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Congenital hernia

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https://www.readbyqxmd.com/read/28087138/corrigendum-to-delayed-presentation-of-congenital-diaphragmatic-hernia-manifesting-as-combined-type-acute-gastric-volvulus-a-case-report-and-review-of-the-literature-j-pediatr-surg-43-3-2008-e35-9-doi-10-1016-j-jpedsurg-2007-11-015
#1
https://www.readbyqxmd.com/read/28081579/predicting-full-enteral-feeding-in-the-postoperative-period-in-infants-with-congenital-diaphragmatic-hernia
#2
Carlos Zozaya, Miryam Triana, Rosario Madero, Steven Abrams, Leopoldo Martinez, Maria Virginia Amesty, Miguel Sáenz de Pipaón
Introduction The objective of the study is to examine the factors associated with time to achieve full enteral feeding after repair of congenital diaphragmatic hernia. Materials and Methods Demographic, clinical, and therapeutic data were retrospectively assessed, and uni- and multivariate Cox regression were performed to examine factors predictive of achieving full enteral feeding that was defined as time to achieve120 mL/kg/d after surgical repair. Results Of 78 infants, 66 underwent intervention before hospital discharge...
January 12, 2017: European Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28079045/hernia-of-the-umbilical-cord-associated-with-a-patent-omphalomesenteric-duct
#3
M Raicevic, I Filipovic, S Sindjic-Antunovic
Congenital hernia of the cord is a different type of ventral abdominal wall defect in which the bowel usually herniates into the base of normally inserted umbilical cord through a patent umbilical ring. It is rare congenital anomaly with incidence of 1 in 5000. Although it was described as a distinct entity since 1920s it is often misdiagnosed as a small omphalocele. We present an unusal case of term male newborn with umbilical cord hernia associated with patent omphalomesenteric duct. The diagnose was made after birth despite antenatal ultrasound scans and it is managed successfully with uneventful recovery...
January 2017: Journal of Postgraduate Medicine
https://www.readbyqxmd.com/read/28078737/3d-assessment-of-the-umbilical-vein-deviation-angle-uvda-for-the-prediction-of-liver-herniation-in-left-congenital-diaphragmatic-hernia
#4
Nicola Volpe, Eleonora Mazzone, Brunella Muto, Alice Suprani, Tiziana Fanelli, Christine Tita Kaihura, Andrea Dall'Asta, Giuseppe Pedrazzi, Carmine Del Rossi, Enrico Maria Silini, Cinzia Magnani, Paolo Volpe, Tullio Ghi, Tiziana Frusca
OBJECTIVES: To introduce a new sonographic marker of intrathoracic liver herniation in fetuses with left congenital diaphragmatic hernia (CDH) METHODS: In a consecutive series of fetuses with isolated CDH an ultrasound volume of the fetal abdomen was acquired. On this the offline calculation of the angle formed by the midline of the abdomen (joining the center of the vertebral body to the abdominal insertion of the umbilical cord) and a second line joining the center of the vertebral body to the intra-abdominal convexity of the umbilical vein was carried out (Umbilical Vein Deviation Angle or UVDA)...
January 12, 2017: Ultrasound in Obstetrics & Gynecology
https://www.readbyqxmd.com/read/28070244/assessment-of-fetus-during-second-trimester-ultrasonography-using-hdlive-software-what-is-its-real-application-in-the-obstetrics-clinical-practice
#5
Gabriele Tonni, Gianpaolo Grisolia, Eduardo Félix Santana, Edward Araujo Júnior
AIM: To show imaging results from application of four-dimensional (4D) ultrasound lightening technique (HDlive™) in clinical obstetrics practice. METHODS: Normal and abnormal fetuses at second and third trimester of pregnancy undergoing routine scan with 4D HDlive™ (5DUS) in the rendering mode are described. Realistic features of fetal structures were provided by 5DUS in the rendering mode. Normal anatomy as well as pathology like cleft lip, hypoplastic face, micrognathia, low-set ears, corpus callosum, arthrogryposis, aortic arch, left congenital diaphragmatic hernia are highlighted in this study...
December 28, 2016: World Journal of Radiology
https://www.readbyqxmd.com/read/28064243/meckel-s-diverticulitis-a-rare-entity-of-meckel-s-diverticulum
#6
Chee S Wong, Leanne Dupley, Haren N Varia, Darek Golka, Thu Linn
Meckel's diverticulum is the most common congenital abnormality of the small intestine that results from incomplete closure of the vitelline (omphalo-mesenteric) duct. This true diverticulum, ~2 ft from the ileocecal valve commonly found on the anti-mesenteric border of the ileum, is benign and majority asymptomatic. Diagnosis challenges arise when it became inflamed or presented in following ways, for example, haemorrhage (caused by ectopic pepsin-and hydrochloric acid-secreting gastric mucosa), intestinal obstruction (secondary to intussusception or volvulus) or the presence of diverticulum in the hernia sac (Littre's hernia)...
