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Protein Starvation Correlated with PWS Lab Results and Symptomology
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| Protein Starvation (kwashiorkor and marasmus) | PWS neonates | PWS infants | PWS children | PWS adults |
| hypotonia | yes | yes | yes | yes |
| low muscle mass | yes | yes | yes | yes |
| lethargy | yes | yes | yes | yes |
| failure to thrive | yes | yes | n/a | n/a |
| developmental delays | yes | yes | yes | n/a |
| cognitive impairment | yes | yes | yes | yes |
| behavioral disturbances | n/a | n/a | yes | yes |
| brain imaging abnormalities | ? | yes | yes | yes |
| anorexia/lack of appetite | yes | yes | n/a | n/a |
| reduced saliva secretion | yes | yes | yes | yes |
| increased tendency to hypothermia | yes | yes | yes | yes |
| reduced fever during infectious illness | yes | yes | yes | yes |
| increased tendency to hypoglycemia | yes | yes | yes | yes |
| angular stomatitis | yes | yes | yes | yes |
| hypopigmentation | yes | yes | yes | yes |
| hypoaminoacidemia | yes | ? | ? | ? |
| hypoproteinemia (low serum protein) | yes | ? | ? | ? |
| low serum albumin hypoalbuminemia | yes | yes | ? | ? |
| low BUN (blood urea nitrogen) | yes | ? | ? | ? |
| hyperammonemia | yes | ? | ? | ? |
| increased urinary 4-hydroxyphenylacetate | yes | ? | ? | ? |
| elevated isobutyrylcarnitine and isovalerylcarnitine | yes | ? | ? | ? |
| low creatinine | yes | ? | ? | ? |
| low alkaline phosphatase (ALP) | ? | ? | ? | ? |
| high WBC (white blood cell count) | no (low) | ? | ? | ? |
| anisocytosis (unequally sized RBCs) | yes | ? | ? | ? |
| elevated ghrelin | ? (probable) | yes | yes | yes |
| low serum leptin | ? (probable) | yes | yes | yes |
| low serum IGF-1 | yes | yes | yes | yes |
| low serum IGFBP-3 | ? | yes | yes | yes |
| high basal growth hormone | ? | ? | ? | ? |
| short stature | ? | yes | yes | yes |
| low fasting insulin | ? (probable) | yes | yes | yes |
| impaired glucose tolerance | ? | yes | yes | yes |
| low serum carnitine | ? (probable) | yes | yes | yes |
| hepatic steatosis | ? | ? | yes | ? |
| hepatomegaly | ? | ? | ? | ? |
| impaired fatty acid oxidation | ? (probable) | ? (probable) | ? (probable) | ? (probable) |
| low luteinizing hormone (LH) | ? | ? | yes/variable | yes/variable |
| low growth hormone response to arginine challenge | ? | yes | yes | yes |
| low sodium | no (high) | ? | ? | ? |
| low potassium | no (high) | ? | ? | ? |
| low chloride | no (high) | ? | ? | ? |
| low bicarbonate (total CO2) | no (high) | ? | ? | ? |
| low lymphocyte count | no (high) | ? | ? | ? |
| low insulin response to arginine challenge | ? | ? | ? | ? |
| impaired carbohydrate absorption | ? | ? | ? | ? |
| low fasting glucose | ? | ? | ? | ? |
| urinary loss of fumarate | ? | ? | ? | ? |
| urinary loss of succinate | ? | ? | ? | ? |
| low prealbumin | ? | ? | ? | ? |
| low plasma urea | ? | ? | ? | ? |
| high cortisol | ? | ? | normal | ? |
| low follicle-stimulating hormone (FSH) | ? | ? | no (variable) | no (variable) |
| high prolactin | ? | ? | ? | ? |
| low cholesterol | ? | ? | ? | ? |
| low serum transferrin | ? | ? | ? | ? |
| low serum ferritin | ? | ? | ? | ? |
| low B12 | ? | ? | ? | ? |
| low folate | ? | ? | ? | ? |
| anemia | ? | yes | ? | ? |
| low serum essential fatty acids | ? | ? | ? | ? |
| impaired delta-6-desaturase/linoleic acid metabolism | ? | ? | ? | ? |
| increased interleukin-6 | ? | ? | ? | ? |
Excerpts from eMedicine - marasmus:
Metabolic changes
Energy metabolism: With reduced energy intake, a decrease in physical activity occurs along with a slower and, ultimately, lack of growth. Weight loss first occurs by a decrease in fat mass, then a decrease in muscle mass, as clinically measured by changes in arm circumference (see Image 1). Muscle mass loss results in a decrease of energy expenditure. Reduced energy metabolism can impair the response of patients with marasmus to changes in environmental temperature, resulting in an increased risk of hypothermia. Furthermore, during infection, fever is reduced compared to a well-nourished patient. In case of nutrient deficiency, the metabolism is redirected to vital function (requiring 80-100 kcal/kg/d). During recovery, the energy cost of catch-up growth has to be added (up to 100 kcal/kg/d). At this stage, energy needs can be massive.
Protein metabolism: Intestinal absorption of amino acids is maintained, despite the atrophy of the intestinal mucosa. Protein turnover is decreased (up to 40% in severe forms), and protein-sparing mechanisms regulated by complex hormonal controls redirect amino acids to vital organs. Amino acids liberated from the loss of muscle mass are recycled in priority by the liver for the synthesis of essential protein. Total plasma proteins, including albumin, are decreased, whereas gamma globulins are often increased by the associated infections.
Albumin: An albumin concentration lower than 30 g/L is often considered as the threshold below which edema develops from decreased oncotic pressure. However, in marasmus, albumin concentration can occasionally be below this value without edema. Prealbumin concentration is a sensitive index of protein synthesis. It decreases with decreased protein intake and rapidly increases in a few days with appropriate nutritional rehabilitation. Insulinlike growth factor 1 (IGF-1) is another sensitive marker of nutritional status.
Carbohydrate metabolism: This has mainly been studied in order to explain the serious and often fatal hypoglycemia occurring in the initial renutrition phase of marasmic children. Glucose level is often low initially, and the glycogen stores are depleted. Also, a certain degree of glucose intolerance of unclear etiology exists, possibly associated with a peripheral resistance to insulin or with hypokalemia. In the initiation of renutrition or in association with diarrhea or infection, a significant risk of profound and even fatal hypoglycemia occurs. Small and frequent meals are recommended, including during the night, to avoid death in the early morning. Furthermore, the digestion of starch is impaired by the decreased production of amylase by the pancreas. Lactose malabsorption is frequent but is generally without clinical consequences. In most cases, renutrition using milk is possible.
Fat metabolism: Dietary fats are often malabsorbed in the initial phase of marasmus renutrition. The mobilization of fat stores for energy metabolism takes place under hormonal control by adrenaline and growth hormone. Blood lipid levels are usually low, and serious dysregulation of lipid metabolism can occur, mainly during KW and rarely during marasmus.
Anatomic changes
Digestive tract
The entire digestive tract from mouth to rectum is affected. The mucosal surface is smooth and thin, and secretory functions are impaired. The decrease in gastric hydrochloric acid (HCl) excretion results in bacterial overgrowth in the duodenum. The peristalsis is slow. Proportionally, the digestive tract is the organ system that loses the largest mass during marasmus. However, these important alterations of the digestive tract interfere only moderately with normal nutrient absorption. Therefore, early enteral renutrition is not contraindicated but is encouraged because some of the nutrients necessary for the recovery of the intestinal mucosa are used directly from the lumen.
In addition to the anatomic changes associated with PEM, the frequent intestinal infections by viruses, bacteria, and toxins also contribute to the changes in the digestive tract. Liver volume usually decreases, as do other organ volumes. An enlarged liver suggests the possibility of other diagnoses, such as KW or hepatitis. Liver synthesis function is usually preserved, although protein synthesis is decreased, as reflected by the decreased albumin and prealbumin levels. The neoglycogenesis is decreased, further increasing the risk for hypoglycemia. The detoxifying function of the liver is impaired with structural changes in the liver cells. Therefore, drugs that are metabolized by the liver should be administered with caution, and liver function should be monitored.
Endocrine system
The main perturbations are observed in the thyroid, insulin, and growth hormone system. As in any stressed state, the adrenergic response is activated. This response is functional in marasmus but less so in KW. Muscle proteins are converted into amino acids, used for the hepatic synthesis of lipoproteins. These lipoproteins contribute to the mobilization of triglycerides from the liver. In contrast, during KW, this function is impaired, resulting in liver steatosis, which is not usually present in marasmus. However, any precipitating factor, such as gastroenteritis or inappropriate renutrition, can disrupt this fragile adaptive mechanism.
Furthermore, in serious marasmus, a significant degree of hypothyroidism, with a decrease in the size of the thyroid gland and repercussions on the brain function and psychomotor development exists. In less severe forms, the impaired thyroid function has fewer clinical consequences. Insulin levels are low and contribute to a certain degree of glucose intolerance, especially during KW. Therefore, high-carbohydrate diets are inappropriate. Growth hormone levels are initially within the reference range, but they progressively decrease with time, explaining the halt in linear growth observed with marasmus. After initiation of renutrition, the substantial anabolism results in a rapid linear growth spurt.
Hematopoietic system
A moderate normochromic or slightly hypochromic anemia is usually present, with normal red blood cell size. Iron and folate deficiencies, intestinal parasites, malaria, and other chronic infections exacerbate the anemia. However, iron stores are present in the liver. Therefore, iron supplementation should not be initially implemented. Oral iron is poorly tolerated by the digestive tract. The other blood cells (eg, thrombocytes, white blood cells) are also affected, but with generally limited clinical consequences. Blood clotting mechanisms are usually preserved, except in the case of serious vitamin K deficiency.
