A case of sarcoidosis with systemic sclerosis

To the editor： Systemic sclerosis （SSc） is an autoimmune or multisystem connective tissue disease. It is characterized by autoimmunity, microangiopathy, and fibrosis in skin and internal organs. Sarcoidosis is a systemic inflammatory disease of unknown cause that affects the skin, respiratory tract, heart, eyes, lymphatic system, nervous system, kidney and endocrine systems.2 Coexistence of these two diseases had been reported, but is rare.3 Here, we describe a case of Chinese woman who developed sarcoidosis 20 years after diagnosis of limited cutaneous SSc （IcSSc）.     

Subcutaneous nodular sarcoidosis and systemic involvement successfully treated with doxycycline

Subcutaneous nodular sarcoidosis is a rare cutaneous manifestation of systemic sarcoidosis. We report a new case in a 45-year-old woman with a 7-year history of subcutaneous nodules and a new onset of dyspnea. She was treated with corticosteroids but her disease recurred upon withdrawal. A 6-month course of doxycycline in a dose of 200 mg/d led to complete remission. We also emphasize the value of systemic work-up and regular screening in such cases.     

Portal hypertension and ascites in systemic mastocytosis

We report a case of systemic mastocytosis (SM) presenting as ascites and portal hypertension. The haematological picture at presentation was suggestive of chronic myelomonocytic leukaemia. Initial difficulties in making a diagnosis of SM were encountered as the cutaneous signs were atypical. The correct diagnosis was established only after tissue sections were appropriately stained for mast cells. The liver biopsy showed portal and sinusoidal mast cell infiltration, portal fibrosis and evidence of hepatic venous outflow obstruction. The disease progressed rapidly and recurrent massive ascites was a dominant problem. This case illustrates again the problems of making a diagnosis of SM especially when the clinical picture is atypical. Ascites as a presenting manifestation of SM has been reported previously in only six patients. Published cases of SM with portal hypertension or ascites or both are reviewed.     

Severe refractory sarcoidosis in a 64-year-old man with persistent leucopenia

We present a case of bone marrow granulomas in a 64-year-old West Indian man who presented with severe leucopenia, anaemia, thrombocytopenia, hepatosplenomegaly, hypercalcaemia, hypercalciuria, elevated angiotensin converting enzyme level and reticulo-nodular shadows on chest X-ray. Bone marrow biopsy revealed numerous non-caseating epithelioid granulomas. A diagnosis of sarcoidosis was made and he was treated with prednisolone 60 mg daily for four weeks and the dose was subsequently reduced to 30 mg daily. Eight months follow-up revealed persistent pancytopenia. Bone marrow granulomas are rare and, when they occur, sarcoidosis is an uncommon aetiology. This case illustrates that severe leucopenia may occur in sarcoidosis and may present therapeutic difficulties.     

Severe systemic effects of Merrem's hump-nosed viper bite

OBJECTIVE: To report unpredictable severe systemic effects of hump-nosed viper envenomation. SETTING: Medical unit, General hospital, Anuradhapura. METHODS: The clinical outcome of seven patients bitten by Merrem's hump-nosed viper were monitored until recovery or death. Limited autopsies were performed on the latter. Offending snakes were positively identified by medical officers and in one instance by a herpetologist. CONCLUSION: Merrem's hump-nosed viper bites, caused an array of potentially fatal systemic manifestations. One patient developed neurological effects, severe Raynaud's syndrome leading to ascending gangrene of distal limbs and adult repiratory distress syndrome (ARDS), in addition to the known complications of severe renal cortical necrosis and haemostatic dysfunction. Two patients who developed acute renal failure and prolonged coagulopathy recovered completely. The combination of extensive renal cortical necrosis, disseminated intravascular coagulation, and ARDS proved fatal in three.     

Association of the 3050G>C polymorphism in the cyclooxygenase 2 gene with systemic sarcoidosis

BACKGROUND: We investigated the potential association between cyclooxygenase-2 (COX-2) gene polymorphisms and clinical manifestations of sarcoidosis. METHODS: This observational cross-sectional study involved seven hospitals in Spain. We diagnosed patients with sarcoidosis according to the International Criteria. The following variables were recorded: age, gender, initial diagnostic methods, serum angiotensin-converting enzyme (ACE) levels, pulmonary function tests, radiological stage, and clinical findings at diagnosis. Manifestations of sarcoidosis were classified as systemic vs. nonsystemic. Genotyping of four COX-2 polymorphisms (COX2.5909T>G, COX2.8473T>C, COX2.926G>C, and COX2.3050G>C) was undertaken on DNA extracted from peripheral blood lymphocytes using fluorescent hybridization probes and melting curves. RESULTS: A total of 131 sarcoid patients (63 males, mean age: 47 +/- 15 years) were studied. One hundred twenty-six of these patients had one or more positive biopsies. The results demonstrated that genotype distribution for the COX2.3050G>C polymorphism was significantly different between patients with systemic sarcoidosis and those with nonsystemic forms (p = 0.046). After adjustment for age, gender, and serum ACE levels, a significant association between the carriage of at least one C allele of the COX2.3050G>C polymorphism and systemic sarcoidosis was observed (odds ratio [OR]: 2.3; 95% confidence interval [CI]: 1.03-5.12, p = 0.031). Other polymorphisms were not associated with either clinical manifestations of the disease or serum ACE levels. CONCLUSIONS: Our results indicate for the first time that the C allele of the COX2.3050G>C polymorphism is associated with systemic sarcoidosis.     

