Failure of clinical and laboratory characteristics to differentiate mesangial proliferative from minimal-change nephrotic syndrome.

Twelve clinical and laboratory characteristics of nephrotic syndrome were compared in 24 children with biopsy-proven mesangial proliferative glomerulonephritis (MesPGN) and 17 children with biopsy-proven minimal-change nephropathy (MCNS). The objective of the study was to determine if these characteristics alone, without renal biopsy, could be used to differentiate the two histopathologic entities. Sex, urinary protein level and IgM immunofluorescence were found to be significantly different in the two groups. Discriminant analysis produced two formulae which gave a discriminant rate of 79% for MesPGN and 76% for MCNS. We conclude that the clinical and laboratory characteristics studied could not differentiate MesPGN from MCNS.

Glucose-galactose malabsorption with renal stones in a Saudi child.

A case of glucose-galactose malabsorption in an 18-month-old Saudi girl is presented. She had associated bilateral renal stones with impaired renal function. Dietary therapy improved her malabsorption and the renal stones were cleared by extracorporeal shortwave lithotripsy.

Henoch-Schönlein syndrome in Saudi Arabia.

A retrospective study was carried out on 40 children from Saudi Arabia with Henoch-Schönlein syndrome to delineate its clinical pattern. More than 50 per cent of the cases occurred in winter. There was no apparent causal relationship with B-haemolytic streptococcal infection. All patients had skin rash. Fifty-eight per cent had gastrointestinal manifestations, 58 per cent had joint manifestations and 38 per cent had renal manifestations. The main clinical features of Henoch-Schönlein syndrome in Saudi children are comparable to those reported elsewhere.

Acute renal failure in neonates: A prospective study.

Babies admitted to a neonatal intensive care unit in whom acute renal failure developed were studied prospectively to determine the etiology, course, and outcome of the disorder, as well as the reliability of renal indices in distinguishing intrinsic from prerenal renal failure. The incidence of neonatal acute renal failure was 3.6% (22/603). The most frequent causes were hypotension (86%), respiratory distress syndrome (41%), sepsis (32%), and asphyxia (27%). Half of the patients were born at less than 32 weeks' gestation and had a birth weight of less than 1500 gm. There was intrinsic renal failure in 15 neonates and prerenal failure in seven. Renal indices (urine-to-plasma ratio of osmolality, fractional excretion of sodium, and renal failure index) were not reliable in differentiating between intrinsix or prerenal failure. Mortality from renal failure was high at 77%. Survivors of neonatal acute renal failure who had no underlying congenital anomalies of the urinary tract did not appear to exhibit residual renal impairment during follow-up evaluation. It is important to identify those clinical indices that can be used in determining the diagnosis and prognosis of acute renal failure in neonates, particularly in very premature, low-birth-weight babies.

Haemostatic measurements in childhood nephrotic syndrome.

Blood coagulation and platelet aggregation were assessed in children with nephrotic syndrome who were divided into the following groups: (1) relapse without treatment: (2) relapse on steroids; (3) early remission; (4) late remission and (5) steroid resistant. The renal histological findings were also recorded. Plasma anti-thrombin III (ATIII) levels were markedly reduced in groups 1 and 2, below normal in group 3 and were normal in groups 4 and 5. There was significant urinary loss of ATIII in groups 1 and 2 as well as in group 5. Plasma fibrinogen fluctuations exhibited the expected negative correlations with plasma ATIII. Reptilase time showed significant prolongation in groups 1, 2 and 3, and was near normal in groups 4 and 5. Platelet aggregation in response to arachidonic acid exhibited aggregation followed by disaggregation in groups 1, 2, 4 and 5, and was normal in group 3. Hyperaggregation in response to decreasing doses of ADP was noted in all patient groups as well as controls with no relationship to serum albumin levels. Aggregation responses to collagen and ristocetin were normal. It is concluded that: 1. The fluctuations in ATIII levels in childhood nephrotic syndrome are determined by the response to steroids and not by the renal histology per se. 2. An acquired fibrin polymerization defect (dysfibrinogenaemia) and an abnormality of the prostaglandin pathway of platelet activation, both reversible, are yet other haemostatic abnormalities in childhood nephrosis. 3. The discrepancies in the literature on haemostatic parameters, specially ATIII in childhood nephrosis, would not have arisen if their fluctuation in relation to steroid therapy as well as the renal histological features of nephrotic syndrome had been documented simultaneously.

Postrenal-biopsy hematoma in infants and children: evaluation by ultrasonography.

Postbiopsy hematoma was assessed using ultrasonography in 106 infants and children (age 1 month to 15 years) who underwent a total of 122 percutaneous renal biopsies. Postbiopsy hematoma occurred in 22 (21%) of 105 successful biopsies. However, only 2 of these were symptomatic. Renal failure, mild and moderate hypertension and inexperience of the operator were not found to increase the incidence of postbiopsy hematoma in infants and children.

The outcome of percutaneous renal biopsy in children: an analysis of 120 consecutive cases.

One hundred and twenty percutaneous renal biopsies performed in 104 patients aged 1 month to 15 years were analysed. Ultrasound examination was used for localization of the kidneys. Adequate renal tissue was obtained in 103 biopsies, with an overall success rate of 85.8%. The most frequent complication was gross haematuria, which occurred in 32 biopsies, but only 5 children required blood transfusion. Nineteen patients developed perirenal haematoma; 2 of these were symptomatic. Arteriovenous fistula was diagnosed in 2 patients. One patient died secondary to intestinal perforation. Serious complications were noted in patients with chronic renal failure. The success and complication rates in 10 patients less than 1 year of age were comparable with the rest of the group. The frequency of serious complications on renal biopsy in the present study was slightly higher than in the more developed countries.

