Testicular adrenal rest tissue in a patient with classical congenital adrenal hyperplasia: color Doppler findings

Congenital adrenal hyperplasia (also known as congenital adrenogenital syndromes) refers to a group of autosomal recessive diseases characterized by altered cortisol production, which may be associated with aldosterone deficiency. The absence of cortisol synthesis stimulates corticotropin production by the adrenal cells and the accumulation of cortisol precursors, which will be diverted for the production of sex hormones. In affected males, ectopic adrenal tissue frequently develops, usually at the testicular level. This tissue is absolutely identical to that of the adrenal gland itself, and its functionality can be stimulated by ACTH and suppressed with glucocorticoid therapy. The authors report the case of a male patient with classic congenital adrenal hyperplasia, who was referred to our staff for evaluation of bilateral testicular tenderness and enlargement. Color Doppler sonography revealed mild enlargement of both gonads, widespread disruption of the testicular echostructure, and hypervascularization. Two months later, when the underlying disease had been controlled, repeat color Doppler ultrasonography revealed markedly decreased vascularity, although no change was noted on the B-mode examination. The color Doppler findings thus represent an early indicator of response to treatment.     

Adrenal gland photoscanning by means of131I-iodinated cholesterol

Summary Photoscanning by means of¹³¹I-19-iodinated cholesterol was performed on 10 patients with various diseases of the adrenal glands. The authors confirmed that the method was suitable for study of the morphology and function of the adrenal glands in disease, especially when combined with stimulation by ACTH or suppression by dexamethasone. The radiation dose absorbed by the thyroid and by the gonads can be controlled during the examination by blocking the iodine trapping and suppression of the gonado-stimulating action, respectively.     

8例新生儿先天性肾上腺皮质增生症的护理

总结了8例新生儿先天性肾上腺皮质增生症的护理.护理要点是入院时急诊处理,快速建立静脉通路,积极准确地补液,高效的标本采集和细致的病情观察,安全有效的激素应用,心理护理和出院宣教等.本组7例好转稳定后出院,1例放弃治疗.     

Clinicoradiologic diagnosis of a rare type of congenital adrenal hyperplasia: A case report from Nepal

Congenital adrenal hyperplasia includes defects in the synthesis of steroid hormones in the adrenal cortex. The implications of this disorder manifest in other genitourinary organs, including ovaries and uterus. The diagnosis may be suspected based on the clinical and radiologic features.     

Primary aldosteronism due to bilateral micronodular hyperplasia and concomitant subclinical Cushing’s syndrome: A case report

BACKGROUNDAdrenal incidentaloma (AI) has been frequently encountered in the clinical setting. It has been shown that primary aldosteronism (PA) or subclinical Cushing’s syndrome (SCS) are the representative causative diseases of AI. However, the coexistence of PA and SCS has been reportedly observed. Recently, we encountered a case of AI, in which PA and SCS coexisted, confirmed by histopathological examinations after a laparoscopic adrenalectomy. We believe that there were some clinical implications in the diagnosis of the present case.CASE SUMMARYA 58-year-old man presented with lower right abdominal pain with a blood pressure of 170/100 mmHg. A subsequent computed tomography scan revealed right ureterolithiasis, which was the cause of right abdominal pain, and right AI measuring 22 mm × 25 mm. After the disappearance of right abdominal pain, subsequent endocrinological examinations were performed. Aldosterone-related evaluations, including adrenal venous sampling, revealed the presence of bilateral PA. In addition, several cortisol-related evaluations showed the presence of SCS on the right adrenal adenoma. A laparoscopic right adrenalectomy was then performed. The histopathological examination of the resected right adrenal revealed the presence of a cortisol-producing adenoma, while CYP11B2 immunoreactivity was absent in this adenoma. However, in the adjacent non-neoplastic adrenal, multiple CYP11B2-positive adrenocortical micronodules were detected, showing the presence of aldosterone-producing adrenocortical micronodules. CONCLUSIONCareful clinical and pathological examination should be performed when a patient harboring AI presents with concomitant SCS and PA.     

