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

Congenital diaphragmatic hernia (CDH) is a life-threatening anomaly with a mortality rate of approximately 40-50%, depending on case selection. It has been suggested that new therapeutic modalities such as nitric oxide (NO), high frequency oxygenation (HFO) and extracorporal membrane oxygenation (ECMO) might decrease mortality associated with pulmonary hypertension and the sequelae of artificial ventilation. When these new therapies indeed prove to be beneficial, a larger number of children with severe forms of CDH might survive, resulting in an increase of CDH-associated complications and/or consequences. In follow-up studies of infants born with CDH, many complications including pulmonary damage, cardiovascular disease, gastro-intestinal disease, failure to thrive, neurocognitive defects and musculoskeletal abnormalities have been described. Long-term pulmonary morbidity in CDH consists of obstructive and restrictive lung function impairments due to altered lung structure and prolonged ventilatory support. CDH has also been associated with persistent pulmonary vascular abnormalities, resulting in pulmonary hypertension in the neonatal period. Long-term consequences of pulmonary hypertension are unknown. Gastro-esophageal reflux disease (GERD) is also an important contributor to overall morbidity, although the underlying mechanism has not been fully understood yet. In adult CDH survivors incidence of esophagitis is high and even Barrett's esophagus may ensue. Yet, in many CDH patients a clinical history compatible with GERD seems to be lacking, which may result in missing patients with pathologic reflux disease. Prolonged unrecognized GERD may eventually result in failure to thrive. This has been found in many young CDH patients, which may also be caused by insufficient intake due to oral aversion and increased caloric requirements due to pulmonary morbidity. Neurological outcome is determined by an increased risk of perinatal and neonatal hypoxemia in the first days of life of CDH patients. In patients treated with ECMO, the incidence of neurological deficits is even higher, probably reflecting more severe hypoxemia and the risk of ECMO associated complications. Many studies have addressed the substantial impact of the health problems described above, on the overall well-being of CDH patients, but most of them concentrate on the first years after repair and only a few studies focus on the health-related quality of life in CDH patients. Considering the scattered data indicating substantial morbidity in long-term survivors of CDH, follow-up studies that systematically assess long-term sequelae are mandatory. Based on such studies a more focused approach for routine follow-up programs may be established.
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PMID:The long-term follow-up of patients with a congenital diaphragmatic hernia: a broad spectrum of morbidity. 1884 73

Lung disease in advanced stages is often accompanied by multiple comorbidities. Often, these processes lead to significant morbidity and occasionally mortality. Symptoms of pulmonary comorbidities, including pulmonary embolus, pulmonary hypertension, sleep-disordered breathing, and lung malignancy, may be intertwined with symptoms of advancing lung disease. However, the occurrence of extrapulmonary processes, including depression, diabetes mellitus, osteoporosis, and gastroesophageal reflux disease, may be subtle. Clinicians caring for advanced lung disease should be aware of the many comorbid processes that may accompany advancing lung disease.
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PMID:Comorbidities of advanced lung disease. 1917 Feb 18

Congenital diaphragmatic hernia (CDH) is associated with pulmonary hypoplasia and pulmonary hypertension. The objective of this study was to assess pulmonary function and exercise capacity and its early determinants in children and adolescents born with high-risk CDH (CDH-associated respiratory distress within the first 24 h) and to explore the relationship of these findings with CDH severity. Of 159 patients born with high-risk CDH, 84 survived. Of the 69 eligible patients, 53 children (mean+/-SD age 11.9+/-3.5 yrs) underwent spirometry, lung volume measurements and maximal cardiopulmonary exercise testing (CPET). Results of the pulmonary function tests were compared with those from a healthy control group matched for sex, age and height. CDH survivors had a significantly lower forced expiratory volume in 1 s (FEV(1)), forced vital capacity (FVC), FEV(1)/FVC, maximum mid-expiratory flow and peak expiratory flow when compared with healthy controls. The residual volume/total lung capacity ratio was significantly higher. Linear regression analysis showed that gastro-oesophageal reflux disease was an independent determinant of reduced FEV(1) and FVC. CPET results were normal in those tested. High-risk CDH survivors have mild to moderate pulmonary function abnormalities when compared with a healthy matched control group, which may be related to gastro-oesophageal reflux disease in early life. Exercise capacity and gas exchange parameters were normal in those tested, indicating that the majority of patients do not have physical impairment.
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PMID:Pulmonary function and exercise capacity in survivors of congenital diaphragmatic hernia. 1928 41

