成人缺铁性贫血致骨髓增生症1例

患者女,45岁.10余年前出现乏力、头晕、无法完成家务等日常活动,无意中发现双下肢对称性增粗,无疼痛、压痛等不适.近来症状加重入院.实验室检查:白细胞 5.62×109/L;中性粒细胞 82.3%;血红蛋白63 g/L;血小板287×109/L;血清铁SI:35 μg/dl,转铁蛋白饱和度15%,铁蛋白SF:12 μg/ml;免疫球蛋白Ig定量(一),抗ENA(一),自身抗体SMA 1∶320.     

Successful treatment of a centenarian with coronavirus disease 2019 (COVID-19) using convalescent plasma

BackgroundBecause treatment options for coronavirus disease 2019 (COVID-19) are very limited, the use of convalescent plasma has bee explored.Case presentation and treatmentA male centenarian with cough and dyspnea for 2 months was diagnosed with COVID-19. Without effective treatments and with the increased risks of antiviral therapy for the elderly, this patient was given convalescent plasma. The viral load, complete blood count, inflammatory indicators, vital signs, and clinical symptoms were observed before and after COVID-19 convalescent plasma transfusion.ResultsAfter convalescent plasma transfusion, significant improvement was observed on laboratory indicators and clinical symptoms of the patient. Concurrently, SARS-CoV-2 viral load decreased sharply after the first transfusion (from 2.55 × 104 to 1.39 × 103 copies/mL) and became undetectable after the second transfusion.ConclusionsWith the substantial increase of COVID-19 in recent months,treatment for elderly patients has become restricted in some countries. The successful treatment of this 100-year-old patient using convalescent plasma suggests that we should consider adding convalescent plasma in th management of the elderly.     

A case of angioedema due to acquired C1 esterase inhibitor deficiency masquerading as suspected peritonitis: a case report

Angioedema due to acquired C1 esterase deficiency is a rare condition and a non-inflammatory disease characterized by episodes of edema of the mucosa of the upper airway or gastrointestinal tract. The purpose of this case report is to heighten awareness among emergency physicians of a peritonitis-like condition that can develop into angioedema due to acquired C1 esterase inhibitor deficiency, and thereby help to prevent false diagnosis resulting in unnecessary surgical intervention. We report the case of a 21-year-old man who presented to the Emergency Department (ED) with abdominal pain. He was later diagnosed with angioedema of the gastrointestinal tract due to acquired C1 esterase deficiency that was initially suspected as peritonitis. Careful evaluation of the acute abdomen in acquired C1 esterase deficiency is very important in the ED to distinguish between medical and surgical causes of an acute abdomen.     

Management of heparin resistance due to antithrombin deficiency in a Chinese pregnant woman: a case report

Untreated individuals with antithrombin (AT) deficiency are at higher risk of thrombosis and adverse pregnancy outcomes. The present recommendations are mostly empirical for treating patients with AT deficiency during pregnancy because of the absence of guidelines. We report a rare case of heparin resistance due to AT deficiency in a pregnant 32-year-old Chinese woman. We also reviewed the English medical literature for AT deficiency and its association with thromboembolism and treatment. This patient suffered two early miscarriages because of thrombosis due to AT deficiency. The patient was administered the combination of adequate low molecular weight heparin with fresh frozen plasma and warfarin because of her heparin resistance. She delivered a healthy female newborn without any adverse effects of the anticoagulation therapy. Our findings suggest that the combination of adequate low molecular weight heparin with fresh frozen plasma and warfarin is effective for preventing thrombus during pregnancy.     

成功救治肺源性心脏病并发胃肠道穿孔、脓毒症休克一例

脓毒症是患者入住ICU的常见病因,也是ICU患者死亡的主要原因之一。急性肾功能损伤是脓毒症常见脏器损伤,且与患者病死率相关。慢性阻塞性肺疾病患者由于长期肺动脉高压会导致肺源性心脏病,并进一步引起右心肥大甚至心功能不全,严重影响患者心肺功能。嘉兴市第二医院收治1例老年肺源性心脏病并发胃肠道穿孔、脓毒症休克患者,术后出现低心排、急性肾功能损伤及体循环淤滞,经相关积极救治后顺利出院,现将本例的临床资料及诊疗体会报道如下。     

Successful treatment of tuberculosis combined with drug-induced myopathy using corticosteroid therapy: a case report

