Chronic Ileum Obstruction, Granulation, and Ulceration With IgG4-Positive Plasma Cell Infiltration in a Six-Year-Old Girl With 21trisomy.

Patients with 21trisomy often develop congenital or acquired gastrointestinal diseases, such as duodenal or anal atresia, celiac disease, intussusception, and constipation. In these patients, it is often challenging to diagnose gastrointestinal diseases because most patients have difficulty explaining their complaints in detail. Furthermore, these patients also possess immunological disorders, such as increased type I interferon activation, innate immune hypersensitivity, and polarization to autoimmune. Here, we present a girl with 21trisomy and constipation who developed severe anemia, occult blood and elevated levels of calprotectin in stool, and chronic ileum obstruction confirmed by computed tomography. The patient underwent surgical resection of the ileum and recovered without complications. Pathological examination demonstrated intussusception, ischemia, ulceration, inflammatory granulation, and massive IgG4-positive plasma cell infiltration. After the surgery, her fecal calprotectin levels were normalized. We assumed that the ileum inflammation caused by ileum dilation generated ulcers and granulation, which could be associated with immunological, gastrointestinal, and intellectual disorders in patients with 21trisomy.

A novel approach to diagnosing crystal-storing histiocytosis: utility of scanning electron microscopy for formalin-fixed paraffin-embedded tissue specimens.

Crystal-storing histiocytosis (CSH) is a rare disorder that shows infiltration of histiocytes with an aberrant cytoplasmic accumulation of crystalline structures and is often accompanied by lymphoproliferative-plasma cell disorders (LP-PCD) as background diseases. The diagnosis of CSH requires identification of crystalline structures that accumulate in the infiltrating histiocytes, which may be challenging by optical microscopy alone. In this case report, we describe an atypical course of systemic CSH with multifocal fibrosclerosis of an unknown background disease that was diagnosed by ultrastructural observation, including transmission electron microscopy (TEM) and scanning electron microscopy (SEM), in pathological autopsy. In addition, crystalline structures were successfully identified by scanning electron microscopic observations using formalin-fixed and paraffin-embedded (FFPE) tissue from biopsy specimens taken before death. Since CSH was identified by SEM in a tiny biopsy specimen, observation of histiocytic infiltrative lesions by SEM using FFPE tissue may lead to early detection of and initiation of treatment for CSH.

Radioprotective Effects of a Semicircular X-ray Shielding Device for Operators During CT Fluoroscopy-Guided Interventional Procedures: Experimental and Clinical Studies.

PURPOSE: To evaluate, experimentally and clinically, the radioprotective effects of a semicircular X-ray shielding device for operators during CT fluoroscopy-guided IR procedures. MATERIALS AND METHODS: During experimentation, the reduction rates of scattered radiation rates from CT fluoroscopy were evaluated using a humanoid phantom. Two shielding device positions were tested: "shielding close to the CT gantry" and "shielding close to the operator". The scattered radiation rate without shielding was also evaluated. The clinical study retrospectively evaluated the operator's radiation exposure during 314 CT-guided IR procedures. With a semicircular X-ray shielding device (with shielding group, n = 119) or without it (no shielding group, n = 195), CT fluoroscopy-guided IR procedures were performed. Radiation dose measurements were taken using a pocket dosimeter placed near the operator's eye. For shielding and no shielding groups, the procedure time, dose length product (DLP), and the operator's radiation exposures were compared. RESULTS: Experimentation revealed the respective mean reduction rates of "shielding close to the CT gantry" and "shielding close to the operator" as 84.3% and 93.5% compared with the no-shielding setting. Although no significant differences were found in the procedure time and the DLP between "no shielding" and "with shielding" groups in the clinical study, the operators' radiation exposure in the "with shielding" group (0.03 ± 0.04 mSv) was significantly lower than in the "no shielding" group (0.14 ± 0.15 mSv; p < .001). CONCLUSION: The semicircular X-ray shielding device provides valuable radioprotective effects for operators during CT fluoroscopy-guided IR.

The Effects of Mucopolysaccharide Polysulfate on Steroid-Induced Tight Junction Barrier Dysfunction in Human Epidermal Keratinocytes and a 3D Skin Model.

