儿童颅咽管瘤术后出现肝肺综合征1例报告

杜婷婷; 姚辉; 李亚坤; 黄小力; 罗杰

doi:10.3969/j.issn.1001-5256.2022.07.030

儿童颅咽管瘤术后出现肝肺综合征1例报告

DOI: 10.3969/j.issn.1001-5256.2022.07.030

华中科技大学同济医学院附属武汉儿童医院遗传代谢内分泌科，武汉 430015

伦理学声明：本例报告已获得患者家属知情同意。

利益冲突说明：所有作者均声明不存在利益冲突。

作者贡献声明：杜婷婷负责撰写论文；杜婷婷、姚辉对研究的思路及设计有关键贡献；李亚坤、黄小力、罗杰参与研究数据的获取分析和解释过程。

详细信息

通信作者:
姚辉，yaohui1024@sina.com

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出版历程
- 收稿日期: 2021-08-06
- 录用日期: 2021-11-19
- 出版日期: 2022-07-20

Hepatopulmonary syndrome after craniopharyngioma operation in children: A case report

Department of Endocrinology and Metabolism, Wuhan Children's Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430015, China

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Corresponding author: YAO Hui, yaohui1024@sina.com(ORCID: 0000-0002-1878-0012)

摘要

- 颅咽管瘤 /
- 肝肺综合征 /
- 非酒精性脂肪性肝病 /
- 儿童
- Craniopharyngioma /
- Hepatopulmonary Syndrome /
- Non-alcoholic Fatty Liver Disease /
- Child

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图 1 术前、术后垂体MRI检查

注: a，术前，鞍上区占位，侧脑室扩大，第三脑室受压扩张；b，术前，增强扫描病变边缘强化；c，术后，鞍区术后改变，垂体柄未见显示。

Figure 1. MRI appearance of the patient's pituitary gland before and after surgery

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图 2 增强经胸超声心动图造影

注：a，注射震荡的生理盐水前；b，注射震荡的生理盐水右心房和右心室出现微泡显像；c，4个心动周期左右左心内出现较浓密的微泡显像。

Figure 2. Contrast-enhanced transthoracic echocardiography of the patient

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图 3 血管造影检查

注：a，肺动脉造影显示，肺小动脉走行不规则、扩张；b，门静脉造影显示门静脉扩张，染色较淡。

Figure 3. Angiography of the patient

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表 1 本例患儿治疗前后代谢指标及垂体功能变化

Table 1. Changes of metabolic indexes and pituitary hormone before and after treatment

项目	治疗前	出院1个月	出院3个月	出院6个月	正常范围
一般情况	不能离氧	间断吸氧	安静状态无需吸氧，行走数百米需吸氧	无氧情况下上3楼，行走数百米
身高(cm)(位于生长曲线百分位数)	135(＜3^rd NGC)	135.6(＜3^rd NGC)	137(＜3^rd NGC)	141(＜3^rd NGC)
体质量(kg)	35.5	35	38	38
ALT(U/L)	82	78	37	52	7~45
AST(U/L)	187	177	61	75	10~50
TC(mmol/L)	2.06	2.77	2.4	2.51	2.8~4.8
TG(mmol/L)	0.99	1.0	1.27	1.36	0.32~1.46
LDL(mmol/L)	1.74	1.71	1.52	1.62	1.55~2.86
TBil(μmol/L)	69.1	59.9	33.4	39.1	2~19
Alb(g/L)	38.9	43.7	44.3	44.1	39~53
血糖(mmol/L)	4.18	4.04	4.39	4.07	3.89~6.11
胰岛素(μIU/mL)	39.3	34.9	29.6	24.8	3~25
IGF-1(ng/mL)	＜25	222	279	361	183~850
TSH(μIU/mL)	2.59	0.743	0.331	0.658	0.51~6.27
FT3(pmol/L)	3.73	3.76	4.80	5.07	5.2~8.6
FT4(pmol/L)	7.34	10.59	12.83	14.32	12~22
ACTH(pg/mL)	12.7	＜10	11.8	＜10	0~46
皮质醇(nmol/L)	＜13.8	509.3	188.47	95.6	145.4~619.4
LH(mIU/mL)	0.03	-	-	＜0.3	0.5~9.0
FSH(mIU/mL)	0.67	-	-	＜0.07	0.4~8.6
E2(pmol/L)	＜43.31	-	-	＜43.31	36.7~1101.0
泌乳素(μIU/mL)	1122.28	-	-	616.73	63.60~305.28
透明质酸(ng/mL)	299	101	145	175	＜100
层粘连蛋白(ng/mL)	60.7	31.5	26.7	27.9	＜50
Ⅲ型前胶原N端(ng/mL)	422	105	325	353	＜30
Ⅳ型胶原(ng/mL)	129	118	108	99	＜30
注：LDL，低密度脂蛋白胆固醇血症；TSH，促甲状腺激素；FT3，游离三碘甲腺原氨酸；FT4，游离四碘甲状腺原氨酸；ACTH，促肾上腺皮质激素；LH，促黄体生成素；FSH，促卵泡刺激素；E2，雌二醇。

