儿茶酚胺增多症的诊断及治疗进展

由于肾上腺嗜铬细胞瘤、肾上腺外的嗜铬细胞瘤及肾上腺髓质增生(AMH)都分泌过量的儿茶酚胺并由此而产生相似的临床症状，故统称为儿茶酚胺增多症。由于本病确认的历史较短，临床上又比较少见。有必要对本病中的一些重要的概念性问题再加阐述。     

儿茶酚胺增多症(附95例报告)

目的 提高儿茶酚胺增多症的诊断和治疗水平。方法 报告95例儿茶酚胺增多症的临床资料，其中嗜铬细胞瘤74例，肾上腺髓质增生2l冽。嗜铬细胞瘤组有高血压67例(90．5％)，24h尿VMA增高者62例(83．8％)，尿儿茶酚胺增高者67例(90．5％)；影像学检查均有阳性发现。肾上腺髓质增生患者均有高血压，24h尿VMA均增高，大部分(15／16)患者尿儿茶酚胺高于正常；21例中14例B超有阳性发现。结果 74例嗜铬细胞瘤患者均行手术治疗，其中异位嗜铬细胞瘤9例；病理及随访证实5例为恶性嗜铬细胞瘤。21例肾上腺髓质增生者中，18例行手术治疗，其中12例单侧肾上腺切除，6例双侧肾上腺切除，术后病理诊断肾上腺髓质增生。结论 嗜铬细胞瘤的诊断重点在定性诊断，而儿茶酚胺症的诊断难点在定位诊断。肾上腺髓质增生作为独立疾病概念的建立，丰富和完善了儿茶酚胺增多症的理论。     

肾上腺髓质增生的研究进展

肾上腺髓质增生是一种罕见的病变，临床表现和内分泌检查结果与嗜铬细胞瘤结果相同^[1]。本病作为一种独立的疾病，由吴阶平于20世纪70年代首次提出以来，人们对肾上腺髓质增生的认识日趋深入，特别是近10年来在基础和临床研究方面进行了大量工作。     

肾上腺髓质疾病42例分析

本文报道４２例肾上腺髓质外科疾病，其中嗜铬细胞瘤３３例（７８．０％），肾上腺髓质增生７例（１６．７％），髓质骨髓脂肪瘤２例（４．７％）。结合文献对其发病情况、临床表现、实验室检查、影像学检查、病理学诊断等分别进行了讨论。     

肾上腺意外瘤126例分析

目的 探讨肾上腺意外瘤的诊断和治疗方法。方法对126例肾上腺意外瘤进行分析,其中98例行手术治疗。结果各类意外瘤中,以腺瘤为最多,共52例(41．3％);43例(34．1％)有功能,以嗜铬细胞瘤及原发性醛固酮增多症为主;无功能腺瘤的最大直径均在6cm以下,皮质癌均在6cm以上。结论术前应明确意外瘤的功能状态及良恶性。对于无功能意外瘤,肿物大小是判断其良恶性及决定手术与否的主要指标。     

肾上腺皮髓质混合性病变(附四例报告)

目的 探讨肾上腺皮髓质混合性病变（醛固酮－儿茶酚胺症）的临床特点和诊治方法。酮血症,２例分别伴有高代谢和高血糖症,血浆肾素、血管紧张素活性正常或略增高,尿儿茶酚胺及其代谢产物水平正常,经Ｂ超、ＣＴ或ＭＲＩ诊断为肾上腺肿瘤。行患侧肾上腺切除术,术后血钾、血糖恢复正常,高血压消失或明显缓解。病理诊断为肾上腺皮质增生或腺瘤伴髓质增生或嗜铬细胞瘤。结论 肾上腺皮髓质混合性病变均有原醛症表现,部分病例同时有     

肾上腺髓质增生

肾上腺是人体中一对十分重要的内分泌腺体 ,主要分泌肾上腺糖皮质激素、盐皮质激素、性激素和儿茶酚胺。肾上腺的病变可以造成这些激素分泌的增多或减少 ,从而导致人体组织器官损害和严重的代谢紊乱。早在 15 6 3年 ,意大利外科医师Eu stachius发现人的肾脏上方有一类似三角形帽子样的特殊结构 ,并绘制成图谱 ,但对其功能一无所知。180 0～ 180 6年 ,Nichol和Cuvior在组织学上明确了这一结构由皮质和髓质二种不同的组织构成 ,并称其为肾上腺。二十世纪 6 0年代前 ,医学家们一直认为 :引起肾上腺髓质机能亢进的只有嗜铬细胞瘤 ;无肿瘤的肾…     

