超声心动图对体肺分流术的评价和随访

目的:体肺分流术是复杂紫绀型先天性心脏病(先心病)的主要姑息手术之一,主要包括改良体肺分流术(BT)和中央分流术(CS)两类.本文回顾性总结超声心动图对不同体肺分流术式和体肺分流术后的评价及中期随访.方法:选择体肺分流术后随访6个月以上的患者资料共87例,其中改良体肺分流术(BT组)42例,中央分流术(CS组)45例,超声心动图检查分别记录手术前后的肺动脉内径及左心室舒张末期前后径变化,分流管道的血流速度和管道通畅情况.结果:两组共7例患者术后当天接受急诊手术对管道进行重新处理.BT组术后平均随访(22.3±12.1)个月,CS组术后平均随访(26.0±21.3)个月.随访期间前者(BT组)有8例发生堵塞,后者(CS组)有6例.分流手术后,左心室前后径扩大CS组为(8.9±3.5)mm较BT组(6.6±5.1)mm显著增加,差异有统计学意义(P=0.03);而两组主肺动脉内径增加值比较差异无统计学意义[(3.1±2.2)mm vs.(1.7±1.8)mm)].术后随访期间接受后期矫治手术两组分别为BT组24例和CS组27例,手术率比较差异无统计学意义[57.1%(24/42)v8 60.0%(27/45),P>0.05].结论:本研究表明两组体肺分流术式都可以促进肺血管和左心室的发育.超声心动图检查在不同术式的体肺分流的术后近期监测及远期随访研究中起着重要的作用.     

原发性心内膜弹力纤维增生症患儿的超声心动图随访

目的：了解原发性心内膜弹力纤维增生症（简称心弹）患儿的心内结构及心功能改变的情况及其指导临床诊断、治疗及预后的价值。方法：心弹组随诊患儿２３例（均经地高辛治疗），年龄３～１７（７．２２±４．２０）岁。距初诊时间３年以上，设配对对照组年龄、性别、身长、体重一致的正常儿童２３名。应用Ｍ型超声心动图、二维超声心动图和多普勒彩色超声心动图测量心脏房室腔的大小、心室壁的厚度及运动，各瓣口血流频谱参数及心功能指数。结果：心弹患儿左心房、左心室明显缩小。除一例１４岁患儿心脏恢复正常外，其余仍轻度扩大。左心室收缩功能与对照组仍有差别。结论：心弹患儿经过３年以上的治疗病情明显好转，但左心收缩功能仍较对照组略低，左心亦较对照组大，应坚持长期治疗。     

埋置心脏复律除颤器患者的随访与药物辅助治疗

为了解埋藏式心脏复律除颤器（ＩＣＤ）埋置后病人及ＩＣＤ工作的情况，以便及时调整ＩＣＤ的诊断及治疗程序从而给予病人更适当的治疗，对８例接受ＩＣＤ治疗的病人分别在出院前及出院后每３～６个月进行一次随访观察。８例病人随访１１．４±７．０个月，共发生心律失常事件２５次。抗心动过速起搏（ＡＴＰ）治疗６７次，第１次成功２２次，占３２．８％。高能量除颤１０次，第１次成功６次，占６０．０％。总成功率９１．３％。１例病人室性心动过速发作频繁、１例病人ＩＣＤ不能有效终止心动过速、２例病人出现甲状腺功能障碍。经调整药物及ＩＣＤ工作方式后，心动过速控制良好。表明对接受ＩＣＤ治疗的病人进行定期随访十分重要，及时调整ＩＣＤ程序设置和辅以药物治疗，可提高ＩＣＤ治疗的成功率。     

