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OHVIRA Syndrome and Ureteral Ectopy Draining in the Ipsilateral Hemiuterus, Diagnosed in the Prepubertal Age Group: Case-Report and Literature Review.
Medicina (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 PDFContinence management in children with severe caudal regression syndrome: role of multidisciplinary team and long-term follow-up.
Pediatr Surg Int
October 2022
Division of Neuro-Urology, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.
Purpose: Caudal regression syndrome (CRS) is a rare congenital abnormality including orthopedic deformities, urological, anorectal, and cardiac malformations. The clinical spectrum of CRS varies in severity, therefore multiple surgeries and complex medical care may be required with the efforts and support of a multidisciplinary team to ensure the most accurate treatment and the best outcome. The aim of our retrospective study was to evaluate the role of a multidisciplinary treatment and the long-term outcome in patients with severe CRS.
View Article and Find Full Text PDFProspective Study on Several Urinary Biomarkers as Indicators of Renal Damage in Children with CAKUT.
Eur J Pediatr Surg
April 2019
Pediatric Surgery Unit, University of Foggia, Foggia, Italy.
Purpose: The aim of the study was to investigate urinary levels of monocyte chemotactic protein-1 (MCP-1), epidermal growth factor (EGF), β-2-microglobulin (β2M), and FAS-ligand (FAS-L) in children with congenital anomalies of kidney and urinary tract (CAKUT) disease at risk of developing glomerular hyperfiltration syndrome. For this reason, we selected patients with multicystic kidney, renal agenesia and renal hypodysplasia, or underwent single nephrectomy.
Materials And Methods: This prospective, multicentric study was conducted in collaboration between the Pediatric Surgery Unit in Foggia and the Pediatric Nephrology Unit in Bari, Italy.
Unilateral renal agenesis and abrupt onset diabetes: an unfrequent form of MODY type diabetes.
Med Clin (Barc)
January 2019
Servicio Endocrinología y Nutrición, Hospital Puerta del Mar, Cádiz, España.
Introduction: MODY diabetes encompasses heterogeneous group of monogenic forms of diabetes with low prevalence. It is not easily diagnosed because of the increase in obesity and family history of diabetes in the general population.
Patients And Methods: We present a clinical case with cardinal symptoms, diabetes,renal insufficiency with no acidosis and with a family history of diabetes and renal agenesis.
Clinical and radiological findings for early diagnosis of Herlyn-Werner-Wunderlich syndrome in pediatric age: experience of a single center.
Gynecol Endocrinol
January 2018
a Pediatric Surgery Unit , Salesi Children's Hospital, Università Politecnica delle Marche, Ancona , Italy.
Objective: The authors present their experience in the management of pediatric patients with Herlyn-Werner-Wunderlich syndrome (HWWS) considering clinical classification and anatomical characteristics of the malformation.
Methods: All the data of the patient presented at our Pediatric Surgery Unit from February 2010 to August 2015 were collected. According to the type of malformations, patients were divided in 3 groups: A (completely obstructed hemivagina), B (incompletely obstructed hemivagina), and C (communication between the duplicated cervices).
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