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Congenital hernia

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https://www.readbyqxmd.com/read/28941930/cannulating-the-contraindicated-effect-of-low-birth-weight-on-mortality-in-neonates-with-congenital-diaphragmatic-hernia-on-extracorporeal-membrane-oxygenation
#1
Patrick T Delaplain, Lishi Zhang, Yanjun Chen, Danh V Nguyen, Matteo Di Nardo, John Patrick Cleary, Peter T Yu, Yigit S Guner
BACKGROUND/PURPOSE: Restrictions for ECMO in neonates include birth weight less than 2kg (BW <2kg) and/or gestational age less than 34weeks (GA <34weeks). We sought to describe their relationship on mortality. METHODS: Neonates with a primary diagnosis code of CDH were identified in the Extracorporeal Life Support Organization (ELSO) registry, and logistic regression models were used to examine the effect of BW <2kg and GA <34weeks on mortality. RESULTS: We identified 7564 neonates with CDH...
September 2, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28933239/diaphragmatic-hernia-associated-with-absent-ductus-venosus-and-anomalous-connection-of-an-obliterated-umbilical-vein-to-the-coronary-sinus
#2
Peter C Chen, Julia C Swanson, Prakash Masand, Jose R Rodriguez, Carlos M Mery
Umbilical vein anomalies are a rare congenital defect, which have been associated with absent ductus venosus, with few cases also involving a congenital diaphragmatic hernia. We describe a case of postnatal development of an anterior diaphragmatic hernia of Morgagni in a four-year-old patient diagnosed prenatally with mesocardia, absent ductus venosus with a large umbilical vein, a large secundum atrial septal defect, and patent ductus arteriosus.
January 1, 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28932585/an-unusually-large-paraesophageal-hernia-mimicking-a-bochdalek-hernia
#3
Daneish Selvakumar, Karan Sian, Ajay J Iyengar, Ross Mejia
Hiatal hernias are due to defects in the esophageal hiatus in the diaphragm and can be classified into sliding or paraesophageal hernias. A 31-year-old male raised a suspicion of a Bochdalek hernia but at surgery had a large paraesophageal hernia. Bochdalek hernia, a congenital diaphragmatic hernia presents in adulthood asymptomatically or with vague abdominal symptoms. It is paramount to confirm the diagnosis and rule out any fatal complications with imaging studies. Prompt surgical management with large complicated hernias, such as in our case presentation would ensure the most favorable outcome...
August 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28931575/case-report-of-clitoral-hypertrophy-in-2-extremely-premature-girls-with-an-ovarian-cyst
#4
Anne-Laure Nerré, Pierre Bétrémieux, Sylvie Nivot-Adamiak
Neonatal clitoromegaly is mainly attributed to in utero androgen exposure secondary to congenital adrenal hyperplasia. We report on 2 extremely premature girls with clitoromegaly, increased androgen levels, no salt wasting syndrome, and ovarian cyst. In case 1, the cyst liquid was aspired during ovarian hernia surgery and revealed high androgen levels. After aspiration, serum androgen levels decreased, as did clitoral size. In case 2, an ovarian cyst was seen on pelvic ultrasound. Aspiration was not indicated...
September 20, 2017: Pediatrics
https://www.readbyqxmd.com/read/28925604/epidemiology-and-prognosis-of-congenital-diaphragmatic-hernia-a-population-based-cohort-study-in-utah
#5
Hari Shanmugam, Luca Brunelli, Lorenzo D Botto, Sergey Krikov, Marcia L Feldkamp
BACKGROUND: Congenital diaphragmatic hernia (CDH) is a relatively frequent and severe malformation. Population-based data on clinical presentation and associated mortality are scarce. We examined a state-wide cohort of infants with a clinically validated diagnosis of CDH to assess their clinical profile, sociodemographic patterns, and infant mortality. METHODS: We identified CDH cases from Utah's statewide population-based surveillance program among the cohort of all pregnancy outcomes (live births, stillbirths, and pregnancy terminations) delivered from 1999 to 2011...
