Hemolytic uremic syndrome in children in Puerto Rico: a rare disease with atypical features.

Hemolytic Uremic Syndrome (HUS) consists ofa triad of acquired hemolytic anemia, thrombocytopenia, and renal failure that occurs acutely in otherwise healthy individuals. HUS may be divided into two broad categories, typical, preceded by a diarrheal prodrome, and atypical. The clinical symptoms of HUS as well as its course, prognosis, and response to treatment appear to be significantly influenced by a number of factors, including age at onset, type and severity of underlying infections, and/or systemic diseases. A retrospective case series review of five patients diagnosed with Hemolytic Uremic Syndrome at the Pediatric University Hospital in Puerto Rico between 1997-2007 was performed. The study showed that the incidence of HUS in children in Puerto Rico is lower than other countries. However, the majority of cases have an atypical presentation, which places our patients at higher risk for life-threatening complications.

Lupus nephritis in Puerto Rican children and adolescents.

BACKGROUND: Lupus nephritis is associated with an unfavorable outcome. Its incidence is higher in children. It has been the impression of pediatric nephrologists and rheumatologists in Puerto Rico that the clinical manifestations in Puerto Rican children differ from what has been described in other geographic regions. The potential deleterious effects of sun exposure could suggest that Puerto Rican children living in the Island are at high risk for severe nephritis. PURPOSE/METHOD: To describe the manifestations and identify predictors of patient and renal survival, we reviewed the medical charts of children diagnosed with lupus nephritis in Puerto Rico between 1987-2001. RESULTS: Mesangial glomerulonephritis (Type II) was the most common histopathological lesion observed. We observed a high incidence of nephritis among males, with a male: female ratio of 1:1.9. CONCLUSIONS: The presence of type IV nephritis and need for acute dialysis at presentation were identified as predictors of poor clinical outcome (development of end stage renal disease or death).