Protease inhibitor-associated angiolipomatosis.

Treatment with protease inhibitors in some persons infected with HIV-1 is associated with a syndrome of lipodystrophy manifesting as peripheral lipoatrophy, relative central adiposity, insulin resistance, and serum lipid abnormalities. We report 3 cases of HIV-1 infected patients who experienced symptomatic angiolipomas shortly after starting antiretroviral therapy including the protease inhibitor indinavir. The mechanism behind this observation may be similar to that of previously reported protease inhibitor-associated fat redistribution, but instead involving the adipose tissue of discrete uncommon benign tumors.

Outcome after surgical repair of junctional epidermolysis bullosa-pyloric atresia syndrome: a report of 3 cases and review of the literature.

BACKGROUND: Junctional epidermolysis bullosa-pyloric atresia syndrome is recognized as a distinct autosomal recessive entity. Affected infants present with skin fragility and inability to feed due to intestinal obstruction. Despite successful surgical repair of the anatomical defect, the outcome is poor owing to poor feeding, malabsorption, failure to thrive, and sepsis. OBSERVATIONS: In 70 cases of intestinal obstruction and epidermolysis bullosa reported in the medical literature and the 3 reported here, surgical intervention was attempted 51 times. In all except 16 infants, death occurred before age 11 months (mean age, 70 days). Renal involvement and continued failure to thrive accompanied the skin disease in survivors, who ranged in age from 30 days to 16 years (mean age, 4.0 years). CONCLUSIONS: The poor prognosis of this condition must be considered when decisions are made regarding surgical correction. Attempting surgical correction may be warranted in individual circumstances, but withholding surgical intervention and providing palliative support is an acceptable alternative.