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Query: UMLS:C0017168 (gastroesophageal reflux disease)
 11,783 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The histological and histochemical findings in the respiratory muscles of a patient with severe neonatal nemaline myopathy are described. The patient suffered from frequent pneumonia associated with vomiting due to gastroesophageal reflux and died at 3 months from respiratory failure. The diaphragm was moderately involved and the intercostal muscles mildly involved. Core/targetoid structures were observed in the diaphragm and intercostal muscles.
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PMID:Severe neonatal nemaline myopathy--histological and histochemical studies of respiratory muscles. 162 19

We analyzed the course of 79 adult patients treated for achalasia between 1977 and 1988. Sixty-six patients (84%) had pneumatic dilatation as the primary therapy. Fifty-three patients (80%) had immediate improvement in swallowing. Three patients required immediate redilatation, 2 developed pulmonary aspiration, and 8 (12%) suffered esophageal perforation. Esophageal perforation was treated by closure plus Heller's myotomy in 3 patients, closure only in 3, chest tube in 1, and antibiotics and nasogastric suction in 1. At 4 years' follow-up, 50% of patients who had dilatation remained asymptomatic, 30% had symptoms of gastroesophageal reflux, and 20% had persistent dysphagia. Eight Heller myotomies were performed, with excellent results in 7 and 1 postoperative death from respiratory failure. Seven additional patients with disabling esophageal symptoms after multiple operations for achalasia were ultimately treated by esophagectomy (n = 5), hemigastrectomy and Roux-en-Y gastrojejunostomy (n = 1), and repeated myotomy (n = 1). All recovered and are able to eat solid food. Thus, our experience indicates that pneumatic dilatation remains unperfected (ie, the line between undertreatment and overtreatment is finer than generally recognized), and unless improvements can be made, the role for surgery may need to be reexpanded.
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PMID:The treatment of achalasia. A current perspective. 275 5

A severe infantile form of nemaline myopathy has a high mortality rate when untreated because of subsequent malnutrition and respiratory failure. Three infants with this condition demonstrated persistent vomiting, poor weight gain, and recurrent pneumonias. Esophageal manometry demonstrated decreased lower esophageal sphincter pressures and low amplitude peristalsis; 24-hour esophageal pH monitoring revealed significant gastroesophageal reflux. Medical therapy was ineffective in relieving symptoms. After antireflux surgery, vomiting and respiratory symptoms ceased, and there was no longer significant gastroesophageal reflux during pH monitoring. Our experience indicates that in some infants with nemaline myopathy a severe form of gastroesophageal reflux develops that is not responsive to medical therapy. Early surgical intervention may decrease life-threatening complications associated with gastroesophageal reflux in these infants.
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PMID:Gastroesophageal reflux associated with nemaline myopathy of infancy. 333 76

Gastroesophageal reflux (GER) was initially diagnosed in two black infants, aged 5 and 9 months, as a cause of their chronic lung disease and failure to thrive. Both infants were treated with bethanechol chloride as part of the management of their GER, but respiratory failure developed in both patients and they required ventilatory support. Both infants had severe air trapping, CO2 retention, difficulty in being weaned from mechanical ventilation, and Staphylococcus aureus cultured from their respiratory tract secretions. These factors led to the suspicion of cystic fibrosis (CF), and this diagnosis was subsequently confirmed by sweat test. The condition of both infants improved substantially on withdrawal of bethanechol therapy and the institution of a regimen of CF care. The early diagnosis of GER in these infants may have led to a delay in diagnosis and treatment of CF.
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PMID:Cystic fibrosis and gastroesophageal reflux in infancy. 396 86