埋藏式心脏复律除颤器治疗恶性室性心律失常的随访观察

对２２例埋置了埋藏式心脏复律除颤器（ＩＣＤ）的有晕厥史的恶性室性心律失常患者进行了随访，观察ＩＣＤ的治疗效果。２２例中扩张型心肌病８例、肥厚型心肌病２例、冠心病７例、ＱＴ延长综合症１例，４例未发现器质性心脏病。所有患者均经临床证实有室性心动过速或（和）心室颤动（ＶＴ／ＶＦ）发作。ＩＣＤ具有多项治疗及信息储存记忆功能（即第三代）。随访时通过体外程控仪调出ＩＣＤ储存的资料进行分析。平均随访７．１（１～２３）个月，１１例患者（５０．０％）ＶＴ／ＶＦ发作１１８次，其中５４次为非持续性ＶＴ，均自行终止；接受ＩＣＤ治疗的６４次为ＶＴ／ＶＦ发作，４５次为ＶＴ，２２次由ＩＣＤ的抗心动过速起搏（ＡＴＰ）终止、１６次由低能量（２～１５Ｊ）转复终止、２次发作为窦性心动过速（误判为ＶＴ），其余５次ＶＴ在ＡＴＰ治疗过程中加速转为ＶＦ，由高能量除颤终止。ＩＣＤ诊断的ＶＦ发作共有１９次，发生于４例患者，有１例患者接受除颤１６次，４次系为频率超过ＶＦ感知频率的ＶＴ。１９次发作均被ＩＣＤ有效除颤终止。２例扩张型心肌病患者，１例术后３个月死于心功能衰竭，另１例术后２个月反复发作ＶＦ，多次接受ＩＣＤ除颤治疗，最后死于心功能衰竭。结果表明常规进行?     

埋藏式心脏复律除颤器临床应用分析

９例药物难治性室性心动过速（ＶＴ）病人接受埋藏式心脏复律除颤器（ＩＣＤ）治疗，其中冠心病７例，扩张型心肌病、房间隔缺损修补术后各１例。８例术前有电击复律／除颤史。９例术前、术后均接受胺碘酮治疗，随访９．８±８．０个月，７例经ＩＣＤ储存资料证实共发生ＶＴ６６６次，１００％被ＩＣＤ有效终止，首次治疗程序成功率为９７％。４例ＶＴ经电击复律，２例因窦性心动过速而误放电，６例有疼痛与恐惧感。１例同时埋置了ＩＣＤ与单极电极导线起搏器，随访１６个月未发现任何不良的相互作用。心理障碍是ＩＣＤ治疗值得重视的问题，医生应协助病人尽量缩短心理调整过程，使之尽快对埋置的ＩＣＤ进行适应。     

Beneficial Effect of Granulocyte Transfusions in Patients with Defects in Granulocyte Function and Severe Infections

A 3-year-old boy (patient A) with a congenital and a 24-year-old man (patient B) with an acquired granulocyte function defect received supportive granulocyte transfusions for the management of severe infections. The boy had suffered from recurrent infections since birth. His granulocytes showed in vitro almost no chemotactic responsiveness, an impaired phagocytosis and reduced intracellular killing of Candida albicans. Family studies suggested that it was an inherited autosomal recessive defect. The child developed a Pseudomonas pneumonia at the age of 3 years, which did not respond to antibiotic therapy. Granulocyte transfusions were then started and soon after the fever and pneumonia disappeared. Patient B showed the haematological signs of a preleukaemic state. He had 3 recurrent episodes of furunculosis which led each time to cellulitis and septic temperatures accompanied by symptoms of an enterocolitis. Tests of granulocyte function in vitro showed reduced intracellular killing of Staphylococcus aureus. Granulocyte transfusions were started, since no clinical improvement could be attained by antibiotics. With transfusion therapy, fever, cellulitis and enterocolitis disappeared each time.     

Metabolic and Functional Studies of Monocytes in Congenital Neutropenia

S ummary . Children with the severe form of congenital neutropenia usually die from infection by 2 yr of age. Metabolic and functional studies of monocytes from three patients with this disease were undertaken to understand further the role of monocytes in the host's defences against bacterial infection. The bactericidal capacity of monocytes compared favourably to the bactericidal capacity of control neutrophils (PMN). Metabolic studies of monocytes and control leucocytes showed similar stimulation of oxygen consumption, hexose monophosphate pathway activity arid ¹²⁵iodide fixation during phagocytosis. Further, myeloperoxidase (MPO) activity of patients'monocytes was comparable to the activity of control leucocytes. Using the Rebuck skin window, very few of the patients'mononuclcar cells were seen to migrate during the initial 2–4 hr of observation. During this time, the PMN migration of controls was maximal. Following the relatively acellular period observed in patients, a brisk mononuclear cell infiltration occurred apparently independent of the presence of neutrophils. Epinephrine injection into patients stimulated a marked increase of blood monocytes, suggesting the existence of a marginal monocyte pool. An injection of hydrocortisone caused an unexplained 3–8-fold decrease in circulating monocytes. The delay of phagocytic cell migration to an inflammatory site, and the apparent sensitivity of monocytes to hydrocortisone may account in part for the increased susceptibility to infection in patients with severe congenital neutropenia.     