Immune system
Immune impairment and infections are usually associated with marasmus. Thymus atrophy is a characteristic manifestation of marasmus, but all T lymphocyte–producing tissues are affected. However, B-lymphocyte tissues, such as Peyeri plaques, the spleen, and the tonsils, are relatively preserved. Cellular immunity is most affected, with a characteristic tuberculin anergy. However, antibody production is maintained. In marasmus, a general acquired immunodeficiency occurs, with a decrease in secretory immunoglobulin A (IgA) and an impairment of the nonspecific local defense system, such as mucosal integrity and lymphokine production. Bacteriemia, candidiasis, and Pneumocystis carinii infection are frequently present. Immune impairment is less frequent with moderate malnutrition. Immunological recovery is generally rapid, except if measles is associated.
Brain and nervous system
Cerebral tissue is usually preserved during marasmus. Brain atrophy with impairment of cerebral functions is only present in severe forms of marasmus. Effects on the brain are more important if malnutrition takes place during the first year of life or during fetal life. Irritability and apathy are characteristic of marasmus, but they improve rapidly with recovery. The permanent developmental consequences of marasmus are difficult to evaluate, but several ongoing studies are evaluating these long-term consequences as well as the benefit of nutritional supplementation with various vitamins and minerals.
Cardiovascular system
Cardiac muscle fiber is thin, and the contractility of the myofibrils is impaired. Cardiac output, especially systolic function, is decreased in the same proportion as the weight loss. Bradycardia and hypotension commonly occur in severe forms of malnutrition. Electrolyte imbalances present during marasmus modify the ECG findings. With this impaired cardiac function, any increase of intravascular volume during rehydration or blood transfusion can result in a significant cardiac insufficiency. With the rapid metabolic, energy, and electrolyte changes of the initial phase of renutrition, this period is also a period of high risk for arrhythmia or cardiac arrest. Therefore, close clinical monitoring is critical in children with circulatory compromise.
Clinical
History: Signs and symptoms of marasmus vary with the importance and duration of the energy deficit, age at onset, associated infections (eg, gastrointestinal infections), and associated nutritional deficiencies (eg, iron deficiency, iodine deficiency). Diets and deficiencies may vary considerably between different geographical regions and even within a country. The AIDS epidemic has also significantly changed the clinical course of classic marasmus. Marasmus is typically observed in infants who are breastfeeding when the amount of milk is markedly reduced or, more frequently, in those who are artificially fed. Failure to thrive is the earliest manifestation, associated with irritability or apathy. Chronic diarrhea is the most frequent symptom, and infants generally present with feeding difficulties. Presentation may be accelerated by an acute infection.
The most perceptible and frequent clinical feature in marasmus is the loss of muscle mass and subcutaneous fat mass. Some muscle groups, such as buttocks and upper limb muscles, are more frequently affected than others. Facial muscles are usually spared longer. Facial fat mass is the last to be lost, resulting, in severe cases, in the characteristic elderly appearance of children with marasmus. Anorexia is frequent and interferes with renutrition. An irritable and whining child who cannot be comforted or separated from the mother demonstrates behaviors often observed with marasmus. Apathy is a sign of serious forms of marasmus: children are increasingly motionless and seem to "let themselves die." In contrast, during rehabilitation, even the slightest smile is a positive sign of recovery. Children's behavior is probably one of the best clinical signs of the severity and evolution of marasmus.
Several clinical signs must be assessed in order to detect complications, with special attention to infectious complications (see checklist below). The physical examination must be very thorough because even small abnormalities can be clinically significant. Clinical signs of serious complication can be very subtle in children with marasmus. A body temperature of 37.5°C can correspond to a fever of 39-40°C in a child without marasmus, and a small cough can be the only sign of a serious pneumonia. After history and physical examination, diagnosing the type and severity of the malnutrition, as well as diagnosing associated infections and complications affecting organs or systems, such as the gastrointestinal, neurological, or cardiovascular system, are critical. This set of diagnoses results in optimal planning of the complementary evaluation and therapeutic strategy.
Drug metabolism during marasmus
Absorption and bioavailability of oral drugs are decreased by the structural and functional changes of the digestive tract. Drug distribution depends on the fluid distribution, organ perfusion, and albumin level and is therefore significantly modified by marasmus. The hepatic metabolism is altered in marasmus; therefore, drugs metabolized in the liver must be used with caution. Renal elimination of drugs is also impaired with the changes in glomerular filtration and tubular secretion. Consequently, patients generally have a decrease of drug elimination, increase in plasmatic concentration, and increase in risk for toxicity. Drug metabolism perturbations improve rapidly with rehabilitation.
See also eMedicine - malnutrition
Clin Biochem. 2007 Feb.
Relationships between leptin, insulin, IGF-1 and IGFBP-3 in children with energy malnutrition.
Haspolat K, Ece A, Gurkan F, Atamer Y, Tutanc M, Yolbas I.
Department of Pediatrics, Dicle University, Medical School, Diyarbakir, Turkey.
Abstract
OBJECTIVES:: Leptin has a key role in energy homeostasis and there may be a link between leptin and insulin-like growth factor-1 (IGF-1) system. The aim of this study was to analyze the relationships between long-lasting insufficient caloric intake (marasmus), leptin and IGF-1 system. DESIGN AND METHODS:: The study group consisted of 30 marasmic children and control group included 28 healthy children. After an overnight fasting; leptin, insulin, IGF-1 and IGFBP-3 levels were measured. RESULTS:: Marasmic children had significantly lower body weight, height, mid-arm circumference (MAC), skinfold thickness, mean serum leptin, insulin, IGF-1 and IGFBP-3 levels compared with healthy subjects (P<0.05). Serum IGF-1 and IGFBP-3 levels were significantly correlated with insulin, MAC and height Z score in patients (P<0.05). In controls, significant positive correlations were found between BMI, IGF-1 and leptin (P<0.05). CONCLUSIONS:: Energy malnutrition is characterized by the important decreases in the leptin, insulin, IGF-1 and IGFBP-3 levels. Understanding details of these changes may lead to new therapeutic approaches in disease states associated with malnutrition.
Endocrinology. 2005 Oct.
Hormonal and metabolic defects in a Prader-Willi syndrome mouse model with neonatal failure to thrive.
Stefan M, Ji H, Simmons RA, Cummings DE, Ahima RS, Friedman MI, Nicholls RD.
Center for Neurobiology and Behavior, Department of Psychiatry, University of Pennsylvania School of Medicine, Philadelphia, USA.
"A number of endocrine abnormalities occur in children and adults with PWS, including GH, IGF-I, and IGF binding protein (IGFBP)-3 deficiencies, and insulin levels are relatively decreased compared with individuals with common obesity."
Clin Biochem. 2004 May.
The evaluation of serum leptin level and other hormonal parameters in children with severe malnutrition.
Kilic M, Taskin E, Ustundag B, Aygun AD.
Department of Pediatrics, Firat University Faculty of Medicine, Elazig, Turkey.
Abstract
OBJECTIVES: Protein-energy malnutrition (PEM) is a clinical problem caused by inadequate intake of one or more nutritional elements, and remains as one of the most important health problems in developing countries. The aim of this study is to determine the relationship among leptin concentrations, body weight and concentrations of some serum hormones, e.g., basal GH, IGF-1, basal cortisol and IGF-BP3, in severe malnourished children, and to determine the effects of leptin in malnourished children. DESIGN AND METHODS: The study group consisted of 36 children diagnosed with PEM. Thirty healthy children were enrolled as the control group. After an overnight fast and before initiation of feedings, fasting venous blood samples were obtained from a forearm vein with needle technique for routine tests, and leptin, IGF-1, IGF-BP3, basal GH and cortisol levels were measured. Tests were carried out in the laboratories of the Department of Biochemistry by commercial kits. RESULTS: Serum leptin levels of infants with marasmus and kwashiorkor were significantly lower than that of the controls (2.09 +/- 0.93 and 2.27 +/- 1.01, 6.82 +/- 2.28 ng/ml, respectively, P < 0.001). However, there was no significant difference between serum leptin levels in children with marasmus and those with kwashiorkor (P > 0.05). Serum IGF-1 and IGF-BP3 levels were significantly lower in malnourished children (P < 0.001, both). Also, basal GH and cortisol levels were significantly higher in malnourished children (P < 0.001, both). There was a positive correlation among serum leptin levels and IGF-1 and IGF-BP3 levels and also a negative correlation among serum leptin levels and basal GH and cortisol levels in children diagnosed with marasmus or kwashiorkor and the control group. CONCLUSIONS: The decrease of energy intake and adipose tissue and serum IGF-1 levels in children with PEM may result in decrease of leptin secretion. Decrease in serum leptin levels may initiate food intake by increasing appetite and stimulating the secretion of cortisol and GH that might increase energy expenditure through an autocrine mechanism. Moreover, serum leptin level may be an important signal to reflect the metabolism of children with PEM.
J Nutr. 2003 Oct.
Fetal and early postnatal protein malnutrition cause long-term changes in rat liver and muscle mitochondria.
Park KS, Kim SK, Kim MS, Cho EY, Lee JH, Lee KU, Pak YK, Lee HK.
Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Korea.
