Functional Evaluation of Bubble CPAP for Neonates Using a Leak Model

Article信州医学雑誌 61(2):65-73(2013)journal articl

Rituximab-combination chemotherapy achieves a 10th cycle of remission for Burkitt's lymphoma.

A 14-year-old girl with multiple intra-abdominal tumors was diagnosed with stage III Burkitt's lymphoma. She achieved complete remission after multi-drug chemotherapy, but she relapsed after six courses. Autologous peripheral blood stem cells (PBSC) or allogeneic PBSC harvested from an HLA-identical sibling were insufficient, and her family did not agree to bone marrow collection from the sibling. Although the patient relapsed nine times (the relapses involved intra-abdominal organs or bone) during the following 4 years 7 months, treatment with rituximab monotherapy or in combination with ifosphamide, carboplastin, and etoposide, or local irradiation (33.8-40.0 Gy) to treat the bone metastases, proved effective, resulting in complete or partial remission. At the time of writing, the patient was in a 10th cycle of remission lasting 1 year 6 months and had not required transplantation. Thus, a chemotherapy regimen including rituximab might be effective for Burkitt's lymphoma in patients experiencing multiple relapse

Long-term efficacy of bevacizumab and irinotecan in recurrent pediatric glioblastoma.

A 5-year-old boy with glioblastoma relapsed soon after postoperative irradiation in combination with temozolomide. Second-line chemotherapy was also ineffective; therefore, the bevacizumab and irinotecan were given after a third gross-total resection of the tumor. Treatment was interrupted for 1 month due to development of posterior reversible encephalopathy syndrome, but was re-initiated at a lower dose of bevacizumab with prolonged intervals between treatments. The patient was alive and disease free 2 years after initial diagnosis. Bevacizumab and irinotecan are a promising regimen for pediatric cases of recurrent glioblastoma after gross-total resection, although the optimal treatment schedule must be determined on a patient-by-patient basis

CD146 is a potential immunotarget for neuroblastoma

Neuroblastoma, the most common extracranial solid tumor of childhood, is thought to arise from neural crest-derived immature cells. The prognosis of patients with high-risk or recurrent/refractory neuroblastoma remains quite poor despite intensive multimodality therapy; therefore, novel therapeutic interventions are required. We examined the expression of a cell adhesion molecule CD146 (melanoma cell adhesion molecule [MCAM]) by neuroblastoma cell lines and in clinical samples and investigated the anti-tumor effects of CD146-targeting treatment for neuroblastoma cells both in vitro and in vivo. CD146 is expressed by 4 cell lines and by most of primary tumors at any stage. Short hairpin RNA-mediated knockdown of CD146, or treatment with an anti-CD146 polyclonal antibody, effectively inhibited growth of neuroblastoma cells both in vitro and in vivo, principally due to increased apoptosis via the focal adhesion kinase and/or nuclear factor-kappa B signaling pathway. Furthermore, the anti-CD146 polyclonal antibody markedly inhibited tumor growth in immunodeficient mice inoculated with primary neuroblastoma cells. In conclusion, CD146 represents a promising therapeutic target for neuroblastoma

Mortality and pulmonary complications in patients undergoing surgery with perioperative SARS-CoV-2 infection: an international cohort study

