Tumor necrosis factor receptor-associated factor 3 (TRAF3) is an anti-inflammatory molecule that negatively regulates the non-canonical nuclear factor-κB pathway. Although TRAF3 haploinsufficiency (TRAF3 HI) can influence innate and adaptive immune cells, its effect on inflammatory bowel disease (IBD) development remains unclear. Here, we report the first case of severe early-onset IBD with a novel TRAF3 variant leading to HI, successfully treated with ustekinumab. A 6-year-old girl with a recurrent parotitis, otitis media, tonsilitis, and atopic dermatitis developed IBD involving the stomach, small intestine, and colon. At diagnosis, the immunoglobulin (Ig)G and IgA levels were relatively high, and lymphocyte subsets showed increased counts of plasmablasts, class-switch recombination B cells, and circulating T-follicular helper cells. Treatment with azathioprine and infliximab failed to maintain remission marked by several relapses accompanied by erythema nodosum and arthritis; however, ustekinumab, an anti-interleukin (IL)-12/23p40 antibody, led to long-term clinical remission, normalizing the Ig level and reducing abnormal lymphocyte counts. Whole-exome sequencing revealed a novel heterozygous mutation in TRAF3 [p.(Pro487Leufs*8)], resulting in TRAF3 under-expression. Our case may highlight the contribution of TRAF3 HI to the development of IBD and provide insights into IBD pathophysiology, suggesting the involvement of the IL-12/23-T-follicular helper cell pathway affected by genetic mutations.

Objective: Environmental deprivation, a type of childhood maltreatment, has been reported to constrain the cognitive developmental processes such as associative learning and implicit learning, which may lead to functional and morphological changes in the ventral pallidum (VP) and pessimism, a well-known cognitive feature of major depression. We examined whether neonatal isolation (NI) could influence the incidence of learned helplessness (LH) in a rat model mimicking the pessimism, and the number of vesicular glutamate transporter 2 (VGLUT2)-expressing VP cells and Penk-expressing VP cells. Methods: The number of escape failures from foot-shocks in the LH test was measured to examine stress-induced depression-like behavior in rats. The number of VGLUT2-expressing VP cells and Penk-expressing VP cells was measured by immunohistochemistry. Results: In NI rats compared with Sham rats, the incidence of LH in adulthood was increased and VGLUT2-expressing VP cells but not Penk-expressing VP cells in adulthood were decreased. VGLUT2-expressing VP cells were decreased only in the LH group of NI rats and significantly correlated with the escape latency in the LH test. Conclusion These findings suggest that the aberrant VP neuronal activity due to environmental deprivation early in life leads to pessimistic associative and implicit learning. Modulating VP neuronal activity could be a novel therapeutic and preventive strategy for the patients with this specific pathophysiology.

Methods: This study included 42 patients with ASD (two men and 40 women; 112 segments; mean, 68.5±8.4 years; and mean follow-up, 31.6±17.0 months) who underwent LIF and posterior correction surgery. The concave slot of the LIF cage was filled with an autologous iliac crest bone graft (IBG), and the convex slot with a porous hydroxyapatite/collagen (HAp/Col) composite was soaked with bone marrow aspirate. Endplate injury, the gap between vertebral endplate and cage in the coronal or sagittal plane, and fusion status were evaluated using computed tomography multiplanar reconstruction at 12 months after surgery. Moreover, the associated risk factors for pseudoarthrosis were analyzed. Results: Fusion at LIF segments were observed in 71.4% segments at 12 months after surgery. Fusion on the concave slot (autologous IBG side), convex slot (porous HAp/Col composite side), and both concave and convex slots were observed in 66.1%, 37.5%, and 36.6% of patients, respectively. Moreover, pseudoarthrosis was observed in 28.6% at 12 months after surgery. Consequently, logistic regression analysis of the fusion at the LIF segment revealed that the gap between the LIF cage and endplate in the coronal plane (p=0.030; odds ratio, 0.183; 95% confidence interval, 0.030–0.183) was significantly associated with pseudoarthrosis at the LIF segments. Conclusions ASD surgery fusion rate using LIF cages was 71.4% at 12 months after surgery. The fusion rate was higher on the concave slot filled with autologous IBG than on the convex slot filled with a porous HAp/Col composite. The gap in the coronal plane was a risk factor for pseudoarthrosis at the LIF segment.

Methods: A total of 24 patients with postsurgical chronic pain and/or numbness Numeric Rating Scale (NRS) scores of ≥4 were enrolled. All patients underwent spine or spinal cord surgery at Keio University Hospital and received daily administration of DLX for more than 3 months. The mean postoperative period before the first administration of DLX was 35.5±57.0 months. DLX was administered for more than 3 months at a dose of 20, 40, or 60 mg/day, and the degree of pain and numbness was evaluated using the NRS before administration and 3 months after administration. Effectiveness was defined as more than a 2-point decrease in the NRS score following administration. Results: In terms of the type of symptoms, 15 patients experienced only numbness, eight experienced both pain and numbness, and one experienced only pain. Of the 24 patients, 19 achieved effective relief with DLX. DLX was effective for all patients with postsurgical chronic pain (n=9), and it reduced postsurgical chronic numbness in 18 of 23 patients. No significant difference was observed in background spinal disorders. DLX was not effective for five patients who complained only of postsurgical chronic numbness. Conclusions This study reports the effectiveness of DLX for postsurgical chronic neuropathic disorders. Although DLX reduced postsurgical chronic pain (efficacy rate=100%) and numbness (78.3%) in certain patients, further investigation is needed to determine its optimal use.