January 6, 2017: Journal of Surgical Case Reports
https://www.readbyqxmd.com/read/28056166/nitric-oxide-for-respiratory-failure-in-infants-born-at-or-near-term
#7
REVIEW
Keith J Barrington, Neil Finer, Thomas Pennaforte, Gabriel Altit
BACKGROUND: Nitric oxide (NO) is a major endogenous regulator of vascular tone. Inhaled nitric oxide (iNO) gas has been investigated as treatment for persistent pulmonary hypertension of the newborn. OBJECTIVES: To determine whether treatment of hypoxaemic term and near-term newborn infants with iNO improves oxygenation and reduces rate of death and use of extracorporeal membrane oxygenation (ECMO), or affects long-term neurodevelopmental outcomes. SEARCH METHODS: We used the standard search strategy of the Cochrane Neonatal Review Group to search the Cochrane Central Register of Controlled Trials (CENTRAL; 2016, Issue 1), MEDLINE via PubMed (1966 to January 2016), Embase (1980 to January 2016) and the Cumulative Index to Nursing and Allied Health Literature (CINAHL; 1982 to January 2016)...
January 5, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28055024/prenatally-diagnosed-congenital-diaphragmatic-hernia-optimal-mode-of-delivery
#8
C M Burgos, B Frenckner, M Luco, M T Harting, P A Lally, K P Lally
OBJECTIVE: To evaluate if the delivery mode of infants with prenatally diagnosed congenital diaphragmatic hernia (CDH) affects the outcome. STUDY DESIGN: Data from the CDH Study Group database of infants with prenatal diagnosis between 2001-2015 were divided into four delivery mode groups: vaginal spontaneous, vaginal induced, elective caesarean section, and emergent caesarean section. Outcomes were analyzed in relation to the time of day of delivery and the gestational age at birth...
January 5, 2017: Journal of Perinatology: Official Journal of the California Perinatal Association
https://www.readbyqxmd.com/read/28051921/tube-thoracostomy-at-the-time-of-congenital-diaphragmatic-hernia-repair-reassessing-the-risks-and-benefits
#9
Avraham Schlager, Kelly Arps, Ragavan Siddharthan, Matthew S Clifton
PURPOSE: Postoperative pneumothorax and effusion remain a concern following congenital diaphragmatic hernia (CDH) repair. Despite a recent trend away from intraoperative thoracostomy, few studies have actually compared outcomes with and without a chest tube. Rationale commonly cited for the more minimalistic approach include the presumed low likelihood of postoperative complications, potential risk of patch infection, and prolonged intubation. We evaluate these theories, as well as the implications of intraoperative chest tube (IOCT) placement...
January 4, 2017: Journal of Laparoendoscopic & Advanced Surgical Techniques. Part A
https://www.readbyqxmd.com/read/28050602/exome-sequencing-identifies-de-novo-pathogenic-variants-in-fbn1-and-trps1-in-a-patient-with-a-complex-connective-tissue-phenotype
#10
Diane B Zastrow, Patricia A Zornio, Annika Dries, Jennefer Kohler, Liliana Fernandez, Daryl Waggott, Magdalena Walkiewicz, Christine M Eng, Melanie A Manning, Ellyn Farrelly, Paul G Fisher, Euan A Ashley, Jonathan A Bernstein, Matthew T Wheeler
Here we describe a patient who presented with a history of congenital diaphragmatic hernia, inguinal hernia, and recurrent umbilical hernia. She also has joint laxity, hypotonia, and dysmorphic features. A unifying diagnosis was not identified based on her clinical phenotype. As part of her evaluation through the Undiagnosed Diseases Network, trio whole-exome sequencing was performed. Pathogenic variants in FBN1 and TRPS1 were identified as causing two distinct autosomal dominant conditions, each with de novo inheritance...
