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4.
J Am Vet Med Assoc ; 259(9): 1009-1024, 2021 11 01.
Artigo em Inglês | MEDLINE | ID: mdl-34647474

RESUMO

OBJECTIVE: To characterize the association between peritoneopericardial diaphragmatic hernia (PPDH) or congenital central diaphragmatic hernia (CCDH) and ductal plate malformations (DPMs) in dogs and cats. ANIMALS: 18 dogs and 18 cats with PPDH or CCDH and 19 dogs and 18 cats without PPDH or CCDH. PROCEDURES: Evaluation of clinical details verified PPDH or CCDH and survival times. Histologic features of nonherniated liver samples were used to categorize DPM. Immunohistochemical staining for cytokeratin-19 distinguished bile duct profiles per portal tract and for Ki-67-assessed cholangiocyte proliferation. Histologic features of herniated liver samples from PPDH or CCDH were compared with those of pathological controls (traumatic diaphragmatic hernia, n = 6; liver lobe torsion, 6; ischemic hepatopathy, 2). RESULTS: DPM occurred in 13 of 18 dogs with the proliferative-like phenotype predominating and in 15 of 18 cats with evenly distributed proliferative-like and Caroli phenotypes. Congenital hepatic fibrosis DPM was noted in 3 dogs and 2 cats and renal DPM in 3 dogs and 3 cats. No signalment, clinical signs, or clinicopathologic features discriminated DPM. Kaplan Meier survival curves were similar in dogs and cats. Bile duct profiles per portal tract in dogs (median, 5.0; range, 1.4 to 100.8) and cats (6.6; 1.9 to 11.0) with congenital diaphragmatic hernias significantly exceeded those in healthy dogs (1.4; 1.2 to 1.6) and cats (2.3; 1.7 to 2.6). Animals with DPM lacked active cholangiocyte proliferation. Histologic features characterizing malformative bile duct profiles yet without biliary proliferation were preserved in herniated liver lobes in animals with DPM. CONCLUSIONS AND CLINICAL RELEVANCE: DPM was strongly associated with PPDH and CCDH. Because DPM can impact health, awareness of its coexistence with PPDH or CCDH should prompt biopsy of nonherniated liver tissue during surgical correction of PPDH and CCDH.


Assuntos
Doenças do Gato , Doenças do Cão , Hérnias Diafragmáticas Congênitas , Animais , Gatos , Cães , Hérnias Diafragmáticas Congênitas/veterinária , Cirrose Hepática/veterinária
5.
BMC Surg ; 21(1): 364, 2021 Oct 12.
Artigo em Inglês | MEDLINE | ID: mdl-34641847

RESUMO

BACKGROUND: Symptomatic Bochdalek hernias are found mainly in infants in respiratory distress and occur rarely in adults. CASE PRESENTATION: We report a rare case of Bochdalek hernia associated with developmental abnormalities in an adult who exhibited acute chest pain and dyspnea on exertion. CONCLUSIONS: This case highlights the importance of the differential diagnosis of acute left-sided chest pain and antenatal examination.


Assuntos
Hérnias Diafragmáticas Congênitas , Adulto , Dor no Peito/etiologia , Diagnóstico Diferencial , Feminino , Hérnias Diafragmáticas Congênitas/diagnóstico por imagem , Hérnias Diafragmáticas Congênitas/cirurgia , Humanos , Gravidez
6.
BMC Pediatr ; 21(1): 460, 2021 10 20.
Artigo em Inglês | MEDLINE | ID: mdl-34666738

