| Fat embolism from the grossly fatty liver of an infant with kwashiorkor is described. Globules of fat were seen at necropsy in the hepatic veins, in the right atrium and ventricle of the heart and in the pulmonary artery. Sections of the lung showed fat emboli in numerous branches of the pulmonary artery. Examination of lung sections taken at necropsy from 40 patients who had severe kwashiorkor failed to show similar fat embolism. ( info) |
2/35. A case of kwashiorkor in the UK. A case of kwashiorkor in a British child of Caucasian origin is described. The 5-year-old boy was referred to hospital for investigation of a persistent anaemia, but on examination was found to have classical features of kwashiorkor. He was stunted with both height and weight below the fifth centile and had mild pitting oedema in both legs. His hair was pale and easily pluckable and a soft liver edge was palpable. plasma albumin concentration was 16 g/l and the plasma amino acid pattern, which revealed markedly reduced levels of essential but normal to high non-essential amino acids, was similar to that described in kwashiorkor in uganda. A dietary history revealed that for about 2 years the child's diet had contained very little protein but adequate energy and had been supplemented with multivitamin pills. There was no evidence of other pathology, neglect or abuse and the child responded rapidly to refeeding with a balanced diet. ( info) |
3/35. Dermatosis in a child with kwashiorkor secondary to food aversion. kwashiorkor is a common affliction of children worldwide. It occurs less often in developed countries, but has been reported under a variety of circumstances, including poverty, neurologic disease, and malabsorption. Because of its rare occurrence in the united states and because the affected child has an edematous rather than wasted appearance, physicians often do not consider it as a diagnostic entity. This article describes a case of kwashiorkor in a child with food aversion that manifested as "flaky paint dermatitis." Our discussion will attempt to delineate underlying conditions that may predispose to kwashiorkor. In addition, biochemical and cellular etiologic factors that may be linked with classical and nonclassical skin findings of kwashiorkor are considered. Finally, we present a differential diagnosis for any child with a generalized eczematous or desquamative rash. Our aim is to increase the ability of health care providers to identify and treat children with kwashiorkor in a timely manner. ( info) |
4/35. Malnutrition in infants receiving cult diets: a form of child abuse. Severe nutritional disorders, including kwashiorkor, marasmus, and rickets, were seen in four children and were due to parental food faddism, which should perhaps be regarded as a form of child abuse. All disorders were corrected with more normal diets and vitamin supplements. In view of the potentially serious consequences of restricted diets being fed to children, families at risk should be identified and acceptable nutritional advice given. When children are found to be suffering from undernutrition due to parental food faddism a court order will normally be a necessary step in providing adequate treatment and supervision. ( info) |
| kwashiorkor, a form of severe protein-energy malnutrition that entails loss of lean body weight, occurs endemically among children in many parts of the world but also has been documented in adults. We report a case of kwashiorkor in an hiv-positive adult male. Cutaneous findings are striking, and skin, hair, and nails are affected. Although kwashiorkor occurs in patients with hiv-AIDS, the skin manifestations have not been emphasized in the dermatologic literature. Indeed, dermatologists may play a vital role in diagnosing this treatable condition. ( info) |
6/35. Severe nutritional deficiencies in toddlers resulting from health food milk alternatives. 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. He was full-term at birth and was breastfed until 10 months of age, when he was weaned to a soy health food beverage, which was not fortified with vitamin d or calcium. Intake of solid foods was good, but included no animal products. Total daily caloric intake was 114% of the recommended dietary allowance. Dietary vitamin d intake was essentially absent because of the lack of vitamin d-fortified milk. The patient lived in a sunny, warm climate, but because of parental career demands, he had limited sun exposure. His dark complexion further reduced ultraviolet light-induced endogenous skin synthesis of vitamin d. The patient grew and developed normally until after his 9-month check-up, when he had an almost complete growth arrest of both height and weight. The parents reported regression in gross motor milestones. On admission the patient was unable to crawl