A 39-year-old woman was admitted to our hospital on 19 January 2019 because of a 10-day history of intolerance to oils in her food, fatigue, and yellowing of the skin and sclera. In December 2018, the patient had been diagnosed with tuberculous pleurisy at a local hospital and received quadruple anti-tuberculosis treatment. Ten days before presentation to our hospital, she had developed anorexia, fatigue, nausea, loss of appetite, cough, and shortness of breath. She visited a local hospital, where she was considered to have drug-induced hepatitis. She discontinued the anti-tuberculosis drugs and liver protection treatment. After 3 days, her symptoms had not substantially improved. She visited the infection department of our hospital for further diagnosis and treatment. After 6 days of treatment, the patient’s symptoms were not significantly improved, her liver and muscle enzyme concentrations were further increased, and her limbs had become weaker and more difficult to move. We considered diagnoses of drug-induced hepatitis and drug-induced myopathy. The patient was treated with intravenous methylprednisolone at 40 mg once a day for 16 days and other symptomatic treatments. Her symptoms significantly improved and she was discharged.     

Successful treatment of a guitarist with a finger joint injury using instrument-assisted soft tissue mobilization: a case report

Finger injuries are common and can greatly affect a musician’s quality of life. A 55-year-old man, who had injured the proximal interphalangeal joint of the left index finger 6 months prior to any intervention, was treated with a manual therapy approach incorporating instrument-assisted soft tissue mobilization (IASTM). Initial examination findings included self-reported pain and functional limitations and physical impairments that significantly impeded his ability to play the acoustic guitar. He was treated once a week for 6 weeks with IASTM, joint mobilization, therapeutic exercise, and ice massage. Additionally, a home exercise program and self-care instructions were provided. The patient gained positive outcomes with improvements in pain (Numerical Pain Rating Scale while playing the guitar: initial 5/10, discharge 1/10) and function (Disability Arm Shoulder Hand Sports-Performing Arts Optional Module: initial 75; discharge 6·25), each reaching a minimum clinically important difference. Importantly, he was able to play the guitar with minimal to no pain as desired. Physical measures also improved, including an immediate gain in finger range of motion with IASTM alone. Manual therapy approaches integrating IASTM may provide an effective conservative treatment strategy for patients with finger/hand conditions in the performing arts and other patient populations.     

Successful treatment of a guitarist with a finger joint injury using instrument-assisted soft tissue mobilization: a case report

Finger injuries are common and can greatly affect a musician’s quality of life. A 55-year-old man, who had injured the proximal interphalangeal joint of the left index finger 6 months prior to any intervention, was treated with a manual therapy approach incorporating instrument-assisted soft tissue mobilization (IASTM). Initial examination findings included self-reported pain and functional limitations and physical impairments that significantly impeded his ability to play the acoustic guitar. He was treated once a week for 6 weeks with IASTM, joint mobilization, therapeutic exercise, and ice massage. Additionally, a home exercise program and self-care instructions were provided. The patient gained positive outcomes with improvements in pain (Numerical Pain Rating Scale while playing the guitar: initial 5/10, discharge 1/10) and function (Disability Arm Shoulder Hand Sports-Performing Arts Optional Module: initial 75; discharge 6·25), each reaching a minimum clinically important difference. Importantly, he was able to play the guitar with minimal to no pain as desired. Physical measures also improved, including an immediate gain in finger range of motion with IASTM alone. Manual therapy approaches integrating IASTM may provide an effective conservative treatment strategy for patients with finger/hand conditions in the performing arts and other patient populations.     

Bile lipid composition and haemostatic variables in a case of high density lipoprotein deficiency (Tangier disease)

A 62-year-old man with clinical and biochemical findings consistent with homozygous Tangier disease is presented. Widespread atherosclerosis was present. Bile lipid analysis showed a low molar percentage of cholesterol with a low saturation index. The data suggest that high density lipoprotein cholesterol may act as a preferential precursor of biliary cholesterol. Coagulation and platelet studies indicated that the patient's platelets were hyper-responsive to aggregating agents and produced an increased amount of thromboxane B2. A platelet storage pool deficiency was also found.     

Glucose-phosphate isomerase deficiency due to a new variant (GP I Barcelona) and to a silent gene: biochemical, immunological and genetic studies.

A 12-year-old girl of Spanish origin was found to be double heterozygote for a deficient GP I variant (GP I Barcelona) and for a silent GP I gene. The mother was heterozygote for GP I Barcelona and the father was heterozygote for the silent gene. GP I Barcelona was a fast variant (116%) with an increased isoelectric point (9.55), lability to heat and to urea, and shift of the pH curve towards the acidic pH. The other kinetic characteristics were normal. The ratio of enzymatic activity to immunological reactivity was normal in erythrocytes and white blood cells of the father and the mother but decreased to 75% of normal in blood cells of the daughter. The genetic and molecular mechanisms of GP I deficiency of this patient are discussed.     

