Bartter's syndrome in Arabic children: review of 13 cases.

BACKGROUND: Bartter's syndrome (BS) is an inherited disease of renal potassium wasting characterized by hypokalemic alkalosis, normal blood pressure, vascular insensitivity to pressor agents and elevated plasma concentrations of renin and aldosterone. It is caused by generalized hyperplasia of the juxtaglomerular apparatus at the site of renin production caused by mutations in the Na-K-2Cl cotransporter gene, NKCC2. The objective of our study is to establish the prevalence and incidence of BS in Kuwait and to assess treatment modalities for it. METHODS AND RESULTS: Bartter's syndrome was diagnosed in 13 Kuwaiti children over a 14 year period (1981-1995) with the estimated incidence of 1.7/100,000 live births. The mean age at diagnosis was 9.3 months (range 2-32 months). There were five males and eight females (ratio 1:1.6). The mean duration of follow up was 5.6 years (1-14 years). Both consanguinity and familial history among our patients were high (69 and 54%, respectively). All patients had hypokalemia, hypochloremia with metabolic alkalosis, hyperreninemia and were normotensive. Clinical presentation was essentially similar to that in other series. Eleven patients (85%) had growth failure, two had nephrocalcinosis (15%) and one had renal failure. All patients were treated with supplemental potassium, an aldosterone antagonist (spironolactone) and a prostaglandin synthetase inhibitor (indomethacin or aspirin) sequentially. Significant catch-up of growth (four patients) and increases in serum potassium (eight patients) were recorded after administration of indomethacin therapy. One patient died of severe pneumonia with respiratory failure from hypokalemic myopathy. Clinical presentation, inheritance, complications and therapy of BS are briefly discussed. CONCLUSION: Bartter's syndrome is a rare disease, but should be considered in the differential diagnosis of other disorders with growth failure and/or hypokalemia. Early diagnosis, close follow up and compliance with treatment may lead to appropriate growth and development.

Acute rheumatic polyarthritis. The duration of secondary prophylaxis.

Antistreptococcal prophylaxis was discontinued in a group of 44 children who had acute rheumatic polyarthritis with no carditis or chorea during the initial attack and who had completed a mean period of 5 years of secondary prophylaxis and freedom from recurrences. Patients were then followed up for a mean period of 5 years (an observation period of 216 patient-years). Recurrence of disease occurred in 3 patients, with a recurrence rate of 1.4 per 100 patient-years. There was no evidence of carditis during these recurrences and all patients remained free of rheumatic heart disease 10 years after the initial attack. These data demonstrate the relative safety with which continuous antistreptococcal prophylaxis can be discontinued in a selected group of children with rheumatic fever.

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.

Microbial etiology of acute gastroenteritis in hospitalized children in Kuwait.

During a period of 15 months 621 hospitalized children with acute gastroenteritis and 152 control children were investigated for etiologic agents of the disease. Putative enteropathogens were identified in 86% of the patients and 10% of the controls. Common viral agents associated with gastroenteritis among children included rotaviruses (45%) and enteric adenoviruses (4%). Bacterial pathogens infecting children were Salmonella serotypes (24%), enterotoxigenic Escherichia coli (9%), Campylobacter jejuni (7%), enteropathogenic E. coli (7%), Shigella (4%) and enterotoxigenic Aeromonas sp. (1%). The highest incidence of infections was observed in the 3- to 25-month age group. Mixed infections were observed in 12% of the patients. Viral gastroenteritis was clinically mild and of short duration. Upper respiratory tract infections, vomiting and watery stools were common features. In contrast bacterial gastroenteritis was more severe; stools were frequently bloody and abdominal pain, cramps, shock, convulsions and milk intolerance were predominant clinical features. Comparative analysis revealed differential features of bacterial and viral gastroenteritis which should help clinicians to make a tentative diagnosis and to start treatment early.

