Fetal, perinatal, and infant death with congenital renal anomaly

Scott, J. E.

doi:10.1136/adc.87.2.114

Cited by 39 publications

(27 citation statements)

References 5 publications

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“…CAKUT was detected antenatally in 76% of cases by Melo et al [2], 66% of cases by Scott [12], and 37% of cases by Soliman et al [8]. In our study, we also found that third-trimester USGs were more sensitive in diagnosing CAKUT antenatally.…”

Section: Discussionsupporting

confidence: 76%

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: Discussionsupporting

confidence: 76%

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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“…Prognostication and management are based largely on case series suggesting that nearly all affected infants die shortly after birth. [6][7][8][9][10][11][12][13][14][15][16] However, reports of occasional long-term survivors [21][22][23][24] and of decreased mortality with intensive care 25,26 raise questions about the use of the term 'lethal' in this context. Especially, as parents access available knowledge, policies that limit medical treatment are being questioned.…”

Section: Discussionmentioning

confidence: 99%

“…First, we elicited the expert opinion of knowledgeable clinicians (three perinatologists, three neonatologists, one geneticist and two genetics counselors) who reviewed International Classification of Diseases, Ninth Revision codes (ICD-9 codes 740 to 759) 5 and identified the congenital diagnoses they considered lethal. We then performed MEDLINE and Google searches of the diagnoses listed by two-thirds or more of the clinicians, reviewed articles, [6][7][8][9][10][11][12][13][14][15][16] reference texts 17 and websites, 18,19 and retained those diagnoses that were consistently reported to be associated with death before a year of life in nearly all (X85%, when statistics were available) cases. We excluded conjoined twins because of the confounding effect of one infant's condition on the other.…”

Section: Methodsmentioning

confidence: 99%

Length of life and treatment intensity in infants diagnosed prenatally or postnatally with congenital anomalies considered to be lethal

2010

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Objectives: The objectives of this study were (1) to compare age at death and the intensity and cost of medical treatment for infants diagnosed prenatally or postnatally with congenital anomalies considered to be lethal.(2) To determine whether greater treatment intensity is associated with longer life.Study Design: This is a retrospective cohort study of all fetuses and neonates with congenital anomalies classified as lethal who were diagnosed or treated at the University of North Carolina Hospitals from January 1998 to December 2003. Result:The cohort consisted of 192 fetuses and infants: 160 were diagnosed prenatally, 2 were diagnosed perinatally, and 30 were diagnosed postnatally. In all, 115 (72%) pregnancies were terminated. Of the liveborn infants, 75% died before 10 days of age and 90% before 4 months of age. Compared with postnatally diagnosed infants, prenatally diagnosed infants received less intense treatment (median average daily Neonatal Therapeutic Intervention Scoring System score 8.3 versus 14.0; P ¼ 0.02), at less cost (median direct cost of hospitalization $1550 versus $8474; P ¼ 0.03) and died sooner (median age at death <1 day versus 4 days; P ¼ 0.01). Greater treatment intensity did not correlate with longer survival (r ¼ À0.04; P ¼ 0.66). Conclusion:Although some kinds of medical therapy may be appropriate for newborns with lethal congenital anomalies, highly aggressive interventions did not prolong survival and should not be offered. Even when pregnancy termination is not elected, infants diagnosed prenatally receive less intense care.

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“…(34,35) Such improper connections result in abnormalities of the urinary tract including megaureter, vesico-ureteral reflux and uteroceles, which are among the most common birth defects in humans. (5,36,37) Genetic studies in mice are revealing that the apparently simple morphogenetic event of bud outgrowth actually requires a complex network of positive and negative regulatory mechanisms, with GDNF at its nexus.…”

Section: The Role Of Localized Gdnf/ret Signaling In Ureter Formationmentioning

confidence: 99%

GDNF/Ret signaling and the development of the kidney

2006

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SummarySignaling by GDNF through the Ret receptor is required for normal growth of the ureteric bud during kidney development. However, the precise role of GDNF/Ret signaling in renal branching morphogenesis and the specific responses of ureteric bud cells to GDNF remain unclear. Recent studies have provided new insight into these issues. The localized expression of GDNF by the metanephric mesenchyme, together with several types of negative regulation, is important to elicit and correctly position the initial budding event from the Wolffian duct. GDNF also promotes the continued branching of the ureteric bud. However, it does not provide the positional information required to specify the pattern of ureteric bud growth and branching, as its site of synthesis can be drastically altered with minimal effects on kidney development. Cells that lack Ret are unable to contribute to the tip of the ureteric bud, apparently because GDNF-driven proliferation is required for the formation and growth of this specialized epithelial domain.

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Fetal, perinatal, and infant death with congenital renal anomaly

Cited by 39 publications

References 5 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

Length of life and treatment intensity in infants diagnosed prenatally or postnatally with congenital anomalies considered to be lethal

GDNF/Ret signaling and the development of the kidney

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