Objective Rectal lymphoid follicular aphthous (LFA) lesions are related to ulcerative colitis (UC) and can be initial lesions of UC. We investigated the clinical course and prognosis of rectal LFA lesions.

Methods This is a retrospective analysis of the clinical records at a single center.

Patients Thirteen consecutive cases with LFA lesions treated at Hiroshima University Hospital between 1998 and 2015 were evaluated. Another 49 consecutive cases with ulcerative proctitis treated in the same period were enrolled as the control group. The clinical course and prognosis of both groups were evaluated.

Results The group with LFA lesions included 9 women and 4 men with a median age of 39.9 years (range, 21-70 years). A total of 11 cases progressed to typical UC at 5-51 months. Proximal extension of these typical UC lesions was observed in 7 (53.8%) cases, which was significantly higher than in the control group (10 cases, 20.8%). Three cases (5-year accumulation incidence rate, 27.3%) progressed to steroid-intractable UC, a significantly higher incidence than that of the control group (3 cases; 5-year accumulation incidence rate, 6.9%).

Conclusion Rectal LFA lesions frequently progress to typical UC with proximal extension, some of which become intractable to corticosteroid treatment.

Objective This historical control study was performed to evaluate i) the rebleeding rate of bleeding colon diverticula treated with endoscopic band ligation (EBL) versus endoscopic clipping (EC) and ii) risk factors for rebleeding of diverticula initially treated by endoscopic hemostasis.

Methods From January 2010 to December 2012, 68 patients were treated with EC, and from January 2013 to August 2016, 67 patients were treated with EBL. All patients in each group were followed up for one year to check for rebleeding.

Results The rebleeding rate was lower in the EBL group (7 of 67, 10%) than in the EC group (21 of 68, 31%; p<0.01). This difference was mainly due to the lower rebleeding rate from the same hemorrhagic diverticulum initially treated by hemostasis (EBL: 4 of 67, 6%; EC: 15 of 68, 22%; p<0.01). The time span until rebleeding in the EBL group was ≤1 week. A multivariate analysis indicated that bleeding from the diverticula on the right side of the colon was a high-risk factor for rebleeding from the diverticula (odds ratio, 4.48; 95% confidence interval, 1.22-16.46; p=0.02).

Conclusion The low rebleeding rate in the EBL group was attributed to the low degree of rebleeding from the same diverticulum, indicating that EBL was superior to EC in preventing rebleeding of an initially treated diverticulum.

Objective Tolvaptan is a class of diuretics that reduce body water through aquaresis. One of the most prominent characteristics of these agents is that worsening of the renal function is less likely to occur. We investigated the underlying mechanism concerning the change in the intracellular fluid (ICF) when the body fluid is reduced.

Methods In this retrospective observational study, five overhydrated chronic kidney disease (CKD) patients with edema or pleural effusion treated with tolvaptan were assessed by the bioelectrical impedance method twice: once before and once after tolvaptan therapy. The changes in the ICF rate were compared with those in 11 hemodialysis patients undergoing body fluid reduction by hemodialysis.

Results Removal of the body fluid either by tolvaptan or by hemodialysis increased the post/pre-ratio of ICW/total body water (TBW). Tolvaptan reduced the ICF more efficiently than hemodialysis.

Conclusion Tolvaptan treatment lessens body fluid by the efficient reduction of the ICF.

Objective The intrarenal renin-angiotensin system (RAS) is activated in clinical settings, such as chronic kidney disease (CKD), as well as in CKD animal models, and kidney transplant donors have a greater risk of end-stage renal disease than healthy controls. However, whether or not the intrarenal RAS is activated immediately after kidney donation in kidney transplant donors is unclear, and the mechanism underlying intrarenal RAS activation is unknown.

Methods We investigated 10 kidney transplant donors (4 men and 6 women, 58.6±9.0 years of age). Their blood pressure (BP), estimated glomerular filtration rate (eGFR), plasma angiotensinogen (AGT) and plasma angiotensin II (AngII) levels (which reflect circulating RAS activation), urinary albumin excretion, and urinary AGT excretion (which reflects intrarenal RAS activation) were evaluated before kidney donation (-1.2±0.40 days) and after kidney donation (7.5±1.7 days).