[ Free full text ]
Epidemiologic data suggest a strong association between low birth weight and increased risk of metabolic syndrome in adult life. However, the underlying mechanisms are largely unknown. To test the hypothesis that mitochondrial changes may serve as a link between poor nutrition in early life and insulin resistance in later life, we investigated the effect of protein malnutrition during gestation and lactation on mitochondria of the liver and skeletal muscle. Female offspring of Sprague-Dawley rats fed a low protein diet (casein, 80 g/kg) were randomly divided into two groups and weaned onto either the low protein diet or a control diet (casein, 180 g/kg). As a control group, offspring of rats fed the control diet were weaned onto the control diet. The rats in each group were randomly divided into four groups that were killed at 5, 10, 15 and 20 wk of age. Both mitochondrial DNA content and the expression of mitochondrial DNA-encoded genes in liver and muscle were measured. Mitochondrial transcription factor A and antioxidant enzyme activities were also determined. The mitochondrial DNA content of the liver and skeletal muscle were reduced in fetal and early postnatal malnourished rats even when proper nutrition was supplied after weaning. These changes were accompanied by a decrease in mitochondrial DNA-encoded gene expression; however, they were not dependent on mitochondrial transcriptional factor A. Our findings indicate that poor nutrition in early life causes long-lasting changes in mitochondria
Mediators Inflamm. 2002 Dec.
Pro-inflammatory cytokines in Turkish children with protein-energy malnutrition.
Dulger H, Arik M, Sekeroglu MR, Tarakcioglu M, Noyan T, Cesur Y, Balahoroglu R.
Department of Biochemistry, School of Medicine, University of Yuzuncu Yil, Tip Fakultesi, Biyokimya Anabilim Dali, Maras Caddesi, 65300 Van, Turkey.
Abstract
BACKGROUND: Protein-energy malnutrition (PEM) results from food insufficiency as well as from poor social and economic conditions. Development of PEM is due to insufficient nutrition. Children with PEM lose their resistance to infections because of a disordered immune system. It has been reported that the changes occurring in mediators referred to as cytokines in the immune system may be indicators of the disorders associated with PEM. AIMS: To determine the concentrations of pro-inflammatory cytokines in children with PEM, and to find out whether there was an association with the clinical presentation of PEM. METHODS: The levels of serum total protein, albumin, tumour necrosis factor-alpha, and interleukin-6 were measured in 25 patients with PEM and in 18 healthy children as a control group. PEM was divided into two groups as kwashiorkor and marasmus. The kwashiorkor group consisted of 15 children and the marasmus group consisted of 10 children. RESULTS: Levels of serum total protein and albumin of the kwashiorkor group were significantly lower than both the marasmus group and controls (p < 0.05). In view of tumour necrosis factor-alpha levels, there was no difference between groups (p > 0.05). While levels of interleukin-6 in both the marasmus group and the kwashiorkor group were significantly higher compared with controls (p < 0.05), there was no significant difference between the groups of marasmus and kwashiorkor (p > 0.05). CONCLUSIONS: It was observed that the inflammatory response had increased in children with malnutrition.
Nutrition. 2002 Jan.
Leptin levels and IgF-binding proteins in malnourished children: effect of weight gain.
Palacio AC, Perez-Bravo F, Santos JL, Schlesinger L, Monckeberg F.
Molecular Biology Laboratory and Public Nutrition Unit, Nutrition and Food Technology Institute, Universidad de Chile, Santiago, Chile.
Abstract
OBJECTIVES: Although it is well known that leptin reflects body fat content in adults, the regulation of leptin levels during childhood malnutrition is poorly understood. Insulin-like growth factor I (IGF-I) and the IGF-binding proteins (IGFBPs) may play important roles in the regulation of body composition. We investigated the relation between leptin, IGF-I, and IGFBPs in children with protein-energy malnutrition (PEM; before and after recovering 10% of their initial body weights) in comparison with well-nourished children. METHODS: Fifteen PEM and 16 healthy children were studied on admission and after 10% weight gains in the malnourished group. Leptin was measured with radioimmunoassay, IGF-I and IGFBPs were measured with immunoradiometric assay. RESULTS: Patients with PEM had a significantly lower body mass index and percentage of body fat than did the control children. Before weight gain, leptin, IGF-I, and IGFBP-3 were significantly lower and IGFBP-1 was elevated in the malnourished group compared with the control group. Among PEM patients, after 10% weight gains, the levels of leptin, IGF-I, and IGFBP-3 were significantly higher and IGFBP-1 significantly lower compared with the control group. Leptin correlated significantly with IGF-I in the normal children (r(s) = 0.86, P < 0.005). On admission, no correlation was observed between leptin and IGF-I (r(s) = 0.08, P < 0.16) and between leptin and IGFBP-3 (r(s) = 0.02, P < 0.27) in the malnourished group, but those levels improved after 10% recovery of their body weights (r(s) = 0.47, P < 0.002 and r(s) = 0.42, P < 0.005, respectively). In the PEM group, IGF-I correlated significantly with IGFBP-3 when the children gained weight (before: r(s) = 0.006, P < 0.31; after: r(s) = 0.32, P < 0.01). Our study showed results similar to those of anorexia nervosa studies, but the normalization of study variables was obtained in considerable less time for the same weight gain. CONCLUSIONS: The main finding of this study was that, after refeeding with only a 10% weight gain, the PEM children normalized their leptin, IGF-I, and IGFBP-3 levels. These results provide evidence that leptin can function as link between this hormonal response and improved nutrition status.
Neuro Endocrinol Lett. 2001 Oct.
The relationship between neuropeptides and hormones in starvation.
Baranowska B, Chmielowska M, Wolinska-Witort E, Roguski K, Wasilewska-Dziubinska E.
Neuroendocrinology Department, Medical Centre of Postgraduation Education, Warsaw, Poland.
OBJECTIVES: Some hormonal disturbances were demonstrated in starvation. Leptin, NPY and galanin play an important role in the control of appetite and in the mechanism of hormone release. METHODS: In order to evaluate the effect of starvation on the relationship between leptin, neuropeptide Y (NPY) galanin and pituitary and gonadal hormones release, plasma leptin, NPY and galanin as well as serum LH, FSH, prolactin (PRL), estradiol, progesterone levels in non-starved female rats (in diestrus) and after 72 hrs of starvation were measured with RIA methods. Effects of leptin, NPY and galanin administration on pituitary and gonadal hormones were investigated in vivo and in vitro experiments. RESULTS: Plasma leptin, NPY and galanin as well as serum estradiol and progesterone concentrations were significantly lower in starved rats as compared with non-starved rats. However serum prolactin level was significantly higher in starved rats. Opposite effects after leptin and NPY administration on hormone release in vivo and in vitro experiments were observed in non-starved rats. However, in starved rats we did not find changes in pituitary and gonadal hormones release after leptin, NPY and galanin injection or the hormonal response was blunted. CONCLUSIONS: 1) The disturbances in neuropeptides activity and in hormones release were observed in starvation. 2) Leptin, NPY and galanin have direct and indirect effects on pituitary and gonadal hormones release. 3) In starvation the hormonal response to leptin, NPY and galanin is impaired.
Pediatrics. 2001 Apr.
Severe nutritional deficiencies in toddlers resulting from health food milk alternatives.
Carvalho NF, Kenney RD, Carrington PH, Hall DE.
Scottish Rite Pediatric and Adolescent Consultants, Childrens Healthcare of Atlanta, Atlanta, Georgia, USA.
Abstract: It is widely appreciated that health food beverages are not appropriate for infants. Because of continued growth, children beyond infancy remain susceptible to nutritional disorders. We report on 2 cases of severe nutritional deficiency caused by consumption of health food beverages. In both cases, the parents were well-educated, appeared conscientious, and their children received regular medical care. Diagnoses were delayed by a low index of suspicion. In addition, nutritional deficiencies are uncommon in the United States and as a result, US physicians may be unfamiliar with their clinical features. Case 1, a 22-month-old male child, was admitted with severe kwashiorkor. He was breastfed until 13 months of age. Because of a history of chronic eczema and perceived milk intolerance, he was started on a rice beverage after weaning. On average, he consumed 1.5 L of this drink daily. Intake of solid foods was very poor. As this rice beverage, which was fallaciously referred to as rice milk, is extremely low in protein content, the resulting daily protein intake of 0.3 g/kg/day was only 25% of the recommended dietary allowance. In contrast, caloric intake was 72% of the recommended energy intake, so the dietary protein to energy ratio was very low. A photograph of the patient after admission illustrates the typical features of kwashiorkor: generalized edema, hyperpigmented and hypopigmented skin lesions, abdominal distention, irritability, and thin, sparse hair. Because of fluid retention, the weight was on the 10th percentile and he had a rotund sugar baby appearance. Laboratory evaluation was remarkable for a serum albumin of 1.0 g/dL (10 g/L), urea nitrogen <0.5 mg/dL (<0.2 mmol/L), and a normocytic anemia with marked anisocytosis. Evaluation for other causes of hypoalbuminemia was negative. Therapy for kwashiorkor was instituted, including gradual refeeding, initially via a nasogastric tube because of severe anorexia. Supplements of potassium, phosphorus, multivitamins, zinc, and folic acid were provided. The patient responded dramatically to refeeding with a rising serum albumin and total resolution of the edema within 3 weeks. At follow-up 1 year later he continued to do well on a regular diet supplemented with a milk-based pediatric nutritional supplement. The mortality of kwashiorkor remains high, because of complications such as infection (kwashiorkor impairs cellular immune defenses) and electrolyte imbalances with ongoing diarrhea. Children in industrialized countries have developed kwashiorkor resulting from the use of a nondairy creamer as a milk alternative, but we were unable to find previous reports of kwashiorkor caused by a health food milk alternative. We suspect that cases have been overlooked. Case 2, a 17-month-old black male, was diagnosed with rickets. ...
Metabolism. 2000 Jul.
Serum leptin concentrations during severe protein-energy malnutrition: correlation with growth parameters and endocrine function.
Soliman AT, ElZalabany MM, Salama M, Ansari BM.
Department of Child Health, University of Alexandria, Egypt.
