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)

Recurrent bleeding, the high occurrence of encephalopathy, and the impairment of hepatic function in the successful cases of portasystemic shunts have led to increasing dissatisfaction with these procedures in recent years. Between March 1974 and November 1990 we have operated on 15 children for bleeding esophageal varices using the Sugiura procedure (esophageal transection with paraesophagogastric devascularization). In two cases the entire procedure was performed via the thoracic approach. The spleen was left in place in five cases. We have had no mortality. Operative complications included bleeding in the early postoperative period in two children and partial leakage from the esophageal suture in two others. Follow-up was from 4 years 3 months to 16 years 8 months with an average of 10 years 4 months. Long-term results have been gratifying in 12 patients (80%) with disappearance of the varices and no evidence of recurrent bleeding. Three children (20% of the patients) had recurrent bleeding 4 1/2 years, 4 years 2 months, and 2 1/2 years after the surgical procedure. In all cases there was no evidence of esophageal stenosis, gastroesophageal reflux, or hiatal hernia, nor signs of encephalopathy nor impairment of hepatic function. In our opinion the Sugiura procedure is a valid procedure in the surgical treatment of esophageal varices bleeding in the pediatric age with a high rate of success (80%) and without late complications.
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PMID:Sugiura procedure in the surgical treatment of bleeding esophageal varices in children: long-term results. 147 3

Multiple level esophageal pH studies were performed in 23 neurologically damaged infants and children for evaluation of gastroesophageal reflux (GER) and feeding difficulties. The patients were placed in one of three anatomic groups based on the extent of their neurologic injury. Seven children had an acute cerebral injury due to closed head trauma or infections. Six patients with perinatal asphyxia or progressive encephalopathy had a global CNS insult. Eight children with CNS malformations or intraventricular hemorrhage had subacute cerebral damage. Two patients with generalized seizure disorders could not be anatomically classified. In all groups, abnormalities detected at the distal esophagus were also noted at more proximal levels. The middle esophageal probe demonstrated a significant difference (P less than .02) for the longest reflux episode between patients with subacute cerebral injury and those with a global insult. The difference (P less than .02) for the longest reflux episode detected by the distal pH sensor in globally damaged children compared with those with acute cerebral injury also persisted at the middle and proximal esophageal levels. Comparing these same groups, a difference (P less than .02) in acid clearance time and percentage of time pH less than 4 was noted only at the proximal esophageal level. Only the middle and proximal pH probes detected differences (P less than .02) for acid clearance time between patients with both types of cerebral damage and those with a global injury. In infants and children with CNS damage and suspected GER, monitoring the proximal and middle esophageal pH provides important information not detected by the distal esophageal sensor.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:The importance of acid reflux patterns in neurologically damaged children detected by four-channel esophageal pH monitoring. 341 77

Rebleeding, the long time high occurrence of encephalopathy and the impairment of hepatic function in the successful cases have led to increasing dissatisfaction in the last years with portasystemic shunt procedures. In the past 12 years we have operated on 14 children for bleeding esophageal varices using the Sugiura procedure (esophageal transection with paraesophagogastric devascularization); in two cases the entire procedure was performed through the thoracic approach. We had no mortality. Complications include bleeding in the early postoperative period in two children and partial leakage from the esophageal suture in two others. Patient follow-up has been between 16 months and 11 1/2 years with an average of 6 1/2 years. The long term controls have been gratifying in 12 patients with disappearance of the varices and no evidence of rebleeding, esophageal strictures, gastroesophageal reflux, encephalopathy or impairment of the hepatic function; in two other patients the operations was unsuccessful. In our opinion the Sugiura procedure should be the elected operation in the surgical treatment of esophageal varices bleeding in pediatric age, after an attempt with sclerotherapy.
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PMID:[Sugiura's operation in the treatment of hemorrhaging esophageal varices]. 349 85

