Dabrafenib and steroids for the treatment of Erdheim-Chester disease with extensive CNS involvement: a case report

Erdheim-Chester disease (ECD) is an exceedingly rare non-Langerhans cell CD68+ CD1a- S100- histiocytic multi-organ disease. Diagnosis of ECD is often delayed due to non-specific radiographic findings and heterogeneous lesional tissue. Increasingly, the role of genomic alterations is being recognized for both diagnosis and treatment of ECD. More than half of ECD patients harbor the BRAFV600E mutation. Evaluation for this mutation be can falsely negative on immunohistochemical staining and confirmation with molecular analyses is recommended. We present a case of the 44 year-old male with BRAFV600E-positive ECD treated successfully with steroids followed by single-agent dabrafenib

Liver transplantation in children: The experience of Queen Mary Hospital, Hong Kong

Seven living-related liver transplants (LRLT) and two reduced-size liver transplants (RSLT) were performed on eight children who suffered from end- stage liver disease, having previously undergone one to three abdominal operations. Their ages at initial transplantation ranged from 8 months to 11 years (mean 35 months, median 12 months). Excluding the two older children aged 7 and 11 years, respectively, the rest of the children weighed 6 to 9.5 kg (mean 7.3 kg) at the time of the initial transplantation. Seven left lateral segments (S2 + 3) and two left lobes (S2 + 3 + 4) were used; of these the smallest graft had a graft-to-recipient body weight ratio of 0.9%. The volunteer living donors were four mothers, two fathers and one sister who were selected after medical and psychiatric evaluations, and their suitability was confirmed by hematological, biochemical, and radiological criteria. During a follow-up period of 3 to 30 months, all eight children are alive and well with normal liver function, one of them having undergone a retransplant LRLT because of hepatitis of undetermined etiology following a RSLT 1.5 years earlier. All seven donors had an uneventful postoperative course and were discharged on day 4 to 7 postoperatively. They have all resumed normal day-to-day activities. There were no complications in the donor group. A variety of complications occurred in the recipients, all of which were overcome. Operating microscope was used to perform all the arterial anastomoses using microvascular techniques. This method has proven to be a major factor in preventing arterial thrombosis even with the smallest of arterial anastomosis where a 1.5-mm diameter recipient artery was anastomosed to a 2.5-mm diameter donor hepatic artery.link_to_subscribed_fulltex

Protocol driven assessment programme effectively shortens new case waiting time

Service Priorities and Programmes Free Papers: SPP4.6 Quality and Safety in Healthcare 1Conference Theme: Consolidating Health Care - 固本培員, 健行不息Introduction: The waiting time for new cases in endocrine clinic has been rising due to increasing demand. The average waiting time has increased to 26.3 ± 5.5 weeks in February 2012. To improve the situation, a protocol driven assessment (PDA) programme has been established and incorporated into the triage pathway starting from April 2012. Objectives: (1) To shorten the waiting time of new case at the Endocrine Clinic; and (2) to enhance efficient work flow of triage system and improve patient care. Methodology: Protocols for endocrine diseases including obesity, hyperprolactinaemia, hypercalcaemia, hypopituitarism and hypogonadism were developed. Patients referred for such conditions were triaged to the PDA programme in which history taking, assessments, investigations and early interventions were carried out according to the protocols set by an endocrine nurse and subsequently followed by endocrinologists. Results: 225 referrals were screened from May 2012 to December 2012. 64 patients were triaged to the programme. The referralto-nurse and referral-to-endocrinologist times for the programme were 5.9 ± 4.9 and 9.8 ± 5.3 weeks respectively. This showed a significant improvement when compared with their original referral-to-endocrinologist time (26.6 ± 5.7 weeks, p<0.05). Referral-to-endocrinologist time for patients not recruited into the programme also showed significant improvement (10.3 ± 9.0 vs. 26.2 ± 5.4 weeks, p< 0.05) and the PDA programme was one of the measures that contributed to this success. Early intervention has been initiated during the initial nursing assessment of the programme. 18 obese patients received prompt referrals to relevant allied health disciplines before assessments by specialists. Four patients, referred for hyperprolactinaemia, had normal serum prolactin level after re-checked by endocrine nurse under a controlled non-stressed condition. They could be discharged from clinic at the first specialist assessment. More urgent conditions, such as visual field defects and high blood pressure, had also been detected early in the nursing assessment stage and resulted in prompt treatment action. The establishment of PDA programme, conducted by an experienced nurse, not only shortens waiting time but also provides patients with a more streamlined, timely and efficient model of care

