Hematopoietic stem cell transplantation (SCT) recipients are exposed to a large amount of anti-cancer drugs, immunosuppressors, and irradiation during the peri-SCT period. Thus, they have to overcome serious adverse events related to unavoidable but toxic procedures, including organ disorders. In particular, acute kidney injury (AKI) is one of the most critical complications, because it influences the mortality of patients. A few patients who survive AKI may develop nephrotic syndrome, and precedent AKI is also closely associated with chronic and progressive loss of the renal function in post-SCT patients. These kidney diseases place a heavy burden on SCT patients, both medically and economically. Therefore, hematologists who evaluate SCT should be fully aware of the development of these kidney diseases after SCT. We herein review the common course of kidney disease development following allogeneic SCT to provide healthcare professionals with practical information on renal disease in SCT patients.

Objective Although esophageal granular cell tumors have been reported to present as hypoechoic tumors, we noticed that their echogenicity is similar to that of the submucosal layer. We investigated the sonographic features of esophageal granular cell tumors and the diagnostic accuracy of the features.

Methods Seven patients with esophageal granular cell tumors who underwent endoscopic ultrasonography were retrospectively reviewed. Thirteen patients with esophageal leiomyoma were selected as historical control subjects. The brightness of the tumor on ultrasonography images was measured and the echogenicity was standardized according to the echogenicity of the proper muscle and submucosal layers. Ten board-certified endoscopists then independently evaluated the endoscopic pictures of the 20 patients (Test 1), as well as the endoscopic ultrasonography images together with endoscopic pictures of the same patient set (Test 2).

Results The standardized echogenicity in granular cell tumors was significantly higher than that in leiomyomas. The diagnostic accuracy of the 10 evaluators using endoscopic pictures alone (Test 1) was 72.0%. The addition of endoscopic ultrasonography images (Test 2) significantly improved the accuracy to 93.0%.

Conclusion The echogenicity of granular cell tumors was similar to that of the submucosal layer, and it was significantly higher than that of leiomyomas. Endoscopic ultrasonography images facilitate the accurate identification of esophageal granular cell tumors.

Objective Endoscopic self-expandable metallic stent (SEMS) placement and gastrojejunostomy (GJY) are palliative treatments for malignant gastric outlet obstruction (GOO). The aim of the present study was to compare the palliative effects of these treatments and identify predictors of a poor oral intake after treatment.

Methods and Patients In total, 65 patients with GOO at multiple centers in Saga, Japan, were evaluated. Thirty-eight patients underwent SEMS placement, and 27 underwent GJY from January 2010 to December 2016. The characteristics and outcomes of the two groups were compared to detect predictors of treatment failure.

Results No significant differences in the technical success, clinical success, post-treatment total protein, hospital discharge, duration from eating disability to death, or post-treatment overall survival were present between the SEMS and GJY groups. More patients in the GJY group than in the SEMS group received chemotherapy (51.4% vs. 26.3%, respectively; p=0.042). The period from treatment to the first meal was longer in the GJY group than in the SEMS group (4.5 vs. 3.0 days, respectively; p=0.013). The present study did not identify any risk factors for failure of SEMS placement. Although the stent length tended to be associated with a poor prognosis, the correlation was not statistically significant (odds ratio: 0.60, 95% confidence interval: 0.36-1.01, p=0.053).

Conclusion Patients with GOO started meals more promptly after SEMS than after GJY, but the clinical outcomes were not markedly different between the SEMS and GJY groups. These findings suggest that endoscopic uncovered SEMS placement might be a feasible palliative treatment for GOO.

Objective Autoimmune pancreatitis (AIP) has been recognized as a benign disease, which that shows a prompt response to corticosteroid treatment (CST). It was previously believed to not be associated with cyst formation; however, a few cases of AIP-associated pancreatic cyst (PC) have been reported. Some cases were reported to have been effectively treated by CST, while others were refractory to CST. Many of the patients received interventional treatment. Until now, there has been no consensus on the therapeutic strategies for AIP-associated PC. The aim of the present study is to describe a therapeutic strategy for this condition.

Methods We conducted a retrospective study of 5 cases of AIP-associated PC that were treated by endoscopic ultrasonography-guided pancreatic fluid collection drainage (ESPD) or CST at Tokyo Medical University Hospital between March 2012 and October 2016, analyzed the therapeutic outcomes, and performed a literature review.

