A Logistic Regression Model for Predicting the Risk of Subsequent Surgery among Patients with Newly Diagnosed Crohn's Disease Using a Brute Force Method.

Surgery avoidance is an important goal in Crohn's disease (CD) treatment and predicting the risk of subsequent surgery is important to determine adequate therapeutic strength for patients with newly diagnosed CD. Herein, we aimed to construct a prediction model for the risk of subsequent surgery based on disease characteristics at the patients' initial visit. We retrospectively collected disease characteristic data from 93 patients with newly diagnosed CD. A logistic regression model with a brute force method was used to maximize the area under the receiver operating characteristic curve (auROC) by employing a combination of potential predictors from 14 covariates (16,383). The auROC remained almost constant when one to 12 covariates were considered, reaching a peak of 0.89 at four covariates (small-bowel patency, extensive small-bowel lesions, main lesions, and the number of poor prognostic factors), and it decreased with increasing covariate size. The most significant predictors were small-bowel patency, extensive small-bowel lesions, and age or major lesions. Therefore, this prediction model using covariates may be helpful in determining the likelihood that a patient with newly diagnosed CD will require surgery, which can aid in appropriate treatment selection for high-risk patients.

Higher abundance of Campylobacter in the oral microbiome of Japanese patients with moyamoya disease.

Genetic factors alone cannot explain the pathophysiology of moyamoya disease (MMD), and environmental factors such as an immune response are thought to be involved. Oral and gut microbiomes have attracted attention as environmental factors in the pathophysiology of some vascular and autoimmune diseases. However, the relationship between MMD and these microbiomes is yet to be thoroughly investigated. This prospective case-control study aimed to compare the microbiomes of Japanese patients with MMD with those of healthy individuals to identify the specific bacteria involved in MMD. Saliva and fecal samples were collected from 16 patients with MMD who had not undergone revascularization surgery. Fifteen healthy individuals were matched for age, sex, and body mass index. The microbiomes were determined using 16S rRNA sequencing and analyzed using QIIME2. Differentially abundant microbes were identified using LEfSE and ANCOM-BC. In the oral microbiome, the two analytical methods showed that Campylobacter was more abundant in patients with MMD than in healthy individuals. Differences in the gut microbiome were smaller than those in the oral microbiome. In conclusion, the oral microbiome profiles of patients with MMD significantly differ from those of healthy individuals. Campylobacter spp. could be a substantial environmental factor in the pathophysiology of MMD.

Real-world data of clazosentan in combination therapy for aneurysmal subarachnoid hemorrhage: a multicenter retrospective cohort study.

Aneurysmal subarachnoid hemorrhage (aSAH) may lead to cerebral vasospasm, significantly associated with morbidity and mortality. In double-blind, placebo-controlled phase 3 studies, clazosentan reduces cerebral vasospasm-related morbidity and all-cause mortality in patients with aSAH. There are no reports about the clinical efficacy of clazosentan combination therapy with some other drugs. Initially, we explored the efficacy of clazosentan combination therapy with cilostazol, statin, and antiepileptic drugs. Subsequently, we assessed the add-on effect of fasudil to clazosentan combination therapy for aSAH patients. This multicenter, retrospective, observational cohort study included Japanese patients with aSAH between June 2022 and March 2023. The primary outcome was the ordinal score on the modified Rankin Scale (mRS; range, 0-6, with elevated scores indicating greater disability) at discharge. Among the 47 cases (women 74.5%; age 64.4 ± 15.0 years) undergoing clazosentan combination therapy, 29 (61.7%) resulted in favorable outcomes. Overall, vasospasm occurred in 16 cases (34.0%), with four cases (8.5%) developing vasospasm-related delayed cerebral ischemia (DCI). Both hypotension and vasospasm-related DCI were related to unfavorable outcome at discharge. Fasudil were added in 18 (38.3%) cases. Despite adding fasudil to clazosentan combination therapy, the incidence of aSAH-related vasospasm did not decrease. Added-on fasudil to combination therapy related to pulmonary edema, vasospasm, and vasospasm-related DCI, and unfavorable outcomes. Clazosentan combination therapy could potentially result in favorable outcomes for aSAH patients to prevent post-aSAH vasospasm-related DCI. The add-on effect of fasudil to combination therapy did not demonstrate a significant impact in reducing aSAH-related vasospasm or improving outcomes at discharge.