September 19, 2017: Birth defects research
https://www.readbyqxmd.com/read/28923474/beyond-the-inhaled-nitric-oxide-in-persistent-pulmonary-hypertension-of-the-newborn
#6
REVIEW
Mei-Yin Lai, Shih-Ming Chu, Satyan Lakshminrusimha, Hung-Chih Lin
Persistent pulmonary hypertension (PPHN) is a consequence of failed pulmonary vascular transition at birth and leads to pulmonary hypertension with shunting of deoxygenated blood across the ductus arteriosus (DA) and foramen ovale (FO) resulting in severe hypoxemia, and it may eventually lead to life-threatening circulatory failure. PPHN is a serious event affecting both term and preterm infants in the neonatal intensive care unit. It is often associated with diseases such as congenital diaphragmatic hernia, meconium aspiration, sepsis, congenital pneumonia, birth asphyxia and respiratory distress syndrome...
August 10, 2017: Pediatrics and Neonatology
https://www.readbyqxmd.com/read/28922348/stem-cell-biology-and-regenerative-medicine-for-neonatal-lung-diseases
#7
REVIEW
Martin Kang, Bernard Thébaud
Lung diseases remain one of the main causes of morbidity and mortality in neonates. Cell therapy and regenerative medicine have the potential to revolutionize the management of life-threatening and debilitating lung diseases that currently lack effective treatments. Over the past decade, the repair capabilities of stem/progenitor cells has been harnessed to prevent/rescue lung damage in experimental neonatal lung diseases. Mesenchymal stromal cells and amnion epithelial cells exert pleiotropic effects and represent ideal therapeutic cells for bronchopulmonary dysplasia, a multifactorial disease...
September 18, 2017: Pediatric Research
https://www.readbyqxmd.com/read/28915526/-exit-a-possible-intervention-for-new-and-earlyborn-babies-with-severe-hydrops-fetalis-and-hydrothoraces-on-both-sides
#8
Sandra Koch, Jochen Essers, Ortraud Beringer, Frank Reister, Helmut Hummler, Anja Moewes
The EXIT (ex utero intrapartum treatment) procedure is an established method of respiratory protection, originally used in the delivery of fetuses with congenital obstructive airway diseases (tumors in the throat area, hygromas, so-called congenital high airway obstruction syndrome (CHAOS)). Meanwhile, the procedure is also carried out in large perinatal centers for pronounced diaphragmatic hernia or other special indications (EXIT to ECMO, congenital lung airway malformations (CCAM), pulmonary atresia). We present our experience with adapted EXIT procedures in 5 preterm infants with secondary generalized hydrops fetalis and pronounced bilateral hydrothoraces...
September 15, 2017: Zeitschrift Für Geburtshilfe und Neonatologie
https://www.readbyqxmd.com/read/28904661/-occlusion-secondary-to-congenital-internal-transmesenteric-hernia-about-2-cases
#9
Souleymane Ouedraogo, Salam Ouedraogo, Jean Luc Kambire, Maurice Zida, Adama Sanou
Internal hernia due to mesenteric defect or transmesenteric hernia is a rare cause of acute intestinal obstruction. Its diagnosis is most often done during surgery. The knowledge of its clinical peculiarities allows the preoperative diagnosis. We here report 2 cases of acute intestinal obstruction secondary to congenital transmesenteric hernia in two adult patients. This study aims to highlight the clinical peculiarities of this rare form of internal hernia.
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/28898547/hlx-is-a-candidate-gene-for-a-pattern-of-anomalies-associated-with-congenital-diaphragmatic-hernia-short-bowel-and-asplenia
#10
Sandra A Farrell, Sandi Sodhi, Christian R Marshall, Andrea Guerin, Anne Slavotinek, Tara Paton, Karen Chong, Wilma L Sirkin, Stephen W Scherer, Félix-Antoine Bérubé-Simard, Nicolas Pilon
Isolated congenital diaphragmatic hernia is often a sporadic event with a low recurrence risk. However, underlying genetic etiologies, such as chromosome anomalies or single gene disorders, are identified in a small number of individuals. We describe two fetuses with a unique pattern of multiple congenital anomalies, including diaphragmatic hernia, short bowel and asplenia, born to first-cousin parents. Whole exome sequencing showed that both were homozygous for a missense variant, c.950A>C, predicting p...