Results The initial treatments included CST (n=2) and ESPD (n=3). All of the PCs disappeared after treatment In 1 of the patients who received CST case and 3 of the patients who received ESPD; however, the PC did not disappear in one of the patients who received CST (corticosteroid maintenance therapy), even after the dose of corticosteroids was increased; ESPD was eventually performed and the PC disappeared. There were no procedure-related complaints.

Conclusion We propose that CST be administered as the first-line treatment for AIP-associated PC, particularly in cases of PC without a history of CST. However, ESPD can be applied to treat cases of corticosteroid refractory PC.

Objective Interferon-free regimens of direct-acting antiviral agents have improved the treatment response for chronic hepatitis C virus (HCV) infection, and improvement in the serum albumin level during interferon-free therapy has been reported. The aim of this study was to identify the factors that influence the improvement in the serum albumin level in patients receiving interferon-free antiviral therapy.

Methods This retrospective, multicenter study consisted of 471 Japanese patients with chronic hepatitis and compensated liver cirrhosis infected with HCV who completed 12-week interferon-free sofosbuvir (SOF)-based therapy [SOF plus ledipasvir for genotype 1 (n=276) and SOF with ribavirin for genotype 2 (n=195)]. We evaluated the changes in the serum albumin level from baseline to the end of treatment (ΔAlb).

Results When compared with the normal-albumin group (baseline serum albumin >35 g/L, n=406), the low-albumin group (baseline serum albumin ≤35 g/L, n=65) showed a significant increase in the mean ΔAlb (5.5 g/L vs. 1.0 g/L, p<0.001). In the low-albumin group, a multivariate logistic regression analysis extracted diabetes mellitus as a negative predictive factor of median ΔAlb >5.0 g/L (odds ratio: 0.19, 95% confidence interval: 0.048-0.79, p=0.020). In the low-albumin group, the mean ΔAlb was significantly lower in the diabetic patients (n=14) than in the non-diabetic patients (n=51) (3.9 g/L and 5.7 g/L, p=0.049).

Conclusion Interferon-free SOF-based therapy significantly improved the serum albumin in the low-albumin group patients with chronic HCV infection. However, the improvement in the serum albumin level was significantly lower in the diabetic patients than in the non-diabetic patients.

Objective Patients with acute hepatitis B sometimes develop acute liver failure (ALF), which has a poor prognosis. The efficacy of nucleoside analogue (NA) monotherapy for ALF due to transient hepatitis B virus infection (HBV-ALF) remains controversial. Further investigations are necessary in nations with a shortage of donor livers for liver transplantation. In the present study, we aimed to clarify the efficacy of combination therapy with corticosteroid (CS) and NA in the treatment HBV-ALF.

Patients We examined the clinical and biochemical features of 19 patients with HBV-ALF who were treated in the early stage of the disease between 2000 and 2015.

Results Fourteen patients received CS and NA (CS + NA group) and 5 received NA monotherapy (NA group). Eleven patients (58%) survived and 8 (42%) died. The survival rates in the CS + NA and NA groups were 64% and 40%, respectively (p=0.60). The mean alanine aminotransferase (ALT) levels declined significantly at week 2 in both groups. The mean PT activities improved significantly at weeks 1 and 2 in the CS + NA group (p<0.05) but not in the NA group. None of the surviving patients developed persistent infection.

Conclusion Combination therapy with CS and NA induces the rapid resolution of inflammation leading to a rapid recovery of the liver function. When it is administered at a sufficiently early stage, it would have a survival benefit and prevent persistent infection in HBV-ALF.

Objective Obstructive sleep apnea (OSA) is assumed to influence the circadian blood pressure (BP) fluctuation, particularly causing nocturnal hypertension and changing the dipping pattern of nocturnal BP. This study aimed to clarify the triggers of the non-dipper pattern in nocturnal BP in Japanese patients with severe OSA (the apnea-hypopnea index ≥30/h).

Methods Of 541 patients with OSA diagnosed using polysomnography (PSG) and ambulatory BP monitoring (ABPM), 163 patients <60 years of age (Younger group) and 101 patients ≥60 years of age (Older group) were stratified into the dipper or non-dipper pattern groups.

Results A logistic regression analysis was performed using a non-dipper pattern as a dependent variable. A multivariate analysis demonstrated that the cumulative percentage of time at saturation below 90% was the only independent risk factor for the non-dipper and riser patterns in the Younger group (odds ratio, 1.022; 95% confidence interval, 1.001-1.044; p=0.035), whereas slow-wave sleep (odds ratio, 0.941; 95% confidence interval, 0.891-0.990; p=0.019) and the use of angiotensin-converting enzyme inhibitors or angiotensin II receptor blockers (odds ratio, 2.589; 95% confidence interval, 1.051-6.848; p=0.039) were risk factors in the Older group.

