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Rationale: Paraganglioma (PGL), an extra-adrenal pheochromocytoma, is a rare tumor, especially in children. While hypersecretion of catecholamines causes the classic triad of headaches, palpitations, and profuse sweating, prompt diagnosis is still challenging., Patient Concerns: For 7 months, an 8-year-old boy complained of polyuria and weight loss, followed by proteinuria and headache for 1 month prior to admission. He was admitted to our hospital due to an afebrile seizure., Diagnosis: His blood pressure remained markedly elevated even after cessation of the convulsion. Magnetic resonance imaging of the brain revealed posterior reversible encephalopathy syndrome. Abdominal computed tomography showed a mass lesion encasing the left renal artery, measuring 41 mm in length along its major axis. The plasma and urine levels of normetanephrine were elevated. Additionally, iodine-123-metaiodobenzylguanidine scintigraphy showed an abnormal uptake in the abdominal mass with no evidence of metastasis. Based on these findings, we tentatively diagnosed him with PGL., Intervention: Substantial alpha- and beta-blocking procedures were performed, followed by a tumor resection and an extended left nephrectomy on day 31 of hospitalization. Pathological findings confirmed the diagnosis of PGL., Outcome: The postoperative course was uneventful, and his blood pressure normalized without the use of antihypertensive agents. Genetic testing revealed a known SDHB germline mutation. The same mutation was also detected on his father and paternal grandfather without any history of hypertension or malignant tumor., Lesson: It remains challenging to diagnose pheochromocytoma/paraganglioma (PPGL) promptly because PPGL can present with a variety of symptoms. Preceding symptoms of the presented case might be caused by PGL. Although PPGL is a rare disease, especially in children, it should be considered in differential diagnosis when various unexplained symptoms persist., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.)

Emicizumab is a humanized anti-FIXa/FX (factor IXa/X) bispecific monoclonal antibody that mimics FVIIIa (activated factor VIII) cofactor function. The hemostatic efficacy of emicizumab has been confirmed in clinical studies of patients with hemophilia A, irrespective of the presence of FVIII inhibitors. Emicizumab differs in some properties from FVIIIa molecule. Emicizumab requires no activation by thrombin and is not inactivated by activated protein C, but emicizumab-mediated coagulation is regulatable and maintains hemostasis. A small amount of FIXa (activated factor IX) is required to initiate emicizumab-mediated hemostasis, whereas tissue factor/FVIIa (activated factor VII)-mediated FXa (activated factor X) and thrombin activation initiates FVIIIa-mediated hemostasis. Fibrin formation, followed by fibrinolysis, appears to be similar between emicizumab- and FVIIIa-mediated hemostasis. These results suggest possible future uses of emicizumab for treating hemorrhagic diseases other than hemophilia A and reveal previously unobservable behaviors of procoagulation and anticoagulation factors in conventional hemostasis. Here, we have reviewed novel insights and new developments regarding coagulation highlighted by emicizumab.

Purpose: The infiltration of macrophages in skeletal muscle during exhaustive exercise promotes inflammation, myofiber lesion, and muscle injury. Although neutrophils upregulate macrophage infiltration in skeletal muscles during exercise, the role of neutrophils in promoting muscle injury after exhaustive exercise remains unclear. In this study, we investigated the effects of preexercise neutrophil depletion with antineutrophil antibody treatment on muscle injury, inflammation, and macrophage infiltration after exhaustive exercise., Methods: Male C57BL/6J mice were randomly assigned to four groups, namely, sedentary with control antibody (n = 10), sedentary with antineutrophil antibody (n = 10), exhaustive exercise with control antibody (n = 10), and exhaustive exercise with antineutrophil antibody (n = 10). The mice were given intraperitoneal injection of the antineutrophil antibody (anti-Ly-6G, clone 1A8) or the control antibody (anti-Ly-6G, clone 2A3), and remained inactive or performed exhaustive exercise on a treadmill 48 h after the injection. Twenty-four hours after the exhaustive exercise, the gastrocnemius muscles were removed for histological and polymerase chain reaction (PCR) analyses. Infiltration of neutrophils and macrophages was evaluated with Ly-6G and F4/80 immunohistochemistry staining procedures. Muscle fiber injury was detected based on the number of IgG staining fiber. The mRNA expression levels of proinflammatory cytokines and chemokines were evaluated with real-time reverse transcription PCR., Results: Exhaustive exercise increased neutrophil infiltration into the gastrocnemius muscle substantially by 3.1-fold and caused muscle injury, but these effects were markedly suppressed by preexercise treatment with antineutrophil antibody (neutrophil infiltration, 0.42-fold, and muscle injury, 0.18-fold). Treatment with antineutrophil antibody also decreased macrophage infiltration (0.44-fold) and mRNA expression of tumor necrosis factor-α (0.55-fold) and interleukin-6 (0.51-fold) in the skeletal muscle after exhaustive exercise., Conclusion: These results suggest that neutrophils contribute to exacerbating muscle injury by regulating inflammation through the induction of macrophage infiltration.

The life expectancy continues to gradually increase worldwide. Laparoscopic liver resection (LLR) was recently reported to be a relatively safe procedure for treatment of liver disease because of the development of new techniques and instruments. The aim of this study was to evaluate the surgical outcomes of elderly patients who underwent LLR. Of these 61 patients who underwent LLR in our institute from January 2010 through April 2014, 21 were aged 75 years and above (group E), and 40 were aged below 75 years (group NE). Patient characteristics were retrospectively analyzed between the 2 groups. The results showed that patient characteristics were similar between the 2 groups, although the incidence of hypertension was significantly greater among patients in group E. There were no significant differences in the incidence of postoperative complications or length of hospital stay between the 2 groups. In conclusion, LLR for elderly patients was a feasible procedure compared with nonelderly patients.

