嗜铬细胞瘤的131I-MIBG显像诊断与病理对照分析

目的 评价＾１３１Ｉ－间位碘卞胍（＾１３１Ｉ－ＭＩＢＧ）显像对嗜铬细胞瘤的诊断价值。方法 观察２１例经病理证实为嗜铬细胞瘤患者的Ｂ超、ＣＴ、尿香草基杏仁酸（ＶＭＡ）和＾１３１Ｉ－ＭＩＢＧ显像结果,并与病理结果进行回顾性对照分析。结果 Ｂ超、ＣＴ、尿ＶＭＡ对嗜铬细胞瘤的检出率分别为８１％、９０．５％和６８．４％,＾１３１Ｉ－ＭＩＢＧ显像对嗜铬细胞瘤的检出率达１００％,＾１３１Ｉ－ＭＩＢＧ显像的敏感性     

急性心肌梗塞心电图QRS积分与血浆儿茶酚胺浓度关系的探讨

作者对急性心肌梗塞（ＡＭＩ）患者用ＱＲＳ积分法来确定心肌梗塞范围，同时测定血浆儿茶酚胺浓度。结果证实ＱＲＳ积分与血浆儿茶酚胺浓度呈正相关。说明交感肾上腺髓质的活动在ＡＭＩ的发生与发展中起重要作用。     

帕金森病猴模型纹状体多巴胺D2受体的显像研究

目的 应用碘标左旋－２－羟基－６－甲氧基－Ｎ－「（１－乙基－２－吡咯烷）甲基」苯酰胺（＾１３１Ｉ－ＩＢＺＭ）及４－氨基－Ｎ－（１－乙基－２－甲氢吡咯基）甲基－５－碘－２－甲氧基苯甲酰胺（＾１２３Ｉ－ＡＩＢＺＭ）单光子发射体层摄影术（ＳＰＥＣＴ）观察一侧帕金森病（ＰＤ）猴模型纹状体多巴胺Ｄ２受体多巴胺Ｄ２受体（Ｄ２Ｒ）功能变化。探讨＾１２３Ｉ－ＡＩＢＺＭ ＳＰＥＣＴＤ２Ｒ功能显像的临床意义及其可行性。方法 恒河猴Ｍ１、Ｍ２、Ｍ３,其中Ｍ１、Ｍ２为经右侧颈总动脉注射１－甲基－４－苯基－１,２,３,６－四氢吡啶（ＭＰＴＰ）建立的一侧ＰＤ模型猴,Ｍ３为正常猴,静脉注射＾１３１Ｉ－ＩＢＺＭ或＾１２３Ｉ－ＡＩＢＺＭ后进行ＳＰＥＣＴ显像,并进行＾１３１Ｉ－ＩＢＺＭ放射自显影观察。高效液相色谱－电化学法检测离体尾、壳核及黑质多     

棘皮分析地形图在癫痫定位诊断中的应用价值

目的 探讨棘波分析地形图在癫痫定位诊断中的应用价值。方法选用癫痫患者８６例，其中６５例为原发性癫痫，２１例为继发性癫痫进行棘波分析地图形（Ｓ－ＢＥＡＭ）、ＥＥＧ、ＢＥＡＭ检查，结果 通过Ｓ－ＢＥＡＭ与ＥＥＧ、ＢＥＡＭ、脑ＣＴ对比研究表明：Ｓ－ＢＥＡＭ定位与脑ＣＴ－一致，但病灶范围比脑ＣＴ大，比ＢＥＡＭ小。此外地形图尚能动态显示病灶的起源、扩展、传播过程。结论棘波分析地图莆（Ｓ－ＢＥＡＭ）不仅同屏不     

Saliva secreting functions in patients with TCM-Piyinxu

ＳａｌｉｖａｓｅｃｒｅｔｉｎｇｆｕｎｃｔｉｏｎｓｉｎｐａｔｉｅｎｔｓｗｉｔｈＴＣＭＰｉｙｉｎｘｕＧＵＡＮＸｕｅＺｈｏｎｇ，ＷＥＩＭｕＸｉｎ，ＣＨＥＮＤｅＺｈｅｎ，ＧＵＹｕＣｈｕｎ，ＳＵＮＺｈｅｎＨｅａｎｄＢＥＩＳｈｕＹｉｎｇＤｅｐａｒｔ...     

