keyword
MENU ▼
Read by QxMD icon Read
search

Congenital hernia

keyword
https://www.readbyqxmd.com/read/29785371/case-report-clitoromegaly-as-a-consequence-of-congenital-adrenal-hyperplasia-an-accurate-medical-and-surgical-approach
#1
Augusto Rafael Fernandez-Aristi, Andre Alonso Taco-Masias, Luis Montesinos-Baca
We present a case of a woman with a history of Congenital Adrenal Hyperplasia (CAH) diagnosed at the age of 12, who was referred to our unit for surgical treatment. Despite the initial diagnosis was an indirect inguinal hernia, it was a misdiagnosis. Once in our service, this was corrected into clitoromegaly secondary to CAH. Physical examination and imaging test discarded other abnormalities, such as secondary effects androgenization. Regarding surgical treatment, the techniques used were Spencer and Allen combined with Kumar, which are the most used for clitoroplasty but also less used in Peru...
May 2018: Urology Case Reports
https://www.readbyqxmd.com/read/29784517/treprostinil-improves-persistent-pulmonary-hypertension-associated-with-congenital-diaphragmatic-hernia
#2
Kendall M Lawrence, Holly L Hedrick, Heather M Monk, Lisa Herkert, Lindsay N Waqar, Brian D Hanna, William H Peranteau, Natalie E Rintoul, Rachel K Hopper
OBJECTIVE: To evaluate the effect of continuous treprostinil in infants with severe pulmonary hypertension associated with congenital diaphragmatic hernia (CDH) on specific markers of pulmonary hypertension severity and to report the safety and tolerability of treprostinil. STUDY DESIGN: We conducted a retrospective cohort study of infants with CDH-associated pulmonary hypertension treated with treprostinil from January 2011 to September 2016. Severity of pulmonary hypertension was assessed by echocardiogram and serum B-type natriuretic peptide (BNP) by using time points before initiation and 24 hours, 1 week, and 1 month after treprostinil initiation...
May 18, 2018: Journal of Pediatrics
https://www.readbyqxmd.com/read/29783140/congenital-diaphragmatic-disease-an-unusual-presentation-in-adulthood-case-report
#3
Angela Gurrado, Roberta Maria Isernia, Alessandro De Luca, Valentina Ferraro, Daniela Virgintino, Anna Napoli, Giuseppe Cavallaro, Eugenio Maiorano, Angela Pezzolla, Mario Testini
INTRODUCTION: Congenital diaphragmatic disease is a quite common condition that usually occurs in the neonatal period, and the diagnosis of congenital diaphragmatic disease in adulthood is rare. CASE PRESENTATION: A 64-years-old Caucasian woman was admitted in emergency at our Department, due to a bowel obstruction and dyspnea. A CT-scan showed a diaphragmatic herniation in the left area, with malposition of dilated transverse and descending colon in the chest. An emergency laparatomy was performed, showing a toxic megacolon, in the absence of a true diaphragmatic hernia, and a left diaphragm and left liver hypoplasia...
May 7, 2018: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/29779390/-prenatally-diagnosed-case-of-pallister%C3%A2-killian-syndrome
#4
Zsolt Tidrenczel, Erika P Tardy, Edina Sarkadi, Judit Simon, Artúr Beke, János Demeter
Pallister-Killian syndrome (PKS) is a rare, sporadic genetic disorder that is caused by the mosaic presence of a supernumerary marker chromosome, isochromosome 12p. The syndrome is a polydysmorphic condition characterized by mental retardation, craniofacial dysmorphism, hypotonia, seizures, epilepsy and certain organic malformations (diaphragmatic hernia, congenital heart disease). Prenatal diagnosis is challenging due to the mosaic tissue-specific distribution of the chromosomal disorder and highly variable phenotype...
