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Congenital diaphragmatic hernia

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https://www.readbyqxmd.com/read/28922348/stem-cell-biology-and-regenerative-medicine-for-neonatal-lung-diseases
#1
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
#2
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/28898547/hlx-is-a-candidate-gene-for-a-pattern-of-anomalies-associated-with-congenital-diaphragmatic-hernia-short-bowel-and-asplenia
#3
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
#4
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
#5
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/28870723/managing-malnourishment-in-pregnancy-after-bariatric-surgery
#6
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
#7
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
#8
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
#9
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
#10
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/28850156/fosterkirurgi-%C3%A3-r-en-v%C3%A3-xande-multidisciplin%C3%A3-r-subspecialitet-etablerad-behandling-vid-ryggm%C3%A3-rgsbr%C3%A3-ck-%C3%A2-i-framtiden-skymtar-artificiell-placenta-och-stamcellsterapi
#11
Carmen Mesas Burgos, Peter Conner, Georgios Papatziamos, Eleonor Tiblad
Advances in fetal surgery Fetal surgery is a subspeciality that is evolving rapidly with focus on improving the natural history of congenital malformations and conditions that are either life threatening or cause severe disability. Fetal surgery for myelomeningocele has been shown to improve neurologic outcome, motor function and to reduce the need of ventriculo-peritoneal shunting after birth compared to postnatal care. However, it conveys an increased risk of preterm birth and maternal morbidity. The role of prenatal intervention with endoscopic tracheal occlusion in congenital diaphragmatic hernia is currently the focus of an ongoing multicenter randomized controlled trial...
August 29, 2017: Läkartidningen
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
#12
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)
https://www.readbyqxmd.com/read/28841740/-massive-morgagni-hernia-as-a-reason-for-lung-function-impairment
#13
A Gillissen, M Paparoupa, T Zimmermann
Hernia of Morgagni is the most rare of the four types of congenital diaphragmatic hernia (2 % - 3 % of all cases). In adults, it commonly presents with non-specific symptoms. In severe cases patients complain about shortness of breath and in lung function analysis a restrictive pattern can be observed. This paper presents a rare case of a massive diaphragmatic hernia of the right thorax which remained undiagnosed over many years and gives an up-to-date overview of the literature. The transabdominal approach using laparoscopic repair is favored in adults especially in non-acute cases...
August 25, 2017: Pneumologie
https://www.readbyqxmd.com/read/28817240/prenatal-presentation-of-mabry-syndrome-with-congenital-diaphragmatic-hernia-and-phenotypic-overlap-with-fryns-syndrome
#14
Kara K Reynolds, Jane Juusola, Gregory M Rice, Philip F Giampietro
We report on a family in which initial features were compatible with Fryns syndrome. The first sibling was a stillborn female with a left diaphragmatic hernia (DH). Her clinical features overlapped with Fryns syndrome. The second pregnancy, a male fetus, was followed for polyhydramnios, hypoplastic mandible, mild enlargement of the fetal bladder, hydronephrosis, and rocker bottom foot deformities. He had facial features similar to his sibling and a large cleft of the secondary palate, small jaw, and secundum atrial septal defect...
August 17, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28812464/congenital-diaphragmatic-hernia-with-liver-herniation-into-the-pericardial-sac-in-a-30-week-gestation-infant
#15
Nisha Patel, Christina Sollinger, Carl T D'Angio, Jeffrey M Vinocur, Kate G Ackerman, Philip J Katzman
Anterior diaphragmatic defects with pericardial involvement are extremely rare and diagnostically challenging entities encountered perinatally. While a majority of diaphragmatic defects occur in isolation, others are associated with multiple defects forming a complex of syndromes such as Pentalogy of Cantrell. Liver herniation into the pericardial sac poses a particular challenge and can mimic a pericardial tumor on prenatal ultrasound, yielding a different management course. The following case is an unusual presentation of a 30-week gestation female with an anterior midline diaphragmatic defect with liver herniation mimicking as a pericardial tumor, diagnosed at time of autopsy...
