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一名患有动脉血栓形成和先天性肾缺如的新生儿暴发性紫癜患者

Neonatal Purpura Fulminans in a Patient With Arterial Thrombosis and Congenital Renal Agenesis.

作者信息

Abbaker Mawada M, Edupuganti Srujan, Elmukashfi Sheimaa T, Elsheikh Taha Ahmed E

机构信息

Pediatrics, Hurley Medical Center, Flint, USA.

Internal Medicine/Pediatrics, Hurley Medical Center, Flint, USA.

出版信息

Cureus. 2024 Jan 18;16(1):e52503. doi: 10.7759/cureus.52503. eCollection 2024 Jan.

DOI:10.7759/cureus.52503
PMID:38371013
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10874227/
Abstract

Neonatal purpura fulminans (PF) is an uncommon skin disorder characterized by acute disseminated intravascular coagulation, tissue necrosis, and small vessel thrombosis. Here, we present a case of a seven-day-old male who was admitted to the Neonatal Intensive Care (NICU) Unit at Gaafar Ibn Auf Tertiary Hospital, in January 2023. He presented with black bullous lesions on the plantar surface of the left foot, deep bluish discoloration over the right buttock and right lower abdomen, and gangrenous changes in the right foot with clear demarcation. Birth history was not significant other than mild pallor and icterus. His blood workup was consistent with severe anemia, thrombocytopenia, elevated prothrombin time, and partial thromboplastin time with decreased protein C and S levels; blood culture yielded no growth. A Doppler ultrasound scan of lower extremities confirmed distal occlusion of the right dorsalis pedis artery. The abdominal ultrasound revealed a free left renal bed and left-sided renal agenesis. We came to a diagnosis of neonatal PF and started administering blood and fresh frozen plasma and subcutaneous heparin injections, but unfortunately, the patient eventually passed away. Hence, we decided to report this case to emphasize the significance of the clinical picture in assisting with early diagnosis, despite limited access to genetic testing. We also want to highlight the importance of a "high index of suspicion" that might be mandatory for better outcomes.

摘要

新生儿暴发性紫癜(PF)是一种罕见的皮肤疾病,其特征为急性弥散性血管内凝血、组织坏死和小血管血栓形成。在此,我们报告一例7日龄男性病例,该患儿于2023年1月入住加菲尔·伊本·奥夫三级医院的新生儿重症监护病房(NICU)。他的左脚足底出现黑色大疱性皮损,右臀部和右下腹部有深青色变色,右脚出现坏疽性改变且界限清晰。除轻度苍白和黄疸外,出生史无其他特殊情况。他的血液检查结果显示为严重贫血、血小板减少、凝血酶原时间延长、部分凝血活酶时间延长,蛋白C和S水平降低;血培养无细菌生长。下肢多普勒超声扫描证实右足背动脉远端闭塞。腹部超声显示左肾床游离和左侧肾缺如。我们诊断该患儿为新生儿PF,并开始输注血液和新鲜冰冻血浆以及皮下注射肝素,但不幸的是,患儿最终死亡。因此,我们决定报告此病例,以强调尽管基因检测条件有限,但临床表现对协助早期诊断的重要性。我们还想强调“高度怀疑指数”的重要性,这可能是获得更好治疗结果所必需的。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c014/10874227/dca156c527ff/cureus-0016-00000052503-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c014/10874227/13dfe7ba5539/cureus-0016-00000052503-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c014/10874227/8337472663cc/cureus-0016-00000052503-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c014/10874227/c5bc75c9f5ea/cureus-0016-00000052503-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c014/10874227/dca156c527ff/cureus-0016-00000052503-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c014/10874227/13dfe7ba5539/cureus-0016-00000052503-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c014/10874227/8337472663cc/cureus-0016-00000052503-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c014/10874227/c5bc75c9f5ea/cureus-0016-00000052503-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c014/10874227/dca156c527ff/cureus-0016-00000052503-i04.jpg

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Case Report: Successful Long-Term Management of a Low-Birth Weight Preterm Infant With Compound Heterozygous Protein C Deficiency With Subcutaneous Protein C Concentrate Up to Adolescence.病例报告:低出生体重早产复合杂合子蛋白C缺乏婴儿采用皮下注射蛋白C浓缩物进行长期成功管理直至青春期
Front Pediatr. 2021 Sep 28;9:591052. doi: 10.3389/fped.2021.591052. eCollection 2021.
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Neonatal purpura fulminans caused by Acinetobacter baumannii: Unusual occurrence of three coincident cases.
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Compound heterozygous protein C variants undetectable by common laboratory testing causing purpura fulminans after the neonatal period.
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