1/82. Non-insulin-dependent diabetes mellitus with type I multiple symmetrical lipomatosis: a case report.Type I multiple symmetrical lipomatosis (MSL; Madelung's disease) is characterized by lipomas in the nape of the neck and the supraclavicular and deltoid regions, resulting in a bull-necked appearance (Madelung's collar). It is most common in alcoholic men between 35 and 50 years of age. Type I MSL has been reportedly associated with hyperinsulinemia, but its association with diabetes mellitus is rarely discussed. We describe a case of non-insulin-dependent diabetes mellitus (NIDDM) associated with type I MSL. A 47-year-old alcoholic man presented with a seven-year history of hyperglycemia and progressive neck swelling with dysphagia for one year. physical examination showed diffuse and symmetrical swelling of the bilateral posterior aspects of the neck. biochemistry profiles revealed elevated concentrations of fasting serum glucose (276 /- 16 mg/dl), triglycerides (358 /- 79 mg/dl) and total cholesterol (323 /- 28 mg/dl). Endocrinologic studies showed normal thyroid function. neck sonography revealed diffuse thickening and swelling of the fatty structures of both sides of the neck. Normal sonography showed no fatty deposition in the liver. Maxillary and neck computerized tomography revealed diffuse fat accumulation in the submental and posterior neck regions, with no extension to the superior mediastinum. Fine needle aspiration cytology of the neck masses showed only fat cells. The patient received an oral hypoglycemic agent (glibenclamide 5 mg bid) for blood glucose control and lovastatin (20 mg before bed-time) for hyperlipidemia, and ceased drinking alcohol. The neck swelling resolved markedly after 15 months of medical treatment. This suggests that, in addition to the cessation of alcohol consumption, the reduction of blood glucose and lipid concentrations by medication may also assist in resolving the accumulated fat of type I MSL in patients with NIDDM.- - - - - - - - - - ranking = 1keywords = fat (Clic here for more details about this article) |
2/82. Triglyceride-induced diabetes associated with familial lipoprotein lipase deficiency.Raised plasma triglycerides (TGs) and nonesterified fatty acid (NEFA) concentrations are thought to play a role in the pathogenesis of insulin-resistant diabetes. We report on two sisters with extreme hypertriglyceridemia and overt diabetes, in whom surgical normalization of TGs cured the diabetes. In all of the family members (parents, two affected sisters, ages 18 and 15 years, and an 11-year-old unaffected sister), we measured oral glucose tolerance, insulin sensitivity (by the euglycemic-hyperinsulinemic clamp technique), substrate oxidation (indirect calorimetry), endogenous glucose production (by the [6,6-2H2]glucose technique), and postheparin plasma lipoprotein lipase (LPL) activity. In addition, GC-clamped polymerase chain reaction-amplified dna from the promoter region and the 10 coding LPL gene exons were screened for nucleotide substitution. Two silent mutations were found in the father's exon 4 (Glu118 Glu) and in the mother's exon 8 (Thr361 Thr), while a nonsense mutation (Ser447 Ter) was detected in the mother's exon 9. Mutations in exons 4 and 8 were inherited by the two affected girls. At 1-2 years after the appearance of hyperchylomicronemia, both sisters developed hyperglycemia with severe insulin resistance. Because medical therapy (including high-dose insulin) failed to reduce plasma TGs or control glycemia, lipid malabsorption was surgically induced by a modified biliopancreatic diversion. Within 3 weeks of surgery, plasma TGs and NEFA and cholesterol levels were drastically lowered. Concurrently, fasting plasma glucose levels fell from 17 to 5 mmol/l (with no therapy), while insulin-stimulated glucose uptake, oxidation, and storage were all markedly improved. Throughout the observation period, plasma TG levels were closely correlated with both plasma glucose and insulin concentrations, as measured during the oral glucose tolerance test. These cases provide evidence that insulin-resistant diabetes can be caused by extremely high levels of TGs.- - - - - - - - - - ranking = 0.4keywords = fat (Clic here for more details about this article) |
