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Renal involvement is common in patients with bacterial endocarditis. The most common bacteria are staphylococci and streptococci, and the commonest renal histopathological lesion is a diffuse proliferative and exudative type of glomerulonephritis. Very rarely, patients may present with an extensive glomerular epithelial crescent formation with a rapid deterioration in the renal function. This study reviews the published literature on diffuse crescentic glomerulonephritis in bacterial endocarditis and reports a 24-year-old male patient with endocarditis due to Capnocytophagia species, a gramnegative facultative anaerobic bacillus, which normally inhabits the oral cavity. Appropriate antibiotic therapy is essential to eradicate the infection. A brief course of corticosteroid therapy may be helpful in those with deteriorating renal function. Plasmapheresis may be useful in those with persistent hypocomplementemia, increased circulating immune complexes, and a progressive deterioration in the renal function. Removal of vegetation or valve replacement may be necessary. Prognosis is generally good.

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Connexins (Cx) are a family of proteins that constitute the intercellular membrane channels of gap junctions. These junctions permit intercellular movement of ions and other molecules between cells, a property vital to organogenesis. Cx43 is a member of the family of channel-forming proteins that are essential for cell-cell communication of developmental signals. Studies demonstrate that Cx43 is observed in mesenchymal cells of 12-day gestation mouse kidney, a crucial period of renal development. In order to study the significance of Cx43 on renal developmental morphology, we evaluated the kidneys of embryos lacking the gene encoding for Cx43. Polymerase chain reaction (PCR) from tail specimens identified wild-type (WT), heterozygote (HT) and knockout (KO) progeny. In situ RT-PCR displayed abundant Cx43 staining in glomeruli, vasculature, and tubules in kidneys obtained from WT progeny. In contrast, Cx43 expression was completely absent in kidneys isolated from the KO. Renal histology in all three groups displayed no significant differences. Renal size was similar and there was no evidence of dysplasia or cyst formation in the KO. Our results indicate that absence of Cx43, heretofore considered essential for renal development, does not affect early renal morphological development.

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Relapses are common in children with idiopathic nephrotic syndrome, and this prospective study looks into the possibility of decreasing the frequency of relapses in a selected group of such patients. The study includes 36 children with a steroid-dependent, relapsing nephrotic syndrome on a maintenance prednisone therapy of about 0.5 mg/kg every other day. They were prospectively divided into two groups with comparable age and sex distribution and the number of those who had previously received cyclophosphamide therapy. Group 1 patients were advised to take daily prednisone for 5 days, starting at the time of the onset of an upper respiratory tract infection (URI). No such advice was given to those in group 2, and they remained on alternate-day prednisone during URI. At the end of a 2-year follow-up period, the total number of relapses in group 1 was 40 with a mean of 2.2 +/- 0.87 per patient as compared with 99 with a mean of 5.5 +/- 1.33 per patient in group 2 (p = 0.04). We conclude that an increased maintenance prednisone during URI helps decrease significantly the number of relapses in those on alternate-day therapy.

