Unlabelled: The association of multicystic renal dysplasia with ureteropelvic junction obstruction is noteworthy in newborn. However, the possibility of conservative management is still a primary requirement, unless there are complications that suggest surgery. The authors are studying a case of a newborn in which an incorrect nephrostomy led to complications that required emergency surgery.
Case Presentation: A newborn girl with left-side ureteropelvic junction obstruction and an enlarged and multicystic right kidney was operated on at an early age by unexpert hands, leading to complications. Was monitored daily, and an emergency procedure was done. Following up shows the success of the emergency operation.
Clinical Discussion: The age and the precise timing of intervention are controversial. In this case, several postnatal diagnostic tests were made due to the severity of the hydronephrosis in the antenatal period, which resulted in the implementation of percutaneous nephrostomy.
Conclusion: Authors suggest that it is better to not operate as long as the patient's condition is stable.
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http://dx.doi.org/10.1097/MS9.0000000000000210 | DOI Listing |
Cureus
December 2024
Department of Pediatrics, Division of Neonatology, Blythedale Children's Hospital, Valhalla, USA.
Retroperitoneal teratomas are rare neoplasms in neonates, presenting with nonspecific symptoms and variable clinical features, making diagnosis challenging. Radiological investigations, particularly fetal ultrasound and contrast-enhanced computed tomography, play a critical role in their detection. Differential diagnoses include neuroblastoma, adrenal hemorrhage, and congenital cystic lesions, which share overlapping clinical and imaging features.
View Article and Find Full Text PDFMedicina (Kaunas)
November 2024
Department of Anatomy and Embryology, "Iuliu Hatieganu" University of Medicine and Pharmacy, 400012 Cluj-Napoca, Romania.
Müllerian (paramesonephric) duct anomalies (MDA) are a rare condition, occurring in 5.5% of female newborns. One of the most complex malformations is represented by Obstructed Hemivagina and Ipsilateral Renal Anomalies (OHVIRA) syndrome, also known as Herlyn -Werner-Wunderlich (HWW) syndrome.
View Article and Find Full Text PDFBMJ Case Rep
December 2024
Dermatology, Case Western Reserve University School of Medicine, Cleveland, Ohio, USA
Hydroa vacciniforme lymphoproliferative disorders (HVLPD) fall within the clinical spectrum of chronic active epstein barr virus (EBV) disease (CAEBVD), ranging from localised and/or indolent forms (classic HVLPD) to systemic disease with fever, hepatosplenomegaly and lymphadenopathy (systemic HVLPD). A preadolescent male with 47XYY, multicystic dysplastic kidney, autism spectrum disorder and Attention-deficit/hyperactivity disorder (ADHD) presented with photodistributed non-pruritic, non-painful necrotic papulovesicles accompanied by non-febrile intermittent fatigue and lymphadenopathy. The patient had a history of EBV pneumonia in infancy confirmed by CT scan and was later diagnosed with CAEBV.
View Article and Find Full Text PDFClin Exp Nephrol
December 2024
Department of Pediatric Nephrology, Ulus Maternity and Child Health and Diseases Training and Research Hospital, Dr. Sami, Ankara, Turkey.
Background: Patients diagnosed with congenital kidney malformations are at an increased risk of developing hypertension, proteinuria, and progressing to chronic kidney disease (CKD). The present study aimed to determine the frequency of masked hypertension and ambulatory arterial stiffness index (AASI) in patients with congenital kidney malformations.
Methods: The study included 174 patients with congenital kidney malformations (48 patients with unilateral renal agenesis (URA), 40 patients with ectopic kidney (EK), 36 patients with horseshoe kidney (HK), 31 patients with multicystic dysplastic kidney (MCDK), 19 patients with unilateral renal hypoplasia (URH), and 45 healthy controls.
Nephrol Dial Transplant
December 2024
Centre de Néphrologie et transplantation rénale, Assistance publique-Hôpitaux de Marseille, La Conception Hospital, Marseille, France.
Background: Alport Syndromes (AS) are the second leading genetic cause of Kidney Failure (KF). Whether multiple kidney cysts (MKC) phenotype belongs to the AS spectrum remains debated.
Methods: This multicenter retrospective study focused on patients genotyped with pathogenic COL4A3, COL4A4, or COL4A5 variants (classified as ACMG-AMP 4 or 5) between January 2011 and January 2023 across four French university hospitals.
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