Since 1974, 112 patients with ruptured esophageal varices, have undergone resection-anastomosis of the supracardial esophagus using the circular suture stapler. Recently, preliminary splenic artery ligature has also been associated, if possible, with systematic ligature of the gastric coronary vein and followed by cardioplasty, to prevent gastro-esophageal reflux and block subcardial venous flow. This highly selective portal decompression (HSPD) procedure provides lasting reduction in blood pressure (confirmed by manometric recordings) in the esophago-cardial region, without any reduction in the distal hepatic flow (no portocaval shunt) or increase in the proximal flow (raised portal pressure). Results were compared with those of the initial, already encouraging, protocol, and demonstrated a tangible improvement after more than one year follow-up. In 50 cases (Child A:16, B:29 and C:5), postoperative mortality was 10% (5 cases) during the first month and 7.5% (3 cases/40) during the first year. There was no specific morbidity due to the additional procedure nor cases of portocaval encephalopathy. During the first postoperative year, the frequency of hemorrhagic complications was one tenth of that during the year before surgery. These very encouraging results suggest the possibility of extending the indications for HSPD in the treatment of recurrent digestive hemorrhages from ruptured esophago-cardial varices, replacing porto-systemic shunts which are sometimes well tolerated but always anti-physiological.
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PMID:Highly selective portal decompression for bleeding esophageal varices. 387 25

We describe an A-to-G transition at nucleotide 10044 in the tRNA(Gly) gene of mitochondrial DNA in a sibship in which the proband died at age 8 years after a severe encephalopathy, a brother died of sudden and unexpected death, and the other six siblings had a combination of symptoms, including apparent life-threatening events and gastroesophageal reflux. This novel mutation was very abundant (> 90%) in liver and muscle of the proband and in several tissues, including blood, from his affected siblings (range 91-99%) but was less abundant in blood from the asymptomatic mother (88%) and maternal grandmother (85%). Our findings further enlarge the spectrum of clinical presentations associated with mitochondrial DNA mutations.
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PMID:Novel mutation in the mitochondrial DNA tRNA glycine gene associated with sudden unexpected death. 888 49

A 15-year-old girl with a former clinical diagnosis of cerebral palsy was found to have isolated deficiency of 3-methylcrotonyl-CoA carboxylase (MCC) on gas chromatography-mass spectrometry (GC/MS) analysis and enzyme determination. Her symptoms included marked growth retardation from birth, profound mental retardation, tonic seizures, rigospastic quadriplegia with opisthotonic dystonia, gastroesophageal reflux with poor esophageal peristalsis, and recurrent episodes of aspiration pneumonia. Brain MRI revealed marked brain atrophy, involving both the gray and white matter. Although she did not exhibit acute metabolic decompensation or acute encephalopathy, her neurological symptoms continuously worsened. This patient is the oldest among reported cases of MCC deficiency who had symptoms at birth, and this case may have the severest sequelae of the longest known natural course of this inborn error of metabolism.
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PMID:Isolated 3-methylcrotonyl-CoA carboxylase deficiency in a 15-year-old girl. 918 84

Conventional management of gastroesophageal reflux (GER) and hiatus hernia in children affected with encephalopathy can deteriorate their pulmonary function, already compromised by their leaning position and their spine deformations. The results after laparoscopic surgery for GER in 6 encephalopathy children are reviewed; their ages ranged from 9 to 14 years, their accompanying diseases were: spastic tetraparesia, hydrocephalus, scoliosis, epilepsy and Reye's syndrome. The laparoscopy procedures followed the same surgical steps as open surgery; the surgical time was 3 to 5 hours; the average hospital stay was 3 days. Oral intake started 8 hours after surgery. The successful results of these first cases in this group of patients with neurologic anomalies, suggest that laparoscopic Nissen funduplication is the treatment of choice for GER and hiatus hernia.
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PMID:[Laparoscopic Nissen fundoplication in children with encephalopathy]. 937 34