3/82. Nontraumatic clostridial myonecrosis.We describe three cases of nontraumatic clostridial myonecrosis seen at the Victorian Institute of forensic medicine. Nontraumatic clostridial myonecrosis is an uncommon and often fatal condition that requires immediate institution of appropriate medical and surgical therapy. It is most commonly caused by clostridium perfringens and clostridium septicum and is associated with gastrointestinal and hematologic malignancies, diabetes mellitus, and peripheral vascular disease. The clinical features include a rapidly evolving acute illness with severe pain, marked tachycardia, and brawny discoloration of the skin with bullae formation and crepitus, followed by hypotension and acute renal failure. Features at autopsy include reddish brown skin discoloration with bullae formation and necrotic skeletal muscle. Radiographs may be of use prior to the postmortem in detecting gas within the soft tissues. Gram stain and microbiologic culture are important in establishing a definitive diagnosis; although the major factors in suggesting the diagnosis are the recognition of the typical clinical history and macroscopic autopsy findings.- - - - - - - - - - ranking = 0.2keywords = fat (Clic here for more details about this article) |
4/82. Necrotizing fasciitis after cosmetic blepharoplasty.PURPOSE: To report a case of necrotizing fasciitis after cosmetic blepharoplasty. METHOD: Case report. A 74-year-old woman with history of type II diabetes mellitus underwent bilateral upper eyelid blepharoplasty. Postoperatively she developed fever, grayish discoloration of the skin, violaceous bullae, and a right facial nerve palsy. Necrotizing fasciitis was diagnosed and treated with intravenous antibiotics, debridement of necrotic tissue, and hyperbaric oxygen therapy. RESULTS: The infection resolved, but the patient required reconstruction for correction of cicatricial ectropion. CONCLUSION: Necrotizing fasciitis is a potentially fatal infection that typically occurs in the setting of trauma. Early recognition of its pathognomonic signs and aggressive management are paramount.- - - - - - - - - - ranking = 0.2keywords = fat (Clic here for more details about this article) |
5/82. Accelerated conversion of dehydroepiandrosterone sulfate to estrogen in a patient with Crow-Fukase syndrome and diabetes mellitus.About 28% of patients with the Crow-Fukase syndrome exhibit glucose intolerance which may be induced by low serum levels of dehydroepiandrosterone (DHEA). We report a patient with the Crow-Fukase syndrome who exhibited non-insulin dependent diabetes mellitus (NIDDM) worsened prior to admission. He received the DHEA sulfate (DHEA-S) infusion test to evaluate aromatase activity. This patient exhibited an increase in aromatase activity measured by the conversion of the intravenously loaded DHEA-S to estrogen, and low serum levels of DHEA and DHEA-S. These abnormalities returned to nearly normal during the administration of prednisolone, 60 mg per day. No adverse effect on his diabetes was observed during the corticosteroid treatment. Five control patients with diabetes but without the Crow-Fukase syndrome showed no increase in the conversion of DHEA-S to estrogen, which suggests that aromatase activity is normal in diabetes. The increase in aromatase activity in our patient may have led to a low serum concentration of DHEA that in turn caused glucose intolerance and a deterioration of the diabetes prior to admission. Glucocorticoid therapy may be beneficial in Crow-Fukase syndrome to improve the distorted metabolism of DHEA with no adverse effect on the diabetes.- - - - - - - - - - ranking = 1keywords = fat (Clic here for more details about this article) |
6/82. Subacute hepatic failure associated with a new antidiabetic agent, troglitazone: a case report with autopsy examination.An autopsy case of fatal subacute hepatic failure after administration of troglitazone is described. The liver dysfunction developed about five months after the patient, a sixty-three-year-old woman, had been initially treated with troglitazone. The patient developed hepatic failure and died despite various hepatic auxiliary treatments such as plasmapheresis. autopsy findings revealed focal liver cell necrosis, cholestasis and steatosis with infiltration of lymphocytes and neutrophils and lack of regenerative activity. The causative mechanism of liver dysfunction may be metabolite aberration, as a result of accumulation of hepatotoxic metabolite(s), in a category of idiosyncratic liver injury. It is proposed to monitor liver function strictly and periodically for the diabetic patients prescribed troglitazone.- - - - - - - - - - ranking = 0.2keywords = fat (Clic here for more details about this article) |