Takayasu arteritis presenting with fever of unknown origin and bilateral carotid artery tenderness: A case report.

A 51-year-old otherwise healthy woman was referred to our hospital with a fever of unknown origin, liver dysfunction, and anemia. One month prior, she had persistent and spontaneous anterior neck pain, with no exacerbation during swallowing or neck movements. Physical examination revealed no pharyngeal or tonsillar abnormalities, heart murmur, arthritis, skin rash, or lymphadenopathy, except for mild bilateral common carotid artery tenderness at the level of the thyroid cartilage. Blood tests showed nonspecific chronic inflammatory findings, anemia, and liver damage, whereas blood cultures, viral antibodies, interferon-γ release assay, and antibodies specific for any collagen disease showed negative results. Echocardiography and computed tomography without contrast of the neck, chest, abdomen, and pelvis showed no apparent abnormalities. She was subsequently diagnosed with Takayasu arteritis using positron emission tomography. Identifying a characteristic history of bilateral carotid artery tenderness and subsequent positron emission tomography can be useful for diagnosing Takayasu arteritis.

Vitamin A Promotes the Fusion of Autophagolysosomes and Prevents Excessive Inflammasome Activation in Dextran Sulfate Sodium-Induced Colitis.

Vitamin A ensures intestinal homeostasis, impacting acquired immunity and epithelial barrier function; however, its role in innate immunity is mostly unknown. Here, we studied the impact of vitamin A in different dextran sulfate sodium (DSS)-induced colitis animal models. Interestingly, more severe DSS-induced colitis was observed in vitamin A-deficient (VAD) mice than in vitamin A-sufficient (VAS) mice; the same was observed in VAD severe combined immunodeficient mice lacking T/B cells. Remarkably, IL-1ß production, LC3B-II expression, and inflammasome activity in the lamina propria were significantly elevated in VAD mice. Electron microscopy revealed numerous swollen mitochondria with severely disrupted cristae. In vitro, non-canonical inflammasome signaling-induced pyroptosis, LC3B-II and p62 expression, and mitochondrial superoxide levels were increased in murine macrophages (RAW 264.7) pretreated with retinoic acid receptor antagonist (Ro41-5253). These findings suggest that vitamin A plays a crucial role in the efficient fusion of autophagosomes with lysosomes in colitis.

Moyamoya disease-specific extracellular vesicle-derived microRNAs in the cerebrospinal fluid revealed by comprehensive expression analysis through microRNA sequencing.

PURPOSE: To examine the specific changes that occur in the expression levels of extracellular vesicle-derived microRNAs (miRNAs) in intracranial cerebrospinal fluid (CSF) in moyamoya disease. METHODS: Patients with arteriosclerotic cerebral ischemia were used as controls to eliminate the effects of cerebral ischemia. Intracranial CSF was collected from moyamoya disease and control patients during bypass surgery. Extracellular vesicles (EVs) were extracted from the CSF. Comprehensive expression analysis of miRNAs extracted from EVs by next-generation sequencing (NGS) and validation by quantitative reverse transcription-polymerase chain reaction (qRT-PCR) was performed. RESULTS: Experiments were conducted on eight cases of moyamoya disease and four control cases. In the comprehensive miRNA expression analysis, 153 miRNAs were upregulated, and 98 miRNAs were downregulated in moyamoya disease compared to the control cases (q-value < 0.05 and |log2 fold change|> 1). qRT-PCR performed on the four most variable miRNAs (hsa-miR-421, hsa-miR-361-5p, hsa-miR-320a, and hsa-miR-29b-3p) associated with vascular lesions among the differentially expressed miRNAs gave the same results as miRNA sequencing. On gene ontology (GO) analysis for the target genes, cytoplasmic stress granule was the most significant GO term. CONCLUSIONS: This study is the first comprehensive expression analysis of EV-derived miRNAs in the CSF of moyamoya disease patients using NGS. The miRNAs identified here may be related to the etiology and pathophysiology of moyamoya disease.

Tacrolimus Up-regulates Expression of TGFß Receptor Type II via ERK, Providing Protection Against Intestinal Epithelial Injury.

