Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0017168 (gastroesophageal reflux disease)
11,783 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two cases of recurrent and persistent respiratory symptoms after primary repair of esophageal atresia and tracheoesophageal fistula are described. The diagnostic workup included barium swallow examination, radionuclide study for gastroesophageal reflux, and bronchoscopy. The tracheal diverticulum was detected on bronchoscopy. Treatment consisted of endoscopic cauterization of the mucosa of the diverticulum and its orifice using electrocoagulation in case 1 and the Nd:YAG laser in case 2. Electrocoagulation produced tracheal edema, which caused severe respiratory distress necessitating temporary tracheostomy. Cauterization with the Nd:YAG laser was safe, effective, and uncomplicated.
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PMID:Endoscopic treatment of tracheal diverticulum after primary repair of esophageal atresia and tracheoesophageal fistula. 972 17

The management of esophageal atresia (EA) and tracheoesophageal fistula (TEF) has improved markedly over the years, with a current overall survival of 80%-90%. This however, is not the case in developing countries, where mortality continues to be high. The results of treatment of 41 consecutive cases EA and/or TEF have been analyzed to determine factors pertinent to the outcome. The distribution of anomalies and a postoperative survival of 86.8% were comparable to those from developed countries. Aspiration pneumonia was unusually frequent in our patients (78%), but did not adversely influence outcome. Associated congenital anomalies as well as low birth weight continue to be important predictors of outcome in our setting. Our post-operative complications were similar to those from developed countries apart from a high incidence of stricture formation. This was attributed to gastroesophageal reflux, non-ventilation post-operatively, and the use of silk sutures to construct the anastomosis.
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PMID:Esophageal atresia and tracheoesophageal fistula: success and failure rates in the United Arab Emirates. 988 Jul 53

Gastroesophageal reflux is common in children after successful repair of esophageal atresia (EA), and may be related to a congenital neuronal abnormality of the esophagus. This study employed a fetal rat model of adriamycin-induced EA to investigate whether the innervation of the esophagus is abnormal in EA. The fetal rats were divided into four groups: (1) normal controls; (2) a saline-injected controls; (3) adriamycin administered but without the development of EA; and (4) adriamycin-induced EA. The distal esophageal segments were immunostained with a general neural marker, protein gene product 9.5 (PGP). Immunoreactivity per cross-sectional area (/xsa) was measured with an image analyzer. The extent of the esophageal circumference encircled by PGP-stained nerve tissue was assessed. While there was no significant difference in PGP immunoreactivity/xsa between the groups, the near-complete ring of nerve tissue along the plane of the myenteric plexus was replaced by clusters of nerve tissue in the atretic group (normal vs EA, P = 0. 001, Mann-Whitney U test). The abnormal distribution of nerve tissue in the atretic esophagus may be contributing factor in the esophageal dysmotility seen in EA.
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PMID:Abnormal enteric nerve morphology in atretic esophagus of fetal rats with adriamycin-induced esophageal atresia. 991 43

Oesophageal atresia (OA) and tracheo-oesophageal fistula (TOF) are life-threatening malformations of generally undefined cause. Previous reports of familial cases suggest a genetic contribution. The pattern of inheritance appears non-Mendelian, i.e., multifactorial. Individuals with OA/TOF often have other malformations and medical problems. The aim of this study was to determine the association in OA/TOF cases and healthy control subjects of associated malformations, midline defects, and medical conditions. We also investigate the relationships of these conditions in the relatives of the cases and controls. The results show that infants with OA/TOF frequently have VACTERL anomalies (vertebral, 17%; anal, 12%; cardiac, 20%; renal, 16%; limb, 10%) and other midline defects (cleft lip and palate, 2%; sacral dysgenesis, 2%; urogenital anomalies, 5%). The following medical problems were also reported: oesophageal dysmotility, 21%; gastro-oesophageal reflux, 22%; chest infections, 6%; and autonomic dysfunction, 0.5%. The first-degree relatives of children with OA are much more likely to have one of the aforementioned malformations or medical conditions when compared with the control group: one or more VACTERL anomalies (P < 0.01), gastro-oesophageal reflux (P < 0.05), recurrent respiratory infections (P < 0.05), and autonomic dysfunction (P < 0.001). The more distant relatives also show an increased incidence of such problems although in this case the data must be viewed with caution. The results confirm that the associated malformations and related medical problems occur significantly more frequently in the relatives of individuals with OA/TOF. These families may prove valuable for linkage analysis in an attempt to determine the genetics of OA/TOF.
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PMID:Oesophageal atresia, related malformations, and medical problems: a family study. 1037 10

The authors report a series of eight cases of isolated tracheoesophageal fistula without esophageal atresia (or an H type fistula), treated in three pediatric ENT departments. This is a rare malformation whose diagnosis requires investigation for associated anomalies. The clinical signs are mainly respiratory but also digestive and the symptomatology can be severe. The diagnosis can be made with a barium swallow combined with cineradiography, but a tracheoesophageal endoscopy remains the investigation of choice. The treatment is surgical. In most cases, the fistula is accessible by a right or left cervicotomy, depending on the surgeon's practice, with a much lower postoperative morbidity as compared to a thoracotomy. The postoperative management was straightforward in most of our cases. We discuss the role of gastro-esophageal reflux with respect to postoperative morbidity as well as systematic treatment for reflux peri-operatively. The pros and cons of the various surgical approaches are also discussed.
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PMID:Congenital tracheoesophageal fistula without esophageal atresia. 1040 20

