Abstract：
OBJECTIVE: To retrospectively analyze the clinical characteristics of 193 Chinese patients with McCune-Albright syndrome (MAS).
METHODS: By using keywords \"McCune-Albright syndrome\", \"Albright syndrome\", or \" fibrous dysplasia \" as the search terms, 193 cases of MAS reported in China from January 1990 to November 2022 from the Wanfang data, CNKI, VIP, PubMed, and Embase databases were obtained, and their clinical data was retrospectively analyzed. Intergroup comparisons were carried out by using t test, Mann-Whitney U test, and X2 test.
RESULTS: The 193 MAS patients had included 42 males and 151 females, with the median first-visit age of females being younger than males. The typical triad group had accounted for 46.1% of patients, and the middle first-visit and diagnosis age was younger than the atypical group. The primary reason for first-visit in males of MAS was fibrous dysplasia (FD), whilst that in females of MAS was peripheral precocious puberty (PPP). FD has occurred in 84.5% of the patients, with an average age of onset age being 6.1 years old, and 90% was ≤ 16 years of age. Endocrine hyperfunction was found in 79.3% of the patients, with a higher proportion in females compared with males (P < 0.05). Pituitary involvement was seen in 21.8% of the patients, and the incidence of craniofacial FD and cranial nerve compression was significantly higher in those with elevated growth hormone (GH) than without (P < 0.05). Café-au-Lait Spots were noted in 86.5% of the patients, and 28.3% (28/99) had located on the different side of FD.
CONCLUSIONS: Most MAS patients had atypical manifestations and multi-systemic involvement. It is more common and occurs earlier in females. The most common reasons for initial diagnosis in male and female patients were FD and PPP, respectively. Patients with elevated GH should be examined for cranial nerve compression.
METHODS: By using keywords \"McCune-Albright syndrome\", \"Albright syndrome\", or \" fibrous dysplasia \" as the search terms, 193 cases of MAS reported in China from January 1990 to November 2022 from the Wanfang data, CNKI, VIP, PubMed, and Embase databases were obtained, and their clinical data was retrospectively analyzed. Intergroup comparisons were carried out by using t test, Mann-Whitney U test, and X2 test.
RESULTS: The 193 MAS patients had included 42 males and 151 females, with the median first-visit age of females being younger than males. The typical triad group had accounted for 46.1% of patients, and the middle first-visit and diagnosis age was younger than the atypical group. The primary reason for first-visit in males of MAS was fibrous dysplasia (FD), whilst that in females of MAS was peripheral precocious puberty (PPP). FD has occurred in 84.5% of the patients, with an average age of onset age being 6.1 years old, and 90% was ≤ 16 years of age. Endocrine hyperfunction was found in 79.3% of the patients, with a higher proportion in females compared with males (P < 0.05). Pituitary involvement was seen in 21.8% of the patients, and the incidence of craniofacial FD and cranial nerve compression was significantly higher in those with elevated growth hormone (GH) than without (P < 0.05). Café-au-Lait Spots were noted in 86.5% of the patients, and 28.3% (28/99) had located on the different side of FD.
CONCLUSIONS: Most MAS patients had atypical manifestations and multi-systemic involvement. It is more common and occurs earlier in females. The most common reasons for initial diagnosis in male and female patients were FD and PPP, respectively. Patients with elevated GH should be examined for cranial nerve compression.
摘要：
目的：回顾性分析193例McCune-Albright综合征(MAS)患者的临床特点。
方法：使用关键字\"McCune-Albright综合征\",“奥尔布赖特综合征”，或“纤维发育不良”作为搜索词，从万方数据来看，1990年1月至2022年11月在中国报告了193例MAS，CNKI,VIP,PubMed,并获得了Embase数据库，并对其临床资料进行回顾性分析。组间比较采用t检验,Mann-WhitneyU测试,和X2测试。
结果：193名MAS患者包括42名男性和151名女性，女性的首次就诊年龄中位数比男性年轻。典型三合会组患者占46.1%,中间初诊和诊断年龄比非典型组年轻。MAS男性首次就诊的主要原因是纤维发育不良（FD），而MAS的女性为外周性早熟（PPP）。84.5%的患者发生FD,平均发病年龄为6.1岁，90％≤16岁。79.3%的患者发现内分泌功能亢进,女性比例高于男性(P<0.05)。21.8%的患者有垂体受累，生长激素（GH）升高者的颅面FD和颅神经压迫发生率明显高于无生长激素（P<0.05）。在86.5%的患者中发现了Café-au-Lait斑点，28.3%(28/99)位于FD的不同侧。
结论：大多数MAS患者表现不典型，多系统受累。它更常见，并且在女性中更早发生。男性和女性患者最初诊断的最常见原因是FD和PPP，分别。应检查GH升高的患者是否有颅神经压迫。
方法：使用关键字\"McCune-Albright综合征\",“奥尔布赖特综合征”，或“纤维发育不良”作为搜索词，从万方数据来看，1990年1月至2022年11月在中国报告了193例MAS，CNKI,VIP,PubMed,并获得了Embase数据库，并对其临床资料进行回顾性分析。组间比较采用t检验,Mann-WhitneyU测试,和X2测试。
结果：193名MAS患者包括42名男性和151名女性，女性的首次就诊年龄中位数比男性年轻。典型三合会组患者占46.1%,中间初诊和诊断年龄比非典型组年轻。MAS男性首次就诊的主要原因是纤维发育不良（FD），而MAS的女性为外周性早熟（PPP）。84.5%的患者发生FD,平均发病年龄为6.1岁，90％≤16岁。79.3%的患者发现内分泌功能亢进,女性比例高于男性(P<0.05)。21.8%的患者有垂体受累，生长激素（GH）升高者的颅面FD和颅神经压迫发生率明显高于无生长激素（P<0.05）。在86.5%的患者中发现了Café-au-Lait斑点，28.3%(28/99)位于FD的不同侧。
结论：大多数MAS患者表现不典型，多系统受累。它更常见，并且在女性中更早发生。男性和女性患者最初诊断的最常见原因是FD和PPP，分别。应检查GH升高的患者是否有颅神经压迫。
