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

Long-term outcome was studied in 233 patients who had undergone renal artery revascularization (51 with balloon angioplasty, 182 with surgery) between 1976 and 1992. Patients (excluding renal transplants) were treated for renal vascular hypertension without or with renal insufficiency (serum creatinine > 1.6 mg/dl. All patients still alive (n = 188) were contacted to determine current blood pressure, medications, serum creatinine, and subsequent significant medical events. In patients who had died the cause of death was determined and renal function status at the time of death noted from medical records. Some follow-up information was obtained on all 233 patients; follow-up serum creatinine data were obtained in 193 (82.8%) patients. Some 24 patients (10.3%) became dialysis-dependent. Using a multiple logistic regression analysis only, preoperative creatinine maintained significance (P < 0.001) for increased dialysis risk. There was no statistically significant association of dialysis for type of revascularization (percutaneous transluminal angioplasty, autogenous artery, saphenous vein, endarterectomy or synthetic material), simultaneous or previous aortic or other vascular surgery (carotid endarterectomy, femoropopliteal bypass, etc.), pathology (atherosclerosis or fibromuscular dysplasia), number of renal arteries stenosed or treated, length of follow-up, age, coronary artery disease, congestive heart failure, stroke, chronic lung disease or type II diabetes. It is concluded that, in patients with renal artery stenosis, the timing of renal artery revascularization relative to the level of renal function is the most important determinant for long-term renal salvage.
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PMID:Late renal function in patients undergoing renal revascularization for control of hypertension and/or renal preservation. 890 17

Femoral hypoplasia/unusual facies syndrome (FH/UFS) is a rarely considered clinical entity that has a strong association with maternal diabetes. A case that demonstrates the prenatal morphologic and biometric abnormalities, and postnatal findings are presented. The pathogenesis and natural history of FH/UFS is discussed and contrasted with the caudal dysplasia syndrome.
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PMID:Femoral hypoplasia and maternal diabetes: consider femoral hypoplasia/unusual facies syndrome. 896 Jun 13

Cebocephaly and sirenomelia are uncommon birth defects. Their association is extremely rare; however, the presence of spina bifida with both conditions is not unexpected. We report on a female still-birth with cebocephaly, alobar holoprosencephaly, cleft palate, lumbar spina bifida, sirenomelia, a single umbilical artery, and a 46,XX karyotype, but without maternal diabetes mellitus. Our case adds to the examples of overlapping cephalic and caudal defects, possibly related to vulnerability of the midline developmental field or axial mesodermal dysplasia spectrum.
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PMID:Cebocephaly, alobar holoprosencephaly, spina bifida, and sirenomelia in a stillbirth. 954 Nov 20

Wolcott-Rallison syndrome is a rare autosomal recessive condition characterized by diabetes mellitus arising in early infancy and multiple epiphyseal dysplasia. To date, nine cases have been described in the world literature. We report an affected girl who died at the age of 4 years and on whom a full autopsy was performed. In addition to neonatal diabetes mellitus and epiphyseal dysplasia, this child had mental retardation and recurrent episodes of self-limiting hepatic failure. Autopsy revealed severe pancreatic hypoplasia and markedly abnormal pancreatic histology, while histology of the bone was consistent with epiphyseal dysplasia. There was laryngeal stenosis and pulmonary hypoplasia. The heart was enlarged with mitral value dysplasia and stenosis, left atrial dilatation, left ventricular hypertrophy, and endocardial fibroelastosis. Examination of the central nervous system showed arrhinencephaly and cerebellar cortical dysplasia. The liver showed minor histological abnormalities but no features were present to account for the recurrent hepatic failure. In addition to Wolcott-Rallison syndrome this child had a deletion at 15q11-12 in 65% of her cells.
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PMID:Autopsy findings in the Wolcott-Rallison syndrome. 918 26