Although infectiological stimuli, environmental factors and genotypic features are known to contribute to the initiation and perpetuation of systemic sclerosis (SSc), its etiology still remains to be enigmatic, and less elusive insights are to be achieved by ongoing and future investigations. Being characterized, however, as chronic autoimmune disease with excessive collagen accumulation in skin, synovia and visceral organs such as lung, heart, and digestive tract along with obliterating angiopathy, the pathophysiology of SSc can be summarized as being based on imbalances of the cellular and humoral immune system, vascular dysfunction and activation of resident connective tissue cells. A complex interplay between these major components manages to establish and maintain the inability of the vasculature to adequately react to the need for dilatation, constriction and growth of new vessels, to cause the increased deposition of extracellular matrix constituents as well as to facilitate immunological disarrangement. Despite parallels to the chicken and egg causality dilemma, all of these account for what later clinicians observe in patients suffering from Raynaud's phenomenon, digital ulcers, sclerodactyly, rigidity of the face, microstomia, sicca syndrome, dyspnea, dry cough, pulmonary hypertension, palpitations, syncopes, renal insufficiency, dysphagia, gastroesophageal reflux, dyspepsia, generalized arthralgias, but also dyspareunia, or erectile dysfunction.
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PMID:The pathogenesis of systemic sclerosis revisited. 2008 91

Pregnancy in women diagnosed with systemic sclerosis is an infrequent situation with a generally favourable outcome according to the most recent studies. Women with systemic sclerosis who wish to become pregnant should obtain preconception counselling in order to withdraw teratogenic drugs and to carefully assess the disease activity. Severe organ impairment, early diffuse systemic sclerosis and pulmonary hypertension ought to discourage patients from pregnancy, as these situations are at high risk of complications for both mother and fetus during pregnancy and puerperium. During pregnancy, the most frequent maternal complication is worsening of gastroesophageal reflux, and the most severe complications are renal crisis and flaring arterial pulmonary hypertension. Angiotensin converting enzyme inhibitors are indicated at any stage of the pregnancy if renal crisis is suspected. Adverse obstetric outcomes are dominated by prematurity which may be the consequence of intra-uterine growth restriction or preeclampsia. Those complications might account for systemic sclerosis-associated vasculopathy, or for the presence of antiphospholipid antibodies. Patients with a previous history of placental insufficiency might benefit from treatment with aspirin, low dose anticoagulants or even nitric oxide donors. During the pregnancy, abnormal bilateral uterine artery Doppler is a predictor of vascular insufficiency and might guide initiation of further preventive treatments. Multicentric prospective cohort studies are warranted to identify more precisely the predictors of pregnancy-related complications and to define the best management of these patients.
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PMID:[Systemic sclerosis and pregnancy]. 2063 63

Obstructive sleep apnea (OSA) may be associated with myriad clinical consequences such as increased risk of systemic hypertension, coronary vascular disease, congestive heart failure, cerebrovascular disease, glucose intolerance, impotence, obesity, pulmonary hypertension, gastroesophageal reflux, and impaired concentration. Nonetheless, OSA remains undiagnosed in 82% of men and 93% of women with the condition. Early identification and treatment of OSA provides significant relief for individuals, prevents complications of OSA, and reduces overall health care costs. Better understanding of the pathogenesis, risk factors, diagnosis, and treatment of OSA has the potential to improve early recognition of OSA and prevention of adverse effects on the individual and society.
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PMID:Snoring and obstructive sleep apnea. 2073 12

Down syndrome (DS) is strongly associated with pulmonary hypertension, but there are many causes requiring a multi-disciplinary approach to the problem. Nearly half of children with DS have upper airway obstruction and the same proportion have congenital heart disease, both of which may cause pulmonary hypertension. Additional problems include pulmonary hypoplasia, structural lung disease and gastro-oesophageal reflux. It is no longer acceptable to ignore these symptoms as early treatment may be preventative.
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PMID:Management of pulmonary hypertension and Down syndrome. 2217 18