May 2018: Orvosi Hetilap
https://www.readbyqxmd.com/read/29770109/fetoscopic-endoluminal-tracheal-occlusion-and-reestablishment-of-fetal-airways-for-congenital-diaphragmatic-hernia
#5
Lennart Van der Veeken, Francesca Maria Russo, Luc De Catte, Eduard Gratacos, Alexandra Benachi, Yves Ville, Kypros Nicolaides, Christoph Berg, Glenn Gardener, Nicola Persico, Pietro Bagolan, Greg Ryan, Michael A Belfort, Jan Deprest
Background: Congenital diaphragmatic hernia (CDH) is a congenital anomaly with high mortality and morbidity mainly due to pulmonary hypoplasia and hypertension. Temporary fetal tracheal occlusion to promote prenatal lung growth may improve survival. Entrapment of lung fluid stretches the airways, leading to lung growth. Methods: Fetal endoluminal tracheal occlusion (FETO) is performed by percutaneous sono-endoscopic insertion of a balloon developed for interventional radiology...
2018: Gynecological Surgery
https://www.readbyqxmd.com/read/29766307/the-laparoscopic-inguinal-and-diaphragmatic-defect-lidd-model-a-validation-study-of-a-novel-box-trainer-model
#6
Damir Ljuhar, Samuel Alexander, Sarah Martin, Ramesh Nataraja
BACKGROUND: Paediatric laparoscopic procedures are now becoming routine practice. Therefore, there is a need for simulated laparoscopic models to acquire part-procedural competency prior to direct patient contact in a safe learning environment. For this reason, we chose two paediatric conditions; inguinal hernia (IH) and congenital diaphragmatic hernia (CDH), which were combined to create the laparoscopic inguinal and diaphragmatic defect (LIDD) model. Our aim was to assess this novel surgical simulation model by determining its construct and content validity...
May 15, 2018: Surgical Endoscopy
https://www.readbyqxmd.com/read/29763850/laparoscopic-surgery-for-a-bochdalek-hernia-triggered-by-pregnancy-in-an-adult-woman-a-case-report
#7
Shotaro Matsudera, Masanobu Nakajima, Masakazu Takahashi, Hiroto Muroi, Maiko Kikuchi, Yosuke Shida, Keisuke Ihara, Satoru Yamaguchi, Kinro Sasaki, Takashi Tsuchioka, Hiroyuki Kato
INTRODUCTION: A Bochdalek hernia (BH) is a type of congenital diaphragmatic hernia. We herein describe an adult woman with a BH triggered by pregnancy and treated by laparoscopic surgery. PRESENTATION OF CASE: A 26-year-old woman was referred to our hospital because of abdominal pain and dyspnea resulting from a left diaphragmatic hernia. She was diagnosed with a BH and underwent laparoscopic surgery. Her postoperative progress was satisfactory, and no recurrence was found at follow-up approximately 1 year later...
May 1, 2018: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/29759035/a-novel-missense-mutation-in-slc5a5-gene-in-a-sudanese-family-with-congenital-hypothyroidism
#8
Yui Watanabe, Reham Shareef Ebrhim, Mohamed A Abdullah, Roy E Weiss
Thyroid hormone synthesis requires the presence of iodide. The sodium iodide symporter (NIS) is a glycoprotein which mediates the active uptake of iodide from the blood stream into the thyroid grand. NIS defects due to SLC5A5 gene mutations are known to cause congenital hypothyroidism (CH). The proposita is a 28-year-old female whose origin is the North Sudan where neonatal screening for CH is not available. She presented with severe constipation and a goiter at the age of 40 days. Laboratory testing confirmed CH and she was started on levothyroxine (L-T4)...
May 15, 2018: Thyroid: Official Journal of the American Thyroid Association
https://www.readbyqxmd.com/read/29754878/extracorporeal-membrane-oxygenation-ecmo-risk-stratification-in-newborns-with-congenital-diaphragmatic-hernia-cdh
#9
Tim Jancelewicz, Mary E Brindle, Matthew T Harting, Elizabeth A Tolley, Max R Langham, Pamela A Lally, Ankush Gosain, Stephanie A Storgion, David W Kays
BACKGROUND: A means for early postnatal stratification of ECMO risk in CDH newborns could be used to comparatively assess the utilization and outcomes of ECMO use between centers. While multiple CDH mortality risk calculators are available, no validated tool exists specifically for prediction of ECMO use. The purpose of this study was to derive and validate an ECMO risk stratification model. METHODS: The study population was obtained from CDH Study Group registry for the period between 2007 and 2016...