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OBJECTIVE: . To review some of the recent recommendations on blood pressure (BP) cuff selection, including those by the Task Force and its update, and to survey the perceptions on cuff selection among practitioners. METHODS: The study was conducted in two parts. In the first, we selected three brands of commonly used infant, child/pediatric, small adult, adult, and large adult BP cuffs. From the median width of each, we derived the required upper arm length (UAL) for the cuff to cover three quarters or two thirds of the UAL, and with the help of a published normal UAL at various ages, we matched the derived UAL with the corresponding age at the 50th percentile. Similarly, we derived the midupper arm circumference (UAC) so that the available cuffs would cover 40% of the UAC, and by using the published normal UAC at various ages, we matched the derived UAC with the corresponding age at the 50th percentile. The second part of the study involved a survey by multiple choice questionnaire mailed to 400 hospital- and office-based pediatricians, residents, and nurses at the Children's Hospital of Michigan. Included in the survey were questions about the age at which practitioners would choose the cuffs described above; the minimum age they would consider using an adult cuff in a pediatric patient with an average height and weight; selecting a cuff using UAL as a criterion; selecting a large versus a small cuff when the appropriate cuff size is not available; and the Task Force definition of hypertension. RESULTS: Using three quarters of the UAL as a criterion, it seems that a large adult cuff should be appropriate for an average-size 6-year-old child and that using two thirds of UAL as a criterion, the cuff should be appropriate for an average-size 7- to 8-year-old child. Similarly, by using 40% of the mid-UAC as a criterion, an adult cuff would be of no use in an average pediatric patient at any age. Our survey revealed that 57% of practitioners would consider using a neonatal cuff for patients up to 1 month of age, 65% would use an infant cuff for those 1 year of age, 49% would use a child/pediatric cuff for those 5 years of age, and 84% would use a small adult cuff for those 10 years of age and older. Most (83%) of the practitioners would consider using an adult cuff in children 11 years of age and older. Practitioners are likely to use a smaller cuff than is appropriate by two thirds or three quarters of UAL criteria, and a larger cuff than is appropriate, particularly in older children, by 40% of UAC criteria. Using UAL as a criterion, a majority (59%) of practitioners use cuffs that cover two thirds of the distance between the axilla and the cubital fossa. Ninety-two percent of practitioners believe that a smaller cuff causes a moderate to significant increase in the BP reading, and 55% believe that a larger cuff causes a similar decrease in the BP reading. A significant number of practitioners (44%) did not know the Task Force definition of hypertension, including 42% of attendings, 44% of residents, and 50% of nurses. CONCLUSIONS: The Task Force and the Working Group recommendations on BP cuff selection need to be reviewed. A new multicenter study, using uniform criteria for cuff selection, may be necessary to establish the accuracy of the published nomogram on normal BP in children. More awareness is required on part of practitioners of the current recommendations on BP measurement and the definition of hypertension. Finally, the labeling of BP cuffs as infant, pediatric, small adult, adult, and large adult is misleading, and such designations should be eliminated. Cuff sizes should be standardized, indicate bladder size, and be uniformly color-coded for convenience.

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A significant number of renal diseases in children occur as a result of genetic susceptibility. It is believed that such diseases, particularly those with an autosomal recessive transmission, may be more common in places with a high rate of consanguineous marriages as in the Middle East including the Kingdom of Saudi Arabia (KSA). Although a comparison of the published literature from the KSA with that from other countries outside the region support such a possibility, too few studies have been published from the region to definitely answer the question. Preliminary observations indicate that the children in the Kingdom probably have a higher incidence of polycystic kidney disease, familial juvenile nephronophthisis, congenital urological anomalies and familial nephrotic syndrome. It is reasonable to speculate that whereas some diseases may be common, others may be rare because of a low gene frequency in the local population.

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Acquired renal cystic disease (ARCD) is a well-known complication of end-stage renal disease (ESRD). We studied 24 patients, aged 8-27 years (mean 19.8 +/- 5.3 years), on chronic maintenance dialysis in our service. The duration of dialysis ranged between 13 and 192 months (mean 77.8 +/- 44.3 months). High-resolution ultrasonography revealed ARCD in 11 (45.8%) patients. No cysts were seen in 7 (29.1%) patients and solitary cysts in one or both kidneys were seen in 6 (25%) patients. Renal malignancy was diagnosed in 2 patients. One, 15 years old, had renal cell carcinoma after being on dialysis for 6 years. She did well after bilateral nephrectomy, left salpingo-oophorectomy, and regional lymphadenectomy. The second patient, 23 years old, had been on dialysis for 16 years when she developed renal oncocytoma. She died of congestive cardiomyopathy 6 months later. We conclude that ARCD is common in children and young adults with ESRD. Neoplastic transformation, although rare, is a potential complication. Annual follow-up with ultrasonography with selective use of computed tomography or magnetic resonance imaging is advised.

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A 6-year-old girl had a group A beta-haemolytic streptococcal (GABS) throat infection and Henoch-Schonlein purpura (HSP). The clinical course was complicated by nephrotic syndrome due to crescentic glomerulonephritis, transient neurological symptoms due to focal ischaemia of the brain, and congestive cardiac failure due to myocarditis. The clinical presentation highlights the diversity of systemic involvement in HSP, the transient nature of apparently serious central nervous system involvement, and a possible role of GABS in its aetiology.

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Thyroid function indices were studied in five children with nephrotic syndrome in the 1st year of life. Four had primary hypothyroidism as defined by low serum free tri-iodothyronine (FT3) and free thyroxine (FT4), and high serum thyroid-stimulating hormone (TSH) levels. One patient with low serum FT3 and FT4 had a normal TSH level. T4 replacement therapy lowered TSH to normal levels in all four patients and normalized FT4 in three of them. There were no significant changes in serum FT3 levels. Adrenal function was studied in three patients, none had adrenal calcification or hypoadrenocorticism. This study supports the existence of a hypothyroid state in some infants with nephrotic syndrome. Routine thyroid screening and early replacement therapy is recommended.

