Bilateral femoral hernias in a male child as the initial presentation of Ehlers-Danlos syndrome
Publication date: Available online 27 August 2020Source: Journal of Pediatric Surgery Case ReportsAuthor(s): Melissa Wong, Patrick J. Javid
Publication date: Available online 8 October 2020Source: Journal of Pediatric Surgery Case ReportsAuthor(s): Elize W. Lockhorst, Jasper van der Slegt, Eelco J. Veen, Dagmar I. Vos
ConclusionA review of patient outcomes after hybrid AWR highlights a trend towards shorter length of stay, lower hospital cost, and fewer complications without significant addition to operative time. Long-term studies on a larger number of patients are definitively needed to characterize the comprehensive benefits of this approach.
Condition: Ventral Hernia Interventions: Procedure: Repair of ventral hernia using biologic mesh; Procedure: Repair of ventral hernia using suture repair or synthetic mesh Sponsor: The University of Texas Health Science Center, Houston Not yet recruiting
ConclusionFemoral hernias causing bowel obstruction are associated with greater time between the development of symptoms, hospitalization, and with a longer hospital stay.
This study highlights the impact of the size of deleted regions, as they may affect the clinical picture of patients with 16p11.2 microdeletion syndrome. Reported cases indicates the key role of the interdisciplinary approach in 16p11.2 microdeletion syndrome diagnostics, as the care of patients with this deletion is based on regular health assessment and adjustment of the nervous system develo pment therapy
ConclusionIn our hands, a significantly higher recurrence rate exists for children undergoing the PIRS method over LICPV techniques when treating inguinal hernias.
AbstractIntroductionMagnetic sphincter augmentation (MSA) is a safe and effective treatment for patients with gastroesophageal reflux disease (GERD). MSA was initially indicated for patients with GERD and concomitant hiatal hernias 2 cm) on videoesophagram or endoscopy.ResultsSeventy-nine patients (53% female) with a median age of 65.56 (58.42 –69.80) years were included. Median follow up was 2.98 (interquartile range 1.90–3.32) years. Median DeMeester scores decreased from 42.45 (29.12–60.73) to 9.10 (3.05–24.30) (p
Mutations of theCYP21A2 gene encoding adrenal 21-hydroxylase cause congenital adrenal hyperplasia (CAH). TheCYP21A2 gene is partially overlapped by theTNXB gene, which encodes an extracellular matrix protein called Tenascin-X (TNX). Mutations affecting both alleles ofTNXB cause a severe, autosomal recessive form of Ehlers-Danlos syndrome (EDS). Rarely, patients with severe, salt-wasting CAH have deletions ofCYP21A2 that extend intoTNXB, resulting in a “contiguous gene syndrome” consisting of CAH and EDS. Heterozygosity forTNXB mutations causing haploinsufficiency of TNX may be associated with the mild “hy...
In conclusion, time-onset and particular conditions associated with GIP should lead to a high index of suspicion for internal hernias in order to achieve appropriate diagnosis and therapy.