September 12, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28891233/fetoscopic-tracheal-occlusion-for-treatment-of-non-isolated-congenital-diaphragmatic-hernia
#11
Viola Seravalli, Eric B Jelin, Jena L Miller, Aylin Tekes, Luca Vricella, Ahmet A Baschat
Fetoscopic endotracheal occlusion (FETO) is a prenatal treatment that may increase survival in severe congenital diaphragmatic hernia (CDH). In the USA, FETO is offered for isolated severe left-sided CDH in the context of an FDA-approved feasibility study. FETO in non-isolated cases of severe CDH is only performed with a compassionate use exemption from US regulatory bodies. Anomalies frequently associated with CDH include congenital cystic lesions of the lung and cardiac defects. We describe two cases of non-isolated severe left-sided CDH that underwent prenatal FETO, survived after birth and underwent postnatal surgical repair...
August 11, 2017: Prenatal Diagnosis
https://www.readbyqxmd.com/read/28887119/respiratory-syncytial-virus-associated-hospitalizations-in-children-with-congenital-diaphragmatic-hernia
#12
Bernhard Resch, Krisztina Liziczai, Friedrich Reiterer, Thomas Freidl, Michaela Haim, Berndt Urlesberger
BACKGROUND: To evaluate the risk of RSV infection in infants and children with congenital diaphragmatic hernia (CDH) over two consecutive RSV seasons. METHODS: Retrospective, single-center cohort study from southern Austria including infants with CDH born between 1993 and 2012. Infants were retrieved by searching via ICD-10 codes Q79.0 and Q79.1 and by using a local electronic database. Children were followed over 2 years of life including at least two consecutive RSV seasons (November to April)...
August 16, 2017: Pediatrics and Neonatology
https://www.readbyqxmd.com/read/28877794/morgagni-hernia-is-it-just-a-congenital-hernia-or-a-cause-of-emergency-surgery
#13
Tayfun Yoldas, Mehmet Ustun, Can Karaca, Ozgur Firat, Cemil Caliskan, Erhan Akgun, Mustafa Korkut
No abstract text is available yet for this article.
November 1, 2016: American Surgeon
https://www.readbyqxmd.com/read/28876575/neonatal-surgery-a-study-of-two-years-at-nelson-mandela-academic-hospital-mthatha-eastern-cape
#14
A Delgado, A Cejas, D Bangasa
BACKGROUND: Advances in diagnostic techniques and perioperative care have greatly improved the outcome of neonatal surgery. Despite this, disparity still exists in the outcome of neonatal surgery between developed and developing countries. METHOD: We performed a prospective study of neonates admitted and treated due to surgical congenital diseases and other conditions in our hospital from April 2015 to April 2017. RESULTS: There were 19 (28,7%) females and 47 (70,3%) males in this group...
September 2017: South African Journal of Surgery. Suid-Afrikaanse Tydskrif Vir Chirurgie
https://www.readbyqxmd.com/read/28870723/managing-malnourishment-in-pregnancy-after-bariatric-surgery
#15
Rachel K Harrison, Charles Berkelhammer, Victor Suarez, Helen H Kay
BACKGROUND: Little information exists to guide monitoring and treatment of malnourishment during pregnancy after bariatric surgery. Here we present a case with severe deficiencies and recommendations for testing and treatment. CASE: Our patient underwent a duodenal switch procedure resulting in significant weight loss and numerous deficiencies. She then experienced a neonatal demise with multiple congenital abnormalities, including diaphragmatic hernia, possibly related to severe vitamin A deficiency...
