A case of idiopathic diabetes insipidus presented with bilateral hydroureteronephrosis and neurogenic bladder: A pediatric case report and literature review

Abstract

Diabetes insipidus (DI) is a condition with heterogeneous clinical symptoms characterized by polyuria (urine output >4 mL/kg/hr) and polydipsia (water intake >2 L/m 2 /d). In children, acquired nephrogenic DI (NDI) is more common than central DI (CDI). Diagnosis is based on the presence of high plasma osmolality and low urinary osmolality with significant water diuresis. A water deprivation test with vasopressin challenge, though has limitations, is done to differentiate NDI from CDI and diagnose their incomplete forms. Neonates and young infants are better managed with hydration therapy alone. Older children with CDI are treated with desmopressin (1-deamino-8-D-arginine vasopressin, dDAVP). Its oral form is safe, highly effective and has dosing flexibility. We report a case of an 8-year-old male patient with CDI with severe bilateral non-obstructive hydronephrosis and megaureter. Dramatic clinical and radiological responses to dDAVP treatment were achieved and therapy reduced urine volume and led to marked radiological improvement in hydronephrosis.

Resolution of Diabetes Insipidus After Pyeloplasty: A Case Report and Review of the Literature

“…Nephrogenic diabetes insipidus (NDI) is a rare cause of polyuria and polydipsia in children. Its diagnosis requires dilute urine in the presence of elevated serum plasma osmolality [1]. The majority of cases are inherited, but the condition can be acquired as a result of medications, biochemical abnormalities, and/or obstructive uropathy [2].…”

A need for a systematic genetic evaluation of hereditary polyuric patients

“…This is certainly useful for this transplanted patient bearing, with other affected members of his family, a mutation in his AVP gene responsible for autosomal dominant central diabetes insipidus rather than having NDI as previously thought. This hereditary form of central diabetes insipidus is easily treated with dDAVP; this will decrease urine output to less than 2 L per day in an adult and prevent large dilation of the urinary tract and obstructive nephropathy with renal failure, a complication observed with long-term increases in urinary output including psychogenic polydipsia [ 2 ], and nephrogenic [ 3 , 4 ] and central diabetes insipidus [ 5 ].…”

“…Misdiagnoses have also led to chronic renal failure due to obstructive nephropathy. With dDAVP administration, regression of dilation has been observed in central diabetes insipidus [ 5 ] and frequent urination and double voiding urinary dilation also decreases in NDI [ 9 , 10 ].…”

Magnetic Resonance Imaging Findings of Central Diabetes Insipidus in a Child

“…Dysfunction of the system that maintains water homeostasis may result in life-threatening hypernatremia, seizures, dehydration, and failure to thrive [2]. CDI is the end result of a number of conditions that affect the hypothalamo hypophyseal region [1,2]. MR images allow us to clearly delineate the clinoids, the clivus, and the two components of the pituitary gland.…”

“…CDI is characterized by polyuria, polydipsia, and the inability to concentrate urine [1]. Dysfunction of the system that maintains water homeostasis may result in life-threatening hypernatremia, seizures, dehydration, and failure to thrive [2].…”