Peritonitis in continuous ambulatory peritoneal dialysis in children living in Saudi Arabia.

The clinical aspects of peritonitis and catheter infections were reviewed in 64 children on continuous ambulatory peritoneal dialysis living in Saudi Arabia over a period of 6 years. Peritonitis occurred in 41 children (64%). The mean time from starting dialysis to the first episode of peritonitis was 7.2 months. The incidence of peritonitis was 1 episode in 9 treatment months. Gram-negative organisms were responsible for the majority of episodes (42%), followed by Gram-positive organisms (20%), and Candida albicans (6%); 32% were culture negative. Recurrent peritonitis was present in 20 cases. Catheter was replaced in 24 patients: 44% due to recurrent peritonitis. Peritoneal membrane loss occurred in 7 patients, 3 had Candida peritonitis and 3 had recurrent peritonitis due to Pseudomonas. The mortality rate was 4.6% but none of the deaths were related to peritonitis or dialysis.

Brucella endocarditis associated with glomerulonephritis and renal vasculitis.

We report a patient with a peculiar association of brucella endocarditis, glomerulonephritis, and renal vasculitis, with immunopathological features different from those of endocarditis glomerulonephritis. Renal biopsy revealed moderate diffuse hypercellularity involving the mesangium as well as capillary loops, immunoflurescence revealed no staining for IgG, IgM, IgA, C3, and fibrinogen. The clinical and biochemical evidence of glomerulonephritis disappeared after antibiotic treatment combined with steroid therapy. No similar case has been previously reported in the literature.

Thin-section computed tomography scans detect medullary cysts in patients believed to have juvenile nephronophthisis.

Juvenile nephronophthisis (NPH) and medullary cystic kidney disease are an important cause of chronic renal failure in children and young adults. Medullary cysts are regarded as the hallmark of this condition. The diagnostic techniques that were used previously were not conclusive in most cases to demonstrate medullary cysts. We studied the role of thin-section (1.5-mm) computed tomography (CT) scan as an optimal imaging diagnostic technique. Three children who were admitted to our hospital consecutive with a clinical diagnosis of NPH were included. We found that the thin-section CT scan demonstrates the medullary cysts in all patients. We recommend CT scan as the investigation of choice in patients with clinical features suggestive of nephronophthisis-cystic renal medulla complex.

Hemolytic uremic syndrome - clinical aspects and outcome of an outbreak: Report of 28 cases.

Hemolytic uremic syndrome (HUS) is characterized by microangiopathic hemolytic anemia, thrombocytopenia and acute renal failure. There are two main subgroups: the typical form of HUS follows a diarrheal prodrome (D+HUS) and the atypical form is without the diarrheal prodrome (D-HUS). We have studied 28 children with HUS over a period of 15 months between 1992 and 1993. The median age was 2.2 years (range from six months to six years). All children had prodromal diarrhea. Hypertension was present in 71% and neurological complications in 39%. All the patients had oliguria or anuria (16 oliguric and 12 anuric). The mean duration of anuria was 16 days (range seven to 42 days). Serum creatinine on admission ranged between 112 and 1064 Amicromol/L (mean 453 Amicromol/L). The lowest hemoglobin level and platelet count during hospitalization ranged between 38 and 87 g/L and 7 to 147x109/L respectively. Leukocytosis on admission was present in 22 patients, low C3 was documented in 11 patients (34%), and four patients had low C4. All patients received fresh frozen plasma transfusion, a total of 25 patients received dialysis therapy, 19 patients were treated with peritoneal dialysis (PD), one patient had hemodialysis (HD), and five patients had both HD and PD. The mean duration of dialysis was 18 days (range three to 56 days). Only one patient died (mortality rate 3%). The median duration of hospital stay was 28 days (range eight to 90 days). We conclude that HUS is emerging as an important clinical and public health problem and that early comprehensive management including dialysis therapy, aggressive management of hypertension, fresh frozen plasma transfusion, and nutritional support all improve the outcome and decrease the mortality and morbidity in patients with HUS.

Sister chromatid exchange analysis in monitoring chlorambucil therapy in primary nephrotic syndrome of childhood.

Sister chromatid exchange (SCE) analysis is the most sensitive method for assessing chromosome damage induced by chemical mutagens. We report the SCE of peripheral blood lymphocytes in children with primary nephrotic syndrome (NS) treated with chlorambucil. Group I consisted of 20 normal children, group 2 of 14 children with primary NS who had never received a cytotoxic drug and group III of 7 children with primary NS who had received chlorambucil, which was discontinued 6-36 months prior to the study. Group IV consisted of 4 nephrotic children who were receiving chlorambucil therapy during the study. There was no significant increase in SCE in group III compared with group I or group II (P much greater than 0.05). A significant rise in SCE (P less than 0.05) was seen in all patients in group IV.

Evaluation of chlorambucil therapy in steroid-dependent and cyclophosphamide-resistant children with nephrosis.

A prospective study was performed to evaluate the efficacy of chlorambucil in inducing long-term remission in children with steroid-dependent or cyclophosphamide-resistant primary nephrotic syndrome (NS). The 16 steroid-dependent children had a relapse rate of 5.3/patient per year (range 4-8) and a mean age of 8.4 years (range 2-13). The chlorambucil dosage was 0.2 mg/kg per day for a total of 8 weeks. Nine patients (56%) remained in complete remission for an average of 39.2 months (range 16-70). The interval to the first relapse in the remaining 7 ranged from 4 to 41 months and the relapse rate decreased significantly to 0.6 relapses/patient per year (P less than 0.05). Five children had steroid- and cyclophosphamide-resistant NS, 4 with focal segmental glomerulosclerosis and 1 with mesangiol proliferative glomerulonephritis. Chlorambucil was given in a dose of 0.2 mg/kg per day for 8-16 weeks. Complete remission was obtained in 4 patients; 2 patients relapsed. No serious long-term complications were observed in our patients.

