Clotilde Druck Garcia

Peripheral gangrene complicating idiopathic and recessive hemolytic uremic syndromes

Abstract Three patients with hemolytic uremic syndrome (HUS) developed peripheral gangrene. Bilateral carotid artery thromboses occurred in one of these patients after recovery from HUS. One patient had a long history of juvenile rheumatoid arthritis. In the second patient, a flu-like illness preceded the onset of HUS. The third was one of two sisters, with the HUS appearing more than 1 year apart. None had evidence of disseminated intravascular coagulation or infection with Streptococcus pneumoniae. The patient with rheumatoid arthritis had renal cortical necrosis but recovered moderate renal function after treatment with dialysis and plasmapheresis for 6 months. The child with a genetic form of HUS died of renal failure and had massive cortical necrosis and vascular thrombosis at autopsy. This is the first report of peripheral gangrene in children with idiopathic HUS and autosomal recessive HUS.

Paediatric dialysis and renal transplantation in the state of Rio Grande do Sul, Brazil

Renal replacement therapy (RRT) for Brazilian children with uraemia has been utilized since 1970 in the state of Rio Grande do Sul. One hundred and eighty patients receiving this therapy between 1970 and 1988 have been reviewed. The annual acceptance rate of new paediatric patients in this period increased from 0.6 to 6.5 patients per million child population. Glomerulonephritis (36.1%) and pyelonephritis including urological anomalies (31.7%) were the most frequent causes of end-stage renal disease. Outpatient hospital haemodialysis was the primary form of dialytic treatment in patients 5–15 years of age. Continuous ambulatory peritoneal dialysis was more often used in patients less than 5 years of age. The survival after 1 year on dialysis was 79.9% for children aged 5–15 years starting dialysis during the period 1985–1988. Fluid overload with congestive heart failure and infection were the main causes of death in children on dialysis. Eighty-four children received 93 grafts; only 14 (15%) were from cadaveric donors. One-year patient and graft survival of first living-related donor transplants were 92.2% and 78.5% respectively during the period 1985–1988. Infection accounted for 43.5% of deaths after transplantation. We conclude that RRT is becoming increasingly successful for children in our region but that greater emphasis upon patient compliance with all forms of RRT and upon cadaver kidney donation is needed.

Lipoprotein glomerulopathy: a case report of a rare disease in a brazilian child

Lipoprotein glomerulopathy (LPG) is a rare autosomal recessive glomerulopathy associated with the deposition of lipoprotein thrombi in the capillary lumina due to apoE gene mutations. Abnormal plasma lipoprotein profile and marked increase in serum apoliprotein E (apoE) are characteristic clinical data. The compromised patients can present nephrotic syndrome, hematuria, and progressive renal failure. Herein, the authors present the first described case of LPG in a Brazilian male patient, 11 years, who presented with a steroid-resistant nephrotic syndrome. Renal function was normal. Kidney biopsy showed markedly enlarged glomerulus, with dilated capillary loops and weak eosinophilic lipoprotein thrombi in the capillary lumina. Interstitium, tubules, arteries, and veins showed normal histologic aspect. Genotypic study for the apoE gene showed the presence of the alleles E3 and E4. The diagnosis of LPG was then performed. The patient received lipid-lowering treatment. After 2 years of follow-up, renal function is gradually decreasing, with persisting heavy proteinuria, despite a marked decrease in serum cholesterol and triglycerides levels.