A prematurely born 5-year-old boy with chronic lung disease, hypoxic-ischemic encephalopathy, cerebral palsy, repeated aspiration pneumonia, and stroke underwent percutaneous endoscopic jejunostomy (PEJ) to alleviate repeated aspiration pneumonia. Studies, including 24-hour esophageal pH monitoring, 99mTc gastric emptying time, upper gastroesophageal barium radiography, and endoscopic examinations showed severe gastroesophageal reflux and prolonged gastric emptying. Percutaneous endoscopic gastrostomy (PEG) was performed first, followed by placement of a polyurethane J-tube (9 French) through the preexisting gastrostomy site. We passed the style-guided J-tube through the pyloric ring endoscopically and advanced it to the jejunum. The position of the J-tube was confirmed by radiologic study. Feeding with an elemental formula, 20 mL/hour, commenced immediately after the procedure, and the rate was gradually increased to 50 mL/hour. No further episodes of aspiration pneumonia have occurred since J-tube placement. Our initial experience with jejunal feeding through a PEJ is encouraging.
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PMID:Pediatric percutaneous endoscopic jejunostomy by J-tube extension through a preexisting gastrostomy site: a preliminary report. 988 93

The TRIAD of encephalopathy, subdural haemorrhages, and retinal haemorrhages is commonly considered diagnostic of Shaken Baby Syndrome, but the original paper describes a statistically linked QUADRAD of features, the fourth of which is a previous history of feeding difficulties (dysphagia). Recent reviews of giving pacifiers (dummies) to infants during sleeping periods have found a significant reduction in the incidence of Sudden Infant Death Syndrome. Stimulation of swallowing is a possible connection with dysphagia, which is examined here, illustrated by a well documented case. Although amniotic fluid passes freely through the larynx of fetal mammals during fetal breathing, application of pure water to the laryngeal epithelium in infants causes choking and laryngeal closure. "Water sensors" in the surface respond to lack of chloride ions and adapt very slowly or not at all. Others have found in puppies that following application of pure water only 32% resume breathing in less than 30-40s. The rest needed at least one saline flush, and some required artificial ventilation in addition. These receptors also respond to high potassium concentrations and acid or alkaline solutions. Normally, airway closure during swallowing or vomiting prevents entry of feed or oesophageal reflux, but in some forms of dysphagia leakage can occur, causing paroxysmal coughing, reflex laryngeal closure, and so prolonged apnoea. Recently, it has been realised that the TRIAD injuries can also result from high intracranial vascular pressures transmitted from intra-thoracic pressure surges during paroxysmal coughing, choking, etc. Triggering of such pressure surges by dysphagic accidents provides a physiological link to injuries commonly considered diagnostic of Shaken Baby Syndrome, completing the statistically identified QUADRAD of features. Further dysphagic research might reveal predictive factors, and preventative measures such as feeds of optimal pH.
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PMID:Dysphagia as a risk factor for sudden unexplained death in infancy. 1679 62

This article deals with the neurological basis of brainstem-related symptoms in disabled children. Synaptic interactions of respiratory and swallowing centers, which are briefly reviewed in this study, highlight the significance of the nucleus of solitary tract (NTS) in the stereotyped motor events. Coordination mechanisms between these two central pattern generators are also studied with a focus on the inhibitory action of decrementing expiratory neurons that terminate the inspiratory activity and become activated during swallowing. Dorsal brainstem lesions in hypoxic-ischemic encephalopathy (HIE) affect the area including NTS, and result in symptoms of apneusis, facial nerve paresis, dysphagia, gastroesophageal reflux, and laryngeal stridor. Leigh syndrome patients with similar distributions of medullary lesions show increased sighs, post-sigh apnea, hiccups, and vomiting in addition to the symptoms of HIE, suggesting pathologically augmented vagal reflex pathways. The present article also discusses the pathophysiology of laryngeal dystonia in xeroderma pigmentosum group A, self-mutilation in Lesch-Nyhan syndrome, and sudden unexpected death in Fukuyama congenital muscular dystrophy. Close observation and logical assessment of brainstem dysfunction symptoms should be encouraged in order to achieve better understanding and management of these symptoms in disabled children.
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PMID:Reflections on the brainstem dysfunction in neurologically disabled children. 1932 67


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