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

Werner's syndrome (adult progeria) is a rare autosomal recessive condition characterized mainly by a characteristic habitus (short stature, light body weight) scleroderma like changes of the limbs and premature aging. Chronic leg ulcers appears in about fifty per cent of the patients. These ulcers can be related to the combination of mechanical factors on atrophic subcutaneous tissue and skin of the feet and leg associated with early arteriosclerosis (20%) and diabetes mellitus (60%).
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PMID:[Leg ulcers in Werner's syndrome. Report of one case]. 179 75

Photopheresis, the process by which peripheral blood is exposed in an extracorporeal flow system to photoactivated 8-methoxypsoralen (8-MOP), is an effective new treatment for certain disorders caused by aberrant T lymphocytes. It has become a standard therapy for advanced cutaneous T cell lymphoma and shows promise in the treatment of four autoimmune disorders (pemphigus vulgaris, the progressive systemic sclerosis form of scleroderma, rheumatoid arthritis, systemic lupus erythematosus) and in reversal of immunologic rejection of transplanted organs. Positive immunologic alterations observed in patients with AIDS-related complex merit further investigation, and preliminary trials in the management of patients with multiple sclerosis, myasthenia gravis and autoimmune insulin-dependent diabetes mellitus have recently been initiated. The inability of the treatment to meaningfully alter the course of the B cell malignancy, chronic lymphocytic leukemia, suggests that B cell proliferations, at least those involving malignant cells, may be more resistant to this treatment. The mechanism of action of photopheresis is likely to be multifaceted, but at least in experimental systems appears to involve an immunization against the pathogenic T cells, in a highly specific manner. Photoactivated 8-MOP initiates a cascade of cellular events by forming covalent photoadducts with nuclear DNA, with cell surface molecules and possibly with other cytoplasmic components of the ultraviolet exposed leukocytes. For reasons not yet clear, exposure of populations of T cells containing expanding a clone(s) of pathogenic T cells to photoactivated 8-MOP alters these cells so that their reinfusion induces a therapeutically significant immunologic reaction that targets unirradiated T cells of the same pathogenic clone(s). It is suggested that the specificity of the induced immunologic reaction may result, in sequence, from the exquisitely titratable damage that 8-MOP inflicts upon cells of the pathogenic clone(s), the return of these cells to an immunocompetent individual, the removal of the photo-damaged cells from the blood by the reticuloendothelial system and the preferential induction of an immune response against cells of the pathologically expanded clone(s).
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PMID:Photopheresis: a clinically relevant immunobiologic response modifier. 179 5

The scleroderma-like syndrome (SLS) of diabetes consists of limited joint mobility and digital sclerosis. It is described in 8-50% of insulin-dependent diabetes mellitus patients and is the earliest clinically apparent long-term complication of diabetes in children and adolescents. The frequency of this finding appears to be related to the duration of diabetes and increasing age. Less clear are the relationships between this syndrome and glycaemic control or genetic factors. SLS is an easily identifiable marker for those young patients who may be at increased risk for the development of early microvascular complications. Recognition of this characterization will permit the clinician to focus on newer and improved techniques for long-term physiological control of the diabetes, in an attempt to prevent microvascular disease.
Diabetes Metab Rev 1991 Jun
PMID:The scleroderma-like syndrome of insulin-dependent diabetes mellitus. 179 57

Patients with Type I diabetes may develop a scleroderma-like syndrome, including limitation of joint mobility. This syndrome, cherioarthropathy, is considered a complication of diabetes, but its cause is unknown. We examined 30 Jewish and 13 Arab patients in our juvenile diabetes clinic for skin and joint involvement. Signs of cherioarthropathy in both hands were found in 13 children (30.2%); all had skin changes and 6 (13.5%) also had articular involvement of the hands. There was no correlation between the presence of cherioarthropathy and the patient's age or the duration of diabetes. The syndrome was significantly more frequent among Arabs (8/15, 61.5%) than Jewish children (5/30, 16.6%), p less than 0.01. There was a indirect correlation between incidence of cherioarthropathy and adequacy of glycemic control, but no difference in glycemic control between Arab and Jewish children. This may indicate a genetic factor in the development of cherioarthropathy in juvenile diabetes.
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PMID:[Diabetic hand syndrome in juvenile diabetes]. 193 31

247 patients with juvenile diabetes mellitus, aged 3-37 years, were examined for diabetic hand syndrome. 68 (27%) had 1 or more of the manifestations of diabetic hand syndrome. In 45 (18%) flexion contractures were found, 41 (17%) had skin changes resembling those of scleroderma and digital sclerosis, and 12 (5%) suffered from trigger finger. We found an association between diabetic hand syndrome and diabetes control as evaluated by serial levels of hemoglobin A1c measured during the years of follow-up. A high relative risk for microvascular complications was found in those who had diabetic hand syndrome, compared to the others. The relative risk for retinopathy was 2.5 times greater in patients with diabetic hand syndrome (p less than 0.001). These results show that diabetic hand syndrome is a common presentation of juvenile diabetes mellitus and can be utilized as a marker for some of its complications.
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PMID:[Diabetic hand syndrome in juvenile diabetics]. 222 68