BACKGROUND/AIM: Transforming growth factor ß (TGFß) signaling plays a key role in modulating intestinal epithelial cell (IEC) homeostasis. The present study aimed to investigate the direct effect of tacrolimus on TGFß signaling in IECs. MATERIALS AND METHODS: The protective effects of tacrolimus, with or without anti-TGFß antibody, in dextran sulfate sodium (DSS)-induced colitis were evaluated. RESULTS: Tacrolimus ameliorated IEC apoptosis-mediated mucosal destruction despite anti-TGFß treatment. TGFß receptor type II (TGFß-RII), phosphor-SMAD family members 2/3, and phosphor-extracellular signal-regulated kinase (ERK) expression in IECs was enhanced in tacrolimus-treated mice, and these positive effects were maintained despite anti-TGFß treatment. Moreover, tacrolimus induced TGFß-RII up-regulation through ERK activation. CONCLUSION: Our data indicate that tacrolimus directly activated TGFß-SMAD signaling via the ERK pathway in IECs, thereby providing protection against apoptosis-mediated intestinal epithelial injury.

Transcriptome-wide analysis of intracranial artery in patients with moyamoya disease showing upregulation of immune response, and downregulation of oxidative phosphorylation and DNA repair.

OBJECTIVE: Moyamoya disease (MMD) is a rare cerebrovascular disease characterized by progressive occlusion of the internal carotid artery and the secondary formation of collateral vessels. Patients with MMD have ischemic attacks or intracranial bleeding, but the disease pathophysiology remains unknown. In this study, the authors aimed to identify a gene expression profile specific to the intracranial artery in MMD. METHODS: This was a single-center, prospectively sampled, retrospective cohort study. Microsamples of the middle cerebral artery (MCA) were collected from patients with MMD (n = 11) and from control patients (n = 9). Using microarray techniques, transcriptome-wide analysis was performed. RESULTS: Comparison of MCA gene expression between patients with MMD and control patients detected 62 and 26 genes whose expression was significantly (p < 0.001 and fold change > 2) up- or downregulated, respectively, in the MCA of MMD. Gene set enrichment analysis of genes expressed in the MCA of patients with MMD revealed positive correlations with genes involved in antigen processing and presentation, the dendritic cell pathway, cytokine pathway, and interleukin-12 pathway, and negative correlations with genes involved in oxidative phosphorylation and DNA repair. Microarray analysis was validated by quantitative polymerase chain reaction. CONCLUSIONS: Transcriptome-wide analysis showed upregulation of genes for immune responses and downregulation of genes for DNA repair and oxidative phosphorylation within the intracranial artery of patients with MMD. These findings may represent clues to the pathophysiology of MMD.

Effective Endoscopic Closure of Cholecysto-duodenal and Transverse Colon Fistulas Due to Squamous Cell Carcinoma of the Gallbladder Using Polyglycolic Acid Sheets and a Covered Metal Stent.

An 81-year-old woman presented with abdominal distension and right hypochondrial pain. Abdominal contrast computed tomography and magnetic resonance imaging revealed an 11-cm gallbladder tumor. The patient was diagnosed with squamous cell carcinoma of the gallbladder by endoscopic ultrasound-guided fine-needle aspiration from the gastric antrum. Thereafter, the gallbladder tumor enlarged, and cholecysto-duodenal and transverse colon fistulas were formed. A covered metal stent was placed on the transverse colon, and polyglycolic acid sheets were injected into the duodenum to close the fistulas endoscopically. Endoscopic closure is less invasive than surgery and considered effective for patients with poor general health conditions.

Cyclosporine protects from intestinal epithelial injury by modulating butyrate uptake via upregulation of membrane monocarboxylate transporter 1 levels.

