[An autopsy case of polyarteritis nodosa accompanied with multiple immune-specific autoantibodies and rhabdomyolysis].

A 72-year-old male complained of fever lasting 1 month and developed muscle weakness and paresthesia in the legs. He presented with muscle weakness, grasping pain, decreased deep tendon reflexes in the extremities, and reduction of tactile sensation in the distal parts of the left leg muscles. Blood tests revealed leukocytosis and inflammatory reactions. Collagen-disease-specific autoantibodies including anti-double-stranded DNA and anti-Scl-70 antibodies were positive, but antineutrophil cytoplastic antibodies were negative. Nerve conduction studies revealed asymmetric axonal degeneration, indicating multiple mononeuropathy. We started intravenous methylprednisolone pulse and plasma exchange therapies. However, the patient developed intestinal necrosis and perforation, and he died 44 days after the onset of fever. An autopsy revealed vasculitis in small- to medium-sized vessels in multiple organs as well as myoglobin casts in the renal tubules, which were suggestive polyarteritis nodosa (PAN) accompanied with rhabdomyolysis. Positivity for collagen-disease-specific autoantibodies and accompanying rhabdomyolysis are atypical findings with PAN. This patient was not clinically diagnosed as PAN, and so promptly starting immunotherapies should be considered when a case presents with evidence of vasculitis.

A facelift procedure for resection of a branchial cleft cysts.

OBJECTIVES: Branchial cleft cysts (BCCs) are common in daily practice, however, BCC patients suffer aesthetic problems due to postoperative scars on visible parts after surgery. To analyze the feasibility, surgical outcomes and possible risks and complications encountered during a facelift procedure for patients with BCC. METHODS: This retrospective analysis examined patients who had undergone surgery for branchial cleft cyst using a facelift procedure (n = 16) or conventional transcervical resection (n = 20) at our institutes between April 2015 and August 2021. RESULTS: There was no significant difference between the groups that underwent the facelift procedure or conventional transcervical resection as to the average size of the cysts, operating time, bleeding, drain out, or recurrence. None of the patients needed to switch from the facelift procedure to conventional transcervical resection. In all the patients in the facelift procedure group, postoperative scars were fully concealed by the auricle and hair. However, four patients in the facelift procedure group experienced a transient auricular complication after surgery. CONCLUSION: The facelift procedure provides adequate visualization, workspace and excellent cosmetic results in suitably selected cases with BCC.

A Case of Esophagojejunal Variceal Rupture after Total Gastrectomy and Esophagojejunostomy Successfully Treated with Percutaneous Transhepatic Obliteration under Dual-balloon Occlusion of Feeding and Draining Veins.

We present the case of a man in his 60s with bleeding esophagojejunal varices occurring after gastrectomy for gastric carcinoma. Percutaneous transhepatic portography depicted the esophagojejunal varices originated from the jejunal vein and drained into the azygos vein. A 5-French occlusion balloon catheter was wedged into the jejunal vein and a 3-French occlusion balloon catheter into one drainage channel of the esophagojejunal varices via the azygos vein. Selective antegrade jejunal venography under dual-balloon occlusion revealed entire esophagojejunal varices with good stagnated and well-opacified contrast medium. Subsequently, 12 mL of 5% ethanolamine oleate-contrast medium mixture was slowly injected into the esophagojejunal varices. He was discharged without complications one week after the procedure, and abdominal computed tomography demonstrated the disappearance of the esophagojejunal varices six months after the procedure.

Cervical Liposarcoma Revisited: A Case Report and Scoping Literature Review.

The commonest sites for liposarcoma are the retroperitoneum and lower extremities. Liposarcoma of the head and neck region is a rare and potentially life-threatening malignancy. Tumors originating in the right cervical space cause special diagnostic and therapeutic difficulties. In the present report, we describe a case of differentiated liposarcoma of the right cervical region. The tumor continued to grow slowly over 3 years before a definitive diagnosis was established. Extended extirpation of the tumor was performed and proved efficacious in that no recurrence has been observed for 4 years. Recommendations for earlier and accurate diagnosis and treatment of this rare neoplasm are discussed.

