[A case of primary central nervous system post-transplant lymphoproliferative disease 14 years after living donor liver transplantation].

The patient was a 51-year-old man who had undergone living donor liver transplantation for type B cirrhosis at the age of 37 years, and had a history of immunosuppressive drug use. He had developed focal seizures starting from his right upper limb, and MRI showed a lesion in the subcortical white matter of his left parietal lobe. Sensory disturbance and paralysis progressed in his right upper and lower limbs, and his brain lesion rapidly enlarged. A brain biopsy revealed diffuse large B-cell lymphoma, and Epstein-Barr virus-encoded small RNA in situ hybridization was positive. The patient was diagnosed with primary central nervous system post-transplant lymphoproliferative disorder (PCNS-PTLD) with no lesions in other organs. There are few reports of PCNS-PTLD cases after living donor liver transplantation in Japan. Although rare, it is nevertheless important to consider this disease in patients receiving immunosuppressive drugs after organ transplantation who develop brain lesions, regardless of which organ was transplanted.

Buckwheat Allergy in Asia.

PURPOSE OF REVIEW: Buckwheat (BW) allergy is a significant issue in Asia. This review delves into three types of BW allergy: immediate food allergy; food-dependent, exercise-induced anaphylaxis (FDEIA) as a subset of immediate food allergy; and food protein-induced enterocolitis syndrome (FPIES); by comparing data from Asian and non-Asian countries. RECENT FINDINGS: Most studies on BW have been published in Japan and Korea, and only a few studies on the topic have been done outside Asia. To date, seven components of common BW (Fagopyrum esculentum) and four components of Tartary BW (Fagopyrum tartaricum) have been implicated in BW allergy. Although BW-sIgE has limited utility for evaluating immediate BW allergy, Fag e 3-specific IgE, one of the components of common BW, and the skin prick test are diagnostically useful. The present review aims to shed light on the current state of knowledge, highlight research gaps, and suggest future directions in the management and understanding of BW allergy.

Intrathoracic Needle Migration from the Mediastinum into the Thoracic Cavity.

Intrathoracic needles are rarely used in clinical practice. They can migrate within the body, injure large blood vessels and other organs, and cause severe complications. We report an interesting case of intrathoracic needle removal using video-assisted thoracoscopic surgery. The needle was inserted under the left clavicle, penetrated the mediastinum, and migrated into the right thoracic cavity. Although pneumothorax developed during the disease course, no severe complications were observed. This rare case illustrates the course of needle migration from the mediastinum into the thoracic cavity. Prompt imaging and surgical removal of foreign bodies are necessary in cases of intrathoracic foreign bodies.

Prognostic significance of ground-glass areas within tumours in non-small-cell lung cancer.

OBJECTIVES: To validate or refute the hypothesis that non-small-cell lung cancers (NSCLC) with ground-glass areas (GGA+) within the tumour on high-resolution computed tomography are associated with a more favourable prognosis than those without GGA (GGA-). METHODS: We analysed data from a multicentre observational cohort study in Japan including 5005 patients with completely resected pathological stage I NSCLC, who were excluded from the Japan Clinical Oncology Group (JCOG) 0707 trial on oral adjuvant treatment during the enrolment period. The patients' medical and pathological records were assessed retrospectively by physicians and re-staged according to the 8th tumour, node, metastasis edition. RESULTS: Of the 5005 patients, 2388 (48%) were ineligible for the JCOG0707 trial and 2617 (52%) were eligible but were not enrolled. A total of 958 patients (19.1%) died. Patients with GGA+ NSCLC and pathological invasion ≤3 cm showed significantly better overall survival than others. In patients with tumours with an invasive portion ≤4 cm, GGA+ was associated with better survival. The prognoses of patients with GGA+ T2a and GGA- T1c tumours were similar (5-year overall survival: 84.6% vs 83.1%, respectively). The survival with T2b or more tumours appeared unaffected by GGA, and GGA was not prognostic in these larger tumours. CONCLUSIONS: Patients with GGA+ NSCLC on high-resolution computed tomography and ≤4 cm invasion size may have a better prognosis than patients with solid GGA- tumours of the same T-stage. However, the presence or absence of radiological GGA has little impact on the prognosis of patients with NSCLC with greater (>4 cm) pathological invasion.