EGG AS A PHYSIOLOGICAL MARKER OF MOTIONSICKNESSINASIANANDNON＿ASIANSUBJECTS．

ＥＧＧＡＳＡＰＨＹＳＩＯＬＯＧＩＣＡＬＭＡＲＫＥＲＯＦＭＯＴＩＯＮＳＩＣＫＮＥＳＳＩＮＡＳＩＡＮＡＮＤＮＯＮ＿ＡＳＩＡＮＳＵＢＪＥＣＴＳ．ＲｏｂｅｒｔＭ．Ｓｔｅｒｎ，ＤｅｐａｒｔｍｅｎｔｏｆＰｓｙｃｈｏｌｏｇｙ，ＴｈｅＰｅｎｎｓｙｌｖａｎｉａＳｔａｔ...     

NORMAL AND DISTURBED MOTILITY OF GALLBLADDERANDSPHINCTEROFODDI．

ＮＯＲＭＡＬＡＮＤＤＩＳＴＵＲＢＥＤＭＯＴＩＬＩＴＹＯＦＧＡＬＬＢＬＡＤＤＥＲＡＮＤＳＰＨＩＮＣＴＥＲＯＦＯＤＤＩ．Ａ．Ｊ．Ｐ．Ｍ．Ｓｍｏｕｔ，Ｇ．Ｐ．ｖａｎＢｅｒｇｅ＿Ｈｅｎｅｇｏｕｗｅｎ，ａｎｄＭ．Ｓａｍｓｏｍ．ＤｅｐａｒｔｍｅｎｔｏｆＧａｓｔｒ...     

硝酸甘油对哮喘患者一氧化氮内皮素的影响及机制

目的　了解哮喘患者肺泡巨噬细胞（ＡＭ） 、支气管上皮细胞（ＢＥＣ） 源性一氧化氮（ＮＯ）、内皮素（ＥＴ）的分泌状态及硝酸甘油（ＮＴＧ）对哮喘患者ＡＭ、ＢＥＣ产生ＮＯ、ＥＴ的影响及机制。方法　分离纯化了１５ 例轻、中度哮喘发作期患者、７ 名健康受试者ＡＭ、ＢＥＣ,并分为哮喘未干预组、哮喘ＮＴＧ干预组和健康对照组,用放射免疫法和镀铜镉还原法分别测定ＡＭ、ＢＥＣ培养４８ 小时上清液中ＥＴ、ＮＯ·２／ＮＯ·３ 浓度,用原位杂交的方法检测ＡＭ、ＢＥＣｉＮＯＳｍＲＮＡ、ＥＴｍＲＮＡ 的表达。结果　（１） 健康受试者ＡＭ、ＢＥＣ分泌少量ＮＯ和ＥＴ及少量ｉＮＯＳｍＲＮＡ 、ＥＴｍＲＮＡ表达;（２）哮喘患者ＡＭ、ＢＥＣ源性ＮＯ、ＥＴ水平及ＡＭ、ＢＥＣｉＮＯＳｍＲＮＡ、ＥＴｍＲＮＡ表达与各组比较差异有显著性（ Ｐ均＜ ０－０５）;（３）ＮＴＧ 促进哮喘患者ＡＭ、ＢＥＣ源性ＮＯ产生（ Ｐ均＜０－０５）,明显抑制ＥＴ产生和ＥＴｍＲＮＡ 的表达,与对照组比较差异均无显著性（ Ｐ均＞ ０－０５） ,ＮＴＧ同时抑制哮喘患者ＡＭ、ＢＥＣｉＮＯＳｍＲＮＡ的表达,与健康对照组、哮喘未干预组比较差异有显著性（Ｐ均＜０－０５） ;（４） 除哮喘ＮＴＧ     

乙型肝炎肝窦病变的免疫组织化学及电镜观察

目的　研究乙型肝炎（ ＨＢ） 肝窦病变的免疫组化表达及超微结构变化的病理意义．方法　ＨＢ 肝活检标本３００ 例， 用ＦＮ，ＬＮ，ＣｏⅣ，αＳＭＡ 及ＶＥＧＦ 单克隆抗体作免疫组化（ＳＰ 法） 染色；另对６５ 例按照电镜技术制样，作超微结构观察．结果　ＨＢ 肝窦有狭窄、阻塞、扩张及肝窦毛细血管化４ 种病变． ＡＨＢ 以肝窦狭窄及阻塞较重者占多数（９５ ％ ） ，而ＣＨＢ 则以肝窦扩张及毛细血管化最明显，两者差异显著（ Ｐ＜ ０－０１） ．肝窦狭窄时肝细胞水肿、微绒毛断裂、线粒体畸型、窦内皮细胞增生或损伤，窗孔减少，基膜物质增多，以αＳＭＡ，ＶＥＧＦ 强表达较前者显著（ Ｐ ＜ ０－０１） ． ＦＮ ，ＬＮ，ＣｏⅣ及αＳＭＡ 表达随肝窦扩张程度升降，与ＶＥＧＦ 表达明显不同（ Ｐ＜ ０－０１） ． 肝窦内皮细胞可出芽形成管腔，基膜物质增多而ＶＥＧＦ 则下降．结论　肝窦病变免疫组化及超微结构变化与ＨＢ 演变有关．     