RESUMO

BACKGROUND: Right-sided congenital diaphragmatic hernia (RCDH) is relatively rare compared with left-sided congenital diaphragmatic hernia (LCDH). Clinical data of RCDH, especially with respect to antenatal prediction of neonatal outcome, are lacking. The aim of this study was to report the treatment outcomes of patients with antenatally diagnosed RCDH and to evaluate the predictability of observed-to-expected lung area-to-head circumference ratio (O/E LHR) for perinatal outcomes, focused on mortality or extracorporeal membrane oxygenation (ECMO) requirement. METHODS: We retrospectively reviewed the medical records of newborn infants with isolated RCDH. We analyzed and compared the clinical and prenatal characteristics including the fetal lung volume, which was measured as the O/E LHR, between the survivors and the non-survivors. RESULTS: A total of 26 (66.7%) of 39 patients with isolated RCDH survived to discharge. The O/E LHR was significantly greater in survivors (64.7 ± 21.2) than in non-survivors (40.5 ± 23.4) (P =.027). It was greater in survivors without ECMO requirement (68.3 ± 15.1) than non-survivors or those with ECMO requirement (46.3 ± 19.4; P = .010). The best O/E LHR cut-off value for predicting mortality in isolated RCDH was 50. CONCLUSIONS: The findings in this study suggest that O/E LHR, a well-characterized prognostic indicator in LCDH, could be applied to a fetus with antenatally diagnosed RCDH. A large cohort study is required to verify the association between O/E LHR values and the graded severity of RCDH.


Assuntos
Hérnias Diafragmáticas Congênitas , Feminino , Idade Gestacional , Hérnias Diafragmáticas Congênitas/diagnóstico por imagem , Hérnias Diafragmáticas Congênitas/terapia , Humanos , Lactente , Recém-Nascido , Pulmão/diagnóstico por imagem , Gravidez , Prognóstico , Estudos Retrospectivos , Ultrassonografia Pré-Natal
8.
Pediatr Surg Int ; 37(11): 1499-1504, 2021 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-34505169

RESUMO

PURPOSE: In congenital diaphragmatic hernia (CDH), ultrasound (U/S) measurements of the contralateral lung commonly provide the observed-to-expected lung-to-head ratio (O/E LHR) and are used to determine the severity of pulmonary hypoplasia. Fetal magnetic resonance imaging (MRI) measurement of the observed-to-expected total lung volume (O/E TLV) has been used as an adjunct to O/E LHR in predicting outcomes. Since O/E LHR only measures the contralateral lung, we sought to investigate if MRI measurements of the contralateral lung volume (O/E CLV) can accurately predict outcomes in CDH. We hypothesize that O/E CLV is a better predictor of CDH outcomes than O/E LHR. METHODS: We identified all infants with a prenatal diagnosis of CDH at our fetal center who had both MRI and U/S measurements. Using lung volume ratios of right-left 55:45, we calculated O/E CLV from O/E TLV. We used receiver-operating characteristic (ROC) curves to calculate the area under the curve (AUC) to compare the predictive accuracy of O/E CLV to O/E LHR for ECMO support, as well as survival to both discharge and 1 year. RESULTS: Seventy-four patients had complete prenatal imaging with 39% requiring ECMO support. The median O/E CLV was 48.0% and the median O/E LHR was 42.3%. O/E CLV was a better predictor of the need for ECMO support (AUC 0.81 vs. 0.74). O/E CLV was a better predictor of survival to discharge (AUC 0.84 vs. 0.64) and 1-year survival (AUC 0.83 vs. 0.63) than O/E LHR. CONCLUSION: O/E LHR is a well-validated standard for predicting outcomes and guiding prenatal counseling in CDH. We provide evidence that fetal MRI measurements of the contralateral lung volume corrected for gestational age were more accurate in predicting the need for ECMO and survival. Future prospective studies validating O/E CLV regarding outcomes and ECMO utilization are warranted. LEVEL OF EVIDENCE: Level III, retrospective comparative study.


