1/14. Hepatic glycogenosis: reversible hepatomegaly in type 1 diabetes.OBJECTIVE: To describe the aetiology, clinical features and appropriate treatment for hepatic glycogenosis in poorly controlled type 1 diabetes. methods: A review of three adolescents with poor diabetes control, hepatomegaly and elevated serum liver transaminase concentrations. RESULTS: Symptoms included abdominal pain, anorexia, nausea and vomiting. All had tender hepatomegaly; two had splenomegaly. Liver biopsy was performed on two patients. histology revealed hepatic glycogenosis in both; one also demonstrated macrovesicular steatosis. With improved glycaemic control, all three showed resolution of their symptoms, organomegaly and elevated serum liver transaminase concentrations. CONCLUSIONS: Insulin-reversible hepatic glycogenosis is the most common cause of hepatomegaly and raised serum liver transaminase concentrations in children and adolescents with type 1 diabetes. Having excluded other causes of hepatic dysfunction, a 4 week therapeutic trial of improved glycaemic control is recommended prior to more invasive investigations.- - - - - - - - - - ranking = 1keywords = diabetes (Clic here for more details about this article) |
2/14. septo-optic dysplasia with congenital hepatic fibrosis.This article reports a 7-year-old female with septo-optic dysplasia and congenital hepatic fibrosis. She manifested nystagmus and severe hepatosplenomegaly. brain magnetic resonance imaging revealed agenesis of the septum pellucidum, optic nerve hypoplasia, pituitary gland stalk hypoplasia, and absence of the posterior pituitary gland. She was diagnosed with growth hormone deficiency, hypothyroidism, diabetes insipidus, and adrenal insufficiency. Thus, this case was regarded as septo-optic dysplasia. No mutation was evident in the coding and boundary regions of the homeobox gene HESX1. Percutaneous biopsy of the liver demonstrated the presence of broad septa of fibrous tissue containing abundant bile ducts without inflammatory cell infiltrates, a finding compatible with congenital hepatic fibrosis. Although there is an association between septo-optic dysplasia and neonatal cholestasis, believed to be related to hypopituitarism, this case of septo-optic dysplasia with congenital hepatic fibrosis is apparently the first reported in the English literature.- - - - - - - - - - ranking = 0.14285714285714keywords = diabetes (Clic here for more details about this article) |
3/14. Intermittent and recurrent hepatomegaly due to glycogen storage in a patient with type 1 diabetes: genetic analysis of the liver glycogen phosphorylase gene (PYGL).We report a 19-year-old woman who had a history of type 1 diabetes with recurrent glycogen accumulation in the liver. During her infantile period she presented with no hepatomegaly nor growth retardation. On admission she was diagnosed with diabetic ketoacidosis (DKA). She also had hepatomegaly and elevated transaminase levels, but these abnormalities had resolved after administration of insulin. However, 4 weeks after DKA marked hepatomegaly and elevated transaminases were reappeared with simultaneous hypoglycemia which suggested an impaired glycogenolysis in the extraordinary conditions. We supposed the partial deficiency of liver glycogen phosphorylase activity in this patient and analyzed the liver glycogen phosphorylase gene (PYGL). Deduced amino acid sequence of the PYGL in this patient was completely identical to that reported by Burwinkel et al. (Y15233), however, the nucleotide sequence of PYGL cDNA was heterozygous for substitutions at positions Asp339 (GAT to GAC) on exon 9 and Ala703 (GCT to GCC on exon 17, respectively. These SNPs were also screened in 51 Japanese normal subjects by PCR-based direct sequencing or PCR-RFLP method. The same genotype observed in this patient was detected in 2 of 51(3.9%) normal subjects. These results suggest that the structure of PYGL coding sequence in this patient is unlikely to account for her excessive liver glycogen accumulation. Further studies including genetic analysis on the promoter region of the gene are necessary to clarify the etiology of susceptibility to excessive liver glycogen storage in patients with type 1 diabetes.- - - - - - - - - - ranking = 0.85714285714286keywords = diabetes (Clic here for more details about this article) |
4/14. Nonalcoholic steatohepatitis associated with psoriasis vulgaris.A 24-year-old man was admitted to our hospital because of liver dysfunction. He had been diagnosed as having psoriasis vulgaris at 18 years of age. physical examination demonstrated obesity, general erythema, and hepatomegaly. Laboratory data revealed elevated serum levels of aspartate aminotransferase, alanine aminotransferase, lactate dehydrogenase, and glucose. A histological examination of the liver revealed macrovesicular fatty change and infiltration of inflammatory cells, including lymphocytes and polymorphonuclear cells, within the liver lobules. Pericentral fibrosis and pericellular fibrosis were also recognized. He was diagnosed as having nonalcoholic steatohepatitis (NASH), based on the fact that he had no habit of drinking alcohol, as well as psoriasis vulgaris and diabetes mellitus. We herein report a very rare case of NASH associated with psoriasis vulgaris.- - - - - - - - - - ranking = 0.58490429400207keywords = diabetes mellitus, diabetes, mellitus (Clic here for more details about this article) |