A multicenter prospective study of the treatment and outcome of patients with gastroduodenal peptic ulcer bleeding in Japan.

Gastroduodenal peptic ulcers are the main cause of nonvariceal upper gastrointestinal bleeding (UGIB). We believe that recent advances in endoscopic techniques and devices for diagnosing upper gastrointestinal tract tumors have advanced hemostasis for UGIB. However, few prospective multicenter studies have examined how these changes affect the prognosis. This prospective study included 246 patients with gastroduodenal peptic ulcers treated at 14 participating facilities. The primary endpoint was in-hospital mortality within 4 weeks, and the secondary endpoints required intervention and refractory bleeding. Subsequently, risk factors affecting these outcomes were examined using various clinical items. Furthermore, the usefulness of the risk stratification using the Glasgow-Blatchford score, rockall score and AIMS65 based on data from the day of the first urgent endoscopy were examined in 205 cases in which all items were complete there are two periods. Thirteen (5%) patients died within 4 weeks; and only 2 died from bleeding. Significant risk factors for poor outcomes were older age and severe comorbidities. Hemostasis was required in 177 (72%) cases, with 20 cases of refractory bleeding (2 due to unsuccessful endoscopic treatment and 18 due to rebleeding). Soft coagulation was the first choice for endoscopic hemostasis in 57% of the cases and was selected in more than 70% of the cases where combined use was required. Rockall score and AIMS65 predicted mortality equally, and Glasgow-Blatchford score was the most useful in predicting the requirement for intervention. All scores predicted refractory bleeding similarly. Although endoscopic hemostasis for UGIB due to peptic ulcer had a favorable outcome, old age and severe comorbidities were risk factors for poor prognosis. We recommend that patients with UGIB should undergo early risk stratification using a risk scoring system.

Endoscopic Treatment of Sinonasal Leiomyosarcoma: A Case Report in Light of the Literature.

A 49-year-old Japanese man presented with a rare case of sinonasal leiomyosarcoma with left nasal bleeding for 12 months. He had no history of irradiation or malignancies, including retinoblastoma. Preoperative histological examination suggested vascular leiomyoma. Complete resection with endoscopic surgery was performed. Histological examination during the operation suggested that the tumor was a leiomyoma. However, immunohistochemical staining for α smooth muscle actin and desmin were helpful in establishing a definitive diagnosis of sinonasal leiomyosarcoma. The resection margins were positive for tumor cells. Staging with CT of the neck and thorax, ultrasound of the abdomen, and MRI of the head ruled out metastases. Second endoscopic tumor resection surgery was performed for positive resection margins. The patient's condition was successfully managed with additional excision, and he remains well with no evidence of recurrence and metastasis 36 months after treatment. Endoscopic management should be considered in suitable cases.

Treatment of oral ranula in HIV-positive patient.

HIV-associated salivary gland disease refers to the pathology in head and neck lesions such as ranula, salivary gland swelling, xerostomia, and benign lymphoepithelial cysts in the parotid gland. Here, we present a unique case of the ranula patient with HIV infection treated with OK-423 sclerotherapy. Case report: The patient was a 42-year-old Japanese male with a few months history of oral floor swelling. Computed tomography (CT) showed a low-density area limited within the right floor of the mouth. Magnetic resonance imaging (MRI) revealed a distinct T2-high intensity area localized on the same location. The puncture fluid was bloody mucus, and the cytology was no malignancy. We diagnosed a simple ranula. He was, however, found to be HIV-antibody positive at the examination before treatment by chance. He was referred to the department of infectious diseases and definitively diagnosed HIV infection by western blot. We chose OK-432 sclerotherapy because of its minimally invasive and the risk of HIV infecting medical staff. Two times OK-432 injection made the lesion disappear. Conclusion: The case indicated that OK-432 sclerotherapy could be effective for ranula related to HIV.