Results The renal function after kidney donation was significantly lower than before donation. There were no significant differences in the BP during 24-h ambulatory BP monitoring, plasma AngII levels, or urinary albumin excretion after kidney donation. In contrast, the levels of plasma AGT and urinary AGT excretion were significantly increased after kidney donation. The urinary AGT excretion after kidney donation did not show a significant relationship with the systolic BP, plasma AGT, plasma AngII, or urinary albumin excretion. In addition, the percentage change in urinary AGT excretion after kidney donation was not associated with the percentage change in other clinical parameters.

Conclusion The intrarenal RAS is activated in kidney transplant donors immediately after kidney donation, independent of the systemic BP and filtration of increased plasma AGT, due to augmented inflammation.

An 18-year-old man diagnosed with ileocolonic Crohn's disease with circumferential strictures of the ascending colon started treatment with mesalazine and subsequently underwent right hemicolectomy. After surgery, the patient was started on adalimumab, and the clinical course was favorable. Nine months postoperatively, colonoscopy revealed granular mucosa with circumferential and continuous involvement from the transverse colon down to the rectum, findings which resembled ulcerative colitis. Mesalazine allergy was suspected, and the inflammatory findings resolved after discontinuing mesalazine. In patients of inflammatory bowel disease receiving mesalazine with an atypical clinical course, the possibility of mesalazine allergy must be borne in mind.

The incidence of carcinoid tumor in the small intestine is increasing; however, its preoperative diagnosis is difficult. We recently experienced three cases of multiple carcinoid tumors in the small intestine successfully detected using capsule endoscopy (CE), followed by a pathological diagnosis using double-balloon enteroscopy (DBE). To diagnose multiple carcinoid in the small intestine appropriately, we reviewed the information of five cases reported to date along with our three recent cases. The literature review demonstrated that CE and DBE are useful for detecting and diagnosing small intestinal carcinoids and tumor multiplicity, which aids in determining the appropriate resection range.

A 70-year-old man was admitted for treatment of a single liver nodule that was detected by contrast-enhanced computed tomography. Twenty years earlier, the patient had been diagnosed with myelodysplastic syndrome-refractory anemia and secondary hemochromatosis but had not received erythrocyte transfusions. The current histological, computed tomography, and magnetic resonance imaging findings revealed hepatocellular carcinoma (HCC) and non-cirrhotic liver hemochromatosis. The liver tumor was treated using radiofrequency ablation therapy. Secondary hemochromatosis may be a risk factor for HCC, even if the liver is not cirrhotic. In such cases, additional surveillance may be required to detect the development of HCC.

Both autoimmune hepatitis (AIH) and eosinophilic fasciitis (EF) are known to be complicated by other autoimmune diseases. However, AIH complicated by EF has never been reported. We experienced a 58-year-old man with AIH complicated by EF. He was admitted to our hospital with acute hepatic injury and edema of the legs in April 201X. The etiologies of these symptoms were histologically proven as AIH and EF. The administration of prednisolone (PSL) drastically improved his liver injury and edema of the legs. When we make a diagnosis of AIH, we should carefully evaluate the physical findings, including the appearance of the legs, in order to detect other coexisting autoimmune diseases.

The effects of bariatric/metabolic surgery on glycemic control in obese type 1 diabetic patients are controversial. We herein report a case of a morbidly obese 35-year-old woman who completely recovered from slowly progressive type 1 diabetes (SPIDDM) following laparoscopic sleeve gastrectomy. Preoperatively, her body mass index (BMI) was 49.8 kg/m² and hemoglobin A1c was 5.7% with intensive insulin therapy. Six months after bariatric/metabolic surgery, her BMI decreased to 33.2 kg/m² and her glycemic control was normal despite the discontinuation of all diabetic medicine. This case demonstrates the usefulness of bariatric/metabolic surgery for achieving glycemic control in morbidly obese patients with SPIDDM in Japan.

A 61-year-old man was diagnosed with sarcoidosis involving the lungs, eyes, parotid gland and extrathoracic lymph nodes complicated by chronic kidney injury and hypercalcemia. Kidney biopsy showed non-specific interstitial nephritis and nephrosclerosis. However, immunohistochemical staining of cell surface markers revealed a multinucleated giant macrophage surrounded by T-cells, suggesting granulomatous interstitial nephritis. Corticosteroid improved the kidney function, and reduced the serum levels of calcium and angiotensin-converting enzyme. Sarcoid nephropathy may be caused by the combination of several sarcoidosis-associated pathophysiological conditions and a comprehensive kidney examination should be performed to assess the type of injury when determining a treatment strategy.

