Response to cyclophosphamide and rituximab therapy in idiopathic inflammatory myopathies: A single center experience

Aim of the workTo evaluate the clinical response to cyclophosphamide (CYC), and rituximab (RTX) following CYC regarding muscle power and creatine kinase (CK) level changes, and the ability to manage cardiac and bulbar affection in dermatomyositis (DM) and polymyositis (PM) patients.Patients and methodsTwenty two idiopathic inflammatory myopathies (IIM) patients: 14 (63.6%) DM and 8 (36.4%) PM were categorized into CYC group (n = 17) received only CYC, and RTX group received RTX following refractory CYC (n = 5). History taking and clinical examination were done, and Medical Research Council Sum Score (MRC-SS) and CK were evaluated at baseline visit, by end of monthly CYC, and 18 months after CYC or RTX. Improvement of cardiac affection or bulbar manifestations was checked.ResultsPatients were 16 females and 6 males (F:M 2.7:1) and mean age was 34.9 ± 14.9 years. The patients were followed up for 7.7 ± 4.3 years. MRC–SS increased in (CYC group) and in (RTX group) (p < 0.001, and p = 0.018 respectively). CK levels decreased significantly in CYC group (p < 0.001) but not in RTX group. MRC-SS changes in males, females, DM, PM patients were (p = 0.007, p < 0.001, p < 0.001, p = 0.006 respectively), and CK changes (p = 0.17, p < 0.001, p < 0.001, p = 0.006 respectively). The two DM cardiac patients and the three PM patients with bulbar manifestations in (RTX group) showed improvement in their extra-muscular manifestations.ConclusionCYC can be an effective drug in the treatment of IIM muscle affection. RTX may be promising for refractory myositis, cardiac and bulbar manifestations, so these patients should be a target for its early administration.     

Myeloablative therapy and bone marrow transplantation in Jehovah's Witnesses with malignancies: single center experience

Hematological malignancies in Jehovah's Witnesses are often difficult to cure since these patients deny transfusions. By a retrospective analysis, we report the possibility of treating some tumors, mostly hematological, with either autologous or allogeneic bone marrow transplantation (BMT) without blood support. Eight patients were evaluated, including lymphoma (two patients), acute lymphoblastic (one patient) and myeloblastic (one patient) leukemia, chronic lymphocytic leukemia (one patient), refractory anemia with blasts in transformation (one patient), chronic myeloid leukemia (one patient) and metastatic breast cancer (one patient). All patients experienced a severe cytopenia with no major side effects or life-threatening complications. We had four deaths: three from relapse and progression of the disease (at 5, 8 and 15 months after the stem cell infusion), and one from acute intestinal GVHD (at 2 months after the stem cell infusion). Four patients are in complete clinical remission (at 8, 10, 16 and 26 months after the stem cell infusion), and this was related to the disease outcome. We conclude that autologous and allogeneic BMT are feasible without the support of transfusions. We believe that this should be performed as soon as possible in the course of the disease.     

Recurrence after microwave ablation of liver malignancies: a single institution experience

BackgroundMicrowave ablation (MWA) is increasingly used to achieve local control for liver tumours. This study sought to examine a monocentric experience with MWA, with a primary hypothesis that primary tumour histology was a significant predictor of early recurrence.MethodsRetrospective single‐institution review identified consecutive patients with liver tumours treated by MWA. Cox proportional hazards models assessed significance of prognostic variables.ResultsSeventy‐two patients (43 female, 60%) underwent 83 MWA procedures for 157 tumours. Tumour histologies included hepatocellular cancer (10 operations), colorectal metastases (39), metastatic carcinoid (20) and other (14). The median tumour size was 2.0 cm. A concomitant liver resection was performed in 50 cases (60%). Crude peri‐operative morbidity and mortality rates were 16% and 1%, respectively. The median follow‐up was 16 months. Ablations were complete for 149 out of 157 tumours (95%). The median overall and recurrence‐free survivals were 36 and 18 months, respectively. There was no difference in time to recurrence between the primary tumour types. In multivariable models, recurrence‐free survival was independently associated with the use of neoadjuvant [hazard ratio (HR): 2.90, 95% confidence interval (CI): 1.09–7.76, P = 0.034] and adjuvant chemotherapy (HR: 0.36, 95% CI: 0.15–0.82, P = 0.016).ConclusionsMWA is a safe and feasible approach for local control of liver tumours. While chemotherapy administration was associated with time to recurrence after MWA, larger studies are needed to corroborate these findings.     