Assuntos
Hérnias Diafragmáticas Congênitas , Feminino , Idade Gestacional , Hérnias Diafragmáticas Congênitas/diagnóstico por imagem , Hérnias Diafragmáticas Congênitas/cirurgia , Humanos , Pulmão/diagnóstico por imagem , Imageamento por Ressonância Magnética , Gravidez , Prognóstico , Estudos Prospectivos , Estudos Retrospectivos , Ultrassonografia Pré-Natal
9.
Pediatr Surg Int ; 37(12): 1667-1673, 2021 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-34487208

RESUMO

PURPOSE: Predicting lethal pulmonary hypoplasia in infants with congenital diaphragmatic hernia (CDH) before extracorporeal membrane oxygenation (ECMO) initiation is difficult. This study aimed to predict lethal pulmonary hypoplasia in patients with CDH prior to ECMO. METHODS: This was a multicenter cohort study involving neonates prenatally diagnosed with isolated unilateral CDH (born 2006-2020). Patients who required ECMO due to respiratory insufficiency were included in this study. Patients who underwent ECMO due to transient disorders were excluded from analysis. Blood gas analysis data within 24 h of birth were compared between survivors and non-survivors. Predictive abilities were assessed for factors with significant differences. RESULTS: Overall, 34 patients were included (18 survivors and 16 non-survivors). The best pre-ductal PaO2 was significantly lower in non-survivors than in survivors (50.4 [IQR 30.3-64.5] vs. 67.5 [IQR 52.4-103.2] mmHg, respectively; p = 0.047). A cutoff PaO2 of 42.9 mmHg had a sensitivity, specificity, and positive predictive value of 50.0%, 94.4%, and 88.9%, respectively, to predict mortality. CONCLUSION: The best PaO2 within 24 h after birth predicted mortality following ECMO initiation. This should be shared to families and caregivers to optimize the best interests of the infants with CDH.


Assuntos
Oxigenação por Membrana Extracorpórea , Hérnias Diafragmáticas Congênitas , Estudos de Coortes , Hérnias Diafragmáticas Congênitas/terapia , Humanos , Lactente , Recém-Nascido , Japão/epidemiologia , Estudos Retrospectivos
10.
Neonatal Netw ; 40(5): 305-312, 2021 Aug 01.
Artigo em Inglês | MEDLINE | ID: mdl-34518382

RESUMO

Congenital diaphragmatic hernia (CDH) is a developmental defect in the diaphragm that allows abdominal viscera to herniate into the thoracic cavity. Pulmonary hypoplasia and pulmonary hypertension are consequences of this disease process. The incidence is approximately 2.4-4.1/10,000 births, and survival rate is estimated at 70-90 percent. To avoid potentially devastating delays in care, it is crucial that neonatal nurses and care providers in both tertiary and nontertiary care centers be familiar with the pathogenesis of CDH and the standard of care for initial stabilization of the neonate. Novel fetal and postnatal surgical repair techniques are also described here.


Assuntos
Hérnias Diafragmáticas Congênitas , Feminino , Feto , Hérnias Diafragmáticas Congênitas/diagnóstico , Hérnias Diafragmáticas Congênitas/terapia , Humanos , Recém-Nascido , Gravidez , Cuidado Pré-Natal
11.
BMJ Case Rep ; 14(9)2021 Sep 03.
Artigo em Inglês | MEDLINE | ID: mdl-34479892

RESUMO

We present a rare case of premature low birthweight neonate with right diaphragmatic hernia and transposition of great vessels requiring balloon atrial septostomy. Congenital diaphragmatic hernia poses a unique challenge to umbilical venous catheterisation. Based on the radiographic position of umbilical vein catheter, umbilical venous cannulation was attempted; however, the catheter could not be navigated to the right atrium. Saline contrast echocardiography was used to delineate the abnormal umbilical and ductus venosus drainage. Eventually, the procedure was successfully completed via the femoral venous approach. We emphasise the importance of defining ductus venosus anatomy and umbilical venous drainage using a simple tool like saline contrast echocardiography before performing catheterisation using the umbilical venous access in such cases.