INTRODUCTION: The long-term use of topical corticosteroids (TCS) is associated with side effects such as skin atrophy and barrier deterioration. Moisturizers, such as mucopolysaccharide polysulfate (MPS), have been reported to prevent relapses in atopic dermatitis (AD) when used in combination with TCS. However, the mechanisms underlying the positive effects of MPS in combination with TCS in AD are poorly understood. In the present study, we investigated the effects of MPS in combination with clobetasol 17-propionate (CP) on tight junction (TJ) barrier function in human epidermal keratinocytes (HEKa) and 3D skin models. METHODS: The expression of claudin-1, which is crucial for TJ barrier function in keratinocytes, and transepithelial electrical resistance (TEER) was measured in CP-treated human keratinocytes incubated with and without MPS. A TJ permeability assay, using Sulfo-NHS-Biotin as a tracer, was also conducted in a 3D skin model. RESULTS: CP reduced claudin-1 expression and TEER in human keratinocytes, whereas MPS inhibited these CP-induced effects. Moreover, MPS inhibited the increase in CP-induced TJ permeability in a 3D skin model. CONCLUSION: The present study demonstrated that MPS improved TJ barrier impairment induced by CP. The improvement of TJ barrier function may partially be responsible for the delayed relapse of AD induced by the combination of MPS and TCS.

Use of bronchoalveolar lavage in diagnosing angioimmunoblastic T-cell lymphoma: A case report.

Angioimmunoblastic T-cell lymphoma (AITL) is a type of peripheral T-cell tumour that belongs to the group of non-Hodgkin's lymphomas. Pulmonary lesions can be found in 7%-10% of AITL cases. Imaging findings of the lungs varied; however, immunoblastic infiltration in the lungs is rare. Our patient was a 73-year-old man who received repeated chemotherapy for AITL. Fourth-line therapy using romidepsin controlled the illness, but the patient was hospitalized for dyspnoea and an infiltrative shadow. We performed bronchoalveolar lavage (BAL), and the culture was positive for Haemophilus influenzae. The patient was initially discharged with antibiotic therapy, but hospitalized again. Antibiotics were ineffective and the patient required mechanical ventilation. BAL was performed again, after which fluid cytology revealed immunoblast-like atypical cells. Therefore, the patient was diagnosed with pulmonary infiltration due to AITL. Steroid therapy proved ineffective, and the patient died. BAL was used to effectively diagnose pulmonary AITL infiltration.

Sweat-gland carcinoma with neuroendocrine differentiation (SCAND): a clinicopathologic study of 13 cases with genetic analysis.

Low-grade neuroendocrine carcinoma of the skin (LGNECS) was proposed in 2017 as a new primary cutaneous neoplasm with neuroendocrine differentiation; however, it is not yet well known due to its rarity. Herein, we perform a detailed clinicopathologic analysis of 13 cases as well as panel DNA sequencing in three cases. The study included 12 males and 1 female with a median age of 71 (43-85) years. All lesions occurred on the ventral trunk. The mean tumor size was 2.2 (0.8-11.0) cm. The histopathology resembled that of well-differentiated neuroendocrine tumors (NETs) in other organs, but intraepidermal pagetoid spreading was seen in 8 (61.5%) cases and stromal mucin deposits in 4 (30.8%). Immunoreactivity for CK7, CK19, EMA, BerEP4, CEA, chromogranin A, synaptophysin, INSM1, GCDFP15, GATA3, ER, and bcl-2 were present in varying degrees in all tested cases. PTEN c.165-1G>A splice site mutation was detected by panel sequencing in one case, and GATA3 P409fs*99 and SETD2 R1708fs*4 in another case. Lymph node metastasis was seen significantly in cases with tumor size >2.0 cm [8/8 (100%) vs. 1/5 (20%)]. All three cases with size >3.0 cm were in unresectable advanced-stage [3/3 (100%) vs. 1/10 (10%)], and two of the three patients succumbed to the disease. The two cases of death revealed mild nuclear atypia (mitosis: 1/10 HPFs) and moderate nuclear atypia (2/10 HPFs). Thus, tumor size would be a better prognostic factor than nuclear atypia, mitotic count, and Ki67 index, unlike in NETs. These clinicopathologic and immunohistochemical features would represent the characteristics as skin adnexal tumors with apocrine/eccrine differentiation rather than NETs; therefore, we rename it as sweat-gland carcinoma with neuroendocrine differentiation (SCAND).