Juvenile onset systemic sclerosis: a single center experience of 23 cases from Asia

The aim of this paper was to study the spectrum of juvenile scleroderma (JSSc) seen at a tertiary care referral center in Asia. Retrospective analysis of case records of patients with systemic sclerosis, having age of onset less than 16 years and seen at our hospital from 1988 to 2004, was done. Patients with linear scleroderma and morphea were excluded. There were 23 patients (19 girls, 4 boys) with median age of onset of 12 years (range 5–16 years). The median age at presentation was 17 years (range 10–34 years). The median time from first symptoms to presentation was 4 years (range 0.2–26 years). Among these, 14 had diffuse systemic sclerosis (DSSc), while 9 had limited scleroderma (LSSc). The clinical features seen at presentation in patients were: Raynaud’s phenomenon in 19, digital ulcers in 14, loss of finger tip pulp in 12, reflux in 8, dysphagia in 7, arthritis in 8, digital gangrene in 2, and pulmonary artery hypertension in 1. Antinuclear antibody was positive in 15 out of 18 patients tested. Interstitial lung disease was seen in 15 patients, 6 of whom had diffuse disease. The median skin score was 22 (range 7–48) .One patient died of primary pulmonary hypertension within 1 year of onset of symptoms. At a mean follow-up of 34 months, 14 patients were stable or had improvement in skin score or dyspnea on exertion. DSSc and LSSc in childhood have a clinical presentation similar to adult patients, with cardiopulmonary involvement being the major predictor of outcome. The short-term prognosis of JSSc is good.     

Duodenal GIST: a single center experience

Purpose

The duodenum as primary site for gastrointestinal stromal tumors (GISTs) is rare and mitotic rate, tumor size, type of mutation and number of chromosomal aberrations have prognostic implications.

Methods

We analyzed the outcome of 13 patients with duodenal GISTs who underwent surgical tumor resection. Either segmental duodenectomy or pylorus-preserving duodenopancreatectomy was performed. The tumors were histopathologically examined and the risk of progression was assessed based on tumor size and mitotic count. Additionally, mutation analysis of the KIT and PDGFRA receptor tyrosine kinase genes and comparative genomic hybridization (CGH) were performed in all cases.

Results

Eight patients underwent segmental duodenectomy and five patients were treated with pylorus-preserving duodenopancreatectomy. None of the five GISTs with low or no risk for malignancy according to the Miettinen classification developed tumor progress. In contrast, five of eight cases (62.5%) with high-risk tumors revealed tumor progress, and four of these patients died (50%). The median overall survival for all patients was 66 months, and the median disease-free survival 41 months. The operative procedure and type of mutation did not correlate with long-term survival. CGH analysis displayed ?15q in 12/13 tumors, and ?1p in 11/13 cases as characteristic chromosomal aberrations for intestinal origin. Notably, ?22q was present in three of four cases with tumor progress.

Conclusions

Both segmental duodenectomy and pylorus-preserving duodenopancreatectomy are appropriate options to treat duodenal GIST and should be implemented depending on resectability and the patient's performing state. The Miettinen classification and CGH findings correlate with the clinical course.     

Candidemia in patients with hematologic malignancies: analysis of 7 years' experience in a single center

We report 45 incidents of candidemia in 45 patients diagnosed with hematologic malignancies between 1997 and 2004. A large majority of species isolated were non-albicans and there was an unexpectedly high incidence of Candida tropicalis. The attributable mortality (15%) was interestingly low in this population of severely immunocompromised patients.     

Pneumatosis cystoides intestinalis: a single center experience

AIM: To share our experience of the management and outcomes of patients with pneumatosis cystoides intestinalis (PCI).METHODS: The charts of seven patients who underwent surgery for PCI between 2001 and 2009 were reviewed retrospectively. Clinical features, diagnoses and surgical interventions of patients with PCI are discussed.RESULTS: Seven patients with PCI (3 males, 4 females; mean age, 50 ± 16.1 years; range, 29-74 years) were analyzed. In three of the patients, abdominal pain was the only complaint, whereas additional vomiting and/or constipation occurred in four. Leukocytosis was detected in four patients, whereas it was within normal limits in three. Subdiaphragmatic free air was observed radiologically in four patients but not in three. Six of the patients underwent an applied laparotomy, whereas one underwent an applied explorative laparoscopy. PCI localized to the small intestine only was detected in four patients, whereas it was localized to the small intestine and the colon in three. Three patients underwent a partial small intestine resection and four did not after PCI was diagnosed. Five patients were diagnosed with secondary PCI and two with primary PCI when the surgical findings and medical history were assessed together. Gastric atony developed in one case only, as a complication during a postoperative follow-up of 5-14 d.CONCLUSION: Although rare, PCI should be considered in the differential diagnosis of acute abdomen. Diagnostic laparoscopy and preoperative radiological tests, including computed tomography, play an important role in confirming the diagnosis.     