BACKGROUND AND AIMS: A relationship between treatment outcomes and intestinal microbiota in patients with inflammatory bowel diseases has been demonstrated. Cyclosporine treatment leads to rapid improvement in severe ulcerative colitis. We hypothesized that the potent effects of cyclosporine would be exerted through relationships between intestinal epithelial cells (IECs) and the host microbiota. The present study was designed to elucidate the effects of cyclosporine on monocarboxylate transporter 1 (MCT1) regulation and butyrate uptake by IECs. METHODS: Colitis was induced in C57BL6 mice via the administration of 4% dextran sulfate sodium in drinking water, following which body weights, colon lengths, and histological scores were evaluated. To examine the role of butyrate in the protective effects of cyclosporine, MCT1 inhibitor and an antibiotic cocktail was administered and tributyrin (TB; a prodrug of butyrate) was supplemented; MCT1 protein expression and acetylated histone 3 (AcH3) signals in IECs, as well as the MCT1-membrane fraction of Caco-2 cells, were evaluated. To explore butyrate uptake, as s butyrate derivatives, 3-bromopyruvic acid (3-BrPA) and 1-pyrenebutyric acid were used. RESULTS: Treatment with cyclosporine inhibited body weight loss and colon length shortening. However, treatment with MCT1 inhibitor and the antibiotic cocktail negated the efficacy of cyclosporine, whereas TB supplementation restored its protective effect. Furthermore, cyclosporine upregulated MCT1 expression in the membrane and the AcH3 signal in IECs, while also inducing higher anti-inflammatory cytokine production compared to that in the vehicle-treated mice. The transcription level of MCT1 mRNA in IECs and Caco-2 cells did not increase with cyclosporine treatment; however, cyclosporine treatment increased membrane MCT1 expression in these cells and uptake of butyrate derivative. CONCLUSION: Cyclosporine treatment modulates butyrate uptake via the post-transcriptional upregulation of membrane MCT1 levels in IECs.

Diagnostic approach for patients with unidentified fever according to the classical criteria of fever of unknown origin in the field of autoimmune disorders.

Fever of unknown origin (FUO) is caused by various diseases, making differential diagnosis difficult. This study aimed to determine the clinical features of patients with FUO for use in daily medical practice. Medical records of patients who first visited our department for FUO between January 2008 and December 2017 were reviewed. We classified the diagnostic categories as infection, non-infectious inflammation, neoplasm, others, and unidentified through definitive diagnosis and compared the clinical characteristics of patients who fulfilled the criteria of classical FUO and those who did not. The most prevalent diseases in patients who fulfilled the criteria were adult-onset Still's disease, Behçet's disease (BD), and polymyalgia rheumatica, which do not have any specific image inspection or specific serological markers. BD and familial Mediterranean fever were most prevalent in patients who did not fulfill the criteria. All neoplasms fulfilled the criteria of classical FUO. The most useful diagnostic procedure was determined according to the criteria of each disease. The key factor that did not fulfill the criteria was periodic fever continuing for less than 3 weeks. When examining patients with FUO, we should strictly diagnose in accordance with the criteria of each disease and consider diseases that cause periodic fever.

Long-term maintenance of the mucosal healing induced by azacitidine therapy in a patient with intestinal Behçet's-like disease accompanied with myelodysplastic syndrome involving trisomy 8.

Myelodysplastic syndromes (MDSs) are a group of myeloid neoplasms characterized by blood cell deformation and dysfunction, and MDS with trisomy 8 is closely linked with intestinal Behçet's-like diseases. Intestinal Behçet's-like disease is refractory to conventional therapies, including prednisolone, immunomodulators, and anti-tumor necrosis factor α agents. Here, we describe a 56-year-old woman with intestinal Behçet's-like disease ascribed to MDS with trisomy 8 who had multiple intractable intestinal ulcers. She presented with periodic fever and abdominal pain. The genetic analysis showed a heterozygous E148Q mutation in the Mediterranean fever gene. The patient did not tolerate treatment with colchicine because of diarrhea; therefore, azacitidine therapy was initiated. One cycle of azacitidine therapy improved the multiple intestinal ulcers, and the periodic fever and abdominal pain gradually disappeared. After eight cycles of azacitidine therapy, ileocolonoscopy, histological assessment and capsule endoscopy revealed mucosal healing. Azacitidine therapy was continued, and mucosal healing was maintained for more than 2 years. This case suggests that azacitidine therapy which has immunoregulatory effects and epigenetic modulations, might control intestinal Behçet's-like disease associated with MDS involving trisomy 8.

A case of celiac disease with type I enteropathy-associated T-cell lymphoma in a Japanese male patient.

