婴儿梅毒性肝炎--肝病学家必须了解的被遗忘的疾病:从系列病例到文献修订。

IF 3 3区 医学 Q1 PEDIATRICS European Journal of Pediatrics Pub Date : 2024-11-01 Epub Date: 2024-09-20 DOI:10.1007/s00431-024-05698-x
Valeria Delle Cave, Geremia Zito Marinosci, Dolores Ferrara, Francesco Esposito, Andrea Lo Vecchio, Marco Sciveres, Claudia Mandato, Daniele De Brasi, Paolo Siani, Giusy Ranucci
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引用次数: 0

摘要

先天性梅毒(CS)的临床表现包括伴有/不伴有肝功能受损的肝病,即梅毒性肝炎。肝脏受累可能很严重;因此,早期诊断对于提供治疗和防止致命后果至关重要。近年来,全球范围内又出现了 CS 病例。我们报告了因肝脏疾病住院并最终诊断为CS的婴儿的系列病例,强调了肝脏受累的广泛性、晚期诊断病例的快速进展以及治疗这种被遗忘但又重新出现的疾病的陷阱。我们对 2008-2023 年间出现肝脏病变的 CS 患者进行了回顾性分析。我们收集了五例病例(三例女性),中位年龄为 13.8 天(范围为 1-84 天)。其中三例患者的母亲在怀孕期间未接受梅毒筛查,两例患者的母亲在怀孕头三个月的筛查中血清反应呈阴性。没有人在孕期接受过特殊治疗。肝脏受累的特点是肝脾肿大,其中四例伴有胆汁淤积性黄疸,三例伴有肝功能衰竭。所有病例的母亲和婴儿的快速血浆凝集素(RPR)和苍白螺旋体血凝试验(TPHA)均呈阳性。CS表现为多器官受累,其中一例患者死亡:结论:对于患有胆汁淤积症和急性肝功能衰竭的婴儿以及孤立性肝脏肿大的患病婴儿,考虑 CS 非常重要。早期识别患有 CS 的婴儿对于发现孕期漏诊病例并开始早期治疗至关重要。
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Syphilitic hepatitis in infants, the forgotten disease that hepatologists have to brush up on: from a case series to a revision of literature.

Clinical manifestations of congenital syphilis (CS) include liver disease with/without impaired liver function, identified as syphilitic hepatitis. Hepatic involvement may be dramatic; therefore, early diagnosis is crucial to provide treatment and prevent fatal outcomes. A new resurgence of CS cases has been described in recent years worldwide. We reported our experience with a case series of infants hospitalized for liver disease with a final diagnosis of CS, highlighting the wide spectrum of liver involvement, the rapid progression in cases with late diagnosis, and the pitfalls of the management of this forgotten but reemerging disease. A retrospective analysis of CS patients with hepatic presentation in the period 2008-2023 was conducted. We collected five cases (three female) with a median age of 13.8 days (range 1-84 days). In three cases, mothers were not screened for syphilis during pregnancy, and in two cases, they were seronegative in the first trimester screening. None practiced specific therapy during pregnancy. Hepatic involvement was characterized by hepatosplenomegaly, in four cases associated with cholestatic jaundice and in three cases with liver failure. Rapid plasma reagin (RPR) and Treponema pallidum hemagglutination assay (TPHA) were positive in all cases in mothers and infants. CS presented with multiorgan involvement and was fatal in one case.Conclusions: It is important to consider CS in infants with cholestasis and acute liver failure, but also in sick infants with isolated hepatomegaly. Early recognition of infants with CS is critical to identify missed cases during pregnancy and to start early treatment.

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来源期刊
CiteScore
5.90
自引率
2.80%
发文量
367
审稿时长
3-6 weeks
期刊介绍: The European Journal of Pediatrics (EJPE) is a leading peer-reviewed medical journal which covers the entire field of pediatrics. The editors encourage authors to submit original articles, reviews, short communications, and correspondence on all relevant themes and topics. EJPE is particularly committed to the publication of articles on important new clinical research that will have an immediate impact on clinical pediatric practice. The editorial office very much welcomes ideas for publications, whether individual articles or article series, that fit this goal and is always willing to address inquiries from authors regarding potential submissions. Invited review articles on clinical pediatrics that provide comprehensive coverage of a subject of importance are also regularly commissioned. The short publication time reflects both the commitment of the editors and publishers and their passion for new developments in the field of pediatrics. EJPE is active on social media (@EurJPediatrics) and we invite you to participate. EJPE is the official journal of the European Academy of Paediatrics (EAP) and publishes guidelines and statements in cooperation with the EAP.
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