Objective：Patients with vertebral compression fracture first visit the acute care hospital for a diagnosis, but cannot be admitted and return home with a corset and analgesics. Because of severe lower back pain, they stay in bed for a significant period of time, and their skeletal muscles suffer from disuse atrophy. We aimed to actively admit these patients for recovery phase rehabilitation by setting up a hotline.Methods：The backgrounds of each case including the major laboratory findings were investigated for 1 year. Health professionals were able to use the hotline to request admission for the patients.Results：One hundred twenty-seven patients (38 males and 89 females, aged 84±7.5 years) were admitted. The percentages of phone calls from acute-care hospitals, clinics, and regional care managers were 46%, 31%, and 20%, respectively. With regard to degree of disability 36％ of the patients were at C1, 31% were at B2, and 20% were at C2 indicating that they needed almost complete assistance. Fifty one percent of the patients lived alone, and 20% were couples without assistance from others. Because they were elderly patients with many complications, more than six agents had been prescribed on average. However, osteoporosis medication had been prescribed to only 23%. The prognosis was favorable with ratio of returned home being 91%, even though the outcome of rehabilitation is limited by ageing and deteriorated cardiac and renal functions in these patients.Conclusion：Services to support these patients during the recovery phase of rehabilitation is crucial, in order to improve community-based health care.

Objective：Patients with vertebral compression fracture first visit the acute care hospital for a diagnosis, but cannot be admitted and return home with a corset and analgesics. Because of severe lower back pain, they stay in bed for a significant period of time, and their skeletal muscles suffer from disuse atrophy. We aimed to actively admit these patients for recovery phase rehabilitation by setting up a hotline.Methods：The backgrounds of each case including the major laboratory findings were investigated for 1 year. Health professionals were able to use the hotline to request admission for the patients.Results：One hundred twenty-seven patients (38 males and 89 females, aged 84±7.5 years) were admitted. The percentages of phone calls from acute-care hospitals, clinics, and regional care managers were 46%, 31%, and 20%, respectively. With regard to degree of disability 36％ of the patients were at C1, 31% were at B2, and 20% were at C2 indicating that they needed almost complete assistance. Fifty one percent of the patients lived alone, and 20% were couples without assistance from others. Because they were elderly patients with many complications, more than six agents had been prescribed on average. However, osteoporosis medication had been prescribed to only 23%. The prognosis was favorable with ratio of returned home being 91%, even though the outcome of rehabilitation is limited by ageing and deteriorated cardiac and renal functions in these patients,Conclusion：Services to support these patients during the recovery phase of rehabilitation is crucial, in order to improve community-based health care.

Methods: Eighty patients who underwent surgical resection at Keio University and Fujita Health University in Tokyo, Japan between 2003 and 2015 with more than 2 years of follow-up were enrolled. A good surgical result was defined as an improvement in the modified McCormick Scale score by one grade or more or having the same clinical grade as was observed preoperatively. Meanwhile, a poor result was defined as a reduction in the McCormick Scale score of one grade or more or remaining in grade IV or V at final follow-up. Univariate and multivariate logistic regression analyses of the following factors were performed in the two groups: sex, age, preoperative Visual Analog Scale (VAS), tumor location, the extent of tumor resection, hemosiderin caps, cavity length, and tumor length on magnetic resonance imaging. Results: At final follow-up, 15 patients were included in the poor results group and 65 in the good results group. In the univariate analysis, the factors related to poor results were as follows: higher age, preoperative McCormick Scale score severity, higher preoperative VAS, thoracic location, hemosiderin capped, and non-gross total resection (GTR). A multiple logistic regression analysis was conducted and showed that age, worse preoperative McCormick Scale score, and non-GTR were significant factors for poor prognosis. Conclusions The independent risk factors for motor deterioration after ependymoma resection were age, worse preoperative McCormick Scale score, and non-GTR. Early surgery for patients with even mild neurological disorders could facilitate functional outcomes. These results may contribute to determining the optimal timing of surgery for spinal intramedullary ependymoma.

In this report, we describe a case involving a 34-year-old woman who showed good treatment outcomes with long-term stability after multidisciplinary treatment for unilateral cleft lip and palate (CLP), maxillary hypoplasia, severe maxillary arch constriction, severe occlusal collapse, and gingival recession. A comprehensive treatment approach was developed with maximum consideration of strong scar constriction and gingival recession; it included minimum maxillary arch expansion, maxillary advancement by distraction osteogenesis using an internal distraction device, and mandibular setback using sagittal split ramus osteotomy. Her post-treatment records demonstrated a balanced facial profile and occlusion with improved facial symmetry. The patient's profile was dramatically improved, with reduced upper lip retrusion and lower lip protrusion as a result of the maxillary advancement and mandibular setback, respectively. Although gingival recession showed a slight increase, tooth mobility was within the normal physiological range. No tooth hyperesthesia was observed after treatment. There was negligible osseous relapse, and the occlusion remained stable after 5 years of post-treatment retention. Our findings suggest that such multidisciplinary approaches for the treatment of CLP with gingival recession and occlusal collapse help in improving occlusion and facial esthetics without the need for prostheses such as dental implants or bridges; in addition, the results show long-term post-treatment stability.
Adult ; Cicatrix ; Cleft Lip* ; Constriction ; Dental Implants ; Esthetics ; Female ; Gingival Recession* ; Humans ; Hyperesthesia ; Lip ; Osteogenesis, Distraction* ; Osteotomy, Sagittal Split Ramus* ; Palatal Expansion Technique ; Palate* ; Prostheses and Implants ; Recurrence ; Tooth ; Tooth Mobility

No abstract available.
Aorta, Thoracic ; Transcatheter Aortic Valve Replacement