1/5. Phrynoderma: a manifestation of vitamin a deficiency?...The rest of the story.Phrynoderma is a distinctive form of follicular hyperkeratosis associated with nutritional deficiency. Although originally thought to represent vitamin a deficiency, several studies have demonstrated multiple etiologies. Characteristic skin lesions are hyperkeratotic papules that first appear on the extensor surfaces of the extremities, shoulders, and buttocks. We report a 14-month-old boy with malnourishment and hyperkeratotic papules and plaques with histologic changes typical of phrynoderma. Despite an extensive evaluation, a specific nutritional deficiency was not identified. Phrynoderma is believed to be a manifestation of severe malnutrition, not necessarily accompanying low vitamin A levels. While the literature supports a link between phrynoderma and vitamins E, B, A, and essential fatty acids general malnutrition seems to be the strongest association. The clinical picture typically improves with enhanced nutritional status. Phrynoderma must be considered in the differential diagnosis in patients with extensor surface hyperkeratotic papules and plaques in the setting of malnourishment and should prompt the clinician to evaluate cell markers of nutritional status, not just vitamin A. We believe this patient exemplifies the conundrum that faces clinicians in evaluating patients with extensor surface predominant hyperkeratotic papules and plaques in the setting of malnourishment.- - - - - - - - - - ranking = 1keywords = malnutrition (Clic here for more details about this article) |
2/5. Advanced xerophthalmia as a presenting sign in cystic fibrosis.xerophthalmia is a common complication of vitamin a deficiency in communities where malnutrition is found. We report on a 16-month-old infant with severe photophobia and failure to thrive. On examination, her major presenting sign was corneal xerosis, with corneal and conjunctival keratinization, and corneal stromal edema with opacification. Based on these findings, vitamin a deficiency secondary to fat malabsorption was suspected, and a workup confirmed the diagnosis of cystic fibrosis. With parenteral vitamin A supplementation, she had complete resolution of her ocular signs and symptoms. This case illustrates the value of a complete ophthalmic examination in the diagnosis of fat malabsorption syndromes.- - - - - - - - - - ranking = 0.5keywords = malnutrition (Clic here for more details about this article) |
3/5. Keratomalacia in a child with familial hypo-retinol-binding proteinemia.Retinol-binding protein (RBP) is a plasma protein with a molecular weight of 21,000 synthesized in the hepatocytes, binding with retinol (vitamin A), and transporting retinol to peripheral tissues. Keratomalacia is caused by a deficiency of vitamin A itself and/or protein-calorie malnutrition. In the latter condition, production of RBP is inhibited. We report herein familial hypo-retinol-binding proteinemia in a child aged 19 months who developed keratomalacia during measles infection in spite of good nourishment. To the best of our knowledge this is the first description of such a case. The patient, her sister and mother showed persistent low levels, about half the normal levels, of retinol and RBP which were unresponsive to oral vitamin A and protein-rich diet. They had normal liver function tests, normal serum levels of other proteins and fat-soluble vitamins and lipids. This hypo-retinol-binding proteinemia was thought to predispose the child to develop keratomalacia during measles. family members would be heterozygous with one normal RBP gene and one defective RBP gene.- - - - - - - - - - ranking = 0.5keywords = malnutrition (Clic here for more details about this article) |
4/5. Benign intracranial hypertension in an older child with cystic fibrosis.Despite having normal height and weight, a 6-year-old girl had frequent bowel movements and slight recurrent chest infections since the age of 4 years and headache for 1 year. The patient appeared healthy, but examination of the ocular fundus revealed papilledema. Cranial computed tomography appeared normal. Lumbar puncture disclosed an elevated opening cerebrospinal fluid pressure, with normal biochemical, cellular, and bacteriologic findings. Laboratory investigations indicated pathologic steatorrhea, elevated electrolytes in 3 sweat tests, and low serum levels of vitamins A and E. The diagnosis of pseudotumor cerebri in a patient with cystic fibrosis was made. After treatment with prednisone (1 mg/kg/day), pancreatic extracts, and vitamin supplements, headache and papilledema resolved and serum vitamin A and E levels subsequently became normal. Older children with cystic fibrosis rarely have benign intracranial hypertension, but when present it is often due to hypervitaminosis during correction of malnutrition. In this child, pseudotumor cerebri and associated hypovitaminosis improved after combined corticosteroid and vitamin treatment.- - - - - - - - - - ranking = 0.5keywords = malnutrition (Clic here for more details about this article) |
5/5. Bilateral severe keratomalacia after acute pancreatitis.A rare bilateral severe keratomalacia occurred in an emaciated alcoholic, 57-year-old white man with acute necrotic pancreatitis. The patient had a 10-year history of chronic alcoholism. Laboratory and clinical findings were consistent with the diagnosis of a fat malabsorptive and malnutrition syndrome secondary to chronic and acute alcoholic pancreatitis. visual acuity of both eyes was limited to light perception. Bilateral corneal necrosis after acute pancreatitis has not previously been reported.- - - - - - - - - - ranking = 0.5keywords = malnutrition (Clic here for more details about this article) |