肾上腺结核合并Addison’s病42例临床分析

目的 探讨肾上腺结核合并Addison’s病的临床特点。方法 回顾性分析 4 2例肾上腺结核合并Addison’s病的临床资料。结果 4 2例肾上腺结核合并Addison’s病病例中 ,30例 (71 4 % )伴有肾上腺外结核 ;39例化验红细胞沉降率 ,2 6例增快 ;2 7例行PPD试验 ,2 0例阳性 ;2 5例行CT扫描 ,2 0例 (80 % )有肾上腺钙化 ,18例 (72 % )肾上腺增大。结论 肾上腺结核是我国Addison’s病的常见原因 ,且常伴有肾上腺外结核 ,CT扫描显示肾上腺钙化、增大对结核性Addison’s病的诊断具有特征性。

以Addison’s病首诊的肾上腺淋巴瘤临床特点分析——附5例报告并文献复习

目的：肾上腺淋巴瘤在临床上少见，以Addison’s病首诊者更少见，本文拟探讨以Addison’s病首诊的肾上腺淋巴瘤临床特征、诊治要点及预后，以提高临床医师对其认识。方法回顾性地分析解放军总医院1995年至2014年间收治的以Addison’s病首诊并经病理确诊的5例肾上腺淋巴瘤患者病例资料，统计其症状、体征、病程、肿瘤大小、病理类型及实验室检查结果，并结合国内外文献进行分析。结果5例均为老年患者，年龄（66.4±6.6）岁，男女比例为4∶1。5例患者均有典型Addison’s病的表现，如皮肤变黑、消瘦、乏力、食欲减退。均诊断为原发性肾上腺非霍奇金淋巴瘤，病理类型为B细胞来源，4例明确为弥漫大B细胞型。术后给予R-CHOP方案化疗，其中2例目前健在，发病至今已分别存活了10个月和20个月，随访效果尚可，1例存活3年，余2例存活时间均不足1年。结论以Addison’s病首诊的肾上腺淋巴瘤多发生于老年男性患者，双侧肾上腺受累多见，确诊需依赖穿刺病理诊断，病理类型主要为弥漫大B细胞型，预后较差。

肾上腺结核继发Addison’s病36例临床分析

目的探讨肾上腺结核继发Addison’s病的临床特点。方法回顾性分析36例肾上腺结核继发Addison’s病的临床资料,归纳总结并比较各临床表现发生情况,以发生率表示。结果36例肾上腺结核继发Addison’s病病例中32例(88.9%)伴有肾上腺外结核;33例行CT检查,随着病程的延长肾上腺由增大逐渐萎缩且钙化范围逐渐增大;综合临床,影像学表现和痰菌等判断,23例(63.9%)为活动性结核。结论肾上腺结核是我国Addison’s病的常见原因,且常伴有肾上腺外结核;肾上腺结核的CT特征与病程长短有关;如有活动性结核者,在用激素替代治疗的同时,应积极给予抗结核治疗。

Addison's病的CT诊断

Addison′s病即原发性肾上腺皮质功能减退症,由双侧肾上腺皮质破坏所致,其原因主要为自体免疫所致的特发性肾上腺皮质萎缩及肾上腺结核。本文通过11例Addison′s病的报告,总结了本病的CT表现,强调肾上腺由双侧增大向小的钙化的腺体演变的过程,提出CT可在Addison′s病早期显示出病变,故对早期诊断、早期治疗以使肾上腺功能恢复具有重要意义。

Occult Adrenal Insufficiency in Patients with Chronic Renal Disease

Background: Addison’s disease is a rare disorder of the adrenal cortex that leads to inadequate production of cortisol initially followed by aldosterone and androgens. Its manifestations are usually slow and non-specific with potential for life-threatening adrenal crisis following hypermetabolic demands (infection, trauma, surgery). Patients: Over the past 10 years, 19 CRD-patients were diagnosed with occult PAI in our center. Results: Unprovoked hypotension was the most common manifestations of occult PAI and was the unmasking event in 11 (58%). It was without significant cardiac and/or severe systemic sepsis and was refractory to isotonic saline infusions. Equal number of the remaining patients (n = 2) presented with persistent and inexplicable electrolytes abnormalities viz. 1) hyponatremia despite restricted oral fluid intake, lack of dehydration and massive fluid overload, as well as 2) hyperkalemia despite potassium-restricted diet, hyperkalemic drugs and adequate therapy with Furosemide and low-potassium dialysis-baths. On the other hand, similar proportions presented with unprovoked 3) progressive weight loss, decrease appetite and cachexia as well as 4) frequent hypoglycemic attacks. All patients were treated and were medically stable after 29 (2 - 60) months of follow up. Autoantibodies to 21-hydroxylase enzyme were positive in 16 (90%). At diagnosis, and subsequent follow up, only 7 patients (37%) had multi-endocrine dysfunction of whom 2 with type 1 and 5 with type 2. Conclusion: High index of suspicion should be exerted in diagnosis of PAI in patients with CRD, since its clinical picture is similar to CRD manifestations and complications. In those patients, confirmatory tests and specific management can save their lives. .

