Systemic lupus erythematosus with acanthosis nigricans, hyperpigmentation, and insulin receptor antibody.

Acanthosis nigricans, insulin receptor antibody, and systemic lupus erythematosus are associated in the potentially lethal syndrome of type B insulin resistance. Hyperpigmentation has been reported rarely, while glucose intolerance is common in these patients. We report an adolescent girl with acanthosis nigricans, hyperpigmentation, insulin receptor antibody, and systemic lupus erythematosus without glucose intolerance. Insulin resistance may be mild or transient in some patients with type B insulin resistance. Resolution of skin lesions was noted during therapy of SLE, and was associated with disappearance of insulin receptor antibody.

Amebic liver abscess in infancy: case report and review.

Amebic liver abscess (ALA) is uncommon in the United States and is potentially lethal when acquired during the first year of life. The clinical course of ALA in a 10-month-old infant is presented and the characteristics of 18 previously described cases of infants with ALA from the United States are reported; this infant population (including our patient) is compared with a population of adults with ALA. In contrast to the initial signs in adults, those in infants are nonspecific and include fever, hepatomegaly, anemia, and elevated levels of transaminases. Colitis may occur in infants, but results of examinations for ova or parasites are usually negative. On presentation the amebic serology for one-third of infants with ALA is negative. The clinical course of ALA in infants is usually fulminant and is associated with a mortality rate of nearly 50%.

Chronic renal failure in sickle cell disease: risk factors, clinical course, and mortality.

OBJECTIVE: To determine the incidence, clinical course, and risk factors associated with the onset of chronic renal failure in sickle cell anemia and sickle C disease. DESIGN: A prospective, 25-year longitudinal demographic and clinical cohort study. A matched case-control study was conducted to determine risk factors. PATIENTS: A total of 725 patients with sickle cell anemia and 209 patients with sickle C disease who received medical care from the hematology service in a large municipal hospital. Most were observed from birth or early childhood. MEASUREMENTS: Thirty-six patients developed sickle renal failure: 4.2% of patients with sickle cell anemia and 2.4% of patients with sickle C disease. The median age of disease onset for these patients was 23.1 and 49.9 years, respectively. Survival time for patients with sickle cell anemia after the diagnosis of sickle renal failure, despite dialysis, was 4 years, and the median age at the time of death was 27 years. Relative risk for mortality was 1.42 (95% Cl, 1.12 to 1.81; P = 0.02) compared with patients who did not develop renal insufficiency. MAIN RESULTS: Histopathologic studies showed characteristic lesions of glomerular "drop out" and glomerulosclerosis. Case-control analysis showed that ineffective erythropoiesis with increasingly severe anemia, hypertension, proteinuria, the nephrotic syndrome, and microscopic hematuria were significant pre-azotemic predictors of chronic renal failure. The risk for sickle renal failure was increased in patients who had inherited the Central African Republic beta s-gene cluster haplotype. CONCLUSIONS: The pre-azotemic manifestations of hypertension, proteinuria, and increasingly severe anemia predict end-stage renal failure in patients with sickle cell disease. The rate of progression of renal insufficiency is genetically determined. Treatment of the uremic phase has been dismal, underscoring the need for the development of useful pre-azotemic therapeutic modalities.

Phosphate poisoning complicating treatment for iron ingestion.

A 15-month-old child was treated for iron intoxication with a hypertonic sodium phosphate mixture. Clinical deterioration manifested by fever, obtundation, abdominal distention, dehydration, and hypotension followed soon after the administration of this mixture. Such symptoms may occur with either iron overdosage or with phosphate poisoning. At this time, the patient's serum chemistry values included: iron, 49 micrograms/dL; phosphorus, 24.6 mg/dL; and calcium, 4.5 mg/dL. The hypocalcemia, hyperphosphatemia, and dehydration were treated with parenteral gluconate calcium, intravenous fluids, and general supportive measures. Although the child had an uneventful recovery despite severe phosphate poisoning, therapeutic alternatives, such as sodium bicarbonate, should be used as adjuncts in the treatment of acute iron ingestion.

Rhabdomyolysis with severe hypernatremia.

Three children with severe hypernatremia presented with profound generalized weakness and biochemical evidence of rhabdomyolysis and myoglobinuria. These findings in combination have not been previously reported, to our knowledge, in children with severe hypernatremia. Unusual complications included respiratory failure in one child and cardiac arrhythmias in two children. All three children had acute renal insufficiency; one required peritoneal dialysis.