Autosomal recessive osteopetrosis in Arab children.

Nineteen Arab children including six boys and 13 girls in ten sibships were diagnosed as having osteopetrosis over a 5-year period in various hospitals in Kuwait. Eighteen patients had an isolated autosomal recessive form and one had autosomal recessive osteopetrosis associated with renal tubular acidosis. The mean age of diagnosis was 24 months. Parental consanguinity was high amongst them (68%). Anaemia, hepatosplenomegaly, failure to thrive, recurrent infections and neurological manifestations were common. Associated congenital abnormalities were found in 26%. Deafness, hydrocephalus and dental caries were relatively less common. A high mortality (37%) owing to infection was noted. The medical management and recommendations for patient care are discussed briefly.

Acute rheumatic fever in Kuwait: The declining incidence.

The incidence of acute rheumatic fever in children aged 5-14 years in Kuwait was studied prospectively over a period of five years (1984 through 1988). The mean annual incidence in the study period was 2.9/100,000 children. There was a decline in the incidence from 3.7/100,000 in 1984 to 2.5/100,000 in 1988. Twenty-seven percent of children with acute rheumatic fever presented as recurrences in 1985; this also declined to 11% in 1988.

Trends in pediatric casualties in a regional hospital of Kuwait.

The purpose of this study was to statistically analyze the emergency care services of the Paediatric Department of Farwania Hospital, Kuwait. The study was conducted from 1 July 1987 until 30 June 1988, during which a total of 77,497 cases were recorded (41,594 [53.7%] males; 35,903 [46.3%] females). Kuwaiti children represented 40,738 (52.5%) of the total. Children aged 2 to 5 years constituted 22,805 (29.4%) of the cases, and the age group 1 month to 1 year comprised the second largest group (15,475; 19.9%). Admissions peaked in February (8,425 [10.8%]) and Friday was the busiest day (13,561 [17.5%]). There were 61,242 (79.02%) self-referrals. Respiratory diseases were the most common reason for emergency room visits, and totaled 51,583 (66.5%), followed by gastroenteritis with 19,898 (25.7%). This study points up an alarming increase in the number of visits to the casualty clinics of Farwania Hospital. If this increase continues, this will not only markedly increase the burden to staff but will also adversely affect the quality of services.

Cystic fibrosis and Helicobacter pylori gastritis, megaloblastic anaemia, subnormal mentality and minor anomalies in two siblings: a new syndrome?

Two siblings of consanguineous Arab parents were found to have cystic fibrosis and gastritis associated with Helicobacter pylori, folate deficiency megaloblastic anaemia, subnormal mentality and minor anomalies. The association of H. pylori and megaloblastic anaemia has not been described in patients with cystic fibrosis. H. pylori infection and gastritis is probably more common in patients with cystic fibrosis than appreciated. We believe that the constellation of features in the two sibs represent a possible new autosomal recessive cystic fibrosis-like syndrome.

Agalactosyl IgG, antibodies to heat shock proteins, and acute rheumatic fever.

In rheumatoid arthritis an increased proportion of the N-linked oligosaccharides on serum IgG terminate with N-acetylglucosamine (agalactosyl IgG). It has recently been shown that group A streptococcal cell wall peptidoglycan/polysaccharide complex may be used to raise monoclonal antibodies which bind to this glycoform of IgG. Patients with rheumatoid arthritis also have increased levels of antibody to the 65 kD and 70 kD families of heat shock proteins, particularly to a bacterial (Mycobacterium bovis) homologue of heat shock protein hsp65. Streptococci must contain similar heat shock proteins. Acute rheumatic fever follows infection with group A streptococci, and these organisms might theoretically evoke antibody to heat shock proteins or changes in the levels of agalactosyl IgG, which is antigenically cross reactive with their cell walls. It is shown here that serum samples from patients with acute rheumatic fever do not differ from those from normal children by these criteria.

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.

Familial hypophosphataemic rickets: experience with 24 children from Kuwait.

Between 1982 and 1988, familial hypophosphataemic rickets (FHR) was diagnosed in 24 children, in nine during screening of the families of index patients. The average annual incidence was 0.2/1000 live births. There were 16 boys and 8 girls in 10 families, of which nine had more than one affected child. Their ages at the onset of the disease ranged between 10 months and 14 years (mean 6.9 yrs). Growth retardation and bowing of the legs were the most prominent features, observed in all index patients and in four of the patients diagnosed by screening. Treatment with 1 alpha-hydroxyvitamin D3 and phosphates was associated with acceleration of growth in all children, healing of rickets in 21, and normalization of the serum phosphate in 22. Two children with late diagnosis are now older than 16 years with a final height below the 3rd centile. Three more pubertal children are also shorter than the 3rd centile. In areas where nutritional rickets is common, FHR is likely to be missed and the treatment delayed with grave consequences; in particular, growth retardation and bone deformity.