Devastating salt‐wasting crisis in a four‐month‐old male child with congenital adrenal hyperplasia,highlighting the essence of neonatal screening

Congenital adrenal hyperplasia (CAH) is a rare condition usually referred to as a group of genetic disorders resulting due to a deficiency of steroid enzymes required by adrenal glands to produce cortisol and mineralocorticoid hormones. It has an autosomal recessive mode of inheritance and is further categorized into two types—Classic and Non‐Classic. Non‐Classic CAH is a more common milder form that presents late after puberty. Classic CAH, although more severe, is rare and detected at birth and is associated with the life‐threatening adrenal crisis in both sexes and virilization of the external genitalia in females (46, XX) patients, whereas in males, no overt abnormality of the external genitalia is present. We present a case of a four‐month‐old male child with the classic form of CAH who was brought with complaints of loose stools, projectile non bilious vomiting, decreased urine output, and failure to feed for 3 days. The child had a clinical presentation of salt wasting with hypoglycemia and hyperpigmentation of his genitalia. The USG findings revealed increased anteroposterior diameter of renal pelvis indicative of a growth in the suprarenal area. 17‐hydroxyprogesterone (17‐OHP) was found to be elevated confirming the diagnosis. He was treated with hydrocortisone with gradual improvement in his glucose and electrolytes. The patient was discharged home on replacement therapy consisting of oral prednisolone and fludrocortisone acetate and followed up as outpatient with significant improvement in the clinical findings. The fact that the child was not screened for CAH at birth led to the critical consequences of the disease in this case. To prevent life‐threatening adrenal crisis and help perform appropriate sex assignments for affected female patients, newborn screening (NBS) programs for the classical form of CAH should be made mandatory even in low‐ and middle‐income countries.     

Metastatic adrenal tumor from clear-cell renal cell carcinoma: a pitfall of chemical shift MR imaging

We present a case of adrenal metastasis from clear-cell renal cell carcinoma in which presence of a small amount of fat was shown on chemical shift gradient-echo magnetic resonance imaging. Radiologists should be aware that signal loss of the adrenal tumor on out-of-phase gradient-echo images does not always suggest the diagnosis of benign adenoma, particularly in patients with a history of renal cell carcinoma. Received: 16 May 2000/Accepted after revision: 13 September 2000     

Prescription of Medroxyprogesterone Acetate to a Patient with Pedophilia, Resulting in Cushing’s Syndrome and Adrenal Insufficiency

This article provides a case report of a patient with pedophilia who was treated over a 4-year period with medroxyprogesterone acetate (MPA) at a dose of 300 mg/day and as a consequence developed Cushing's Syndrome and adrenal insufficiency, for which he was treated and from which he recovered. He also reported a hypersexual reaction to his own past cessation of MPA. Gonadotropin-releasing hormone agonists, which have a more benign side-effect profile than MPA, are suggested as an alternative to MPA.     

Bilateral hemorrhagic adrenal cysts in an incomplete form of Beckwith-Wiedemann syndrome: MRI and prenatal US findings

Benign hemorrhagic adrenal cysts are a cause of subdiaphragmatic mass in the fetus and neonate with Beckwith-Wiedemann syndrome. Characteristic imaging features on ultrasonography, color Doppler, and magnetic resonance imaging help differentiate adrenal hemorrhage from neonatal neuroblastoma and help avoid unnecessary surgery in these patients. Bilateral adrenal hemorrhage is self-limiting, and spontaneous resolution is the usual outcome. This report presents this rare condition with prenatal ultrasonographic and magnetic resonance imaging findings and reviews the differential diagnosis of neonatal adrenal masses.     

Long-term survival of a patient with a large adrenal primitive neuroectodermal tumor: A case report

BACKGROUND Adrenal primitive neuroectodermal tumor(PNET) is an extremely rare malignant tumor with poor prognosis and of neural crest origin. Herein, we report a case of adrenal PNET and summarized its clinical and pathological characteristics on the basis of 16 patients reported recently.CASE SUMMARY A female patient aged 25 years presented with right lumbago for 12 mo, and preoperative computed tomography showed a huge right adrenal mass. She received tumorectomy, and post-operative pathological examination showed adrenal PNET. After surgery, she underwent adjuvant chemotherapy and was followed up 31 mo after surgery. She received brachytherapy for right paracolic and hepatic metastases. She was alive and followed up for 60 mo. In available studies, only 57.14%(4/7) and 44.44%(4/9) were positive for the expression of neuron-specific enolase and synaptophysin, respectively, although CD99 expression was found in all the patients(100%; 10/10).CONCLUSION It is concluded that adrenal PNET is very rare and highly malignant, and histology is a golden standard in its diagnosis. Surgery and adjuvant therapy is the main treatment.     