Thymoma-Related Stiff-Person Syndrome with Successfully Treated by Surgery.

INTRODUCTION: Stiff-person syndrome (SPS) is a rare autoimmune neurological disorder. Paraneoplastic SPS associated with malignant tumors such as thymoma occurs in approximately 5% of all SPS cases. We present a rare case of thymoma accompanied by SPS successfully treated using surgery. PRESENTATION OF CASE: A 26-year-old woman presented with lower limbs convulsions and gait disturbance and complained of leg pain. Cerebrospinal fluid and blood test results showed a high level of anti-glutamic acid decarboxylase (GAD) antibodies. Computed tomography showed anterior mediastinal tumor suggestive of a thymoma. She underwent extended thymectomy, and her symptoms gradually improved after surgery. No evidence of recurrent thymoma and SPS has been observed over 44 months. CONCLUSION: Surgical treatment would be effective for patients with SPS and thymoma.

[Usefulness of Combining Post-processing Scatter Correction and an Anti-scatter Grid in Chest Standing Radiography].

PURPOSE: The aim of this study was to evaluate the usefulness of combining post-processing scatter correction (IG) and an anti-scatter grid (RG) in chest radiography. METHOD: To determine the combination protocol (Hyb) that was closed to RG 12:1 (RG12), we measured the content rate of scattered radiation for each combination (RG12, IG12, RG3-12+IG3-12). Task-based modulation transfer function (MTF_Task) and SDNR were evaluated using RG12, IG12, and Hyb. Additionally, seven radiologists performed visual evaluation by using chest phantom. RESULT: The protocol of Hyb was RG8+IG3. In SDNR, Hyb (RG8+IG3) was equal to or higher than RG12, and MTF_Task was equal in all grid systems. Hyb (RG8+IG3) was significantly superior to RG12 in visual evaluation. CONCLUSION: The combining post-processing scatter correction should be useful for improving inspection throughput and reducing the risk of grid's damage.

[Comparison of Detectability for the New Image Processing and Conventional Image Processing by ROC Analysis: A Phantom Study of Chest Radiography].

The purpose of this study is to compare the detectability of diseases the new image processing and the conventional image processing by receiver operating characteristic (ROC) analysis and to show the usefulness of the new image processing. Radiographs with and without nodular cancer models in the chest phantom were used for observation samples. Totally 200 radiographs were evaluated by 10 radiological technologists (each readers had over 20 years or under 4 years of experience). The mean area under the curve (AUC) calculated from the over 20 years group was 0.754 for the new processing and 0.771 for the conventional processing (p value=0.651, 95% confidence interval=-0.084/0.049 (lower bound/upper bound)). On the other hand, the average AUC calculated from under 4 years group was 0.819 for the new processing and 0.678 for the conventional processing (p value= 0.041, 95% confidence interval=0.019/0.262 (lower bound/upper bound)). New image processing provides high detectability in less than 4 years group compared to conventional processing.

[Measurement of Head and Neck Exposure in CT Guided IVR].

In April 2011, the International Radiological Protection Committee recommended that "The equivalent dose of the crystalline lens should not exceed 20 mSv/year, averaged over defined periods of 5 years, with no single year exceeding 50 mSv". Based on this recommendation, it is predicted that the equivalent dose limit of our crystalline lens can be lowered in the near future. Therefore, it is important to grasp the current situation of radiation exposure. The purpose of this study is to measure the crystalline lens of surgeons by focusing on the CT-fluoroscopy guided interventional radiology's (IVRs). We also examined whether the exposure dose of the crystalline lens can be correctly evaluated by measuring the unequal exposure dose of the neck, which is usually used for the unequal exposure measurement. Results of the analysis of 200 CT-fluoroscopy guided IVR procedures showed that the unequal exposure dose of the neck was significantly correlated with the exposure dose of the crystalline lens which was measured near the left eye ball (R=0.83). However, the exposure dose of the crystalline was 33% lower than those of the neck. Therefore, although the individual dosimeter worn on the neck can be used as the useful index of the exposure dose of the crystalline lens, the results can be overestimated.

