Harsch Igor Alexander
Department of Internal Medicine II, Division of Endocrinology and Metabolism, Rainweg 68, Saalfeld 07318, Germany.
Case Rep Endocrinol. 2021 Jan 20;2021:8885348. doi: 10.1155/2021/8885348. eCollection 2021.
. The Waterhouse-Friderichsen Syndrome (WFS) is a course of bacterial meningitis with a lethality rate that is still high today. One hallmark of the clinical course is intravascular coagulopathy. This causes hemorrhagic infarctions in the adrenal glands, rapidly causing a primary adrenal insufficiency. Only few reports highlight the course of the remaining adrenal insufficiency or adrenal restitution in survivors. . After 3 weeks in an intensive care unit, a 45-year-old male survived WFS with necroses on the legs and forefeet and with primary adrenal insufficiency confirmed by the ACTH stimulation test. The substitution therapy with hydrocortisone and fludrocortisone could be gradually discontinued after nine months due to a further positive clinical course. Although the patient reported good mental and physical performance further on, the cortisol response in ACTH testing showed tiny incremental rises of the stimulated serum cortisol, but to reach a formally normal level, it took about five years. . The report demonstrates a case with a relatively fast clinical remission. A remission of the corticotrophic response occurred in small increments during an observational period of five years. The data suggest that not only a clinical remission is possible but also a complete biochemical remission, although this process may take a much longer timespan.
华-弗综合征（WFS）是一种细菌性脑膜炎病程，其致死率至今仍然很高。临床病程的一个标志是血管内凝血病。这会导致肾上腺出血性梗死，迅速引发原发性肾上腺功能不全。仅有少数报告着重指出了幸存者中剩余肾上腺功能不全或肾上腺恢复的病程。在重症监护病房治疗3周后，一名45岁男性从华-弗综合征中存活下来，腿部和前足出现坏死，促肾上腺皮质激素（ACTH）刺激试验证实存在原发性肾上腺功能不全。由于临床病程进一步好转，9个月后氢化可的松和氟氢可的松替代疗法可逐渐停用。尽管患者随后报告精神和身体状况良好，但ACTH检测中的皮质醇反应显示，刺激后的血清皮质醇仅有微小的增量上升，不过要达到正式的正常水平大约需要5年时间。该报告展示了一个临床缓解相对较快的病例。在5年的观察期内，促肾上腺皮质激素反应以小幅度逐渐缓解。数据表明，不仅可能实现临床缓解，而且也可能实现完全的生化缓解，尽管这个过程可能需要更长的时间跨度。
