Spontaneous neonatal gastric perforation.

Over a 7-year period (1990-1997) spontaneous gastric perforation was diagnosed in five neonates. The mean gestational age and birth weight were 33/40 weeks and 1.83 kg, respectively. All patients presented with severe abdominal distention and frank pneumoperitoneum on roentgenograms. All perforations were on the anterior wall of the greater curvature and were managed by prompt laparotomy and primary closure of the perforation. No gastrostomy was used, however, peritoneal drainage was used in all cases. There was no mortality or morbidity. An attempt to understand the possible etiology together with a review of the literature is presented.

Extravasation of parenteral alimentation fluid into the renal pelvis--a complication of central venous catheter in a neonate.

Many complications of central venous catheters, which include perforation of the vessel walls and extravasation of the infusate into pericardial, pleural, and peritoneal cavities, have been reported. We report an infant with a central venous catheter in inferior vena cava who experienced extravasation of parenteral alimentation fluid into the right renal pelvis secondary to perforation of the renal vein. To our knowledge, this rare complication has not been reported earlier.

Prostaglandin induced cortical hyperostosis in neonates with cyanotic heart disease.

OBJECTIVE: To study the side effects of prolonged prostaglandin therapy especially hyperostosis and other musculoskeletal changes. METHODS: Case files of the neonates, with cyanotic heart disease, who had received prostaglandin infusion from early days of life, were reviewed. Patients with periosteal changes were identified. Their radiographs, serum alkaline phosphatase activity, duration and dose of prostaglandin and other side effects related to the prostaglandin were studied. RESULTS: Ten patients developed hyperostosis, who had received PGE1 infusion for a period of 9 to 195 days. Two babies developed coarse facial features, hypertrichosis, and edema of extremities and digital swelling. Serum alkaline phosphatase activity was significantly raised in the patients, with hyperostosis. Besides long bones, ribs and scapulae, the clavicles were also involved. The involvement of clavicles has not been previously reported. CONCLUSION: Hyperostosis is a common side effect of prolonged prostaglandin therapy. Abnormal facial features, hypertrichosis and coarse skin are additional adverse effects of long term therapy. Serum alkaline phosphatase activity can be used as a marker of the hyperostosis. In addition to other bones clavicles can also be involved. The above effects seem to be both dose and duration dependent.

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.

Spectrum of renal pathology in 100 selected children with nephrotic syndrome.

The study includes 100 renal biopsies from an equal number of patients referred to us with nephrotic syndrome (NS). Their ages ranged from one month to 13 years (medial five years) and included 59 males and 41 females. Indications for kidney biopsy were steroid resistance in 41 patients, steroid dependence with frequent relapses on maintenance steroid therapy in 38, NS associated with gross hematuria, hypertension, azotemia, and/or systemic manifestations in eight and age of less than one year at the onset of the disease in 13 patients. In 90 patients, NS was idiopathic in origin and in eight, it was secondary to IgA nephropathy, hemolytic-uremic syndrome, post streptococcal glomerulonephritis, antiglomerular basement membrane antibodies, Henoch-Schonlein nephritis and systemic lupus erythematosus. Two patients had hepatitis B infection and its relationship with NS could not be elucidated. Primary NS is the predominan type of NS in the local pediatric population. The disease in more common in the first year of life. A relatively high incidence of diffuse mesangioproliferative glomerulonephritis (MesPGN) and rarity of infection-related NS are noted.

Etiology, presentation and management of acute renal failure in Saudi children.

One hundred children with acute renal failure (ARF) were admitted in our Nephrology Unit, Maternity & Children's Hospital over a four year period. Male patients outnumbered females with a ratio of 3:2. The ages of patients ranged from 1 month to 13 years with a mean age of 3.9 years. Fifty-five were suffering from intrinsic renal factors, 32 from post-renal and 13 were due to pre-renal factors. Among the intrinsic causes: acute glomerulonephritis (27 patients) and hemolytic-uremic syndrome (15 patients) were most common. Posterior urethral valve (20 patients) was the leading cause of post-renal failure. All pre-natal failure was due to diarrheal disease. Forty patients with ARF required dialysis (23 peritoneal dialysis, 17 hemodialysis). The remaining 60 patients were treated conservatively. The outcome in our patients were as follows; 60% cured, 29% continued to have variable degrees of renal failure and only 11% died. The latter was due to sepsis and cardiorespiratory failure.

Nephrotic syndrome in 1st year of life and the role of unilateral nephrectomy.

The study includes 17 infants with nephrotic syndrome. Their ages at the onset of disease ranged from 10 days to 10 months (median 2 months), and included 10 males and 7 females. Ten patients had consanguineous parents and 4 had a history of similar illness in the sibling. Renal biopsy revealed diffuse mesangial proliferation in 10 patients, diffuse mesangial sclerosis in 3, Finnish microcystic disease in 2, and focal segmental glomerulosclerosis and atypical glomerulopathy in 1 each. Unilateral nephrectomy was performed in 8 patients with intractable proteinuria associated with multiple complications. Five are well after 4-36 months follow-up, 1 died of septicaemia soon after the procedure, 1 needed dialysis and is stable at 26 months and 1 showed no reduction in the degree of proteinuria. We conclude that unilateral nephrectomy is a better alternative to bilateral nephrectomy in some infants with severe nephrotic syndrome.

Acute renal failure in non-fulminant hepatitis A infection.

A 7-year-old boy developed acute renal failure during the icteric phase of non-fulminant hepatitis A infection. He needed peritoneal dialysis for 3 days, which was followed by a rapid recovery in renal function which was normal when he was discharged 4 weeks later.