September 1, 2017: Journal of Obstetrics and Gynaecology Canada: JOGC, Journal D'obstétrique et Gynécologie du Canada: JOGC
https://www.readbyqxmd.com/read/28868233/intestinal-perforation-during-the-stabilization-period-in-a-preterm-infant-with-congenital-diaphragmatic-hernia
#16
Zbyněk Straňák, Karel Pýcha, Simona Feyereislova, Jaroslav Feyereisl, Michal Rygl
Background  Delayed surgery after stabilization of infants with congenital diaphragmatic hernia (CDH) is an accepted strategy. However, the evidence favoring delayed versus immediate surgical repair is limited. We present an extremely rare case of a very low-birth-weight infant with prenatally diagnosed left-sided CDH and unexpected transmural bowel perforations developing within the postnatal stabilization period. Case Report  A neonate born at 31st week of gestation with a birth weight of 1,470 g with antenatally diagnosed left-sided CDH presented with bowel dilation leading to transmural bowel perforations on the 2nd day of life...
January 2017: European Journal of Pediatric Surgery Reports
https://www.readbyqxmd.com/read/28866670/clinical-outcome-for-congenital-diaphragmatic-hernia-at-the-age-of-1-year-in-the-era-of-fetal-intervention
#17
Eva Van Ginderdeuren, Karel Allegaert, Herbert Decaluwe, Jan Deprest, Anne Debeer, Marijke Proesmans
BACKGROUND: Congenital diaphragmatic hernia (CDH) is an abnormal development of the diaphragm leading to high neonatal mortality and morbidity. Beyond the neonatal period, prospective data on overall long-term outcome in CDH survivors is scarce. In particular, for those treated with fetoscopic endoluminal tracheal occlusion (FETO), a promising new technique to increase survival chances for severe cases, the outcomes are even less documented. AIM: To prospectively document the clinical outcome of CDH at 1 year including FETO-treated infants in relation to ante- and postnatal variables...
September 2, 2017: Neonatology
https://www.readbyqxmd.com/read/28860014/feasibility-and-safety-of-intact-cord-resuscitation-in-newborn-infants-with-congenital-diaphragmatic-hernia-cdh
#18
Caroline Lefebvre, Thameur Rakza, Nathalie Weslinck, Pascal Vaast, Véronique Houfflin-Debarge, Sébastien Mur, Laurent Storme
BACKGROUND: Starting resuscitation before clamping the umbilical cord at birth may progressively increase pulmonary blood flow while umbilical venous blood flow is still contributing to maintenance of oxygenation and left ventricle preload. OBJECTIVE: To evaluate the feasibility, safety, and effects of intact cord resuscitation (ICR) on cardiorespiratory adaptation at birth in newborn infants with CDH. STUDY DESIGN: Prospective, observational, single-center pilot study...
August 30, 2017: Resuscitation
https://www.readbyqxmd.com/read/28853592/diaphragmatic-eventration-presenting-as-a-recurrent-diaphragmatic-hernia
#19
REVIEW
C Shwaartz, E Duggan, D S Lee, C M Divino, E H Chin
Diaphragmatic eventration is an uncommon condition, usually discovered incidentally in asymptomatic patients. Even in symptomatic patients, the diagnosis can be challenging and should be considered among the differential diagnoses of diaphragmatic hernia. The correct diagnosis can often only be made in surgery. We describe the case of a 31-year-old patient with diaphragmatic eventration that was misdiagnosed as a recurrent congenital diaphragmatic hernia and review the corresponding literature.
September 2017: Annals of the Royal College of Surgeons of England
https://www.readbyqxmd.com/read/28845456/outcome-analysis-of-congenital-diaphragmatic-hernia-cohort-before-and-after-implementation-of-standardized-protocol-in-a-tertiary-neonatal-unit
#20
Amitava Sur, Adjemoke Awoseliya, Alok Sharma
Despite evolving evidence and increased understanding, there is a strong argument that best outcomes in managing congenital diaphragmatic hernia (CDH) patients are achieved in centers which have a high admission rate of such patients and follow standardized operating protocols of management. Pneumothorax and air leak syndromes are one of the main causes of pre- and postoperative morbidity in these patients and experts believe that delivery room sedation and gentle ventilation strategies can minimize this. We observed a significant drop in incidence of pneumothorax and reduction of mortality following implementation of standardized guidelines at the neonatal unit at Southampton which is a tertiary-level neonatal care in the southern United Kingdom and a regional referral center for CDH patients...
July 2017: Surg J (N Y)
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