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The relationship between nephritogenic strains of group A beta-hemolytic streptococci (GABS) and acute poststreptococcal glomerulonephritis (APSGN) is well established. Recent studies have cast some doubts on the importance of the M-antigen which is widely used in classifying the nephritogenic strains of GABS. The renal injury occurs as a result of an immune-mediated process which involves the complement system. This leads to deposition of circulating immune complexes and/or their in-situ formation in the kidney resulting in renal damage. Newer antigenic fractions have been identified in GABS, which include; endostreptocin (ESS), nephritis strain associated protein (NSAP), and pre-absorbing antigen (Pa-Ag). These developments have allowed a better insight into the pathogenesis of APSGN.

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The study includes 15 children (8 males, 7 females) with autosomal recessive polycystic kidney disease (ARPKD) whose ages at diagnosis ranged from 2 days to 7 years (median 10 months). Eleven (73.3%) patients were hypertensive on admission and 1 developed hypertension 4 months later; 5 patients became normotensive after receiving treatment for 18-36 months (mean 23.2 months). Patients were followed for a period of 1-48 months (mean 20.9 months). Glomerular filtration rate remained normal in 7 patients, improved in 4 and deteriorated in 1. Two patients died soon after diagnosis and 1 was lost to follow-up and is assumed dead. Of the 4 patients less than 6 months old at the time of diagnosis, only 1 is alive compared with 10 of 11 presenting after 6 months of age. The cumulative chance of survival from the time of diagnosis was 85% at 3 months and 77% at 6 months. The study highlights the reversible nature of hypertension in ARPKD. Survival is better in patients older than 6 months at the time of diagnosis and those surviving 6 months follow-up.

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Three newborn babies with acute renal failure were started on peritoneal dialysis 3, 4 and 3 days after abdominal surgery for intestinal perforation, intestinal malrotation with volvulus and hepatic laceration, respectively. Peritoneal access was achieved by Trocath catheters in the first two and via a Jackson-Pratt peritoneal drain inserted during surgery in the third baby. Two babies survived after dialysis for 9 and 6 days. The third baby was dialysed for 16 days, had partial recovery in renal function and died of multisystem failure at the age of 35 days. Peritoneal dialysis is possible in neonates with recent major abdominal surgery. Occasionally, a peritoneal drain inserted during surgery may provide an easy access for the procedure.

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Eighty children with End Stage Disease (ESRD) were treated in our unit over a six year period. Forty-eight were treated with CAPD (mean age = 5.8 years) and thirty-two with HD (mean age = 8.2 years). The average duration of treatment was 14.8 months in the CAPD group and 14.2 months in the HD group. There were 22 failures of peritoneal catheter in the CAPD group out of 70 catheters compared to 19 failures of vascular access devices out of 45 in the HD group. Peritoneal catheter failure was due to resistance or recurrent peritonitis in 10 (45.4%) and obstruction in nine (41%), whereas vascular access devices failure was due to thrombosis in six (31.5%) and infection in five (26.3%). Fifteen (31.3%) of the CAPD patients died and eight (16.7%) transferred to HD, whereas five (15.6%) HD patients died and four (12.5%) transferred to CAPD. The three year actuarial rates for CAPD were 81% at one year, 55% at two years and 42% at three years, while for HD was 94%, 85% and 64%, respectively. In this unique experience at the Kingdom so far, we found that a fully integrated service of dialysis including both CAPD and HD are essential. Such a system allows the optimal mode of treatments to be chosen for a child at any time and allows the child to move freely from one treatment to another when needed.

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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.

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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.

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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.

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The study includes 30 children, 10 each with minimal change nephropathy (MCNS), diffuse mesangial proliferative glomerulonephritis (MesPGN) and focal segmental glomerulosclerosis (FSGS). Indications for kidney biopsy were: steroid resistance in 12 cases, and frequent relapses on maintenance steroid therapy in 18 cases. A 2-year remission was achieved by cyclophosphamide therapy in 6 patients with FSGS, 8 patients with MCNS and 8 patients with MesPGN. In terms of initial response to corticosteroids, only 3 of 8 patients with early steroid resistance responded to cyclophosphamide therapy compared with 3 of 4 with late steroid resistance and 16 of 18 with frequent relapses on maintenance steroid therapy. Response to cyclophosphamide therapy correlates better with the initial corticosteroid response than renal histopathology. A kidney biopsy prior to administration of cyclophosphamide is not essential in patients who respond to initial corticosteroid therapy.

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