Over an 8-year period (January 1996 to December 2003), a total of 171 patients below the age of 15 years were diagnosed with chronic renal failure. The mean incidence rate of CRF in Kuwaiti children was found to be 38.2 per million children per year, with a peak incidence of 55 per million children per year. While the mean age at diagnosis was 33+/-12 months (range: 1 month to 15 years), the male:female ratio was 2.7:1. Etiological factors for chronic renal failure included congenital urological malformation (61.9%), chronic glomerulopathies (5.2%), hereditary nephropathies (21%), multi-system disease (0.5%), chronic pyelonephritis (without VUR) (4.6%), tumors (0.6%), ischemic renal disease (1.1%) and unknown etiology (1.7%). Thirty percent of patients reached end-stage renal disease within a mean of 18 months following diagnosis. The overall mortality before reaching ESRD was reported to be 4%. Kuwait has one of the highest incidence and prevalence rates of CRF in children. It is likely that genetic and hereditary factors are the cause of these high rates.
The distinctiveness of IgM nephropathy (IgMN) as a clinicopathologic entity is controversial. Twenty-seven children (16 males, 11 females) with IgMN as defined immunohistochemically by diffuse mesangial staining of glomeruli for IgM were compared to a group of 63 children (40 males, 23 females) with minimal change disease (MCD). While mesangial expansion was significantly greater in IgMN than in MCD (p = 0.0014), there were no significant differences between the two groups with respect to the other biopsy factors. IgMN showed a significantly higher incidence of hypertension at presentation. More than 90% of patients in both groups presented with the nephrotic syndrome which in most initially responded to prednisone. Frequently relapsing/steroid-dependent nephrotic syndrome was the most common indication for biopsy in both groups. Approximately 60% of patients from both groups received cytotoxic therapy. Eight percent of IgMN and 7% of MCD patients failed to respond to therapy. Relapse rates and mean dose of prednisone at relapse were very similar in both groups prior to biopsy. Relapse rates diminished significantly after treatment in the postbiopsy interval, but mean dose of prednisone at relapse did not change appreciably over time. None of the patients developed renal failure or hypertension in the follow-up period. At last visit 23% of IgMN and 27% of MCD had proteinuria. The results indicate that IgMN and MCD are indistinguishable clinically in children who are biopsied for the nephrotic syndrome.
We have studied the effect of HLA-DRB1 alleles on the clinical presentation of 61 Kuwaiti Arab children with idiopathic nephrotic syndrome. DR7(*0701) was the most prevalent DR allele, found in 41/61 patients (67%) compared with 10/59 healthy controls (17%) (p<0.001). DR3(*0301-0308) allele was the second most common, found in 25% of patients compared with 26% of controls (not significant). There was no significant difference between DRB1*0701(DR7)-positive and DRB1*0701-negative patients in terms of steroid sensitivity, steroid dependency, or steroid resistance. Nevertheless, the former group had a significantly lower mean age of onset (35 months vs 53 months) and a shorter remission period following treatment with cyclophosphamide or chlorambucil (8 months vs 29 months). Our data highlight the role of the DRB 1*0701 allele in predisposing Kuwaiti Arab children with idiopathic nephrotic syndrome to a more prolonged course of the disease.
Our data suggest an association of D allele of the ACE gene insertion/deletion polymorphism and congenital urological abnormalities, which result in parenchymal damage in Kuwaiti Arab children.
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