A Review of Infections in Patients with Left Ventricular Assist Devices: Prevention,Diagnosis and Management

Since the advent of ventricular assist devices with smaller configurations and continuous-flow technology, survival rates for patients with end-stage heart failure have dramatically improved. While the burden of infectious complications is decreased in patients on continuous-flow ventricular assist devices compared to bulkier pulsatile-flow devices, infection remains one of the most common causes of morbidity and mortality. The majority of infections occur at the driveline exit site, beginning with a disruption or trauma to the barrier between the skin and driveline and sometimes spreading deeper. Once infections develop, they can be difficult to eradicate. Depending on the degree of infection, treatment options may include local wound care, antibiotics, or surgical treatment. Preventive strategies and careful surveillance are crucial to improve patient outcomes.     

When and How to Cover for Fungal Infections in Patients with Severe Sepsis and Septic Shock

Candida species remain the most frequently isolated fungi in intensive care unit (ICU) patients with severe sepsis or septic shock. Delayed antifungal therapy in these patients is a recognized risk factor for mortality. However, the diagnosis of invasive candidiasis remains difficult and is frequently delayed. Clinical scores have been proposed to assess the risk of development of invasive candidiasis or candidemia. Laboratory tools for early diagnosis are disappointing or still under development. Triazoles, polyenes, and echinocandins are the key drugs used to treat invasive candidiasis in ICU patients with similar efficacy, but very variable tolerability. The increasing incidence of fluconazole-resistant and susceptible-dose dependent strains and the safety profile of antifungal agents must be taken into account when selecting empiric therapy, frequently leading to the initial use of echinocandins in ICU patients with severe sepsis or septic shock.     

Neutrophil Marrow Profiles in Patients with Rheumatoid Arthritis and Neutropenia

Neutrophil marrow cellularity was determined in 14 neutropenic patients with rheumatoid arthritis (RA) from measurements of neutrophil-normoblast ratios in marrow biopsies and ferrokinetic estimates of marrow normoblasts. A marrow profile was developed for each patient comprising the numbers of promyelocytes and myelocytes, of metamyelocytes and bands, and of segmented neutrophils in whole marrow. In each case a maturation ratio was calculated by dividing the number of metamyelocytes and bands by the number of promyelocytes and myelocytes. The physiologic marrow response to loss of neutrophils from circulation was assumed to be an increase in promyelocytes and myelocytes due to proliferation and influx, a reduction in segmented cells due to early release, and a normal maturation ratio. The results were interpreted in the light of the 95% confidence limits for data previously obtained from 13 normal subjects: in patients with neutropenia reduced or basal numbers of promyelocytes and myelocytes were interpreted as absence of the anticipated proliferative response; increased numbers of marrow segmented cells were attributed to failure of release; a low maturation ratio was assessed to reflect intramedullary cell loss. The pattern in two patients with Felty's syndrome was consistent with a physiological response to neutrophil destruction. The other 12 patients had neutrophil marrow abnormalities. Seven patients with Felty's syndrome and four patients without splenomegaly had absolute or relative hypoplasia of neutrophil marrow or low maturation ratios. One patient with a normal spleen size had an increased number of marrow segmented cells yet failed to mobilize cells normally in response to dialysis coil-activation of C3. Abnormalities of neutrophil marrow may contribute to neutropenia in RA irrespective of the presence of splenomegaly. Recognition of neutrophil marrow abnormalities in these patients may be of value in prognosis and management.     