A 66-year-old man was transferred to our hospital for pneumonia that was resistant to sulbactam/ampicillin and levofloxacin therapy. Chest computed tomography showed the rapidly progressive formation of multiple cavities. Methicillin-resistant Staphylococcus aureus (MRSA) was isolated, and the patient was diagnosed with necrotizing pneumonia caused by community-acquired MRSA (CA-MRSA). The MRSA strain had type IV staphylococcus cassette chromosome mec and genes encoding Panton-Valentine leucocidin (PVL). CA-MRSA necrotizing pneumonia with the PVL gene is rare; only three cases have been previously reported in Japan. We administered anti-MRSA antibiotics and the patient achieved complete clinical and radiological improvement.

Nivolumab-induced multiple organ immune-related adverse events (irAEs) have been described in some case reports. The symptoms of endocrinological irAEs are especially nonspecific. A 63-year-old man with a postoperative recurrence of pulmonary adenocarcinoma who was treated with nivolumab presented fever, anorexia and fatigue after the 7th cycle. He underwent a rapid adrenocorticotrophic hormone test, four-hormone tolerance test and thyroid gland scintigraphy. The results were consistent with destructive thyroiditis, hypophysitis and secondary adrenal insufficiency. Nivolumab was restarted following glucocorticoid and thyroid hormone replacement treatment. When a patient presents nonspecific symptoms, the possibility of endocrinological irAEs should be considered as it may enable their early detection.

We herein report a 78-year old man with squamous cell carcinoma of the lungs treated with pembrolizumab. At 10 days after the administration of pembrolizumab, he showed progressive anemia and increased levels of bilirubin. Because the findings of a direct coombs test and cold hemagglutinin were positive, we diagnosed the patient with autoimmune hemolytic anemia and treated him with prednisolone. Subsequently, he was admitted to our hospital owing to fatigue, a high fever, and jaundice. His clinical findings met the diagnostic criteria of hemophagocytic lymphohistiocytosis, and he was rescued with a high dose of glucocorticoids. Marked tumor regression was obtained and has been maintained since then.

The central nervous system is a common site of relapse in patients receiving crizotinib, which is presumed to be associated with the low concentration of crizotinib in the cerebrospinal fluid (CSF). Our patient received surgical treatment for anaplastic lymphoma kinase-positive stage IIA lung adenocarcinoma. His cancer recurred with brain metastases and carcinomatous meningitis. We started whole-brain radiation therapy (WBRT) and subsequently administered crizotinib. The concentration of crizotinib on day 15 in the plasma was 158 ng/mL, and that in the spinal fluid was 4.32 ng/mL. WBRT may elevate the CSF/plasma crizotinib concentration ratio; clinicians may therefore consider performing WBRT prior to crizotinib initiation.

A 45-year-old woman was diagnosed with hepatosplenic T-cell lymphoma (HSTCL), a rare subtype of peripheral T-cell lymphoma. She received different types of chemotherapy, but disease progression was observed. To reduce the tumor burden before an unrelated bone marrow transplantation, combination chemotherapy consisting of the gemcitabine, carboplatin, and dexamethasone (GCD) was administered as bridging therapy, resulting in a reduction in the number of lymphoma cells. We were then able to perform bone marrow transplantation. Although she experienced some adverse events, she successfully achieved long-term remission. We herein report a successful case of HSTCL treated with unrelated stem cell transplantation following the GCD regimen as bridging chemotherapy.

We herein report the case of an 84-year-old woman with transthyretin (TTR) Val30Met-associated familial amyloid polyneuropathy (FAP-ATTR Val30Met), representing a very old case. The patient had muscle weakness and sensory disturbances in her extremities caused by severe peripheral neuropathy. She also had vitreous opacity and orthostatic hypotension, and pyrophosphate scintigraphy showed a myocardial accumulation. Esophagogastroduodenoscopy revealed mucosal amyloid deposits, positive in anti-TTR antibody staining. A TTR gene analysis isolated the Val30Met mutation. More than a few cases of FAP-ATTR develop late, like our own, and their familial histories are often obscure in non-endemic areas, which might make a diagnosis difficult.

