Focal Segmental Membranoproliferative Glomerulonephritis: A Histological Variant of Denys-Drash Syndrome.

Introduction: Denys-Drash Syndrome (DDS) consists of a triad of pseudohermaphroditism, Wilms'tumor and nephropathy. This condition may manifest as a complete triad or in an incomplete form; with either one or a combination of the above features. The characteristic glomerular abnormality in DDS is diffuse mesangial sclerosis (DMS).Case report: We report two cases of DDS with focal membranoproliferative glomerulonephritis (MPGN). Both of our cases were males with ambiguous genitalia. They had a similar heterozygous germline mutation in exon 9 of WT1, c.1180C>T, p.R394W; a known mutation hotspot for DDS. Case 1 had nephropathy at the age of 4 years and Case 2 at 2.5 years with different rates of progression to end-stage renal failure. Conclusion: Our findings, in combination with other reports, illustrate the clinicopathological heterogeneity of DDS. There are no universal recommendations for optimal management of patients with DDS due to the inability to accurately predict affected individuals' progress.

Cochrane Eyes and Vision Group (CEVG).

This is a new column for reviews from the Cochrane Eyes and Vision Group (CEVG). Its main aim is to promote access to systematic reviews of all the interventions used to prevent or treat eye diseases. It is based at the London School of Hygiene & Tropical Medicine in London, UK, and Johns Hopkins Bloomberg School of Public Health in Baltimore, Maryland, USA.

Variation of blood pressure during topical phacoemulsification.

BACKGROUND: The changes of blood pressure in patients undergoing cataract procedure are not well studied. The blood pressures of cataract patients often become uncontrolled intraoperatively causing the procedure to be postponed. Intraoperative rise in blood pressure has been associated with ocular complications such as suprachoroidal haemorrhage and can be fatal from stroke or even myocardial infarction. We attempt to study the changes in blood pressure of patients undergoing cataract surgery. AIM: Prospective study on the variation of blood pressure pre- and intra-operatively. METHOD: The blood pressure readings of patients going for cataract procedures were measured in five stages during the pre-assessment clinic, on arrival at the day surgery, at the anaesthetic room, on the operating table intraoperatively; and postoperatively. The readings were compared to their age, sex, past medical history, and use of antihypertensive medication. RESULTS: The rise in systolic blood pressures between intraoperative readings and those at the preassessment and anaesthetic room was highly statistically significant.Being older (>65 years of age), taking more than two antihypertensive drugs, being hypertensive, or having a family history of hypertension does not appear to increase the risk. CONCLUSION: Our study showed that there is a significant rise in systolic blood pressures especially in females during topical phacoemulsification. Further studies are needed on the factors that determine this increase and the changes in blood pressures during different stages of the procedure.

Lupus nephritis in children in Malaysia.

OBJECTIVES: To determine the pattern of renal histology, clinical outcome of children with lupus nephritis and to identify any associated risk factors predicting renal failure in these children. METHODS: Retrospectively, 27 children under 16 years of age with lupus nephritis who had renal biopsies done at Sultanah Aminah Hospital Johor, Malaysia from 1994 to 2002 were studied. The renal histology was graded according to WHO classification system (1982). The medical records, laboratory data and the clinical outcome of the patients were studied. RESULTS: There were 24 cases of WHO Class IV, two cases of WHO Class II and a case of WHO Class V. Twenty children were in the good renal outcome group while six children progressed into the poor renal outcome group and required renal replacement. One child was lost to follow-up. All six children in the poor renal outcome group had WHO Class IV histology. The 5-year patient and renal survival rates were 84% and 75%, respectively. Age, sex, activity and chronicity indices in the renal histology, anaemia, elevated serum creatinine, depressed levels of C3 and C4, heavy proteinuria or presence of urinary active sediments were not associated with progression to renal failure. CONCLUSIONS: Presently, children with lupus nephritis appeared to have better patient and renal survival rates. Assessment of renal histology in these children was important for diagnosis, treatment and probably prognosis. In this study, there was a 25% incidence of loss of renal function over 5 years in children with WHO Class IV renal histology.

Biopsy-proven childhood glomerulonephritis in Johor.

BACKGROUND: There has been no published study of biopsy-proven childhood glomerulonephritis in Malaysia. OBJECTIVES: To determine the pattern of childhood glomerulonephritis in Johor, Malaysia from a histopathological perspective and the various indications used for renal biopsy in children. MATERIALS AND METHODS: Retrospective study was done of all renal biopsies from children under 16 years of age, received in Sultanah Aminah Hospital, Johor between 1994 and 2001. The histopathological findings were reviewed to determine the pattern of biopsy-proven glomerulonephritis. The indications for biopsy, mode of therapy given after biopsy and the clinical outcome were studied. RESULTS: 122 adequate biopsies were received, 9 children had repeat biopsies. Of the 113 biopsies, minimal change disease formed the most common histopathological diagnosis (40.7%) while lupus nephritis formed the most common secondary glomerulonephritis (23.0%). The main indications for biopsy were nephrotic syndrome (50.8%), lupus nephritis (25.4%) and renal impairment (13.1%). The mode of therapy was changed in 59.8% of the children. Of 106 patients followed-up, 84 children were found to have normal renal function in remission or on treatment. 4 patients developed chronic renal impairment and 16 reached end stage renal disease. Five of the 16 children with end stage disease had since died while 11 were on renal replacement therapy. Another 2 patients died of other complications. CONCLUSIONS: The pattern of childhood GN in our study tended to reflect the more severe renal parenchymal diseases in children and those requiring more aggressive treatment. This was because of our criteria of selection (indication) for renal biopsy. Renal biopsy where performed appropriately in selected children may not only be a useful investigative tool for histological diagnosis and prognosis but may help clinicians plan the optimal therapy for these children.