应用激光鞘拔除电极导线(附五例报道)

目的探讨应用激光鞘拔除电极导线的安全性、可行性。方法回顾分析北京大学人民医院应用激光鞘拔除电极导线的5例患者资料,总结电极导线拔除原因、电极导线特点、手术相关并发症、手术时间、曝光时间、曝光量等。结果 5例患者(2例女性),年龄(62.8±16)岁,其中1例为Ⅱa类适应证,4例为I类适应证,共拔除电极导线8根(2根除颤电极导线),均成功拔除,无并发症发生,手术时间、曝光时间、曝光量的中位数分别为4 min、1min 35 s、1.305 mgy。结论对于大的、有经验的中心,应用激光鞘拔除电极导线是安全、可行的。     

Amiodarone therapy for atrial rhythm control: insights gained from a single center experience

Introduction: Amiodarone has been advocated as an effective "long-term" therapy for atrial rhythm control in patients with atrial fibrillation (AF). We sought to assess the sustainability of this therapeutic strategy.
Methods and Results: Retrospective analysis of a consecutive, single-center cohort (n = 168) with symptomatic AF who were treated with amiodarone and followed for 3 years. The incidence of amiodarone cessation was evaluated at 1, 2, and 3 years and attributed principally to drug inefficacy, intolerance, or toxicity. A gradual diminution in the number of patients on therapy was observed, such that by 3 years, only 45% remained. This was attributable to inefficacy (25%), intolerance (12%), or toxicity (18%). Pulmonary toxicity was surprisingly common, occurring in at least 7% of patients.
Conclusions: These data challenge the notion of amiodarone as a reasonable "destination" therapy for AF.     

Microscopic colitides: a single center experience in Mexico

Background/aim Microscopic colitis is characterized by chronic diarrhea and specific microscopic changes in a macroscopically normal colonic mucosa. In this paper, we report the clinical and histological features of a group of Mexican patients with microscopic colitis. Materials and methods Patients were identified from 10 years of data in the pathology database of our institution. A pathologist reassessed all biopsy material. We included only patients who met strict histopathological criteria. Clinical data were obtained from medical records. Results Microscopic colitis was diagnosed in 26 cases. The female/male ratio was 1.8:1. The median age at diagnosis was 56.5 ± 15.7 (range, 26–85) years. The most frequent symptoms were diarrhea in all, weight loss in 22 (84%), and abdominal pain in 18 (69%) patients. Sixteen (61%) patients reported associated diseases; autoimmune thyroid disorders were the most common. Drug-induced microscopic colitis was suspected in 12 (46%) patients. More than 80% of the treated patients improved or had clinical remission. Conclusion Microscopic colitis is an uncommon colonic disease at our institution, representing less than 1% of the final diagnoses in mucosal colonic biopsies. It is the largest case series of microscopic colitis in the Mexican population.     

Causes of mortality after liver transplantation: a single center experience in mainland china

Background/Aims: To analyze the causes of death, both in the short- and long-term after cadaveric liver transplantation (LT) from a single center in mainland China. Methodology: Clinical data of 472 adult patients who underwent primary cadaveric LT from February 1999 to December 2009 at our center were identified. All patients were followed-up until December 2010. Mortalities in three periods were described: the postoperative mortality (within the first 90 postoperative days or more if the patient remained hospitalized); the early mortality (death between 4-12 months after transplantation); the late mortality (death after one year following transplantation). Results: The survival of the 472 patients at 3 months, 1, 3 and 5 years was 85.2%, 73.5%, 61.2% and 59.7%, respectively. The postoperative mortality, the early mortality and the late mortality were 34.8% (70/201), 27.4% (55/201) and 37.8% (76/201), respectively. Infection, multi-organ failure and technical complications were the major causes of death during the first period, and the recurrence of malignancy was the main cause of death during the early and late periods after LT. Conclusions: Recurrence of malignancy was the major cause of the early and late mortality in our study, which is higher than that of other reports. The serious preoperative condition of the recipient, the waiting timing for the operation and the tumor selection criteria for LT may account for this.     