Assuntos
Hérnias Diafragmáticas Congênitas , Transposição dos Grandes Vasos , Cateterismo , Ecocardiografia , Humanos , Recém-Nascido , Transposição dos Grandes Vasos/diagnóstico por imagem , Transposição dos Grandes Vasos/cirurgia , Veias Umbilicais/diagnóstico por imagem
12.
Int J Mol Sci ; 22(17)2021 Aug 31.
Artigo em Inglês | MEDLINE | ID: mdl-34502408

RESUMO

Oxidative stress plays a pathological role in pulmonary hypoplasia and pulmonary hypertension in congenital diaphragmatic hernia (CDH). This study investigated the effect of molecular hydrogen (H2), an antioxidant, on CDH pathology induced by nitrofen. Sprague-Dawley rats were divided into three groups: control, CDH, and CDH + hydrogen-rich water (HW). Pregnant dams of CDH + HW pups were orally administered HW from embryonic day 10 until parturition. Gasometric evaluation and histological, immunohistochemical, and real-time polymerase chain reaction analyses were performed. Gasometric results (pH, pO2, and pCO2 levels) were better in the CDH + HW group than in the CDH group. The CDH + HW group showed amelioration of alveolarization and pulmonary artery remodeling compared with the CDH group. Oxidative stress (8-hydroxy-2'-deoxyguanosine-positive-cell score) in the pulmonary arteries and mRNA levels of protein-containing pulmonary surfactant that protects against pulmonary collapse (surfactant protein A) were significantly attenuated in the CDH + HW group compared with the CDH group. Overall, prenatal H2 administration improved respiratory function by attenuating lung morphology and pulmonary artery thickening in CDH rat models. Thus, H2 administration in pregnant women with diagnosed fetal CDH might be a novel antenatal intervention strategy to reduce newborn mortality due to CDH.


Assuntos
Hérnias Diafragmáticas Congênitas/tratamento farmacológico , Hidrogênio/farmacologia , Animais , Animais Recém-Nascidos , Antioxidantes/farmacologia , Óxido de Deutério/farmacologia , Modelos Animais de Doenças , Feminino , Hérnias Diafragmáticas Congênitas/metabolismo , Hérnias Diafragmáticas Congênitas/patologia , Hidrogênio/metabolismo , Hipertensão Pulmonar/metabolismo , Pulmão/patologia , Masculino , Organogênese/efeitos dos fármacos , Éteres Fenílicos/efeitos adversos , Éteres Fenílicos/farmacologia , Gravidez , Artéria Pulmonar , Surfactantes Pulmonares/metabolismo , Ratos , Ratos Sprague-Dawley , Remodelação Vascular/efeitos dos fármacos
13.
Ultrasound Q ; 37(3): 272-277, 2021 Sep 01.
Artigo em Inglês | MEDLINE | ID: mdl-34478427

RESUMO

ABSTRACT: To determine if lung to liver MR T2 signal ratio is predictive of neonatal outcome in fetuses with congenital diaphragmatic hernia (CDH).After Interal Review Board approval, the PACS systems at the University of Washington and University of Utah were searched for cases having an in utero fetal MR examination diagnostic of CDH. Inclusion criteria were at least 1 prior ultrasound demonstrating a CDH and an MR obtained within 1 week of that prior ultrasound.A total of 69 patients from the University of Utah and 13 from the University of Washington satisfied the inclusion criteria for a total of 82. After adjusting for gestational age and contralateral lung volume, there was little apparent association between contralateral lung to liver MR T2 signal and 5-minute Apgar score and neonatal mortality When considering neonatal Apgar and mortality, increasing contralateral lung volume was significantly associated with lower risk (hazard ratio, 0.40 per doubling; 95% confidence interval, 0.24-0.69; P = 0.001) as expected.Our data demonstrate that the lung to liver MR signal ratio was not predictive of outcome. The measurement of contralateral lung area, and gestational age at the time of the examination (time of diagnosis) are still the best predictors of poor neonatal outcome.


