Renal outcome in adults with renal insufficiency and irregular asymmetric kidneys

Neild, Guy H.; Thomson, G E; Nitsch, Dorothea; Woolfson, Robin G.; Connolly, John; Woodhouse, Christopher

doi:10.1186/1471-2369-5-12

Cited by 46 publications

(28 citation statements)

References 28 publications

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“…For primary reflux, the development of progressive renal failure is rare unless the GFR is below the range of 40-50 ml/min per 1.73 m 2 [7] and, at any level of function, the rate of loss of function is minimal, while there is little or no proteinuria [2,4,6,8]. We have previously reported similar results from young adults with CAKUT [9].…”

Section: Introductionsupporting

confidence: 74%

What do we know about chronic renal failure in young adults? II. Adult outcome of pediatric renal disease

Neild

2009

Pediatr Nephrol

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Congenital abnormalities of the kidney and urinary tract (CAKUT) account for more than half of all renal failure in children. For young adults with CAKUT two questions are paramount: what is the prognosis and what is the best management to improve outcome? The paediatric literature shows that prognostic factors are glomerular filtration rate (GFR) and the presence of proteinuria. We reviewed data from 101 young adult patients with either primary vesico-ureteric reflux and renal dysplasia or obstructive uropathy. Patients had an estimated GFR (eGFR) of 35 ml/min. The ACEI benefit increased with time. Rate of decline was slower than reported for other diseases and was only -2.4 ml/min per year for those reaching the start of dialysis. Outcome is predictable by the level of residual renal function (GFR). Nevertheless, function remains stable while proteinuria is minimal. Short-term studies overestimate rates of deterioration.

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Section: Introductionsupporting

confidence: 74%

What do we know about chronic renal failure in young adults? II. Adult outcome of pediatric renal disease

Neild

2009

Pediatr Nephrol

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“…In the literature, eGFR of less than 30 ml/min per 1.73 m 2 is a poor prognostic factor for deterioration of renal function [8]. In adult patients with renal failure secondary to dysplasia, a GFR of more than 40 ml/min per 1.73 m 2 was a good prognostic factor for continuing stable renal function or a slow progression [27].…”

Section: Discussionmentioning

confidence: 99%

Progression of chronic renal failure in children with dysplastic kidneys

2007

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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.

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“…A nephrectomy can cure the symptoms. Neild et al 30 investigated the effect of angiotensin-converting enzyme inhibitor in patients who had chronic renal failure due to renal dysplasia with or without reflux. This study suggests that there is a watershed glomerular filtration rate of 40 to 50 mL/min, at which angiotensin-converting enzyme inhibitor treatment can be successful in improving renal function.…”

Section: Current Treatment and Prognosismentioning

confidence: 99%

Renal Dysplasia

Chen

Chang

2015

Archives of Pathology &Amp; Laboratory Medicine

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Renal dysplasia is an aberrant developmental disease usually diagnosed during the perinatal and childhood years. Prevalence is estimated at 0.1% of infants (via ultrasound screening) and 4% of fetuses and infants (via autopsy study). Occurrences may be combined with abnormalities in the collecting system or associated with complex syndromes. Histopathology shows primitive tubules surrounded by a fibromuscular collar. The differential diagnosis includes renal dysplasia, hypoplasia, and renal atrophy. Immunohistochemical expression of the paired box genes 2 and 8 (PAX2/8) and Wilms tumor 1 (WT1) is increased in the primitive ducts and fibromuscular collar, respectively. Renal dysplasia pathogenesis is not well understood, but may be caused by a nephron-inductive deficit due to ampullary inactivity or abnormal budding of the ureteric bud from the mesonephric duct. Either the PAX2 mutation only or cross-talk with the p53 pathway is involved in this deficit. Nephrectomy is the treatment of choice for symptomatic renal dysplasia.

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Renal outcome in adults with renal insufficiency and irregular asymmetric kidneys

Cited by 46 publications

References 28 publications

What do we know about chronic renal failure in young adults? II. Adult outcome of pediatric renal disease

What do we know about chronic renal failure in young adults? II. Adult outcome of pediatric renal disease

Progression of chronic renal failure in children with dysplastic kidneys

Renal Dysplasia

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