Analysis of autoimmune hepatitis with acute presentation in the early stage of illness.

Background And Aim: There is no gold standard for making the diagnosis of autoimmune hepatitis (AIH), and the diagnosis of acute onset AIH (A-AIH) is most challenging. A-AIH sometimes develops into acute liver failure with poor prognosis if the diagnosis is delayed. Therefore, it is most important for the better prognosis to diagnose non-severe A-AIH early and treat appropriately.

Surgical treatment in the chronic phase for uncomplicated Stanford type B aortic dissection.

Background: The most appropriate surgical method for patients with uncomplicated type B aortic dissection (UTBAD) in the chronic phase remains controversial. This study evaluated the outcomes of patients with UTBAD who needed aortic treatment as well as the impact of the treatment method or indication criteria on their prognosis.

Methods: This retrospective review of 106 consecutive patients with aortic events in the chronic phase who underwent initial treatment for UTBAD between 2004 and 2021 comprised three groups: 19 patients who underwent endovascular repair (TEVAR), 38 who underwent open aortic repair and the medication group that included 49 patients.

Immunogenicity and influence on disease activity of recombinant zoster vaccine in patients with rheumatoid arthritis treated with DMARDs.

Objectives: This study aimed to determine the immunogenicity and the influence on disease activity of an adjuvanted recombinant varicella-zoster virus (VZV) subunit vaccine (RZV) in patients with rheumatoid arthritis (RA) treated with disease-modifying antirheumatic drugs (DMARDs).

Methods: This prospective longitudinal study enrolled 53 patients with RA (aged ≥50 years) treated with DMARDs (conventional synthetic (cs)DMARDs 20, biological (b)DMARDs 23 and targeted synthetic (ts)DMARDs 10) and 10 control individuals. The participants received two intramuscular RZV 2 months apart.

A Case of Postthoracic Endovascular Aortic Repair Stent-Graft Infection Caused by .

An 81-year-old Japanese man who had distal aortic arch dilatation at age 77 had thoracic endovascular aortic surgery. The patient developed a fever and was taken to the hospital. Reduced diffusion in the descending aortic wall along the stent graft was discovered using whole-body diffusion-weighted imaging with background body signal suppression, and stent-graft infection was identified.

The long-term effect of biologics in patients with ulcerative colitis emerging from a large Japanese cohort.

To gain a better understanding of the effects of biologics, we evaluated clinical outcomes in patients with moderate to severe exacerbations of ulcerative colitis (UC). This retrospective, multicenter study retrieved the entire clinical courses of UC patients who began treatments between 2004 and 2018. All exacerbations and clinical parameters, including treatment details for exacerbations and both remission and re-exacerbation dates, were identified during the observation period.

Growth potential of small residual tumors after vestibular schwannoma surgery: comparison between remnants and the natural history of small tumors.

Objective: Due to the heterogeneous definitions of tumor regrowth and various tumor volume distributions, the nature of small remnants after vestibular schwannoma (VS) surgery and the appropriate timing of adjuvant stereotactic radiosurgery for these remnants remain unclear. In this study, the growth potential of small remnants (< 1 cm3) after VS surgery was compared with that of treatment-naïve (TN) small VSs.

Methods: This retrospective single-center study included 44 patients with VS remnants following subtotal resection (STR) of a large VS (remnant group) and 75 patients with TN VS (< 1 cm3; TN group).

Arachnoid cyst alone causes hemifacial spasm: illustrative case.

Background: Hemifacial spasm (HFS) due to an arachnoid cyst at the cerebellopontine angle is rare. Here, the authors reported such a case and analyzed the mechanism of facial nerve hyperactivity by reviewing the literature.

Observations: A 40-year-old man presented with right HFS for the past 3 years.

An aberrant venous channel mimicking the perilabyrinthine cells in the petrous bone of a patient with vestibular schwannoma: illustrative case.

Background: Anatomical variations, such as high jugular bulbs and air cell development in the petrosal bone, should be evaluated before surgery. Most bone defects in the internal auditory canal (IAC) posterior wall are observed in the perilabyrinthine cells. An aberrant vascular structure passing through the petrous bone is rare.

