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Query: UMLS:C0011854 (type 1 diabetes)
 20,749 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Cystic fibrosis is the most common lethal genetic disease occurring in the white population. It is estimated that 3.5% of the 20,000 individuals with cystic fibrosis in North America will die each year of end-stage lung disease. Lung transplantation (heart-lung or double lung) is becoming more frequent as more patients are referred for this procedure. Since January 1988, we have evaluated 60 patients with cystic fibrosis for lung transplantation and have accepted 30 (50%). Nine patients (30%) died while awaiting a donor. Fifteen patients underwent transplantation (13 heart-lung and two double lung procedures). Actuarial survival at 1, 2, and 3 years is 76%. All survivors are without physical limitations. Pulmonary function, as determined by forced vital capacity, forced expiratory volume in 1 second, and arterial blood gas determinations, is within the normal range. Comparing these data with those of a group of patients without cystic fibrosis who underwent transplantation during the same period did not reveal any significant differences with respect to infection, rejection, and outcome. Preliminary data suggest that obliterative bronchiolitis is less prevalent at 1 year in patients with cystic fibrosis (19%) than in those without cystic fibrosis (41%). Patients with cystic fibrosis present a number of challenges. The problems of pleural adhesions from repeated infections, pleurodesis, and previous thoracic procedures are now readily approached through the bilateral thoracosternotomy (clam shell) incision. Insulin-dependent diabetes mellitus and low-dose corticosteroid therapy are no longer considered absolute contraindications. Both septic lungs must be removed at operation, either with heart-lung transplantation or with double lung transplantation. These data support the therapeutic efficacy of lung transplantation for patients with cystic fibrosis.
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PMID:Cystic fibrosis. Target population for lung transplantation in North America in the 1990s. 156 53

Structure elucidation of a specific fluorophore from the aging extracellular matrix revealed the presence of a protein crosslink formed through nonenzymatic glycosylation of lysine and arginine residues. The unexpected finding that a pentose instead of a hexose is involved in the crosslinking process suggested that the crosslink, named pentosidine, might provide insight into abnormalities of pentose metabolism in aging and disease. This hypothesis was investigated by quantitating pentosidine in hydrolysates of 103 human skin specimens obtained randomly at autopsy. Pentosidine level was found to increase exponentially from 5 to 75 pmol/mg collagen over lifespan (r = 0.86, P less than 0.001). A three- to tenfold increase was noted in insulin-dependent diabetic and nondiabetic subjects with severe end-stage renal disease requiring hemodialysis (P less than 0.001). Moderately elevated levels were also noted in some very old subjects, some subjects with non-insulin dependent diabetes, and two subjects with cystic fibrosis and diabetes. The cause of the abnormal pentose metabolism in these conditions is unknown but may relate to hemolysis, impaired pentose excretion, cellular stress, and accelerated breakdown of ribonucleotides. Thus, pentosidine emerges as a useful tool for assessment of previously unrecognized disorders of pentose metabolism in aging and disease. Its presence in red blood cells and plasma proteins suggests that it might be used as a measure of integrated pentosemia in analogy to glycohemoglobin for the assessment of cumulative glycemia.
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PMID:End-stage renal disease and diabetes catalyze the formation of a pentose-derived crosslink from aging human collagen. 229 12

Urinary enzyme excretion and proteinuria were studied in 316 children with different underlying diseases. Activities on N-acetyl-beta-D-glucosaminidase and alanine aminopeptidase decreased progressively with age in the urine of 66 healthy prematures, newborns, infants or children. In 51 children with nephrotic syndrome, tubulopathies or chronic renal failure, excretion of NAG and AAP rose 3 to 30 fold. Contrary to molecular weight dependent protein analysis, determination of enzymuria did not allow to differentiate between glomerular and tubular disorders. After renal transplantation, 31 out of 52 children had a pathological enzymuria. NAG and AAP were more frequently elevated during treatment with cyclosporine A (21/29), than with azathioprine (10/23). The influence of nephrotoxic drugs upon enzymuria was documented in 14 children with cystic fibrosis or septicaemia treated with tobramycin. Activities of NAG and AAP rose transiently, whereas proteinuria remained almost unchanged. Only three out of 45 children receiving nonsteroidal antiinflammatory drug therapy for juvenile rheumatoid arthritis or spondylarthritis showed a pathological increase in enzymuria. Mean urinary NAG and AAP excretion in 154 children with insulin dependent diabetes mellitus were not different from controls and were unrelated to either duration of disease or HbA1 concentration. The determinations of urinary enzymes as non-invasive tests of renal integrity in medicine and toxicology provide a very sensitive indicator of renal damage. The assays of NAG and AAP have proven to be most valuable; however, due to a lack of specificity for the type and origin of renal dysfunction, these urinary enzyme assays are most useful when carried out in conjunction with electrophoretic analyses of proteinuria.
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PMID:[Enzymuria and kidney diseases in childhood]. 288 Nov 98

