Highlights Segmental Hypoplasia with Hypertension (Ask-Upmark Kidney)
We report 27 patients who presented with segmental hypoplasia (SH) and hypertension. Twenty were girls and hypertension was rarely discovered before 8 years of age. Corticopapillary scarring was unilateral in 7 cases and bilateral in the remaining 20. Nephrectomy performed in 11 cases led to normalization of blood pressure (BP) in only 4. Death occurred in 2, BP was well controlled in 2 and poorly controlled in 3. The remaining 16 children were treated with hypotensive drugs. At latest followup, 8 had progressed to terminal failure and 6 had a well controlled BP with stable renal function. In one patient there was a decreased GFR while BP was well controlled and in the remaining one, renal function was stable while BP was poorly controlled.