Olsson Daniel S, Andersson Eva, Bryngelsson Ing-Liss, Nilsson Anna G, Johannsson Gudmundur
Department of Endocrinology (D.S.O., A.G.N., G.J.), Sahlgrenska University Hospital, SE-413 45 Gothenburg, Sweden; Department of Occupational and Environmental Medicine (E.A.), Sahlgrenska University Hospital, SE-405 30 Gothenburg, Sweden; and Department of Occupational and Environmental Medicine (I.-L.B.), Örebro University Hospital, SE-701 85 Örebro, Sweden.
J Clin Endocrinol Metab. 2015 Feb;100(2):467-74. doi: 10.1210/jc.2014-3525. Epub 2014 Nov 6.
Craniopharyngiomas (CPs) in adults have been associated with excess mortality.
The aim of the study was to investigate mortality and morbidity in patients with childhood-onset and adult-onset CP.
PATIENTS with CP were identified and followed in Swedish national health registries, 1987 through 2011. The inclusion criteria for the CP diagnosis were internally validated against patient records in 28% of the study population.
This was a nationwide population-based study.
A total of 307 patients (151 men and 156 women) were identified and included (mean follow-up, 9 years; range, 0-25 years). The inclusion criteria had a positive predictive value of 97% and a sensitivity of 92%.
There were no interventions.
Standardized mortality ratios (SMRs) and standardized incidence ratios (SIRs) with 95% confidence intervals were calculated using the Swedish population as the reference.
During the study, 54 patients died compared with the expected number of 14.1, resulting in an SMR of 3.2 (2.2-4.7) for men and 4.9 (3.2-7.2) for women. PATIENTS with childhood-onset (n = 106) and adult-onset (n = 201) CP had SMRs of 17 (6.3-37) and 3.5 (2.6-4.6), respectively. PATIENTS with hypopituitarism (n = 250), diabetes insipidus (n = 110), and neither of these (n = 54) had SMRs of 4.3 (3.1-5.8), 6.1 (3.5-9.7), and 2.7 (1.4-4.6), respectively. The SMR due to cerebrovascular diseases was 5.1 (1.7-12). SIRs were 5.6 (3.8-8.0) for type 2 diabetes mellitus, 7.1 (5.0-9.9) for cerebral infarction, 0.7 (0.2-1.7) for myocardial infarction, 2.1 (1.4-3.0) for fracture, and 5.9 (3.4-9.4) for severe infection. The SIR for all malignant tumors was 1.3 (0.8-2.1).
This first nationwide population-based study of patients with CP demonstrated excess mortality that was especially marked in patients with childhood-onset disease and among women. Death due to cerebrovascular diseases was increased 5-fold. Hypopituitarism and diabetes insipidus were negative prognostic factors for mortality and morbidity. PATIENTS with CP had increased disease burden related to type 2 diabetes mellitus, cerebral infarction, fracture, and severe infection.
成人颅咽管瘤（CPs）与过高的死亡率相关。
本研究旨在调查儿童期发病和成人期发病的CP患者的死亡率和发病率。
1987年至2011年期间，在瑞典国家卫生登记处对CP患者进行识别和随访。CP诊断的纳入标准在28%的研究人群中根据患者记录进行了内部验证。
这是一项基于全国人群的研究。
共识别并纳入307例患者（151例男性和156例女性）（平均随访9年；范围0 - 25年）。纳入标准的阳性预测值为97%，敏感性为92%。
无干预措施。
以瑞典人群为参照，计算标准化死亡率（SMRs）和标准化发病率（SIRs）及其95%置信区间。
研究期间，54例患者死亡，而预期死亡人数为14.1，男性的SMR为3.2（2.2 - 4.7），女性为4.9（3.2 - 7.2）。儿童期发病（n = 106）和成人期发病（n = 201）的CP患者的SMR分别为17（6.3 - 37）和3.5（2.6 - 4.6）。患有垂体功能减退症（n = 250）、尿崩症（n = 110）以及两者都没有的患者（n = 54）的SMR分别为4.3（3.1 - 5.8）、6.1（3.5 - 9.7）和2.7（1.4 - 4.6）。脑血管疾病导致的SMR为5.1（1.7 - 12）。2型糖尿病的SIR为5.6（3.8 - 8.0），脑梗死为7.1（5.0 - 9.9），心肌梗死为0.7（0.2 - 1.7），骨折为2.1（1.4 - 3.0），严重感染为5.9（3.4 - 9.4）。所有恶性肿瘤的SIR为1.3（0.8 - 2.1）。
这项首次基于全国人群的CP患者研究表明存在过高死亡率，在儿童期发病患者和女性中尤为明显。脑血管疾病导致的死亡增加了5倍。垂体功能减退症和尿崩症是死亡率和发病率的负面预后因素。CP患者与2型糖尿病、脑梗死、骨折和严重感染相关的疾病负担增加。