Epidemiology of juvenile chronic arthritis and other connective tissue diseases among children in Kuwait.

An 8-year hospital-based retrospective study on the epidemiology of juvenile chronic arthritis (JCA), systemic lupus erythematosus (SLE) and other connective tissue diseases among children in Kuwait is described. There were 108 children with JCA, 20 children with SLE, 23 children with other connective tissue diseases and 24 children with arthritis of familial Mediterranean fever (FMF). The average annual incidence of JCA was 2.84 cases/10(5) children under the age of 12 years and the 1988 prevalence was 18.7/10(5). The polyarticular, systemic and oligoarticular onset types were observed in 42, 29 and 29%, respectively. The incidence and prevalence of SLE were 0.53 and 3.37/10(5), respectively. The findings are compared with those from other countries.

Vitamin-D-deficiency rickets in Kuwait: the prevalence of a preventable disease.

Two hundred and fifty children with clinical, biochemical and radiological evidence of vitamin-D-deficiency rickets were studied over a period of 5 years. Their ages ranged from 1 month to 2 years. Breastfed infants formed 63% of total cases. Intramuscular therapy with vitamin D in a dose of 600,000 IU, deep intramuscular, proved to be safe and effective. In contrast, oral vitamin D did not provide such satisfactory results, presumably owing to poor patient/parental compliance. This report reveals that vitamin-D-deficieny rickets is common in Kuwait in spite of abundant sunlight all through the year because children are wrapped up and kept indoors. Insufficient intake of vitamin D is another important factor in the pathogenesis of vitamin-D-deficiency rickets in Kuwait.

The value of red cell distribution width in the diagnosis of anaemia in children.

The red cell distribution width index (RDW) was determined in 103 normal children, 69 iron-deficient (ID) patients, 73 with the thalassaemia trait, and 71 with other haemoglobinopathies. Elevated RDW values were found in anaemic patients, the highest values in ID anaemia, sickle thalassaemia, sickle cell anaemia, and beta-thalassaemia trait in decreasing order. The normal RDW in children was 13.2 +/- 0.9 and an elevated RDW reflects active erythropoiesis. The RDW was elevated and the MCV low in all 69 patients with ID anaemia. The RDW was also elevated in 11/13 children with sickle cell anaemia, in 25/29 patients with sickle cell-thalassaemia, and in all patients with thalassaemia major. The ID anaemia could be differentiated from the thalassaemia trait by the markedly elevated RDW in ID anaemia (mean = 20.7 +/- 3.2) and the mildly elevated RDW (mean 15.4 +/- 1.4) in thalassaemia trait. The RDW index discriminated better than Mentzer's index, discriminant function or the Shine Lal index. Thus the RDW is a good indicator of anisocytosis, and a good screening index especially for ID anaemia and the thalassaemia trait.

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)

Congenital chloride diarrhoea in Kuwaiti children.

Congenital chloride diarrhoea was diagnosed in 16 Kuwaiti children over a 7 year period (1980-1986) with an estimated incidence of 7.6 per 100,000 live births. The mean age at diagnosis was 3.2 months (range 1 week to 5 months). There were 9 boys and 7 girls with a mean age of 3 years 10 months (range 10 months to 7 years). All children had a shortened gestational period, abdominal distension and chronic diarrhoea. The serum electrolytes in all patients prior to treatment showed hyponatraemia, hypokalaemia, hypochloraemia and metabolic alkalosis. The diagnosis was confirmed by a stool chloride content that exceeded the sum of faecal sodium and potassium. Fifteen patients survived and showed catch-up growth with adequate replacement therapy and 1 died with renal failure.

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.

Probable transmission of brucellosis from breast milk to a newborn.

The first case of neonatal brucellosis is described. The baby was fed breast milk only when the mother became acutely ill with fever, arthralgia and malaise. At the same time the baby was febrile. Blood cultures grew Brucella melitensis in both mother and child. The brucella agglutination titre in the breast milk was 1:2560. Both mother and child recovered during treatment with streptomycin.