Rituximab: rescue therapy in life-threatening complications or refractory autoimmune diseases: a single center experience

Rituximab (RTX) is a chimeric anti-CD20 antibody, approved for rheumatoid arthritis (RA) patients who failed anti-Tumor Necrosis Factor therapy. It has been used occasionally for life-threatening autoimmune diseases (AID). We report our center experience in the use of RTX in life-threatening complications or refractory AID. Clinical charts of patients treated with RTX at our center were reviewed, cases treated for life-threatening complications or refractory AID were analyzed. Acute damage to vital organs such as lung, heart, kidney, nervous system with severe functional impairment were defined as life-threatening complications; treatment failure with high-dose corticosteroids, cyclophosphamide, IVIG, plasmapheresis was defined as refractory autoimmune disease. During the years 2003–2009, 117 patients were treated with RTX, most of them for RA. Nine patients (6 females, mean age 51.5 years, mean disease duration 6.3 years) answered the criteria. The indications were as follows: pulmonary hemorrhage (1 patient with cryoglobulinemic vasculitis, 1 with systemic sclerosis, 1 with ANCA-associated vasculitis), catastrophic anti-phospholipid syndrome (2 SLE patients), non-bacterial endocarditis and pulmonary hypertension (1 patient with mixed connective tissue disease), vasculitis and feet necrosis (1 patient with systemic lupus erythematosus), severe lupus demyelinative neuropathy and acute renal failure (1patient), and severe rheumatoid lung disease with recurrent empyema and pneumothorax (1patient). B cell depletion was achieved in all patients. The median time since starting of complications to RTX administration was 3 weeks (range 2–15 weeks). Complete remission (suppression of the hazardous situation and return to previous stable state) was seen in 7 out of 9 patients. Partial remission (significant improvement) was achieved in the remained. The median time to response was 3 weeks (range 1–8 weeks), mean follow-up 47.2 months (range 6–60 months). A rapid tapering off of steroids was achieved in all patients. Two patients relapsed and were successfully retreated with RTX: the patient with severe RA lung relapsed after 3 years, one of the patients with ANCA-associated pulmonary alveolar hemorrhage relapsed after 10 months. There were no side effects during RTX infusion. Two episodes of serious infections were registered: fatal Gram-negative sepsis 6 months after RTX treatment, and septic discitis 4 months after receiving RTX. RTX serves as a safe, efficient, and prompt rescue therapy in certain life-threatening conditions and resistant to aggressive immunosuppression AID. RTX when administrated at an earlier stage, prevented irreversible vital organ damage, and allowed rapid steroid tapering off in already severe immunodepressed patients.     

RETRACTED ARTICLE: Unicompartmental knee arthroplasty: a review of literature

There has been a resurgence of interest in unicompartmental knee arthroplasty (UKA) for treatment of medial unicompartmental knee osteoarthritis (OA). Improved prosthetic design, minimally invasive surgical techniques, and strict patient selection criteria have resulted in improved survivorship and functional outcomes. A review of orthopedic literature was conducted regarding the advantages of UKA versus total knee arthroplasty (TKA), UKA indications, survivorship, conversion of UKA to TKA, rehabilitation, and outcomes. The UKA appears to be a viable option for patients with knee medial compartment OA, including younger and active patients. Survivorship rates of 94% to 97% at 10 years have been reported.     

Systemic sclerosis after interferon alphacon-1 therapy for hepatitis C

A 66-year-old woman developed systemic sclerosis (SSc) after receiving interferon alphacon-1 (IFNalphacon-1; consensus interferon) therapy for chronic hepatitis C. She suffered from Raynauds phenomenon about 1 year after a course of IFNalphacon-1 therapy. The combination of proximal scleroderma, Raynauds phenomenon, and ANA positivity led to a diagnosis of systemic sclerosis (SSc). IFN has multiple effects on the immune system and is known to trigger the development of autoantibodies, as well as the onset or exacerbation of autoimmune disease. We suspect that the immunomodulatory effects of IFNalphacon-1 triggered the clinical manifestations of SSc in this patient. To our knowledge this is only the third case of SSc after IFNalpha therapy, and it is the first case associated with IFNalphacon-1 therapy.