Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0036690 (sepsis)
 59,461 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 29-year-old female, with chronic renal failure and chronic bilateral emphysema, was admitted with severe uremia and septicemia secondary to multiple abscesses in the right kidney. Her condition improved after right nephrectomy. Pulmonary function studies showed marked obstructive and restrictive lung disease consistent witht the diagnosis of primary emphysema. On biochemical and histological examination, the liver was found to be normal. Alpha1-antitrypsin could not be demonstrated in the patient's serum at normal pH by any of the known techniques, but protein molecules with alpha1-antitrypsin antigencity were found at pH 4.8; this suggests a pH-dependent structural difference in alpha1-antitrypsin protein. Starch gel electrophoresis gave a multibanding pattern not previously described. A new form of apparent total alpha-1-antitrypsin deficiency is postulated.
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PMID:Apparent total alpha1-antitrypsin deficiency: report of a case. 97 22

74 premature infants with a birth weight of 1000 g or less were hospitalized in the period 1963-1975. 20 babies survived the first 4 weeks of life and 2 died during the second month of life from progressive lung disease. 77% of all patients were mechanically ventilated and 9 ventilated patients survived. The main causes of death were cerebral hemorrhage, severe asphyxia and septicemia. Prognosis was impaired by hypothermia, IRDS, gestational age below 27 weeks and premature rupture of membranes with bacterial infection. At the age of 1 year 8 out of 12 survivors followed up showed normal mental and motoric development. 3 children had minor or obvious signs of cerebral paresis and one further child has partial retrolental fibroplasia. Due to modern intensive care the survival rate of these very small premature babies has now reached 40%. The prognosis is favourably influenced by optimal obstetric and neonatal care, while special precautions should be undertaken to avoid hypothermia. Our results with mechanical ventilation justify the administration of full intensive care to these very small premature babies.
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PMID:[The premature infant below 1000 g]. 99 16

Significant progress has been made since the first successful human heart-lung transplantation (HLT) for pulmonary vascular disease performed in 1981. The refinement of surgical techniques, use of cyclosporin as the main immunosuppressant, technique of distant organ procurement to expand the donor organ pool, and improved diagnosis and management of pulmonary infection and rejection have all contributed to this accomplishment. This has inevitably coincided with the extension of this procedure to other groups of patients with end stage heart and lung disease. Initially, HLT was offered to patients with cardiac disease associated with pulmonary hypertension. Because of the success, consideration was given to transplantation for parenchymal pulmonary diseases, initially pulmonary fibrosis and emphysema, and then suppurative lung disease such as in cystic fibrosis (CF). However, the application of HLT to patients with CF lagged behind because of concern related to the risk of sepsis, the systemic nature of the disease, malnourishment, and fear of recurrence of the epithelial CF defect in the transplanted lungs.
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PMID:Lung transplantation for cystic fibrosis. 145 9

A 59-yr-old man with bullous lung disease developed a refractory bronchopleural fistula involving the right upper lobe. Despite independent lung and high-frequency jet ventilation, a large air leak persisted. Following the introduction of a bronchial blocker into the right upper lobe bronchus via the tracheal lumen of a left-sided endobronchial tube, oxygenation and ventilation improved, and the airleak was reduced by 90%. The presence of pneumonia led to an inexorably downhill course with death from overwhelming sepsis.
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PMID:Lobar bronchial blockade in bronchopleural fistula. 154 1

Cystic fibrosis (CF) is the commonest, fatal, autosomal recessive disorder and is associated with lung sepsis, pancreatic failure and elevated sweat electrolytes. The CF gene on chromosome 7 encodes a protein identified as CF transmembrane conductance regulator (CFTR) which regulates chloride ion transport in epithelial cell membranes. Almost 100 mutations have been identified in this gene which cause defective chloride-channel control. Recently, this abnormality has been reversed in affected CF cells in vitro by retrovirus-mediated transfer of a normal gene. Fifty years ago, most cases died in childhood, but now up to 80% reach adulthood. Chronic lung sepsis is the principal cause of death, and intensive antibiotic therapy with chest physiotherapy is used to control this. Advanced lung disease can be successfully treated by heart-lung transplantation. Nebulised recombinant DNase and antineutrophil elastase agents such as alpha-1-antitrypsin and secretory leucoprotease inhibitor are potentially promising new therapies. Pancreatic insufficiency is managed by high-calorie diets and enteric coated enzyme supplements. Other prominent gastrointestinal complications include meconium ileus equivalent, biliary cirrhosis and cholelithiasis. Specially dedicated CF centres have led to improved survival rates and allow experienced staff to treat the many complications of CF while promoting research in this multisystem disorder.
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PMID:Cystic fibrosis in adolescents and adults. The coming of age of cystic fibrosis. 155 Dec 44

Despite the generally salutary experience in recent years of managing suppurative pleuropulmonary disease, empyemas and lung abscesses have persisted and increased in incidence in hospitals such as Queens Hospital Center that serve large numbers of the socioeconomically disadvantaged. This study documents the etiology, clinical presentation, treatment, and treatment results of suppurative pleuropulmonary disease at Queens Hospital Center, which serves a large segment of the urban poor, many of whom are black. Results indicate that contributory or antecedent etiologic factors include a history of prior disease (specifically pneumonia, lung abscess, obstructive lung disease, pulmonary neoplasia, and tuberculosis); a predisposition to constitutional or immunologic deficiencies (specifically, alcoholism, anemia/malnutrition, drug abuse, and acquired immunodeficiency syndrome [AIDS]); conditions contributing to tracheobronchial aspiration (specifically, alcoholism and seizure disorders); and a miscellaneous group such as prior surgery, cardiovascular disease, and sepsis syndrome. The patients in this study were young with maximal incidence occurring in the third to fifth decades of life. Patients were predominantly male (75%) and black (66%). There were 18 deaths (23%), with sepsis being the cause in 10 (56%). Most surgical interventions were conservative, ie, bronchoscopies (48), thoracenteses (43), and tube thoracotomies (39). Thirty-one open thoracotomies were performed for drainage, decortication, or pulmonary resection. The surgical mortality was three cases or 5% of the patients who underwent surgery. The designated incidence of proven AIDS in this series (29%) was low, undoubtedly because many patients refused testing, and the multiple gram-positive and gram-negative infections that were seen did not conform to the Centers for Disease Control criteria for diagnosis and case reporting for AIDS.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:The role of surgery in treating pleuropulmonary suppurative disease--review of 77 cases managed at Queens Hospital Center between 1986 and 1989. 160 13