A 45-year-old Japanese male patient who was diagnosed with celiac disease (CeD) developed type I enteropathy-associated T-cell lymphoma (EATL). In 2013, the patient was admitted to our hospital with worsening of diarrhea and weight loss. Pathological examination of biopsy specimens from the duodenum and ileum led to a diagnosis of suspected EATL. A previous total colonoscopy (TCS) indicated villous atrophy in the terminal ileum. The patient was changed to a gluten-free diet, and the nutritional status gradually improved. In September 2014, he experienced acute right lower abdominal pain. He underwent urgent surgery, and a perforation was identified in the ileum. A diagnosis of type I EATL was made following histopathological examination. After eight courses of CHOP therapy, the patient entered complete remission. TCS and esophagogastroduodenoscopy with magnifying narrow-band imaging performed in 2015 identified villous regrowth in the distal ileum and duodenum. Capsule endoscopy also found villous regrowth in the entire small intestine. To our knowledge, this is the first case of type I EATL following CeD with villous atrophy before EATL occurrence in a Japanese HLA-DQ2 carrier. The possibility of type I EATL occurring after CeD should be recognized, although CeD is quite rare in Japan.

Successful adalimumab treatment and usefulness of capsule endoscopy for gut inflammation concomitant with ankylosing spondylitis.

Here we describe a 20-year-old man with ankylosing spondylitis and gut inflammation, who was successfully treated with adalimumab. Capsule endoscopy and ileocolonoscopy showed multiple erosions and aphthoid ulcers in the ileum and the ileocecal valve. Immunohistochemical analysis of the terminal ileum demonstrated that the number of IL-23p19 expressing macrophages was increased. Adalimumab was administered, and his back pain and abdominal symptoms improved. Adalimumab might be an effective treatment for gut inflammation related to ankylosing spondylitis.

Gastric Focal Neutrophil Infiltration and Wide Duodenal Gastric Foveolar Metaplasia Are Histologic Discriminative Markers for Crohn's Disease and Behçet's Disease.

BACKGROUND/AIMS: Behçet's disease (BD) with intestinal lesions and Crohn's disease (CD) share clinical features. However, no report has compared the 2 diseases with regard to lesions of the upper gastrointestinal tract (UGT). We aimed to evaluate endoscopic and histologic findings of UGT in CD and BD. METHODS: We retrospectively assessed the endoscopic records and biopsy samples of 84 Helicobacter pylori-negative patients (50 CD, 34 BD). In duodenal samples, MUC5AC immunohistochemical analysis was performed to identify gastric foveolar metaplasia. RESULTS: In endoscopic findings, bamboo joint-like appearance (17/50 CD, 0/34 BD) and erosions (14/50 CD, 2/34 BD) were significantly more frequent in CD gastric lesions (p < 0.001, and p = 0.012). In histologic findings of stomach, focal neutrophil infiltration in lamina propria (15/48 CD, 1/34 BD) was significantly more frequent in CD (p < 0.001). In that of duodenum, wide gastric foveolar metaplasia (19/49 CD, 1/34 BD) was significantly more frequent in CD duodenal lesions (p = 0.013). The mean maximum width of the gastric foveolar metaplasia was 114.0 ± 10.6 and 29.5 ± 4.5 µm for CD and BD respectively (p = 0.003). CONCLUSIONS: In H. pylori-negative patients, gastric focal neutrophil infiltration and wide duodenal gastric foveolar metaplasia were important for distinguishing CD from BD.

Prediction of Intracranial Arterial Stenosis Progression in Patients with Moyamoya Vasculopathy: Contrast-Enhanced High-Resolution Magnetic Resonance Vessel Wall Imaging.