Abstract: Circulating leptin, insulin, insulin-like growth factor-I (IGF-I), cortisol, and albumin concentrations and the growth hormone (GH) response to provocation were measured in 30 children with severe protein-energy malnutrition (PEM), 20 with marasmus and 10 with kwashiorkor, as well as 10 age-matched normal children (body mass index [BMI] >50th and <90th percentile for age and sex) and 10 prepubertal obese children (BMI >95th percentile for age and sex). Patients with PEM had a significantly lower BMI, midarm circumference (MAC), and skinfold thickness (SFT) compared with the age-matched control group. Basal cortisol and GH concentrations were significantly higher in the malnourished groups versus controls. Leptin and IGF-I were significantly lower in the marasmic and kwashiorkor groups versus normal children. Fasting insulin levels were significantly decreased in the kwashiorkor group compared with marasmic and normal children. The BMI correlated significantly with leptin (r = .77, P < .001), basal insulin (r = .61, P < .001), and IGF-I (r = .77, P < .001) and negatively with basal GH (r = -.52, P < .001). These findings suggest that during prolonged nutritional deprivation, the decreased energy intake, diminished subcutaneous fat mass, and declining insulin (and possibly IGF-I) concentration suppress leptin production. In support of this view, serum leptin levels were positively correlated with triceps, scapular, and abdominal SFT (r = .763, .75, and .744, respectively, P < .0001) in all of the children. Moreover, basal insulin and circulating IGF-I were correlated significantly with leptin concentrations (r = .47 and .62, respectively, P < .001). Basal levels of cortisol and GH were significantly elevated in the 2 groups with severe PEM. It is suggested that low leptin levels can stimulate the hypothalamic-pituitary-adrenal (HPA) axis and possibly the hypothalamic-pituitary-GH axis to maintain the high cortisol and GH levels necessary for effective lipolysis to ensure a fuel (fatty acids) supply for the metabolism of brain and peripheral tissue during nutritional deprivation. In summary, during prolonged PEM, the decreased synthesis of IGF-I and the low level of insulin and/or its diminished effect due to an insulin-resistant status in the presence of high circulating GH and cortisol levels ensure substrate diversion away from growth toward metabolic homeostasis. Leptin appears to be an important signal in the process of metabolic/endocrine adaptation to prolonged nutritional deprivation.
J Trop Pediatr. 1999 Oct.
Plasma and urine carnitine levels and carnitine supplementation in children with malnutrition.
Alp H, Orbak Z, Akcay F, Tan H, Aksoy H.
Department of Pediatrics, Ataturk University Medical Faculty, Erzurum, Turkey.
Abstract: The purpose of the present investigation was to determine serum and urinary carnitine levels in children suffering from protein-energy malnutrition (PEM) before and after dietary treatment and carnitine supplementation, and to compare them with those in healthy children. Plasma and urine carnitine levels were lower in patients with marasmus and kwashiorkor than in controls. There was no statistical difference between groups with and without carnitine supplementation on the first day. On the fifth day, in groups receiving carnitine supplementation, plasma and urine carnitine levels were significantly higher than in groups without supplementation (p < 0.01). On the 15th day there was no statistical significance between groups with PEM and controls.
East Afr Med J. 1999 Oct.
Serum free carnitine levels in children with kwashiorkor.
Aseo I, Tindimwebwa G, Agu E, Iputo JE.
Department of Paediatrics, University of Transkei Medical School, Umtata, South Africa.
Abstract
OBJECTIVE: To determine the serum free carnitine concentration in normally nourished children and in children with kwashiorkor and to relate the carnitine concentration to the ability to oxidise exogenous long chain fatty acids in the body. STUDY DESIGN: A cross-sectional comparative study of two age-matched groups. SUBJECTS: Forty seven children with kwashiorkor and 47 age-matched normally nourished children. MAIN OUTCOME MEASURES: Fasting blood samples were enzymatically analysed for free carnitine levels. 13C labelled hiolein was administered orally and the recovery of 13C from the breath air was monitored after administration of the feed. The cumulative per cent dose (CUMPD) recovery of 13C 16 hours after the ingestion of labelled hiolein was determined. RESULTS: Normal children had significantly higher free carnitine concentrations (mean = 60.7 mumol/l; 95% confidence interval of the mean = 42.7-77.8) than the kwashiorkor children (mean = 16.5 mumol/l; 95% confidence interval of the mean = 11.3-19.8)(p < 0.001). There was no correlation between serum free carnitine concentration and serum albumin in kwashiorkor subjects, but there was a significant correlation between serum free carnitine concentration and the degree of weight loss as indicated by the weight: weight for age and sex ratio. The greater the weight loss, the lower the serum carnitine concentration amongst the kwashiorkor children (r = 0.46; p < 0.01). There was a linear relationship between serum free carnitine and hiolein oxidation (r = 0.89; p < 0.001). CONCLUSION: There is carnitine deficiency in kwashiorkor, and that the impaired lipid oxidation in kwashiorkor is related to this deficiency.
J Trop Pediatr. 1999 Apr.
Plasma fatty acids in children with grade III protein-energy malnutrition in its different clinical forms: marasmus, marasmic kwashiorkor, and kwashiorkor.
Franco VH, Hotta JK, Jorge SM, dos Santos JE.
Department of Pediatrics, Faculty of Medicine of Ribeirao Preto, Sao Paulo, Brazil.
Abstract: Plasma fatty acid patterns were analysed in 15 children aged 2 to 42 months (median = 12 months) admitted to the University Hospital, Faculty of Medicine of Ribeirao Preto, University of Sao Paulo, suffering from grade III protein-energy malnutrition in its different clinical forms: marasmus (n = 5), marasmic kwashiorkor (n = 5), and kwashiorkor (n = 5). A control group of eight children from the same population aged 3 to 22 months (median = 12 months) was also studied. Plasma fatty acids were analysed by gas liquid chromatography. The plasma fatty acid patterns detected in grade III protein-energy malnutrition indicated changes characteristic of essential fatty acid deficiency, with lower linoleic acid (C18:2) levels in the three groups (p < 0.05). The major metabolite of linoleic acid, arachidonic acid (C20:4), was lower in the kwashiorkor group than in the control group (p < 0.05). Linoleic acid (C18:2) levels were lower in the marasmus group than in the kwashiorkor group (p < 0.05). Several factors may have contributed to these changes, such as a decreased content of linoleic acid in the diet, plus malabsorption and/or poor utilization of fats, and changes in the synthesis or degradation of these fatty acids. Although many questions remain unanswered, we suggest that an adequate amount of linoleic acid be added to the rehabilitation diet of these children in the form of vegetable fat.
Pediatr Res. 1998 Sep.
Urinary organic acids in infant malnutrition.
Teran-Garcia M, Ibarra I, Velazquez A.
Unidad de Genetica de la Nutricion, Instituto de Investigaciones Biomedicas, Universidad Nacional Autonoma de Mexico, Mexico City.
Abstract: The metabolic derangements in severe protein-energy malnutrition (PEM) are only partially known, due to the limitations of blood collection in these patients. Urinary excretion of organic acids was studied by gas chromatography-mass spectrometry in 39 infants with four types of PEM: 1) upon hospital admission, as soon as eventual infections had been cleared, and salt and water deficits corrected, but before oral feeding was started; 2) after start of protein alimentation; 3) on the day of discharge. All of the patients showed an increased excretion of various organic acids at some point of their hospital stay, regardless of the clinical type of PEM. In nearly half of the malnourished children, results were suggestive of blocks in the pathways of propionate (15.4% with increased methylmalonate and 25.6% with 2-methylcitrate), of fatty acid beta-oxidation (30.8% with raised dicarboxylic acids with low or low normal 3-hydroxybutyrate), or of both pathways (12.8%). These abnormalities may have been caused by cofactor deficiencies (biotin, vitamin B12, riboflavin, carnitine, niacin). Dicarboxylic acids were excreted in high amounts since the initial sample, probably due to increased mobilization of fatty acids. Increased 2-methylcitrate and methylmalonate excretion was observed more frequently once patients started to be orally fed. The accumulation of potentially toxic acyl-CoA precursors of these compounds could contribute to the known clinical worsening of some malnourished infants after suddenly increased protein intake. Other less specific metabolites, such as 3-hydroxybutyrate, lactate, 4-hydroxyphenyllactate, fumarate, succinate, and 4-hydroxyphenylacetate, were also abnormally excreted in some patients. The analysis of urinary organic acids provides a new approach for the metabolic study of PEM and may have diagnostic and therapeutic implications.
J Trop Pediatr. 1998 Jun.
Blood sugar and serum insulin response in protein-energy malnutrition.
Das BK, Ramesh J, Agarwal JK, Mishra OP, Bhatt RP.
Department of Pediatrics, Banaras Hindu University, Varanasi, India.
Abstract: Blood sugar and serum insulin levels in the fasting state and following an oral glucose load in children with protein-energy malnutrition (PEM) were studied. Twenty-nine children with PEM (15 marasmus, 7 kwashiorkor, and 7 marasmic kwashiorkor) and eight healthy children aged between 6 and 60 months were the subjects of the study. Fasting samples were collected after a 6 h fast. Post-glucose samples were collected after an oral glucose load of 1.75 g/kg. Serum insulin was estimated by radioimmunoassay and glucose by the glucose oxidase method. In malnourished children, the mean fasting blood glucose levels were significantly lower. Two hours following an oral glucose load, only marasmus and marasmic kwashiorkor patients showed significantly higher blood glucose levels. Similarly, the fasting serum insulin levels were significantly lower in malnourished children. Two hours after the oral glucose load, serum insulin levels increased significantly in malnourished children but fell well short of the control values. The insulin:glucose ratio was consistently low in all cases but was more marked in PEM patients, both in the basal state as well as oral glucose loading
Clin Chim Acta. 1996 Aug 30.
Acylcarnitine removal in a patient with acyl-CoA beta-oxidation deficiency disorder: effect of L-carnitine therapy and starvation.
Fontaine M, Briand G, Vallee L, Ricart G, Degand P, Divry P, Vianey-Saban C, Vamecq J.