Profile of Autoimmune Polyendocrinopathies at the Medical Clinic II of the Abass Ndao Hospital: About 40 Cases

Introduction: Autoimmune polyendocrinopathies (AP) represent a group of rare concomitant pathologies, making them underdiagnosed. The objective was to study their profile at the Medical Clinic II of the Abass Ndao Hospital. Patients and Methods: This was an observational, descriptive and analytical study, lasting 24 months, from January 1, 2020 to December 31, 2022. We assessed the epidemiological, clinical and paraclinical characteristics of the patients and classified the APs found. Results: We included 40 patients divided into type III (38 cases) and IV (2 cases). A female predominance was noted with a sex ratio of 0.21. The mean age was 38.6 years. A family history of component diseases of autoimmune polyendocrine syndrome (APS) was found in 62.5%. Goiter (80%) was the main clinical sign present. All 38 patients with ISAP-3 had autoimmune thyroiditis, including 29 cases of Graves’ disease (72.5%) and 9 cases of Hashimoto’s thyroiditis (22.5%). They were associated with either type 1 diabetes (57.9%), Biermer’s disease (21.1%), vitiligo + alopecia (18.4%), lupus (2.6%). The 2 patients with AP-4 had Biermer’s disease associated with either Addison’s disease or type 1 diabetes. Management depended on the pathologies present and their possible complications. The immunological phenomena were also controlled. Conclusion: This series is globally similar to the literature. The polymorphous character of the clinical pictures requires a better collaboration between specialists leading to a clinical and holistic synthesis.

Adrenal Insufficiency by Adrenoleukodystrophy

The X-linked adrenoleukodystrophy (ALD) is a severe neurodegenerative disorder due to mutations in the ABCD1 gene. Objective: To report a case of a 19-year-old man with adrenal insufficiency due to adrenoleukodystrophy. Method: Case report and literature review. Result: A previously healthy 19-year-old male patient was admitted to the emergency room with nausea and vomiting for 5 days, who progressed to abdominal pain, severe asthenia, and fever (38.5°C). He referred progressive darkening of the skin, oral mucosa, tongue and nail bed of the hands and feet, observed in the last 6 years. Emergency laboratory evaluation showed severe hyponatremia and hyperkalemia, which, together with decreased plasma cortisol, directed the investigation to causes of adrenal insufficiency. High ACTH (Adrenocorticotropic hormone) and very long chain fatty acid levels closed the diagnosis. Discussion: ALD is characterized by progressive demyelination in the central and peripheral nervous system and adrenal insufficiency consequence to the accumulation of very long chain fatty acids (VLCFA) in the adrenal. The overall incidence of ALD is 1:17,000. Adrenal insufficiency may be the first symptom of ALD in boys and adults. The diagnosis is based on the measurement of VLCFA plasma levels. Allogeneic bone marrow transplantation is the only treatment that provides a permanent cure when the procedure is performed at an early stage of brain demyelination, i.e. when patients are asymptomatic, although brain magnetic resonance imaging (MRI) is abnormal. Treatment of Addison’s disease is obligatory, but does not change the course of neurological symptoms.

后腹腔镜肾上腺结核切除术经验（附5例病例报告）

目的：探讨后腹腔镜。肾上腺结核切除术的安全性、可行性及临床应用价值。方法：2001年2月-2008年12月，经后腹腔镜手术治疗肾上腺结核5例，男2例，女3例，年龄32—64岁，平均约47岁，双侧2例，右2例，左侧1例。结节大小3．2—5．5cm，平均3．5cm．术前均予以抗结核治疗，皮质激素替代治疗，其他术前准备同常规手术。结果：本组5例手术均获成功，无中转开放手术病例。手术时间45-240分钟，平均118分钟，术中出血量约30—150ml，平均为75ml，均未输血，无一例中转开放手术，手术完整切除病变侧肾上腺，术后均经病理证实为肾上腺结核：肾上腺干酪样坏死，少量残余肾上腺组织。5—7天出院。未发现术后并发症及再次住院治疗。结论：后腹腔镜肾上腺结核切除术对于直径〈6cm的肾上腺结核的是安全有效的手术方式。