Metastatic Renal Cell Carcinoma to the Left Sphenoid Sinus: A Case Report in Light of the Literature.

A 79-year-old Japanese woman presented with a rare case of metastatic renal cell carcinoma to the left sphenoid sinus with left nasal bleeding. She had previously had right radical nephrectomy for renal cell carcinoma at the age of 64 years and brain and spinal cord infarction at 74 years. Endoscopic examination revealed no mass in the nasal cavity. CT and MRI revealed a tumor in the left sphenoid sinus. The size of the tumor increased gradually from 12 to 15 years after the radical nephrectomy. Complete resection with endoscopic surgery was performed without preoperative embolization. The tumor cells had clear cytoplasm and were arranged in a trabecular pattern lined by a layer of endothelial cells. These findings were identical to the pathological findings of the surgical specimen of the renal cell carcinoma from 15 years previous. A pathological diagnosis of metastatic renal cell carcinoma of clear cell type (grade 1) was made. PET-CT demonstrated no metastasis. The patient's condition was successfully managed with excision of the tumor, and she remains well with no evidence of recurrence and metastasis 36 months after treatment. Metastatic renal cell carcinoma to the sphenoid sinus is rare, but it might be considered in the differential diagnosis of masses in the paranasal sinus even long after initial treatment of renal cancer.

A Facelift Procedure for Resection of Benign Parapharyngeal Tumors.

<b>Objective:</b> The feasibility, surgical outcomes and possible risks and complications encountered during a facelift procedure for patients with parapharyngeal space (PPS) tumor were analyzed. <br><b>Method:</b> This retrospective analysis examined 10 patients who underwent surgery for PPS tumor using a facelift incision at our institutes between April 2015 and August 2019. <br><b>Results:</b> This study included four retro-styloid (benign nerve sheath tumor) and six pre-styloid tumors (pleomorphic adenoma). Mean tumor dimensions were 4.1 x 4.2 x 3.8 cm respectively. None of the patients needed conversion to conventional open resection. Transient sensory changes in the auricle occurred in 30% of the patients; however, all recovered within four months. In all the patients, postoperative scars were fully concealed by the auricle and hair. No recurrences were detected during a mean follow-up period of 16.6 months. <br><b>Conclusion: </b>The facelift procedure provides adequate visualization, workspace and excellent cosmetic results in properly selected cases.

Nodal Merkel Cell Carcinoma in Head and Neck Lesions with an Unknown Primary: A Case Report in Light of the Literature.

Merkel cell carcinoma (MCC) is a rare but aggressive neuroendocrine skin cancer. To diagnose nodal MCC with an unknown primary disease is challenging, and it has to be separated from other nodal metastatic neoplasms. We report a unique case of nodal MCC in head and neck lesions with an unknown primary. A 70-year-old woman was admitted to our department with a right submandibular mass. Fine needle aspiration biopsy was performed and indicated malignancy. F-18-fluorodeoxyglucose positron emission tomography (PET) demonstrated abnormal accumulation in the right submandibular lymph node, right palatine tonsil, and right thyroid gland. For diagnostics and treatment, bilateral selective neck lymph node dissection, right tonsillectomy, and right thyroidectomy were performed. Histopathological examination revealed that most parts of the submandibular lymph node were occupied by diffuse sheets of tumor cells. Contrary to our expectation, malignant cells were not detected in the right palatine tonsil and right thyroid. Immunohistochemistry demonstrated a marked positive reaction for AE1/AE3, chromogranin A, synaptophysin, cytokeratin 20 (CK20) and CD56 and a negative reaction for vimentin, leucocyte common antigen (LCA), thyroid transcription factor-1 (TTF1) and cytokeratin 7 (CK7) in the tumor cells. Immunostaining of Merkel cell polyomavirus-large T antigen (MCPyV-LT) showed a positive reaction and MCPyV-positive MCCs were assessed by PCR analysis, demonstrating that viral copy number was 12.8 copies per cell. These histological findings confirmed the diagnosis of Merkel cell carcinoma of the lymph node. In cases of tumors in the lymph node with a neuroendocrine appearance in head and neck lesions, it is necessary to eliminate the possibility of metastasis from MCC.