Laboratoire de Biochimie, Hopital Huriez, Centre Hospitalo-Universitaire de Lille, France.
Carnitine levels and acylcarnitine profiles in a patient with mild multiple acyl-CoA dehydrogenase deficient beta-oxidation were compared with control results. Whereas blood and urine total carnitine levels were moderately decreased, blood esterified carnitine levels in the patient were about 2-fold higher than in controls. Urinary acylcarnitine profiles presented with a larger variety of carnitine esters than in controls and included propionylcarnitine, butyrylcarnitine, 2-methylbutyrylcarnitine, hexanoylcarnitine and octanolycarnitine. Total carnitine levels in body fluids were similarly affected by chronic oral L-carnitine administration in patient and controls. By contrast, esterified carnitine level increase was 2-fold more important in controls than in patient. Whereas no qualitative changes in urinary acylcarnitine profiles were induced by L-carnitine therapy in controls, several alterations of these profiles were observed in the patient. The effect of starvation on metabolites was also studied, especially beta-oxidation rates assessed by free fatty acids to 3-hydroxybutyric acid ratios in blood from the patient in the untreated and L-carnitine treated states. In the L-carnitine-supplemented patient, the effect of starvation on the time course of carnitine levels and acylcarnitine profiles could also be documented. The ability of chronic oral L-carnitine administration to remove relatively less important amounts of acylcarnitines in the patient than in controls is further discussed, as well as qualitative alterations of acylcarnitine profiles induced by this therapy in the pathological condition.
Metabolism. 1996 Feb.
Distinction of dicarboxylic aciduria due to medium-chain triglyceride feeding from that due to abnormal fatty acid oxidation and fasting in children.
Tserng KY, Griffin RL, Kerr DS.
Medical Research Service, Veterans Affairs Medical Center, Cleveland, OH, USA.
Increased amounts of dicarboxylic acids are excreted in human urine under conditions of medium-chain triglyceride (MCT) feeding, abnormal fatty acid oxidation (FAO) and fasting. Criteria to distinguish dicarboxylic aciduria originating from MCT feeding and other conditions are needed in urinary organic acid profiling for detecting inborn errors of metabolism. Patterns of dicarboxylic aciduria in children under various conditions were compared. The relative amounts of medium-chain saturated dicarboxylic acids in urine are not reliable for identifying MCT-induced dicarboxylic aciduria. On the other hand, low ratios of unsaturated to saturated dicarboxylic acids (<0.1) and 3- hydroxydecenedioic to 3-hydroxydecanedioic acids were found to be useful in identifying dicarboxylic aciduria due to MCT ingestion. Additional unique features of dicarboxylic aciduria from MCT are low ratios of 3-hydroxydodecanedioic to 3-hydroxydecanedioic acid (<0.14) and 3-hydroxyadipic to adipic acid (<0.02).
Indian Pediatr. 1995 Mar.
Serum amino acids and genesis of protein energy malnutrition.
Phadke MA, Khedkar VA, Pashankar D, Kate SL, Mokashi GD, Gambhir PS, Bhate SM.
Department of Pediatrics, B.J. Medical College, Pune.
Abstract: Twenty four patients of classical marasmus and kwashiorkor along with equal number of healthy controls were selected for the study. Their serum amino acid patterns analysis revealed a mean ratio of glutamate to alanine in fasting samples of normal individuals to be 0.33, while it as 9.3 in kwashiorkor and 1.6 in marasmus. This differences in controls, kwashiorkor and marasmus was statistically significant. This observation may explain evolution of marasmus and kwashiorkor in children with similar diets. On the basis of the present observation it is postulated that in kwashiorkor, the conversion of pyruvate to alanine in presence of glutamate, an aminogroup donor does not proceed normally, resulting in accumulation of glutamate and low alanine. Thus the development of marasmus and kwashiorkor may not be related to dietary inadequacy alone but also to the transaminase function. This could be genetic in origin.
Journal of Dental Research. 1994.
Saliva composition in Indian children with chronic protein-energy malnutrition.
I. Johansson, M. Lenander-Lumikari and A. K. Saellstrom
Department of Cariology, University of Umea, Sweden.
The composition of paraffin-stimulated and unstimulated whole saliva was compared between two groups of 8-12-year-old Indian children-one group with severe to moderate chronic protein-energy malnutrition (PEM group) and an age- and sex-matched control group with normal protein status or mild PEM. The classification of PEM was based on anthropometric measurements compared with Indian standards. Stimulated saliva was analyzed for the following variables: electrolytes (Na+, K+, Ca2+, Cl-, and PO4(3-)), total protein, hexosamines, fucose, sialic acid, and amylase. Unstimulated saliva samples were analyzed for total protein, salivary and myeloperoxidase, thiocyanate, lactoferrin, lysozyme, a bacteria-agglutinating protein (BAGP), total IgG, total IgA, and specific anti-S. mutans IgA. The results show that the PEM group had a reduced secretion rate of stimulated but not unstimulated saliva. Further, the Ca2+ and Cl- concentrations in stimulated saliva were significantly lower in the PEM group compared with the control group, but the other electrolyte levels were similar. No differences were found in total protein concentration or glycoprotein bound carbohydrates in stimulated saliva between the two groups, but the quantity of total protein secreted per min was reduced by 20% in the PEM group. Significantly lower levels of lactoferrin, BAGP, and anti-S mutans IgA were found in unstimulated saliva from children in the PEM group, but significantly higher levels of total IgG. We conclude that children with severe or moderate PEM, who have reduced secretion rate, buffer capacity, lower Ca2+, and protein secretion in stimulated saliva, also have impaired immunological and agglutinating defense factors in unstimulated saliva.
J Inherit Metab Dis. 1993.
Marked elevation of urinary 3-hydroxydecanedioic acid in a malnourished infant with glycogen storage disease, mimicking long-chain L-3-hydroxyacyl-CoA dehydrogenase deficiency.
Bergoffen J, Kaplan P, Hale DE, Bennett MJ, Berry GT.
Division of Human Genetics, Children's Hospital of Philadelphia, PA.
An infant with glycogen storage disease and prolonged malnourishment showed a urinary organic acid profile during an episode of fasting hypoglycaemia with inappropriate hypoketotic dicarboxylic aciduria that was indistinguishable from that reported in long-chain L-3-hydroxyacyl-CoA dehydrogenase deficiency. Although there was a striking elevation of urinary 3-hydroxydecanedioic acid, the ratios between hydroxydicarboxylic acids were consistent with values reported to be indicate of medium-chain acyl-CoA dehydrogenase deficiency. We suspect that the fasting 3-hydroxydicarboxylic aciduria was attributable to secondarily impaired enzyme activities, the consequence of malnutrition, early infancy, and/or glycogen storage disease. Caution is advised in the interpretation of urinary organic acid patterns that indicate a 3-hydroxydicarboxylic aciduria, as well as an inappropriate hypoketotic dicarboxylic aciduria, as they may represent non-specific findings.
East Afr Med J. 1992 Aug.
Plasma biochemical parameters in Nigerian children with protein energy malnutrition.
Taiwo OO, Thomas KD.
Department of Paediatrics, Faculty of Health Sciences, Obafemi Awolowo University, Ile-Ife, Nigeria.
Abstract: Eleven plasma biochemical parameters were estimated in a total of 28 children with protein-energy malnutrition (PEM): 7 children each category of marasmus, kwashiorkor, marasmic-kwashiorkor and undernutrition with ages between 8 and 48 months. The estimations were performed on admission and 8 to 24 days after treatment at the Obafemi Awolowo University Teaching Hospitals Complex, Nigeria. Plasma sodium, potassium, chloride, bicarbonate and albumin levels were significantly (p < 0.05) higher after treatment than on admission. Calcium however, showed no significant change. Total protein and cholesterol were significantly (p < 0.05) raised after treatment for all the PEM types except undernutrition and kwashiorkor respectively. Globulin, urea and creatinine were significantly (p < 0.05) raised after treatment for kwashiorkor. These biochemical findings support the claims of clinical improvement in PEM children after a minimum of 18 days of treatment at the OAUTHC in Nigeria.
PIP: 11 plasma biochemical parameters were estimated in 28 children aged between 8 and 48 months with protein-energy malnutrition (PEM): 7 children each in the categories of marasmus, kwashiorkor, marasmic-kwashiorkor, and undernutrition. 8 ml of blood was obtained from each of the 7 children with marasmus, kwashiorkor, marasmic-kwashiorkor, and undernutrition, respectively, on admission to the children's ward of the Obafemi Awolowo University Teaching Hospital Complex (OAUTHC), Ile-Ife, Nigeria, and put into lithium heparinized tubes. The children were hospitalized for 18 and 24 days and fed the diet for PEM in the OAUTHC. 8 ml of blood was again taken from the children just before they were discharged. The percentage of recovery weight ranged between 74.4 and 86.4. Analysis of variance showed significance (p 0.05) for all types of PEM for plasma sodium, bicarbonate, urea, total protein, albumin, and globulin. The levels of plasma electrolytes comprising potassium, sodium, chloride, and bicarbonate were significantly (p 0.05) higher after recovery for all 4 types of PEM. There was also a significant (p 0.05) increase in plasma albumin levels in all 4 types of PEM. Plasma total protein levels were significantly increased in all types of PEM except undernutrition. The plasma globulin fraction which contains immune elements for body defense may be more tenaciously retained in PEM than albumin and only kwashiorkor showed a significant (p 0.05) increase after treatment. Plasma urea levels were significantly (p 0.05) higher after treatment than before for marasmus and kwashiorkor, the most protein deficient states in PEM. Plasma creatinine levels after treatment were significantly (p 0.01) lower for marasmic-kwashiorkor after treatment than on admission. The observed rise in kwashiorkor may reflect the increase in muscle formation and activity, while its decrease in marasmic-kwashiorkor possibly reflects increased renal function and efficiency.