Conclusion These findings suggested that the degree of desaturation in young OSA patients and sleep quality in old OSA patients might influence the dipping patterns in nocturnal BP.

Objective Evidence suggests that the eating rate is positively associated with the body weight and blood pressure. Furthermore, people who are overweight or obese tend to have higher salt intakes than those of normal weight. To investigate whether or not the eating rate is also associated with the salt intake, a cross-sectional study was conducted using health examination survey data collected in 2014 from 7,941 residents of Sado City, Niigata, Japan.

Methods The eating rates were evaluated using a questionnaire; 11.7% of participants rated themselves as slow eaters, 65.6% as normal eaters, and 22.7% as fast eaters. The salt intake was estimated from sodium and creatinine spot urine measurements using Tanaka's formula. Associations with eating rate were evaluated using multivariate linear regression analyses, with normal eaters as the reference (set at 0).

Results Self-reported eating rates were positively associated with the salt intake after adjustment for age and sex [β coefficient (95% confidence interval) for slow -0.51 (-0.67, -0.35); fast 0.18 (0.05, 0.30) ]. Further adjustment for the body mass index showed that slower eaters had lower salt intakes than normal eaters, but there was no marked difference in the salt intake between normal and fast eaters. The association between slower eating and a lower salt intake persisted after further adjustment for comorbidities [slow -0.33 (-0.49, -0.18) ].

Conclusion Our results suggest that reducing eating rates may be an effective strategy for reducing dietary salt intake as well as preventing obesity.

Objective The purpose was to evaluate the association between bite instability and comorbidities, comprehensive geriatric evaluations, or disabilities in elderly people.

Methods A dentist examined the oral function, such as the bite stability, number of teeth, and the use of dentures, in 119 patients (93 women, mean age: 86.7±7.8) in 2 nursing homes for the elderly. The association between the oral function and the prevalence of diseases, including hypertension, diabetes mellitus, and dementia, was analyzed.

Results The median number of teeth was 0 [0, 4]. The patients were divided into a bite-stable group (n=78, 66%) and bite-unstable group (n=41, 34%). The prevalence of hypertension was significantly higher in the bite-stable group than in the bite-unstable group (83% vs. 63%, respectively; p=0.0149), whereas the prevalence of diabetes mellitus was significantly lower in the bite-stable group than in the bite-unstable group (10% vs. 27%, respectively; p=0.0190). The prevalence of a cognitive function decline was significantly lower in the bite-stable group as well (59% vs. 83%, p=0.0082). According to the simplified comprehensive geriatric assessment 7, the bite-stable group scored significantly higher for instrumental activities of daily living (ADL) than the bite-unstable group (54% vs. 24%, respectively; p=0.0021). A multivariate logistic regression analysis demonstrated that bite instability was independently correlated with hypertension, diabetes mellitus, and instrumental activities of daily living.

Conclusion Bite instability was independently associated with a decreased prevalence of hypertension or increased prevalence of diabetes mellitus and low levels of instrumental ADL in the elderly.

Sodium polystyrene sulfonate (SPS: Kayexalate^®) is an ion-exchange resin used to treat hyperkalemia in patients with chronic kidney disease. It is known that this resin sometimes causes colonic necrosis and perforation, but there are few reports about small bowel necrosis associated with SPS. We herein report the case of a patient who developed SPS-induced small bowel necrosis, which was diagnosed based on the examination of a small bowel endoscopic biopsy specimen. The SPS-induced small bowel necrosis was resistant to conservative treatment including the cessation of SPS, and finally required surgical bowel resection.

The patient was a 72-year-old woman whose alkaline phosphatase levels had been elevated since she was 56 years old. Liver biopsies obtained when the patient was 64 and 66 years of age led to a suspicion of cholangitis caused by vasculitis. Furthermore, proteinase-3 anti-neutrophil cytoplasmic antibody positivity led to a suspicion of granulomatosis with polyangiitis, but subjective symptoms and disorders in other organs were absent, so this suspicion was not confirmed. Cholangitis caused by vasculitis rarely occurs without vasculitis in other organs. We herein report this case in which we obtained distinctive laparoscopic and imaging findings that raised suspicions of liver circulatory failure.