Analysis of non-severe acute onset autoimmune hepatitis according to the presence of radiological heterogeneity.

Aim: Diagnosis of acute onset autoimmune hepatitis (A-AIH) has been difficult in that patients may not have typical clinicopathological features of AIH. In our previous reports of severe and fulminant AIH, two-thirds of them showed radiological heterogeneity: hepatic heterogeneous hypoattenuation on unenhanced computed tomography (CT) reflecting heterogeneous distribution of massive hepatic necrosis (severe centrilobular necrosis), which would be beneficial for the diagnosis. In the present study, we analyzed non-severe A-AIH patients with or without radiological heterogeneity and tried to find novel clinical features for supporting the early diagnosis.

False lumen being larger than true lumen is associated with late aortic events in uncomplicated type B aortic dissection.

Objectives: In uncomplicated type B aortic dissection, a large false lumen (FL) is reportedly a risk factor for late aortic events. However, it is unclear how the relationship between the false and true lumen (TL) diameters affects the dissected aorta. This study aimed to evaluate the impact on clinical outcomes of the FL being larger than the TL.

Immunogenicity and safety of routine 13-valent pneumococcal conjugate vaccination outside recommended age range in patients with hematological malignancies and solid tumors.

Hematological malignancy and solid tumor are major risks for invasive pneumococcal disease. Thirteen-valent pneumococcal conjugate vaccine (PCV13) is recommended for immunocompromised patients aged 6 years and older and adults who had not received the vaccine previously. However, vaccination for these individuals is not publicly subsidized in Japan.

Tubulointerstitial nephritis and uveitis syndrome following meningitis and systemic lymphadenopathy with persistent Toxoplasma immunoglobulin M: a case report.

Background: Tubulointerstitial nephritis and uveitis syndrome is a rare lymphocyte-related oculorenal inflammatory disease presumed to be associated with drug use and infectious agents. Toxoplasma gondii is one of such pathogens that could exhibit encephalitis, meningitis, and uveitis in immunocompromised or in some immunocompetent individuals. If the immunoglobulin M of Toxoplasma is positive on screening, the interpretation of the result is not simple, especially when immunoglobulin M stays positive persistently.

Pneumococcal serotype-specific IgG and opsonophagocytic activity in young Japanese patients with asplenia.

Patients with asplenia are at high risks of severe infections caused by encapsulated bacteria, particularly . Thirteen-valent pneumococcal conjugate vaccine (PCV13) and 23-valent pneumococcal polysaccharide vaccine (PPSV23) are recommended for invasive pneumococcal disease prevention; however, little is known about the immunity to pneumococci in young patients with asplenia. We measured pneumococcal serotype-specific IgG (Pn-IgG) levels and pneumococcal opsonophagocytic activity (Pn-OPA) against some PCV13-contained serotypes (1, 3, 5, 6A, 7 F, 19A) in 23 young patients with asplenia using surplus serum samples.

Paediatric primary cough headache with internal jugular phlebectasia.

Primary cough headaches (PCHs) are mainly observed in people aged >40 years, but cough-induced headaches are potentially symptomatic in children. We report a case of a child diagnosed with PCH without an intracranial disease. A 7-year-old boy presented with cough due to pertussis and powerful cough-induced headaches.

Isolated Hypoglossal Nerve Palsy Due to an Osteophyte with Atlantoaxial Dislocation.

Isolated hypoglossal nerve palsy (IHP), or hypoglossal nerve palsy without any other neurological signs, is rare. We report a woman with atlantoaxial dislocation (AAD) who presented with IHP due to hypoglossal nerve compression by an osteophyte at the hypoglossal canal. A 77-year-old woman presented with speech difficulties and the feeling of a swollen tongue on the left side for 3 days.

Detection of the intimal tear in aortic dissection and ulcer-like projection in intramural hematoma: usefulness of full-phase retrospective ECG-gated CT angiography.

Purpose: To compare the accuracy of non-electrocardiogram (ECG)-gated CT angiography (CTA), single-diastolic-phase ECG-gated CTA, and full-phase ECG-gated CTA in detecting the intimal tear (IT) in aortic dissection (AD) and ulcer-like projection (ULP) in intramural hematoma (IMH).