Frequent relapser minimal change nephrosis: an unrecognized X-linked disorder?

We report three brothers who developed nephrosis between the age of 3-10 years. The parents were nonconsanguineous and of Arab descent. The mother's sister had a son with a similar condition. Patients were steroid responders and frequent relapsers. Renal biopsies in the three brothers showed findings of minimal change nephrosis. This family may suggest the existence of an X-linked recessive nephrosis which provides further evidence for genetic heterogeneity of familial nephrosis.

Serum concentrations of vitamin D metabolites in rachitic Libyan children.

Twenty-two consecutive patients with rickets were studied in Benghazi, Libya. All were less than 2 years old. Rickets was associated with traditional cultural habits that limited sunshine exposure of the mothers and their infants, and with breast-feeding. Serum concentrations of the vitamin D metabolites 25-hydroxyvitamin D (25-OHD), 1,25-dihydroxyvitamin D, and 24,25-dihydroxyvitamin D, and other parameters of mineral metabolism were typical of vitamin D deficiency disease, as was the biochemical and clinical response to treatment. Minimum safe serum levels of 25-OHD (20 nmol/L), and the serum levels of vitamin D metabolites in response to vitamin D treatment, were identical to previously obtained results from native Norwegian and Norwegian immigrant children with rickets, suggesting lack of racial differences in response to vitamin D. Fifty percent of the patients had adequate levels of vitamin D metabolites at the time of diagnosis, indicating that they had recently received oral vitamin D or cutaneous exposure to sunshine. Many cases of rickets in the area may, therefore, be spontaneously cured when the children's maturity allows adequate mobility and independence to achieve exposure to sunshine.

Long-term, small dose prednisone therapy in frequently relapsing nephrotic syndrome of childhood. Effect on remission, statural growth, obesity, and infection rate.

A prospective study was performed to evaluate the effect of long-term small-dose prednisone therapy in frequently relapsing nephrotic syndrome (NS); 37 patients were included, with a relapse rate 4.6/patient/year (range, 3-8) (mean age, 6.7 years; range, 2-15 years). Prednisone was started 2 mg/kg/day once remission was induced. Prednisone was progressively reduced over weeks until 10 mg/day was reached, and then the daily dose was changed to 10 mg on alternate days. On follow-up (mean, 25.4 months; range, 10-58 months), only five had subsequent relapses and their relapse rate decreased significantly to 1.2/patient/year (p less than 0.05). Forty-six episodes of infection were associated with exacerbation of NS; 41 of these excerbations remitted spontaneously without an increase in the dose of prednisone. Serial height and weight measurements revealed evidence of improved height velocity and obesity persisted in only two of 13 initially obese children.

Perinatal outcome at Benghazi and implications for perinatal care in developing countries.

PIP: Using 1984 perinatal mortality rates as indicators of the level of maternal and newborn health care service quality and efficiency, Libya's high perinatal loss is compared to perinatal losses of 9 other developing countries. Timely antenatal care is identified as the essential component needed to reduce perinatal loss. Libyan perinatal, still birth, and early neonatal death rates were 26.3, 11.4, and 14.9/100, respectively. Perinatal death rates of other countries in the study ranged from 18.8 to 100/thousand. The major causes of still births in Libya included antepartum hemorrhage, cord accidents, maternal diabetes mellitus, and fetal malformations. The effect of timely obstetric care in reducing still birth rates (SBR) is evidenced by comparing SBRs of 16.8 to 63.8 in pregnant women receiving or not receiving minimal antenatal care at a peripheral health center, respectively. The clinical causes of early neonatal death were major congenital malformation (24.9%), hyaline membrane disease and aspiration syndrome (26.1%), birth asphyxia and injury (17.9%), very low birth weight (17.2%), and sepsis/meningitis (13.1%). High general fertility rates of developing countries leads to increased proportions of women under 20 and over 35 years of age bearing children. These women are prone to bearing offspring comparatively more vulnerable to early neonatal death. Consanguineous marriages leading to congenital malformation, and lack of maternal immunization with tetanus toxoid are also cited as factors contributing to high perinatal mortality. In closing, the authors call for future community-based studies, and recognize socioeconomic level as a main determinant in obtaining obstetric care.^ieng

Mechanical ventilation in newborn infants.

A 20-month experience of mechanical ventilation (MV) in the newborn infants (birth weight greater than or equal to 1500 g) from a developing country is described. A total of 41 neonates (4.1% of total admissions to the Neonatal Intensive Care Unit) were treated with MV. The mode of MV was intermittent positive pressure ventilation and continuous positive airway pressure via nasotracheal intubation. The mean birth weight and gestational age were 2544 g and 36.2 weeks, respectively. The mean age at the start of MV was 141 h and the mean duration was 54 h. The indications for MV were respiratory distress syndrome (18), aspiration pneumonia (8), non-aspiration pneumonia (6), apnoea (8) and tetanus neonatorum (1). The complications encountered during MV were sepsis (26.8%), pulmonary haemorrhage (21.9%), congestive heart failure (17.1%), pneumothorax (14.6%) and intraventricular haemorrhage (7.3%). Post-extubation atelectasis was observed in 29.6% of cases. The overall survival rate was 43.9%. The risk factors for a poor outcome were birth weight less than 2000 g, prematurity and late referrals to the Neonatal Intensive Care Unit.