PIP: During a 15-month period, 621 hospitalized children with acute gastroenteritis and 152 control children were investigated for etiologic agents of the disease. Putative enteropathogens were identified in 86% of the patients and 10% of the controls. Common viral agents associated with gastroenteritis among children included rotaviruses (45%) and enteric adenoviruses (4%). Bacterial pathogens infecting children were Salmonella serotypes (24%), enterotoxigenic Escherichia coli (9%), Campylobacter jejuni (7%), enteropathogenic E. coli (7%), Shigella (4%), and enterotoxigenic Aeromonas sp. (1%). The highest incidence of infections was observed in the 3-25 month age group. Mixed infections were observed in 12% of the patients. Viral gastroenteritis was clinically mild and of short duration. Upper respiratory tract infections, vomiting, and watery stools were common features. In contrast, bacterial gastroenteritis was more severe; stools were frequently bloody and abdominal pain, cramps, shock, convulsions, and milk intolerance were predominant clinical features. Comparative analysis revealed differential features of bacterial and viral gastroenteritis which should help clinicians to make a tentative diagnosis and to start treatment early.

Pattern of protracted diarrhoea among children in Kuwait.

A total of 92 children with protracted diarrhoea were studied over a 3-year period (October 1983 to September 1986). The diagnosis was postgastro-enteritis syndrome in 61 (66.2%), coeliac disease in 17 (18.5%), Giardia lamblia infestation in 5 (5.4%), immune deficiency in 4 (4.4%), congenital chloride diarrhoea in 2 (2.2%), and glucose-galactose malabsorption, Shwachmann syndrome and intestinal lymphangiectasia in one each. Children with the postgastro-enteritis syndrome were young, malnourished and bottle-fed, and suffered a high incidence of antecedent bacterial infections. Six children died, a case fatality ratio of 6.5%.

Antibiotic misuse in a paediatric teaching department in Kuwait.

Of 3813 children admitted to the paediatric teaching unit in Mubarak Hospital during 1985, 716 (19%) received either therapeutic (672) or prophylactic (44) antibiotics. The indications were respiratory infections (400), urinary infections (78), septicaemia (58), meningitis (42), gastroenteritis (32), other infections (62) and prophylaxis in rheumatic fever or splenectomy (44). Penicillins were prescribed in 553 children, ampicillins being the most frequently prescribed drugs (295, 41%). Antibiotic use was assessed as unnecessary in 282 children, of whom 203 (72%) had respiratory infections. Overall mistakes were encountered in 352 cases, of which 238 (68%) were in respiratory infections and 302 (86%) involved a penicillin, single or in combination. Mistakes were also commoner with combination than with single antibiotic therapy (66% and 44%, respectively). A departmental policy for the use of antibiotics in gastro-enteritis led to their prescription in only 4% of cases. A written justification for cefotaxime reduced its use by 90%. The lack of similar policies for antibiotic use in respiratory infections leads to high rates of antibiotic use and misuse, probably world-wide.

Acute gastroenteritis: clinical features according to etiologic agents.

The clinical manifestations in 595 children hospitalized with gastroenteritis during a 15-month time frame were studied. They were divided into eight groups according to etiologic agent: rotavirus (203 patients); Salmonellae (98); Escherichia coli (55); Campylobacter (36); Shigella (22); combined rotavirus and salmonellae (44); combined rotavirus and other bacteria (26); and no pathogen (111). The mean duration of diarrhea was shortest in the rotavirus and "no pathogen" groups (4.8 and 5.6 days, respectively) and longest with pure and mixed salmonella infections (12.3 and 12.9 days, respectively). Associated manifestations were most frequent with salmonellae and least frequent with rotavirus and E. coli infections. Malnutrition also was most common with salmonellae and lowest with rotavirus and E. coli. There were no differences in the frequency of hypernatremia. Hyponatremia was most frequently encountered with salmonella (25% compared to 9% in the rest of the patients). Evidence of septicemia was found in 22 patients, 21 of whom were in the salmonella groups. The four deaths in this series (0.7%) also were in the salmonellae groups. The clinical severity of salmonella infection in developing countries, particularly in young and malnourished children, warrants attention to more intensive management. The selective use of antibiotics may help reduce the mortality and morbidity of gastroenteritis.

