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 277,896 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Because the lifespan of patients with cystic fibrosis is now longer, both pediatricians and adult care physicians are involved in the health care strategy. Respiratory manifestations occur due to bronchial dilatation and chronic bronchial infection, mainly due to Staphylococcus aureus and Pseudomonas. Episodes of adult infection are frequent and death usually results from respiratory failure. Characteristically, the disease also involves exocrine pancreas insufficiency. Other intestinal tract manifestations include meconial ileus and liver disease which may reach the stage of biliary cirrhosis. Nutritional disorders are frequent. Clinically there are respiratory and digestive tract disorders, pansinusitis and frequent nasal polyposis, sometimes associated with diabetes mellitus or joint pain. Male sterility results from bilateral agenesia of the vas deferens and in the female, fertility is decreased although pregnancy is possible. Clinical presentation suggests the diagnosis which is confirmed by a sweat test and genetic analysis. Care should be provided by a centre specialized in cystic fibrosis. The main treatments rely on respiratory physical therapy, antibiotics and gastroprotected pancrease extracts.
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PMID:[Mucoviscidosis: in children and adults]. 756 75

Corticotropin-releasing hormone (CRH) is well-known to be a centrally acting respiratory stimulant after systemic application both in healthy subjects and in patients suffering from respiratory failure. In order to study the effects of CRH on sleep EEG and respiratory parameters during sleep, 14 healthy male volunteers were investigated in a single-blind placebo controlled design. After an adaptation night, polysomnography was performed during two successive nights between 23.00 hrs. and 7.00 hrs. During one night placebo was applied, on the other 50 micrograms ovine CRH was administered intravenously as a bolus every hour from 0.00 hrs. to 6.00 hrs. For the assessment of respiration, blood oxygen saturation and thoracic wall movements were measured, as well as nasal and oral airflow using the thermistor method. Sleep efficiency parameters and subjective perception of sleep quality were not affected following CRH. The following alterations were found regarding sleep architecture: REM sleep as well as slow wave sleep showed a tendency to decrease under CRH, whereas light sleep tended to increase. After an injection of CRH a stimulation of respiration could be observed, with an increase of tidal volume over a time interval of a few minutes. Blood oxygen saturation was only slightly increased. Cortisol and ACTH concentrations were found to be constantly elevated. These results indicate that respiration during sleep is clearly affected by CRH with only slight alterations of global sleep parameters. No association was found between stimulation of ventilation and the occurrence of arousals; the respiratory analeptic effect of CRH thus appears to be specific.(ABSTRACT TRUNCATED AT 250 WORDS)
Exp Clin Endocrinol Diabetes 1995
PMID:Effects of corticotropin-releasing hormone on respiratory parameters during sleep in normal men. 758 29

A 75-g oral glucose tolerance test (OGTT) was performed on 18 patients with chronic respiratory failure and without fasting hyperglycemia, positive urine glucose, or hepatic/pancreatic disorders. Underlying diseases in these patients were pulmonary emphysema (11 cases, 61%), pulmonary tuberculosis (5 cases, 28%), and chronic bronchial asthma (2 cases, 11%). The body mass index (mean +/- SD, 17.6 +/- 2.2 kg/m2, P < 0.001) in these patients was significantly lower than that (23.8 +/- 3.1 kg/m2) in normal subjects. The OGTT results showed an impaired glucose tolerance pattern in 9 cases (50%) and a diabetes mellitus pattern in 6 cases (34%). The mean two-hour plasma glucose value in the patients was 9.8 mmol/L. However, insulin secretion responded well to glucose loading. These results suggest that a high proportion of chronic respiratory failure patients may have an intolerance for glucose loading but a normal insulin secretion pattern.
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PMID:Glucose intolerance in chronic respiratory failure. 797 7