Background: The impact of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) on postoperative recovery needs to be understood to inform clinical decision making during and after the COVID-19 pandemic. This study reports 30-day mortality and pulmonary complication rates in patients with perioperative SARS-CoV-2 infection. Methods: This international, multicentre, cohort study at 235 hospitals in 24 countries included all patients undergoing surgery who had SARS-CoV-2 infection confirmed within 7 days before or 30 days after surgery. The primary outcome measure was 30-day postoperative mortality and was assessed in all enrolled patients. The main secondary outcome measure was pulmonary complications, defined as pneumonia, acute respiratory distress syndrome, or unexpected postoperative ventilation. Findings: This analysis includes 1128 patients who had surgery between Jan 1 and March 31, 2020, of whom 835 (74·0%) had emergency surgery and 280 (24·8%) had elective surgery. SARS-CoV-2 infection was confirmed preoperatively in 294 (26·1%) patients. 30-day mortality was 23·8% (268 of 1128). Pulmonary complications occurred in 577 (51·2%) of 1128 patients; 30-day mortality in these patients was 38·0% (219 of 577), accounting for 81·7% (219 of 268) of all deaths. In adjusted analyses, 30-day mortality was associated with male sex (odds ratio 1·75 [95% CI 1·28–2·40], p\textless0·0001), age 70 years or older versus younger than 70 years (2·30 [1·65–3·22], p\textless0·0001), American Society of Anesthesiologists grades 3–5 versus grades 1–2 (2·35 [1·57–3·53], p\textless0·0001), malignant versus benign or obstetric diagnosis (1·55 [1·01–2·39], p=0·046), emergency versus elective surgery (1·67 [1·06–2·63], p=0·026), and major versus minor surgery (1·52 [1·01–2·31], p=0·047). Interpretation: Postoperative pulmonary complications occur in half of patients with perioperative SARS-CoV-2 infection and are associated with high mortality. Thresholds for surgery during the COVID-19 pandemic should be higher than during normal practice, particularly in men aged 70 years and older. Consideration should be given for postponing non-urgent procedures and promoting non-operative treatment to delay or avoid the need for surgery. Funding: National Institute for Health Research (NIHR), Association of Coloproctology of Great Britain and Ireland, Bowel and Cancer Research, Bowel Disease Research Foundation, Association of Upper Gastrointestinal Surgeons, British Association of Surgical Oncology, British Gynaecological Cancer Society, European Society of Coloproctology, NIHR Academy, Sarcoma UK, Vascular Society for Great Britain and Ireland, and Yorkshire Cancer Research

直腸粘膜下層浸潤(sm)癌リンパ節転移例および再発例に関する検討

[目的]直腸sm癌は,内視鏡切除で根治可能なものから外科切除後再発するものと多様で,外科切除の術式も,経肛門的腫瘍切除術から腹会陰式直腸切断術と多岐にわたり,術後QOLにも大きな差異が生ずる.そこで今回,直腸sm癌のリンパ節転移例および再発例の検討を行い,治療方針に関して考察した.[対象と方法]1988～2003年までに経験した直腸sm癌108例を初回治療別に,内祝鏡切除5例,局所切除11例および外科切除(リンパ節郭清を含めた腸管切除術)44例に分け,リンパ節転移例と再発例を検討した.[成績](1)内視鏡切除 : 53例中14例に追加腸切除を行い,リンパ節転移は1例(7.1%)みられた.この症例はIs病変(20mm)でnon-lifting sign陽性の分割摘除例(高分化腺癌,ly (+))であった.追加腸切除例に再発はなく,経過観察した39例中2例(5.1%)に再発を認め,いずれも分割切除例であった.(2)局所切除 : 11例中1例に追加腸切除を行った.経過観察した10例中,経肛門的切除の1例に2年後局所リンパ節再発を認めた.(3)外科切除 : 44例中リンパ節転移を4例(9%)に認めた.全例sm2以深癌でかつ脈管侵襲陽性であった.再発は2例(5%)に認めた.[まとめ]内視鏡切除および局所切除例で,sm2以深癌かつ脈管侵襲を有している場合は追加腸切除を考慮すべきで,とくに内視鏡的分割切除の適応は慎重にすべきである.sm癌はリンパ節転移術中診断は困難で,迅速病理診などを行い,転移陽性の場合は十分なリンパ節郭清を行うべきと考えられる.[Background] It has been possible to resect early colorectal cancer by endoscopy because of the progress of colonoscopic diagnosis and technology. Therefore most cases of colorectal mucosal cancer and benign tumor have been resected by endoscopy only. Surgical procedures for rectal submucosal invasive carcinoma include transanal tumor resection and abdominoperineal resection of the rectum. Therefore, there is a variation in quality of life. The aim of this study was to evaluate the management of rectal submucosal invasive carcinoma by analyzing lymph node metastases and recurrence of rectal submucosal carcinomas. [Methods] From 1988 to 2003, 108 patients who had rectal submucosal invasive carcinoma were studied clinicopathologically. Fifty-three patients underwent endoscopic resection (ER), 11 patients underwent local resection, and 44 patients underwent surgical resection with lymph node dissection. Patients with recurrence after resection and patients with lymph node metastasis after surgical resection were analyzed. [Results] (1) Of 53 patients who underwent ER, 14 underwent additional bowel resection, and one (7.1 %) had lymph node metastasis. Histopathological findings showed well-differentiated tubular adenocarcinoma and lymphatic invasion. No recurrence occurred in the patients who underwent additional bowel resection. Recurrence was seen in 2 (5.1%) of 39 patients who had been followed up without additional bowel resection. (2) Of 11 patients who underwent local resection, one had recurrence after transsacral tumor resection. (3) Of 44 patients who underwent surgical resection with lymph node dissection, 4 patients (9%) had lymph node metastasis. All 4 patients had submucosal invasion deeper than sm2 and positive lymphatic invasion. Recurrence after surgical resection occurred in 2 patients. [Conclusion] In patients with rectal submucosal invasive carcinoma, additional bowel resection after ER or local resection is thought to be indicated if histopathological findings reveal submucosal invasion deeper than sm2 and positive vascular invasion. Patients who underwent endoscopic piecemeal resection should be followed up carefully. It was often difficult to diagnose lymph node metastasis intra-operatively. Therefore, radical resection with adequate lymph node dissection for advanced cancer is indicated if lymph node metastasis is positive on quick histopathological diagnosis

