婴儿朗格汉斯细胞组织细胞增生症

IF 2.7 3区 医学 Q1 PEDIATRICS Pediatric Pulmonology Pub Date : 2024-10-28 DOI:10.1002/ppul.27352
Diya Ye, Tianming Yuan, Mizu Jiang
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引用次数: 0

摘要

一名 2 个月大的男婴因持续轻微咳嗽和左眼睑下的粉红色丘疹就诊。影像学检查发现右肺中叶有弥漫性肺不张和囊性阴影,但无细菌或真菌感染迹象。皮肤活检显示 CD207/Langerin 和 CD1a 免疫组化染色阳性,诊断为朗格汉斯细胞组织细胞增生症(LCH)。化疗后,婴儿的肺大泡消退了,但患者仍在接受监测,以防复发。朗格汉斯细胞组织细胞增生症因其炎症性和恶性特征而闻名,通常表现为多系统受累,包括肺部表现。及时诊断和治疗对于控制这种罕见的婴儿疾病至关重要。
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Langerhans cell histiocytosis in an infant.

A 2-month-old male infant presented with a persistent mild cough and a pink papule beneath the left eyelid. Imaging studies revealed diffuse lung opacities and a cystic shadow in the right middle lobe, with no evidence of bacterial or fungal infection. Skin biopsy demonstrated positive immunohistochemical staining for CD207/Langerin and CD1a, leading to a diagnosis of Langerhans-cell histiocytosis (LCH). The infant's pulmonary bullae resolved following chemotherapy, and the patient is under surveillance for recurrence. LCH, recognized for its inflammatory and malignant characteristics, often presents with multisystemic involvement, including pulmonary manifestations. Timely diagnosis and treatment are crucial for managing this rare disorder in infants.

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来源期刊
Pediatric Pulmonology
Pediatric Pulmonology 医学-呼吸系统
CiteScore
6.00
自引率
12.90%
发文量
468
审稿时长
3-8 weeks
期刊介绍: Pediatric Pulmonology (PPUL) is the foremost global journal studying the respiratory system in disease and in health as it develops from intrauterine life though adolescence to adulthood. Combining explicit and informative analysis of clinical as well as basic scientific research, PPUL provides a look at the many facets of respiratory system disorders in infants and children, ranging from pathological anatomy, developmental issues, and pathophysiology to infectious disease, asthma, cystic fibrosis, and airborne toxins. Focused attention is given to the reporting of diagnostic and therapeutic methods for neonates, preschool children, and adolescents, the enduring effects of childhood respiratory diseases, and newly described infectious diseases. PPUL concentrates on subject matters of crucial interest to specialists preparing for the Pediatric Subspecialty Examinations in the United States and other countries. With its attentive coverage and extensive clinical data, this journal is a principle source for pediatricians in practice and in training and a must have for all pediatric pulmonologists.
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