小儿紫癜性肾炎诊治进展

过敏性紫癜 (ＨＳＰ)是儿童最常见的毛细血管变态反应性疾病之一 ,以全身广泛性小血管无菌性炎症为病理基础。该病常侵犯肾脏 ,发生率报告不一 ;以尿常规改变诊断肾损害发生率为２０ %～６０% ,而以尿酶改变或病理改变分析其发生率为１００ %。因而紫癜性肾炎 (ＨＳＰＮ)已成为儿童时期肾脏疾病的主要病种之一 ,严重危害儿童健康。现将该病有关病因、发病机理、诊断及治疗综述如下。病因与发病机理一、病因ＨＳＰ主要因各种致敏因素 ,如各种感染 ,富含蛋白质的食物、药物 ,其他如花粉、油漆、预防接种等使机体发生变态反应而引起。另外 ,机…     

儿童慢性特发性血小板减少性紫癜治疗进展

儿童慢性特发性血小板减少性紫癜是由约 2 0 %～ 2 5 %的急性ITP发展而来 ,其治疗特点呈慢性难治性 ,目前治疗包括肾上腺皮质激素、静脉免疫球蛋白、抗D抗体、α -干扰素治疗、脾脏切除及其它免疫抑制治疗。肾上腺皮质激素间歇短程大剂量冲击疗法副作用小、花费低且能获得几乎 5 0 %的有效率 ;静脉免疫球蛋白已广泛用于临床特别对急危重症其疗效可达83～ 88% ;抗D抗体有效率可达 80 % ,且能持续保持良好反应 ;α -干扰素的治疗可用于延迟小儿脾切除或者脾切除术前提高血小板数 ;抗CD2 0 单抗可尝试用于治疗儿童慢性、难治性ITP。脾切除是治疗慢性ITP的有效方法 ,其完全缓解率可达 70～ 80 % ,但应严格掌握指征     

儿童紫癜性肾炎诊断和治疗进展

过敏性紫癜的基本病变是弥漫性血管炎 ,肾损害是本症的基本症状之一。紫癜性肾炎为儿科常见的继发性肾小球疾病 ,也属免疫复合物性肾炎。近年随着对紫癜性肾炎的深入研究 ,在诊断方面已不只限于传统的临床诊断 ,而是包括从病因、临床、病理及转归等４个方面系统地进行诊断。在治疗方面也有新的进展。兹将有关临床诊断及治疗 ,结合文献分述如下。临床诊断紫癜性肾炎肾脏损害主要表现为血尿、蛋白尿。其症状可出现于过敏性紫癜的整个病程 ,但多数发生于紫癜出现后２周～４周。某些病例以肉眼血尿为首发症状 ,易于误诊 ,故不明原因的血尿 ,紫癜…     

紫癜性肾炎的诊断与治疗

紫癜性肾炎(HSPN)是儿科常见病,发病率呈逐年升高趋势,越来越引起儿科医师的重视.现对本病的临床表现、辅助检查和治疗的新进展进行论述,以期对临床工作者有所帮助.     

小儿弥散性血管内凝血43例临床分析

弥散性血管内凝血 (ＤＩＣ)是一种获得性的临床综合征 ,发生在许多疾病的病理过程中。是许多危重症常见并发症 ,重症者死亡率可高达 80 %。但如能及时诊治 ,尚能取得满意效果。本文将我院近 10年来收治的小儿ＤＩＣ 4 3例总结如下 ,以供临床诊治参考。1　临床资料1.1　一般资料     

婴儿暴发性紫癜1例

患儿男,5月龄,主因"发热3d,四肢出血性皮疹伴水肿、少尿1d"于2006-10-15入院.患儿入院前3d无明显诱因出现发热,当地医院给予静点林可霉素、病毒唑抗感染治疗.入院前1d患儿双下肢出现紫色皮疹,对称性分布.     

紫癜性肾炎治疗进展

紫癜性肾炎 (HSPN)是继发于过敏性紫癜(HSP)的肾炎 ,是直接影响HSP病程和预后的决定因素之一 ,因此近年HSPN的研究越来越受到人们的重视 ,对其治疗的研究也有一定新的进展 ,现综述如下。一、肾外表现的治疗根据症状采取相应的治疗措施 ,发热和关节肿痛可以用解热镇痛药;有荨麻疹样皮疹、水肿及腹痛时 ,可用抗组胺及解痉药物(如阿托品、654 -2等)。近年来有报道H2 受体阻滞剂西咪替丁治疗本病 ,对控制皮疹及减轻内脏损伤有利[1]。其机制为竞争性拮抗组胺 ,改善血管通透性 ,从而减轻皮肤粘膜及内脏器官的水肿出血。肾上腺皮质激素对缓解…     

Postvaricella purpura fulminans

Purpura fulminans (PF) is an infrequent complication of varicella characterized by the progressive development of purpuric or painful ecchymotic lesions associated with biochemical alternations typical of consumption coagulopathy. Activation of coagulation is due to a marked and prolonged decrease in protein S, which is probably secondary to the formation of antiprotein S antibodies. The mechanism responsible for the synthesis of these autoantibodies is unknown. We present three cases of postvaricella PF and review the clinical and biochemical characteristics of this entity, as well as current diagnostic and therapeutic recommendations.     