直腸粘膜下層浸潤(sm)癌リンパ節転移例および再発例に関する検討

[目的]直腸sm癌は,内視鏡切除で根治可能なものから外科切除後再発するものと多様で,外科切除の術式も,経肛門的腫瘍切除術から腹会陰式直腸切断術と多岐にわたり,術後QOLにも大きな差異が生ずる.そこで今回,直腸sm癌のリンパ節転移例および再発例の検討を行い,治療方針に関して考察した.[対象と方法]1988～2003年までに経験した直腸sm癌108例を初回治療別に,内祝鏡切除5例,局所切除11例および外科切除(リンパ節郭清を含めた腸管切除術)44例に分け,リンパ節転移例と再発例を検討した.[成績](1)内視鏡切除 : 53例中14例に追加腸切除を行い,リンパ節転移は1例(7.1%)みられた.この症例はIs病変(20mm)でnon-lifting sign陽性の分割摘除例(高分化腺癌,ly (+))であった.追加腸切除例に再発はなく,経過観察した39例中2例(5.1%)に再発を認め,いずれも分割切除例であった.(2)局所切除 : 11例中1例に追加腸切除を行った.経過観察した10例中,経肛門的切除の1例に2年後局所リンパ節再発を認めた.(3)外科切除 : 44例中リンパ節転移を4例(9%)に認めた.全例sm2以深癌でかつ脈管侵襲陽性であった.再発は2例(5%)に認めた.[まとめ]内視鏡切除および局所切除例で,sm2以深癌かつ脈管侵襲を有している場合は追加腸切除を考慮すべきで,とくに内視鏡的分割切除の適応は慎重にすべきである.sm癌はリンパ節転移術中診断は困難で,迅速病理診などを行い,転移陽性の場合は十分なリンパ節郭清を行うべきと考えられる.[Background] It has been possible to resect early colorectal cancer by endoscopy because of the progress of colonoscopic diagnosis and technology. Therefore most cases of colorectal mucosal cancer and benign tumor have been resected by endoscopy only. Surgical procedures for rectal submucosal invasive carcinoma include transanal tumor resection and abdominoperineal resection of the rectum. Therefore, there is a variation in quality of life. The aim of this study was to evaluate the management of rectal submucosal invasive carcinoma by analyzing lymph node metastases and recurrence of rectal submucosal carcinomas. [Methods] From 1988 to 2003, 108 patients who had rectal submucosal invasive carcinoma were studied clinicopathologically. Fifty-three patients underwent endoscopic resection (ER), 11 patients underwent local resection, and 44 patients underwent surgical resection with lymph node dissection. Patients with recurrence after resection and patients with lymph node metastasis after surgical resection were analyzed. [Results] (1) Of 53 patients who underwent ER, 14 underwent additional bowel resection, and one (7.1 %) had lymph node metastasis. Histopathological findings showed well-differentiated tubular adenocarcinoma and lymphatic invasion. No recurrence occurred in the patients who underwent additional bowel resection. Recurrence was seen in 2 (5.1%) of 39 patients who had been followed up without additional bowel resection. (2) Of 11 patients who underwent local resection, one had recurrence after transsacral tumor resection. (3) Of 44 patients who underwent surgical resection with lymph node dissection, 4 patients (9%) had lymph node metastasis. All 4 patients had submucosal invasion deeper than sm2 and positive lymphatic invasion. Recurrence after surgical resection occurred in 2 patients. [Conclusion] In patients with rectal submucosal invasive carcinoma, additional bowel resection after ER or local resection is thought to be indicated if histopathological findings reveal submucosal invasion deeper than sm2 and positive vascular invasion. Patients who underwent endoscopic piecemeal resection should be followed up carefully. It was often difficult to diagnose lymph node metastasis intra-operatively. Therefore, radical resection with adequate lymph node dissection for advanced cancer is indicated if lymph node metastasis is positive on quick histopathological diagnosis