Materials And Methods: A total of 81 consecutive patients with AD and IMH of the thoracic aorta were included in this single-center retrospective study. Non-ECG-gated CTA, single-diastolic-phase ECG-gated CTA, and full-phase ECG-gated CTA were used to detect the presence of the IT and ULP in thoracic aortic regions including the ascending aorta, aortic arch, and proximal and distal descending aorta.

Risk score system for late aortic events in patients with uncomplicated type B aortic dissection.

Objective: Several risk factors for late aortic events in patients with uncomplicated type B aortic dissection (UTBAD) have been reported; however, they remain controversial. We developed and validated a new risk prediction score system for late aortic events in patients with UTBAD.

Methods: We reviewed 187 consecutive patients diagnosed with UTBAD from 2004 to 2017 at 2 centers (derivation cohort) and 219 consecutive patients diagnosed with UTBAD from 2012 to 2016 in 4 other centers (validation cohort).

Long-term observation of acute-onset autoimmune hepatitis presenting clinically and radiologically as acute hepatitis.

Background: There is yet no gold standard for the diagnosis of acute-onset autoimmune hepatitis (A-AIH), especially histologically acute AIH. As a result, long-term observation of A-AIH has been difficult and the nature is not well known. We retrospectively analyzed the clinicopathological features of A-AIH over a long prospective follow-up period.

Impact of initial aortic diameter and false-lumen area ratio on Type B aortic dissection prognosis.

Objectives: Medical treatment is the gold standard for uncomplicated acute Type B aortic dissection (ATBAD). Although endovascular treatment could become an alternative therapy, it is unclear which ATBAD patients should undergo endovascular intervention. We aimed to evaluate the outcomes of patients with uncomplicated ATBAD and identify the risk factors for major adverse events.

Intrathecal Noggin administration in rats temporally ameliorates mechanical allodynia induced by a chronic constriction injury.

Chronic intractable neuropathic pain after central or peripheral nervous system injury remains refractory to therapeutic intervention. Using microarray and RT-qPCR methods, we found that Noggin mRNA is downregulated in the lumbar enlargement 2 weeks after chronic constriction injury (CCI) in rats. Eight-week-old female Sprague Dawley rats were used for the CCI model.

Analysis of infectious complications and timing for emergency liver transplantation in autoimmune acute liver failure.

Background: Autoimmune hepatitis (AIH) is one of major etiologies of acute liver failure (ALF), and the survival rate without liver transplantation (LT) of patients with fulminant AIH is especially poor worldwide. We investigated the clinicopathological features of infectious complications in autoimmune ALF retrospectively and tried to determine when to continue corticosteroid (CS) treatment or abandon it for LT.

Methods: Twenty patients with autoimmune ALF, comprising five severe hepatitis, 13 fulminant hepatitis and two late onset hepatic failure, were analyzed.

High level of persistent liver injury is one of clinical characteristics in treatment-naïve acute onset autoimmune hepatitis: experience in a community hospital.

Background: There is, as yet, no gold standard for making the diagnosis of acute onset autoimmune hepatitis (A-AIH). Novel histological characteristics have been reported, but etiologies other than AIH could show similar histological pattern. We attempted to determine what clinical characteristics we should consider as A-AIH different from other etiologies, and to whom histological characteristics should be applied for the diagnosis.

Severe and fulminant hepatitis of indeterminate etiology in a Japanese center.

Aim: The outcome of acute liver failure (ALF) is influenced by its etiology, making etiological consideration of ALF important. However, specific etiology could not be identified in 30-40% of adult patients in a Japanese nationwide survey. We examined our patients with severe (SH) and fulminant hepatitis (FH) of indeterminate etiology for the better understanding of ALF.

[Long-term survival in a case of recurrent gastrointestinal stromal tumor treated with intermittent or low-dose imatinib].

A 65-year-old woman with a gastrointestinal stromal tumor(GIST)underwent a total gastrectomy in 1999. In 2004, she was diagnosed with an intra-abdominal recurrence and was treated with 300mg/day of imatinib. Because of the side effects of imatinib, we interrupted the treatment and were forced to reduce the dose from 300mg/day to 100mg/day.