January 2017: Cold Spring Harbor Molecular Case Studies
https://www.readbyqxmd.com/read/28049534/case-report-maternal-mosaicism-resulting-in-inheritance-of-a-novel-gata6-mutation-causing-pancreatic-agenesis-and-neonatal-diabetes-mellitus
#11
Daphne Yau, Elisa De Franco, Sarah E Flanagan, Sian Ellard, Miriam Blumenkrantz, John J Mitchell
BACKGROUND: Haploinsufficiency of the GATA6 transcription factor gene was recently found to be the most common cause of pancreatic agenesis, a rare cause of neonatal diabetes mellitus. Although most cases are de novo, we describe three siblings with inherited GATA6 haploinsufficiency and the rare finding of parental mosaicism. CASE PRESENTATION: The proband was born at term with severe intrauterine growth restriction, the first child of non-consanguineous parents...
January 3, 2017: Diagnostic Pathology
https://www.readbyqxmd.com/read/28045687/irreducible-indirect-inguinal-hernia-in-premature-infant-with-ectopic-uterus-and-bilateral-adnexa
#12
Hassan Ibrahim, Mohga Khaled, Thomas Gates, Hilary Trice, Guillermo Sangster
Indirect inguinal hernia is one of the most common congenital anomalies in children, with a reported prevalence of 0.8-4.4%.1 About 15-20% of hernias in female infants contain ovary, and in rare cases a fallopian tube.2 However, only a few cases contain the uterus and both ovaries in the hernia sac.3 The normal anatomy is altered when an ovary is trapped in a hernia sac, and these changes make torsion more likely and increase the risk of infertility. Although an irreducible ovary is not at great risk of compression of its blood supply, in these occurrences, ovarian torsions have been reported in 2%-33%, emphasizing the importance of early surgical repair in irreducible hernias, even in asymptomatic patients...
November 2016: Journal of the Louisiana State Medical Society: Official Organ of the Louisiana State Medical Society
https://www.readbyqxmd.com/read/28043315/right-sided-congenital-diaphragmatic-hernia-and-myelomeningocele-a-rare-association
#13
Syed Rehan Ali, Shakeel Ahmed
Congenital diaphragmatic hernia (CDH) is a rare birth defect with a prevalence of < 0.5 per 1,000 live births. Majority of these defects are left-sided as most studies suggest that frequency of right-sided CDH was 10% of the total. The association of CDH with myelomeningocele (MMC) is extremely rare; as in Sweed's study of 116 consecutive cases of CDH, the incidence of associated MMC with CDH was stated as 4.3%. There has been one previous case report of leftsided CDH, MMC and hydrocephalus prenatally diagnosed; but to the best of authors' knowledge, this is the first reported case of the above constellation with a right-sided CDH diagnosed prenatally...
December 2016: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/28040456/exhaled-breath-condensate-analysis-from-intubated-newborns-by-nano-hplc-coupled-to-high-resolution-ms
#14
A S Kononikhin, N L Starodubtseva, V V Chagovets, A Y Ryndin, A A Burov, I A Popov, A E Bugrova, R A Dautov, A O Tokareva, Y L Podurovskaya, O V Ionov, V E Frankevich, E N Nikolaev, G T Sukhikh
Invasiveness of examination and therapy methods is a serious problem for intensive care and nursing of premature infants. Exhaled breath condensate (EBC) is the most attractive biofluid for non-invasive methods development in neonatology for monitoring the status of intubated infants. The aim of the study was to propose an approach for EBC sampling and analysis from mechanically ventilated neonates. EBC collection system with good reproducibility of sampling was demonstrated. Discovery-based proteomic and metabolomic studies were performed using nano-HPLC coupled to high resolution MS...
December 23, 2016: Journal of Chromatography. B, Analytical Technologies in the Biomedical and Life Sciences
https://www.readbyqxmd.com/read/28040139/prenatal-diagnosis-of-paternal-duplication-of-11p15-5%C3%A2-14-3-its-implication-of-beckwith-wiedemann-syndrome
#15
Kuan Ju Chen, Yu Mei Liu, Chien Hong Li, Yao Lung Chang, Shuenn Dyh Chang
OBJECTIVE: To characterize a prenatally detected chromosomal aberration with molecular cytogenetic approaches and explore its relationship with Beckwith-Wiedemann syndrome (BWS). CASE REPORT: A 33-year-old woman, gravida 2, para 0, was referred to our prenatal clinic at 20+ weeks due to an abnormal amniocentesis karyotyping finding, which showed 46,XY,add(11)(q24.2)dn. The mother conceived through in vitro fertilization-intracytoplasmic sperm injection (IVF-ICSI), then embryo transfer...
