Progression of chronic renal failure in children with dysplastic kidneys

Abstract

The aim of this study is to describe progression of chronic renal failure (CRF) in children with renal malformations and to study factors influencing this progression. We reviewed retrospectively 176 children with CRF secondary to renal dysplasia, reflux nephropathy or renal obstruction with at least 5 years of follow-up. Serum creatinine was recorded at least every third month, and an estimated glomerular filtration rate (eGFR) was calculated. Number of febrile urinary tract infections (UTI), blood pressure, albuminuria (UaUc), and number of functioning kidneys was also recorded. We found that the development of renal function could be separated into three time periods: (1) During the first years of life, 82% of the children showed early improvement of their kidney function, which lasted until a median age of 3.2 years (median improvement 6.3 ml/year). (2) From the age of 3.2 years until 11.4 years, 52.5% of the studied children showed a stable kidney function, whereas in 47.5%, kidney function immediately started to deteriorate. (3) Around puberty, 42.9% started deterioration in kidney function, whereas 57.1% even after puberty showed a stable function. Patients with UaUc >200 mg/mmol deteriorated faster (-6.5 ml/min per 1.73 m(2) per year compared with -1.5 ml/min per 1.73 m(2) per year) in those with UaUc <50 mg/mmol. Children with more than two febrile UTIs, hypertension or an eGFR at onset of less than 40 ml/min per 1.73 m(2) deteriorated faster than the others. Most children experienced early improvement of kidney function. The further prognosis, early or late deterioration of kidney function or stable function during the whole follow-up, was related to albuminuria, number of febrile UTIs, eGFR at onset of deterioration, hypertension and puberty.

Timing and Outcome of Renal Replacement Therapy in Patients with Congenital Malformations of the Kidney and Urinary Tract

“…In the context of these changed ontologic concepts, the biphasic pattern of RRT incidence observed in the first two decades of life in patients with obstructive nephropathy and isolated renal dysplasia, with a dip around age 5-9 years, is consistent with the notion that early renal survival depends on the severity of renal dysplasia (10). Although children with severe dysplastic kidney disease require RRT from early infancy, those with less severe malformations characteristically undergo a transient period of stable or even increasing GFR resulting from compensatory hypertrophy of the remnant functioning nephrons.…”

“…The natural course of CAKUT is very heterogeneous (10,11). Although the most severely affected newborns progress to ESRD within the first few months of life, kidney function improves in most children born with CAKUT, typically reaching a peak around age 3-4 years.…”

“…Subsequently, kidney function remains stable in most children until puberty. During adolescence, accelerated progression of CKD to ESRD is frequently observed (10). Altogether, approximately 25% of children born with bilateral CAKUT and kidney dysfunction require RRT during the first two decades of life (2,10,11).…”

HNF1B Mutations Associate with Hypomagnesemia and Renal Magnesium Wasting

“…42 GFR was estimated using the Schwartz-Haycock formula, optimized for children with renal malformations assessed in our hospital. 43 Plasma total magnesium was interrogated only when the renal disease was staged 3 CKD or less severe (GFR Ն30 ml/min per 1.73 m 2 ) to avoid potentially confounding magnesium retention when GFR is decreased below 25% of normal. 44 Values were not included once patients received magnesium supplementation.…”

Natural Course of Children With Dysplastic and Hypoplastic Kidney

“…Furthermore. the natural history and long-term renal prognosis of these diseases are poorly defined, thus making it difficult to predict renal outcomes of any individual patient with dysplastic or hypoplastic kidney [5][6][7][8].…”