We report the case of a 28-year-old woman attending for hirsutism and diabetes mellitus. Diabetes was a casual finding 2 years before consulting and was treated with diet and antidiabetic drugs. Acromegalic appearance, facial acne, penty, curled and rude hair, hypertrichosis, ade I diffuse goitre, prominent abdomen with umbilical hernia, severe hepatomegaly, prominent muscles and veins with normal genitalia appeared in the physical examination. No other abnormalities were found. Hypophysis, thyroid, suprarenal and ovaric hormonal functional studies were normal. An insulin-resistant diabetes mellitus was found in the metabolic study. Ultrasound and TAC showed severe diffuse hepatomegaly and visceral fat lack. Bone radiographies showed diffuse lesions compatible with polyostotic dysplasia. Subcutaneous, hepatic and bone biopsy revealed lack of fat tissue, hepatic steatosis and osteal fibrosis. Patient s diagnosis was Berardinelli-Seip syndrome, Seip-Lawrence or lipoatrophic diabetes associated with polyostotic fibrotic dysplasia. Case is studies and bibliographic references are reviewed.
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PMID:[Seip-Lawrence syndrome associated with polyostotic fibrous dysplasia. Report of a case]. 923 83

The aim of this report is to describe four cases of non-autoimmune diabetes that presented in infancy. Three had transient neonatal diabetes mellitus (TNDM) with diabetic ketoacidosis at onset, followed by complete remission after several months of insulin treatment. While the fourth case was initially diagnosed as TNDM, she had renal, hepatic and pancreatic dysplasia. These cases illustrate that diabetes in infants can be difficult to diagnose and that patients with TNDM can have a recurrence of diabetes several years later.
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PMID:Different faces of non-autoimmune diabetes of infancy. 951 Apr 56

Cobalamin (vitamin B12) deficiency is more common in the elderly than in younger patients. This is because of the increased prevalence of cobalamin malabsorption in this age group, which is mainly caused by (autoimmune) atrophic body gastritis. Cobalamin supplementation is affordable and nontoxic, and it may prevent irreversible neurological damage if started early. Elderly individuals with cobalamin deficiency may present with neuropsychiatric or metabolic deficiencies, without frank macrocytic anaemia. An investigation of symptoms and/or signs includes the diagnosis of deficiency as well as any underlying cause. Deficiency states can still exist even when serum cobalamin levels are higher than the traditional lower reference limit. Cobalamin-responsive elevations of serum methylmalonic acid (MMA) and homocysteine are helpful laboratory tools for the diagnosis. The health-related reference ranges for homocysteine and MMA appear to vary with age and gender. Atrophic body gastritis is indirectly diagnosed by measuring serum levels of gastrin and pepsinogens, and it may cause dietary cobalamin malabsorption despite a normal traditional Schilling's test. The use of gastroscopy may also be considered to diagnose dysplasia, bacterial overgrowth and intestinal villous atrophy in healthy patients with atrophic body gastritis or concomitant iron or folic acid deficiency. Elderly patients respond to cobalamin treatment as fully as younger patients, with complete haematological recovery and complete or good partial resolution of neurological deficits. Chronic dementia responds poorly but should, nevertheless, be treated if there is a metabolic deficiency (as indicated by elevated homocysteine and/or MMA levels). Patients who are at risk from cobalamin deficiency include those with a gastrointestinal predisposition (e.g. atrophic body gastritis or previous partial gastrectomy), autoimmune disorders [type 1 (insulin-dependent) diabetes mellitus and thyroid disorders], those receiving long term therapy with gastric acid inhibitors or biguanides, and those undergoing nitrous oxide anaesthesia. To date, inadequate cobalamin intake has not proven to be a major risk factor. Intervention trials of cobalamin, folic acid and pyridoxine (vitamin B6) in unselected elderly populations are currently under way.
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PMID:Age-related changes in cobalamin (vitamin B12) handling. Implications for therapy. 957 92