双侧肾上腺肿瘤的诊治

目的 探讨双侧肾上腺肿瘤诊断和治疗。方法 分别对17例嗜铬细胞和2例原发性醛固醇增多症的临床特征、定性和定位检查及手术径路进行分析。结果 17例双侧肾上腺嗜铬细胞瘤在临床特征、生化、内分泌检查和术前准备方面与单侧或肾上腺外嗜铬细胞瘤类似,术前应注意合并肾上腺外嗜铬细胞瘤可能。经腹切口有利于一期切除双侧肾上腺肿瘤。结论 对双侧肾上腺肿瘤应注意定性分析,I^131-MIBG和NP59有益于肾上腺髓质和皮质肿瘤的鉴别诊断,双侧肋缘下弧形切口有利于切除双侧肾上腺肿瘤。对小的、无功能肾上腺肿瘤应予以随访。     

肾上腺

20050764腹膜后腹腔镜手术治疗原发性醛固酮增多症130例／张旭…∥中华外科杂志．-2004．42(18)．-1093～1095     

Adrenal cortical carcinoma

Adrenal cortical carcinoma is a rare endocrine tumor for which complete surgical resection is the only potentially curative treatment. Accurate preoperative evaluation (biochemical and radiographic) of the patient who presents with an adrenal mass maximizes the opportunity for the patient to undergo a complete, margin-negative resection of the primary tumor, which is the most powerful prognostic variable for long-term survival. The response to chemotherapy or mitotane is modest in patients with advanced disease. Hopefully, an improved understanding of the molecular pathogenesis of this challenging tumor will lead to the development of more effective therapies in the future.     

肾上腺皮质癌诊治方法的探讨

目的 探讨肾上腺皮质癌的诊断和治疗方法。方法 回顾１９７８－１９９８年收治的１５例肾上腺皮质癌,其中有内分泌功能８例,无内分泌功能７例,均经手术和病理证实。结果 有内分泌功能性上肾上腺皮质癌具有典型肾上腺皮质功能增强的临床表现,如库兴综合征、原发性醛固酮增多症或性征异常;无内分泌功能性肿瘤为腺外表现;如急性腹痛、高血压等,尿１７－羟、１７－酮和血、尿皮质醇、醛固酮的测定及ＣＴ和Ｂ超检查均有助于诊断     

Adrenal cortical cell transplantation

Purpose

The adrenal cortex is a critical component of the hypothalamic-pituitary-adrenal/gonadal axis that coordinates the stress response and maintains homeostasis. The authors hypothesize that adrenal cortical cells can be transplanted in adrenal insufficiency states to regenerate the adrenal cortex.

Methods

Murine adrenal glands were dissociated into adrenal cortical cells. Cells cultured in a collagen matrix were transplanted under the renal capsule. The implants were retrieved 1, 2, 4, and 8 weeks later. Total RNA was extracted from the retrieved specimens and was analyzed by real-time polymerase chain reaction.

Results

All animals survived the surgical procedure. At implant retrieval, a distinct organoid could be visualized under the renal capsule. The expressions of adrenal-specific markers including Sf1, Dax1, Star, Cyp11a, Cyp11b1, and Cyp21 were detectable in the retrieved specimens up to 8 weeks posttransplantation.

Conclusion

Primary adrenal cortical cells retained their gene expressions after heterotopic transplantation. Ex vivo gene transfer followed by adrenal cortical cell transplantation may lead to curative therapy for patients with adrenal insufficiency.     

Adrenal cortical tumors in children

A review of the literature reveals 230 cases of adrenal cortical tumors in children. We add an additional 7 cases. Signs, symptoms, laboratory studies, and treatment of this rare rate tumor are discussed.     

Adrenal medullary phaeochromocytoma and adrenal medullary hyperplasia]

From 1979 to 1989, 8 cases of adrenal medullary phaeochromocytoma (AMP) and 6 cases of adrenal medullary hyperplasia (AMH) were treated surgically. We reviewed the two groups of patients, and the results suggested that though the patients of AMP are similar to those of AMH in clinical symptoms and signs, AMP and AMH are virtually two different diseases of the adrenal medulla. CT and echography are helpful in distinguishing AMP from AMH, but the determination of the two diseases depends on the histological findings. The diagnostic criterion of pathology for AMP and AMH, as well as the treatment of the two diseases are discussed.