Coeliac disease among children in Kuwait: difficulties in diagnosis and management.

Twenty children with coeliac disease were diagnosed over a five year period in an area with 10,000-12,000 births per year. The average annual incidence was 1:3000 births. All children presented with severe symptoms and rickets was not uncommon (25%). Mean age at onset of symptoms was 38 months (range 6-120) and 72 months at the time of diagnosis (range 13-192), with a mean delay of 34 months. No cases were diagnosed during infancy. The difficulties in the differentiation of coeliac disease from the more common causes of chronic diarrhoea, and problems with diagnosis and management are discussed.

Children with acute rheumatic fever and acute poststreptococcal glomerulonephritis and their families in a subtropical zone: a three-year prospective comparative epidemiological study.

Over a period of three years (December 1980 through November 1983) the incidence and epidemiological features of acute rheumatic fever (ARF) and acute poststreptococcal glomerulonephritis (AGN) were studied prospectively in two regional hospitals in Kuwait serving a childhood population of 225,000. The study included 146 children with ARF and 256 family members and 125 children with AGN and 199 family members. The annual incidence of ARF and AGN were 19.6 and 17.8 respectively per 100,000 childhood population (7.3 and 6.7 respectively per 100,000 total population). Both diseases occurred sporadically throughout the year with a similar peak in winter. The clinical profile of ARF was essentially similar to that reported from temperate climates, and AGN followed mainly throat infections. Microscopic haematuria was detected in ten ARF family members (4%) and in 20 AGN family members (10%). Haematuria and low C3 were found in two (0.8%) and in seven (3.5%) ARF and AGN family members respectively. Of the nine family members with subclinical nephritis the group C streptococcus was isolated from three (33%). The geometric mean titre (GMT) of antistreptolysin O (ASO) and of antihyaluronidase (AH) in ARF and AGN patients were markedly elevated. Although the median age of ARF and AGN family members were 13 and 15 years respectively, yet the GMT of ASO and AH in the family members were slightly higher than those of the normal childhood population.

The concurrent associations of group A streptococcal serotypes in children with acute rheumatic fever or pharyngitis-associated glomerulonephritis and their families in Kuwait.

A group of 146 children with acute rheumatic fever and 256 members from their families, and a group of 125 children with post-streptococcal glomerulonephritis and 199 family members, together with a group of 145 children with non-complicated group A streptococcal pharyngitis were examined over a period of three years. The purpose of the study was to explore the concurrent association and distribution of group A streptococcal serotypes among the three groups. Strains isolated from the children with non-complicated group A streptococcal pharyngitis represented the prevalent strains of group streptococci in the childhood community during the period of study. Rheumatic fever was encountered in a non epidemic situation. As expected, the recovery of group A streptococci was low. The strains however belonged mainly to two patterns, namely type M1 and M non-typable strains. Nephritis was pharyngitis-associated and occured also sporadically throughout the year. Types M12 and 49 accounted for the majority of the isolates. Type M12 accounted for 34.4% of the group A isolates from family members of children with nephritis and was totally absent in family members of children with rheumatic fever (p less than 0.001). The T pattern 8/25/Imp. 19 accounted for 40% of the group A isolates from family members of rheumatic patients compared to 3.3% from family members of nephritis patients (p less than 0.005). Data from this study show that the group A streptococcal serotypes, concurrently isolated from children with acute rheumatic fever and their families are disparately different from those of children with pharyngitis-associated glomerulonephritis and their families in the same population. These findings support the concept of "nephritogenicity" and "rheumatogenicity" and indicated the important role of the biological characteristics of the streptococcal serotypes in the aetiology of acute rheumatic fever and acute poststreptococcal glomerulonephritis.