Differences in Characteristics Between Patients Who Met or Partly Met the Diagnostic Criteria for Food Protein-Induced Enterocolitis Syndrome (FPIES).

BACKGROUND: Some patients with food protein-induced enterocolitis (FPIES)-like allergy do not completely fulfill the diagnostic criteria of the international consensus guideline for FPIES. However, it is unclear whether such FPIES-like patients represent a completely different population from FPIES. OBJECTIVE: This study aimed to clarify differences in characteristics between patients with FPIES who fully met diagnostic criteria and those who partly met them. METHODS: This was a cross-sectional study using data at the time of registration in multicenter, prospective studies of patients with FPIES in Japan. Children who had delayed emesis within 1 to 4 hours and/or diarrhea within 5 to 10 hours after ingestion of food were recruited between March 2020 and February 2022. We examined their compatibility with the diagnostic criteria of the international consensus guideline and their detailed clinical characteristics, including trigger foods, the serving size that elicited symptoms, and antigen-specific IgE antibody titers. RESULTS: Of the 225 patients with FPIES, 140 fully met the diagnostic criteria whereas 79 patients did not fully meet them but demonstrated reproducible symptoms. The frequencies of pallor, lethargy, and diarrhea were significantly higher in those who met the criteria fully, whereas the age at onset, trigger foods, comorbidity, and perinatal information were comparable. Analysis of patients with FPIES to hen's egg revealed significantly higher levels of egg white- and egg yolk-specific IgE in patients who partly met criteria, whereas the serving size eliciting symptoms was comparable. CONCLUSIONS: Patients who partly met the diagnostic criteria may have a milder phenotype of FPIES, but this needs to be validated in further studies using biomarkers reflecting the pathophysiology.

Lymphocyte stimulation test for diagnosing hen's egg yolk-induced enterocolitis syndrome.

Background: There is currently little research into factors predicting the results of an initial diagnostic oral food challenge (OFC) test for food protein-induced enterocolitis syndrome (FPIES). Objective: The present study aimed to identify predictors of the diagnosis of hen's egg yolk-induced FPIES (HEY-FPIES). Methods: The present monocentric study was performed at Tokyo Metropolitan Children's Medical Center and included patients who underwent hen's egg yolk OFC (HEY-OFC) between March 2018 and March 2023 to assess for HEY-FPIES. The baseline characteristics of the groups and HEY-OFC positivity or negativity were then compared. Univariate analysis was conducted by using the Mann-Whitney U test or Fisher exact test. Receiver operator characteristic analysis was used to create probability curves. Results: In total, 35 patients were analyzed; of these, 17 were HEY-OFC-positive. No significant difference was observed between the HEY-OFC-positive and HEY-OFC-negative groups in terms of background factors except for the HEY-LST value, which was significantly higher in the HEY-LST group (P = .027). Receiver operator characteristic analysis demonstrated that the area under the curve for HEY-OFC positivity using the HEY-LST value was 0.719 (95% CI = 0.541-0.897). The statistically optimal cutoff value for the HEY-LST was 610%, which had a clinical sensitivity and specificity of 64.7% and 83.3%, respectively. Conclusions: The present study demonstrated that the HEY-LST may be a useful predictor of the result of an initial OFC for HEY-FPIES.

Treatment of new-onset primary central nervous system lymphoma in elderly patients using RMPV chemotherapy: a single-institution experience.

