Although angiotensin-converting enzyme inhibitors are mentioned as a potential treatment, they have not been studied sufficiently to provide dosing guidelines. Oligomeganephronia is a chronic progressive renal disease leading invariably to renal failure. Assessment of renal function, biochemical status, and growth is necessary to manage this condition and provide optimal care. Complications of chronic renal disease include growth failure, metabolic bone disease, anemia, metabolic acidosis, and other disorders of fluid and electrolyte metabolism. If an associated syndrome is present, offer genetic counseling and education to the patient and family.
Genetic counseling is very important in such families.
Renal ultrasound from a newborn with spontaneous pneumothorax, preauricular pits, and branchial cysts. The right kidney was absent, and the left kidney was hyperechoic and hypoplastic. Oligomeganephronic renal hypoplasia. Oligomeganephronic renal hypoplasia with tapetoretinal degeneration. Report of one case with ultrastructural study of the renal biopsy.