5/14. Reversible hepatomegaly and diabetes mellitus in an adult with disseminated histiocytosis X.histiocytosis X rarely disseminates in an adult. The authors describe an unusual patients who presented with multiple areas of cutaneous and bone involvement. During the course of his disease he developed massive hepatomegaly. Aggregates of vacuolated histiocytes were found on liver biopsy. He subsequently developed diabetes mellitus complicated by ketoacidosis. Both his hepatomegaly and diabetes resolved spontaneously. No pancreatic nor pituitary abnormalities were identified. The combination of histiocytosis X, hepatomegaly, and diabetes mellitus has not been previously reported. The medical literature is reviewed with an emphasis on disseminated histiocytosis X in adults and the mechanism of glucose intolerance in liver disease.- - - - - - - - - - ranking = 3.6522829068696keywords = diabetes mellitus, diabetes, mellitus (Clic here for more details about this article) |
6/14. Elevated aminotransferases and alkaline phosphatases in unstable diabetes mellitus without ketoacidosis or hypoglycemia.Four patients with unstable diabetes mellitus and pronounced elevations of serum aminotransferases and alkaline phosphatases are reported. Thorough investigations revealed no cause for the abnormalities. The enzyme elevations were associated with hepatomegaly, and in some instances, abdominal pain and leg edema. Liver biopsies in all patients showed only marked accumulation of glycogen in the hepatocytes.- - - - - - - - - - ranking = 2.9245214700104keywords = diabetes mellitus, diabetes, mellitus (Clic here for more details about this article) |
7/14. The Mauriac syndrome.An insulin-dependent diabetic was diagnosed at the age of 7 years. After two years of satisfactory control she began to have several bouts of hospitalization with hyperglycaemic ketoacidosis, and developed tender hepatomegaly, which persisted to age 11 years. With restabilisation of her diabetes, the liver regressed and she continued to maintain good health for another 1 1/2 years when she died suddenly while asleep. Post-mortem examination by the coroner revealed ascites in the abdomen, hepatomegaly and fatty metamorphosis of the liver. Her diabetes control required up to 2.3 i.u. insulin per kg body weight per day plus a 1,900 calorie diet. Her growth was well below the tenth percentile, weight for height (Harvard charts). This clinical picture of high insulin dosage, hepatomegaly, unstable diabetes and growth failure approximates to the Mauriac syndrome.- - - - - - - - - - ranking = 0.52061833036403keywords = diabetes, insulin-dependent (Clic here for more details about this article) |
8/14. An unusual case of poems syndrome.A 58-year-old woman presented with a history of premature onset of menopause, longstanding hepatosplenomegaly, monoclonal gammopathy, lower limb polyneuropathy of recent onset, diabetes mellitus, excessive perspiration and leg edema. Polyneuropathy and excessive perspiration improved following a course of prednisone and melphalan. The clinical and pathophysiological features fit the rare entity known as poems syndrome.- - - - - - - - - - ranking = 0.58490429400207keywords = diabetes mellitus, diabetes, mellitus (Clic here for more details about this article) |
9/14. hepatomegaly due to self-induced hyperinsulinism.Repeated hypoglycaemic attacks, associated with transient hepatomegaly, in a 12-year-old insulin-dependent diabetic girl continued despite reduction in dose and, later, complete discontinuance of insulin. The attacks ceased while she was in hospital, necessitating reinstitution of insulin. The hepatomegaly resolved when surreptitious additional insulin injections were discovered and stopped. hepatomegaly in diabetics should arouse suspicion of overdosage with insulin.- - - - - - - - - - ranking = 0.092046901792597keywords = insulin-dependent (Clic here for more details about this article) |
10/14. Pericentral hepatic fibrosis and intracellular hyalin in diabetes mellitus.Five patients with maturity-onset diabetes mellitus and hepatomegaly were studied. This group in cluded moderately obese females in whom hepatomegaly and abnormal liver studies were associated with a marked elevation in the erythrocyte sedimentation rate. Liver biopsies from all 5 showed fatty steatosis and pericentral fibrosis. In 3 of the 5, fibrous bridging between central veins and portal tracts was present. collagen surrounded swollen hepatocytes, some of which contained intracellular hyalin indistinguishable from Mallory's hyalin. Polymorphonuclear neutrophils and regenerating nodules were not present. None of the patients had a history of alcohol use, and in 2 followed closely for 2 yr, alcohol was never detected in random blood samples. The recognition of this morphologic lesion may help to clarify the significance of this finding as an intermediate lesion between fatty steatosis and cirrhosis in diabetics.- - - - - - - - - - ranking = 2.9245214700104keywords = diabetes mellitus, diabetes, mellitus (Clic here for more details about this article) |
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