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Query: UMLS:C0011849 (diabetes)
 277,896 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The authors report a case of Steinert's disease in a woman and discuss the endocrine profile of this disease after giving an account of the criteria of diagnosis. Disorders of gonad function are mild in women, primary testicular atrophy is very frequent in man with reduction in 17-ketosteroids and testosterone. Thyroid function was normal but, in a few cases, a low fixation curve was found (our case) corrected by TSH stimulation. The frequency of cataract emphasizes the interest of this sign for detection. Diabetes, associated with hyperinsulinism, seemed more frequent than in a population without Steinert's disease. The pathogenesis of these endocrine disorders appears secondary and is ill explained if one considers it as a single disease. Better knowledge, no doubt linked to progress in biochemistry of normal and myopathic muscle, will help to explain the pathogenesis.
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PMID:[Endocrine aspects of Steinert's disease]. 18 36

Muscle capillary basement membrane width (MCBMW) was measured in 18 myotonic dystrophy patients and compared with that in age- and sex-matched normal and diabetic subjects. The MCBMW in myotonic dystrophy patients (773 +/- 258 A) was significantly thinner than in normal subjects (925 +/- 181 A, P less than 0.05) or in diabetics (1224 +/- 614 A, P less than 0.01). An increase in MCBMW with advancing age was present in all groups but was greatest in the myotonic dystrophy groups (r = +0.59, P less than 0.01). There was no relation between MCBMW and either the degree of glucose intolerance or insulin hypersecretion in the myotonic dystrophy group, though none had fasting hyperglycemia. This is the first report of a condition associated with thinner-than-normal capillary basement membrane.
Diabetes 1979 Jul
PMID:Thin muscle capillary basement membranes in myotonic dystrophy. 44 22

A 71-year-old woman complained of "smoky" vision, which was found to be caused by a hyphema with blood dripping from a vascular tuft located in the 12 o'clock meridian of the iris. Fluorescein angiography delineated vascular tufts and argon laser photocoagulation eradicated one of the tufts that bled. Histopathologic studies of iris obtained at the time of cataract extraction showed an aggregate of small vessels at the pupillary margin. Most patients with vascular tufts of the pupillary margin have no systemic disease but they are also observed in diabetes mellitus and myotonic dystrophy.
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PMID:Vascular tufts of pupillary margin of iris. 86 91

Internalized capillaries limited to type 1 muscle fibers were noted in seven patients. They occurred in each case in association with a similar admixture of neurogenic and myopathic features that included atrophic and hypertrophic fibers, internal nuclei, fiber splitting, and endomyseal and perimyseal fibrosis. Internalized capillaries in enlarged type 1 fibers arose from fiber splits on step section study of four patients. They occurred in the gastrocnemius, quadriceps, and soleus muscles from patients with a variety of disorders that included Becker dystrophy, diabetes mellitus and strenuous leg activities, Achilles tendon rupture, and myotonic dystrophy. Exercise-induced myalgias were noted in the four patients with the most plentiful intramuscular capillaries, and in three of these muscle hypertrophy was present. The concurrence of internalized myofiber capillaries and exercise-induced myalgias may represent an associated biochemical/pathological defect.
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PMID:Internalized myofiber capillaries: observations on their origin and clinical features. 272 49

The association of motor nerve conduction velocity (MNCV) to (1) duration of symptoms, (2) deep tendon reflex responses, (3) clinical muscle atrophy, and (4) ultrastructure of quadriceps muscle was studied in 18 patients with myotonia dystrophica of Steinert and nine normal controls. These patients had neither diabetes mellitus nor any other type of muscle dystrophy. Ultrastructural features of muscle fibers and intercellular spaces between atrophic fibers provided a basis for identifying degenerative changes and evaluating them semi-quantitatively. Our study indicates presence of an association between the pattern of muscle degeneration and both MNCV (correlation coefficients, gamma = +0.60) and duration of symptoms (gamma = -0.62), but not between MNCV and duration of symptoms (gamma = +0.28). Further analysis of the association between the degeneration of quadriceps and the MNCV of a distant peroneal nerve (which does not innervate quadriceps) suggested that the systemic nerve degeneration occurred in some groups of myotonia patients. Our study indicates that while in some patients the muscle degeneration may have been associated with the impairment of neurogenic elements, in others it occurred in the absence of any MNCV abnormality. Our findings favor the role of both neuropathic and myopathic factors in the muscle degeneration seen in myotonia dystrophica.
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PMID:Ultrastructural relationship of quadriceps muscle degeneration with a distant peroneal nerve conduction in human myotonia dystrophica. 298 Jul 38

