HLA antigens in Arab children with steroid-responsive nephrotic syndrome.

Forty-eight Arab patients with steroid-responsive childhood nephrotic syndrome were studied for the frequency of HLA-A, -B, -C and -DR antigens. HLA-DR7 was significantly increased in the patient group (63% vs. 28%, P = 0.0002) confirming reports of a DR7 association in other ethnic groups and indicating a universal association with this antigen. HLA-CW6 was also significantly increased (44% vs. 21%, P corr. = 0.042). HLA-DQW1 was significantly reduced in the patients (29% vs. 57%, P corr. = 0.012) as was HLA-CW4 (6% vs. 24%, P corr. = 0.042).

Cutaneous leishmaniasis in Kuwait. Clinical experience with itraconazole.

Twenty-four patients suffering from single or multiple lesions of cutaneous leishmaniasis were included in this study. Most of the lesions were on the extremities. The patients were randomly divided into two groups. Most of the patients in the first group who were given oral itraconazole for a period of 6-8 weeks showed excellent clinical response. On the other hand, only one patient in the second control group who was given placebo showed good clinical improvement. Systemically administered itraconazole may prove to be a valuable modality for the treatment of cutaneous leishmaniasis.

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.

Successful pregnancies following remission in childhood acute lymphoblastic leukaemia--case report.

We report two successful pregnancies in a 17-year-old Arab girl who had received modern combination chemotherapy and central nervous system minimal disease therapy for childhood acute lymphoblastic leukaemia at the age of 9.5 years.

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.

Congenital hypothyroidism in Kuwait.

During a 7-year period (1981-1987), 25 children with congenital hypothyroidism were diagnosed in a regional hospital in Kuwait. The calculated incidence was 1:3476 live births. The diagnosis relied on clinical manifestation and confirmed by thyroid function tests. The age at diagnosis ranged between 3 weeks and 7 years with a mean of 18.5 months. Seven patients (28 per cent) were diagnosed in the first months, six in the following 5 months (24 per cent), and 12 diagnosed after the age of 6 months (48 per cent). Thyroid scan (TC-99M) was performed for 13 patients, four had thyroid aplasia, three had ectopia, and six had thyroid in normal position. In 1986-1987, two patients were detected by neonatal screening. Mental development was the best in the children who received thyroid hormone replacement in the first 6 months of life. Until a neonatal screening programme is widely available in developing countries, increased clinical awareness will remain the main way for the detection of congenital hypothyroidism.

Prolonged and secondary fever in childhood bacterial meningitis.

In order to study the causes of prolonged and secondary fever in bacterial meningitis, a group of 102 infants and children with proven bacterial meningitis were studied. The causative agent was Haemophilus influenzae in 58% of patients, Streptococcus pneumoniae in 25% and Neisseria meningitidis in 17%. Prolonged fever was observed in 12% of the patients. The established causes include, in order of frequency, subdural effusion, drug fever, otitis media, gastroenteritis and urinary tract infection. Secondary fever was noted in 18% of the patients. The causes, in order of frequency, were urinary tract infection, subdural effusion, otitis media, phlebitis, pneumonia and drug fever. Neither relapse of the meningitis nor inadequate response to antibiotic therapy was the cause for prolonged or secondary fever. Neurological sequalae were observed in 21 patients. There was no correlation between prolonged or secondary fever and neurological sequalae. We conclude that prolonged and secondary fever in patients with treated bacterial meningitis is rarely caused by the primary infection.

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.

Macrosomia, microphthalmia, +/- cleft palate and early infant death: a new autosomal recessive syndrome.

An Arab girl with macrosomia, severe microphthalmia and early infant death is reported. Four other sibs were similarly affected; three of them had median cleft palate. All five sibs showed respiratory infections in early life and died either unexpectedly or because of a documented overwhelming infection. Parental consanguinity and affected sibs of both sexes strongly suggest autosomal recessive inheritance in this apparently new syndrome.

A new autosomal recessive disorder resembling Weaver syndrome.

Most reported cases of Weaver syndrome are sporadic, and the mode of inheritance is still unclear. We describe two (male and female) sibs born to consanguineous Bedouin parents with manifestations resembling Weaver syndrome. Both sibs had accelerated growth of prenatal onset, hypotonia, psychomotor retardation, excess loose skin, peculiar craniofacial and acral anomalies, dental dysplasia and/or serrated gums, joint laxity, and hoarse low-pitched cry. One of them had an accelerated harmonic skeletal maturation. Differentiating features from Weaver syndrome are discussed, and autosomal recessive inheritance is suggested.

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.

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)

New autosomal recessive faciodigitogenital syndrome.

Most pedigrees of Aarskog's faciodigitogenital syndrome have suggested X linked inheritance. However, sex influenced autosomal dominant inheritance is also a possibility in some families. We describe an Arab family of normal consanguineous parents with five children (three males and two females) with some features of Aarskog syndrome in addition to some unusual hair changes. The possibility that this family represents a distinct previously unrecognised faciodigitogenital syndrome with short stature and hair abnormalities is suggested and discussed.