Paraganglioma of the larynx

A laryngeal paraganglioma in a 58 years female is reported with a reference to 12 cases reported in literature.     

German populations with infrequent CHEK2*1100delC and minor associations with early-onset and familial breast cancer

CHEK2*1100delC is associated with a twofold increased breast cancer risk. This was shown in a collaborative analysis of European populations, but not in other populations from Europe and the US. Accordingly, there is a need to clarify the role of CHEK2*1100delC in breast cancer.We established its prevalence in two German populations GENICA (Northrhine-Westphalia, n = 724) and KORA (Bavaria, n = 600) and in women with breast cancer. The latter included cases (n = 688) from the GENICA breast cancer case-control study, patients with early-onset breast cancer (n = 86) and patients with familial breast cancer (n = 71). The latter patient groups were previously investigated for BRCA1/2-mutations and tested negative. Mutation analysis was performed by combined PCR/DHPLC methodology.CHEK2*1100delC was found in 0.9% of GENICA controls and was absent in the KORA controls indicating a significant difference between the two populations (P = 0.03). The frequency of CHEK2*1100delC in age-matched cases of the GENICA collection was 0.8% and thus not different from controls (OR 0.88, 95% CI 0.21–3.50). In patients with early-onset disease CHEK2*1100delC was found at a frequency of 2.3% referring to an increased breast cancer risk of 2.56 (95% CI 0.25–14.58). In patients with familial disease the frequency was 1.4% referring to an increased risk of 1.53 (95% CI 0.03–12.93).Our data showed variations in CHEK2*1100delC prevalence within German populations suggesting possible inaccuracies in breast cancer risk assessments from non population-based studies. In patients with a high-risk profile however, CHEK2*1100delC was indicative for this risk and highest for early-onset breast cancer.     

Posttherapy pathologic stage predicts survival in patients with esophageal carcinoma receiving preoperative chemoradiation

BACKGROUND: In patients with locoregional carcinoma of the esophagus or esophagogastric junction who underwent preoperative chemoradiation, it is unclear whether survival was better predicted by pretherapy clinical stage or by posttherapy pathologic stage. METHODS: The authors studied 235 consecutive patients with pretherapy clinical Stage II, III, or IVA (according to American Joint Committee on Cancer criteria) carcinoma of the esophagus or esophagogastric junction who were treated with chemoradiation followed by esophagectomy. Posttherapy cancer status was classified using pathologic stage and semiquantitative assessment of residual carcinoma. Clinicopathologic features, residual carcinoma status, and pretherapy and posttherapy stage were compared with disease-free and overall survival. RESULTS: Posttherapy pathologic stage was Stage 0 in 29% of patients, Stage I in 11% of patients, Stage II in 34% of patients, Stage III in 20% of patients, and Stage IV in 6% of patients. Cancer downstaging occurred in 56% of patients. In univariate analysis, disease-free and overall survival were predicted by posttherapy pathologic stage (both with P < 0.001), margin status (P = 0.002 and P = 0.01, respectively), extent of residual carcinoma (both with P < 0.001), and downstaging (both with P = 0.001), but not by age, gender, type of cancer, pretherapy clinical stage, or preoperative regimen. However, in multivariate analysis, disease-free and overall survival were independently predicted by posttherapy pathologic stage (both with P = 0.02). Extent of residual carcinoma was a marginally significant predictor of overall survival (P = 0.04). CONCLUSIONS: Posttherapy pathologic stage was the best available predictor of outcome for patients with locoregional carcinoma of the esophagus or esophagogastric junction who underwent chemoradiation therapy followed by esophagectomy. The findings in the current study supported the concept of downstaging by preoperative therapy.     

Interferon alpha therapy for patients with essential thrombocythemia: final results of a phase II study initiated in 1986

BACKGROUND: In 1986, a Phase II trial of recombinant interferon-alpha (IFN-alpha) was initiated as therapy for patients with essential thrombocythemia (ET). METHODS: Patients were treated with subcutaneous IFN-alpha at a dose of 5 x 10(6) units/m(2) daily. In responding patients, the therapy lasted at least 3 years. RESULTS: Twenty-three patients (14 females and 9 males; median age, 41 years; age range, 20-63 years) with a median platelet count of 1350 x 10(9)/L were treated. After a median follow-up of 174 months (14.5 years), 15 of 20 evaluable patients (75%) responded, including 14 patients who achieved a complete hematologic response (CHR) (6 of them with bone marrow remission) and 1 patient who demonstrated a partial response. The median time to response was 6 months (range, 0.5-36 months), and the median response duration was 48 months (range, 5-114 months). Seven patients who achieved a CHR and were taken off therapy after they completed 3 years of maintenance therapy sustained their response for a median of 28 months. No symptoms or signs of thrombosis or hemorrhage were observed in responding patients. Eleven of 14 patients (78%) who achieved a CHR developed a recurrence, and 2 of 5 patients with recurrences who were rechallenged with IFN-alpha achieved a second response. The treatment was tolerated relatively well. CONCLUSIONS: IFN-alpha was safe and effective therapy for patients with ET, and the ability of IFN-alpha to reverse disease pathology and possibly modify the clinical course of patients with ET warrants its investigation in larger, prospective trials.     