Pancreatic desmoid tumor in a 4-year-old male with hemihypertrophy

We report the first case of a pancreatic desmoid tumor detected during follow-up for hemihypertrophy in a 4-year-old boy. Hemihypertrophy is a rare disorder in which one side of the body grows more than the other, causing asymmetry, and well-known complications include embryonal tumors. However, there has been no report of desmoid tumors in patients with hemihypertrophy, and these tumors are rare and poorly characterized in the literature, especially the cystic variant. For this patient, the lesion was diagnosed as a desmoid tumor based on immunostaining positive for beta-catenin and mutation of the beta-catenin gene (CTNNB1). This case suggests that desmoid tumors should be considered a possible etiology of pancreatic cystic lesions in patients with hemihypertrophy

Treatment of hemolytic uremic syndrome related to Bordetella pertussis infection —is plasma exchange or eculizumab use necessary?

Abstract Background Bordetella pertussis infection is a known trigger of atypical hemolytic uremic syndrome (HUS). For patients suspected of having atypical HUS, prompt plasma exchange/infusion (PE/PI) or eculizumab (ECZ) treatment is recommended. Case presentation We report a 1-month-old female infant who was admitted with a severe cough and a B. pertussis-positive sputum culture. She was born at 38 weeks gestation and did not have a family history of renal diseases. Hemophagocytic syndrome was suspected and she was transferred to our hospital 17 days after her initial admission. One day later, she developed acute kidney injury and was diagnosed with HUS triggered by B. pertussis infection. Her plasma complement levels were low and her kidney function continued to worsen over the next few days. However, prior to starting ECZ treatment, her kidney function improved spontaneously; she did not receive PE/PI or ECZ. She was discharged 46 days after her initial hospitalization, without complications. A genetic workup revealed no mutations in CFH, CFI, CFB, C3, MCP, THBD, or DGKE. Conclusions This case demonstrates that B. pertussis infection-related HUS may resolve spontaneously. The decision to treat during the acute phase is challenging because B. pertussis often affects infants suspected of having atypical HUS. However, ECZ may not be the first treatment option for patients with B. pertussis infection-related HUS unless they show an indicated genetic abnormality; if ECZ is used, early discontinuation should be considered