Familial dysautonomia (FD) is a rare incurable genetic disorder with multisystem involvement. Most of its clinical manifestations are related to disorders of the autonomic nervous system. The disease is associated with specific disturbances of the upper gastrointestinal tract: pharyngoesophageal dyskinesia, gastroesophageal reflux, and prolonged gastric emptying. About 40% of the dysautonomic children manifest repeat vomiting crises. In view of the extensive gastrointestinal symptomatology, children with FD are prone to repeated aspiration pneumonia and chronic respiratory failure, while inadequate calory and fluid intake may lead to a chronic state of hypovolemia and severe failure to thrive. Control of vomiting, prevention of aspiration due to abnormal swallowing, and the assurance of adequate calory intake are three major objectives in the treatment of the dysautonomic child. Medical treatment of the gastrointestinal disorders using different drugs has had limited success. This study reviews the surgical experience in ten children with FD. The type of the procedure used was determined by the severity of the upper GI disturbances. Nine children underwent gastroesophageal Nissen fundoplication and gastrostomy. In seven of them, a pyloroplasty was added. Gastrostomy alone was done in one patient only. Postoperative complications included transient dysphagia in four patients, gastric dilatation in four patients, and dumping syndrome in one. There has been no incidence of immediate postoperative death. One child died 6 months after operation from severe and irreversible respiratory failure. Following operation, the patients still suffered from dysautonomic crises but these were not associated with vomiting.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:The surgical management of children with familial dysautonomia. 408 89

We report a unique case of coexisting exogenous lipoid pneumonia, endogenous lipoid pneumonia (ELP), and pulmonary alveolar proteinosis (PAP) in a 5-year-old patient with severe neurodevelopmental disease. The patient presented with gastroesophageal reflux and presumed chronic lung disease resulting from recurrent aspiration pneumonias and succumbed to respiratory failure. The autopsy showed lipid-laden macrophages and periodic acid-Schiff-positive granular material in alveolar spaces and multilamellated structures within both alveolar macrophages and extracellular debris. These findings were similar to those in previous reports of coexisting ELP and PAP in the setting of gastroesophageal reflux. However, the present case differed by the presence of scattered large osmiophilic extracellular lipid vacuoles. Besides strengthening the association between ELP and PAP and their relationship to gastroesophageal reflux, this case suggests that they may arise together with exogenous lipoid pneumonia, through related mechanisms, in the setting of neurodevelopmental disease.
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PMID:Coexisting endogenous and exogenous lipoid pneumonia and pulmonary alveolar proteinosis in a patient with neurodevelopmental disease. 806 6

We describe an 18 year old male with pulmonary interstitial and intra-alveolar cholesterol granulomas (PICG), which developed to severe respiratory failure over 15 yrs. The histological diagnosis was made on the basis of open lung biopsy findings at the age of 3 yrs and autopsy at 18 yrs of age. Although the pathological features of the lung were similar to that of paediatric patients with lipoid pneumonia coexisting with pulmonary alveolar proteinosis (PAP), gastro-oesophageal reflux (GOR) and a diverse group of severe primary diseases, the patient lacked evidence for any of these. We believe the present case provides a new example of a diffuse-type of lipoid pneumonia coexisting with pulmonary alveolar proteinosis, which we call cholesterol granulomas.
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PMID:Diffuse progressive pulmonary interstitial and intra-alveolar cholesterol granulomas in childhood. 894 94