Endoscopic Treatment of Sinonasal Glomangiopericytoma: A Case Report in Light of the Literature.

A 71-year-old Japanese male patient presented with a rare case of Glomangiopericytoma (GPC) of the left nasal with obstruction. Complete resection with endoscopic surgery was performed. Immunohistochemical staining for smooth muscle actin, ß catenin, cyclin D1, vimentin, and factor 13 were helpful in establishing a definitive diagnosis. Extranasal treatment has been traditionally performed for successful management. However, recent advances in endoscopic treatment have enabled complete endoscopic resection of GPC, minimizing morbidity and facilitating subsequent surveillance for recurrence. Endoscopic management should be considered in suitable cases.

The expression of 11 beta hydroxysteroid dehydrogenase in severe allergic rhinitis.

OBJECTIVE: To clarify the roles of 11 beta-HSD in resistance to glucocorticoid therapy for allergic rhinitis, a case series study was conducted. METHODS: The patient group consisted of 20 subjects with allergic rhinitis, aged from 21 to 46 years (mean age 26.5), who showed persistent GC resistance necessitating surgical removal of the inferior turbinate after 6 months' GC treatment. The patients with poor response to GC treatment for 6 months' were defined as GC resistance. The control group consisted of 10 subjects aged from 16 to 39 years (mean age 24.5) who underwent maxillofacial surgery, from whom nasal tissues were taken and who did not receive GC treatment. Nasal mucosal tissues from patients and cntorol subjects were examined immunohistochemically. The sections were washed with 0.01 M phosphate-buffered saline (PBS; pH 7.2) containing 0.15 M NaCl and 0.01% Triton X-100, and incubated for 2 h with rabbit polyclonal anti-11 beta HSD1 and 11 beta-HSD2 antibody (Santa Cruz Biotechnology, Inc., Santa Cruz, CA, USA), each diluted 1:200 in PBS containing 0.1% bovine serum albumin. Immunostained sections were assessed under an Olympus microscope with an eyepiece reticule at 200 X magnification. Cell counts are expressed as means per high-power field (0.202 mm2). Control group means (arithmetic mean ± SD) were compared with patient group means by Mann-Whitney U-test at P = 0.05. RESULTS: Although 11 beta-HSD1 was expressed to a similar extent in patients and controls, 11 beta-HSD2 was expressed significantly more in patients with severe allergic rhinitis, resulting in a increased HSD-1/HSD-2 ratio. The significantly increased expression of 11 beta-HSD2 in the nasal epithelium and submucosal inflammatory cells of patients with severe nasal allergy were observed in the present study. CONCLUSION: Our findings suggest that 11 beta-HSD2 plays an important role in resistance to glucocorticoid therapy for allergic rhinitis, and its expression might be used as an additional parameter indicating steroid resistance in allergic rhinitis.

[A Case of Merkel Cell Carcinoma Complicated with Severe Thrombocytopenia Treated with Carboplatin/Etoposide Regimen after Surgery].

Thrombocytopenia is often caused by myelosuppression during chemotherapy. However, when platelet transfusions are required, pathological conditions such as idiopathic thrombocytopenic purpura(ITP)and thrombotic thrombocytopenic purpura( TTP)also occur. We report a case of Merkel cell carcinoma complicated with severe thrombocytopenia treated with carboplatin/etoposide regimen after surgery. The patient's platelet count did not increase in spite of platelet transfusions. However, the platelet count increased after steroid treatment was chosen under the diagnosis of ITP. Subsequent examinations revealed that the patient had HLA antibody, which caused the platelet transfusion refractoriness. When the platelet count does not increase in spite of platelet transfusions during chemotherapy, the possibility that the platelet transfusion refractoriness is due to the presence of HLA antibody should be considered.