or roll over. He could maintain a sitting position precariously when so placed. Conversely, his language, fine motor-adaptive, and personal-social skills were well-preserved. Generalized hypotonia, weakness, and decreased muscle bulk were present. Clinical features of rickets present on examination included: frontal bossing, an obvious rachitic rosary (photographed), genu varus, flaring of the wrists, and lumbar kyphoscoliosis. The serum alkaline phosphatase was markedly elevated (1879 U/L), phosphorus was low (1.7 mg/dL), and calcium was low normal (8.9 mg/dL). The 25-hydroxy-vitamin d level was low (7.7 pg/mL) and the parathyroid hormone level was markedly elevated (114 pg/mL). The published radiographs are diagnostic of advanced rickets, showing diffuse osteopenia, frayed metaphyses, widened epiphyseal plates, and a pathologic fracture of the ulna. The patient was treated with ergocalciferol and calcium supplements. The published growth chart demonstrates the dramatic response to therapy. Gross motor milestones were fully regained within 6 months. The prominent neuromuscular manifestations shown by this patient serve as a reminder that rickets should be considered in the differential diagnosis of motor delay. (ABSTRACT TRUNCATED) ( info) |
7/35. kwashiorkor in the united states: fad diets, perceived and true milk allergy, and nutritional ignorance. BACKGROUND: kwashiorkor is the edematous form of protein-energy malnutrition. It is associated with extreme poverty in developing countries and with chronic malabsorptive conditions such as cystic fibrosis in developed countries. Rare cases of kwashiorkor in affluent countries unrelated to chronic illness have been reported. We present 12 cases of kwashiorkor unrelated to chronic illness seen over 9 years by pediatric dermatologists throughout the united states, and discuss common causative themes in this easily preventable condition. OBSERVATIONS: Twelve children were diagnosed as having kwashiorkor in 7 tertiary referral centers throughout the united states. The diagnoses were based on the characteristic rash and the overall clinical presentation. The rash consisted of an erosive, crusting, desquamating dermatitis sometimes with classic "pasted-on" scale-the so-called flaky paint sign. Most cases were due to nutritional ignorance, perceived milk intolerance, or food faddism. Half of the cases were the result of a deliberate deviation to a protein-deficient diet because of a perceived intolerance of formula or milk. Financial and social stresses were a factor in only 2 cases, and in both cases social chaos was more of a factor than an absolute lack of financial resources. Misleading dietary histories and the presence of edema masking growth failure obscured the clinical picture in some cases. CONCLUSIONS: physicians should consider the diagnosis of kwashiorkor in children with perceived milk allergies resulting in frequent dietary manipulations, in children following fad or unorthodox diets, or in children living in homes with significant social chaos. The presence of edema and "flaky paint" dermatitis should prompt a careful dietary investigation. ( info) |
8/35. Can the growth of a neuroblastoma be influenced by a child's nutritional state? Observations in a patient treated for kwashiorkor and later given a restricted diet. A 17-month-old Thai female with neuroblastoma presented with an abdominal mass and the classical findings of kwashiorkor. Concomitant with effective repair of the child's protein deficit, the mass enlarged dramatically and metastases were noted. This is the first known report of such an occurrence. ( info) |
| growth failure is a common presentation of patients with pancreatic insufficient cystic fibrosis. However, full blown kwashiorkor is extremely rare. cystic fibrosis is also considered to be rare in the South Asian population. This report describes a Sri Lankan infant with cystic fibrosis who presented with clinical features of severe kwashiorkor. ( info) |
10/35. Central pontine myelinolysis central pontine myelinolysis manifesting with massive myoclonus. Central pontine myelinolysis is a rare neurologic disorder defined by symmetric demyelination in the central base of the pons. It usually manifests with neurologic signs such as impaired consciousness, ataxia, spastic quadriparesis, pseudobulbar signs, and the locked-in syndrome which is related to a disconnection syndrome at the pontine level. We report a 17-month-old patient with kwashiorkor and hyponatremia who developed acute massive myoclonus. magnetic resonance imaging revealed a central pontine lesion. Central pontine myelinolysis is rare in infants, with only a few cases reported in the literature so far. This report presents the first infantile case of central pontine myelinolysis manifesting with massive myoclonus. ( info) |