We have reviewed 70 consecutive elderly patients (70 years or older) who underwent pneumonectomy for bronchogenic carcinoma, in order to evaluate morbidity, mortality, and long-term survival. The majority of the patients had stage II (n = 32) or III (n = 25) disease. Fifteen deaths occurred in the peri-operative period (21%). Pre-operative factors associated with peri-operative death included a history of ischaemic heart disease (P = 0.001) and right-sided tumour (peri-operative mortality for right pneumonectomy = 37%, left pneumonectomy = 6%, P = 0.001). Poor lung function (as assessed by pre-operative spirometry), peripheral vascular disease, cerebrovascular disease, diabetes mellitus, and hypertension were not significant risk factors for peri-operative death. Post-operatively, the requirement for ventilation, or the development of post-operative myocardial infarction, adult respiratory distress syndrome and respiratory failure were significantly associated with peri-operative death. Over 60% of the patients developed one or more complications. The absolute survival rates for operative survivors were 51% and 27% at 1 and 5 years, respectively (stage I, 60% and 40%; stage II, 63% and 33%; stage III 33% and 14%). The absolute overall survival rates were 40% and 21% at 1 and 5 years, respectively. We conclude that pneumonectomy is justified in elderly patients but right-sided lesions and ischaemic heart disease should be considered as relative contra-indications.
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PMID:Pneumonectomy for bronchogenic carcinoma in the elderly. 804 86

The aim of this review was to demonstrate that RM function is altered in various endocrinopathies and that RM weakness is a common finding. RM function has been well-studied in diseases such as thyroid dysfunction, and steroid induced RM myopathies. Less well documented reports on RM function were found in parathyroid dysfunctions, disorders of mineralocorticoids and pituitary disturbances. Controversial reports were found in diabetes mellitus. No report was found connecting RM function with androgens, pheochromocytoma or adrenaline deficiency in humans. These diseases could potentially cause RM impairment leading to severe respiratory failure (pump failure) putting life in great danger. Therefore, it is obvious that further studies are needed to investigate the performance of RMs in endocrinopathies. Such studies are extremely urgent in Cushing's and Addison's disease, acromegaly, disorders of the adrenal medulla, and in diabetes insipidus.
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PMID:Respiratory muscles in endocrinopathies. 820 54

Necrotizing fasciitis (NF) is a rapidly progressive disease characterized by extensive necrosis of the skin, fascia, and subcutaneous tissue, with sparing of the underlying muscle. Diabetes mellitus, Bartholin's gland abscess, and recent surgical procedures (including episiotomy) are factors often found in obstetric and gynecologic patients. Mortality in this group of patients is higher than in the general surgical population. Death is usually due to overwhelming sepsis, renal and respiratory failure, and multiple organ failure. The infections are usually polymicrobial, with alpha-hemolytic streptococci, gram-negative coliforms, and anaerobic bacteria. Lower survival has been reported in large series when the groin is involved or when the general nutritional state is poor. From October 1988 to August 1990, we treated five patients with necrotizing fasciitis. Certain important characteristics of such patients have not been discussed in the obstetric and gynecologic literature. Nutritional status, with special emphasis on total protein, albumin, and the effects of alcoholism, has a significant impact on mortality. Nutritional support of these patients may improve survival. To limit the impact of secondary infections, surgical approaches should be modified by the anatomic location of the initial lesions. More frequent debriding in the operating room and early fecal diversion are recommended.
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PMID:Necrotizing surgical infection and necrotizing fasciitis in obstetric and gynecologic patients. 827 12

A surgical experience between October 1983 and December 1990, with 1467 consecutive patients 65 years of age and over, was used to compare patients receiving single internal mammary artery grafts (n = 736) with those receiving bilateral internal mammary artery grafts (n = 731). The mean age in the single-graft group was 73.2 years and 70.9 years in the bilateral-graft group (p < 0.001). Various clinical parameters were analyzed that revealed that the single-graft group had more women and more patients with unstable angina, a history of previous myocardial infarction, and emergency surgery (p < 0.05). There was no significant difference in cigarette smoking, hypertension, diabetes mellitus, hyperlipidemia, triple vessel coronary artery disease, left main coronary artery stenosis, or left ventricular function between the two groups. Hospital mortality for the single-graft group was 6.4% (47 patients) and 3.1% (23 patients) for the bilateral-graft group (p < 0.004). No significant difference was observed between the groups in the rate of reoperation for bleeding, sternal infection, respiratory failure, stroke, or perioperative infarction. Follow-up was obtained in 663 hospital survivors (96.4%) in the single-graft group and in 691 (97.7%) in the bilateral-graft group. Mean follow-up in the single-graft group was 42.5 months (range, 1 to 92.4 months) and 43.0 months (range, 1 to 90.3 months) in the bilateral-graft group. In the single-graft group, survival was 60.7% +/- 4.8% (+/- standard error of the mean) at 8 years (60 patients at risk); it was 67.9% +/- 8.1% (18 patients at risk) for the bilateral-graft group (p < 0.028). This comparative study demonstrates that bilateral internal mammary artery grafting can be accomplished in elderly patients with low operative risk and hospital morbidity. Moreover, patients in both groups had a reduction in cardiac events and significant functional improvement.
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PMID:Coronary bypass grafting in the elderly. Single versus bilateral internal mammary artery grafts. 832 Sep 91