腹水回输术治疗顽固性腹水临床效果研究

目的 探讨腹水回输治疗顽固性腹水的有效性、安全性.方法 对2005年1月至2008年12月收治的33例顽固性腹水病例资料进行统计分析.结果 本组病例33例,其中男18例,女15例,治愈26例,症状明显缓解7例.结论 腹水回输治疗肾性顽固性腹水效果好,操作简便,安全.     

Splenectomy in patients with undiagnosed splenomegaly.

Of splenectomies performed in the Cork Regional Hospital over an 11 year period, ten were undertaken primarily for diagnostic purposes. A definitive histological diagnosis was established in nine patients, seven of whom had lymphoma, two with Hodgkin''s disease and five with non-Hodgkin''s lymphoma. The weight of the excised spleen in all patients with lymphoma exceeded 1 kg; in all those with a diagnosis other than lymphoma, the spleen weighed less than 1 kg. A majority of patients also had symptomatic improvement from reversal of hypersplenism and from relief of the mechanical pressure effects of an enlarged spleen. Operative mortality was zero. Diagnostic splenectomy is a worthwhile procedure. Most patients will have lymphoma.     

腹腔热灌注联合全身化疗治疗恶性腹腔积液的疗效观察

目的观察腹腔热灌注(IPCH)联合全身化疗对恶性腹腔积液的治疗效果,探讨恶性肿瘤的辅助治疗措施。方法选择具有中至大量恶性腹腔积液的晚期肿瘤患者102例,随机分为治疗组(腹腔热灌注联合全身化疗,n=52)和对照组(全身化疗,n=50),观察两组的近期疗效及不良反应。结果治疗组患者的完全缓解率、部分缓解率及临床受益率(分别为38.5%、42.3%及90.3%)均高于对照组(分别为14.0%、16.0%及66.0%)(P<0.05),两组不良反应比较,差异无统计学意义(P>0.05)。结论 IPCH联合全身化疗能有效地控制恶性腹腔积液。     

脾肿大的诊断和处理策略

脾肿大判断的金指标是脾脏的重量,正常成人的脾脏50～250g,随着年龄的增长趋于减轻。但这种方法仅限于脾切除术后及尸检的患者,因此临床实用性不强。     

脾脏肿大慢加急性肝衰竭病人的临床特点

目的了解脾脏肿大慢性肝病患者发展为慢加急性肝衰竭后的临床特点和短期预后。方法回顾性收集慢性乙型肝炎病
毒感染相关的慢加急性肝衰竭病例,描述和分析脾脏肿大病人的临床特点。结果共纳入149例病人,4周死亡率48.3%。与无
脾脏肿大者相比,脾脏肿大的病人外周血小板计数（G/L）降低（78 vs 113,P=0.001）,丙氨酸氨基转移酶（ULN）水平低（1.98 vs
4.73,P=0.005）,凝血酶原时间国际标准化率低（2.03 vs 2.33,P=0.010）,肝性脑病总体发生率接近（39.8% vs 38.8%,P=1.000）,但
脾脏肿大者多为1~2级肝性脑病（P<0.001）。脾脏肿大者4周死亡率低（P=0.034）,但90 d死亡率接近。脾脏肿大是4周死亡的
独立保护因素（RR=1.939,P=0.038）,其他与4周死亡独立相关的因素包括中性粒细胞比例大于70%（RR=1.791,P=0.049）、肝性
脑病（RR=1.806,P=0.001）、血清肌酐水平（RR=1.457,P=0.004）及凝血酶原时间国际标准化率（RR=1.205,P=0.018）。新模型的
预测价值优于终末期肝病模型（曲线下面积0.860 vs 0.792,P=0.017）。结论脾脏肿大的慢加急性肝衰竭病人具有相对独特的
临床特征。
     

腹水超滤浓缩回输治疗顽固性腹水患者及护理方法

1 资料与方法 1.1 对象 治疗对象30例为我院住院确诊的肝硬变合并顽固性腹水患者,所有病例均经限水限钠利尿治疗两周以上效果不佳,腹胀症状明显.男22例,女8例,年龄23～78岁,平均46.5岁.肝炎后肝硬变26例,酒精性肝硬化4例,均无重度黄疸、肝昏迷及上消化道出血史.