Pattern of clinical presentation of congenital anomalies of the kidney and urinary tract among infants and children

Soliman, Neveen A.; Ali, Reham I; Ghobrial, Emad E.; Habib, E; Ziada, Ali

doi:10.1111/nep.12414

Cited by 46 publications

(45 citation statements)

References 22 publications

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“…Bondagji [10] found associated nonrenal anomalies in 26.2% of cases and Soliman et al [8] in 31.8% of cases. The incidence of the different types of CAKUT in the current study and other studies is shown in Table 3.…”

Section: Discussionmentioning

confidence: 99%

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Clinico-Biochemical Profile of Children with Congenital Anomalies of the Kidney and Urinary Tract: A Cross-Sectional Study

et al. 2018

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Background: Congenital anomalies of the kidney and urinary tract (CAKUT) are a group of disorders responsible for the majority of pediatric end-stage renal disease cases. There are only a few studies on CAKUT. Objectives: A study was conducted to determine the clinical and biochemical profile of children with CAKUT and to estimate the prevalence and the factors associated with hypertension in CAKUT. Methods: A cross-sectional study was conducted in a tertiary center for 18 months from March 2014 to August 2015. Demographic data were recorded, and clinical examination including blood pressure measurement was performed. Various biochemical parameters including plasma renin activity (PRA), urinary beta-2-microglobulin (B2M), and microalbuminuria were evaluated. Results: A total of 81 patients with CAKUT were studied. Twenty-two (27%) patients were underweight, 4 (5%) patients were stunted, and 26 (32%) were both underweight and stunted. Children with bilateral disease had a higher incidence of underweight (21/44 vs. 8/37; p = 0.04; 95% CI; Fisher exact test), and both underweight and stunted (25/44 vs. 10/37; p = 0.006; 95% CI; Fisher exact test) compared to children with unilateral disease. Hypertension was found in 27% cases. No association was found between hypertension and PRA, BM2, or microalbuminuria in our study. PRA was inversely proportional to the estimated glomerular filtration rate (eGFR) (Pearson test; 95% CI; p = 0.006). Conclusions: Bilateral disease in CAKUT was significantly associated with poor somatic growth. PRA was inversely proportional to eGFR. The prevalence of hypertension was higher in children with CAKUT than in normal children and is possibly multifactorial as it was not associated with elevated PRA, B2M, or microalbuminuria.

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Section: Discussionmentioning

confidence: 99%

“…CAKUT also accounts for 7% of adult ESRD worldwide [8]. CAKUT is seen in 1 in 500 newborns [2] and in 0.3–1.6 per 1,000 live born and stillborn neonates [9].…”

Section: Discussionmentioning

confidence: 99%

Clinico-Biochemical Profile of Children with Congenital Anomalies of the Kidney and Urinary Tract: A Cross-Sectional Study

et al. 2018

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“…Many of them like hypoplasia, ectopic kidneys, and anomalies in shape can be asymptomatic and detected incidentally in adults. Others like renal agenesis, MCDK, bilateral PUJO and cystic renal diseases can present early either antenatally with oligohydramnios or later with urinary tract infection, hypertension, proteinuria, renal impairment, abdominal mass, hematuria or stones [6] . Antenatal screening ultrasonography (US) allows demonstration of fetal kidneys and urinary bladder from early second trimester and enables to detect a number of major congenital anomalies of the urinary system.…”

Section: Approachmentioning

confidence: 99%

Multi-modality imaging review of congenital abnormalities of kidney and upper urinary tract

Ramanathan¹,

Kumar²,

Khanna³

et al. 2016

WJR

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Congenital abnormalities of the kidney and urinary tract (CAKUT) include a wide range of abnormalities ranging from asymptomatic ectopic kidneys to life threatening renal agenesis (bilateral). Many of them are detected in the antenatal or immediate postnatal with a significant proportion identified in the adult population with varying degree of severity. CAKUT can be classified on embryological basis in to abnormalities in the renal parenchymal development, aberrant embryonic migration and abnormalities of the collecting system. Renal parenchymal abnormalities include multi cystic dysplastic kidneys, renal hypoplasia, number (agenesis or supernumerary), shape and cystic renal diseases. Aberrant embryonic migration encompasses abnormal location and fusion anomalies. Collecting system abnormalities include duplex kidneys and Pelvi ureteric junction obstruction. Ultrasonography (US) is typically the first imaging performed as it is easily available, noninvasive and radiation free used both antenatally and postnatally. Computed tomography (CT) and magnetic resonance imaging (MRI) are useful to confirm the ultrasound detected abnormality, detection of complex malformations, demonstration of collecting system and vascular anatomy and more importantly for early detection of complications like renal calculi, infection and malignancies. As CAKUT are one of the leading causes of end stage renal disease, it is important for the radiologists to be familiar with the varying imaging appearances of CAKUT on US, CT and MRI, thereby helping in prompt diagnosis and optimal management.

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“…Among anomalies found at prenatal screening, CAKUT is present in 20%-30% of the cases. 13,14 Renal function ranges from normal to ESRD, but most patients have some degree of impaired kidney function. Urine analysis in general shows no cells and a proteinuria of ,1 g/24 h. Occasionally, renal biopsies are performed in patients that do not present with CAKUT, with the histologic diagnosis often being variable and nonspecific.…”

Section: Hnf1b-associated Kidney Diseasementioning

confidence: 99%

Hepatocyte Nuclear Factor 1β–Associated Kidney Disease

Verhave

Bech

Wetzels

et al. 2016

Journal of the American Society of Nephrology

130

121

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Hepatocyte nuclear factor 1b (HNF1b)-associated disease is a recently recognized clinical entity with a variable multisystem phenotype. Early reports described an association between HNF1B mutations and maturity-onset diabetes of the young. These patients often presented with renal cysts and renal function decline that preceded the diabetes, hence it was initially referred to as renal cysts and diabetes syndrome. However, it is now evident that many more symptoms occur, and diabetes and renal cysts are not always present. The multisystem phenotype is probably attributable to functional promiscuity of the HNF1b transcription factor, involved in the development of the kidney, urogenital tract, pancreas, liver, brain, and parathyroid gland. Nephrologists might diagnose HNF1b-associated kidney disease in patients referred with a suspected diagnosis of autosomal dominant polycystic kidney disease, medullary cystic kidney disease, diabetic nephropathy, or CKD of unknown cause. Associated renal or extrarenal symptoms should alert the nephrologist to HNF1b-associated kidney disease. A considerable proportion of these patients display hypomagnesemia, which sometimes mimics Gitelman syndrome. Other signs include early onset diabetes, gout and hyperparathyroidism, elevated liver enzymes, and congenital anomalies of the urogenital tract. Because many cases of this disease are probably undiagnosed, this review emphasizes the clinical manifestations of HNF1b-associated disease for the nephrologist.

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Pattern of clinical presentation of congenital anomalies of the kidney and urinary tract among infants and children

Cited by 46 publications

References 22 publications

Clinico-Biochemical Profile of Children with Congenital Anomalies of the Kidney and Urinary Tract: A Cross-Sectional Study

Clinico-Biochemical Profile of Children with Congenital Anomalies of the Kidney and Urinary Tract: A Cross-Sectional Study

Multi-modality imaging review of congenital abnormalities of kidney and upper urinary tract

Hepatocyte Nuclear Factor 1β–Associated Kidney Disease

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