Pregnancy complicated with Buerger's disease.

Buerger's disease is an inflammatory occlusive vascular disorder involving small- and medium-sized arteries in the distal extremities and is usually complicated with thrombophlebitis. Since Buerger's disease develops most frequently in men who smoke, pregnancy complicated with this disease is extremely rare. Only three pregnancies have been reported previously. All cases indicate that Buerger's disease worsens during pregnancy. However, anti-coagulant therapy appeared to be effective in this case. Accordingly, careful observation is mandatory in pregnancies complicated with Buerger's disease.     

Efficacy of axillary-axillary bypass grafting for the subclavian artery disease

Axillary-axillary bypass procedure was performed in 5 patients (3: atherosclerosis, 1: rupture of aortic arch aneurysm, 1: trauma) with the total occlusion of the subclavian artery. All the operations were performed with 6 mm ringed expanded polytetrafluoroethylene under general anesthesia. Though several types of extrathoracic procedures such as carotid-subclavian bypass, subclavian-subclavian bypass and axillary-axillary bypass are introduced, the efficacy of axillary-axillary bypass procedure is greater ease of anatomic exposure with no concern of interfering with the carotid circulation. The type of bypass to be used in a given case will depend on the individual circumstances. We suggest that this procedure is minimally invasive as well as effective for subclavian artery occlusion depending on the case.     

Intraclass correlation coefficient of trunk muscle thicknesses in different positions measured using ultrasonography

[Purpose] This study aimed to clarify the required number of measurements to calculate trunk muscle thickness at each position. [Participants and Methods] The participants were 30 elderly males aged >65 years. The right lumbar multifidus (L2), lumbar multifidus (L5), erector spinae, transversus abdominis, internal oblique, and external oblique muscle thicknesses were measured on longitudinal images obtained using ultrasonography in the lying, sitting, and standing positions. Two measurement values for each muscle thickness was used to calculate the intraclass correlation coefficient (1.1–1.5). [Results] The intraclass correlation coefficients of the abdominal muscle thickness measurements with “great reliabilities” were as follows: 1.3–1.5 for the external oblique muscle and 1.2–1.5 for the internal oblique and transversus abdominis muscles in the lying position; 1.3–1.5 for the external oblique and transversus abdominis muscles and 1.2–1.5 for the internal oblique muscle in the sitting position; the intraclass correlation coefficient in the standing position was 1.5 for the external oblique muscle 1.1–1.5 for the internal oblique muscle and 1.3–1.5 for the transversus abdominis muscle. In all the positions, the intraclass correlation coefficient of the measurements of the back-muscle thicknesses ranged from 1.1 to 1.5 for the right lumbar multifidus (L2), lumbar multifidus (L5), and erector spinae. [Conclusion] Depending on the posture, the abdominal muscles require multiple measurements, whereas the back muscles only require a single measurement.Key words: Intraclass correlation coefficient, Trunk muscle thicknesses, Ultrasonography     

Evaluation of apremilast,an oral phosphodiesterase 4 inhibitor,for refractory cutaneous dermatomyositis: A phase 1b clinical trial

Dermatomyositis, an idiopathic inflammatory myopathy, is characterized by cutaneous itchy manifestations, which are frequently refractory and recurrent even after intensive immunosuppressive treatments. To evaluate the effectiveness and safety of apremilast, an oral phosphodiesterase 4 inhibitor, in treating skin-dominant dermatomyositis in which myositis and interstitial lung disease are absent or in remission, we performed this prospective, single-arm, interventional study. A total of five Japanese patients (one male and four females, median [range] age, 64 [37–71] years) with refractory dermatomyositis-associated cutaneous manifestations were recruited and treated with a 12-week course of oral apremilast. Among five enrolled patients, three experienced diarrhea with full-dose apremilast (30 mg twice daily), two of whom withdrew from the study and recovered quickly afterwards. A total of three evaluable female patients (median [range] age, 65 [64–71] years) received apremilast treatment for 12 weeks. A 39.4% reduction from baseline Cutaneous Dermatomyositis Disease Area and Severity Index total activity score, but not the damage score, at week 12 was observed in all three patients. Visual analog scale of itching, and quality of life by Dermatology Life Quality Index were slightly improved in one and two apremilast-treated patients, respectively. As apremilast was effective, with expected and recoverable digestive adverse events (diarrhea), in patients with refractory and recurrent dermatomyositis-associated cutaneous manifestations in this first phase Ib study, it can be suggested as a possible treatment when aggressive immunosuppressive therapies with high-dose systemic corticosteroid and/or immunosuppressive agents for other manifestations, myositis, and interstitial lung disease, are not required.     

Marked hypercalcemia in a patient with hypocalciuric hypercalcemia without a mutation in the calcium-sensing receptor gene

A 60-year-old man was admitted to our hospital with marked hypercalcemia. He had no symptoms that might be caused by hypercalcemia. Plasma concentrations of calcium and intact parathyroid hormone were 15.2 mg/dl and 103 pg/ml, respectively. Radiological examinations revealed no abnormal findings. His calcium-creatinine clearance ratio was calculated to be 0.004, thus he was diagnosed as having hypocalciuric hypercalcemia. Familial hypocalciuric hypercalcemia was a plausible diagnosis, however, gene analysis of his calcium-sensing receptor (CaSR) revealed no mutation. The patient was thought to be a case of hypocalciuric hypercalcemia without mutation in the CaSR gene.     

Regional myocardial perfusion and metabolism assessed by positron emission tomography in children with Kawasaki disease and significance of abnormal Q waves and their disappearance

To clarify the significance of newly appearing abnormal Q waves and their disappearance in patients with Kawasaki disease, regional myocardial perfusion and glucose metabolism at rest in the fasting condition were assessed by positron emission tomography (PET) with ¹³N-ammonia and ¹⁸F-fluorodeoxyglucose (FDG), and regional wall motion by left ventriculography in regions with persistent and transient abnormal Q waves in 14 patients. PET identified 3 groups of abnormal myocardial segments: segments with hypoperfusion without increased FDG uptake, those with hypoperfusion and increased FDG uptake, and those with normal perfusion and increased FDG uptake. Almost all the segments with persistent or transient abnormal Q waves had abnormal PET findings. PET demonstrated evidence of metabolic activity in 57% of segments with persistent abnormal Q waves and 67% of those with transient abnormal Q waves. Regional wall motion, scored from 0 (normal) to 4 (dyskinesia), was not significantly different between segments with persistent and transient abnormal Q waves (2.3 ± 1.3 vs 2.2 ± 1.2). The persistence of abnormal Q waves on serial electrocardiograms was significantly shorter in metabolically active than in inactive segments (19 ± 17 vs 92 ± 27 months). In conclusion, in patients with Kawasaki disease, the new appearance of abnormal Q waves is a reliable clue to the presence of ischemic myocardial injury and a high proportion of them are associated with metabolically active myocardial regions. The disappearance of abnormal Q waves does not necessarily mean the normalization of regional myocardial perfusion, metabolism or function, and their early disappearance may imply “viability” in the associated myocardial region.