7/82. Cannula related suppurative thrombophlebitis in the burned patient.Suppurative thrombophlebitis is a well recognised and potentially fatal complication of intravenous cannulation in burns patients. We report a case of an Afro-Caribbean patient with noninsulin-dependent diabetes who developed signs of systemic sepsis two weeks after a 14% total body surface area flame burn. Despite an initial paucity of clinical signs at the cannulation site, exploratory venotomy revealed frank suppuration within the long saphenous vein from the ankle to the groin. This was treated successfully by total excision of the vein and its tributaries and delayed wound closure. Following this, a retrospective analysis of the measured clinical parameters and blood tests revealed no obvious, missed pointers to the impending sepsis other than a dramatic increase in the overall daily insulin requirement. This had doubled over a 48-h period, preceding the clinical diagnosis by three days. The relevant literature and guidelines for management are reviewed.- - - - - - - - - - ranking = 0.2keywords = fat (Clic here for more details about this article) |
8/82. Acute lymphoblastic leukemia in one of two siblings with alstrom syndrome.alstrom syndrome is a rare autosomal recessive disease; less than 60 cases have been reported. No Chinese patient with this disease has been reported previously in the literature. Here, we describe an 11-year-old Chinese boy with this condition. His elder sister also had alstrom syndrome, and his father had non-insulin-dependent diabetes mellitus. Both siblings had degenerative retinopathy, obesity, mental retardation, perceptive hearing loss, short stature, non-insulin-dependent diabetes mellitus, nephropathy, hyperlipidemia, acanthosis nigricans, and hepatic dysfunction. The boy also developed acute lymphoblastic leukemia, which was confirmed by cytochemistry and immunophenotyping findings. He received chemotherapy and radiotherapy for the malignancy. The present case suggests that acute lymphoblastic leukemia may be coincident with or may be a previously undescribed systemic manifestation of alstrom syndrome.- - - - - - - - - - ranking = 0.2keywords = fat (Clic here for more details about this article) |
9/82. Fatal emphysematous pyelonephritis with gas in the spinal extradural space in a patient with diabetes.BACKGROUND: Emphysematous pyelonephritis (EP) is a rare but life-threatening condition of the upper urinary tract, characterized by the presence of gas in the renal parenchyma and perirenal space. The vast majority of patients with EP (90%) are known to have diabetes, with escherichia coli being the most common causative pathogen. CASE REPORT: We present a case of fatal bilateral EP in a patient with diabetes, with an unusual radiological finding of gas around the spinal cord and in the psoas muscle, with renal parenchymal sparing. Our case serves as an important reminder of this life threatening entity in diabetic patients, which is not well recognized by clinicians because of its rarity.- - - - - - - - - - ranking = 0.2keywords = fat (Clic here for more details about this article) |
10/82. Hepatocellular carcinoma arising in non-alcoholic steatohepatitis.The incidence and significance of hepatocellular carcinoma (HCC) in non-alcoholic steatohepatitis (NASH) has not been previously evaluated in detail. We recently experienced a case of NASH with multicentric HCC in a female patient. At the age of 58 years, the patient was diagnosed with non-insulin-dependent diabetes mellitus, treated by insulin therapy. The patient did not drink alcohol. She was negative for all serological markers of hepatitis b and C virus infection. Because of liver dysfunction, a needle biopsy was performed at the age of 62 years, and pathological findings, such as fatty change, Mallory's body, nuclear glycogen and pericellular fibrosis, suggested a diagnosis of NASH. Subsequently, four nodules were detected in the liver by imaging. liver biopsies were performed from each nodule. One nodule was pathologically diagnosed as a pseudolymphoma, while three other nodules were moderately differentiated HCC (10 years after the diagnosis of non-alcoholic steatohepatitis), well-differentiated HCC (11 years later) and dysplastic nodule (11 years later), suggesting multicentric occurrence of HCC. This case suggests that HCC could be a late complication of NASH.- - - - - - - - - - ranking = 0.2keywords = fat (Clic here for more details about this article) |
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