Assuntos
Hérnias Diafragmáticas Congênitas , Feminino , Feto , Idade Gestacional , Hérnias Diafragmáticas Congênitas/diagnóstico por imagem , Humanos , Recém-Nascido , Pulmão/diagnóstico por imagem , Imageamento por Ressonância Magnética , Espectroscopia de Ressonância Magnética , Gravidez , Ultrassonografia Pré-Natal
16.
J Laparoendosc Adv Surg Tech A ; 31(10): 1168-1174, 2021 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-34569855

RESUMO

Background: Congenital diaphragmatic hernia (CDH) is a relatively common congenital anomaly, typically requiring repair in the neonatal period. Approaches to surgical repair of a CDH are varied. A small defect may be repaired primarily while a large defect often requires a patch repair. Minimally invasive and open techniques have been reported to have varying benefits and outcomes. Materials and Methods: The authors describe their technique of CDH repair and present a review of the literature. Results: In a stabilized neonate with a small-to-moderate CDH defect, a thoracoscopic primary repair with biological mesh underlay (or a patch repair if needed to reduce tension) represents our approach of choice to treat this pathology. However, attention to specific technical details is required to minimize incidence of recurrence. Conclusions: The data favor a minimally invasive approach to CDH repair in the appropriate patients.


Assuntos
Hérnias Diafragmáticas Congênitas , Hérnias Diafragmáticas Congênitas/cirurgia , Herniorrafia , Humanos , Recém-Nascido , Recidiva , Estudos Retrospectivos , Toracoscopia , Resultado do Tratamento
17.
J Laparoendosc Adv Surg Tech A ; 31(11): 1341-1345, 2021 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-34491842

RESUMO

Aim: To assess the severity of persistent pulmonary hypertension (PPH) in congenital diaphragmatic hernia (CDH) neonates solely using oxygenation index (OI). Study Design: A prospective study was carried out from April 2016 to March 2019, where all confirmed CDH neonates were evaluated for the possibility repair through thoracoscopic approach. The severity of PPH was assessed using OI. It is calculated using the equation: mean airway pressure (MAP) × FiO2 × 100 ÷ PaO2. Neonates having OI <5 were considered to have a mild degree of pulmonary hypertension; hence, thoracoscopic repair was offered for them. Results: Thirty-nine CDH cases met the selection criteria; therefore, they underwent thoracoscopic repair. Primary diaphragmatic repair was successfully accomplished thoracoscopically in all neonates without any perioperative complications. Conversion from thoracoscopy to open method occurred in five cases. The causes were due to difficulties encountered during repair and none was due to a pure anesthetic problem or general deterioration during thoracoscopy. Recurrence had occurred in two cases only. Conclusion: OI is a reliable subjective parameter that could be used as an adjuvant to the usually used cardiovascular and pulmonary parameters for thoracoscopic repair decision. With increasing surgical experience, a wider range of neonates may be considered for thoracoscopic CDH repair.


Assuntos
Hérnias Diafragmáticas Congênitas , Hérnias Diafragmáticas Congênitas/cirurgia , Humanos , Recém-Nascido , Estudos Prospectivos , Estudos Retrospectivos , Toracoscopia , Resultado do Tratamento
18.
Am J Hum Genet ; 108(10): 1964-1980, 2021 10 07.
Artigo em Inglês | MEDLINE | ID: mdl-34547244

