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一名未确诊血红蛋白S/β+地中海贫血儿童的致命脂肪栓塞综合征:急性细小病毒B19感染的并发症

Fatal fat embolism syndrome in a child with undiagnosed hemoglobin S/beta+ thalassemia: a complication of acute parvovirus B19 infection.

作者信息

Kolquist K A, Vnencak-Jones C L, Swift L, Page D L, Johnson J E, Denison M R

机构信息

Department of Pathology, Vanderbilt University Medical Center, Nashville, Tennessee 37232-2561, USA.

出版信息

Pediatr Pathol Lab Med. 1996 Jan-Feb;16(1):71-82.

PMID:8963632
Abstract

Anemia, mental status changes, and fatal respiratory failure complicated a febrile illness in a previously healthy 14-year-old black female. At autopsy, widespread fat emboli and bone marrow necrosis were found. Hemoglobin electrophoresis on an antemortem, pretransfusion specimen revealed hemoglobin S/beta+ thalassemia. Acute parvovirus B19 (PV B19) infection was suspected. Postmortem serum and a variety of paraffin-embedded tissues were assayed for PV B19 DNA using the polymerase chain reaction (PCR). The expected PCR product was identified in the serum specimen and in paraffin-embedded sections of bone marrow, kidney, spleen, parathyroid, thyroid, adrenal, and gastrointestinal tract: lung, liver, ovary, fallopian tube, uterus, brain, heart, and pancreas were negative. PV B19 infection is highly contagious and may be rapidly fatal in children with hemoglobinopathies by several mechanisms, including fat embolism. Therefore, there exists the risk of multiple deaths within a family. The acute infection may be easily and expeditiously diagnosed using serum or a variety of paraffin-embedded tissues.

摘要

一名既往健康的14岁黑人女性在发热性疾病后出现贫血、精神状态改变及致命性呼吸衰竭。尸检发现广泛的脂肪栓塞和骨髓坏死。生前输血前标本的血红蛋白电泳显示为血红蛋白S/β+地中海贫血。怀疑为急性细小病毒B19(PV B19)感染。使用聚合酶链反应(PCR)对死后血清及多种石蜡包埋组织检测PV B19 DNA。在血清标本以及骨髓、肾脏、脾脏、甲状旁腺、甲状腺、肾上腺和胃肠道的石蜡包埋切片中鉴定出预期的PCR产物:肺、肝、卵巢、输卵管、子宫、脑、心脏和胰腺为阴性。PV B19感染具有高度传染性,通过包括脂肪栓塞在内的多种机制,可能导致血红蛋白病患儿迅速死亡。因此,家庭中存在多人死亡的风险。使用血清或多种石蜡包埋组织可轻松快速地诊断急性感染。

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