白塞病合并骨髓异常增生综合征的临床特点(附6例报告)

目的：为了解白塞病（ＢＤ）与骨髓增生异常的相关性。方法：回顾分析了６例ＢＤ合并骨髓异常增生综合征（ＭＤＳ）患者的临床资料、血象和骨髓象。２例合并难治性贫血（ＭＤＳＲＡ），２例合并难治性贫血伴原始细胞增多（ＭＤＳＲＡＥＢ），１例合并转化型难治性贫血伴原始细胞过多（ＭＤＳＲＡＥＢｔ），１例合并慢性粒—单细胞白血病（ＭＤＳＣＭＭＬ）。结果：６例均示有贫血，５例白细胞低下，４例血小板减少。６例骨髓均增生活跃。４例ＢＤ发病在前，２例ＢＤ与ＭＤＳ同时出现。结论：认为ＢＤ与ＭＤＳ有相关性     

Pheochromocytoma due to unilateral adrenal medullary hyperplasia

We describe two male patients, aged 17 and 47 years, with clinical and biochemical features of pheochromocytoma. Both patients had normal-sized adrenal glands on abdominal CT scan and abnormal unilateral uptake of I-123 metaiodobenzylguanidine (MIBG) on scintigraphy. The surgical adrenalectomy revealed normal macroscopic glands in both patients. Histological examination showed adrenal medullary hyperplasia with adrenal cortico-medullary ratios of 2 : 1 and 4 : 1. Unilateral adrenalectomy resulted in amelioration of symptoms and normalization of catecholamines excretion. DNA examination for RET proto-oncogene revealed no mutations in exons 10, 11, 13, 14 and 16. Our results suggest that diffuse adrenal medullary hyperplasia may be the initial pathological change in the adrenal gland leading, subsequently, to the development of nodular hyperplasia and adrenal medullary tumor. These results indicate that the syndrome of pheochromocytoma may occur as an unilateral adrenal medullary hyperplasia in patients without evidence for multiple endocrine neoplasia.     

Association of adrenal medullar and cortical nodular hyperplasia: a report of two cases with clinical and morpho-functional considerations

Arterial hypertension of adrenal etiology is mainly attributed to primary hyperaldosteronism. However, subtle expressions of hyperadrenergic or glucocorticoid excess can also generate arterial hypertension. The present report describes two hypertensive patients cataloged as resistant essential hypertensives, in whom adrenal masses were found incidentally, who highlight the need to recognize these tenuous clinical or laboratory presentations. Case 1 was a 50-yr-old female with hyperadrenergic hypertension associated to a left adrenal node, normal cortisol and aldosterone:renin ratio, marginally increased urinary normetanephrine, and a positive 131I MIBG radioisotope scan. Adrenalectomy normalized blood pressure and urinary metanephrines. Pathology showed a hyperplastic adrenal medulla associated to a multinodular cortical hyperplasia. Case 2 was a 62- yr-old female with progressive hypertension, a slight Cushing phenotype, non-suppressible hypercortisolism, normal urinary metanephrines, and bilateral adrenal nodes. Bilateral adrenalectomy and subsequent replacement normalized blood pressure and phenotypic stigmata. Pathology demonstrated bilateral cortical multinodular hyperplasia and medullary hyperplasia. The clinical study in both patients was negative for MEN. The apparently rare association of cortical and medullary lesions presented by both patients is probably overlooked in routine pathology exams, but should be meticulously searched since the crosstalk between the adrenal cortex and medulla may prompt dual abnormalities.     

A case of extraadrenal pheochromocytoma associated with adrenal cortical nodular hyperplasia and papillary thyroid carcinoma.