An evaluation of information dissemination during the severe acute respiratory syndrome (SARS) outbreak among selected rural communities in Kuala Kangsar

To assess the level of knowledge, attitude and practice (KAP) on SARS and its preventive measures among the rural population of Kuala Kangsar district. This KAP study was also done to identify the expectation and preference of rural population upon obtaining health information. This is a cross-sectional study of 201 households from four villages in Kuala Kangsar. Face-to-face interview was done regarding knowledge, attitude and practice on SARS and its preventive measures. Statistical analyses were performed with SPSS (Version 10.0). A scoring system was used to assess the level of knowledge, attitude and practice towards SARS. Ninety one percent of the study population was aware of SARS. Majority of them have good attitude towards SARS based on the formulated scoring system. Television was found to be the first hand information about SARS and most preferred source of information by the rural population. Knowledge and attitude of respondents concerning SARS were good. Television was found to be the preference among the rural population in obtaining health information.     

Cardiac echinococcosis: echocardiographic diagnosis with a fatal clinical outcome

Hydatid disease HD is an endemic disease found in various regions of the world. The organs mostly affected are the liver and lung. Cardiac involvement in HD is rare. The symptoms are mainly due to the mass effect of the calcific hydatid cyst obstructing the blood or the lymphatic vessels. Other manifestations are secondary infection or cyst rupture in the involved organ. Here, we report a middle aged female patient with no history of medical illness who presented to the emergency room with an unrecordable blood pressure. Echocardiogram showed multiple calcific cysts of echinococcosis granulosa in the left ventricle cavity. In the clinical setting, where there is detection of HD elsewhere in the body, an echocardiogram is mandatory as cardiac involvement is serious and may be fatal.     

上海市胆道癌诊治情况的调查和分析

目的评估上海市胆道癌的诊治情况。方法对上海市区1997年6月至2001年5月4年间年龄在35～74岁的658例胆道癌新病例进行流行病学调查,对收集到的390例胆囊癌、195例胆管癌和73例壶腹癌的临床资料进行分析。结果资料显示,胆道癌好发于老年人;胆囊癌男女之比为1：2．61;胆管癌和壶腹癌则男性略多于女性。胆囊癌、胆管癌和壶腹癌分别有68．5％、43．1％和22．4％的患者合并胆结石。胆囊癌的B超诊断准确率为63．1％,意外胆囊癌占20％,ⅣA和IVB期胆囊癌占43．6％。胆管癌和壶腹癌的误诊率较高,分别为19．1％和47、1％,且就诊时大多数患者已出现黄疸。69例(18．2％)胆囊癌、50例(25．6％)胆管癌和54例(74％)壶腹癌行根治性切除术,术后1、3、5年生存率分别为58．5％、42．8％、40．7％,58％、28．3％、11．1％和81．5％、39．2％、26．9％。79例胆管癌行姑息性引流术,大多数患者在术后1年内死亡。38例胆管癌植入金属内支架或塑料内支撑管,平均生存期约7个月。结论胆道癌的早期诊断仍较困难;应重视胆囊癌手术方法的规范化;怀疑胆管癌而无手术禁忌证宜手术探查;壶腹癌宜行胰十二指肠切除术。     

Arterialization of deep dorsal vein of penis for penile ischemia

Penile ischemia, a rare complication of diabetic end-stage renal disease, is usually treated by penectomy once conservative measures fail. We present a patient with diabetes mellitus and end-stage renal disease with penile ischemia that was successfully treated with an arteriovenous interposition bypass graft between the common femoral artery and the deep dorsal vein of the penis. Retrograde flow into the corpus spongiosum resulted in immediate pain relief and healing of the ischemic lesions.     

A phase II dose-increasing study of sitamaquine for the treatment of visceral leishmaniasis in Kenya

Sitamaquine (WR6026) is an 8-aminoquinoline in development for the oral treatment of visceral leishmaniasis (VL). This was an open-label, dose-increasing study to determine the dose-response and safety profile for sitamaquine in Kenyan patients with VL caused by Leishmania donovani. Patients (mean age 15.9 [range = 5-47] years) received sitamaquine daily for 28 days at one of four doses: 1.75 (n = 12), 2.0 (n = 61), 2.5 (n = 12), or 3.0 (n = 12) mg/kg/day. The primary efficacy outcome was cure (absence of parasites on splenic aspirate) in the intent-to-treat population at day 180. Cure was achieved in 79 (83%) of 95 patients overall, and in 11 (92%) of 12, 49 (80%) of 61, 9 (82%) of 11, and 10 (91%) of 11 patients at sitamaquine doses of 1.75, 2.0, 2.5, or 3.0 mg/kg/day, respectively. The most frequent adverse events during active treatment were abdominal pain (12 [12%] of 97) and headache (11 [11%] of 97), and one patient in each of the 2.5 mg/kg/day and 3.0 mg/kg/day dose groups had a severe renal adverse event. The effects of sitamaquine on the kidney need further investigation. Sitamaquine was efficacious and generally well tolerated in Kenyan patients with VL.