RESUMO

Congenital diaphragmatic hernia (CDH) is a severe congenital anomaly that is often accompanied by other anomalies. Although the role of genetics in the pathogenesis of CDH has been established, only a small number of disease-associated genes have been identified. To further investigate the genetics of CDH, we analyzed de novo coding variants in 827 proband-parent trios and confirmed an overall significant enrichment of damaging de novo variants, especially in constrained genes. We identified LONP1 (lon peptidase 1, mitochondrial) and ALYREF (Aly/REF export factor) as candidate CDH-associated genes on the basis of de novo variants at a false discovery rate below 0.05. We also performed ultra-rare variant association analyses in 748 affected individuals and 11,220 ancestry-matched population control individuals and identified LONP1 as a risk gene contributing to CDH through both de novo and ultra-rare inherited largely heterozygous variants clustered in the core of the domains and segregating with CDH in affected familial individuals. Approximately 3% of our CDH cohort who are heterozygous with ultra-rare predicted damaging variants in LONP1 have a range of clinical phenotypes, including other anomalies in some individuals and higher mortality and requirement for extracorporeal membrane oxygenation. Mice with lung epithelium-specific deletion of Lonp1 die immediately after birth, most likely because of the observed severe reduction of lung growth, a known contributor to the high mortality in humans. Our findings of both de novo and inherited rare variants in the same gene may have implications in the design and analysis for other genetic studies of congenital anomalies.


Assuntos
Proteases Dependentes de ATP/genética , Proteases Dependentes de ATP/fisiologia , Anormalidades Craniofaciais/genética , Variações do Número de Cópias de DNA , Anormalidades do Olho/genética , Transtornos do Crescimento/genética , Hérnias Diafragmáticas Congênitas/genética , Luxação Congênita de Quadril/genética , Proteínas Mitocondriais/genética , Proteínas Mitocondriais/fisiologia , Mutação de Sentido Incorreto , Osteocondrodisplasias/genética , Anormalidades Dentárias/genética , Animais , Estudos de Casos e Controles , Estudos de Coortes , Anormalidades Craniofaciais/patologia , Anormalidades do Olho/patologia , Feminino , Transtornos do Crescimento/patologia , Hérnias Diafragmáticas Congênitas/patologia , Luxação Congênita de Quadril/patologia , Humanos , Masculino , Camundongos , Camundongos Endogâmicos C57BL , Camundongos Knockout , Osteocondrodisplasias/patologia , Linhagem , Anormalidades Dentárias/patologia
19.
J Med Case Rep ; 15(1): 424, 2021 Aug 05.
Artigo em Inglês | MEDLINE | ID: mdl-34353367

RESUMO

BACKGROUND: Bochdalek diaphragmatic hernia is a developmental defect of the posterolateral portion of the diaphragm. This defect may allow abdominal contents to abnormally occupy the thoracic cavity, resulting in most cases in the compression of the developing lungs. Signs are typically shown during early childhood since the defect is usually present during development. In exceptional cases, however, Bochdalek diaphragmatic hernia can be observed in asymptomatic adult patients, or in those whose initial diagnosis may include common respiratory pathologies such as asthma. CASE PRESENTATION: Here we describe the case of a 31-year-old Mestizo female patient admitted to the emergency room owing to sudden onset of pain in the left hypochondrium and in epigastrium, as well as signs of respiratory distress. Soon after admission, the patient entered cardiorespiratory arrest, and advanced cardiac life support was provided for 45 minutes without success. The patient was declared dead 1 hour 40 minutes after admission. Clinical autopsy concluded that cause of death was respiratory failure as a complication of a previously undiagnosed Bochdalek diaphragmatic hernia. CONCLUSIONS: We report an exceptional case of Bochdalek diaphragmatic hernia as the cause of rapid-onset respiratory failure and death in an adult. Unfortunately, due to its unusual presentation, Bochdalek diaphragmatic hernia is rarely considered among the list of differential diagnoses when admitting an adult patient with respiratory symptoms. By reporting this case, we encourage the medical community and trainees to consider diaphragmatic defects when approaching a patient with sudden onset of abdominal pain with concomitant respiratory symptoms.


Assuntos
Hérnia Hiatal , Hérnias Diafragmáticas Congênitas , Adulto , Autopsia , Pré-Escolar , Diafragma , Dispneia/etiologia , Feminino , Hérnias Diafragmáticas Congênitas/complicações , Hérnias Diafragmáticas Congênitas/diagnóstico por imagem , Hérnias Diafragmáticas Congênitas/cirurgia , Humanos
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