Endoscopic surgical management of sinonasal inverted papilloma extending to frontal sinuses.

OBJECTIVE: Sinonasal inverted papilloma has been traditionally managed with external surgical approaches. Advances in imaging guidance systems, surgical instrumentation, and intraoperative multi-visualization have led to a gradual shift from external approaches to endoscopic surgery. However, for anatomical and technical reasons, endoscopic surgery of sinonasal inverted papilloma extending to the frontal sinuses is still challenging. Here, we present our experience in endoscopic surgical management of sinonasal inverted papilloma extending to one or both frontal sinuses. METHODS: We present 10 cases of sinonasal inverted papilloma extending to the frontal sinuses and successfully removed by endoscopic median drainage (Draf III procedure) under endoscopic guidance without any additional external approach. RESULTS: The whole cavity of the frontal sinuses was easily inspected at the end of the surgical procedure. No early or late complications were observed. No recurrence was identified after an average follow-up period of 39.5 months. CONCLUSION: Use of an endoscopic median drainage approach to manage sinonasal inverted papilloma extending to one or both frontal sinuses is feasible and seems effective.

Identification of a major enzyme for the synthesis and hydrolysis of cyclic ADP-ribose in amphibian cells and evolutional conservation of the enzyme from human to invertebrate.

Cyclic ADP-ribose (cADPR), a metabolite of NAD(+), is known to function as a second messenger for intracellular Ca(2+) mobilization in various vertebrate and invertebrate tissues. In this study, we isolated two Xenopus laevis cDNAs (frog cd38 and cd157 cDNAs) homologous to the one encoding the human cADPR-metabolizing enzyme CD38. Frog CD38 and CD157 are 298-amino acid proteins with 35.9 and 27.2 % identity to human CD38 and CD157, respectively. Transfection of expression vectors for frog CD38 and CD157 into COS-7 cells revealed that frog CD38 had NAD(+) glycohydrolase, ADP-ribosyl cyclase (ARC), and cADPR hydrolase activities, and that frog CD157 had no enzymatic activity under physiological conditions. In addition, when recombinant CD38 and frog brain homogenate were electrophoresed on an SDS-polyacrylamide gel, ARC of the brain homogenate migrated to the same position in the gel as that of frog CD38, suggesting that frog CD38 is the major enzyme responsible for cADPR metabolism in amphibian cells. The frog cd38 gene consists of eight exons and is ubiquitously expressed in various tissues. These findings provide evidence for the existence of the CD38-cADPR signaling system in frog cells and suggest that the CD38-cADPR signaling system is conserved during vertebrate evolution.

Prevalence of organic colonic lesions in patients meeting Rome III criteria for diagnosis of IBS: a prospective multi-center study utilizing colonoscopy.

BACKGROUND: It remains unknown whether the Rome III criteria can exclude organic colonic lesions prior to the diagnosis of irritable bowel syndrome (IBS). We evaluated the colonoscopy results of patients meeting the Rome III criteria for the diagnosis of IBS to determine the presence of organic colonic lesions. METHODS: This study was prospectively conducted at 17 centers in Japan. We enrolled 4528 patients who underwent diagnostic colonoscopy examinations. The diagnosis of IBS was evaluated by questionnaire results according to the Rome III criteria. RESULTS: We evaluated 4178 patients (350 were excluded because of incomplete data or previous colonic surgery), of whom 203 met the Rome III criteria (mean age 57.9 years; range 14-87 years) prior to the diagnostic colonoscopy examination. We identified organic colonic diseases in 21 of these 203 patients (10.3 %) , and these disease were also identified in 338 (8.5 %) of 3975 patients who did not fulfill the Rome III criteria. There were no differences in regard to the prevalence of organic colonic diseases between patients who did and did not fulfill the Rome III criteria. CONCLUSIONS: The prevalence of organic colonic diseases in patients who met the Rome III criteria was at an acceptably low level, indicating that the Rome III criteria are adequately specific for the diagnosis of IBS without performing a colonoscopy examination.