Autoimmune hepatitis (AIH) and type 1 diabetes mellitus (T1DM) are thought to be induced by autoimmunity, but their coexistence has rarely been reported. We herein report a case in which a patient with T1DM developed acute-onset AIH. A 26-year-old woman, who had been diagnosed with T1DM in childhood, was transferred to our hospital because of acute liver failure of unknown etiology. The administration of corticosteroids including steroid pulse therapy was effective. Based on the histological finding of massive centrilobular necrosis and a good response to steroid therapy, we diagnosed the patient with acute-onset AIH. This case indicates that AIH can occur in young T1DM patients.

A 40-year-old man was transferred to our hospital following an isolated horse kick injury to the anterior chest wall. The case showed bi-fascicular block, severe tricuspid valve regurgitation due to ruptured chordae tendineae of the anterior leaflet, moderate mitral valve regurgitation due to prolapse of mitral anterior leaflet, and hypokinetic motion of the inferior septal wall. Both tricuspid and mitral insufficiency were completely repaired by a surgical operation. Fortunately, these injuries were not fatal in this case, but the comprehensive assessment of cardiac damage and careful observation are important for managing patients with cardiac injury.

We herein report a case of concurrent cardiac sarcoidosis and large-vessel aortitis detected by ¹⁸F-fluorodeoxyglucose positron emission tomography (FDG-PET) and followed up during immunosuppressive therapy. After high-dose prednisolone administration (1 mg/kg), serial FDG-PET showed that almost all of the abnormal FDG uptake in the heart and extracardiac region, including the abdominal to bilateral iliac arteries, had been disappeared. During the tapering of prednisolone, additive methotrexate therapy was needed to treat the recurrence of cardiac sarcoidosis. FDG-PET is a useful tool for detecting cardiac sarcoidosis concomitant with large-vessel aortitis and monitoring the effectiveness of immunosuppressive therapy.

A 71-year-old woman was admitted with dyspnea. An electrocardiogram revealed ST-segment elevation, and echocardiography showed akinesis in the left ventricular apex with hyperkinesis of the base. Coronary angiography revealed no stenosis, and left ventriculography indicated ballooning of the left ventricular apex and apical ventricular septal perforation. We diagnosed the patient with Takotsubo syndrome complicated by ventricular septal perforation, which was surgically repaired. Although ventricular septal perforation is recognized as a life-threatening complication after acute myocardial infarction, it can also occur after Takotsubo syndrome. The early recognition and management of this condition can help prevent morbidity and mortality.

Cerebrotendinous xanthomatosis (CTX) is a rare, autosomal recessive, inborn disruption in bile acid synthesis characterized by severe systemic xanthomas, cataracts and neurological injuries occurring before adolescence without elevation of the serum cholesterol or triglyceride levels. CTX is caused by a deficiency of the mitochondrial enzyme sterol 27-hydroxylase, which is encoded by the CYP27A1 gene. We herein report a 50-year-old Japanese woman with late-onset CTX who had no relevant symptoms before the development of bilateral Achilles tendon xanthomas in middle age. A genetic analysis revealed a compound heterozygous mutation in the CYP27A1 gene with a previously known missense mutation (NM_000784.3:c.1421 G>A) and a novel frame shift mutation of NM_000784.3:c.1342_1343insCACC.

A 31-year-old woman was admitted to our hospital for thrombotic microangiopathy (TMA). She was diagnosed with systemic lupus erythematosus (SLE) and class V lupus nephritis. She had no aggravated SLE activity, Shiga toxin positivity, ADAMTS13 abnormality, or other causes of secondary TMA. Plasma exchange partially improved TMA, and eculizumab was introduced for suspected atypical hemolytic uremic syndrome (aHUS), as eculizumab was effective in suppressing the TMA activity. A kidney biopsy revealed diffusely organized crescents (pseudotubulization) with glomerular and arteriolar endothelial injury and subepithelial immune deposits. Thus, this was a rare case of lupus nephritis presenting as TMA with pseudotubulization possibly caused by aHUS.

Necrotizing sarcoid granulomatosis (NSG) is a rare disease that is diagnosed based on pathological findings. We herein report the case of a 27-year-old man who had multiple nodular shadows in bilateral lung fields on chest radiography and elevated levels of C-reactive protein (CRP). The pathological evaluation of a lung biopsy specimen showed the infiltration of lymphocytes, granulomas with necrosis and granulomatous angiitis. He was therefore diagnosed with NSG. He has been followed without treatment, as his fever and CRP levels decreased immediately after the surgical lung biopsy. Thereafter, the pulmonary nodular shadows gradually recovered without any treatment within a few months. Our experience suggests the possibility that surgical invasion might trigger an improvement in disease activity.