Sporadic pharyngitis-associated acute poststreptococcal nephritis. A four-year prospective clinical study of the acute episode.

Between December 1980 and November 1984, the acute episode was studied prospectively in 187 children under 14 years presenting with acute poststreptococcal glomerulonephritis. The mean annual incidence was 19.5/10(5) children. The disease was mainly pharyngitis-associated and occurred throughout the year but peaked in the winter. The mean age was 5.4 years, and 71 percent of children were under the age of 8 years. Gross hematuria was the presenting feature in 93.6% of cases and was usually associated with edema. Hypertension was present in 45 percent, circulatory congestion in 27 percent, and heavy proteinuria in 22 percent of cases. The C3 level was low in 97 percent of the patients tested and returned to normal within 6-8 weeks. There were no deaths. Only one patient needed peritoneal dialysis. All except one patient were asymptomatic with normal creatinine within 8 weeks. The authors adopted a very optimistic attitude in the counseling of children with the disease and of their families.

The natural history of acute rheumatic fever in Kuwait: a prospective six year follow-up report.

One hundred and twenty-six children with the initial attack of acute rheumatic fever were followed up prospectively for 6 years. Sixty-six children maintained regular secondary prophylaxis (regular group) and 60 were irregular (irregular group). Two recurrences developed in the regular group with a recurrence rate of 0.005/patient/year follow-up, and 71 recurrences developed in the irregular group with a recurrence rate of 0.2/patient/year follow-up. These findings demonstrate the effect of secondary prophylaxis in reducing the frequency of recurrences. The prevalence rate of rheumatic heart disease in children who had carditis in the initial attack was 42% in the regular group vs 70% in the irregular group (p less than 0.05). These findings demonstrate the deleterious effect of recurrences in the evolution of rheumatic heart disease. The prevalence rate of rheumatic heart disease in children who maintained regular secondary prophylaxis, was 42% in those children who had carditis in the initial attack and 6% in those who had no carditis (p less than 0.05). These findings demonstrate the prognostic significance of presence or absence of carditis during the initial attack, in the subsequent evolution of rheumatic heart disease. The prevalence rate of rheumatic heart disease in the 66 children who maintained regular prophylaxis was 23%. Comparison of these data with those of similarly designed studies shows that the evolution of rheumatic heart disease following the initial attack of acute rheumatic fever, seems to behave similarly in the tropics and subtropics as it did in temperate climates.

Reversible renal failure in a child with brucellosis: a case report.

Bilateral kidney enlargement and renal failure are described in a five-years-old girl with brucellosis. The diagnosis was made on the basis of a rising brucella agglutination titre. Both the function and the size of the kidneys returned to normal after four weeks treatment with co-trimoxazole. Renal brucellosis may stimulate renal tuberculosis or chronic pyelonephritis and should be considered in areas where brucellosis is endemic.

Coeliac disease in monozygotic twin girls. Synchronous presentation.

A pair of monozygotic twin girls with coeliac disease is reported. The diagnosis was made on clinical and biochemical evidence of malabsorption, characteristic histological findings, and clinical, biochemical, and histological response to gluten elimination. Monozygosity was established on finding a single placenta at birth, exact similarity of physical appearance, similar blood group, and histocompatibility antigens, and negative reaction in mixed lymphocyte culture. This is one of six well documented cases of coeliac disease in monozygotic twins and may throw light on the importance of genetic and environmental factors in the causation and expression of the disease.

Basic epidemiological aspects of acute gastro-enteritis in a regional hospital in Kuwait.

Over a 12-month period 1045 children were hospitalized because of acute gastroenteritis. The disease accounted for 26% of admissions and for 20% of the total bed utilization. Prevalence peaked twice (autumn and spring), was low during summer and lowest in winter. Only 20% of children were breastfed on admission and in the great majority of those who were not, breast feeding was stopped under the age of two months. Early cessation of breast feeding may be responsible for the increased frequency of gastroenteritis in very young infants, and should be a cause for major concern in developing countries.