Congenital Diaphragmatic Hernia (CDH) is defined by the presence of an orifice in the diaphragm, more often left and posterolateral that permits the herniation of abdominal contents into the thorax. The lungs are hypoplastic and have abnormal vessels that cause respiratory insufficiency and persistent pulmonary hypertension with high mortality. About one third of cases have cardiovascular malformations and lesser proportions have skeletal, neural, genitourinary, gastrointestinal or other defects. CDH can be a component of Pallister-Killian, Fryns, Ghersoni-Baruch, WAGR, Denys-Drash, Brachman-De Lange, Donnai-Barrow or Wolf-Hirschhorn syndromes. Some chromosomal anomalies involve CDH as well. The incidence is < 5 in 10,000 live-births. The etiology is unknown although clinical, genetic and experimental evidence points to disturbances in the retinoid-signaling pathway during organogenesis. Antenatal diagnosis is often made and this allows prenatal management (open correction of the hernia in the past and reversible fetoscopic tracheal obstruction nowadays) that may be indicated in cases with severe lung hypoplasia and grim prognosis. Treatment after birth requires all the refinements of critical care including extracorporeal membrane oxygenation prior to surgical correction. The best hospital series report 80% survival but it remains around 50% in population-based studies. Chronic respiratory tract disease, neurodevelopmental problems, neurosensorial hearing loss and gastroesophageal reflux are common problems in survivors. Much more research on several aspects of this severe condition is warranted.
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PMID:Congenital diaphragmatic hernia. 2221 68

Congenital diaphragmatic hernia (CDH) is a life-threatening anomaly associated with a variable degree of pulmonary hypoplasia (PH) and persistent pulmonary hypertension (PPH). Despite remarkable advances in neonatal resuscitation and intensive care, and new postnatal treatment strategies, the rates of mortality and morbidity in the newborn with CDH remain high as the result of severe respiratory failure secondary to PH and PPH. Later, lung function assessments show obstructive and restrictive impairments due to altered lung structure and lung damage due to prolonged ventilatory support. The long-term consequences of pulmonary hypertension are unknown. Other problems include chronic pulmonary aspiration caused by gastro-oesophageal reflux and respiratory manifestations of allergy such as asthma or rhinitis. Finally, failure to thrive may be caused by increased caloric requirements due to pulmonary morbidity. Follow-up studies that systematically assess long-term sequelae are needed. Based on such studies, a more focused approach for routine multidisciplinary follow-up programs could be established. It is the goal of the French Collaborative Network to promote exchange of knowledge, future research and development of treatment protocols.
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PMID:[Congenital diaphragmatic hernia: respiratory and vascular outcomes]. 2240 23

A wide range of clinical consequences may be associated with obstructive sleep apnea (OSA) including systemic hypertension, cardiovascular disease, pulmonary hypertension, congestive heart failure, cerebrovascular disease, glucose intolerance, impotence, gastroesophageal reflux, and obesity, to name a few. Despite this, 82 % of men and 93 % of women with OSA remain undiagnosed. OSA affects many body systems, and induces major alterations in metabolic, autonomic, and cerebral functions. Typically, OSA is characterized by recurrent chronic intermittent hypoxia (CIH), hypercapnia, hypoventilation, sleep fragmentation, peripheral and central inflammation, cerebral hypoperfusion, and cerebral glucose hypometabolism. Upregulation of oxidative stress in OSA plays an important pathogenic role in the milieu of hypoxia-induced cerebral and cardiovascular dysfunctions. Strong evidence underscores that cerebral amyloidogenesis and tau phosphorylation--two cardinal features of Alzheimer's disease (AD), are triggered by hypoxia. Mice subjected to hypoxic conditions unambiguously demonstrated upregulation in cerebral amyloid plaque formation and tau phosphorylation, as well as memory deficit. Hypoxia triggers neuronal degeneration and axonal dysfunction in both cortex and brainstem. Consequently, neurocognitive impairment in apneic/hypoxic patients is attributable to a complex interplay between CIH and stimulation of several pathological trajectories. The framework presented here helps delineate the emergence and progression of cognitive decline, and may yield insight into AD neuropathogenesis. The global impact of CIH should provide a strong rationale for treating OSA and snoring clinically, in order to ameliorate neurocognitive impairment in aged/AD patients.
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PMID:Death by a thousand cuts in Alzheimer's disease: hypoxia--the prodrome. 2340 Jun 34


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