Biochim Biophys Acta. 1992 Feb 20.
Formation and degradation of dicarboxylic acids in relation to alterations in fatty acid oxidation in rats.
Mortensen PB.
Department of Medicine A, Rigshospitalet, University of Copenhagen, Denmark.
Dicarboxylic acids are excreted in urine when fatty acid oxidation is increased (ketosis) or inhibited (defects in beta-oxidation) and in Reye's syndrome. omega-Hydroxylation and omega-oxidation of C6-C12 fatty acids were measured by mass spectrometry in rat liver microsomes and homogenates, and beta-oxidation of the dicarboxylic acids in liver homogenates and isolated mitochondria and peroxisomes. Medium-chain fatty acids formed large amounts of medium-chain dicarboxylic acids, which were easily beta-oxidized both in vitro and in vivo, in contrast to the long-chain C16-dicarboxylic acid, which was toxic to starved rats. Increment of fatty acid oxidation in rats by starvation or diabetes increased C6:C10 dicarboxylic acid ratio in rats fed medium-chain triacylglycerols, and increased short-chain dicarboxylic acid excretion in urine in rats fed medium-chain dicarboxylic acids. Valproate, which inhibits fatty acid oxidation and may induce Reye like syndromes, caused the pattern of C6-C10-dicarboxylic aciduria seen in beta-oxidation defects, but only in starved rats. It is suggested, that the origin of urinary short-chain dicarboxylic acids is omega-oxidized medium-chain fatty acids, which after peroxisomal beta-oxidation accumulate as C6-C8-dicarboxylic acids. C10-C12-dicarboxylic acids were also metabolized in the mitochondria, but did not accumulate as C6-C8-dicarboxylic acids, indicating that beta-oxidation was completed beyond the level of adipyl CoA.
Turk J Pediatr. 1992 Jan-Mar.
Effects of cortisol and growth hormone on the metabolism of liver and bone in children with malnutrition.
Guler A, Sapan N, Salantur E.
Department of Biochemistry, Uludag University Faculty of Medicine, Bursa.
Abstract: A protein-energy deficit produces stress in the organism affecting all systems. Proportional to the degree of disease, cortisol and GH are mostly responsible for some of these effects. To investigate the effects on liver and bone, cortisol, GH, AST, ALT, ALP activities and Ca(T) and P(i) in serum were measured in 21 marasmus, nine kwashiorkor and 34 control children. In the marasmus group, we found a positive correlation between cortisol and AST, ALT and Ca(T) and a negative correlation between cortisol and ALP. In the kwashiorkor group there were positive correlations between the same parameters, although, they were of a lesser degree. Furthermore, in the kwashiorkor group we established a positive correlation between GH and ALP. Cortisol stimulates transaminases directly and suppresses ALP activity, thus indirectly increasing Ca(T), whereas GH has no direct effect on these enzymes. As the disease progresses and as liver functions deteriorate, AST, ALT and ALP increase in serum.
Crit Rev Neurobiol. 1991.
Neurological consequences of protein and protein-calorie undernutrition.
Chopra JS.
Department of Neurology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Abstract: Malnutrition is a worldwide problem of enormous magnitude. The growth of the central nervous system in human beings is retarded in case of malnutrition in the very early part of life. Likewise, the peripheral nerves in infants and children and young growing animals appear susceptible to nutritional deprivation including protein as well as protein-calorie deficiency. Motor weakness, hypotonia, and hyporeflexia in infants and children are the essential clinical neurological signs in protein-calorie malnutrition (PCM). Motor and sensory nerve conduction are significantly impaired in children with PCM as well as in animals subjected to protein or protein-calorie deficiency. Histological studies have revealed reduced diameter of myelinated nerve fibers, retardation of myelination, segmental demyelination and remyelination, axonal degeneration, and shortened longitudinal growth of internodes. Diffusion barrier by perineurium may be broken. There is reduction in myelin lipids and impaired synthesis of myelin as shown by the biochemical and radioisotope incorporation studies. Presence of cholesterol esters in the biochemical synthesis of nerves suggests degeneration changes. Experimental studies have revealed that most effects of PCM on peripheral nerves can be reversed by nutritional rehabilitation, although complete recovery in the sensory nerve action potential, fiber size of dorsal nerve roots, and myelin-specific lipids does not occur. Skeletal muscle also shows many changes including muscle fiber atrophy, reduction in duration and amplitude of motor unit potentials, and/or fibrillation on electromyography (EMG) and biochemical estimation of muscle enzymes. These changes may be the reflection of a direct effect of PCM on muscles or secondary to the abnormal structural or biochemical changes in the peripheral nerves. PCM affects the central nervous system, especially the neuropsychological functions, in a lasting manner. Learning deficits and impairment of manual dexterity are the most obtrusive features. Neurotransmitter abnormalities and maturation lag in electroencephalogram have been demonstrated in experimental animals. Spinal cord dysfunction sometimes manifests overtly as clinical myelopathy. Degenerative changes in the cerebellum have been noted.
Metabolism. 1990 Aug.
Fatty acid composition of erythrocyte membrane lipid obtained from children suffering from kwashiorkor and marasmus.
Vajreswari A, Narayanareddy K, Rao PS.
National Institute of Nutrition, Indian Council of Medical Research, Jamai Osmania, Hyderabad.
Abstract: The fatty acid composition of erythrocyte membrane (EM) lipids obtained from normal, kwashiorkor, and marasmic children was analyzed by gas chromatography. The proportion of palmitic acid (16:0) was lower and of oleic acid (18:1) higher in the kwashiorkor group than in the control group. The marasmic group showed lower proportions of eicosatrienoic acid (20:3) and arachidonic acid (20:4) and a higher proportion of oleic acid (18:1) than the control group. A significant difference was found between the marasmic and kwashiorkor groups with respect to arachidonic acid (20:4), which showed a lower proportion in the former group than the latter. The ratio of arachidonic acid to linoleic acid (20:4/18:2) was markedly lower in the marasmic group than the control group, suggesting a possible impairment in the conversion of linoleic acid to arachidonic acid in marasmic children. The ratio of unsaturated fatty acids to saturated fatty acids was markedly elevated in the kwashiorkor group over that of control group, indicating increased fluidity of EM in kwashiorkor. It is suggested that the altered membrane fatty acid composition reflects deranged lipid metabolism and affects the physical and physiological properties of EM and could contribute to changes in the activities of several red blood cell membrane-bound enzymes reported earlier in kwashiorkor children.
Trop Geogr Med. 1989 Jan.
Serum growth hormone, insulin and blood sugar responses to oral glucose in protein energy malnutrition.
Garg SK, Marwaha RK, Ganpathy V, Sialy R, Thapa BR, Walia BN, Dash RJ.
Department of Experimental Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Abstract: Blood sugar (BS), serum immunoreactive insulin (IRI) and growth hormone (GH) were measured in 5 normal children and 15 with varying grades of protein energy malnutrition (PEM) during a standard oral glucose tolerance test (OGTT). Impaired glucose tolerance was noted in grade III and IV PEM but not in grade II. Serum insulin responses were low but appropriate to blood glucose in grade II and grade III PEM and were lower (p less than 0.001) in Grade IV PEM subjects. Higher basal GH was observed in most cases of grade III and IV PEM (p less than 0.001) and the elevated GH was not suppressed following the oral glucose load. Further, a paradoxical rise in GH during OGTT was seen in 3 of the 5 subjects with grade-IV PEM.
J Clin Invest. 1988 Nov.
Binding of straight-chain saturated dicarboxylic acids to albumin.
Tonsgard JH, Mendelson SA, Meredith SC.
Department of Pediatrics, University of Chicago, Illinois.
Dicarboxylic acids are prominent features of several diseases, including Reye's syndrome. Long-chain dicarboxylic acids have profound effects on the function and structure of isolated mitochondria, suggesting that they could contribute to the mitochondrial dysfunction in Reye's syndrome. Binding of fatty acids to albumin and the intracellular fatty acid-binding proteins is important in regulating the transport and metabolism of fatty acids and protects against the toxic effects of unbound fatty acids. We studied the binding of dicarboxylic acids to defatted albumin using equilibrium dialysis to assess to what extent dicarboxylic acids are likely to be bound in the plasma of patients. Dicarboxylic acids bind weakly to albumin in a molar ratio of 3.8, 4.2, 1.6, 0.8, and 0.7 to 1 for octadecanedioic, hexadecanedioic, tetradecanedioic, dodecanedioic, and decanedioic acid, respectively. The dissociation constants for long-chain dicarboxylic acids are 100-1,000-fold larger than those of comparable monocarboxylic acids. Oleate competes with dicarboxylic acid and reduces the moles of dicarboxylic acid bound per mol of albumin to less than 1. Octanoate inhibits dicarboxylic acid binding. Our observations indicate that in Reye's syndrome, substantial concentrations of dicarboxylic acids of patients may be free and potentially toxic to mitochondria and other cellular processes.
Ann Trop Paediatr. 1987 Sep.
Serum carnitine in children with kwashiorkor.
Hammond KD, Tobiansky R, Abrahams OL.
Department of Medical Biochemistry, University of the Witwatersrand Medical School, Johannesburg, South Africa.
Abstract: Concentrations of free and acylcarnitine were measured in serum of children with kwashiorkor and compared with those obtained for well nourished children of similar age. The mean values (S.E.) for both free and acylcarnitine were significantly lower in the kwashiorkor group [32.6 (6.2) and 8.1 (2.2), respectively] than in the controls [53.2 (2.9) and 13.8 (3.1), respectively]. Serum albumin was also low in kwashiorkor patients, but there was no significant correlation with carnitine values.
Pediatr Res. 1986 Nov.
