5例儿童造血干细胞移植后脂肪组织分布异常及代谢异常:获得性部分脂肪营养不良的新病因

IF 1 Q4 ENDOCRINOLOGY & METABOLISM Clinical Pediatric Endocrinology Pub Date : 2013-10-01 DOI:10.1297/cpe.22.53
M. Adachi, Y. Asakura, K. Muroya, H. Goto, H. Kigasawa
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引用次数: 16

摘要

我们报告了5例连续接受造血干细胞移植(HSCT)治疗早期白血病或神经母细胞瘤的患者,这些患者后来表现出异常的脂肪组织分布模式。臀区和四肢脂肪萎缩明显,而脸颊、颈部和腹部皮下脂肪保留。此外,内脏脂肪沉积、肝脏脂肪改变和代谢紊乱(如胰岛素抵抗和高甘油三酯血症)也很明显。这些特征类似于邓尼根型家族性部分脂肪营养不良,这是一种罕见的由LMNA基因突变引起的疾病。这些患者有共同的HSCT病史,包括接受全身照射(TBI)。由于多发转移(n = 3)、复发(n = 3)和重复HSCT (n = 3),他们也接受了强化化疗。我们提出HSCT是获得性部分脂肪营养不良的新病因,并建议在生命早期接受HSCT合并TBI和强化化疗的患者必须仔细观察可能发生的脂肪营养不良和代谢并发症。
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Abnormal Adipose Tissue Distribution with Unfavorable Metabolic Profile in Five Children Following Hematopoietic Stem Cell Transplantation: A New Etiology for Acquired Partial Lipodystrophy
We report five consecutive patients who underwent hematopoietic stem cell transplantation (HSCT) to treat leukemia or neuroblastoma early in their lives and later manifested abnormal patterns of adipose tissue distribution. Lipoatrophy was remarkable in the gluteal regions and extremities, whereas subcutaneous fat was preserved in the cheeks, neck, and abdomen. In addition, visceral fat deposition, fatty changes in the liver, and metabolic derangements such as insulin resistance and hypertriglyceridemia were evident. These features resemble Dunnigan-type familial partial lipodystrophy, which is a rare condition caused by LMNA gene mutation. These patients shared a common medical history involving HSCT, including conditioning with total body irradiation (TBI). They also received intensive chemotherapy because of multiple metastases (n = 3), relapse (n = 3), and repetitive HSCT (n = 3). We propose HSCT as a new etiology for acquired partial lipodystrophy and recommend that patients who undergo HSCT with TBI and intensive chemotherapy early in their lives must receive careful observation for the possible development of lipodystrophy and metabolic complications.
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来源期刊
Clinical Pediatric Endocrinology
Clinical Pediatric Endocrinology ENDOCRINOLOGY & METABOLISM-
CiteScore
2.40
自引率
7.10%
发文量
34
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