Renal transplantation for infantile cystinosis corrects renal failure and prolongs survival. However, after transplantation, the disease may develop in the allograft and continue to progress in nonrenal organs. We studied seven children (6 boys, 1 girl) with infantile cystinosis who received 11 renal transplants (3 cadaver, 8 living-related) between May 1969 and December 1986. The age at transplant ranged from 6 to 12 years (mean, 9.1 years). Four children received second renal transplants at a mean age of 17 years (range, 16 to 22 years). The mean period of follow-up was 138 +/- 47 months. Three children received cysteamine therapy prior to transplantation. Nonrenal complications of infantile cystinosis present before transplantation were photophobia (n = 3), corneal crystals (n = 5), hypothyroidism (n = 1), rickets (n = 6), and short stature (n = 7). Graft and patient survival did not differ from controls matched for the time of transplantation. Two patients died (1 pneumococcal sepsis, 1 respiratory failure due to pulmonary fibrosis) with functioning grafts 5 and 14 years posttransplant. Complications that developed posttransplant included photophobia (n = 1), corneal crystals (n = 2), hypothyroidism (n = 4), polyneuropathy (n = 1), pulmonary fibrosis (n = 1), abnormal electroencephalogram without clinical seizures (n = 1), bladder stones (n = 1), and diabetes mellitus (n = 2). One patient received a corneal transplant. All seven children failed to show improvement in growth following transplantation. Cystine crystals are present in graft-infiltrating cells, but do not seem to affect kidney allograft function.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Renal transplantation for infantile cystinosis: long-term follow-up. 843 88

We report experiences in 3 patients with acromegaly while using the somatostatin analogue octreotide. In case 1, a 44 year old male developed pneumococcal meningitis 3 months after having transphenoidal surgery for a pituitary tumour. This occurred with the re-emergence of communication between the surgical tract and the C.S.F. In case 2 a 52 year old male with insulin resistant diabetes mellitus requiring 240 units/day, with greatly elevated growth hormone concentrations was able to stop insulin within 5 days of starting octreotide. In case 3, a 52 year old male with sleep apnoea syndrome, respiratory failure and resistant heart failure made a dramatic improvement which is maintained 2 years later. All cases were associated with substantial falls in growth hormone and insulin like growth factor-1 concentrations.
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PMID:Experiences with octreotide in acromegaly. 844 80

Wolfram syndrome was originally described as a combination of familial juvenile-onset diabetes mellitus and optic atrophy. Other neurological features subsequently emerged, and "DIDMOAD" (diabetes insipidus, diabetes mellitus, optic atrophy, and deafness) became a commonly accepted acronym. Here, we describe 4 further cases from 2 families, in whom there occurred previously unrecognized neurological features, central apnea and neurogenic upper airway collapse, together precipitating primary respiratory failure (fatal in 1 case), startle myoclonus (in 2 unrelated cases), axial rigidity, and Parinaud's syndrome. Magnetic resonance images revealed striking brainstem atrophy affecting, in particular, the pons and midbrain. The mitochondrial DNA from 3 cases (and relatives) showed no evidence of any of the previously reported abnormalities. These neurological and neuroradiological features, in conjunction with (1) analyses showing the neurodegenerative origin of optic atrophy, deafness, diabetes insipidus, and incontinence, (2) other previously reported neurological complications (including anosmia, ataxia, epilepsy, and neuropsychiatric and cognitive abnormalities), and (3) the very small number of published postmortem studies, indicate that Wolfram syndrome should be reemphasized as a unique hereditary neurodegenerative disorder with prominent optic atrophy and diabetes mellitus.
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PMID:Wolfram syndrome: hereditary diabetes mellitus with brainstem and optic atrophy. 860 54


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