All-trans retinoic acid and a novel synthetic retinoid tamibarotene (Am80) differentially regulate CD38 expression in human leukemia HL-60 cells: possible involvement of protein kinase C-delta.

ATRA and a synthetic RAR agonist tamibarotene (Am80) induce granulocytic differentiation of human acute leukemia HL-60 cells and have been used in antineoplastic therapy. ATRA induces CD38 antigen during HL-60 cell differentiation, which interacts with CD31 antigen on the vascular EC surface and may induce disadvantages in the therapy. We here examined the mechanisms of the ATRA-mediated CD38 induction and compared the difference between ATRA- and tamibarotene-mediated induction. Tamibarotene-induced HL-60 cell adhesion to ECs was 38% lower than ATRA, and NB4 cell adhesion to ECs by tamibarotene was equivalent to ATRA, which induced CD38 gene transcription biphasically in HL-60 cells, the early-phase induction via DR-RARE containing intron 1, and the delayed-phase induction via RARE lacking the 5'-flanking region. In contrast to ATRA, tamibarotene induced only the early-phase induction, resulting in its lower CD38 induction than ATRA. A PKCδ inhibitor, rottlerin, and siRNA-mediated PKCδ knockdown suppressed the ATRA-induced CD38 promoter activity of the 5'-flanking region, whereas a RAR antagonist, LE540, or RAR knockdown did not affect it. Cycloheximide and rottlerin suppressed the delayed-phase induction of CD38 expression by ATRA but did not affect the early-phase induction. Moreover, ATRA, but not tamibarotene, induced PKCδ expression without affecting its mRNA stability. The diminished effect of tamibarotene on CD38-mediated HL-60 cell adhesion to ECs compared with ATRA is likely a result of the lack of its delayed-phase induction of CD38 expression, which may be advantageous in antineoplastic therapy.

Peroxisome proliferator-activated receptor-{gamma} suppresses CYP11B2 expression and aldosterone production.

Peroxisome proliferator-activated receptor-γ (PPARγ) is a nuclear receptor for the antidiabetic agent thiazolidinedione, which exerts various physiological activities, independent of lowering blood glucose. However, the role of PPARγ in aldosterone production has not been clarified. The objective of this study was to investigate the effect of PPARγ on aldosterone synthase gene (CYP11B2) expression and aldosterone production. Localization of PPARγ expression in normal adrenal cortex was determined by immunohistochemistry. Aldosterone production and CYP11B2 expression levels were determined using human adrenocortical carcinoma H295R cells. Pioglitazone suppressed angiotensin II-induced aldosterone secretion and CYP11B2 expression. PPARγ was expressed in zona glomerulosa in human normal adrenal gland. PPARγ overexpression enhanced pioglitazone-mediated CYP11B2 transrepression. The pioglitazone-mediated suppression of aldosterone secretion and CYP11B2 expression were canceled by PPARγ L466A/E469A mutant. Pioglitazone also suppressed potassium-mediated CYP11B2 induction, but not N6-2'-O-dibutyladenosine-3',5'-cyclic monophosphate stimulation. Rosiglitazone and GW1929 also suppressed CYP11B2 transactivation. Mutation analysis revealed that the Ad1/CRE element in CYP11B2 5'-flanking region was responsible for the pioglitazone-mediated transrepression. Pioglitazone suppressed ionomycin and a truncated constitutively active form Ca(2+)/calmodulin-dependent kinase I (CaMKI)-mediated CYP11B2 transcriptional activation. A CaMK inhibitor KN-93 attenuated pioglitazone-mediated CYP11B2 transrepression. PPARγ suppresses CYP11B2 expression and aldosterone secretion.