11/814. A case of acromegaly accompanied by adrenal preclinical Cushing's syndrome. We encountered a 58-year-old woman with acromegaly accompanied by a cortisol-secreting adrenal tumor without clinical features of hypercortisolism. The simultaneous occurrence of these two endocrinopathies in one individual is extremely rare. She was diagnosed as having diabetes mellitus 8 years ago. Afterwards, in spite of insulin therapy, her hyperglycemia could not be well controlled. Her acromegaly and preclinical Cushing's syndrome were histopathologically proven to be due to a pituitary adenoma and an adrenocortical adenoma, respectively. Successful treatment for these endocrinopathies resulted in greatly improved blood sugar control because of a reduction in insulin resistance. In this case of preclinical Cushing's syndrome, replacement therapy with glucocorticoid was able to be discontinued at only 8 weeks after adrenalectomy, so that the period of necessary replacement was much shorter than that for overt Cushing's syndrome. This is the first report describing insulin resistance before and after treatment in a case of acromegaly accompanied by adrenal preclinical Cushing's syndrome. ( info) |
| We report a rare case of acromegaly in which pituitary infarction possibly developed in a GH-producing pituitary adenoma following gastrointestinal bleeding from peptic ulcer. In this case, pituitary infarction resulted in spontaneous remission of acromegaly associated with diabetes mellitus. In addition, detailed histological investigation revealed that clinically silent pituitary apoplexy was mainly an acute ischemic event which occurred recently in a GH-producing adenoma. This event led to massive coagulation necrosis of the tumor and endocrinological improvement. ( info) |
13/814. Nocardial infection in a renal transplant recipient on tacrolimus and mycophenolate mofetil. Infection with nocardia spp. is an uncommon but important cause of morbidity and mortality in organ transplant recipients. Cotrimoxazole prophylaxis against urinary tract infection and pneumocystis carinii pneumonia in these patients usually prevents nocardial infection also. We report the case of a patient on tacrolimus and mycophenolate mofetil who developed drug-induced diabetes mellitus followed by nocardial brain infection. This infection occurred despite conventional cotrimoxazole prophylaxis. physicians should be aware that newer, more potent and more diabetogenic immunosuppressive regimens may increase the risk of opportunistic infections such as nocardiosis, even in the presence of "adequate" antimicrobial preventive measures. ( info) |
14/814. Persistent hemiballism with striatal hyperintensity on T1-weighted MRI in a diabetic patient: a 6-year follow-up study. The combination of hemiballism, hyperglycemia and hyperintensity of the striatum on T1-weighted MRI constitutes a unique syndrome. We report the follow-up of a patient with this disorder whose hemiballism was sustained for over 5 years. High density on CT of the right striatum turned into normodensity in 4 months, and hyperintensity on T1-weighted MRI and hypointensity on T2-weighted MRI of the lesion were resolved in 18 months. A decreased perfusion of the lesion by SPECT remained 37 months after onset. There was no volume change of the lesion during the course of the illness. The radiological features support the possible pathology of either or both petechial hemorrhage and astrocytosis with high protein concentration after ischemic insult. The hemiballism may result from selective damage of GABA/enkephalin-containing neurons in the striatum and can persist without the primary histological changes causing the striatal T1-hyperintensity in this disorder. ( info) |
15/814. Patient case studies. Three contrasting cases of obese patients with type 2 diabetes mellitus are presented, which illustrate the management difficulties faced by clinicians. The first raises the issue of when to commence an oral hypoglycaemic agent in a newly diagnosed but asymptomatic obese patient; the second case addresses the problem of when to commence insulin in the face of continuing weight gain and poor glycaemic control; the final case is an example of the vicious metabolic spiral which so many patients enter, with increasing body weight, poor diabetic control and associated co-morbidities. The discussion that follows each case presentation recognises the considerable cardiovascular risk faced by such patients and provides guidance about possible management pathways including adjunctive anti-obesity pharmacotherapy. ( info) |