[Multi-facility Survey on Accuracy and Calibration Method for Dose Area Product Meter of Angiography Systems].

In many facilities, the displayed dose of dose-area product (DAP) is used for the dose management of interventional radiology. In this study, we investigated the measured dose at the patient entrance reference point (interventional reference point) and the displayed dose on each angiography systems. Also, we investigated the calibration method of each DAP. The errors of the measured dose and the displayed dose were less than 35%, but that dispersion was wide between the systems. The calibration methods varies according to a system. And even in the same manufacturer, the calibration methods were different. Therefore, to use the displayed dose of DAP for patient dose management, we need to compare that with the measured dose regularly. The differences of calibration methods induce the wide dispersion of the errors of the measured and the displayed dose. So the standardization of the calibration method in each manufacturer is expected.

Development of a Three-dimensional Surgical Navigation System with Magnetic Resonance Angiography and a Three-dimensional Printer for Robot-assisted Radical Prostatectomy.

We sought to develop a surgical navigation system using magnetic resonance angiography (MRA) and a three-dimensional (3D) printer for robot-assisted radical prostatectomy (RARP). Six patients with pathologically proven localized prostate cancer were prospectively enrolled in this study. Prostate magnetic resonance imaging (MRI), consisting of T2-weighted sampling perfection with application-optimized contrasts using different flip-angle evolutions (SPACE) and true fast imaging with steady-state precession (true FISP), reconstructed by volume rendering, was followed by dynamic contrast-enhanced MRA performed with a volumetric interpolated breath-hold examination (VIBE) during intravenous bolus injection of gadobutrol. Images of arterial and venous phases were acquired over approximately 210 seconds. Selected images were sent to a workstation for generation of 3D volume-rendered images and standard triangulated language (STL) files for 3D print construction. The neurovascular bundles (NVBs) were found in sequence on non-contrast images. Accessory pudendal arteries (APAs) were found in all cases in the arterial phase of contrast enhancement but were ill-defined on non-contrast enhanced MRA. Dynamic contrast-enhanced MRA helped to detect APAs, suggesting that this 3D system using MRI will be useful in RARP.

Extraskeletal osteosarcoma of the lung following treatment of primary small-cell lung carcinoma with chemoradiotherapy: A case report.

Primary pulmonary osteosarcoma is a rare entity, with only a few cases reported in the literature to date. Moreover, secondary extraskeletal osteosarcoma of the lung following chemoradiotherapy is extremely rare and, to the best of our knowledge, this is the first reported case. We herein present the case of an 80-year-old male patient with small-cell lung cancer (SCLC), who developed what it was initially considered as recurrence of the tumor after chemoradiotherapy. The patient eventually succumbed to the disease, and on autopsy it was discovered that the lung tumor was not in fact SCLC, but rather a secondary osteosarcoma. Osteosarcoma metastasis to the lung is very rare, but must be considered in the differential diagnosis when there is relapse following treatment for primary SCLC.

Palisaded neutrophilic and granulomatous dermatitis as a novel cause of hypercalcemia: A case report.