The prognosis of primary central nervous system lymphoma (PCNSL) in the elderly remains poor. We aimed to evaluate the outcome of rituximab, methotrexate, procarbazine, and vincristine (RMPV) chemotherapy in elderly patients with new-onset PCNSL. Twenty-eight patients aged ≥ 70 years treated for PCNSL between 2010 and 2020 were examined retrospectively. Nineteen patients received RMPV and nine did not qualify. Patients received five to seven cycles of RMPV plus response-adapted whole-brain radiotherapy (WBRT) and cytarabine. Ten of the 19 patients who received RMPV (52.6%) completed the induction, but only four patients (21.1%) completed RMPV chemotherapy, WBRT 23.4 Gy, and cytarabine. Median progression-free survival (PFS) and overall survival (OS) in the RMPV group was 54.4 and 85.0 months, respectively. Both PFS and OS were significantly longer in patients who received RMPV chemotherapy than in those who did not, and in patients who started but did not complete RMPV than in those who did not receive RMPV. Patients who received incomplete RMPV tended to have a favorable prognosis. Initial treatment with RMPV chemotherapy was effective in elderly patients with PCNSL. Adjusting the number of courses of RMPV may improve the prognosis of elderly patients with PCNSL, but further verification is necessary.

Assessing effects of mechanical stimulation of fluid shear stress on inducing matrix Metalloproteinase-9 in cultured corneal epithelial cells.

Blinking is regarded as mechanical stimulation of fluid shear stress on the corneal epithelial cells. Therefore, we evaluated whether fluid shear stress affects matrix metalloproteinases (MMPs) and tissue inhibitors of metalloproteinases (TIMPs) in cultured human corneal epithelial cells (HCECs). No other study has shown the influence of fluid shear stress on HCECs regarding mRNA expression and the protein levels of MMPs. Cultured HCECs were exposed to shear stress (0, 1.2, 12 dyne/cm2) for 12 and 24 h with the parallel-plate type of flow chamber. Gene expression of MMPs and TIMPs was measured by real-time polymerase reaction. Concentrations of MMP-1 and MMP-9 in cell lysates were determined using bead-based amplified luminescent proximity homogenous assay-linked immunosorbent assay. The expression of MMP-9 and MMP-1 in HCECs exposed to low and high flow for 12 and 24 h, respectively, increased significantly compared with those under static conditions. The expression of MMP-9 in the cells exposed to high flow for 24 h increased significantly compared with those under static and low flow conditions. Levels of MMP-9 in cell lysates exposed to fluid flow for 24 h were elevated significantly with increasing shear stress. Fluid shear stress exerted on HCECs affected MMPs, which was associated with inflammation and pathogenesis. Mechanical stress induced by blinking might influence expression of MMPs on the ocular surface. Further studies are warranted to establish the molecular mechanism of shear stress-induced alternations of MMPs.

Prevalence of Allergic Diseases across All Ages in Japan: A Nationwide Cross-Sectional Study Employing Designated Allergic Disease Medical Hospital Network.

Introduction: Allergic diseases affect both children and adults, but generation-specific prevalence rates are unclear. Methods: An online questionnaire was used from December 2021 to January 2022 to survey the prevalence of allergic diseases among staff and their families of designated allergic disease medical hospitals in Japan. In this study, bronchial asthma (BA), atopic dermatitis (AD), food allergies (FAs), allergic rhinitis (AR), allergic conjunctivitis (AC), metal allergies (MAs), and drug allergies (DAs) were the allergic diseases surveyed. Results: In total, 18,706 individuals were surveyed (median age, 36 years; quartile range, 18-50). Allergic disease was reported in 62.2% of respondents. Across all ages, prevalence rates were as follows: BA (14.7%), AD (15.6%), FAs (15.2%), AR (47.4%), AC (19.5%), MAs (1.9%), and DAs (4.6%). The prevalence of BA and AR was higher in male children, whereas that of FAs and AC was higher in adult females. The prevalence of MAs and DAs peaked during adulthood and predominated among females. Conclusions: Our results suggest that approximately two-thirds of the Japanese population may have an allergic disease, with AR being the most prevalent.

