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病态肥胖作为隐匿性下丘脑 - 垂体朗格汉斯细胞组织细胞增多症的早期表现及治疗延迟

Morbid Obesity as Early Manifestation of Occult Hypothalamic-Pituitary LCH with Delay in Treatment.

作者信息

Keates-Baleeiro Jennifer, Rincon Marielisa

机构信息

Department of Pediatric Hematology-Oncology, T.C. Thompson Children's Hospital at Erlanger, 910 Blackford Street, Chattanooga, TN 37403, USA.

Department of Endocrinology, T.C. Thompson Children's Hospital at Erlanger, 910 Blackford Street, Chattanooga, TN 37403, USA.

出版信息

Case Rep Oncol Med. 2015;2015:915716. doi: 10.1155/2015/915716. Epub 2015 Nov 30.

Abstract

Morbid obesity presents unique challenges in managing additional disease processes. A 16-year-old male with a history of central diabetes insipidus (DI) and hypothyroidism developed destructive lesions in both his right mandible and brain, which were not discovered until the patient presented for tinnitus, 8 years after his initial diagnosis with DI. Langerhans cell histiocytosis (LCH) was diagnosed on pathologic biopsy. The patient's initial body mass index (BMI) was 54.5 kg/m(2) so a unique treatment approach with single agent cladribine (2-CdA) was offered as traditional steroid therapy could worsen his endocrine dysfunction. The patient presented with neurodegenerative sequelae from the central LCH, possibly due to a delay in diagnosis and therapy. This case highlights difficulties in managing obese patients in an oncology setting and provides an illustrative case of how obesity may mask other comorbid conditions. Close supervision of complex obese patients with coordinated endocrinology and oncology care is vital. For the primary care practitioner, monitoring abrupt changes in BMI with serial cranial imaging may lead to a prompt diagnosis and prevention of further neurodegenerative effects. The use of 2-CdA was found to successfully bring the patient's LCH into remission without the additional risks of steroid therapy in a morbidly obese patient.

摘要

病态肥胖在管理其他疾病过程中带来了独特的挑战。一名16岁男性,有中枢性尿崩症(DI)和甲状腺功能减退病史,在其右下颌骨和脑部出现了破坏性病变,直到患者因耳鸣就诊时才被发现,此时距离他最初被诊断为DI已有8年。经病理活检诊断为朗格汉斯细胞组织细胞增多症(LCH)。患者最初的体重指数(BMI)为54.5kg/m²,由于传统的类固醇疗法可能会加重其内分泌功能障碍,因此提供了一种独特的单药克拉屈滨(2-CdA)治疗方法。该患者出现了中枢性LCH导致的神经退行性后遗症,可能是由于诊断和治疗的延迟。本病例突出了肿瘤学环境中管理肥胖患者的困难,并提供了一个肥胖可能掩盖其他合并症的实例。对复杂肥胖患者进行密切监测,并协调内分泌和肿瘤学护理至关重要。对于初级保健医生来说,通过连续的头颅影像学监测BMI的突然变化可能会导致及时诊断并预防进一步的神经退行性影响。结果发现,使用2-CdA成功使患者的LCH缓解,而不会给病态肥胖患者带来类固醇疗法的额外风险。

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