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表 2 已报道的6例垂体功能减退伴HPS患者的临床特征

Table 2. Clinical features of six reported cases of hypopituitarism with HPS

项目	病例1^[8]	病例2^[9]	病例3^[10]	病例4^[11]	病例5^[12]	病例6^[13]
诊断年龄	23岁	18岁	16岁	17岁	15岁	35岁
性别	男	女	男	女	男	男
垂体疾患	2岁诊断垂体柄阻断综合征	11岁行颅咽管瘤手术治疗	诊断HPS时发现鞍上区占位，活检未见肿瘤组织	14岁诊断朗格汉斯组织细胞增生症，予放疗及鞘内化疗	11岁时诊断成熟畸胎瘤，手术治疗	31岁时诊断垂体瘤，手术治疗
既往治疗情况	予rhGH及雄激素治疗至18岁停药	予优甲乐、氢化可的松、去氨加压素、雌激素治疗，15岁二甲双胍口服，后失访2年	既往诊断尿崩症，间断予鼻内去氨加压素	去氨加压素、雌二醇贴及生理剂量的糖皮质激素	术后鼻内去氨加压素、糖皮质激素、甲状腺素、rhGH治疗；术后1年半停药	术后未予垂体激素替代治疗；27岁时发现患乙型肝炎，未正规治疗
身高(cm)	172	168.3	160	160.1	-	-
体质量(kg)	60	54.3	68	91.1	95	-
BMI(kg/m²)	20.2	19.2	26.6	35.5	29.3	-
第二性征	无阴毛、第二性征发育不良	Tanner Ⅲ期	无第二性征发育	Tanner Ⅱ~Ⅲ期无初潮	-	-
动脉血气分析	PaCO₂ 49.8 mmHgPaO₂ 39.5 mmHg	-	SO₂ 70%	静息时SO₂ 88%活动时SO₂ 76%	PaO₂ 51 mmHg	PaO₂＜50 mmHg
血脂	TG 72 mg/dL	TG 123 mg/dLTC 113mg/dL	-	描述有代谢综合征	-	-
血糖	82 mg/dL	126 mg/dL	-	描述有代谢综合征	72 mg/dL	-
胰岛素	-	-	-	描述有代谢综合征	22.8 μIU/mLHOMA-IR 4.05	-
肝功能	ALT 42 U/LAST 78 U/LTBil 1.1 mg/dLAlb 3.5 g/dL	ALT 103 U/LAST 104 U/LAlb 4.3 g/dL	ALT 39 U/L描述胆红素和Alb正常范围	ALT 46 U/LAST 67 U/LTBil 0.7 mg/dL	ALT 16 U/LAST 22 U/LTBil 2.9 mg/dLAlb 3.9 g/dL	ALT 191 U/LAST 287 U/LTBil 63.1 μmol/LAlb 19.7 g/L
生长激素	0.03 ng/mL	激发后峰值0.1 ng/mL	全垂体功能低下	描述有垂体功能低下	激发后峰值0.1 ng/mL	-
IGF-1	＜4 ng/mL	67.2 ng/mL	全垂体功能低下	描述有垂体功能低下	71 ng/mL	-
甲状腺功能	促甲状腺激素释放激素刺激试验显示反应延迟	TSH 0.22 μU/mL(0.5~6.5)FT4 0.91 ng/dL(0.8~1.9)	全垂体功能低下	描述有垂体功能低下	TSH 0.152 μIU/mLFT3 1.12 pg/mLFT4 0.69 ng/mL	TSH 2.4 mU/mLFT4 5.06 pmol/L
肾上腺功能	ACTH＜2.0 pg/mL皮质醇24.5 μg/dL	激发后皮质醇峰值0.38 μg/dL(＞20)	全垂体功能低下	描述有垂体功能低下	ACTH 6.0 pg/mL皮质醇14.3 μg/dL	皮质醇4.0 mmol/L
性激素	LH＜0.2 mIU/mLFSH 0.3 mIU/mLTesto＜5.0 ng/mLPRL 7.0 ng/mL	激发后LH峰值3.6 mIU/mL(＞10)；FSH峰值0.18 mIU/mL(＞2)E2 12.6 pg/mL	全垂体功能低下	描述有垂体功能低下	LH＜0.1 mIU/mLFSH 0.06 mIU/mLTesto＜0.03 ng/mL	LH 0.3 mIU/mLFSH 8 mIU/mLTesto 0.06 ng/mL
肝活检	NASH 3~4期	小结节性肝硬化	小结节性肝硬化	伴有再生结节的NASH和明显的纤维化	弥漫性脂肪变性和纤维化	-
治疗	再次予rhGH、Testo、左甲状腺素钠、氢化可的松替代，转氨酶下降；5个月后肝活检示NASH改善；肝纤维化指标下降；无氧情况下可正常活动及工作	予左旋甲状腺素、泼尼松龙、去氨加压素、rhGH、雌激素、醋酸甲羟孕酮、二甲双胍，转氨酶正常，但APTT持续延长，等待肝移植	诊断HPS后予甲状腺激素、rhGH、糖皮质激素、雄激素、去氨加压素治疗，7个月后，低氧血症恶化，肝移植	诊断HPS 6个月后进展为门静脉性肺动脉高压，后予华法林抗凝，西地那非和吸入伊洛前列素等肺血管扩张剂治疗	肝移植；术后1年再次出现脂肪肝，转氨酶升高，术后5年开始rhGH治疗；术后6年，二甲双胍及格列美脲治疗，随访10年，转氨酶正常，脂肪肝无进展	抗病毒、护肝，予糖皮质激素、甲状腺素治疗；呼吸困难有所改善，低氧血症仍存在；等待肝移植
注：Testo，睾酮；HOMA-IR，胰岛素抵抗指数；-，未描述。

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