温心胶囊对冠心病心阳虚证大鼠血小板功能的影响

目的：研究温心胶囊对冠心病心阳虚证大鼠血小板功能的影响。方法：采取高脂饮食，脑垂体后叶素皮下注射及寒冷刺激方法，建立冠心病阳虚证模型。同时观察温心胶囊对血小板功能的影响。结果：温心胶囊组与模型组比较差异显著（P＜0．001）。血浆血管性假血友病因子（VWF）浓度、血栓素B2（TXB2）含量、血小板聚集率、血小板膜糖蛋白GPⅡb/Ⅲa分子数和α颗粒膜蛋白GMP－140含量下降，6－酮－前列环素（6－Keto-PGF1α）含量增高。结论：温心胶囊具有抑制血小板聚集作用。     

Successful treatment of c-kit-positive metastatic Adenoid Cystic Carcinoma (ACC) with a combination of curcumin plus imatinib: A case report

Adenoid cystic carcinoma (ACC) is an aggressive malignant neoplasm of the secretory glands. Conventional chemotherapy has poor effectiveness against metastatic ACC. Thus, a novel effective therapy is needed against metastatic ACC.A majority of ACCs (up to 94%) express c-kit. Imatinib is monoclonal antibody with specific activity against c-kit but has not been found to be effective in treating patients with ACC in which c-kit is overexpressed and activated. The NF-κB and mTOR pathways have been shown that ubiquitously and concurrently activated, indicating that the inhibition of these pathways may represent a novel treatment approach for patients with ACC. Curcumin has been shown to inhibit NF-κB and NF-κB-related pathways. 43-year-old patient was diagnosed ACC from submandibular salivary gland. After complete resection of tumor adjuvant radiotherapy was initiated. Seven years later multiple lung metastases were detected and ACC was confirmed by re-biopsy. First-line chemotherapy failed. NF-κB and c-kit were overexpressed in the metastatic specimens. Therefore, we treated the patient with metastatic chemoresistant ACC with imatinib 400 mg/day and intravenous curcumin 225 mg/m² twice a week plus oral bioavailable curcumin Arantal^® 2 × 84 mg/day. At 24 months, we observed near complete anatomic and complete metabolic response. To our knowledge, this is the first report of a patient with a c-kit-positive ACC that is successfully treated with the combination of imatinib and curcumin in an integrative approach.     

温阳活血化痰法降低冠心病心阳虚证血小板聚集率的机制探讨

目的 :研究温阳活血化痰法对冠心病心阳虚证大鼠血小板功能的影响。方法 :采取高脂饮食、脑垂体后叶素皮下注射及寒冷刺激方法建立冠心病心阳虚证大鼠模型 ;同时观察温阳活血化痰法对血小板功能的影响。结果 :温阳活血化痰法治疗组与模型组比较 ,血浆血管性假血友病因子 ( v WF)浓度、血栓素 B2 ( TXB2 )含量、血小板聚集率、血小板膜糖蛋白 b/ a( GP b/ a)分子数和α颗粒膜蛋白 ( GMP 140 )含量均明显下降 ,6酮前列腺素 F1α( 6 keto PGF1α)含量增高 ( P均 <0 .0 0 1)。结论 :温阳活血化痰法具有抑制血小板聚集的作用。     

Intraperitoneal bleeding due to rupture of the left gastric vein (LGV) in a patient with liver cirrhosis: a case report

Intraperitoneal bleeding from ruptured ectopic varices is a rare and fatal complication in patients with portal hypertension. Although laparotomy with high mortality is performed, it is difficult to detect correct bleeding site and save the patient. This is probably the first case report of rupture from left gastric vein revealed by transjugular intrahepatic portosystemic shunt (TIPS). We propose the use of TIPS for diagnosing and treating intraperitoneal bleeding from ectopic varices.     

Transfusion-related acute lung injury (TRALI) following autologous stem cell transplant for relapsed acute myeloid leukaemia: a case report and review of the literature

A fatal case of transfusion-related acute lung injury (TRALI) in a child post-autologous stem cell transplant for relapsed acute myeloid leukaemia is described. The implicated product was a single unit platelet concentrate containing anti-HLA A2 and granulocyte-specific anti-NA1 antibodies. The recipient typed as HLA A2/A2, NA1/NA1. This is the first reported case of TRALI following a transplant procedure for a haematological condition. It is also unusual in that the patient failed to make a full recovery and that two relevant leucocyte antibodies of clear specificity were identified in the donor plasma. The literature relating to the pathophysiology, clinical sequelae and management of TRALI is reviewed.