匹多莫德治疗儿童过敏性紫癜疗效评价

目的：评价匹多莫德对儿童过敏性紫癜的治疗效果。方法选取90例过敏性紫癜患儿,随机分为实验组(n=45)和对照组(n=45),实验组使用匹多莫德联合常规方法治疗过敏性紫癜,对照组予常规方法治疗,比较两组患儿临床症状消失时间,以及IgG、IgA、IgM和T淋巴细胞亚群变化情况。结果(1)实验组临床症状消失时间及住院时间较对照组明显缩短,差异有统计学意义(P<0.01);实验组皮肤紫癜反复发生率显著低于对照组,差异有统计学意义(P<0.05)。(2)治疗前两组外周血IgG、IgA、IgM水平以及T淋巴细胞亚群差异无统计意义(孕>0.05);治疗结束后,实验组IgA和CD8+水平较对照组显著降低,其IgG、IgM、CD4+和CD4+/CD8+水平较对照组显著升高,差异有统计学意义(P<0.05)。(3)实验组病情复发率较对照组明显降低,差异有统计学意义(P<0.05)。结论匹多莫德联合常规方法治疗HSP效果好,临床治疗中值得借鉴参考。     

Cardiovascular Complications and Their Association With Mortality in Patients With Thrombotic Thrombocytopenic Purpura

BackgroundDespite widespread availability of plasmapheresis, the mortality in thrombotic thrombocytopenic purpura remains high. Cardiovascular complications have been reported as an important cause of morbidity in these patients. The burden and prognostic implications of these complications have not been well studied. We analyzed the rates of cardiovascular complications in thrombotic thrombocytopenic purpura, temporal trends, and studied its impact on in-hospital mortality.MethodsWe analyzed the National Inpatient Sample (NIS) from January 2005 to September 2015 to identify adult patients with thrombotic thrombocytopenic purpura. This group was further refined by excluding patients who did not receive therapeutic plasmapheresis, and other conditions that can mimic thrombotic thrombocytopenic purpura. We identified the age- and sex-stratified rates of cardiac arrhythmias, cardiac conduction system disorders, heart failure, acute coronary syndrome, myocarditis, pericarditis, takotsubo cardiomyopathy, cardiogenic shock, cardiac arrest, and stroke. We also compared in-hospital mortality with and without cardiovascular complications.ResultsAmong 15,054 thrombotic thrombocytopenic purpura hospitalizations (mean age 46.4 years, 69% in the 18- to 54-age group, 66.2% women, and 42.9% white), a cardiovascular complication was observed in 3802 (25.3%) hospitalizations. The following cardiovascular complications were identified: stroke (10.4%), heart failure (8.3%), acute coronary syndrome (6.4%), atrial tachyarrhythmia (5.9%), ventricular tachyarrhythmia (2.0%), cardiogenic shock (0.5%), takotsubo cardiomyopathy (0.1%), atrioventricular block (0.2%), myocarditis or pericarditis (0.3), and cardiac arrest (1.9%). Rates of several cardiovascular complications were significantly higher in patients 55 years or older compared to a younger age group, whereas males had higher rates of acute coronary syndrome and tachyarrhythmias compared to females. Overall, the cardiovascular complication rate was stable during the study period. The presence of a major cardiovascular complication was associated with a significantly higher in-hospital mortality (19.7%) as compared with no major cardiovascular complication (4.1%) (adjusted odds ratio 2.09, 95% confidence interval 1.41-3.09, P <0.001). Results were generally consistent in age and sex subgroups.ConclusionCardiovascular complications were frequently observed at a rate of 1 in 4 in patients hospitalized for thrombotic thrombocytopenic purpura and were associated with substantially higher in-hospital mortality. These findings underscore the need to promptly identify and treat these complications to improve outcomes.     

Neglect‐induced pseudo‐thrombotic thrombocytopenic purpura due to vitamin B12 deficiency

Although thrombotic thrombocytopenic purpura (TTP) is rare, early diagnosis and treatment are important for decreasing the mortality rate. Acquired vitamin B12 deficiency is frequently overlooked because of its rarity in developed countries, particularly in children and adolescents. The hematological changes in vitamin B12 deficiency present as megaloblastic anemia, increased lactate dehydrogenase, vasoconstriction, increased platelet aggregation, and abnormal activation of the coagulation followed by microangiopathy as well as neutropenia and thrombocytopenia. We report herein the case of a 15‐year‐old girl who had been neglected, which might have caused pseudo‐TTP through malnutrition, particularly vitamin B12 deficiency. When we encounter cases of TTP in children, clinicians must be aware of the possibility of malnutrition, particularly with vitamin B12 deficiency, even in developed countries, and investigate the cause of malnutrition including neglect.     