Synthesis, morphology, and properties of hydroxyl Terminated-POSS/polyimide low-k nanocomposite films

Polyhedral oligomeric silsequioxane (POSS), having eight hydroxyl groups for the preparation of nanocomposites with polyimide (PI) was synthesized by the direct hydrosilylation of allyl alcohol with octasilsesquioxane (Q 8M8H) with platinum divinyltetramethyl disiloxane Pt(dvs) as a catalyst. The structure of allyl alcohol terminated-POSS (POSS-OH) was confirmed by FTIR, NMR, and XRD. A high performance, low-k PI nanocomposite from pyromellitic dianhydride (PMDA)-4,4'-oxydianiline (ODA) polyamic acid cured with POSS-OH was also successfully synthesized. The incorporation of POSS-OH into PI matrix reduced dielectric constant of PI without loosing mechanical properties. Furthermore, the effects of POSS-OH on the morphology and properties of the PI/POSS-OH nanocomposites were investigated using UV-vis, FTIR, XRD, SEM, AFM, transmission electron microscope (TEM), TGA, and contact angle. The homogeneous dispersion of POSS particles was confirmed by SEM, AFM, and TEM. The nanoindentation showed that the modulus increased upon increasing the concentration of POSS-OH in PI, whereas the hardness did not increase very much with respect to loading of POSS, due to soft-interphase around POSS molecules in the resulting nanocomposites. Overall results demonstrated the nanometer-level integration of the polymer and POSS-OH. © 2008 Wiley Periodicals, Inc

Il sud-est asiatico nell'anno della tigre. Rapporto 1987 sulla situazione sociale, politica ed economica dell'area

Approfondito rapporto sulla situazione politica ed economica del sud-est asiatico e dei paesi componenti, dopo un periodo di profondi mutamenti geopolitici ed economici dell'area.- Indice #7- Prefazione, Marcello Pacini #11- Introduzione, Kernial S. Sandhu #15- La regione #17- L’anno della Tigre: le grandi tendenze nel Sud-est asiatico, Mohammed Ayoob #19- L’Asia del Sud-est tra cambiamento, continuità e sfide, Muthiah Alagappa #23- Le economie di mercato dell’Asia del Sud-est, Lee Soo Ann #61- Cooperazione economica nell’ASEAN. Circoli viziosi o nuovi orizzonti?, Hans Christoph Rieger #77- Problemi demografici dell’Asia del Sud-est, S. Gunasekaran #95- Tendenze recenti dell’urbanizzazione nella regione dell’ASEAN, Trinidad S. Osteria #123- Birmania #155- La Birmania nell’anno del Serpente, Mya Than #157- Brunei #193- Il Brunei Darussalam. Alla ricerca del “Regno Politico”, K. U. Menon #195- Cambogia #223- La Cambogia. L’ottavo anno della Repubblica Popolare, Tim Huxley #225- Filippine #245- La rivoluzione incompiuta delle Filippine, David G. Timberman #247- La ripresa economica delle Filippine. Alcuni problemi, Aurora Sanchez #287- Indonesia #301- L’Indonesia di Suharto dopo due decenni, Leo Suryadinata #303- L’economia indonesiana e la crisi del mercato del petrolio, Djisman S. Simandjuntak #327- Laos #347- Il Laosnel secondo decennio della ricostruzione nazionale, Ng Shui Meng #349- Malaysia #377- La Malaysia in un contesto recessivo. Rassegna generale, Mohamed Ariff #379- Le elezioni parlamentari nella Malaysia Peninsulare, S. Sothi Rachagan #415- Singapore #447- Singapore. Panorama politico e sociale, Wan Hussin Zoohri #449- L’economia di Singapore. Problemi e prospettive, Ng Chee Yuen #473- Tailandia #495- La Tailandia. Ancora cambiamento e continuità, Ananda Rajah #497- Il cambiamento della leadership militare ed il suo impatto sulla politica tailandese, Suchit Bunbongkarn #529- Vietnam #553- Il Partito Comunista Vietnamita. Iniziative per la riforma del Partito, la lotta per la ripresa economica e la via per il Sesto Congresso Nazionale, Lewis M. Stern #55