September 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28804698/a-novel-technique-of-posterolateral-suturing-in-thoracoscopic-diaphragmatic-hernia-repair
#16
Yoon Jung Boo, Stephan Rohleder, Oliver J Muensterer
Background  Closure of the posterolateral defect in some cases of congenital diaphragmatic hernia (CDH) can be difficult. Percutaneous transcostal suturing is often helpful to create a complete, watertight closure of the diaphragm. A challenge with the technique is passing the needle out the same tract that it entered so that no skin is caught when the knots are laid down into the subcutaneous tissue. This report describes a novel technique using a Tuohy needle to percutaneously suture the posterolateral defect during thoracoscopic repair of CDH...
January 2017: European Journal of Pediatric Surgery Reports
https://www.readbyqxmd.com/read/28803335/intraoperative-ventilation-during-thoracoscopic-repair-of-neonatal-congenital-diaphragmatic-hernia
#17
Tadaharu Okazaki, Manabu Okawada, Junya Ishii, Hiroyuki Koga, Go Miyano, Takashi Doi, Yuki Ogasawara, Geoffrey J Lane, Atsuyuki Yamataka
PURPOSE: To evaluate the optimal ventilation mode during thoracoscopic repair (TR) of neonatal congenital diaphragmatic hernia (CDH), we compared high-frequency oscillatory ventilation (HFOV) with conventional mechanical ventilation (CMV). METHODS: Twenty-three neonatal CDH cases who underwent TR without intraoperative inhalation of nitric oxide at our institution between 2007 and 2016 were reviewed. Patients were initially ventilated with HFOV, which was converted to CMV if the HFOV settings were decreased to FiO2 <0...
August 12, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28796911/re-evaluation-of-lung-to-thorax-transverse-area-ratio-immediately-before-birth-in-predicting-postnatal-short-term-outcomes-of-fetuses-with-isolated-left-sided-congenital-diaphragmatic-hernia-a-single-center-analysis
#18
Saki Kido, Nobuhiro Hidaka, Yuka Sato, Yasuyuki Fujita, Kina Miyoshi, Kouji Nagata, Tomoaki Taguchi, Kiyoko Kato
We aimed to investigate whether the lung-to-thorax transverse area ratio (LTR) immediately before birth is of diagnostic value for the prediction of postnatal short-term outcomes in cases of isolated left-sided congenital diaphragmatic hernia (CDH). We retrospectively reviewed the cases of fetal isolated left-sided CDH managed at our institution between April 2008 and July 2016. We divided the patients into 2 groups based on LTR immediately before birth, using a cut-off value of 0.08. We compared the proportions of subjects within the 2 groups who survived until discharge using Fisher's exact test...
August 10, 2017: Congenital Anomalies
https://www.readbyqxmd.com/read/28795544/-recurrence-of-congenital-diaphragmatic-hernia
#19
R Gillard, M Dirix, M Cannie, P Philippet, M Thimmesch
Through the case report of a child who had had a congenital diaphragmatic hernia (cdh) and then relapsed 8 months after initial surgery, the various risk factors related to a cdh, its pre- and postnatal management as well as some long-term complications are discussed.
July 2017: Revue Médicale de Liège
https://www.readbyqxmd.com/read/28794965/efficacy-of-lung-volume-optimization-maneuver-monitored-by-optoelectronic-pletismography-in-the-management-of-congenital-diaphragmatic-hernia
#20
G Lista, I Bresesti, F Cavigioli, F Castoldi, E Lupo, A LoMauro, A Aliverti
Newborns affected by congenital diaphragmatic hernia (CDH) need cardio-respiratory stabilization before undergoing surgical repair. Open lung strategy is a well-established approach to optimize lung volume in preterm infants with Respiratory Distress Syndrome (RDS), using both High Frequency Oscillatory Ventilation (HFOV) and Conventional Mechanical Ventilation (CMV). We report a case of left CDH with severe lung hypoplasia, managed applying open lung strategy in HFOV (pre-surgery period) and in Assist-Control with Volume Guarantee (post-surgery period), guided by SpO2 changes, TcPO2 and TcPCO2 monitoring...
2017: Respiratory Medicine Case Reports
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