Diffuse pulmonary ossification (DPO) is a rare disease characterized by metaplastic bone formation in the lung. There are few reports with a long-term follow-up of this disease. We herein report a 47-year-old man diagnosed with idiopathic DPO at 30 years of age. The patient's vital capacity was normal until 36 years of age (3.39 L, 82.4% predicted), but it was severely decreased when he visited the hospital again at 47 years of age due to cough and dyspnea (1.98 L, 44.6% predicted). Chest computed tomography showed a significant increase in the number of high-density nodules, suggesting that the progression of DPO had caused restrictive ventilatory impairment.

A patient with a history of lung adenocarcinoma was admitted because of palpitation. Transthoracic echocardiogram revealed a mass (74×42 mm) in the right ventricle. Computed tomography showed a tumor lesion in the right ventricular cavity but no other distant metastasis. Coronary angiography revealed well-developed small branches to the tumor. After right heart catheterization, a pathological analysis of a tumor biopsy demonstrated adenocarcinoma. We diagnosed the patient with right ventricular metastasis of lung cancer. With large cardiac metastasis, a tumor biopsy with a right heart catheter may help obtain a pathological diagnosis and also serve as a re-biopsy to confirm the gene mutation status.

Acquired von Willebrand syndrome (AVWS) is a bleeding disorder caused by an acquired deficiency of von Willebrand factor (vWF). Some patients with AVWS show a low bleeding tendency and are diagnosed by the presence of a mild prolongation of activated partial thromboplastin time (APTT) preoperatively. Another cause of APTT prolongation is the presence of antiphospholipid antibody (aPL). We experienced a case of AVWS due to aortic valve stenosis in a patient with aPL in whom aortic valve replacement surgery was successful with vWF replacement. In patients with AVWS-associated disorders who are identified based on APTT prolongation at the preoperative examination, both vWF and aPL screening tests must be performed.

A 61-year-old woman who had smoked for 41 years developed subacute dizziness, ataxic gait, opsoclonus, and right visual impairment. She had right optic disc swelling and optic nerve gadolinium enhancement on magnetic resonance imaging. She had small-cell lung cancer (SCLC), with CV2/collapsin response mediator protein (CRMP) 5 and HuD antibodies in her serum and cerebrospinal fluid. She was diagnosed with paraneoplastic optic neuropathy (PON) accompanied by paraneoplastic opsoclonus-ataxia syndrome. Her symptoms improved after removing the SCLC. Classical PON is rare in Japan. We recommend assaying for CV2/CRMP5 antibodies and searching for cancer in elderly patients with subacute painless visual impairment.

A 58-year-old man consulted our hospital due to a 2-year history of dysarthria and a 1-month history of blepharospasm. In addition to the ataxic dysarthria and blepharospasm, a neurological examination demonstrated slight ataxia of the trunk and lower limbs. Brain MRI demonstrated atrophy of the upper portion of the cerebellar vermis. Gene analysis established a diagnosis of spinocerebellar ataxia type 31 (SCA31). Single photon emission computed tomography (SPECT) with the three-dimensional stereotaxic ROI template (3DSRT) software program demonstrated hyperperfusion in the lenticular nucleus and thalamus. Although the association between SCA31 and blepharospasm in our patient remains unclear, we considered that this combination might be more than coincidental.

Pregnancy in women with systemic lupus erythematosus (SLE)-associated pulmonary arterial hypertension (PAH) remains a high risk. We successfully managed a pregnancy in a patient with SLE-PAH. A 31-year-old pregnant woman with SLE-PAH had worsening PAH and SLE flare-up during pregnancy and a sudden increase in pulmonary arterial pressure after delivery. SLE-PAH was controlled by continuous intravenous epoprostenol and inhaled nitric oxide therapy combined with high-dose corticosteroids under close hemodynamic monitoring. Women with SLE-PAH should avoid pregnancy. However, in case of a similar event, we recommend our case as a good reference for improving the outcome of pregnancy with SLE-PAH.

We herein report a case of a 31-year-old Japanese man who simultaneously had a positive influenza A virus antigen test result and Vogt-Koyanagi-Harada disease (VKHD), demonstrated by both diffuse multiple early hyperfluorescent points on fluorescein fundus photography and serous retinal detachments on optical coherence tomography. He had meningitis. It was difficult to determine whether the main cause of meningitis was influenza A or VKHD. After initial treatment with peramivir for influenza A and then methylprednisolone pulse with subsequent corticosteroid therapy for VKHD, his symptoms improved gradually. These findings suggest that influenza A virus infection contributes to the onset or exacerbation of VKHD.