Congenital nephrotic syndrome and persistent hypothyroidism after bilateral nephrectomy.

Congenital nephrotic syndrome is commonly associated with hypothyroidism. Thyroid hormone supplementation is recommended as standard of care. The hypothyroidism is postulated to occur secondary to chronic massive proteinuria with loss of thyroid binding globulin, thyroid hormone and iodine. Previous reports have indicated that thyroxin may be discontinued following bilateral nephrectomy. We report our experience with one child with congenital nephrotic syndrome, Finnish type, and hypothyroidism who had a high requirement for thyroxin (100-150 microg/d) from infancy to 4 years of age. Hypothyroidism persisted despite bilateral nephrectomy and later following renal transplantation. However, his thyroxin requirement is now substantially lower (62.5 microg/d) at age 14 years. No goiter was detected clinically and antithyroid antibodies were negative. Thyroid ultrasound and 123I scan revealed a thyroid gland in the anatomically normal location. 123I uptake was elevated, 18% at 6 hours and 51% at 24 hours (normal values: 3-16% at 6 hours and 8-25% at 24 hours). Perchlorate was unavailable for a perchlorate washout study. We speculate that this patient may have an intrinsic problem with thyroid hormone synthesis. It is unclear whether this is related or coincidental to the Finnish nephrotic syndrome. We recommend following thyroid functions closely if thyroxin is discontinued following bilateral nephrectomies in Finnish type congenital nephrotic syndrome.

Acute renal failure.

Acute renal failure (ARF) can be defined as the sudden loss of adequate renal function to clear metabolic wastes and maintain normal fluid and electrolyte balance. ARF may occur in otherwise healthy children, may complicate underlying chronic kidney disease, or may result from multiorgan disorders. The underlying cause of the renal injury remains the major factor that determines outcomes for patients with ARF. Overall mortality in children with ARF varies from 8% to 89%, with greater than 50% mortality associated with three-organ system failure. Management of the adolescent with ARF ranges from conservative management in mild cases to more intensive care in hospitalized patients with complications of fluid overload, hypertension, metabolic acidosis, or life-threatening hyperkalemia.

Chronic kidney disease in adolescents.

Chronic kidney disease (CKD) involves a fixed deficit or progressive deterioration in kidney function, producing variable losses of normal physiologic functions. In adolescents, CKD results from a wide range of causes. Because of the broad spectrum of disease, the physician providing primary care to the adolescent must be knowledgeable about findings leading to the diagnosis of CKD and must understand the physiologic basis for therapeutic management. Complications of normal renal function loss include issues of fluid, electrolyte, and acid-base balance, as well as development of anemia, cardiovascular disease, metabolic bone disease, and growth failure. Goals of therapy include anticipation and replacement of lost physiologic functions so that the adolescent can grow and develop normally.