Chylothorax following transsternal total thymectomy: a case report.

Herein, we report a case of chylothorax following total thymectomy. A 46-year-old woman having an anterior mediastinal tumor underwent a thymectomy via median sternotomy. Seven days after surgery, there was no massive pleural effusion. However, on post-operative day 17, a right massive pleural effusion was detected, and it was diagnosed as chylothorax. She was successfully treated with conservative therapy. Chylothorax following thymectomy is a very rare complication.

A Japanese Patient with Gaucher Disease Treated with the Oral Drug Eliglustat as Substrate Reducing Therapy.

Gaucher disease is a rare genetic disorder caused by the deficiency of acid ß-glucosidase to effectively catalyze the degradation of glucosylceramide to glucose and ceramide. We report here the case of a 31-year-old male Japanese patient with Gaucher disease who switched from enzyme replacement therapy (ERT) to substrate reducing therapy (SRT). Liver dysfunction was identified at a routine medical checkup, and the patient was referred to our hospital with "idiopathic liver disease." Clinical laboratory tests indicated thrombocytopenia and splenomegaly, which are characteristic symptoms of Gaucher disease. To definitively diagnose Gaucher disease, a bone marrow biopsy and acid ß-glucosidase activity measurement were conducted; the results supported a diagnosis of Gaucher disease. This case emphasizes that it is possible for periodic medical checkups in adults to lead to the diagnosis of rare genetic disorders. The patient underwent ERT treatment with imiglucerase for 5 years; the platelet count rapidly increased and the spleen size rapidly decreased, indicating a good response to the drug. However, the patient increasingly felt the burden of visiting the hospital for 2 h of infusion ERT every 2 weeks. Consequently, it was jointly decided that he should switch from ERT to SRT with an oral drug. This switch was successful with no deterioration of laboratory data. This case report is the first to describe a Japanese Gaucher disease patient treated with eliglustat for >2 years. We showed that SRT is a well-tolerated and effective option for the treatment of Gaucher disease.

Recovery from Diabetic Macular Edema in a Diabetic Patient After Minimal Dose of a Sodium Glucose Co-Transporter 2 Inhibitor.

BACKGROUND Diabetic macular edema (DME) causes serious visual impairments in diabetic patients. The standard treatments of DME are intra-vitreous injections of corticosteroids or anti-vascular endothelial growth factor antibodies and pan-photocoagulation. These treatments are unsatisfactory in their effects and impose considerable physical and economic burdens on the patients. CASE REPORT A 63-year-old woman was diagnosed as type 2 diabetes with retinopathy 7 years ago. Before the initiation of an SGLT2 inhibitor, the dipeptidyl peptidase-4 inhibitor, sitagliptin (50 mg daily), and metformin (250 mg dai- ly) were used for her glycemic control. The level of her hemoglobin A1c had been controlled around 7%. She began to feel decreased visual acuity and blurred vision of her left eye 8 months before the visit to our clin- ic. She was diagnosed as DME, which turned out to be corticosteroid-resistant. Her visual acuity further de- creased to 20/50. Metformin was changed to ipraglifl (25mg/day). Her left visual acuity started to improve after 4 weeks of treatment with ipragliflozin and improved to 20/22 after 24 weeks. The macular edema did not change until 12 weeks of the treatment, however, it decreased prominently after 16 weeks. CONCLUSIONS In our patient with steroid-resistant DME, her visual symptoms and macular edema recovered after the initiation of an SGLT2 inhibitor. SGLT2 inhibitors might be a potential candidate for the DME treatment.

Significance of estimating the glomerular filtration rate for the management of hypertension in type 2 diabetes with microalbuminuria.

The Home Blood Pressure for Diabetic Nephropathy (HBP-DN) study is a prospective cohort study to determine the optimal home blood pressure (HBP) required to improve the prognosis of type 2 diabetic patients with microalbuminuria. The glomerular filtration rate (GFR) is heterogeneous in microalbuminuric diabetic patients. The first step of the HBP-DN study explored the significance of estimating GFR for the identification of patients for whom HBP measurement would be more helpful. The patients were divided into two groups based on their estimated GFR (eGFR): a low eGFR group (eGFR <60 ml min(-1) per 1.73 m(2), N=75) and a preserved eGFR group (eGFR 60 ml min(-1) per 1.73 m(2), N=193). HBP, variability of HBP and a previous history of vascular complications were compared between the two groups. The number of antihypertensive drugs used for the patients and the s.d. of home systolic blood pressure (HSBP) in the low eGFR group were significantly higher than those in the preserved eGFR group. A stepwise multiple linear regression analysis also revealed that the low eGFR group was independently correlated with the s.d. of HSBP. A multiple logistic regression analysis revealed that a low eGFR was an independent risk factor for coronary heart disease and advanced retinopathy. Estimating the GFR is important to identify patients with elevated blood pressure variability, as well as those with a high risk of vascular complications. Measuring HBP would be more helpful for the effective and safe treatment of hypertension in patients with a low eGFR than in those with a preserved eGFR.

Short-term effects of ß-adrenergic antagonists and methimazole in new-onset thyrotoxicosis caused by Graves' disease.