Diagnosis and management of neonatal purpura fulminans

Neonatal purpura fulminans is a rare, life-threatening condition, caused by congenital or acquired deficiencies of protein C or S. The condition is often fatal unless there is early recognition of the clinical symptoms, prompt diagnosis, and judicious replacement therapy is initiated. The clinical presentation is that of acute disseminated intravascular coagulation and hemorrhagic skin necrosis. The management includes an acute phase of replacement therapy with fresh frozen plasma or protein C concentrate and a maintenance therapy that includes anticoagulation with warfarin or low molecular weight heparin. This review focuses on the management of severe protein C deficiency.     

Topical nitroglycerin and pain in purpura fulminans

Topical nitroglycerin has been previously described as an adjunctive therapy to increase perfusion to areas of purpura affected in purpura fulminans. We report a case of purpura fulminans in which topical nitroglycerin was found to provide analgesia after its application to purpuric lesions. The broader role for the use of topical nitroglycerin in pain management deserves further study and evaluation.     

Idiopathic purpura fulminans with transient protein S deficiency

Idiopathic purpura fulminans produces rapidly progressive hemorrhagic necrosis of the skin with disseminated intravascular coagulation in individuals without known abnormalities of the protein C pathway or acute infections. The disease mainly affects children and in 90 % of cases is preceded by a benign infection. Its pathogenesis involves a temporary autoimmune protein S deficiency that provokes a state of hypercoagulability. We present the case of a previously healthy 2-year-old boy with hemorrhagic skin lesions characteristic of purpura fulminans and disseminated intravascular coagulation without sepsis. Severe, temporary protein S deficiency was confirmed. The patient received daily replacement therapy with fresh frozen plasma for 12 days and anticoagulation with heparin for 3 months. Evolution was favorable. Although the other parameters returned to normal, protein S remained low for 50 days despite treatment. The patient has made a complete recovery.     

Meningococcal purpura fulminans treated with medicinal leeches.

INTRODUCTION: Meningococcal septicemia remains one of the most common infectious causes of admission to a pediatric intensive care unit. Numerous treatment strategies aimed at the thromboembolic complications inducing purpura fulminans and limb/digital ischemia have been attempted, with variable results. The successful use of medicinal leeches for pneumococcal purpura fulminans has been described, and we present a similar case of meningococcal purpura fulminans. PATIENT AND INTERVENTION: A 5-wk-old female infant with meningococcal meningitis and septicemia and progressive purpura fulminans of the left hand was treated with medicinal leeches. Medicinal leeches were applied to the left dorsal hand on a daily basis for 4 consecutive days. RESULT: The swelling and limited functionality visibly improved after 48 hrs, and by 120 hrs, perfusion in the distal phalanges of the thumb and middle finger was evident. Reperfusion of the distal phalanges was not fully sustained, and at 6 wks the plastic surgery department debrided the distal phalanges of her left hand, excluding the thumb. She fully recovered from the meningococcal septicemic shock; the functionality of her left thumb was preserved, and she has limited functionality of her left hand. CONCLUSION: The unique combination of salivary products in leech therapy has theoretical benefits and requires future study.     

Neonatal purpura fulminans due to homozygous protein C deficiency

Severe and recurrent purpura fulminans developed in a Turkish boy at 1 week of age. Initial coagulation studies performed were compatible with disseminated intravascular coagulation. Subsequent investigations showed that the patient had homozygous and his healthy parents had heterozygous protein C deficiency. The episodes of purpura fulminans were controlled by infusions of fresh frozen plasma and heparinization. Oral anticoagulant therapy was given in the symptom-free period.     

A novel protein C mutation causing neonatal purpura fulminans

Background

Neonatal purpura fulminans due to congenital protein C deficiency is a rare disorder.

Case characteristics

A four-day-old neonate presented with multiple necrotic skin lesions with abnormal coagulation profile.

Intervention and outcome

Skin lesions responded to repeated plasma transfusions but the neonate developed bilateral retinal detachment. A novel homozygous PROC gene mutation was noted in the neonate.

Message

Molecular diagnosis and prenatal counseling in neonatal purpura fulminans are vital considering the poor outcome.