16/814. Renal complications in patients with diabetes mellitus associated with an A to G mutation of mitochondrial dna at the 3243 position of leucine tRNA. The substitution of guanine for adenine at position 3243 of the leucine tRNA gene of mitochondrial dna was originally described in association with MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes). diabetes mellitus associated with the mutation (mitochondrial diabetes) is a different phenotype from MELAS. We identified 11 patients with the mutation among 385 Japanese diabetic patients: two had MELAS and nine had mitochondrial diabetes. We present data on a male patient with mitochondrial diabetes who developed the nephrotic syndrome at the age of 23. light microscopy revealed mesangial expansion, PAS-positive deposits and segmental sclerosis in the glomeruli. Scattered mesangial electron-dense deposits and thickening of the basement membrane were found on electron microscopy, suggesting that diabetic glomerulosclerosis accompanied by focal glomerulosclerosis (FGS). Mitochondrial diabetes may pre-dispose patients to renal complications, including forms of glomerulonephritis, such as FGS. ( info) |
17/814. Acute renal failure in acquired haemophilia following the use of high dose intravenous immunoglobulin. High dose intravenous immunoglobulin (IVIG) is a useful form of treatment in patients with acquired haemophilia, a rare bleeding disorder due to auto-antibodies to factor viii. We describe a patient with acquired haemophilia who developed acute renal failure rapidly after treatment with a high sucrose content IVIG preparation. We speculate that the sucrose content of the preparation was responsible for the renal failure and make recommendations on the use of IVIG in patients with acquired haemophilia. ( info) |
18/814. A gene for congenital generalized lipodystrophy maps to human chromosome 9q34. Congenital generalized lipodystrophy (CGL, Berardinelli-Seip Syndrome, OMIM # 269700) is a rare autosomal recessive disorder characterized by near complete absence of adipose tissue from birth. Affected individuals have marked insulin resistance, hypertriglyceridemia and acanthosis nigricans, and develop diabetes mellitus during teenage years. The genetic defect for CGL is unknown. A semi-automated genome-wide scan with a set of highly polymorphic short tandem repeats (STR) was carried out in 17 well-characterized pedigrees and identified a locus for CGL to chromosome 9q34. The maximum two-point lod score obtained was 3.6 at D9S1818 (theta(max) = 0.05). There was evidence for genetic heterogeneity (alpha = 0.73) and 2 of the pedigrees were unlinked. Multipoint linkage analysis excluding the 2 unlinked families yielded a peak lod score of 5.4 between loci D9S1818 and D9S1826. The CGL1 critical region harbors a plausible candidate gene encoding the retinoid x receptor alpha (RXRA) that plays a central role in adipocyte differentiation. Identification of the CGL gene(s) will contribute to our understanding of the adipocyte differentiation and elucidation of the mechanisms of insulin resistance in disorders of adipose tissue. ( info) |
19/814. Bone amyloidoma in a diabetic patient with morbid obesity. Bone localisations of amyloidosis are rare, usually diffuse and associated with myeloma. We report the case of a patient with massive obesity complicated by diabetes, hypertension, sleep apnea and liver steatosis, who complained of rapidly worsening bilateral polyradiculalgia of the lower limbs. After sufficient weight loss made nuclear magnetic resonance imaging feasible, a spinal tumour was visualised on the 5th lumbar vertebra, extending to soft tissues. Total excision was performed, and pathological studies revealed an amyloid bone tumour with no evidence of myeloma. ( info) |
20/814. Neonatal diabetes mellitus with hypergalactosemia. We report the case of a male, small-for-gestational-age newborn who presented with failure to thrive, severe fluctuation of blood glucose concentrations, and increased serum concentrations of galactose. The infant responded well to a lactose-free diet supplemented with fructose, inulin and corn starch. The metabolic disorder disappeared within 6 months. The transient course, and results of a molecular analysis of the glucose transporter 2 (Glut2) gene seem to rule out Fanconi-Bickel syndrome. ( info) |