OBJECTIVE: Moyamoya vasculopathy (MMV) is characterized by progressive stenosis of the intracranial arteries. MMV currently has no curative treatments, and cerebral ischemia and hemorrhage are the major outcomes. Evaluation of the stroke risk of each patient resulting from the progression of intracranial arterial stenosis is clinically important. METHODS: We prospectively reviewed patients with intracranial arterial stenosis and already diagnosed MMV. High-resolution magnetic resonance imaging using contrast agent is the novel vessel wall imaging (VWI) technique for directly evaluating vascular walls and intracranial artery disease. All patients underwent high-resolution vessel wall imaging and magnetic resonance angiography at the time of registration, and they underwent follow-up magnetic resonance angiography. The Fisher exact test was used to assess associations between the degrees of wall enhancement and between stable and progressive intracranial arterial stenosis. RESULTS: A total of 24 patients (17 female; mean age, 36.1 ± 16.8 years; range 3-67 years) with MMV were consecutively recruited to this study. Progression of stenosis was shown in 6 lesions (66.6%) on strong enhancement, 2 lesions (12.5%) on mild enhancement, and 1 lesion (4.3%) on lack of enhancement. Arterial vessel wall enhancement in MMV patients correlated closely with progression of intracranial arterial stenosis (P = 0.002). CONCLUSIONS: Arterial vessel wall enhancement in MMV patients was closely related to progression of intracranial arterial stenosis. Strong enhancement of the intracranial vessel wall was associated with intracranial arterial stenosis progression, and lack of enhancement correlated with the stability of intracranial arterial stenosis.

Intraoperative evaluation of local cerebral hemodynamic change by indocyanine green videoangiography: prediction of incidence and duration of postoperative transient neurological events in patients with moyamoya disease.

OBJECTIVE: Transient neurological events (TNEs) occur frequently in the acute phase after direct bypass surgery for moyamoya disease (MMD), but there is currently no way to predict them. FlowInsight is a specialized software for analyzing indocyanine green (ICG) videoangiography taken with a surgical microscope. The purpose of this study was to investigate whether intraoperative evaluation of local hemodynamic changes around anastomotic sites using FlowInsight could predict the incidence and duration of TNEs. METHODS: From patients who were diagnosed with MMD in our hospital between August 2014 and March 2017 and who underwent superficial temporal artery-middle cerebral artery bypass surgery, we investigated 25 hemispheres (in 22 patients) in which intraoperative ICG analysis was performed using FlowInsight. To evaluate the local cerebral hemodynamics before and after anastomosis, regions of interest were set at 3 locations on the brain surface around the anastomotic site, and the mean cerebral blood flow (CBF), mean gradation (Grad), mean transit time (MTT), and mean time to peak (TTP) were calculated from the 3 regions of interest. Furthermore, the change rate in CBF (ΔCBF [%]) was calculated using the formula (postanastomosis mean CBF - preanastomosis mean CBF)/preanastomosis mean CBF. ΔGrad (%), ΔMTT (%), and ΔTTP (%) were similarly calculated. RESULTS: Postoperative stroke without TNE occurred in 2 of the 25 hemispheres. These 2 hemispheres (in 2 patients) were excluded from the study, and data from the remaining 23 hemispheres (in 20 patients) were analyzed. For each parameter (ΔCBF, ΔGrad, ΔMTT, and ΔTTP) calculated by FlowInsight, the difference between the groups with and without TNEs was significant. The median values for ΔCBF and ΔGrad were significantly higher in the TNE group than in the no-TNE group (ΔCBF 30.13 vs 3.54, p = 0.0106; ΔGrad 62.05 vs 10.78, p = 0.00435), whereas the median values for ΔMTT and ΔTTP were significantly lower in the TNE group (ΔMTT -16.90 vs -7.393, p = 0.023; ΔTTP -29.07 vs -7.02, p = 0.00342). Comparison of the area under the curve (AUC) for each parameter showed that ΔTTP had the highest AUC and was the parameter with the highest diagnostic accuracy (AUC 0.857). The Youden index revealed that the optimal cutoff value of ΔTTP was -11.61 (sensitivity 77.8%, specificity 71.4%) as a predictor of TNEs. In addition, Spearman's rank correlation coefficients were calculated, and ΔCBF, ΔGrad, ΔMTT, and ΔTTP each showed a strong correlation with the duration of TNEs. The larger the change in each parameter, the longer the TNEs persisted. CONCLUSIONS: Intraoperative ICG videoangiography findings were correlated with the occurrence and duration of TNEs after direct bypass surgery for MMD. Screening for cases at high risk of TNEs can be achieved by ICG analysis using FlowInsight.

Postoperative Cerebral Infarction Risk Factors and Postoperative Management of Pediatric Patients with Moyamoya Disease.