中青年高血压病患者动脉弹性功能与血清脂肪酸

目的 观察中青年高血压病患者和健康对照者基线空腹血清游离脂肪酸(FFA)的差异和大、小动脉弹性功能2年的变化.方法 社区筛选高血压病患者103例(33～60岁),并筛选128例健康非高血压者(年龄匹配),随访2年.采用CVProfilor DO-2020型动脉弹性功能测定仪检测两组2年前后大、小动脉弹性指数(C1和C2),测定基线空腹血FFA、血脂、血糖、胰岛素等.高血压组91例,非高血压组97例得到随访.结果 1)高血压病患者(n=91)的C1、C2均显著低于非高血压病患者[n=97;C1,11.9±3.9比非高血压组:(14.0±4.2)mL/mm Hg×10,P=0.001;C2,4.9±2.2比非高血压病组:(6.1±2.6)mL/mm Hg×100,P=0.001].2)高血压病患者2年的小动脉弹性指数变化值(△C2)显著低于非高血压病患者[-0.24±0.25比非高血压组:(0.75±0.31)mL/mm Hg×100,P=0.014],其大动脉弹性指数变化值(△C1)也降低,但差异无统计学意义(P=0.458).3)与非高血压病患者相比,高血压病患者基线空腹血清饱和脂肪酸、单不饱和脂肪酸水平均显著增高(P均＜0.05),而n-3脂肪酸水平[(96.0±38.9)比非高血压病组:(123.0±36.0)μmol/L,P=0.001]、多不饱和与饱和脂肪酸比值(P/S)(1.60±0.37比非高血压病组:1.90±0.46,P=0.009)显著降低.4)logistic回归分析显示高血压病患者基线空腹血清亚麻酸(18:3 n3)优势比(OR值)和95%可信区间为2.483(1.480～4.166,P=0.001),而DHA(22:6 n3)的为0.329(0.195～0.554,P=0.001).结论 原发性高血压患者动脉弹性年龄相关降低过程加速,空腹血清FFA组成异常(如亚麻酸增高,DHA下降),可能是其高血压发病的危险因素之一.     

中青年高血压病患者动脉弹性功能与血清脂肪酸

目的观察中青年高血压病患者和健康对照者基线空腹血清游离脂肪酸(FFA)的差异和大、小动脉弹性功能2年的变化。方法社区筛选高血压病患者103例(33～60岁),并筛选128例健康非高血压者(年龄匹配),随访2年。采用CVProfilorDO-2020型动脉弹性功能测定仪检测两组2年前后大、小动脉弹性指数(C1和C2),测定基线空腹血FFA、血脂、血糖、胰岛素等。高血压组91例,非高血压组97例得到随访。结果1)高血压病患者(n=91)的C1、C2均显著低于非高血压病患者[n=97;C1,11.9±3.9比非高血压组:(14.0±4.2)mL/mmHg×10,P=0.001;C2,4.9±2.2比非高血压病组:(6.1±2.6)mL/mmHg×100,P=0.001]。2)高血压病患者2年的小动脉弹性指数变化值(ΔC2)显著低于非高血压病患者[-0.24±0.25比非高血压组:(0.75±0.31)mL/mmHg×100,P=0.014],其大动脉弹性指数变化值(ΔC1)也降低,但差异无统计学意义(P=0.458)。3)与非高血压病患者相比,高血压病患者基线空腹血清饱和脂肪酸、单不饱和脂肪酸水平均显著增高(P均<0.05),而n-3脂肪酸水平[(96.0±38.9)比非高血压病组:(123.0±36.0)μmol/L,P=0.001]、多不饱和与饱和脂肪酸比值(P/S)(1.60±0.37比非高血压病组:1.90±0.46,P=0.009)显著降低。4)logistic回归分析显示高血压病患者基线空腹血清亚麻酸(18:3n3)优势比(OR值)和95%可信区间为2.483(1.480～4.166,P=0.001),而DHA(22:6n3)的为0.329(0.195～0.554,P=0.001)。结论原发性高血压患者动脉弹性年龄相关降低过程加速,空腹血清FFA组成异常(如亚麻酸增高,DHA下降),可能是其高血压发病的危险因素之一。     

Successful Unrelated Cord Blood Transplantation Using a Reduced-Intensity Conditioning Regimen in a 6-Month-Old Infant with Congenital Neutropenia Complicated by Severe Pneumonia

Here we report the first successful unrelated cord blood transplantation (CBT) using reduced-intensity conditioning for the 'treatment of congenital neutropenia in a 6-month-old infant with complications of severe pneumonia probably due to Staphylococcus aureus infection. Because the patient showed no response to treatment with granulocyte colony-stimulating factor and had a cytogenetic aberration, unrelated CBT with an HLA-DRB1 genotypic mismatch was performed. The number of infused cells was 15 x 10(7)/kg. The preparative regimen was fludarabine, cyclophosphamide, and 6 Gy of total body irradiation. Teicoplanin was administered for bacterial pneumonia. Neutrophil engraftment was achieved on day 41 and was followed by clinical improvement. The patient gradually caught up on growth and development after the CBT. Unrelated CBT using a reduced-intensity conditioning regimen may be an effective treatment for congenital neutropenia.     

