Prevalence of congenital adrenal hyperplasia in Kuwait.

Between 1978 and 1988 congenital adrenal hyperplasia (CAH) was diagnosed in 60 children in Kuwait with an estimated prevalence of 1:9,000 livebirths, which is higher than that reported from Europe and Canada. In addition, there was presumptive evidence of CAH resulting in the death of 20 other children, giving a prevalence figure of 1:7000. There were 41 girls (68%) and 19 boys (32%). Thirty-one of the girls (75.6%) and 11 of the boys (57.9%) were saltlosers. Fifty-four patients (90%) were diagnosed as 21-hydroxylase deficient, 3 patients (5%) had a deficiency of the 3 beta-hydroxy-steroid dehydrogenase enzyme, and 3 patients (5%) showed a deficiency of 11 beta-hydroxylase.

Pulmonary brucellosis.

Pulmonary abnormalities in brucellosis are rare. We report on nine cases (five adults and four children) with pulmonary brucellosis. All presented with fever, cough and mucopurulent sputum, and most had abnormal signs in the chest. Radiography of the chest showed pneumonic patches or consolidation in five patients, pleural effusion in three, granuloma of the lung in one and a picture of interstitial pneumonitis in one. All the patients had a brucella agglutination titre of 1:320 or more, and an elevated titre in the brucella-specific enzyme linked immunosorbent assay of IgM, IgG and IgA. Blood cultures grew Brucella melitensis in six patients while the pleural fluid aspirate grew the same organism in two of three patients. Treatment with oral oxytetracycline, doxycycline, rifampicin, trimethoprim-sulfamethoxazole alone, in combination with each other or together with intramuscular streptomycin was successful in all patients. All our patients recovered and none relapsed.

Neurobrucellosis in children.

Reports on nervous system involvement in brucellosis are rare in children. We report nine children with neurobrucellosis. The clinical presentation included meningitis in six patients, one with encephalitis, one with meningoencephalitis and one with meningomyeloencephalitis. The blood from all patients showed elevation in Brucella microagglutination test titers (greater than or equal to 1:640) and in Brucella-specific enzyme-linked immunosorbent assay for IgM (greater than or equal to 1:800), IgG (greater than or equal to 1:800) and IgA (greater than or equal to 1:800) antibodies. Brucella melitensis was recovered from the blood in five patients and from the cerebrospinal fluid in three patients. The cerebrospinal fluid showed lymphocytic pleocytosis in eight patients with elevated protein in three, decreased glucose in four and a Brucella microagglutination test titer of greater than or equal to 1:80 in all. Treatment with a combination of oral tetracyclines with intramuscular streptomycin was successful in five patients, rifampin with streptomycin in two, tetracycline with rifampin in one and tetracycline, rifampin and streptomycin in one. No relapses, mortality or sequelae occurred in our patients.

A multicenter therapeutic study of 1100 children with brucellosis.

A 6-year multicenter therapeutic study was performed on 1100 children with brucellosis in order to compare several antibiotic combinations and duration of treatment. The patients were randomized to receive oral therapy with oxytetracycline, doxycycline, rifampin and trimethoprim-sulfamethoxazole (TMP/SMX) either alone or in combination with each other or combined with streptomycin or gentamicin injections. The patients were also randomized into three groups based on the duration of oral therapy: 500 patients were treated for 3 weeks; 350 for 5 weeks; and 250 for 8 weeks. When intramuscular aminoglycosides were used, streptomycin was given for 2 weeks and gentamicin for 5 days. In oral monotherapy oxytetracycline, doxycycline and rifampin showed comparable results with low relapse rates (less than or equal to 9%) and no statistically significant differences were found among 3-, 5- or 8-week durations of therapy. TMP/SMX alone showed an unacceptably high relapse rate (30%) with all durations of therapy. In combined oral therapy rifampin plus oxytetracycline, rifampin plus TMP/SMX and oxytetracycline plus TMP/SMX showed comparable results with low relapse rates ranging from 4 to 8% in patients receiving therapy for 3 or 5 weeks, no relapses occurred in patients treated for 8 weeks. When oral monotherapy was combined with either streptomycin or gentamicin, very few relapses were seen, irrespective of the duration of treatment.(ABSTRACT TRUNCATED AT 250 WORDS)

Neonatal brucellosis.

Three Arab children with neonatal brucellosis are described. The first presented with late neonatal hyperbilirubinaemia, the second with a septicoemia-like picture and the third was born prematurely and presented with respiratory distress. The diagnosis of brucellosis was based on a positive blood culture and on a high or rising titre of antibodies to the Brucella organism. All the three neonates responded well to antibiotic therapy as monitored by a Brucella titre of less than 1:40 and a negative blood culture 10 weeks after the onset of therapy. The three mothers had Brucella infections during pregnancy and the Brucella agglutination titre of the breast milk was high. No Brucella microorganism was isolated from the breast milk. The mode of transmission of brucellosis in neonates is discussed.

Primary hypomagnesaemia. A case report and literature review.

A case of primary hypomagnesaemia with secondary hypocalcaemia in an Arab girl of consanguineous parents is reported. She presented at the age of 3 weeks with generalised convulsions, was treated with magnesium supplements and followed up for 5 years during which she showed normal physical and psychomotor development. In view of the striking male preponderance among the reported cases and the presence of parental consanguinity in a few, the inheritance is discussed and genetic heterogeneity is suggested.