肾上腺皮质、髓质增生症(附9例报告)

目的探讨肾上腺皮、髓质增生症的诊断和治疗。方法测定９例高儿茶酚胺血症患者血浆去甲肾上腺素（ＮＥ）、肾上腺素（Ｅ）、多巴胺（ＤＡ）及２４小时尿３甲氧４羟苦杏仁酸（ＶＭＡ）、１７羟皮质类固醇（１７ＯＨＣＳ）、１７酮类固醇（１７ＫＳ）含量；作肾上腺ＣＴ扫描和同位素间位碘苄胍（１３１ＩＭＩＢＧ）显像；术中作冰冻切片检查。结果ＮＥ、Ｅ、ＤＡ、ＶＭＡ均升高，Ｅ尤为明显，１７ＯＨＣＳ、１７ＫＳ正常或升高。ＣＴ扫描肾上腺弥漫性增大、增厚，准确率８３％；１３１ＩＭＩＢＧ肾上腺显像示踪剂在２４～４８小时呈１～３级浓集，准确率９４％。病理报告：９例１７侧肾上腺皮质束状带及头、体、尾部髓质增生。结论肾上腺皮、髓质增生８９％为双侧病变，临床表现儿茶酚胺增多症，有否皮质醇症取决于皮质增生的类型，弥漫性增生者常出现症状，而结节状增生者症状不明显。认为本病的诊断首选ＣＴ和１３１ＩＭＩＢＧ。主张术中采用连续冰冻切片检查以决定肾上腺的切除范围，对增生明显的一侧腺体作全切除，另一侧作次全切除。

Adrenal cortical and medullary hyperplasia (report of 9 cases)

Objective To study the diagnosis and treatment of adrenal cortical and medullary hyperplasia.Methods In 9 cases of hypercatecholaminemia, determination were made of the contents of plasma noradrenaline (NE),epinephrine(E) and dopamine (DA) and vanillymandelic acid (VMA), 17 hydroxycorticosteroid (17 OHCS), and 17 ketosteroid (17 KS) from 24 hour urine. Adrenal glands were examined with CT scan and 131 I MIBG imaging. Frozen section examination wasperformedduringthe operation. Results NE, E, DA, VMA were all increased, especially E and 17 OHCS and 17 KS were either normal or increased. CT scanning revealed diffuse enlargement with increased thickness of the adrenal glands and the accuracy rate was 83%. In adrenal imaging with 131 I MIBG, the concentration of tracer indicated grades 1 3 in 24 48 hours with accuracy of 94%. Pathological study of 17 glands in 9 cases revealed hyperplasia of both zona fasciculata of cortex and head, body and tail of medulla. Conclusion 89% hyperplasia of adrenal contical and medullary are bilateral. Clinically the disease is manifested in hypercatecholaminemia. Whether or not there is a hypercortisonism depends on the type of cortical hyperplasia. In diffuse hyperplasia, symptoms usually occur while in nodular hyperplasia, there is no obvious symptom. CT and 131 I MIBG are diagnostie measures of first choice. The authors hold that serial frozen sections during surgery should be made to determine the extent of adrenalectomy. A total resection may be performed on the gland with obvious hyperplasia and subtotal resection on the other side.