Children with cerebral palsy are at risk of developing obstructive sleep apnea, which is initially managed by medical therapy but often requires tracheostomy for stabilization of the airway. We report preoperative and postoperative polysomnographic findings in a prospective series of 18 patients with cerebral palsy and obstructive sleep apnea who were refractory to medical management and underwent aggressive surgical treatment of upper airway obstruction. Fifteen of the 18 children (83 percent) in whom tracheostomy was recommended were spared the procedure. Eighteen children with cerebral palsy failed medical management of obstructive sleep apnea and were advised to have tracheostomy. There were 9 boys and 9 girls, ranging in age from 9 months to 17 years and 6 months at the time of operation. Tonsillectomy and adenoidectomy was performed in 9 patients, turbinectomy and/or septoplasty in 9, tongue-hyoid advancement in 13, uvulopalatoplasty in 13, conventional mandibular advancement in 2, distraction osteogenesis of the mandible in 2, and tongue reduction in 7. A concomitant Wilkes-Brody procedure for drooling was performed in 6 patients. Preoperative and postoperative polysomnographic data were compared by means of a paired t test. The mean preoperative apnea index, respiratory disturbance index, and lowest oxygen saturation were 3.61, 7.02, and 73.7, respectively. Mean postoperative apnea index, respiratory disturbance index, and lowest oxygen saturation were 0.67, 1.44, and 88.2, respectively. Lowest oxygen saturation and respiratory disturbance index were both improved significantly, with p values of 0.0367 and 0.0021, respectively. Fifteen patients are tracheostomy-free (83 percent) at a mean follow-up time of 30 months (range 14 to 49 months.) Two (11 percent) of the children ultimately required tracheostomy, and one (6 percent) died from respiratory failure following the parents' decision not to proceed with further treatment. Our results confirm the efficacy of an aggressive surgical approach to the treatment of obstructive sleep apnea in neurologically compromised children. Many children and their families may potentially avoid the long-term commitment and cumulative hazards of tracheostomy. Additional strategies that have been adopted include identification and aggressive management of seizures, esophageal reflux, and excessive oral secretions and the application of mandibular distraction and skeletal expansion whenever feasible. Close postoperative monitoring is necessary with reoperation for recurrent symptoms of obstructive sleep apnea if documented by sleep study and associated with evidence of recurrent or residual morphologic abnormalities.
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PMID:Surgical treatment of obstructive sleep apnea in neurologically compromised patients. 904 81

Respiratory symptoms in children may be associated with underlying gastro-oesophageal reflux (GOR). We reviewed the case notes of 20 children who presented to us from June 1993 to June 1994 with respiratory symptoms and GOR. The patients consisted of 16 Malays, two Chinese and two Indians with equal number of males and females. Their age at diagnosis was less than one year in 17 patients. The earliest age at presentation was at the third day of life. All patients had major respiratory manifestations i.e. recurrent wheezing, recurrent cough and pneumonia. In addition, three patients had stridor and six patients had apparent life threatening episodes (ALTE). Fourteen patients required ventilation because of respiratory failure. Diagnosis of GOR was based on clinical grounds supported by barium oesophagogram in seven patients and ultrasound examination in 11 patients. Eight patients were fundoplicated because of ALTE and recurrent severe bronchospasm. On follow up, 14 patients had hyperactive airways requiring inhaled bronchodilator and steroid therapy.
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PMID:Gastro-oesophageal reflux in children with severe respiratory symptoms--clinical spectrum and management. 1096 86

Chronic lung disease of prematurity (CLD) is largely confined to preterm infants who require mechanical ventilation in the newborn period. Its development is associated with preterm labour and pulmonary inflammation secondary to oxidant stress, barotrauma of mechanical ventilation and antenatally--or postnatally--acquired respiratory tract infection. Pathological studies have shown that infants dying of established CLD have airway wall thickening secondary to increased airway wall smooth muscle mass, alveolar hypoplasia and pulmonary vascular re-modelling. These structural abnormalities are likely to account for the clinical problems of arterial hypoxemia and hypercapnia, tachypnea, recurrent wheezing and decreased exercise tolerance. Severity of the structural components may account for the clinical variation that is observed in a particular child. Management of CLD is aimed at decreasing the effects of hypoxemia and in maximising somatic, and by implication lung, growth. Low flow domiciliary oxygen and bronchodilators are used for arterial hypoxemia and recurrent wheezing. Systemic and inhaled corticosteroids may be beneficial but it is unclear if such treatment alters the natural history of CLD in the developing lung. Gastro-esophageal reflux should be sought in these infants and they should receive immunizations or immunoprophylaxis against respiratory tract pathogens. There is considerable concern that survivors of CLD may develop respiratory failure in early--or late--middle age.
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PMID:Chronic lung disease of prematurity: clinical and pathophysiological correlates. 1166 9


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