SPG5 is a rare subtype of autosomal recessive hereditary spastic paraplegia caused by a homozygous mutation in the oxysterol 7α-hydroxylase gene, CYP7B1. We describe the first Japanese patient with SPG5 with a novel mutation in the CYP7B1 gene. On exome sequencing, we identified a homozygous frameshift mutation, c.741delA, p.K247fs, in exon 3 of the CYP7B1 gene. The patient showed spastic paraparesis with white matter hyperintensities in the bilateral corona radiata and periventricular and subcortical regions on brain magnetic resonance imaging. The present study expands the mutation spectrum of CYP7B1 and provides an opportunity to study the genotype-phenotype correlation in SPG5.

We report the case of a 46-year-old man with hemicrania continua presenting as exacerbations mimicking trigeminal neuralgia. The patient was tentatively diagnosed with trigeminal neuralgia, and treatment with various combinations of drugs was performed after the onset of pain. However, when the condition of the patient did not improve, we suspected hemicrania continua, and treatment with indomethacin was initiated. There was a marked alleviation of his pain within 24 hours. Thus, clinicians should be aware that the duration and frequency of exacerbations of hemicrania continua are variable.

We herein report a sporadic Creutzfeldt-Jakob disease (sCJD) patient followed from the presymptomatic phase to death. A 67-year-old woman had abnormal hyperintense cortical lesions on diffusion-weighted magnetic resonance imaging (MRI) one year before the onset. The levels of 14-3-3 protein and total tau protein, and findings from a real-time quaking-induced conversion test were normal at first but became abnormal after disease onset. Although there are four reports of presymptomatic sCJD identified by MRI, this is the first case report in which all three biomarkers had been assessed before and after the disease onset. MRI might be the most sensitive modality for detecting presymptomatic sCJD patients.

Systemic lupus erythematosus (SLE) may be associated with various types of malignancy. However, SLE occurring with ovarian cancer seems rare, and reliable therapeutic approaches for such cases have yet to be identified. We herein report a case of SLE with ovarian cancer that was successfully treated with corticosteroid, plasmapheresis and chemotherapy. This case may provide new insights into treatment approaches for SLE with ovarian cancer.

Anti-melanoma differentiation-associated gene 5 (MDA5) antibodies are frequently detected in amyopathic dermatomyositis with rapidly progressive interstitial lung disease (RP-ILD). However, the presence of anti-MDA5 antibodies in other connective tissue diseases is not well known. We herein report a case of rheumatoid arthritis complicated with refractory anti-MDA5 antibody-positive ILD. A 75-year-old Japanese woman was referred to our hospital for refractory ILD. Serological testing was positive for anti-MDA5 antibody without any muscle or skin lesions. Immunosuppressive therapy (prednisolone and tacrolimus) ameliorated her symptoms as well as ILD. Anti-MDA5 antibody-positive ILD, as well as dermatomyositis with RP-ILD, can occur in patients with rheumatoid arthritis.

The emergence of a Japan-intrinsic community associated methicillin-resistant Staphylococcus aureus strain (CA-MRSA/J) has been reported. A 70-year-old man with recurrent colon cancer and a history of mitral valve replacement was admitted to the hospital in a state of shock. He was diagnosed with prosthetic valve endocarditis (PVE) caused by MRSA and underwent cardiac surgery. The MRSA isolates belonged to multilocus sequence type 8 and carried staphylococcal cassette chromosome mec IVl and the genes of toxic shock syndrome toxin-1, enterotoxin C, and enterotoxin L. These characteristics indicated a CA-MRSA/J clone. This is the first reported case of PVE caused by CA-MRSA/J.

Exacerbation of liver enzymes after the initiation of feeding in malnourished patients is caused by refeeding syndrome or persistent starvation. There are no definite clinical markers for distinguishing between the two conditions. We herein report a 63-year-old woman with starvation-induced liver enzyme elevation. Her body weight was inversely associated with the liver enzyme levels after refeeding, which was a different course from refeeding syndrome. Normalization of liver enzymes ensued as the caloric intake increased and weight gain progressed. Daily changes in body weight can be a useful clinical marker for distinguishing between refeeding syndrome and starvation-induced liver enzyme elevation.