RATIONALE: Palisaded neutrophilic and granulomatous dermatitis (PNGD) is a benign, inflammatory dermatosis with distinct histopathological features often observed in patients with systemic diseases. There were no reports of PNGD without underlying systemic diseases as an underlying cause of hypercalcemia. Herein, we report a case of a 62-year-old man with hypercalcemia due to PNGD, but with no underlying systemic diseases, including tuberculosis, sarcoidosis, or vasculitis. PATIENT CONCERNS: Laboratory tests showed an elevated C-reactive protein level, an elevated corrected calcium level, a normal 25-hydroxyvitamin D level, and an elevated 1,25-dihydroxyvitamin D level. There were no other abnormalities to explain the hypercalcemia. Positron emission tomography-computed tomography showed abnormal uptake in his skin. Histopathological examination of the skin showed palisaded granulomatous infiltrate in the dermis. Neutrophils, degenerated collagen, and fibrin were present in the centers of the palisades without prominent mucin. There were no eosinophils, central necrosis, or necrotizing vasculitides. These features were consistent with PNGD. DIAGNOSES: A diagnosis of PNGD with hypercalcemia was established. INTERVENTIONS: Oral prednisolone was administered to the patient. OUTCOMES: After treatment, his symptoms resolved, and his calcium, 1,25-dihydroxyvitamin D and CRP levels returned to normal. Skin specimens before and after treatment were assessed using immunohistochemistry for 1a-hydroxylase. Granuloma and epidermal cells were 1a-hydroxylase-positive before treatment. After treatment, the granuloma diminished in size and the 1ahydroxylase-positive areas of the epidermal cells decreased. LESSONS: This case was particularly unique because increased 1a-hydroxylase expression in the granuloma and epidermal cells seemed to result in hypercalcemia due to excessive transformation of 25-hydroxyvitamin D to 1,25-dihydroxyvitamin D. Physicians should consider PNGD as an underlying cause of hypercalcemia.

Echocardiography findings in a case with Ballantyne syndrome.

Marked fluid retention occurs in Ballantyne syndrome, but few reports are available on changes in cardiac morphology in this syndrome. A woman with generalized edema, dyspnea, fetal hydrops (skin edema and ascites), thickened placenta, and elevated plasma B-type natriuretic peptide level (344 pg/mL) was admitted to our hospital at gestational week (GW) 20+3 . Blood pressure remained within the normal range. However, acute increases in left atrial volume index, pulmonary artery systolic pressure, and hyperdynamic left ventricular function (as evidenced by increased left ventricular ejection fraction to 74% with cardiac index of 5.1 L/min/m2 ) occurred preceding fetal death at GW 21+4 in the presence of increased inferior vena cava diameter (23 mm) and relatively low systemic vascular resistance of 752 dyn·s/cm5 . These findings suggested life-threatening heart failure and required cesarean delivery at GW 21+5 resulting in complete recovery. The placenta suggested cytomegalovirus infection.

HIV-associated cystic lesions of the parotid gland.

We present two cases of an HIV-associated parotid gland cyst. One case was a 36-year-old HIV infected woman. She was diagnosed with HIV infection and presented with slowly enlarged parotid gland cysts together with elevation of HIV viral RNA copies/mL in her serum. She was performed parotid gland biopsy under the general anesthesia. The histopathologic analysis revealed negative HIV p24-antigen in her parotid gland tissue. The other case was a 43-year-old man found his parotid gland swelling shortly after highly active antiretroviral therapy (HAART). He was diagnosed with HIV infection 2 years previously. He had started HAART several days before. He showed exceeding elevation of IgE in his serum. We treated him with medication using anti-histamic drugs for his cyst. A computed tomography scan revealed a complete response of his parotid gland cyst 4 weeks after the medication. His serum IgE level was decreased to half of the level before the medication. These findings suggested that the parotid gland swelling associated with HIV was due to various factors including immune reconstitution inflammatory syndrome (IRIS). In case such a parotid gland swelling, we could avoid invasive treatments.

Three simultaneous cases of hypersensitivity pneumonitis and acute lung injury caused by dichloromethane.

Three middle-aged male patients were admitted to our hospital complaining of dry cough, dyspnoea, and fever. All three of them had cleaned a refrigerator at a Chinese noodle mill using paint remover containing dichloromethane (90%) the previous day. Chest X-rays revealed bilateral diffuse infiltrative shadows on the lungs. We performed transbronchial lung biopsies and bronchoalveolar lavage (BAL) via bronchoscopy for each patient. Bronchoalveolar lavage showed increased total cell count as well as lymphocyte and neutrophil fraction. Transbronchial lung biopsy revealed Masson bodies and slight lymphocyte infiltration into the alveolar septa. We diagnosed all three patients with hypersensitivity pneumonitis and acute lung injury caused by exposure to the paint remover containing dichloromethane. Two of these patients ultimately went into respiratory failure, requiring the administration of a corticosteroid. The third patient improved without the need of steroid therapy.