We describe a new familial syndrome in three siblings; it is biochemically characterized by a combined defect of the action of the three related peptides insulin, insulin-like growth factor I (IGF I) and epidermal growth factor (EGF). Clinically, the disease has features of Werner syndrome with lipodystrophy, scleroderma-like alterations of the skin, alterations of the skeleton and contractures of joints. In addition, one of the patients has an insulin-resistant diabetes mellitus. Studies with cultured fibroblasts obtained from skin biopsies show a markedly reduced stimulation of RNA synthesis by the three growth factors and a decreased insulin stimulation of 2-deoxy-D-glucose uptake as compared with normal controls. Receptor binding of the three peptides occurred with normal capacity and affinity. We conclude that the signal transfer of different growth factors has a common denominator at the postreceptor level.
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PMID:A new familial syndrome with impaired function of three related peptide growth factors. 247 23

The course of scleroderma is compared in patients with and without diabetes mellitus. In combined pathology, bullous foci of injury and trophic abnormalities occur more frequently. All the patients were diagnosed to have abnormalities on the part of connective tissue metabolism.
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PMID:[The different clinical variants of scleroderma in diabetics]. 260 76

To establish the character of microvascular changes in psoriatic skin and their specificity, 29 skin biopsies of psoriatics (20 in exacerbation and 9 in a stationary stage) were investigated using histologic, histochemical, immunomorphologic, electron-microscopic, and morphometric methods. Five biopsies of uninvolved skin in scleroderma and five of diabetes mellitus patients were studied with the same technique for comparison. The results showed that structural changes depend on disease stage and the clinical appearance of lesions. Microvascular changes precede papule appearance during exacerbation and gradually increase during papule development. They comprise vascular dilatation, bridged fenestrations and gaps in endothelium, edematous areas in the cytoplasm of endotheliocytes, myocytes and pericytes, basement-membrane-zone thickening, and cell extravasation--signs of increased vascular permeability. Immunoglobulin G deposits in vascular walls, degranulation of mast cells, and extravasation of lymphocytes and neutrophils indicate that inflammation is a basic process during exacerbation and that immune mechanisms play an important role in the pathogenesis of inflammation. Microvascular changes in scleroderma and diabetes mellitus are different in nature and do not resemble those in psoriasis.
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PMID:Morphology of skin microvasculature in psoriasis. 264 67

The cutaneous microvasculature is organized into upper and lower horizontal plexuses with the dermal capillary loops arising from the upper plexus. The arteriolar and venular sides of the microvasculature can be identified by the ultrastructure of the mural basement membrane material. Collecting venules present in the lower dermis contain valves. Periadventitial cells (veil cells) are present around all microvessels. Their size and number appear to correlate with the quantity of mural basement membrane material found in cutaneous vessels in diabetes, actinic damage, and chronological aging. The contractile cells of the vascular wall surround the endothelial cell tube in a manner suggesting specific functions. The smooth muscle cells in the arteriolar segment form a sleeve, whereas each pericyte in the postcapillary venular simultaneously makes many contacts with several underlying endothelial cells. The common telangiectases can be explained by abnormalities in this organization and ultrastructure rather than by neovascularization or random anastomoses. The macular telangiectases seen in scleroderma, generalized essential telangiectasia, and nevus flammeus are produced by dilatation of the postcapillary venules of the upper horizontal plexus. Cherry angiomas are produced by spherical and tubular dilatations of capillary loops in dermal papillae with tortuous cross-connections between individual loops. Angiokeratomas of Fabry and Fordyce have the ultrastructure of collecting venules that contain valves, and appear to represent the ectopic development or placement of small valve-containing collecting veins. The cutaneous lesions of hereditary hemorrhagic telangiectasia represent arteriovenous communications.
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PMID:Ultrastructure and organization of the cutaneous microvasculature in normal and pathologic states. 266 19

Five patients with childhood scleroderma, were studied from a total group of 50 cases with the disease, 39 of them with diffuse systemic sclerosis and 11 with the CREST syndrome. The average age for these five patients when the disease onset was 13 (the age ranged from 5.5 to 16 years) with an average follow-up of 3.6 years (ranging from 1 to 6.5 years). Of the five, four girls were classified as having diffuse systemic sclerosis and the remaining boy, as suffering from the CREST syndrome. We found no family history or personal and occupational antecendents related with the appearance of the illness. Also excluded were conditions associated with changes similar to scleroderma as are seen in cases of diabetes mellitus, phenylketonuria, toxic oil syndrome, or graft-host rejection reactions. The clinical manifestations seen at the start of the disease included the Raynaud phenomenon, subcutaneous edema and muscular-skeletal abnormalities as arthralgia and myalgia with objective data of inflammatory myopathy. Proximal scleroderma was seen in all five patients; three of them, in addition, developed rapidly progressive cutaneous changes, causing the loss of elasticity and cutaneous hardening of the face during the first year of the disease. In all of the cases, the skin biopsy showed histopathological changes compatible with the diagnosis already given. The most important changes seen in the organs of these children were oesophageal dysfunction and fibrosis of the lung. The X-rays of three of the patients showed them to suffer from intestinal malfunction. We found no kidney, liver or nervous system disorders.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Generalized sclerosis (scleroderma) in children]. 269 12


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