December 2016: Taiwanese Journal of Obstetrics & Gynecology
https://www.readbyqxmd.com/read/28040135/pallister-killian-syndrome-cytogenetics-and-molecular-investigations-of-mosaic-tetrasomy-12p-in-prenatal-chorionic-villus-and-in-amniocytes-strategy-of-prenatal-diagnosis
#16
Francesco Libotte, Domenico Bizzoco, Ivan Gabrielli, Alvaro Mesoraca, Pietro Cignini, Salvatore Giovanni Vitale, Ilaria Marilli, Ferdinando Antonio Gulino, Agnese Maria Chiara Rapisarda, Claudio Giorlandino
OBJECTIVE: Pallister-Killian syndrome (PKS) is a rare, sporadic genetic disorder caused by mosaic tetrasomy of the short arm of chromosome 12 (12p). Clinically, PKS is characterized by several systemic abnormalities, such as intellectual impairment, hearing loss, epilepsy, hypotonia, craniofacial dysmorphism, pigmentary skin anomalies, epilepsy, and a variety of congenital malformations. Prenatally, PKS can be suspected in the presence of ultrasound anomalies: diaphragmatic hernia, rhizomelic micromelia, hydrops fetalis, fetal overweight, ventriculomegaly in the central nervous system, congenital heart defects, or absent visualization of the stomach...
December 2016: Taiwanese Journal of Obstetrics & Gynecology
https://www.readbyqxmd.com/read/28029369/management-and-outcomes-of-scoliosis-in-children-with-congenital-diaphragmatic-hernia
#17
Ryan M Antiel, John S Riley, Patrick J Cahill, Robert M Campbell, Lindsay Waqar, Lisa M Herkert, Natalie E Rintoul, William H Peranteau, Alan W Flake, N Scott Adzick, Holly L Hedrick
PURPOSE: The purpose of this study was to evaluate the management and outcomes of CDH patients with scoliosis. METHODS: From January 1996 to August 2015, 26 of 380 (7%) CDH patients were diagnosed with scoliosis. Six (23%) were prenatally diagnosed by ultrasound, and 9 (35%) were diagnosed postnatally. The remaining 11 (42%) developed scoliosis after discharge. Mean follow-up was 6.6years. RESULTS: Among the 15 patients with congenital scoliosis, there were 2 (13%) perinatal deaths...
September 15, 2016: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28018801/thoracoscopic-approach-for-congenital-diaphragmatic-hernia-a-useful-tool-in-diagnosis-and-treatment-of-unexpected-associated-lung-lesion
#18
Mario Lima, Michela Maffi, Tommaso Gargano, Giovanni Ruggeri, Michele Libri, Francesca Destro
We report the case of a neonate affected by prenatally diagnosed congenital diaphragmatic hernia (CDH), treated by thoracoscopy. During the procedure, we detected an associated extralobar pulmonary sequestration (EPS), which was preoperatively undiagnosed, and we removed it. EPS has been shown to be associated with CDH in up to 15 to 40% of cases. A possible explanation hypothesized is that pulmonary sequestration, which develops at 4 to 5 weeks of gestation, can disturb the fusion of the diaphragm and closure of the pleuroperitoneal canal...
December 2016: European Journal of Pediatric Surgery Reports
https://www.readbyqxmd.com/read/28018700/the-cause-of-unexpected-acute-abdomen-and-intra-abdominal-hemorrhage-in-24-week-pregnant-woman-bochdalek-hernia
#19
Yavuz Savas Koca, Ibrahim Barut, İhsan Yildiz, Rasih Yazkan
Bochdalek hernia (BH) is the most common type of congenital diaphragm hernia and is rarely seen in adults. In adult patients, BH often remains asymptomatic or presents with nondiagnostic symptoms and may lead to complications, though rarely. The necrosis and perforations occurring in the hernia may lead to mortality. In this report, we present a 34-year-old pregnant woman at 24 gestational weeks who presented with Bochdalek hernia causing gastric volvulus associated with perforation and intra-abdominal hemorrhage associated with splenic rupture...
2016: Case Reports in Surgery
https://www.readbyqxmd.com/read/28005161/-epidural-anesthesia-for-fetoscopy-retrospective-analysis-of-a%C3%A2-one-year-cohort
#20
N Kiefer, S F Suter, C Berg, U Gembruch, S U Weber
BACKGROUND: The introduction of routine prenatal screening using ultrasound has led to a substantial increase in diagnoses of fetal disorders that are amenable to intrauterine treatment. While an ultrasound guided insertion of small bore cannulas can be performed under local anesthesia, insertion of a fetoscope usually requires anesthetic management for the mother and the fetus. Additionally, the fetus' intrauterine position may have to be manipulated in order to enable access. Such manoeuvres depend on relaxation of the mother's abdominal wall...
December 22, 2016: Der Anaesthesist
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