Surgical Treatment of Ossified Cephalhematoma in Young Child.

Cephalhematoma is a subperiosteal collection of blood. The lesion generally spontaneously resolves, but if cephalhematoma persists beyond this period, it typically begins to ossify and may require surgical treatment for correction. The incidence of ossified cephalhematoma is rare, and its pathogenesis is unclear. There have been reports of surgical treatment of ossified cephalhematoma in newborns and infants, but few reports in 5-year-old children. We experienced the surgical treatment of an ossified cephalhematoma in a 5-year-old boy. We performed periosteal reattachment with onlay bone autograft. Follow-up examination at 2 years revealed a good cosmetic result and a computed tomography scan showed excellent reconstitution of the contour of the skull.

Extracellular matrix gene expression in human trabecular meshwork cells following mechanical fluid flow stimulation.

AIM: To investigate changes in extracellular matrix (ECM) gene expression in human trabecular meshwork (HTM) cells in response to mechanical fluid flow stimulation. METHODS: HTM cells were grown on a glass plate coated with 0.02% type I collagen (COL) and exposed to shear stress (0, 0.2, 1.0 dyne/cm2) for 12h. Changes in genes related to the ECM were evaluated by real-time reverse transcriptase-polymerase chain reaction. Phosphorylation of Smad2 protein was investigated by Western blotting. RESULTS: After mechanical stimulation, COL type 4 alpha 2, COL type 6 alpha 1, and fibronectin-1 mRNA were significantly higher than the static control (P<0.05, <0.05, and <0.01, respectively). The metalloproteinase-2 and plasminogen activator inhibitor-1 mRNA were significantly higher than the static control (P<0.05 and <0.01, respectively), while the differences in the tissue inhibitors of metalloproteinases-2 mRNA were not significant. The phosphorylation of Smad2 levels was significantly higher compared to the static control cells. CONCLUSION: Changes in the expressions of genes associated ECM metabolism result in HTM cells after mechanical stimulation. The mechanical stimulation of the aqueous humor to the trabecular meshwork may promote ECM turnover and contribute to intraocular pressure homeostasis.

Food Protein-induced Enterocolitis Syndrome Due to Rice in a Japanese Infant: A Case Report.

Food protein-induced enterocolitis syndrome (FPIES) is a non-IgE-mediated gastrointestinal food allergy characterized by repetitive vomiting within 1-4 h and/or diarrhea within 24 h after ingesting the causative food. We herein report a rare Japanese case of rice-induced FPIES. A six-month-old, female, Japanese patient presented to the emergency room (ER) with the complaint of vomiting after feeding. Postprandial vomiting had occurred occasionally since she started ingesting solid food at the age of 5 months. Rice-induced FPIES was suspected only after the fourth ER visit based on the characteristic history of recurrent vomiting occurring 1-2 h after ingesting food containing rice. Allergen-specific IgE testing and a skin prick test with an allergen scratch extract were both negative for rice. During an oral food challenge test (OFC), vomiting was observed after the patient ingested 2 g of rice porridge. Based on the OFC results and the entire clinical course, FPIES due to rice was diagnosed. A lymphocyte stimulation test with rice revealed a significantly elevated stimulation index. Rice-induced FPIES is rarely reported among Japanese infants despite rice being a staple in the Japanese diet. The prevalence of rice-induced FPIES differs greatly among populations, suggesting a multifactorial cause associated with its development. Delays in diagnosis are common in FPIES, and our case demonstrates the importance of obtaining a dietary history of food ingested prior to symptom onset in cases of infantile repetitive vomiting.

Ruptured aneurysm-induced pituitary apoplexy: illustrative case.