April 14, 2018: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29754141/unilateral-lung-agenesis-hiatal-hernia-and-atrioventricular-septal-defect-a-rare-combination-of-congenital-anomalies
#10
Sudheer R Gorla, Josaura Fernandez-Sanchez, Ashish Garg, Sethuraman Swaminathan
Unilateral lung agenesis is a relatively rare congenital anomaly with a reported incidence of 1 in 15 000 births. It is frequently associated with other congenital malformations. Some of the sequelae of lung agenesis are potentially life-threatening. Here, we report a case of left lung agenesis in association with hiatal hernia and atrioventricular septal defect, a rare combination of anomalies which have not been described previously in the literature.
May 12, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29753526/prenatally-versus-postnatally-diagnosed-congenital-diaphragmatic-hernia-side-stage-and-outcome
#11
Carmen Mesas Burgos, Björn Frenckner, Matias Luco, Matthew T Harting, Pamela A Lally, Kevin P Lally
AIM: To compare outcomes between prenatally and postnatally diagnosed CDH in a large multicenter database of prospectively collected data and evaluate factors associated with poorer outcome for prenatally diagnosed CDH. MATERIAL AND METHODS: We used information from the multicenter, multinational CDH Study Group database on patients born between 2007 and 2015. We compared differences between prenatally and postnatally diagnosed CDH with respect to survival, side, size, ECMO needs, associated major cardiac malformations and liver position...
April 14, 2018: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29745254/treatment-of-rat-congenital-diaphragmatic-hernia-with-sildenafil-and-ns-304-selexipag-s-active-compound-at-the-pseudoglandular-stage-improves-lung-vasculature
#12
Daphne S Mous, Heleen M Kool, Petra E Burgisser, Marjon J Buscop-van Kempen, Koji Nagata, Anne Boerema-de Munck, Joost van Rosmalen, Oleh Dzyubachyk, Rene Mh Wijnen, Dick Tibboel, Robbert J Rottier
Patients with congenital diaphragmatic hernia (CDH) often suffer from severe pulmonary hypertension and the choice of current vasodilator therapy is mostly based on trial and error. Since pulmonary vascular abnormalities are already present early during development, we performed a study to modulate these pulmonary vascular changes at an early stage during gestation. Pregnant Sprague-Dawley rats were treated with nitrofen at day 9.5 of gestation (E9.5) to induce CDH in the offspring and subsequently the phosphodiesterase-5 inhibitor sildenafil and/or the novel prostaglandin-I receptor agonist selexipag (NS-304) were administered from E17...
May 10, 2018: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/29735873/bochdalek-hernia-and-intrathoracic-ectopic-kidney-presentation-of-two-case-reports-and-review-of-the-literature
#13
M Sarac, U Bakal, T Tartar, S Canpolat, A Kara, A Kazez
Bochdalek hernia is a congenital abnormality with high morbidity and mortality characterized by passage of the abdominal organs into the thoracic cavity through a diaphragmatic defect. Intrathoracic location of abdominal organs such as kidneys is very rare, with a reported incidence of only 0.25% in the literature. Herein, we present two cases of Bochdalek hernia with a herniation of intra-abdominal organ such as kidney that was treated in our clinic and compare this rare case with those in the literature. In both cases, the functionally normal kidneys were left in situ during diaphragmatic repair...
May 2018: Nigerian Journal of Clinical Practice
https://www.readbyqxmd.com/read/29734195/tenascin-x-congenital-adrenal-hyperplasia-and-the-cah-x-syndrome
#14
Walter L Miller, Deborah P Merke
Mutations of the CYP21A2 gene encoding adrenal 21-hydroxylase cause congenital adrenal hyperplasia (CAH). The CYP21A2 gene is partially overlapped by the TNXB gene, which encodes an extracellular matrix protein called Tenascin-X (TNX). Mutations affecting both alleles of TNXB cause a severe, autosomal recessive form of Ehlers-Danlos syndrome (EDS). Rarely, patients with severe, salt-wasting CAH have deletions of CYP21A2 that extend into TNXB, resulting in a "contiguous gene syndrome" consisting of CAH and EDS...
May 7, 2018: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/29728412/challenge-of-determining-true-outcome-of-congenital-diaphragmatic-hernia
#15
EDITORIAL
Carl Davis, Gregor M Walker
No abstract text is available yet for this article.