We analyzed 14 cases of colovesical fistula that had been diagnosed and treated at the urology and gastroenterology and general surgery services of our hospital from 1986-1990. In 85.7% of the cases, the fistula had been caused by a tumor (adenocarcinoma of the colon) and in 14.3% by inflammatory disease (diverticulitis of the colon). All patients presented a varying degree of micturition syndrome; 30% had pneumaturia and 40% fecaluria. Cystoscopy proved to be the most effective in diagnosing colovesical fistula. It permitted visualization of the fistula or passage of fecal material to the bladder in 33% of the cases, while indirect endoscopic signs could be observed in 100%. Furthermore, it permitted the anatomopathological diagnosis of adenocarcinoma of the colon in 5 cases. We performed one-stage en bloc radical surgery in 57% of the cases, shotgun barrel discharge colostomy in 2 cases, exploration laparotomy in 3 and treatment was withheld in 1 case. The overall survival for the group submitted to radical surgery was 19.5 +/- 8.0 months. There were 4 deaths from metastasis, 2 from sepsis originating in the abdomen and the remaining deaths were due to iliofemoral venous thrombosis, cardiovascular disease and pneumopathy.
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PMID:[Vesico-sigmoidal fistulas. Evaluation of the diagnostic performance of the complementary tests in our series]. 181 45

Among 659 infants of 30 weeks' gestation or less born in a regional perinatal centre between 1983 and 1989, 195 were ventilated for four days or more and survived to 28 days, and 87 of these developed chronic lung disease. There was a sevenfold increase in the annual incidence of chronic lung disease over time. During the same period there were significant increases in the number of infants who survived, the incidence of septicaemia, and the use of parenteral lipid emulsions. Chronic lung disease was significantly associated with low birth weight, shorter gestation, duration of ventilation, vaginal delivery, sepsis, and the use of lipid. Respiratory and physiological measurements at 96 hours were significantly worse in infants who subsequently developed chronic lung disease. Initial logistic regression showed that gestation, arterial carbon dioxide tension (PaCO2), and ventilation rate at 96 hours; and birth in 1988 or 1989, were independently associated with chronic lung disease, but when septicaemia and use of lipid during the first 21 days were included, only gestational age (odds ratio 0.64, 95% confidence interval (CI), 0.49 to 0.81 for each week) and use of lipid (odds ratio 8.1, 95% CI, 2.32 to 28.0) remained significantly associated with chronic lung disease. The observed increase in incidence of chronic lung disease in this population was associated with earlier use of parenteral lipids in infants of very low gestation rather than with changes in population, survival, or ventilator treatment of respiratory distress syndrome.
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PMID:Factors associated with chronic lung disease in preterm infants. 173 38

Invasive pulmonary aspergillosis usually occurs in severely immunocompromised or neutropenic patients. Six patients with invasive aspergillosis are described whose only defence impairment was underlying lung disease and corticosteroid treatment. Cough, fever, and sputum production were the usual reasons for presentation and four patients developed the sepsis syndrome. Radiographic findings included de novo cavitation in three patients and rapid radiographic progression in four. Aspergillus species were isolated from respiratory secretions of all patients early in the course of the disease. Treatment was effective in only two patients and the subsequent progress of the others was consistent with a chronic necrotising process. Invasive pulmonary aspergillosis is uncommon in patients with respiratory diseases receiving corticosteroids, but should be considered when pneumonia and cavitary infiltrates occur.
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PMID:Corticosteroid treatment as a risk factor for invasive aspergillosis in patients with lung disease. 158 5

The effect of a 21-day (reducing dose) course of dexamethasone upon platelet numbers, neutrophil numbers and neutrophil morphology in ventilator-dependent preterm infants with chronic lung disease is reported. Forty-one infants, who were sepsis-free throughout the treatment period, received 46 courses of dexamethasone. In these infants the circulating platelet count increased significantly from 235 +/- 157 to 476 +/- 208 X 10(9)/L. Both total neutrophil numbers (7.94 +/- 7.16 to 19.56 +/- 12.40 X 10(9)/L) and immature neutrophil numbers (1.19 +/- 1.22 to 2.68 +/- 2.58 X 10(9)/L) also showed a significant increase reaching a peak on day 7 of treatment and decreasing thereafter. The immature:total (I:T) neutrophil ratio remained essentially unchanged. Eight other infants who developed serious sepsis during the treatment period showed a fall in both platelets (293 +/- 236 to 140 +/- 170 X 10(9)/L) and total neutrophil numbers (18.61 +/- 10.83 to 15.76 +/- 10.53 X 10(9)/L), together with an increase in immature neutrophils (2.30 +/- 2.80 to 8.22 +/- 13.91 X 10(9)/L) and consequent increase in I:T ratio.
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PMID:The effect of dexamethasone upon platelets and neutrophils of preterm infants with chronic lung disease. 188 47


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