Background: Although rectal tenesmus in patients with advanced cancer can have marked negative impact on quality of life, effective treatment has not yet been established. Case: A 71 -year-old man with an inoperable rectal cancer developed tenesmus 11 months after a colostomy. Tenesmus worsened over the following 3 months, and the patient suffered from involuntary straining every 5-15 minutes. After unsuccessful symptom control with radiotherapy to the primary lesion, we started oral amoxapine 25 mg that alleviated symptoms related to tenesmus. As the general condition deteriorated, however, oral intake became difficult. After the discontinuation of amoxapine, the tenesmus recurred even though intravenous administration of clomipramine was initiated. We started continuous infusion of intravenous lidocaine 200 mg/day which successfully relieved tenesmus. The dose of lidocaine was subsequently increased to 290 mg/day for worsening symptoms, which continued to control his distress caused by tenesmus until he died. Consideration/Conclusion: This is the first report that demonstrates the efficacy of oral amoxapine for rectal tenesmus with malignant tumor. After the discontinuation of amoxapine due to the inability of taking medications orally, symptoms remained under adequate control with infusional lidocaine until the patient died. Further studies are warranted to confirm our findings and to propose optimal use of medications in the management of rectal tenesmus.

Mucolipidosis is an autosomal recessive lysosomal storage disorder that demonstrates a clinical resemblance to mucopolysaccharidosis. Accumulation of glycoproteins throughout the body causes dysfunction of several organs, in particular, valvular heart diseases are an important cause of mortality, however, there is no consensus guideline regarding the indications and optimal timing of the surgical repair because of the unclear and short natural history. Here we present 12- and 15-year-old siblings diagnosed with mucolipidosis who underwent aortic valve replacement. The senior sibling received redo-aortic valve replacement for prosthetic valve dysfunction 11 years after the initial surgery. A few surgical valve replacements in patients with mucopolysaccharidosis have been reported, however, there is no published case of aortic valve replacements in two siblings with mucolipidosis.

Objective: To describe the treatment patterns and time to next treatment (TTNT) in newly diagnosed multiple myeloma patients (MM) using a large-scale claims database in Japan.Design: Cohort studyMethods: The patients with newly diagnosed MM from 2008 to 2015 were classified into two groups: age <65 years, and age ≥65 years. Specific regimens and general regimens were identified with a complex algorithm considering interval of no therapy, additional and discontinued agents. Correspondingly, TTNT between the first- and second-line were measured among non-transplant patients with Kaplan-Meier method.Results: A total of 425 patients were eligible to participate in the analysis. The most common regimen for the treatment of MM was bortezomib-based regimens (52.9％ in the first-line, 28.2％ in later lines), followed by melphalan-prednisolone (27.1％ in the first-line, 12.9％ in later lines) and lenalidomide-based regimens (4.7％ in the first-line, 26.1％ in later lines). TTNT between the first- and second-line was 11.4 months and was seen to vary greatly with each regimen. A statistically longer TTNT was observed in subgroups of patients aged 65 years or over compared with patients aged younger than 65 years, but no statistical difference was found between conventional therapy and novel therapy.Conclusion: Based on the data from the study, patients with MM were commonly treated with novel agent-based regimens, especially bortezomib-based regimens. Between the first- and second-line therapies a relatively short TTNT was observed, indicating that therapies in clinical practice poorly complied with treatment guidelines.

A 71-year-old man presented to our hospital with sudden-onset epigastric pain. He reported a history of undergoing the following operations : aortic valve replacement for aortic regurgitation 11 years earlier and graft replacement of the ascending aorta for acute type A aortic dissection, 1 year earlier. His systolic blood pressure was 70 mmHg, and computed tomography revealed a pseudoaneurysm of the distal anastomosis of the ascending aorta with a connection to the right pulmonary artery. Cardiopulmonary bypass was established with cannulation of the right axillary artery and the right femoral vein, and systemic cooling was initiated before sternotomy. We identified an area showing 3 cm dehiscence at the distal aortic anastomosis after hypothermic circulatory arrest and selective cerebral perfusion. The ascending aorta was replaced as hemiarch replacement, and the defect in the right pulmonary artery was closed with bovine pericardium. The patient's postoperative course was uneventful, and he was transferred to a rehabilitation hospital on the 22nd postoperative day.

In acute Stanford type A aortic dissection, except for some thrombosed false-lumen types, graft replacement is a standard treatment. On the other hand, thoracic endovascular aortic repair (TEVAR) might be considered for high-risk patients with retrograde type A aortic dissection when entry is in the descending aorta, although its efficacy in a case of an extensive thrombosed false lumen without obvious entry is unknown. We report a case of successful zone 3 TEVAR using RelayPro NBS for Stanford type A aortic dissection with a localized CT-enhanced false lumen in the proximal descending aorta. An 83-year-old woman was admitted for acute Stanford type A aortic dissection with a thrombosed false lumen of the ascending thoracic aorta. She was initially treated conservatively because of being a high-risk patient for open surgery. One week after hospitalization, the ascending aorta diameter increased and the false lumen in the proximal descending aorta grew sporadically in a CT image. We suspected that the ascending aorta was enlarged due to a partially patent false lumen of the descending thoracic aorta, and performed zone 3 TEVAR using RelayPro NBS to close a possible entry in the proximal descending aorta even though there was no obvious entry. The patient had a good postoperative course and was discharged 15 days after TEVAR. Shrinkage of the false lumen in the ascending aorta was observed in CT images two months after TEVAR.