Serum insulin-like growth factors I and II concentrations and growth hormone and insulin responses to arginine infusion in children with protein-energy malnutrition before and after nutritional rehabilitation.
Soliman AT, Hassan AE, Aref MK, Hintz RL, Rosenfeld RG, Rogol AD.
Abstract: Serum insulin, growth hormone (GH), insulin-like growth factors (IGFs) I and II, cortisol, and albumin concentrations were measured in 15 children with kwashiorkor, 15 with marasmic-kwashiorkor, and 21 with marasmus, before and in the survivors, after nutritional rehabilitation, as well as in 10 underweight and eight normal Egyptian children. We also evaluated arginine-induced insulin and GH secretion. IGF-I concentrations were reduced in the three severely malnourished groups (0.07 +/- 0.03, 0.05 +/- 0.03, and 0.09 +/- 0.09 U/ml, respectively) but returned to normal after refeeding. IGF-II concentrations were low in the kwashiorkor (175 +/- 79 ng/ml), marasmic-kwashiorkor (111 +/- 57 ng/ml), and marasmic children (128 +/- 70.9 ng/ml) and returned to normal after nutritional rehabilitation. Basal GH levels were high in the three severely malnourished groups (21.9, 28.8, and 16.6 ng/ml, respectively) and returned to normal after refeeding (8.1, 6.5, and 6.0 ng/ml, respectively). GH responses to arginine were depressed in the three malnourished groups and improved significantly in marasmic-kwashiorkor and marasmic children after nutritional rehabilitation. Insulin responses to arginine were impaired in kwashiorkor, and marasmic-kwashiorkor children and improved significantly after refeeding. IGF-I levels correlated significantly with percent of expected weight (r = 0.52, p less than 0.001), percent of expected height (r = 0.42, p less than 0.001), and weight/(height)2 index (r = 0.34, p less than 0.01). IGF-I levels correlated positively with insulin levels (r = 0.421, p less than 0.001) and negatively with cortisol concentrations (r = -0.400, p less than 0.001).
Eur J Pediatr. 1986 Apr.
Fatty acid composition of plasma lipids in Nigerian children with protein-energy malnutrition.
Koletzko B, Abiodun PO, Laryea MD, Bremer HJ.
Abstract: The fatty acid (FA) composition of the main plasma lipids was analysed in eight well-nourished, generally healthy Nigerian children aged 14.1 +/- 7.2 months and in 17 malnourished children (8 marasmus, 9 kwashiorkor) aged 14.6 +/- 3.8 months within the first 2 days of admission at the Dept. of Child Health, University of Benin. In comparison to the control group, the malnourished children showed a marked decrease of polyunsaturated FA with low linoleic acid, mainly in sterol esters (STE), and severely reduced linoleic acid metabolites, including arachidonic acid, in all lipid fractions. omega-3-FA were not altered except for a reduction of docosapentaenoic and docosahexaenoic acids in phospholipids. Clearly increased values were found for saturated FA in STE and for the non-essential monoenoic FA in all lipid classes. This pattern indicates the presence of essential fatty acid deficiency in the malnourished children. There was no significant difference between marasmus and kwashiorkor. Eight malnourished children were followed up in the early phase of recovery during hospital treatment 14.0 +/- 3.1 days after obtaining the first sample. Linoleic acid had increased again in STE, but its metabolites were as low or even lower than before. An impaired activity of delta-6-desaturase, the rate limiting enzyme of linoleic acid metabolism, in suggested by elevated substrate-product-ratios of this enzyme in untreated children with protein energy malnutrition and in the early phase of recovery, which may be due to low insulin levels, protein and zinc deficiency. The trientetraen ratio (20:3 omega 9/20:4 omega 6) thus is not a reliable indicator of essential FA status in protein-energy malnutrition.
J Trop Med Hyg. 1984 Dec.
A profile of ammonia-urea values in blood and cerebrospinal fluid in children with protein energy malnutrition.
Abo-Hussein SA, Hussein ZM, Farag SI, Shebl SS, el-Melegy S, Akhnoukh S.
Abstract: The study comprised 25 children suffering from PEM. Ten of them were non-oedematous and the rest were oedematous. A group of 10 children of similar age served as the controls. A state of hyperammonaemia with slightly reduced blood urea was found in both forms of PEM but was more marked in kwashiorkor. There was also a highly significant increase in CSF ammonia in all cases of PEM studied. Some of the mental changes which have been observed in kwashiorkor may be, at least in part, due to ammonia intoxication.
Trop Geogr Med. 1984 Dec.
Carbohydrate malabsorption in children with severe protein energy malnutrition.
Tolboom JJ, Ralitapole-Maruping AP, Mothebe M, Kabir H, Molatseli P, Fernandes J.
Abstract: To determine the incidence of carbohydrate malabsorption, particularly lactose malabsorption in Basotho children with severe PEM during treatment with a dry skim milk (DSM)-sucrose-oil mixture, 105 children with PEM were submitted to a Hydrogen Breath Test (HBT) after administration of the mixture. Carbohydrate malabsorption occurred more frequently in children with kwashiorkor (28/58) than in those with marasmus (5/33), marasmic kwashiorkor (3/15) and healthy controls (8/34). The positive HBT appeared to be due to lactose malabsorption in at least two thirds of the children with kwashiorkor as it turned negative when the challenge was repeated with a lactosefree mixture. In controls malabsorption of carbohydrate (usually lactose) appeared at the age of 22 months, nearly a year later than in PEM. Diarrhoea occurred in 23 children with PEM and particularly in those with carbohydrate malabsorption. In 20 children Giardia was found in the stools without any observable effect on carbohydrate malabsorption, however. The findings support the cautious use of physiological doses of lactose in the treatment of severe PEM.
Fertil Steril. 1982 May.
Circulating gonadotropin profile in severe cases of protein calorie malnutrition.
Chakravarty I, Sreedhar R, Ghosh KK, Card D, Bulusu S.
Abstract: The circulating levels of luteinizing hormone (LH), follicle-stimulating hormone (FSH), and prolactin (PRL) were studied in children of both sexes between 2 and 14 years of age who were suffering from severe protein calorie malnutrition (PCM), namely, kwashiorkor, marasmic kwashiorkor, and marasmus. LH and FSH levels in all the age groups and in all forms of PCM were found to be significantly lowered, thereby explaining the possible delay in the onset of puberty in these children. Circulating PRL levels, on the other hand, were significantly raised in all patients with PCM studied, with values in children with kwashiorkor and marasmic kwashiorkor higher than in children with marasmus, possibly accounting for the presence of edema in the former cases. The present work, therefore, proposes a possible correlation between gonadotropin levels and PCM in children.
Biochim Biophys Acta. 1981 Dec 23.
The biological origin of ketotic dicarboxylic aciduria. In vivo and in vitro investigations of the omega-oxidation of C6-C16-monocarboxylic acids in unstarved, starved and diabetic rats.
Mortensen PB, Gregersen N.
The conversion of radioactive C6-C16-monocarboxylic acids to urinary adipic, suberic, sebacic and 3-hydroxybutyric acids was investigated in vivo in unstarved, starved and diabetic ketotic rats. Hexanoic, octanoic and decanoic acids were converted to C6-, C6-C8- and C6-C10-dicarboxylic acids, respectively, in fed and 72-h-starved rats. Lauric acid was converted to C6-C8-dicarboxylic acids in starved rats but not in unstarved rats. Decanoic and lauric acids were converted to relatively high amounts of C6-C8-dicarboxylic acids compared with myristic acid in myristic acid in ketotic diabetic rats, while radioactivity from [1-14C]-and [16-(14)] palmitic acid was not incorporated into C6-C8-dicarboxylic acids in diabetic ketotic rats. C6-C12-monocarboxylic acids in hydrolysed rat adipose tissue wee determined by gas-liquid chromatography-mass spectrometry (selected ion monitoring). Decanoic and lauric acids were found in amounts of 7.6-9.1 and 85.9-137.5 micrometers/100 mg tissue, respectively, whereas the amounts of hexanoic and octanoic acids were negligible. It is concluded that the biological origin of the C6-C8-dicarboxylic aciduria seen in ketotic rats are C10-C14-monocarboxylic acids, which are initially omega-oxidised solely or partly as free acids and subsequently beta-oxidised to adipic and suberic acids. The in vitro omega-oxidation of C6-C16-monocarboxylic acids to corresponding dicarboxylic acids in the 100,000 Xg supernatant fraction of rat liver homogenate was measured by selected ion monitoring. 0.09, 0.14, 16.1, 5.8, 7.0 and -6.9% of, respectively, hexanoic, octanoic, decanoic, lauric, myristic and palmitic acid were omega-oxidised to dicarboxylic acids of corresponding chain lengths after 90 min of incubation, when correction for the production of dicarboxylic acids in control assays was made. An in vitro production of C12-C16-dicarboxylic acids was detected in all assays ()including control assays), probably formed from"endogenous' monocarboxylic acids preexistent in the homogenate. Ths "endogenous' production of dicarboxylic acids was inhibited by C10-C16-monocarboxylic acids, where palmitic acid had the strongest effect. In fact, palmitic acid inhibited its own omega-oxidation when added in concentrations above 0.6 mM. Starvation of rats for 72 h did not alter the "endogenous' in vitro production of hexadecanedioic acid.
Biochim Biophys Acta. 1981 May 22.
C6-C10-dicarboxylic aciduria in starved, fat-fed and diabetic rats receiving decanoic acid or medium-chain triacylglycerol. An in vivo measure of the rate of beta-oxidation of fatty acids.
Mortensen PB.