A 64-year-old woman was admitted in November, 1996 for fluctuating blood pressure. There was multinodular goiter in her neck. High urine VMA and serum aldosterone were noted. Computed tomography showed an oval lesion in the left adrenal gland. Left adrenalectomy was performed and the pathology was proved to be adrenal cortical nodular hyperplasia. Fluctuating blood pressure and high urine VMA persisted after the operation. CT scan of the abdomen revealed a soft tissue mass in lower abdomen. The patient was admitted again in September, 1997. Laboratory examinations showed normal serum aldosterone, normal plasma renin activity and high urine VMA. Aspiration cytology of the thyroid gland disclosed papillary thyroid carcinoma. [131I]-metaiodobenzylguanidine image revealed a high uptake lesion in the right L-3 paravertebral area. Tumor excision and thyroidectomy were performed. The pathology was reported as extraadrenal pheochromocytoma and papillary thyroid carcinoma. Papillary thyroid carcinoma is rarely associated with pheochromocytoma. To our knowledge, this paper is the first report of a patient with extraadrenal pheochromocytoma associated with papillary thyroid carcinoma and adrenal cortical nodular hyperplasia.     

Clonidine-induced suppression of plasma catecholamines in states of adrenal medulla hyperfunction

To assess adrenal medulla activity in states of hyperfunction, a single 0.3 mg oral dose of clonidine hydrochloride (Catapres) was given to twelve patients with varying evidence of familial adrenal medullary hyperplasia and pheochromocytomas from kindreds with Multiple Endocrine Neoplasia type 2 syndrome (MEN-2), seven patients with sporadic pheochromocytomas and six normal subjects. Mean arterial blood pressure and plasma norepinephrine (NE) levels were lower than baseline values 2 h after clonidine in the normal subjects. Plasma epinephrine (E) rose in one normal but fell in the remainder after clonidine administration. In sporadic pheochromocytoma patients, E fell slightly in 4 and NE fell in 3 while mean arterial blood pressure was not significantly lower than baseline values in 7 patients 2 h after clonidine. In MEN-2, mean arterial blood pressure fell and there was a variable response of plasma E and NE to clonidine, which appears to be related to the presence of detectable anatomic (CT scan) and functional (131I-mlBG scintigraphy) abnormalities of the adrenal medulla. These findings are thus compatible with the spectrum of adrenal medulla dysfunction and the presumed development of pheochromocytoma in this syndrome.     

The role of 6-[18F]fluorodopamine positron emission tomography in the localization of adrenal pheochromocytoma associated with von Hippel-Lindau syndrome

OBJECTIVE: [(123/131)I]metaiodobenzylguanidine (MIBG) scintigraphy is considered as the gold standard in the localization of pheochromocytoma. However, this method has less optimal sensitivity for the detection of pheochromocytoma associated with von Hippel-Lindau (VHL). Our preliminary results suggest that this is partially due to the low expression of cell membrane norepinephrine transporter system in VHL-related pheochromocytoma cells. Another probable cause may be the low affinity that [(123/131)I]MIBG has for these cells. Recently, 6-[(18)F]fluorodopamine ([(18)F]DA) positron emission tomography (PET) has been introduced as a novel functional imaging modality with high sensitivity for pheochromocytoma. Therefore, we investigated whether [(18)F]DA PET is more effective than [(123/131)I]MIBG scintigraphy in the diagnostic localization of VHL-related adrenal pheochromocytoma. MATERIALS AND METHODS: In this study, we evaluated seven VHL patients in whom adrenal pheochromocytomas were confirmed by histopathology results. Adrenal pheochromocytomas were localized using computed tomography (CT), magnetic resonance imaging (MRI), [(123/131)I]MIBG scintigraphy and [(18)F]DA PET. RESULTS: [(18)F]DA PET localized pheochromocytoma in all the seven patients, as did in CT. In contrast, three out of the seven had negative results utilizing [(123/131)I]MIBG scintigraphy and one out of the six patients had negative MRI results. CONCLUSIONS: [(18)F]DA PET was found to show more promising results when compared with [(123/131)I]MIBG scintigraphy in the diagnostic localization of VHL-related adrenal pheochromocytoma, with a 100% rate of localization. Thus, [(18)F]DA PET in conjunction with CT/MRI should be considered as an effective method for the proper localization of VHL-related adrenal pheochromocytoma.     

High permanent plasma adrenaline levels: a marker of adrenal medullary disease in medullary thyroid carcinoma