We have reviewed the hospital records of 24 patients with cystic fibrosis-associated diabetes, and 2 groups of CF patients (1 with normal and the other with abnormal oral glucose tolerance tests) who did not develop symptomatic fasting hyperglycemia, to define the clinical characteristics of the diabetes and to study its effects on the progression of the pulmonary disease, changes in sputum organisms, and mortality. Although maximum blood glucoses ranged from 322 to 1160 mg/dl with a median of 579 mg/dl, only 1 of 24 diabetic patients developed ketoacidosis. This patient developed diabetes 12 years prior to the diagnosis of CF and may have had type 1 diabetes. In contrast, hypoglycemia was frequent and 4 patients were hospitalized with serious neurologic manifestations. Two patients were found to have diabetic retinopathy, 1 with macular edema required laser photocoagulation to improve vision, and the other had multiple microaneurysms. CF-associated diabetes did not influence the deterioration of clinical scores, chest x-ray scores, pulmonary function tests, the number of hospital admissions, the type of organisms found in the sputum, or mortality rates. The development of diabetes in our CF patients was not related to the severity of pulmonary dysfunction, clinical, or chest x-ray scores. Thus, although the development of diabetes is an additional encumbrance upon the already therapeutically burdened existence of a CF patient, it does not appear to affect the course of the disease. Despite the demonstration of diabetic retinopathy in this study, most patients with CF-associated diabetes still do not live long enough to develop microvascular complications from the diabetes.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:The interaction of 2 diseases: diabetes mellitus and cystic fibrosis. 309 55

The prevalence of overt diabetes mellitus and carbohydrate intolerance was studied in 448 patients with cystic fibrosis (CF). Insulin-dependent diabetes (IDDM) developed in 7.6% of patients (13 male and 21 female). Survival was significantly lower (P less than 0.01) in the IDDM-CF group, with fewer than 25% surviving to age 30 years, whereas nearly 60% of the nondiabetic CF population reached this age. A significant deterioration in CF clinical status, based on NIH score, became apparent 2 years before onset of overt IDDM (P less than 0.05 at 2 years prior, P less than 0.01 at IDDM diagnosis). Total glycosylated hemoglobin (HbA1) was significantly (P less than 0.001) higher for the total CF population (7.3% +/- 1.2%) than for the general non-CF population (6.5% +/- 0.7%), and in the IDDM-CF group (P less than 0.05) compared with normoglycemic CF control patients. Female patients had a higher mean HbA1 after 12 years of age than their male counterparts did (P less than 0.02). HBA1 did not predict the development of IDDM, but there was a weak inverse relationship between HbA1 and both NIH clinical score (r = -0.41, P less than 0.02) and standard pulmonary function tests (forced vital capacity, r = -0.25, P less than 0.01) in the general CF population. Therefore, impaired carbohydrate tolerance in CF is associated with progressive clinical deterioration.
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PMID:Diabetes mellitus associated with cystic fibrosis. 334 74

Seven patients with type I diabetes mellitus (Group 1), seven with normoglycemic cystic fibrosis (Group 2), seven with hyperglycemic cystic fibrosis (Group 3), and ten age-matched control subjects underwent corneal fluorophotometry and quantitative specular microscopy. Group 1 had background microangiopathic retinopathy but no evidence of proliferative disease by fluorescein angiography. Significant increases in mean corneal endothelial permeability and mean pump rate occurred in Group 1, indicating a defect in the endothelial barrier function early in type I diabetes mellitus. Similar significant increases in mean corneal endothelial permeability and mean pump rate occurred in both cystic fibrosis groups. The greatest increase was found in Group 3, suggesting a primary defect in the endothelial barrier function in cystic fibrosis, aggravated by the hyperglycemic state. No morphologic abnormalities were noted in Group 1, but both cystic fibrosis groups had smaller mean cell areas than did the control group. There were significant differences in the morphologic and functional correlations between Groups 1 and 3, suggesting different mechanisms for the increased endothelial permeability in these two disorders.
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PMID:A morphologic and fluorophotometric analysis of the corneal endothelium in type I diabetes mellitus and cystic fibrosis. 407 74