Administration of decanoic acid to rats resulted not only in elevated urinary excretions of the C10-dicarboxylic acid (sebacic acid), but also in highly elevated excretions of the beta-oxidation products C8- and C6-dicarboxylic acids (suberic and adipic acids). Activation of the lipid metabolism by starvation, fat-feeding and experimental diabetes increased the excretions of adipic acid and decreased the excretions of sebacic acid, i.e. the rate of oxidation of fatty acids was correlated to the adipic : sebacic acid ratio in urine. Compared with nondiabetic unstarved rats the adipic : sebacic acid ratio was elevated 2-3-, 8-16-, 5-19-, and 22-88-times in rats which were, respectively, starved for 2 days, 4 days, on a fat-diet for 4 days, and ketotic due to streptozotocin-induced diabetes. All rats with ratios above 10 were ketotic (urinary excretions of 3-hydroxybutyric acid over 500 microgram/mg creatinine) and all rats with ratios below 4 were nonketotic, while ketosis was a variable finding in rats with intermediary ratios. Similar changes in the ratio of excreted dicarboxylic acids were found when medium-chain triacylglycerols were fed instead of decanoic acid.
Biochim Biophys Acta. 1981 May 22.
Urinary excretion of C4--C10-dicarboxylic acids and antiketogenic properties of adipic acid in ketogenic-stimulated rats due to diabetes, long-chain and short-chain monocarboxylic acids.
Mortensen PB.
The urinary excretion of C4-C10-dicarboxylic acids (succinic, adipic, suberic and sebacic acids) and the antiketogenicity of adipic acid have been studied in ketogenic-stimulated rats in three biochemically different states: diabetes, fat-feeding (long-chain monocarboxylic acids) and feeding of hexanoic acid (short-chain monocarboxylic acid). In diabetic rats urinary excretions of adipic and suberic acids were elevated before the rise in urinary excretions of 3-hydroxybutyric acid, i.e. before ketosis appeared. In severe diabetic ketosis sebacic acid was below normal values, whereas the excretion of succinic acid was unaltered. Rats, in which ketosis was provoked by hexanoic acid, had preketotic high urinary excretions of adipic and succinic acids. After ketosis the excretions of succinic acid declined again whereas the excretion of adipic acid rose further, together with that of suberic acid. Moreover, when rats which were ketotic due to treatment with long-chain triacylglycerol or hexanoic acid received 500 mg of adipic acid the urinary excretion of succinic acid rose significantly. However, no changes in succinic acid excretion were seen in diabetic ketotic rats treated with the same amount of adipic acid. Exogenously administered adipic acid was strongly antiketogenic towards ketosis caused by long-chain or short-chain monocarboxylic acids, but had no effect on diabetic ketosis.
Biochim Biophys Acta. 1980 Nov 7.
The possible antiketogenic and gluconeogenic effect of the omega-oxidation of fatty acids in rats.
Mortensen PB.
The urinary excretion of C4--C10-dicarboxylic acids and 3-hydroxybutyric acid was examined in rats on ketogenic stimulation due to fat-feeding. The urinary excretion of succinic acid decreased while the urinary excretion of adipic and suberic acids increased prior to the appearance of ketosis, and this pattern of excretion was almost independent of the degree of the subsequent ketosis. After administering adipic acid to the ketotic rats, urinary excretion of succinic acid increased at the same time as ketosis decreased and blood glucose increased. The possibility of a physiological antiketogenic and gluconeogenic effect of the omega-oxidation of fatty acids to dicarboxylic acids is discussed.
Acta Paediatr Scand. 1979 Sep.
The excretion of C6-C10-dicarboxylic acids in the urine of newborn infants during starvation. Evidence for omega-oxidation of fatty acids in the newborn.
Gregersen N, Ingerslev J.
The excretion of C6-C10-dicarboxylic acids, i.e. adipic, suberic and sebacic acids, was measured during the three first days of life in 3 fasting newborns, 2 newborns fed with isocaloric glucose and 2 newborns given mothers'-milk. On the second and third day of life the starved children excreted 27-84 mmol adipic acid/mol creatinine, 6-22 mmol suberic acid/mol creatinine and 4-7 mmol sebacic acid/mol creatinine. The excretion of C6-C10-dicarboxylic acids in the neonates given glucose or mothers'-milk was, for the first three days of life, 0-9 mmol adipic acid/mol creatinine, 0-10 mmol suberic acid/mol creatinine and 0-4 mmol sebacic acid/mol creatinine. The latter amounts are equivalent to the excretion of dicarboxylic acids in older children. It is argued that the detected dicarboxylic acids are formed by omega-oxidation of long-chain monocarboxylic acids followed by beta-oxidation, and that the excreted amounts reflect omega-oxidation activity. It is speculated that the substantial omega-oxidation activity in the starving newborn serve to provide succinyl-CoA-substrate for the citric acid cycle and for gluconeogenesis.
Clin Chim Acta. 1979 May 16.
Dicarboxylic aciduria: the response to fasting.
Truscott RJ, Hick L, Pullin C, Halpern B, Wilcken B, Griffiths H, Silink M, Kilham H, Grunseit F.
The urine of a child who presented with an episode of a disease resembling Reye's syndrome was found to contain large quantities of the dicarboxylic acids adipic and suberic acids, as well as the glycine conjugate of suberic acid, suberyl glycine. A variety of other dicarboxylic acids, both saturated and unsaturated, were also found in the urine at the time of the attack. It was found that the excretion of these unusual metabolites could be markedly increased by fasting for periods of greater than 10 h. These results indicate that the patient may have a defect in fatty acid oxidation which becomes clinically significant during periods of prolonged fasting.
J Nutr. 1979 Jan.
The effect of long-term fasting on the branched chain acylcarnitines and branched chain carnitine acyltransferases.
Choi YR, Fogle PJ, Bieber LL.
The effect of fasting for 8 days on the levels of carnitine acyltransferases in heart, liver, liver mitochondria, skeletal muscle, skeletal muscle mitochondria, kidney, and testes in young adult male rats was determined. The specific activities of acetyl-, octanyl-, isobutyryl-, and isovaleryl-carnitine acyltransferase in mitochondria isolated from the livers of fasted animals were significantly higher than the levels of the transferases isolated from livers of fed animals. Similar results were obtained with the 500 x g supernatant fluids from liver. In contrast, the specific activities of carnitine acyltransferases of 500 x g supernatant fractions isolated from heart, skeletal muscle, kidney, and testes were the same for fed as fasted animals. The total carnitine content of liver, muscle, heart, and kidney was less in animals fasted for 8 days than in fed animals, but the amount/g of organ was higher in the animals fasted for 8 days. The amount of specific short-chain acylcarnitines in liver, muscle, and heart was determined for both fed and fasted animals. The amount of isobutyrylcarnitine and isovalerylcarnitine increased significantly in muscle from fasted animals. These data are consistent with the previous suggestion that carnitine may have a role in the metabolism of the branched-chain amino acids.
Br J Nutr. 1976 Sep.
Serum albumin and transferrin protein-energy malnutrition. Their use in the assessment of marginal undernutrition and the prognosis of severe undernutrition.
Reeds PJ, Laditan AA.
Abstract: 1. Deficits in weight- and length-for-age, and serum albumin and transferrin concentrations were determined for children who were either marginally undernourished (twenty-five children) or suffering from either marasmus (thirty-two children) or kwashiorkor (twenty-six children) defined according to the Wellcome Classification (Waterlow, 1972). The measurements were also made in eight children with kwashiorkor after the loss of oedema, and in sixteen children who were recovering from either marasmus or kwashiorkor. 2. The mean concentration of serum albumin was similar for children from the 'under-nourished' group and from the group with marasmus, but was significantly reduced in those with kwashiorkor. 3. The concentration of serum transferrin was significantly reduced in both the group of children with marasmus and those with kwashiorkor. The serum transferrin concentration was significantly lower in children with kwashiorkor when compared with the level in those with marasmus. 4. Seventeen children (seven with kwashiorkor and ten with marasmus) died. These children were neither lighter nor shorter than the severely malnourished children who survived. The concentration of serum albumin was not lower in the children who died than in those who survived. 5. In contrast to the results for serum albumin concentrations, the children who died had significantly lower levels of serum transferrin than those who survived. 6. There was a significant linear relationship between serum transferrin concentrations and the deficits in length-for-age (P less than 0-05) and weight-for-length (P less than 0-001) in the marginally undernourished children. The deficit in weight-for-length was also linearly related to the serum transferrin concentrations (P less than 0-001) in children recovering from severe malnutrition. 7. It is suggested that the measurement of serum transferrin concentrations provides an index of severity in severely malnourished children, and should prove useful in field assessments of nutritional status.
Gaz Egypt Paediatr Assoc. 1975 Apr.
Biochemical and haematological aspects of anaemia associating protein energy malnutrition (PEM).
Said A, El-Hawary MF, Sakr R, Khalek MK, El-Shobaki FA, Noseir MB.
Abstract: Haematological as well as biochemical parameters assessing anaemia associating protein energy malnutrition PEM were estimated. Data revealed that controls studied have a relatively lower Hb, Ht, and R.B.C.S. count than European standards. Hypochromic anaemia was predominant in 83.3% of moderate Kwashiorkor being normocytic in 50% and microcytic in 33.3%. In severe KWO anaemia was normocytic in 42% and microcytic in 19.2%. Normochromia was found in 9.7% and hypochromia in 29%. Macrocytosis was found in 38.8% in severe KWO. In marasmus, normocytic anaemia was revealed in 74% of the 2nd. grade being normochromic in 82% and hypochromic in 40%. Macrocytic anaemia was detected in 26%. In 3rd. grade subjects normocytic normochromic anaemia occurred in 70% of the cases. Macrocytosis was encountered among 30% of the cases. Serum iron and transferrin level dropped in both KWO and marasmus, the extent of decrease was greater in the former. The percent transferrin saturation was elevated in severe cases. Particularly severe KWO and marasmic KWO.