Assessment, endoscopy, and treatment in patients with acute severe ulcerative colitis during the COVID-19 pandemic (PROTECT-ASUC): a multicentre, observational, case-control study

BackgroundThere is a paucity of evidence to support safe and effective management of patients with acute severe ulcerative colitis during the COVID-19 pandemic. We sought to identify alterations to established conventional evidence-based management of acute severe ulcerative colitis during the early COVID-19 pandemic, the effect on outcomes, and any associations with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection and severe COVID-19 outcomes. MethodsThe PROTECT-ASUC study was a multicentre, observational, case-control study in 60 acute secondary care hospitals throughout the UK. We included adults (≥18 years) with either ulcerative colitis or inflammatory bowel disease unclassified, who presented with acute severe ulcerative colitis and fulfilled the Truelove and Witts criteria. Cases and controls were identified as either admitted or managed in emergency ambulatory care settings between March 1, 2020, and June 30, 2020 (COVID-19 pandemic period cohort), or between Jan 1, 2019, and June 30, 2019 (historical control cohort), respectively. The primary outcome was the proportion of patients with acute severe ulcerative colitis receiving rescue therapy (including primary induction) or colectomy. The study is registered with ClinicalTrials.gov, NCT04411784. FindingsWe included 782 patients (398 in the pandemic period cohort and 384 in the historical control cohort) who met the Truelove and Witts criteria for acute severe ulcerative colitis. The proportion of patients receiving rescue therapy (including primary induction) or surgery was higher during the pandemic period than in the historical period (217 [55%] of 393 patients vs 159 [42%] of 380 patients; p=0·00024) and the time to rescue therapy was shorter in the pandemic cohort than in the historical cohort (p=0·0026). This difference was driven by a greater use of rescue and primary induction therapies with biologicals, ciclosporin, or tofacitinib in the COVID-19 pandemic period cohort than in the historical control period cohort (177 [46%] of 387 patients in the COVID-19 cohort vs 134 [36%] of 373 patients in the historical cohort; p=0·0064). During the pandemic, more patients received ambulatory (outpatient) intravenous steroids (51 [13%] of 385 patients vs 19 [5%] of 360 patients; p=0·00023). Fewer patients received thiopurines (29 [7%] of 398 patients vs 46 [12%] of 384; p=0·029) and 5-aminosalicylic acids (67 [17%] of 398 patients vs 98 [26%] of 384; p=0·0037) during the pandemic than in the historical control period. Colectomy rates were similar between the pandemic and historical control groups (64 [16%] of 389 vs 50 [13%] of 375; p=0·26); however, laparoscopic surgery was less frequently performed during the pandemic period (34 [53%] of 64] vs 38 [76%] of 50; p=0·018). Five (2%) of 253 patients tested positive for SARS-CoV-2 during hospital treatment. Two (2%) of 103 patients re-tested for SARS-CoV-2 during the 3-month follow-up were positive 5 days and 12 days, respectively, after discharge from index admission. Both recovered without serious outcomes. InterpretationThe COVID-19 pandemic altered practice patterns of gastroenterologists and colorectal surgeons in the management of acute severe ulcerative colitis but was associated with similar outcomes to a historical cohort. Despite continued use of high-dose corticosteroids and biologicals, the incidence of COVID-19 within 3 months was low and not associated with adverse COVID-19 outcomes