BACKGROUND: Pituitary apoplexy associated with aneurysmal rupture is extremely rare and may be misdiagnosed as primary pituitary adenoma apoplexy. The authors present a case of a patient with pituitary apoplexy caused by rupture of an anterior cerebral artery aneurysm embedded within a giant pituitary adenoma, and they review the relevant literature. OBSERVATIONS: A 78-year-old man experienced sudden headache with progressive vision loss. Magnetic resonance imaging (MRI) revealed a giant pituitary tumor with abnormal signal intensity. Magnetic resonance angiography immediately before surgery showed a right A1 segment aneurysm, suggesting coexisting pituitary apoplexy and ruptured aneurysm. The patient underwent urgent transsphenoidal surgery for pituitary apoplexy. The tumor was partially removed, but the perianeurysmal component was left behind. Subsequent cerebral angiography showed a 5-mm right A1 aneurysm with a bleb that was successfully embolized with coils. Retrospective review of preoperative dynamic MRI showed extravasation of contrast medium from the ruptured aneurysm into the pituitary adenoma. Histopathologic examination showed gonadotroph adenoma with hemorrhagic necrosis. Postoperatively, the patient's visual function improved. LESSONS: MRI identification of pituitary apoplexy caused by aneurysmal rupture has not been reported previously. Aneurysmal rupture should be considered in the differential diagnosis of pituitary apoplexy. When a ruptured aneurysm is encountered, the authors recommend treating it before addressing pituitary apoplexy.

Primary intracranial aggressive fibromatosis arising in sella turcica: illustrative case.

BACKGROUND: Aggressive fibromatosis is a rare histologically benign but locally infiltrative myofibroblastic tumor. Primary intracranial aggressive fibromatosis (IAF) can exhibit a clinically malignant course. OBSERVATIONS: A 22-year-old otherwise healthy woman presented with left painful ophthalmoplegia. Magnetic resonance imaging (MRI) revealed a left sellar tumor with cavernous sinus invasion. Endoscopic transsphenoidal surgery was performed. The lesion could not be totally resected. An inflammatory myofibroblastic tumor was suspected, so steroid pulse therapy was introduced, but it was ineffective. The tumor recurred after a few months, and she complained of visual acuity loss, abducens nerve palsy, trigeminal neuralgia, and panhypopituitarism. The lesion was diagnosed as primary IAF by a pathological review. Gamma Knife radiosurgery was performed, and chemotherapies were introduced but ineffective. Her consciousness was disturbed, and MRI showed hypothalamic invasion of the tumor, occlusion and stenosis of carotid arteries, and cerebral stroke. Palliative care was introduced, and she died 32 months after the onset. The autopsy revealed tumor invasion to the cavernous sinus, optic nerve, hypothalamus, pituitary, and tonsillar herniation due to massive cerebral stroke. LESSONS: Radical resection can be impossible in patients with IAF. Radiotherapy and chemotherapy are not always effective for residual lesions. Adjuvant therapy for IAF remains to be explored.

A Case of Invasive Sphenoid Sinus Aspergillosis Presenting as Oculomotor Nerve Palsy in a Healthy Patient.

Invasive sphenoid sinus aspergillosis is a rare infection and usually affecting immunocompromised patients. We describe an invasive sphenoid sinus aspergillosis patient with immunocompetent who present progressive ocular dysfunctions. A 66-year-old woman with no history of immune dysfunction was referred to our hospital with orbital complications. Computed tomography (CT) scan and magnetic resonance imaging (MRI) showed a mass lesion extending from the left orbital apex to the sphenoid sinus. Inflammatory diseases were not suspected by laboratory findings, but a transnasal endoscopic biopsy revealed sphenoid sinus aspergillosis. After treatment of antifungal medication, this patient showed improvement and no sign of recurrence during the follow-up period. Diagnosis of invasive sphenoid sinus aspergillosis in an immunocompetent, healthy individual, was challenging. However, if patients have sinus wall deformities and orbital complications, early surgery is necessary to improve their prognosis.