Neonatal diabetes mellitus is a rare condition, the causes of which are mostly unknown. One well defined though very rare entity is the autosomal recessive Wolcott-Rallison syndrome, in which permanent neonatal diabetes, osteopenia, and epiphyseal dysplasia occur. Only five previous families have been reported, and here we describe the second in which parental consanguinity was present. The proband was born to first cousin parents and died at 2 years from the sequelae of poorly controlled diabetes. To test the hypothesis that mutation of PAX4, required in the mouse for pancreatic islet beta cell development, might cause WRS, the structure of the human PAX4 gene was deduced and DNA from two unrelated WRS patients sequenced. No PAX4 mutation was present, though the entire coding region was sequenced in both patients. It therefore appears unlikely that PAX4 is involved in the aetiology of Wolcott-Rallison syndrome, though it remains a good candidate for other forms of neonatal diabetes mellitus.
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PMID:Organisation of the human PAX4 gene and its exclusion as a candidate for the Wolcott-Rallison syndrome. 959 21

Menorrhagia--menstrual periods lasting longer than 7 days and totaling blood losses greater than 80mL--affects 9%-14% of otherwise healthy women, and it can signal cancer, an endocrinologic disorder, or gynecologic disease. Blood loss can be high enough to result in anemia, fatigue, and syncope. Most often, abnormal uterine bleeding such as menorrhagia involves a disruption in the hypothalamic-pituitary axis, the ovary, and/or the uterus. Other identified causes include medications (especially psychotropics) that cross the blood-brain barrier; chronic diseases such as cancer, diabetes, and liver and kidney dysfunction; endocrine disorders, perimenopausal anovulation, polycystic ovary disease, pituitary tumors, and abnormal estrogen cycling caused by morbid obesity; and anatomic abnormalities of the uterus. Routine tests include hematocrit or hemoglobin to detect and evaluate anemia, thyroid stimulating hormone (TSH) level to evaluate thyroid function as a possible cause, and a pregnancy test to rule out an incomplete, spontaneous abortion as a cause. A Pap test is recommended to screen for dysplasia that can suggest a gynecologic cancer cause. Additional screening for endocrine disorders that may be causing menorrhagia include tests of thyroid, liver, and kidney function, and tests of follicle stimulating hormone (FSH), prolactin, and cortisol levels. Treatment can be medical or surgical. Medical treatment includes prostaglandin inhibitors, specifically nonsteroidal antiinflammatory drugs (NSAIDs), and hormonal therapy with estrogen, progesterone, gonadotropin-releasing hormone agonists, or oral contraceptives such as medroxyprogesterone (Depo-Provera). Surgical treatment includes hysteroscopic endometrial ablation by physical agents, laser electrodiathermy, and "roller ball," or surgical, resection. Hysterectomy is the treatment of last resort.
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PMID:Treatment Decisions in the Management of Menorrhagia. 974 72

Constipation is a frequent complaint among patients with different neurological diseases. This review provides a brief account of the numerous conditions affecting the central, peripheral and intrinsic (enteric) nervous systems in which constipation can be the only clinical manifestation or a component of a complex syndrome. Recent neuropathophysiological acquisitions show that any structural or functional impairment of the intrinsic innervation of the gut, including both developmental (i.e., Hirschsprung's disease and intestinal neuronal dysplasia) and acquired (i.e., either degenerative or inflammatory neuropathies) disorders, can be associated with constipation. Constipation may also arise from derangements of the peripheral nervous system, including diabetes and primary chronic autonomic failure (pandysautonomias). Finally, in the central nervous system, a wide array of disorders (post-traumatic, degenerative, ischaemic or neoplastic) are recognized to determine bowel dysfunction, ultimately leading to constipation. Further understanding of the fine pathophysiological mechanisms through which the intrinsic and extrinsic nerve supplies to the digestive system are involved in idiopathic constipation or in diseases generating this symptom will hopefully lead to a better treatment of this frequent pathological condition.
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PMID:Constipation: a common problem in patients with neurological abnormalities. 983 15


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