埋藏式心脏转复除颤器患者的随访

目的报道7例埋藏式心脏转复除颤器(ICD)治疗恶性室性心律失常的情况。方法对6例单腔ICD及1例双腔ICD植入者进行随访,通过ICD程控仪调出储存的资料,了解ICD对恶性心律失常的治疗情况,并对其工作情况进行分析。结果随访5～44个月,平均23.7个月,ICD共检测到持续性室性心动过速(VT)2 129次,心室颤动(VF)6次。采用抗心动过速起搏(ATP)治疗VT的成功率为91.9%,ATP未能终止的VT经低能量转复(CV)治疗95.3%转为窦性心律,少数VT经治疗后加速为VF。高能量除颤(DF)终止VF的成功率为100%。4例12次误将室上性心动过速误判断为室性事件而启动治疗,其中2次心房颤动,10次窦性心动过速,经调整VT的识别参数及联合使用抗心律失常药物后未再发生类似情况。2例随访期间出现晕厥,ICD存储的信息显示发生了VF并复律成功。2例出现焦虑症,经心理治疗改善。结论术后严密随访,及时调整工作参数,同时关注患者的心理健康并给予针对性的心理支持和治疗,对提高ICD的治疗效果至关重要。     

Long-term Follow up of Mesenchymal Hamartoma of Liver- Single Center Study

Background/Aim:

Mesenchymal hamartoma of liver (MHL) is a rare liver tumor of childhood. About 200 cases have been reported till now. Most of the work on MHL is limited to case reports and there are not many long term follow-up studies. We present our 20 years of experience with this uncommon entity. This study aims to highlight clinical features, diagnosis and treatment of MHL.

Materials and Methods:

All patients with a diagnosis of MHL in last 20 years were included in this retrospective study. The patients were evaluated clinically, radiologically and pathologically.

Results

The total number of patients with a diagnosis of MHL was nine. Mean age of the patients was 19.89 ± 2.75 months. Right lobe was involved in eight patients. The prominent clinical features were distension of abdomen and anorexia. Surgical options used were hepatic lobectomy, wedge resection and enucleation. Histopathology of the specimens showed cysts of variable size with normal hepatocytes, bile ducts and connective tissue stroma. Overall mortality was one (11.11%).

Conclusion:

MHL is a benign tumor that can present with various clinical features. It should be differentiated carefully from other liver masses especially malignant ones. The diagnosis can be made with the help of radiology and histopathology. Adequate resection is curative in most of the cases and long-term follow up is satisfactory.     

A Pharmacokinetic Study of Idarubicin in Japanese Patients With Malignant Lymphoma: Relationship With Leukocytopenia and Neutropenia

To clarify the pharmacokinetic properties of idarubicin (IDA) in Japanese patients and to clarify the relationship between the pharmacokinetic parameters of IDA or idarubicinol (IDAol), an active metabolite of IDA, and leukocytopenia or neutropenia, we examined the pharmacokinetics of IDA in patients with malignant lymphoma. Nine of 21 patients registered in an early phase II study of IDA were enrolled in the pharmacokinetic study. IDA (12 or 15 mg/m2) was administered by intravenous infusion for 5 minutes. The elimination half lives (t 1/2) of IDA were 11.0 hours and 12.5 hours after administration of 12 and 15 mg/m2 IDA, respectively. IDAol appeared rapidly both in plasma and in blood cells, and its concentrations exceeded those of IDA within 4 hours. IDAol had a very long t 1/2 (69.2 hours and 70.0 hours for 12 and 15 mg/m2, respectively). The areas under the concentration curves of IDAol in plasma were 3.4 and 5.8 times higher than those of IDA after administration of 12 and 15 mg/m2 IDA, respectively. The t 1/2 of IDAol in plasma correlated significantly with the nadir of neutrophils, and the steady-state volume of distribution of IDA in plasma and in blood cells correlated significantly with the nadirs of white blood cells and neutrophils. These results suggest that both IDA and IDAol play an important role in leukocytopenia or neutropenia. No substantial differences between Japanese and Caucasian people in the pharmacokinetics of IDA were apparent.