Abnormal Adipose Tissue Distribution with Unfavorable Metabolic Profile in Five Children Following Hematopoietic Stem Cell Transplantation: A New Etiology for Acquired Partial Lipodystrophy
Abnormal Adipose Tissue Distribution with Unfavorable Metabolic Profile in Five Children Following Hematopoietic Stem Cell Transplantation: A New Etiology for Acquired Partial Lipodystrophy
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造血干细胞移植后五名儿童的异常脂肪组织分布和不利的代谢特征：获得性部分性脂肪营养不良的新病因
DOI：
10.1297/cpe.22.53
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发表时间：
2013
影响因子：
1.4
通讯作者：
H. Kigasawa
中科院分区：
文献类型：
--
作者：
M. Adachi;Y. Asakura;K. Muroya;H. Goto;H. Kigasawa
我们报告了五个连续的患者谁接受造血干细胞移植（HSCT）治疗白血病或神经母细胞瘤在他们的生命早期，后来表现出脂肪组织分布的异常模式。臀部和四肢的脂肪萎缩明显，而脸颊、颈部和腹部的皮下脂肪保留。此外，内脏脂肪沉积、肝脏脂肪变化和代谢紊乱（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Dunnigan型家族性部分脂肪营养不良，这是一种罕见的LMNA基因突变引起的疾病。这些患者有共同的HSCT病史，包括全身照射（TBI）预处理。他们还接受了强化化疗，因为多个转移（n = 3），复发（n = 3），重复HSCT（n = 3）。我们建议HSCT作为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伴TBI和强化化疗的患者必须仔细观察脂肪营养不良和代谢并发症的可能发展。
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.
影响因子：
15.9
作者：
Brent, Gregory A.
通讯作者：
Brent, Gregory A.
影响因子：
9.8
作者：
Speckman, RA;Garg, A;Bowcock, AM
通讯作者：
Bowcock, AM