A 14-year-old boy presented with Type I diabetes mellitus and subsequently developed pancreatic exocrine insufficiency and systemic sclerosis (SSc). His diabetes had been diagnosed when he was about 5 years old, after the onset of ketoacidosis. Insulin treatment was provided from then until the time he was treated in our department. Exocrine pancreatic insufficiency, which occurred at age 9, was treated with pancreatic extracts. Cystic fibrosis was excluded after a chloride sweat test. The diagnosis of SSc was confirmed at age 14 on the basis of skin sclerosis, sclerodactyly and oesophageal and pulmonary involvement and then at age 18 by the occurrence of Raynaud's disease. Thus, this patient demonstrated a rare and previously unreported association of Type I diabetes mellitus and systemic scleroderma. Limited joint mobility and skin abnormalities are frequent in childhood diabetes mellitus but should not be misdiagnosed as systemic scleroderma.
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PMID:Case report: insulin-dependent diabetes mellitus in childhood associated with scleroderma. 869 7

Cystic fibrosis-related diabetes mellitus (CF-DM) is thought to be secondary to beta-cell destruction by fibrous tissue replacing the exocrine pancreas. The aim of this study was to investigate the hypothesis that other factors may also be responsible. Glutamic acid decarboxylase (GAD) and islet cell (IA-2) antibodies were measured by quantitative ELISA in a group of patients with CF (n=30) in comparison to a group of newly diagnosed DM type 1 (IDDM) patients (n=30) and normal subjects (n=30). GAD antibodies were positive (>32 ng/ml) in 50% of the CF, 93% of the IDDM and 0% of the control group. IA-2 antibodies were detected (>0.9 U/ml) in 40% of the CF, 93% of the IDDM and 0% of the control group. Among the fifteen CF patients with positive GAD and IA-2 antibodies, four already had IDDM and another five abnormally low (<45 mU/l) first phase insulin response (FPIR) indicating a prediabetic state. We conclude that factors other than mechanical may be involved in the development of CFDM. The presence of autoantibodies predicting IDDM supports the hypothesis that CF-DM may have a multifactorial pathogenesis.
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PMID:Islet autoantibodies and insulin dependent diabetes mellitus in cystic fibrosis. 1071 59

This study evaluated paediatric nursing students' knowledge of diet therapy to establish whether it was sufficient to prepare them for practice. A questionnaire sampled 19 1st-year and nine 4th-year students' diet therapy knowledge in relation to chronic renal failure, cystic fibrosis, juvenile diabetes mellitus and liver disease. The knowledge of 1st and 4th-year students was compared and then evaluated against criteria, devised by the researcher to measure whether this knowledge level was sufficient for practice. The Mann-Whitney Utest showed a significant difference between the 1st and 4th-years' diet therapy knowledge. The mean score for overall diet therapy knowledge of 4th-year students was 46 per cent. The results suggest that knowledge of diet therapy is insufficient to prepare nursing students for practice and that this topic needs further emphasis in paediatric nurse education.
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PMID:Diet therapy--a forgotten art? 1122 22

Diabetes mellitus and cystic fibrosis (CF) have been reported before in the literature, but they have never been reported in the same patient in the Middle East. We present the first reported case of insulin dependent diabetes mellitus (IDDM) and CF in 2 siblings of the same family. Both siblings were diagnosed early in life with IDDM, and their diabetes was well controlled on insulin. Cystic fibrosis was diagnosed in the first case one year after IDDM was diagnosed due to history of chronic cough and in the 2nd case by family screening. Both had severe failure to thrive, recurrent chest infections and gastro-esophageal reflux. With treatment both showed clinical improvement, but continued to have moderate lung disease radiologically and by pulmonary function test.
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PMID:Diabetes mellitus and cystic fibrosis in 2 Saudi siblings. 1251 10


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