From 1990 to 1998, 12 patients with columnar metaplasia of the lower esophagus were diagnosed. Only 4 of them displayed a Barrett's epithelium (BE) with a specialized columnar epithelium and globet cells. As it is already published, a male predominance was observed. In three of these patients some predisposing factor to develop BE was detected (i.e. severe central nervous system damage, chronic pulmonary disease, esophageal atresia and chemotherapy). All patients had severe gastroesophageal reflux (GER) with abnormal pH probe. Diagnosis was suggested by characteristic endoscopy images in 2 patients and was confirmed by biopsy in all cases. All patients had been primary treated with proton-bomb inhibitors. Two patients were treated by a Nissen fundoplication, 4 and 6 month after diagnosis, respectively. One patient with severe neurological damage will undergo the same surgery soon. In another patient with caustic esophageal injury, the affected esophagus will be resected, restoring continuity with stomach or colon. In view of the potential oncogenous transformation of BE, the importance of not overlooking this anomaly in all patients with severe GER is highlighted. All cases with predisposing factors to develop BE should be closely followed by periodic examination and multiple biopsies.
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PMID:[Barrett's esophagus: diagnosis and coexistent diseases in childhood]. 1053 57

Over a period of 19 years an antireflux procedure was performed for gastroesophageal reflux in 59 children. Thirty-two patients underwent Nissen fundoplication and 27 children underwent the Boix-Ochoa antireflux procedure. Six patients died between two and 15 months post surgery of unrelated causes. Follow-up period from six months to 18 years was available in 45 (85%) of the surviving patients. This report summarizes the complications and long-term results with the two surgical procedures and their comparisons. The follow-up evaluation included parental interview and physical examination. Upper GI series and pH monitoring were performed only in children with signs and symptoms of recurrent GER or other post-operative complications. At follow-up with a mean period of 8.7 years following Nissen fundoplication, 87.5% showed good results without any residual symptoms. However, the overall complication rate was as high as 50%. Following the Boix-Ochoa antireflux procedure, 17 (81%) children showed excellent results while four children had recurrent GER. This occurred in two neurologically impaired children and two patients following esophageal atresia repair. No other post-operative complications were encountered with the Boix-Ochoa antireflux procedure. In our experience, the Boix-Ochoa antireflux procedure should be the procedure of choice in the surgical treatment of GER in otherwise normal children while the Nissen fundoplication is preferable in neurologically impaired children and in patients with GER following esophageal atresia repair.
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PMID:Nissen fundoplication and Boix-Ochoa antireflux procedure: comparison between two surgical techniques in the treatment of gastroesophageal reflux in children. 1058 85

The improved survival of neonates with esophageal atresia and tracheo-esophageal fistula reflects the advancement in neonatal care and anaesthesia over the years. Chick embryo studies have given new insights in the embryopathy of esophageal atresia. It is now apparent that the various types of esophageal atresia could be explained due to selective discrepancy in the growth of the 3 folds in the region of tracheo-esophageal separation. The early disturbances in organogenesis which result in esophageal atresia also lead to other associated anomalies, the incidence of which varies from 40 to 55%. These anomalies have an important bearing on the survival outcome. The physiological aspects of esophageal atresia such as esophageal dysmotility and gastro-esophageal reflux are also vital in the long term and proper treatment of the associated defects. The criteria for an ideal esophageal substitute in long gap esophageal atresia have been determined and several options are now available with good results, such as: gastric transposition, colon, gastric tube and small intestine. IN developing countries, however, a high mortality is still attributed to late referrals, low birth weight, hypothermia and chest infection.
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PMID:Esophageal atresia and tracheo-esophageal fistula: a review. 1079 38

On the basis of the experience with treatment of esophageal atresia in 86 patients, it was established that cicatricial stenosis of the esophagus after radical correction of a defect occurs as a consequence of anastomotic suture failure, violation of the technique of placing an anastomosis, gastro-esophageal reflux. The recommendations on prevention and treatment of cicatricial esophageal stenosis are given.
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PMID:[The prevention and treatment of cicatricial esophageal stenosis after the correction of atresia]. 1091 66

Symptoms related to esophageal dysmotility are common following successful repair of esophageal atresia (EA) and tracheo-esophageal fistula (TEF). Esophageal manometry was performed in 27 survivors of EA/TEF at a mean age of 30.5 +/- 30.3 months and in 25 age-matched controls (mean age 36.8 +/- 22.6 months). The patients were also evaluated clinically to grade them into 3 result-oriented groups "Excellent", "Good" and "Fair", depending on pre-existing criteria evaluating the ability and ease of swallowing. Gastroesophageal reflux (GER) was evaluated with the help of a radionuclide scan. The mean esophageal and peak esophageal pressures and therefore also the lower esophageal sphincter (LES) pressure were lower in the patients (12.4 +/- 5.5, 34.9 +/- 20.4 and 12.2 +/- 6.8 mm Hg, respectively) compared to the controls (21.1 +/- 6.9, 62.3 +/- 19.3 and 16.8 +/- 4.3 mm Hg, respectively). The mean body pressures were highest with "Excellent" results and lowest with "Fair" results and the LES pressures followed a similar trend. Likewise, in the patients with GER, LES pressure was 12.0 +/- 7.1, 12.3 +/- 3.7, 11.0 +/- 5.7 and 6.9 +/- 5.6 mm Hg with nil, mild, moderate and severe GER, respectively. The pressure and contractility profile of the esophagus was abnormal in the majority of patients, even in the absence of symptoms.
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PMID:Manometric evaluation of postoperative patients of esophageal atresia and tracheo-esophageal fistula. 1180 65


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