May 4, 2018: Archives of Disease in Childhood. Fetal and Neonatal Edition
https://www.readbyqxmd.com/read/29726645/-single-scrotal-incision-orchidopexy-without-ligation-of-processus-vaginalis-for-palpable-undescended-testis
#16
Yi Chen, Jun-Feng Zhao, Fu-Ran Wang, Yan Li, Zhan Shi, Hong-Ji Zhong, Jian-Ming Zhu
Objective: To determine the feasibility and short-term effect of single scrotal-incision orchidopexy (SSIO) without ligation of the processus vaginalis (PV) in the treatment of palpable undescended testis (PUDT). METHODS: This retrospective study included 109 cases of PUDT (125 sides) and 15 cases of impalpable undescended testis (IUDT). The former underwent SSIO without PV ligation (group A, n = 53) or standard inguinal orchidopexy with PV ligation (group B, n = 56) while the latter received laparoscopic exploration (group C)...
August 2017: Zhonghua Nan Ke Xue, National Journal of Andrology
https://www.readbyqxmd.com/read/29720002/early-prenatal-ultrasound-diagnosis-of-congenital-thoracic-malformations
#17
Lea Bentur, Michal Gur, Mordechai Pollak, Kamal Masarweh, Ido Solt, Moshe Bronshtein
OBJECTIVES: To evaluate and describe the spectrum and rate of congenital thoracic malformations (CTMs) diagnosed by early prenatal sonography (gestational age (GA) less than 16 weeks). METHODS: A retrospective, cross-sectional analysis of prenatal ultrasound screening tests in a community-based clinic. RESULTS: In 2001-2017, 31 261 prenatal ultrasound tests detected 31 CTMs at a gestational age of 15.2 (range, 11.6-16.0) weeks. The most common malformation was congenital pleural effusion (CPE) (15 fetuses, 0...
May 2, 2018: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/29714432/extracorporeal-membrane-oxygenation-support-in-neonates-a-single-center-experience-in-turkey
#18
Emel Okulu, F. Begüm Atasay, Gaffari Tunç, Hasan Akduman, Ömer Erdeve, Saadet Arsan, Zeynep Eyileten, Tayfun Uçar, H. Ercan Tutar
Background/aim: Extracorporeal membrane oxygenation (ECMO) is a form of life support for patients with respiratory failure, cardiac failure, or both. The aim of this study was to evaluate neonates supported with ECMO and report our experience as a Turkish neonatal intensive care unit. Materials and methods: We retrospectively reviewed 11 newborn infants treated with ECMO at Ankara University for respiratory and cardiac failure. We reported the demographic, diagnostic, laboratory, and clinical data of the patients...
April 30, 2018: Turkish Journal of Medical Sciences
https://www.readbyqxmd.com/read/29713814/prenatal-intervention-for-the-management-of-congenital-diaphragmatic-hernia
#19
REVIEW
Mariatu A Verla, Candace C Style, Oluyinka O Olutoye
Congenital diaphragmatic hernia (CDH) is the result of incomplete formation of the diaphragm that occurs during embryogenesis. The defect in the diaphragm permits the herniation of abdominal organs into the thoracic cavity contributing to the impairment of normal growth and development of the fetal lung. In addition to the hypoplastic lung, anomalies of the pulmonary arterioles worsen the pulmonary hypertension that can have detrimental effects in severe cases. Most cases of CDH can be effectively managed postnatally...
April 30, 2018: Pediatric Surgery International
https://www.readbyqxmd.com/read/29713311/cryptorchidism-in-boys-with-cerebral-palsy-is-associated-with-the-severity-of-disease-and-with-co-occurrence-of-other-congenital-anomalies
#20
Julia Spencer Barthold, Anton Wintner, Jennifer A Hagerty, Kenneth J Rogers, Md Jobayer Hossain
Background: Cryptorchidism is reported in 40-50% of small case series of cerebral palsy (CP) and attributed to hypothalamic-pituitary-gonadal axis abnormalities, intellectual disability (ID), or cremaster spasticity. We collected demographic and clinical data to define the frequency of cryptorchidism and clinical comorbidities in a large CP population. Methods: Electronic health record data were collected for all male patients ≥7 years of age seen in a large, multidisciplinary CP clinic between 2000 and 2016...
2018: Frontiers in Endocrinology
keyword
keyword
119309
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"