OBJECTIVE: Although revascularization surgery for patients with moyamoya disease can effectively prevent ischemic events and thus improve the long-term clinical outcome, the incidence of postoperative ischemic complications affects patients' quality of life. This study aimed to clarify the risk factors associated with postoperative ischemic complications and to discuss the appropriate perioperative management. METHODS: Fifty-eight revascularization operations were performed in 37 children with moyamoya disease. Patients with moyamoya syndrome were excluded from this study. Magnetic resonance imaging was performed within 7 days after surgery. Postoperative cerebral infarction was defined as a diffusion-weighted imaging high-intensity lesion with or without symptoms. We usually use fentanyl and dexmedetomidine as postoperative analgesic and sedative drugs for patients with moyamoya disease. We used barbiturate coma therapy for pediatric patients with moyamoya disease who have all postoperative cerebral infarction risk factors. RESULTS: Postoperative ischemic complications were observed in 10.3% of the children with moyamoya disease (6 of 58). Preoperative cerebral infarctions (P = 0.0005), younger age (P = 0.038), higher Suzuki grade (P = 0.003), and posterior cerebral artery stenosis/occlusion (P = 0.003) were related to postoperative ischemic complications. Postoperative cerebral infarction occurred all pediatric patients using barbiturate coma therapy. CONCLUSIONS: The risk factors associated with postoperative ischemic complications for children with moyamoya disease are preoperative infarction, younger age, higher Suzuki grade, and posterior cerebral artery stenosis/occlusion. Barbiturate coma therapy for pediatric patients with moyamoya disease who have the previous risk factors is insufficient for prevention of postoperative cerebral infarction. More studies are needed to identify the appropriate perioperative management.

Successful treatment with tacrolimus of refractory pyoderma gangrenosum with pouchitis after restorative proctocolectomy for ulcerative colitis.

We describe herein a case of severe relapsed pyoderma gangrenosum (PG) concomitantly with severe pouchitis treated by tacrolimus. A 25-year-old woman had undergone proctocolectomy with construction of ileo-anal pouch surgery for refractory ulcerative colitis (UC). She first developed PG with refractory pouchitis, and infliximab (IFX) was administered to induce remission due to resistance to glucocorticoid therapy. After achieving remission, IFX was stopped. Five years later, severe skin ulcers concomitantly with severe pouchitis recurred and treatment with 30 mg oral prednisolone (PSL) combined with topical tacrolimus showed partial improvement. When PSL was tapered to 15 mg, the skin ulcers and diarrhea aggravated. Endoscopy revealed multiple ulcers in the ileal pouch. Treatment with oral tacrolimus was initiated for severe pouchitis and refractory PG. Forty days later, all skin ulcers became scars and multiple ulcers in the ileal pouch were also improved. Our case suggests that oral tacrolimus treatment could be a valuable treatment option for UC patients with refractory PG and pouchitis.

Prevention of the Rerupture of Collateral Artery Aneurysms on the Ventricular Wall by Early Surgical Revascularization in Moyamoya Disease: Report of Two Cases and Review of the Literature.

BACKGROUND: Collateral artery aneurysms are a source of intracranial hemorrhage in moyamoya disease. Several reports have shown that surgical revascularization leads to the obliteration of collateral artery aneurysms. However, its effect on the prevention of rebleeding has not been established, and the optimal timing of the operation remains unclear. The purpose of the present study is to evaluate the effects of surgical revascularization and to investigate the optimal operation timing in patients with moyamoya disease who have ruptured collateral artery aneurysms on the ventricular wall. CASE DESCRIPTION: Two patients with moyamoya disease who presented with intraventricular hemorrhage caused by rupture of collateral artery aneurysms on the wall of the lateral ventricle are presented here. In both cases, the aneurysms reruptured approximately 1 month after the initial hemorrhage. Both patients successfully underwent superficial temporal artery-middle cerebral artery anastomosis combined with indirect bypass in the subacute stage. The aneurysms decreased with the development of collateral circulation through the direct bypasses, and rebleeding did not occur after the surgery. CONCLUSIONS: Because ruptured collateral artery aneurysms on the wall of the lateral ventricle in moyamoya disease are prone to rerupture within 1 month, surgical revascularization may be recommended as soon as the patients are stable and able to withstand the operation.