Successful living domino liver transplantation in a child with protein C deficiency

PC is produced in the liver and inhibits blood coagulation by catalyzing active factors V and VIII. PC deficiency causes abnormal blood clotting that is difficult to regulate by anticoagulative treatments. Four reports of PC deficiency treated with LTx have been published; however, no report of DLT as a therapy for PC deficiency is available. We describe a case of a 23‐month‐old girl who received DLT for compound heterozygous PC deficiency. Her PC activity was below 5%. She developed intracranial lesion and frequent refractory purpura fulminans. Both her parents had heterozygous mutations of PC genes and were excluded as living donors. Furthermore, she was a low priority on the waiting list of deceased‐donor transplantation. We performed living DLT using the liver from a patient with MSUD. Activated PC concentrate safely supported the perioperative period. After DLT, she maintained normal PC activities and BCAA levels. This is the first case of PC deficiency successfully treated by living DLT with MSUD. We propose that DLT using liver from patients with MSUD is a treatment option for PC deficiency.     

CTLA-4 exon 1 polymorphism in patients with autoimmune blood disorders

CTLA-4 is a CD28 homologue that plays an important role in negative regulation of T-cell responses. Its transient expression on the surface of activated T cells antagonizes the activating signals and terminates the T-cell response. An A to G polymorphism at position 49 of the CTLA-4 first exon has recently been associated with several autoimmune disorders. In the present study we have examined the prevalence of the A and G alleles of the CTLA-4 gene in 50 patients with autoimmune hemolytic anemia (AIHA), of which 20 had idiopathic AIHA and 30 had AIHA and chronic lymphocytic leukemia (CLL), and in 60 patients with immune thrombocytopenic purpura (ITP). Control subjects were 100 healthy individuals and 100 CLL patients without clinical evidence for an autoimmune disease. The G allele was present at a significantly higher frequency among the patients with AIHA (P = 0.003), whereas no difference was observed between patients with ITP and controls. The G allele frequency was highest among CLL patients who had developed AIHA. The obtained data indicate that the G allele of CTLA-4 predisposes to the development of AIHA, particularly among patients with CLL.     

Splenectomy for the treatment of thrombotic thrombocytopenic purpura

Plasma exchange is the treatment of choice for patients with thrombotic thrombocytopenic purpura (TTP) and results in remission in >80% of the cases. Treatment of patients who are refractory to plasma therapy or have relapsing disease is difficult. Splenectomy has been a therapeutic option in these conditions but its value remains controversial. We report on a series of 33 patients with TTP who were splenectomised because they were plasma refractory (n = 9) or for relapsed disease (n = 24). Splenectomy generated prompt and unmaintained remissions in all except five patients, in whom remission was delayed (n = 4) or who died with progressive disease (n = 1). Four postoperative complications occurred: one pulmonary embolism and three surgical complications. Median follow-up after splenectomy was 109 months (range 28-230 months). The overall postsplenectomy relapse rate was 0.09 relapses/patient-year and the 10-year relapse-free survival (RFS) was 70% (95% CI 50-83%). In the patients with relapsing TTP, relapse rate fell from 0.74 relapses/patient-year before splenectomy to 0.10 after splenectomy (P < 0.00001). Two patients died from first postsplenectomy relapse. Although these results are based on retrospective data and that the relapse rate may spontaneously decrease with time, we conclude that splenectomy, when performed during stable disease, has an acceptable safety profile and should be considered in cases of plasma refractoriness or relapsing TTP to reach durable remissions and to reduce or prevent future relapses.     

Epidermoid cyst in intrapancreatic accessory spleen: A systematic review

Background/Objectives

Due to its rarity, epidermoid cyst in intrapancreatic accessory spleen (ECIPAS) is still a diagnostic dilemma during clinical practice. The aim of this review was to summarize the epidemiologic features and management of ECIPAS.

Nurses best practices for the management of thrombotic thrombocytopenic purpura

A group of Canadian apheresis nurses developed best practice for in the management of thrombotic thrombocytopenic purpura (TTP). The recommendations address issues related to infusion protocols, preventing and managing adverse events, comprehensive patient assessments, treatment procedures, as well as pre- and post- treatment care. The Canadian group encourages institutes to include nurses on committees that examine recommendations for TTP management.