Good's Syndrome Accompanied by Agranulocytosis Following a Rapid Clinical Course.

Good's syndrome is an immunodeficiency disease involving thymoma accompanied by hypogammaglobulinemia. We encountered a case of Good's syndrome accompanied by agranulocytosis that followed a rapid clinical course. A 72-year-old man visited our hospital with a two-week history of a sore throat. Candida albicans was detected in the pharynx, and hypogammaglobulinemia was detected in addition to granulocytopenia. The patient subsequently developed septic shock and followed a rapid clinical course which ended in death. Good's syndrome with agranulocytosis was diagnosed at autopsy. Good's syndrome accompanied by agranulocytosis can follow a rapid clinical course and some cases remain asymptomatic until old age. Its prompt treatment is crucial.

The usefulness of a cholesterol absorption inhibitor in Japanese type 2 diabetes patients with dyslipidemia.

AIM: Cholesterol absorption has been suggested to be an independent risk factor for cerebral and cardiovascular events. We studied the clinical efficacy of ezetimibe in Japanese patients with type 2 diabetes mellitus complicated by dyslipidemia, in whom increased cholesterol absorption had been reported. SUBJECTS AND METHODS: Ninety-six patients with type 2 diabetes complicated by dyslipidemia received ezetimibe at 10 mg/day for 12 weeks. The lipid profile, a cholesterol synthesis marker (lathosterol), and cholesterol absorption markers (cholestanol, sitosterol, and campesterol) were measured before and after the therapy to evaluate the clinical efficacy of ezetimibe. RESULTS: Serum low-density lipoprotein-cholesterol (LDL-C) levels were positively associated with cholesterol absorption markers but not associated with a cholesterol synthesis marker, suggesting that serum LDL-C levels are more strongly related to cholesterol absorption than synthesis. During the 12-week ezetimibe treatment period, cholesterol absorption markers significantly decreased, and serum lipid profiles, including LDL-C levels, significantly improved. The LDL-C-lowering rate was greater in those patients who had been receiving statin therapy and were newly started on ezetimibe additionally than in the ezetimibe monotherapy group (-31.4% vs. -18.4%; P<0.001). CONCLUSIONS: It is suggested that ezetimibe improves the lipid profile in Japanese type 2 diabetes patients with dyslipidemia through the substantial reduction of cholesterol absorption.

[Panhypopituitarism caused by an intrasellar cystic mass in late-onset hypogonadism clinic].

A 74-year-old man who was referred to our late onset hypogonadism clinic presented with sweating and loss of appetite. His aging males' symptoms (AMS) and international index of erectile function (IIEF-5) scores were 59 and 2, respectively. His hormonal examination revealed extremely low free testosterone values. The patient was started on androgen replacement therapy, but his symptoms did not improve. Additional hormonal examinations revealed low values for other anterior pituitary hormones. Magnetic resonance imaging revealed an intrasellar cystic mass with suprasellar extension. We considered this mass caused hypothalamic hypopituitarism. A load test for anterior pituitary hormones revealed panhypogonadism. His symptoms improved after administration of adrenal and thyroid hormones and androgen. Five months after start of drug administration, his AMS score improved to 29, but IIEF-5 score showed little change. As a matter of course, not only androgen but all pituitary-related hormones are needed for hypopituitarism patients.

Association of coronary artery stenosis with carotid atherosclerosis in asymptomatic type 2 diabetic patients.

AIMS: Carotid atherosclerosis assessed by ultrasound is an established surrogate marker for systemic atherosclerosis. This study aimed to evaluate the association between coronary artery stenosis assessed by coronary computed tomography angiography (CCTA) and intima-media thickness (IMT) in asymptomatic type 2 diabetic patients. METHODS: In a cross-sectional study, carotid IMT was measured by ultrasound and CCTA in 169 asymptomatic type 2 diabetic patients. RESULTS: Among 169 patients, 77 (46%) had coronary artery stenosis on CCTA. Multivariate logistic regression analysis revealed four independent predictors of coronary artery stenosis: diabetes duration (OR 4.07, 95%CI 2.34-7.12; p< 0.001), gender (OR 1.66, 95% CI 1.05-2.61; p=0.029), dyslipidemia (OR 2.07, 95%CI 1.34-3.18; p=0.001), and max-IMT (OR 2.71, 95%CI 1.70-4.33; p< 0.001). By ROC curve analyses, the area under the curve (AUC) for max-IMT was 0.73 (95%CI 0.66-0.81; p< 0.001) and mean-IMT was 0.64 (95%CI 0.56-0.73; p=0.001). The cut-off level for the greatest sensitivity and specificity for max-IMT was 1.55 mm (sensitivity 0.90, specificity 0.46), and mean-IMT was 1.05 mm (sensitivity 0.55, specificity 0.72). CONCLUSION: Carotid IMT was associated with coronary artery stenosis assessed by CCTA in asymptomatic type 2 diabetic patients. Measurement of both mean- and max-carotid IMT is useful for selecting asymptomatic type 2 diabetic patients who should undergo CCTA screening.