Increases in urinary, plasma and tumour adrenaline have been previously observed in MEN II patients with phaeochromocytoma. However, the sensitivity of adrenaline for early detection of adrenal medullary disease has not been accurately evaluated. Twenty-five patients with medullary thyroid carcinoma (MTC) histologically confirmed but without clinical or biological evidence of phaeochromocytoma have been studied. Medullary adrenal status was evaluated by adrenal CT-scan. MIBG scintigraphy, determination of urinary VMA, metanephrines and total catecholamine levels, measurement of nyctohemeral plasma adrenaline or noradrenaline concentrations (every 2 h during 24 h) and clonidine suppression test. Four of the 25 patients had evidence of adrenal medullary disease in view of the coexistence of CT-scan, MIBG scintigraphy and plasma adrenaline abnormalities. Moderate adrenal enlargement (unilateral, n = 3; bilateral, n = 1) was observed on scans together with a high adrenal MIBG uptake (bilateral, n = 4). Among the urinary parameters studied, a minor MN increase was observed in only one of the four patients. Plasma adrenaline levels were significantly (P less than 0.01) different from those of the other 21 patients (mean + SD 115 + 110 pmol/l). This plasma adrenaline increase is reproducible and not suppressed by clonidine. Unilateral adrenalectomy performed in one patient confirmed a phaeochromocytoma and induced normalization of plasma adrenaline levels. In contrast, the plasma noradrenaline levels of the four patients were not statistically different from those of the other 21 patients. These data suggest that persistent high plasma adrenaline levels may be selectively increased in MTC patients together with a moderate adrenal CT-scan enlargement and a high adrenal MIBG uptake, despite a normal urinary excretion of total catecholamines and catecholamines metabolites.(ABSTRACT TRUNCATED AT 250 WORDS)     

Multiple endocrine neoplasia type 2A.

A 40-year-old woman was admitted with complaints of headache, palpitation and diaphoresis. She had undergone right hemithyroidectomy 12 years previously. Histological reexamination of the operative specimen revealed a medullary thyroid carcinoma. Abdominal ultrasonography, CT scan and angiography showed bilateral adrenal tumors. Serum catecholamine levels in both adrenal veins were high. Based on these data, bilateral adrenalectomy was performed. Histological examination confirmed the diagnosis of pheochromocytomas. After operation, serum calcitonin and urinary noradrenaline levels were still high. Further examination by 131I-metaiodobenzylguanidine (MIBG) scintigraphy is planned.     

Plasma and tumor dopamine-beta-hydroxylase activity in patients with familial pheochromocytomas

The majority of patients with sporadic pheochromocytomas (pheos) have been noted to have normal plasma dopamine-beta-hydroxylase (DBH) activity. We determined the activity of DBH in the plasma of 8 patients with pheos, secondary to multiple endocrine neoplasia Type 2 (MEN II) (medullary carcinoma of the thyroid [MCT], pheochromocytoma(s), and parathyroid hyperplasia). We also determined the activity of DBH in the pheos of six of the patients. Three of the eight patients (38%) had elevated plasma DBH in the preoperative period. After surgical resection of their pheos, the plasma DBH of two of the subjects, in whom it could be measured, returned to normal. Although two of the other patients had bilateral pheos containing large amounts of DBH and norepinephrine (NE), their plasma DBH was normal in the preoperative and postoperative periods. Despite elevated urinary vanillylmandelic acid (VMA) excretion in all eight patients, their homovanillic acid (HVA) excretion was normal. VMA reflects NE plus epinephrine (E) excretion, while HVA reflects dopamine (DA) excretion. We conclude that the majority of patients with pheos, associated with MEN II, have normal plasma DBH activity.     

A case of sleep apnea syndrome manifesting severe hypertension with high plasma norepinephrine levels

A 55-year-old female was admitted to our hospital with severe hypertension (274/140 mmHg). Endocrinological examination revealed that her plasma levels of norepinephrine (NE) was elevated with high levels of urinary NE, normetanephrine and vanillylmandelic acid (VMA), suggesting the presence of pheochromocytoma. However, neither computed tomography nor MIBG scintigraphy detected any catecholamine-producing tumor in or outside the adrenal glands. She was screened with full polysomnography because of heavy snoring, and the diagnosis of severe obstructive sleep apnea syndrome (OSAS) was made. She was treated with calcium channel blocker for three weeks, but severe hypertension persisted. After treatment with nasal continuous positive airway pressure (CPAP) was added, her blood pressure gradually lowered week by week. Concomitantly, the levels of plasma and urinary NE, urinary normetanephrine and urinary VMA were normalized following nasal CPAP therapy for 2 weeks. Additional treatments with alpha-adrenergic blocker further decreased her home blood pressure. After a year, she continued nasal CPAP therapy and her blood pressure was nearly below 160/100 mmHg. Urinary NE level was slightly above normal range and other catecholamines stayed within the normal range. This case shows that patients with OSAS could develop severe hypertension through elevated sympathetic tone, mimicking pheochromocytoma. Nasal CPAP therapy is recommended not only to improve hypertension and catecholamine excess but also to distinguish the condition from pheochromocytoma.