OBJECTIVE: ß-adrenergic antagonists (ß-blockers) are often used to attenuate the hyperadrenergic symptoms of Graves' disease (GD), including palpitation. Although ß-blockers reduce the heart rate, cardiac output and oxygen consumption, no firm evidence exists regarding the effects of combined therapy with ß-blockers and anti-thyroid drugs. The objective is to elucidate the effects of ß-blockers on anti-thyroid drug therapy in GD. METHODS: Patients newly diagnosed with mild GD were randomly assigned to receive methimazole with or without ß-blockers in a prospective multi-center survey. The heart rate and thyroid function were measured and the quality of life was assessed using original and SF-36 questionnaires at 0 and 4 weeks. RESULTS: A total of 28 patients were enrolled in the study. Fourteen patients (one man, 13 women) were randomly assigned to the group treated with ß-blockers and 14 patients (one man, 13 women) were randomly assigned to the group not treated with ß-blockers. Although no significant differences in the improvement of thyroid function were observed between the two groups, the heart rates improved more significantly in the group treated with ß-blockers. Specific symptoms, such as easy fatigability and shortness of breath, also improved more significantly with the ß-blocker treatment. In addition, 'physical functioning' assessed with the SF-36 questionnaires significantly improved only in the group treated with ß-blockers. CONCLUSION: Although ß-blockers may not reinforce the effects of anti-thyroid drugs on thyroid function, at least during the course of one month, they are effective in reducing heart rates and ameliorating specific symptoms in patients with mild GD.

Gas-containing pericardial abscess in a type 2 diabetic patient.

A 63-year-old male with type 2 diabetes mellitus was admitted to our hospital with fever and chest pain. An echocardiogram, chest CT and MRI showed the gas-containing pericardial abscess located posteriol to the right atrium. He was initially treated by thoracoscopic pericardial fenestration to set a drainage tube in the pericardial abscess. However, the surgical treatment was discontinued because of a large amount of bleeding from the abscess wall. The patient was then treated by continued administration of antibiotics and gamma-globulin. The inflammatory reactions improved and shrinkage of the abscess was confirmed.

Autopsy of a patient with Cushing's Syndrome who was revealed to have pulmonary tumorlets producing ectopic ACTH.

The patient, a 78-year-old female with a 10-year history of type 2 diabetes mellitus, was admitted to our department for evaluation of leg edema and general fatigue. Biochemical investigations revealed hypokalemia and elevated serum cortisol and plasma ACTH levels, with a loss of diurnal rhythm and failure of suppression at high doses (8 mg) of dexamethasone. No pituitary tumor or parasellar tumor was detected by contrast-enhanced computed tomography (CT) or magnetic resonance image scan of the pituitary. High resolution CT of the lung and bronchoscopic examination revealed no abnormalities. Abdominal and pelvic CT indicated bilateral, slightly diffuse, adrenal gland hyperplasia only. These findings led to a diagnosis of ACTH-dependent hypercortisolism from an undefined source. Ten days after admission the patient had a fever and was diagnosed with disseminated intravascular coagulation. Despite intensive treatment about 1 month after admission the patient died from progressive multiple organ failure. At autopsy, a histological examination of the periphery of the right middle lobe of the lung revealed the presence of tumorlets. Immunohistochemical staining of the tumorlets revealed scattered cells containing ACTH and many cells containing chromogranin A that were positive for Grimelius staining. In addition, multiple microabscesses were present throughout most tissues of the body. The ectopic hormonal production observed in the present case suggests that pulmonary tumorlets should thus be considered in the differential diagnosis of Cushing's syndrome, and medical treatment to inhibit steroidogenesis should be started immediately to reduce the risk of complications from hypercortisolism.

Short term intensive insulin therapy improves insulin secretion significantly in type 2 diabetic patients.

To investigate the effects of short-term (1 week) intensive insulin therapy, on glycemic control, insulin secretion, and insulin sensitivity in type 2 diabetic patients, an open prospective study was conducted in sixteen type 2 diabetic patients receiving diet therapy alone or treatment with oral hypoglycemic agents. Of the study subjects, 8 patients were treated with insulin, the remaining 8 patients served as the control group. The metabolic parameters were evaluated once before treatment and once during one of the following treatments : glycemic control as measured by 1,5-anhydro-D-glucitol (1,5-AG) and area under curve of glucose (AUCglucose), insulin secretion as measured by area under curve of daily serum insulin (AUCinsulin), and insulin sensitivity as measured by the K index of the insulin tolerance test (K(ITT)). Post-treatment plasma glucose (AUCglucose) and 1,5-AG levels in patients who had received intensive insulin therapy were comparable to those of the control group. A statistically significant increase in AUCinsulin occurred after intensive insulin therapy for just 1 week, while no change occurred in the control group. Insulin sensitivity (K(ITT)) did not improve significantly in patients treated with insulin or patients from the control group. These results indicate that intensive insulin therapy for 1 week improves insulin secretion remarkably but has little effect on insulin sensitivity in type 2 diabetic patients. Clinically, this suggests that intensive insulin therapy for one week might be one of the initial treatments of choice for such patients.

[Stent-assisted percutaneous transluminal angioplasty for intracranial vertebral and basilar artery stenosis: two case reports].

We report two cases of patients who underwent stent-assisted percutaneous transluminal angioplasty for intracranial vertebral artery (VA) and basilar artery (BA) stenosis. They presented with sudden onset of vertigo and right hemiparesis. Vertebral angiographies revealed severe proximal BA stenosis (case 1) and intracranial VA stenosis (case 2). They underwent angioplasty and stenting, with excellent angiographic results. The availability of new flexible intravascular stents, allowing access to tortuous proximal intracranial vessels, provides a new therapeutic approach for patients with vertebral and basilar artery stenosis.