The aim of this study was to use the difficulty score for a laparoscopic cholecystectomy procedure to predict the surgical difficulty of single-port laparoscopic cholecystectomy. From January 2009 to April 2011, single-port laparoscopic cholecystectomy was performed in 30 patients at our institution. The patients were evaluated using the difficulty score and classified into 3 groups: low, intermediate, and high difficulty. All surgeries were successfully completed without conversion to conventional laparoscopic surgery. A strong relationship was observed between the increasing score and longer surgical time. The mean surgical time was longer and the amount of blood loss was greater in the intermediate-difficulty and high-difficulty groups than in the low-difficulty group. Moreover, the high-difficulty group had a higher rate of insertion of an additional trocar than the low-difficulty group. Thus, the difficulty of single-port laparoscopic cholecystectomy is well predicted using the difficulty score.

Objective: Pancreatic ductal carcinoma frequently recurs postoperatively, and we analyzed clinicopathological features of patients treated by surgical resection to find predictors of postoperative recurrence., Methods: A retrospective cohort study was performed that included 69 patients between 1985 and 2003. Clinicopathologic factors were evaluated for tumor recurrences by univariate and multivariate analyses., Results: Mean survival time and actuarial 5-year disease-specific survival were significantly lower in cases of hepatic metastasis (13 months, 0%) and in cases of peritoneal carcinomatosis (15 months, 6.8%) than in cases of local retroperitoneal recurrence (30 months, 21%). Univariate and logistic regression analyses showed undifferentiated adenocarcinoma to be independently associated with hepatic metastasis (odds ratio, 7.4; 95% confidence interval, 1.5-37.0) and invasion of the portal vein to be independently associated with peritoneal carcinomatosis (odds ratio, 4.0; 95% confidence interval, 1.2-12.8). Multivariate analysis showed undifferentiated adenocarcinoma, invasion of the anterior capsule, and invasion of the portal vein to be independent prognostic factors., Conclusion: Undifferentiated adenocarcinoma and invasion of the portal vein are predictors of poor outcome and are related to hepatic metastasis and peritoneal carcinomatosis, respectively. Postoperative adjuvant chemotherapy, including intra-arterial chemotherapy, should be selected according to prediction of the patterns of recurrence.

We investigated the expression of CD10 by an immunohistochemical method in 51 basal cell carcinomas (BCCs), eight pilomatricomas, five trichoblastomas, two trichofolliculomas, three sebaceomas, five sebaceous carcinomas, ten syringomas, two spiradenomas, ten poromas, four porocarcinomas, one eccrine duct carcinoma (not otherwise specified, NOS), six mixed tumors of apocrine origin, and nine squamous cell carcinomas (SCCs). We detected strong expression of CD10 in tumor cells of BCC (86%), and found that the smaller the number of positive tumor cells, the larger the number of positive stromal cells, in particular in sclerosing BCCs. Spearman's rank correlation test revealed a significant negative correlation in BCCs between the expression of CD10 in tumor cells and that in stromal cells (P = 0.001). In all pilomatricomas (100%) and in four trichoblastomas (80%), strong expression was also detected in tumor cells. There was no detectable expression in trichofolliculomas. One sebaceoma (33%) and two sebaceous carcinomas (40%) expressed CD10 in a similar fashion to BCCs. All tumors of eccrine gland origin, including syringoma, spiradenoma, poroma, porocarcinoma, and eccrine duct carcinoma (NOS), did not express CD10. Five mixed tumors (83%) were immunopositive. In SCC, CD10 was overexpressed only in the stromal cells. These findings support the hypothesis that BCC is derived from the folliculo-sebaceous apocrine unit, especially having the same origin as trichoblastoma and pilomatricoma. CD10 might be an indicator of tumor invasiveness if it is expressed in stromal cells, while it might be a marker of follicular differentiation if it is expressed in the actual tumor cells of cutaneous epithelial neoplasms.

Study Design: This study analyzed biomechanical characteristics of the cervical spinal cord, especially in relation to neck flexion. Intramedullary pressure was measured in different neck positions., Objectives: The results provided a rationale for dynamic changes in intramedullary pressure, with the flexed neck position playing a role in syrinx growth., Summary of Background Data: Dynamic changes in intramedullary pressure in the flexed position have been postulated to play an important role in syrinx growth. However, intramedullary pressure of the spinal cord has not been measured., Methods: The authors designed a balloon method to assess, experimentally, intramedullary pressure dynamics of the spinal cord. A system was incorporated to examine the reliability of the balloons. Using 15 mongrel dogs, two balloons were embedded in the cervical spinal cord. Intramedullary pressure of the spinal cord was measured in several neck positions. In 5 of them, the same measurements were repeated when the spinal cord and roots were transected., Results: When filled with a suitable volume of water, the balloons faithfully transmitted the pressure of the environment. No pressure differences were observed with the neck in the extended or neutral positions. However, when the neck was flexed, intramedullary pressure significantly increased. This increase in intramedullary pressure in the flexed neck position was not observed after spinal cord and roots were transected., Conclusion: The results indicated that the intramedullary pressure of the cervical spinal cord increases when the neck is flexed. This phenomenon might play an important role in syrinx growth.