Shiga-like toxin-producing Escherichia coli O111 and associated hemolytic-uremic syndrome: a family outbreak.

OBJECTIVE: To describe a family cluster of Shiga toxin-producing Escherichia coli O111ac:NM infection. STUDY DESIGN: The index case was identified as part of a United States prospective study of hemolytic-uremic syndrome. Epidemiologic investigation was conducted through interviews. E. coli O111:NM infection was characterized through culture and serology. Shiga toxin 1 and 2 gene sequences were determined with oligonucleotide DNA probes. RESULTS: All three children and both parents had nonbloody diarrhea, vomiting and abdominal cramps, and one child developed hemolytic-uremic syndrome. Shiga toxin 1- and 2-producing E. coli O111ac:NM was isolated from two children. IgG antibodies to E. coli O111 were detected in all three children. CONCLUSIONS: To our knowledge this is the first reported cluster of O111 infection and only the second caused by non-O157 Shiga toxin-producing E. coli in North America.

Marfanoid children. Etiologic heterogeneity and cardiac findings.

The clinical, cardiac, and echocardiographic test results of 20 children with marfanoid features are reviewed. Fifteen were diagnosed as having Marfan syndrome, two had "possible" Marfan syndrome, and three had other diagnoses. On first evaluation, eight patients with Marfan syndrome (53%) had mitral regurgitation and none had aortic regurgitation. Echocardiography showed aortic root enlargement in 12 (80%) of 15 patients and mitral valve prolapse in 12 (80%) of 15. None had a normal echocardiogram. At follow-up examination, one patient had developed aortic root enlargement, and one patient, mitral valve prolapse. Thus, although aortic root enlargement is usually present in early childhood in patients with Marfan syndrome, it is not considered specific because in this study it also occurred in one child with Alport's syndrome and in one with marfanoid features. Four patients with aortic root enlargement were treated with propranolol and their echocardiograms showed no further increase in the aortic root diameter for several years. We recommend echocardiography in the diagnosis and routine management of children in whom Marfan syndrome is suspected.

Membranous glomerulonephritis in a child asymptomatic for hepatitis B virus. Concomitant seropositivity for HBsAG and anti-HBs.

The presence of hepatitis B surface antigen (HBsAg) in association with immunoglobulins and complement components within the glomerular basement membranes of adults having chronic active hepatitis has been well documented. In addition, investigators in Poland have demonstrated HBsAg immune complexes in glomeruli of children who did not have clinical evidence of hepatitis. More recently, a single case of childhood membranous glomerulonephritis in an asymptomatic carrier of hepatitis B virus was cited by observers in Canada. Reported here is the deposition of HBsAg immune complexes in the glomerular basement membranes of a 13-year-old black boy who had membranous glomerulopathy but not clinical evidence of hepatitis. This may be the first reported case in the United States of HbsAg-associated membranous glomerulonephritis in a child asymptomatic for hepatitis B virus, and only the second such case in North America. However, unlike previous studies of childhood glomerulopathy in association with hepatitis B virus, this patient is seropositive for both HBsAg and anti-HBs (antibody for hepatitis B surface antigen). Similar "rare" serologic findings were found for the patient's eldest male sib.

Ammonia encephalopathy secondary to urinary tract infection with Proteus mirabilis.

Hyperammonemia with coma, tachypnea, and respiratory alkalosis developed in a 3-year-old boy with prune"-belly syndrome during a urinary tract infection with Proteus mirabilis. Hyperammonemia is thought to have resulted from the production within the massively dilated urinary tract of excessive amounts of ammonia due to bacterial urease, and its subsequent reabsorption into the systemic circulation. The patient rapidly improved following parenteral antibiotic therapy and continuous catheter drainage of the urinary tract.

Urinary high density lipoprotein in minimal change glomerular disease and chronic glomerulopathies.

Serum lipids and lipoproteins and urinary apolipoprotein A (Apo A) were determined in two groups of patients. One group consisted of 11 children (ages ranging from 4 to 14 years) with minimal change glomerular disease. The other group consisted of 13 patients, eight less than 19 years old five adults, with different types of chronic glomerulopathy. Elimination of urinary lysozyme was a feature of chronic glomerulopathies, and creatinine clearances were also significantly lower in this group. Patients with chronic glomerulopathies had significantly lower HDL cholesterol and Apo A concentrations in their sera. In contrast, urinary Apo A concentrations were significantly higher in patients with chronic glomerulopathies, who also showed significantly lower urinary protein selectivities. Lipoprotein electrophoresis of urines containing Apo A showed distinct high-density lipoprotein (HDL) fractions, suggesting that HDL is eliminated in the urine as a result of increased glomerular permeability. This is also supported by a correlation coefficient of 0.77 between the selectivity indices and the ratio of urinary Apo A to total proteinuria. The determination of urinary Apo A appears to give valuable diagnostic information in patients with glomerular disease. According to our results the absence of urinary Apo A is very suggestive of minimal change glomerular disease.

Renal anomalies in mice prenatally exposed to ethanol.

The current observations confirm and extend earlier data demonstrating the deleterious effects of ethanol in the C57BL/6J mouse. Ethanol given to pregnant mice from gestation-day 5 to gestation-day 11 reduced the number of mice going to full term, decreased the number of pups per litter, and lowered the birthweight of the live pups. Prenatal exposure to ethanol also produced a high incidence of hydronephrosis in the offspring.

Subarachnoid hemorrhage complicating acute poststreptococcal glomerulonephritis.

A 6-year-old boy with seizures and signs of meningeal irritation was found to have a spontaneous subarachnoid hemorrhage. Investigations, including a renal biopsy, were diagnostic of an underlying acute poststreptococcal glomerulonephritis. The patient recovered and is asymptomatic three years later. This case shows that spontaneous subarachnoid hemorrhage can occur in children with acute poststreptococcal glomerulonephritis and should be suspected when meningeal manifestations are prominent.