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http://dx.doi.org/10.1007/s00467-020-04581-9 | DOI Listing |
BMC Pediatr
November 2024
Department of Pediatric and Neonatal Nursing, School of Nursing and Midwifery, Institute of Health Science, Wollega University, Nekemte, Ethiopia.
Cureus
April 2024
Medicine, Indira Gandhi Institute of Medical Sciences, Patna, IND.
BMC Nephrol
April 2024
Laboratory of Genomic Medicine, Sidra Medicine, P.O. Box 26999, Doha, Qatar.
Background: Renal tubular dysgenesis (RTD) is a severe disorder with poor prognosis significantly impacting the proximal tubules of the kidney while maintaining an anatomically normal gross structure. The genetic origin of RTD, involving variants in the ACE, REN, AGT, and AGTR1 genes, affects various enzymes or receptors within the Renin angiotensin system (RAS). This condition manifests prenatally with oligohydramninos and postnatally with persistent anuria, severe refractory hypotension, and defects in skull ossification.
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February 2024
Neonatal Intensive Care Unit, Department of Neonatology, Centro Hospitalar Universitário de São João, Porto, PRT.
The renin-angiotensin-aldosterone system (RAAS) plays a crucial role in the normal development of the fetal kidney. Late pregnancy blockage of the RAAS, through in-utero exposure to angiotensin-converting enzyme inhibitors (ACEIs) or angiotensin II receptor blockers, is associated with poor fetal outcomes, including oligohydramnios, renal tubular dysplasia, postnatal anuric renal failure, and hypotension. The present case describes a 39-year-old primigravida that was referred to the emergency department, at 37 weeks, for the evaluation of intrauterine growth restriction and suspected coarctation of the aorta (CoA).
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June 2024
Division of Nephrology and Rheumatology, National Center for Child Health and Development, 2-10-1, Okura, Setagaya-ku, Tokyo, 157-8535, Japan.
There are no clinical guidelines for performing nephrectomy in patients with autosomal recessive polycystic kidney disease (ARPKD). Few reports have described the clinical course of ARPKD diagnosed in the neonatal period in detail. Here, we report seven patients diagnosed with ARPKD and treated at our center during the neonatal period.
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