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Objective: To evaluate the current outcome of a selected prenatally diagnosed spina bifida group. Materials andMethods: We analyzed and followed up 74 cases of prenatally diagnosed spina bifida. Results: Termination of pregnancy was chosen in 72% of the cases and 28% were live-born. Chromosomal defects were identified in 16%, although only 1.6% in isolated cases. Of the 21 live births, 3 died in the neonatal period. The other 18 (86%) were all alive after an average follow-up of 3 years and 6 months (range 5 months to 7 years and 4 months). From this group 11% are wheelchair-dependent, 87% of the patients older than 2 years of age are walking, 33% have had cerebral shunting and 72% have normal neurodevelopment. There was a better outcome in patients with closed defects; however, the rates of neuropathic bladder (50%) remain a concern. Conclusions: Even with prenatal diagnosis and a tendency towards apparently less severe defects in the cases in which the pregnancies continue, the prognosis in terms of morbidity needs to remain guarded.

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Renal tubular acidification function was studied in 12 patients treated with cyclosporine (Cy) for idiopathic uveitis (IU) and in 5 patients with IU not treated with Cy. After intravenous bicarbonate loading fractional bicarbonate excretion was similar in both groups indicating normal proximal tubular acidification function. Plasma renin activity, plasma aldosterone and transtubular potassium gradient were similar in both groups. Distal hydrogen ion secretion evaluated by the ability to increase urine-blood (U-B) pCC₂ in a highly alkaline urine was impaired in Cy-treated patients (31.8 ± 3.2 mm Hg) as compared to controls (47.9 ± 0.5 mm Hg) (p < 0.005). We conclude that Cy therapy is associated with a distal acidification defect with a low U-B pCO₂ gradient during sodium bicarbonate loading. Because none of our Cy-treated patients spontaneously developed overt metabolic acidosis one could classify them as having an incomplete form of distal tubular acidosis.