Shinji Kamei

Werner Syndrome and Diabetes Mellitus Accompanied by Adrenal Cortex Cancer

Werner syndrome is a rare genetic disease characterized by progeria, diabetes mellitus, cataracts and various types of malignancy. However, there are few reports showing adrenal cortex cancer in subjects with Werner syndrome. We herein report an extremely rare case of Werner syndrome accompanied by adrenal cortex cancer. Based on the data obtained from blood samples, computed tomography, magnetic resonance imaging and 131I adosterol scintigraphy, we diagnosed this subject with adrenal cortex cancer and Cushings syndrome. Since the prognosis of adrenal cancer is very poor, we should be aware of the possibility of adrenal cancer occurring in subjects with Werner syndrome.

Case of iliopsoas abscess that was markedly recovered after percutaneous and surgical drainage in a patient with poorly controlled type 2 diabetes.

We experienced a case of iliopsoas abscess which was markedly recovered after percutaneous and surgical drainage in a subject with poorly controlled type 2 diabetes. When iliopsoas abscess is suspected, physicians should survey patients by CT scan or MRI and should consider invasive treatment including surgical drainage.

Onset of type 1 diabetes mellitus and heparin-induced thrombocytopenia in a patient with Basedow's disease and idiopathic thrombocytopenic purpura: Novel combination as autoimmune polyglandular syndrome

Type 1 diabetes mellitus is often complicated with some other autoimmune disorders, and the complication of various autoimmune disorders is known as autoimmune polyglandular syndrome (APS). We experienced a patient who developed type 1 diabetes mellitus and heparin-induced thrombocytopenia (HIT) in addition to Basedow’s disease and idiopathic thrombocytopenic purpura (ITP). To our best knowledge, this is the first report showing that HIT is observed in APS patients. When the patient was aged 65 years, she had Basedow’s disease. She was treated with thiamazole (30 mg) or propylthiouracil (300 mg), but agranulocytosis was induced after starting the treatment with propylthiouracil. Therefore, she had radioactive iodine treatment (I 6 mCi). After the treatment, she had secondary hypothyroidism and started taking levothyroxine (50 lg/day). During the treatment, her platelets were decreased to 40 9 10/lL and platelet-associated immunoglobulin G was positive. She was diagnosed with ITP, which was well treated with prednisolone. After starting the treatment with prednisolone, the plateletassociated immunoglobulin G level was decreased and after several months it was finally normalized. When she was aged 77 years, she felt thirst, general fatigue, nausea and appetite loss. As such symptoms persisted for several months, she was hospitalized at Kawasaki Medical School Hospital, Kurashiki, Japan. Her height and bodyweight were 150.0 cm and 37.2 kg, respectively. Blood pressure and heart rate were 153/96 mmHg and 140 b.p.m, respectively. Body temperature was 37.2°C, blood glucose level was 737 mg/ dL, glycated hemoglobin was 10.3% and glycoalbumin was 48.4%. Insulin secretion was markedly suppressed: immunoreactive insulin was <1.0 lIU/ mL and serum C-reactive protein was 0.3 ng/mL. Ketone bodies were markedly increased: 3-hydroxybutyric acid was 11,310 lmol/L and acetoacetic acid was 3,850 lmol/L. In an arterial blood gas test, the pH was 7.21. The value of antiglutamic acid decarboxylase antibody in this patient was ≤1.3U/mL. However, considered from the onset speed of diabetes and depletion of insulin secretion, we diagnosed this patient with acuteonset type 1 diabetes mellitus and diabetic ketoacidosis. In addition, as various auto-antibodies (anti-glutamic acid decarboxylase antibody, anti-islet antigen-2 antibody, islet cell autoantibody and zinc transporter 8) were negative, we diagnosed this patients with type 1B diabetes mellitus. Renal dysfunction, probably as a result of dehydration, was observed: creatinine was 1.68 mg/dL and blood urea nitrogen was 73 mg/dL. Liver function and other endocrine hormone levels were within the normal range. As she had various autoimmune disorders, such as Basedow’s disease, ITP and type 1 diabetes mellitus, we diagnosed her with APS type 3. Human leukocyte antigen typing was as follows: DRB1, *04:05, *08:03; DQB1 04:01, 06:01, which were also compatible with type 1 diabetes mellitus and APS type 3. Hyperglycemia and ketoacidosis gradually recovered with fluid replacement and insulin therapy. As the patient had atrial fibrillation, we gave her unfractionated heparin (5,000 U/day for 3 days). After that, her platelets markedly decreased from 90 9 10/lL to 40 9 10/lL within several days. In addition, anti-HIT antibody was positive. After starting the treatment with heparin, purpura was observed in both legs. Therefore, we stopped heparin, and after then the purpura disappeared. Furthermore, this patient had a past history of stroke. Although the patient had completely recovered from stroke without any sequela, it seemed that this stroke was also related to HIT. In addition, the 4Ts score in this patient was 4 points (thrombocytopenia 2 points, timing of platelet count fall 0 points, thrombosis or other sequela 1 point, other causes for thrombocytopenia 1 point). We finally diagnosed the patient with HIT based on its diagnosis criteria. HIT is a serious side-effect of heparin, and is observed in a small percentage of patients treated with heparin. HIT leads to the development of thromboembolism and is a lifethreating disease without appropriate therapy, such as stopping heparin. AntiHIT antibody is an antibody against the complex of platelet factor 4 and heparin, which is thought to lead to the onset of *Corresponding author. Hideaki Kaneto Tel.: +81-86-464-1111 Fax: +81-86-464-1046 E-mail address: kaneto@med.kawasaki-m.ac.jp Received 9 October 2017; revised 10 November 2017; accepted 11 January 2018

Effect of Tofogliflozin on Body Composition and Glycemic Control in Japanese Subjects with Type 2 Diabetes Mellitus

Sodium-glucose cotransporter 2 inhibitor tofogliflozin is a new type of antidiabetic drug for individuals with type 2 diabetes mellitus (T2DM). The aim of this study was to examine in which type of individuals and/or under which conditions tofogliflozin could exert more beneficial effects on body composition and/or glycemic control in Japanese individuals with T2DM. We retrospectively evaluated the effects of tofogliflozin on body composition and/or glycemic control in individuals with T2DM who newly started taking tofogliflozin. After tofogliflozin treatment, body weight was significantly reduced and HbA1c levels were significantly decreased. Body fat mass, skeletal muscle mass, and skeletal muscle index, a marker for sarcopenia, were also reduced after the treatment. In univariate analyses, there was a statistically significant association between the decrease of HbA1c level after tofogliflozin treatment (Δ HbA1c) and the following parameters such as HbA1c levels at baseline, visceral fat area (VFA) at baseline, and reduction of VFA after the treatment (Δ VFA). Furthermore, in multivariate analyses, HbA1c levels at baseline and duration of diabetes were independently associated with Δ HbA1c. These results suggest that tofogliflozin would be more suitable for relatively obese individuals whose duration of diabetes is relatively short.