Iris microhemangiomas (IM) are benign proliferations of small, twisted blood vessels along the pupillary margin. They are usually bilateral and appear to be developmental in nature. IMs most commonly occur in patients with myotonic dystrophy and adult-onset diabetes mellitus, but have also been associated with respiratory disease, congential heart disease, and central retinal vein occlusion. Additionally, they may be found in individuals without obvious ocular or systemic abnormality. The etiology remains obscure. In this report, illustrative cases are followed by a brief discussion outlining typical clinical manifestations, etiological considerations, and possible implications.
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PMID:Iris microhemangiomas. 318 74

A case of hydramnion by congenital myotonic dystrophy is presented. By a 31 years old primipara typical causes of hydramnion as fetal malformations, immunologic or non-immunologic hydrops, diabetes mellitus or intrauterine infections could be excluded. Because of a severe fetal dystrophy with imminent asphyxia a caesarean section has been performed in the 35th. week of pregnancy. The newborn showed all clinical symptoms of congenital myotonic dystrophy and died after four weeks in a respiratory distress. A muscle biopsy of the m. quadriceps confirmed the diagnosis. EMG examination of the parents revealed a conductoring state of the mother. Possible causes of "idiopathic" hydramnion and possibilities of prenatal diagnosis of congenital myotonic dystrophy are discussed.
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PMID:[Polyhydramnios in congenital myotonic dystrophy]. 328 17

Oral glucose administration to normal humans stimulates insulin release and simultaneously enhances the action of insulin by producing a rapid increase in tissue insulin sensitivity by a mechanism separate from the amount of hormone released. We determined whether insulin-resistant patients with myotonic dystrophy lose the ability to produce the normal rapid increase in tissue insulin action after oral glucose. Nine ambulatory, nonobese men with myotonic dystrophy were studied with 120-min euglycemic insulin infusions (20 mU X m-2 X min-1) given before and after glucose ingestion (4 and 5 patients received 15- and 25-g loads, respectively). Identical studies were performed in nonobese normal volunteers (16 and 13 patients received 15- and 25-g oral glucose loads, respectively). Glucose infusion rates at 20-120 min (GIR20-120) during euglycemic insulin infusions without prior glucose were 2.87 +/- 0.6 mg X kg-1 X min-1 in patients with myotonic dystrophy compared to 4.70 +/- 0.3 mg X kg-1 X min-1 in normal subjects. Euglycemic insulin infusions after glucose ingestion were begun after arterialized blood glucose values had returned to baseline. After glucose ingestion by normal subjects, GIR20-120 increased by 44.4 +/- 7.1% (P less than .0001) and by 46.8 +/- 8.6% (P less than .0002) with 15- and 25-g glucose loads, respectively. GIR20-120 in the nine patients with myotonic dystrophy showed no significant increase after glucose ingestion. These results confirmed the existence of a decrease in whole-body insulin sensitivity in myotonic dystrophy and indicated that the patients lack the normal mechanism that enhances insulin action after oral glucose.(ABSTRACT TRUNCATED AT 250 WORDS)
Diabetes 1987 Jun
PMID:Lack of rapid enhancement of insulin action after oral glucose challenge in myotonic dystrophy. 355 1

The management of a patient with myotonic dystrophy undergoing Caesarean section for delivery of twins in breech position is reported. Anaesthetic management must reflect the multi-system nature of the disease in addition to the implications of pregnancy. Known triggers of myotonic crisis (succinylcholine, shivering) must be avoided. Attention to respiratory reserve is necessary in view of the restrictive defect this disease can impose. Cardiomyopathy and conduction system disturbances may be features. Upper gastrointestinal incoordination can superimpose the added risk of aspiration on the pregnant myotonic. Ineffective uterine contraction can cause vigorous post partum haemorrhage. Endocrine disturbances, especially diabetes mellitis, may be present. There may be increased sensitivity to sedative and narcotic agents. The newborn myotonic will need the expertise of neonatal care.
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PMID:Caesarean section for twins in a patient with myotonic dystrophy. 402 70

Iris neovascular tufts are biomicroscopic capillary outgrowths from the pupillary margins. Iris tufts have been reported to occur in the elderly, in diabetes patients, and in myotonic dystrophy patients. Three cases have also been described in association with retinal vein occlusion. This paper describes a patient with unilateral acute retinal branch vein occlusion who had bilateral iris neovascular tufts. The bilaterality of this elderly patient's tufts suggested an underlying acquired systemic disorder. The patient denied medical illness, except for hypertension. Medical evaluation disclosed an insulin tolerance test diagnostic of diabetes, although a glucose tolerance test was not markedly abnormal. Iris tufts appeared to be associated with abnormal insulin secretion. Their occurrence with branch vein occlusion may be coincidental.
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PMID:Iris neovascular tufts. 616 13


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