Clinicopathological features of childhood nephrotic syndrome in northern Nigeria.

The clinicopathological features of childhood nephrotic syndrome in northern Nigeria were studied in 100 consecutive patients. The patients presented with gross anasarca and very low serum albumin, which was less than 15 g/l in 30 patients. The three most frequent histological diagnoses in 98 renal biopsies were membranoproliferative glomerulonephritis (25), quartan malarial nephropathy (20), and proliferative glomerulonephritis (19): together they accounted for 65 per cent of all biopsies. Only nine patients had minimal change nephropathy. Antigens were detected by immunofluorescence in the glomeruli of 70 of 76 biopsies (92 per cent): Plasmodium malariae was detected in 25 per cent and hepatitis B surface antigen in 24 per cent. The disease was characterized by progressive deterioration in renal function and a high mortality rate of 13 per cent. Nine of the 13 deaths occurred within one year of diagnosis.

Childhood renal disorders in Nigeria.

The common childhood renal disorders in Nigeria are post-infectious acute glomerulonephritis (PIAGN), nephrotic syndrome, hypertension, congenital anomalies and urinary tract infection. Children with PIAGN often present with hypertension, circulatory overload and hypoalbuminaemia. Nephrotic syndrome is characterized by a paucity of minimal change and a poor prognosis. Posterior urethral valves and hydronephrosis are the most frequent congenital anomalies. Children with congenital anomalies present late with advanced disease. The commonest malignancy is nephroblastoma, but Burkitt's lymphoma of the kidney also occurs. Both acute and chronic renal failure present depressing and distressing clinical problems, with an aggressive course and a high morbidity and mortality. Poor socio-economy has adverse effects on the presentation, management and follow-up of Nigerian children with renal disorders.

Clinicopathological features of childhood nephrotic syndrome in Saudi Arabia.

The clinicopathological features are described in 119 Arab children in Saudi Arabia with the nephrotic syndrome. The clinical and laboratory data are similar to those described in other parts of the world. However, mesangial proliferative glomerulonephritis (MesPGN) was found in 21 of 66 biopsies (31.8%), giving a frequency of 17.6% of all children with the nephrotic syndrome. Minimal-change nephrotic syndrome (MCNS) was diagnosed in 17 biopsies (25.8%) and in 58 patients (48.7%). Onset of the nephrotic syndrome was at less than 1 year of age in 17 patients (14.3%). Seven children had 11 episodes of peritonitis. Seven children had positive hepatitis B surface antigen (HBsAg) in their serum: renal biopsy carried out on four of them showed membranous glomerulonephritis (MGN) in three, and four of the seven patients developed end-stage renal disease (ESRD). There were nine deaths, all in patients with end-stage renal disease: six of the deaths occurred in infants. The pattern of childhood nephrotic syndrome in Saudi Arabia is different from the pattern in tropical countries.

Proteinuria selectivity index in Saudi children with nephrotic syndrome.

Proteinuria selectivity index (PSI) was determined in 54 Saudi children with nephrotic syndrome whose renal biopsy showed minimal change in 13 and non-minimal histologic changes in 41 patients. The selectivity was determined with two pairs of proteins, IgG/transferrin and IgG/albumin. There was considerable overlap in the values of PSI in patients with minimal and non-minimal histological changes. The clinical utility of PSI in differentiating minimal change nephrotic syndrome and non-minimal change nephrotic syndrome is low.

Nephrotic syndrome and primary tubular amyloidosis in a child: a case report.

Nephrotic syndrome in a 19-month-old child was associated with an unusual form of primary renal amyloidosis. The amyloid deposit was in the tubulo-interstitium and not in the glomeruli. Although the deposit did not stain with Congo red, it had the ultrastructural characteristics of amyloid. The patient had resistant nephrotic syndrome, and he subsequently developed microhaematuria and glucosuria.

Nephrotic syndrome in Saudi infants in the first year of life.

Sixteen Saudi children with onset of nephrotic syndrome in infancy were seen at King Khalid University Hospital, Riyadh over a 5-year period. This figure represented 17% of the 92 cases of childhood nephrotic syndrome seen during the period. Onset of the nephrotic syndrome was less than or equal to 3 months of age in four patients. Ten of the patients developed renal failure. Eight patients died, seven of them by 1 year of age. Two patients given renal transplants have functioning grafts without recurrence of the disease. Renal biopsy in 12 patients showed congenital nephrotic syndrome of the Finnish type (4 cases), 2 each of congenital glomerulosclerosis, mesangioproliferative glomerulonephritis and minimal change disease, and 1 each of focal segmental glomerulosclerosis and tubular amyloidosis. Prognosis of infancy-onset nephrotic syndrome in Saudi children was poor. A significant prognostic factor appeared to be the age of onset of the disease rather than the histological lesion.

Hepatitis B surface antigen associated nephrotic syndrome.

Hepatitis B virus (HBV) has been reported in association with the nephrotic syndrome from different parts of the world, but its role as a cause of the pathological findings of nephrotic syndrome is still controversial. We report seven nephrotic children with positive hepatitis B markers in which members of their families were also positive for the markers but without clinical, renal or hepatic involvement. Four showed haematuria at onset and three developed hypertension later in the course of the disease. Only two were responsive to steroid therapy. Renal biopsy was performed in four, of whom three showed membranous nephropathy and the other showed mesangioproliferative glomerulonephritis. Four patients developed end-stage renal disease. We conclude that in our environment HBV, when detected in children with nephrotic syndrome, should not be considered as a chance finding, but may have a definite role in its pathogenesis. Moreover, the prognosis of HBV-associated nephrotic syndrome appears poor.