原发性高血压合并无功能肾上腺意外瘤患者代谢综合征患病情况分析

目的：探讨原发性高血压合并无功能肾上腺意外瘤（NFAI）患者代谢综合征（MS）发生情况及其与皮质醇分泌水平的相关性。方法：复旦大学附属中山医院内分泌科2014年10月至2015年4月收治的原发性高血压合并NFAI患者56例（NFAI组）,原发性高血压肾上腺形态正常者58例（EH组）。收集身高、体质量、腹围、血压等体格检查参数;测定电解质,血脂谱,空腹及餐后2 h血糖水平,以及隔夜1 mg地塞米松试验（ONDST）后皮质醇水平。根据有无代谢综合征（MS）,分别将NFAI组和EH组患者分为MS组（MS组）和非MS组（NMS组）,比较各组ONDST后皮质醇水平。结果：NFAI组患者舒张压、体质指数（BMI）、腹围、餐后2 h 血糖、三酰甘油、ONDST后皮质醇水平高于EH组患者（P<0.05）。56例NFAI组患者中,22例（39.2%）合并MS,高于EH组患者（14例,24.1%,P<0.05）。NFAI组患者中,MS亚组患者ONDST后皮质醇水平高于NMS亚组患者（P<0.05）。随着NFAI组患者MS组分数量异常增加,ONDST后皮质醇水平逐渐升高。结论：原发性高血压合并NFAI患者MS患病率较高,其MS发生与ONDST后皮质醇水平相关。     

Association of rare intestinal malformations: Colonic atresia and intestinal neuronal dysplasia

A single case of colonic atresia (CA) associated with type A intestinal neuronal dysplasia has been reported in the literature. This article describes a newborn with CA associated with diffuse type B intestinal neuronal dysplasia. A 2-day-old fullterm boy presented with marked abdominal distention, milk intolerance, and bilious vomiting. Type III CA was detected at laparotomy, and a mucous fistula colostomy was performed. The colostomy functioned poorly postoperatively, so an ileostomy was performed. Pathology reported that ganglion cells were evident in the specimens, however, the ileostomy did not function adequately. A second laparotomy was performed, and a re-stoma was fashioned. All previous pathology slides were reviewed, and diffuse type B intestinal neuronal dysplasia was detected. The patient died of septicemia at 34 d old. Specimens of patients with CA should be examined carefully so that dysganglionoses can be ruled out.     

Power Doppler imaging and evaluation of the resistive index in focal nodular hyperplasia of the liver

Focal lesions of the liver represent a significant diagnostic problem for various imaging modalities. The aim of this study was to assess the value of power Doppler sonography versus conventional color Doppler imaging in the depiction of hypervascular focal nodular hyperplasia (FNH) of the liver and to investigate the resistive index (RI) in the lesions' feeding arteries. Eighteen histologically proved FNHs in 14 patients were evaluated by gray-scale ultrasound, conventional color Doppler, and power Doppler sonography. With conventional color Doppler, a feeding arterial vessel could be depicted in only 4/18 lesions and hypervascularization was detected in 6/18 lesions. Power Doppler was more sensitive in detecting feeding arteries (16/18) within hypervascular lesions (15/18). RI values in the feeding arteries (mean = 0.51) significantly differed from those in the main hepatic artery or its intraparenchymal branches (mean = 0.68) in the same patient. The mean RI-difference was 0.19, suggesting hemodynamically significant arteriovenous shunting. Power Doppler sonography significantly increases sensitivity in the diagnosis of focal nodular hyperplasia of the liver and reliably permits the distinction of these lesions from hepatocellular carcinomas. Received: 17 January 1996/Accepted after revision: 8 May 1996     

国内首例达芬奇机器人单孔肾上腺肿瘤切除术的护理配合

目的：探讨国内首例行达芬奇机器人单孔肾上腺肿瘤切除术的护理配合方法。方法：总结我院1例达芬奇机器人单孔肾上腺肿瘤切除术患者的手术护理配合方法。结果：患者手术过程顺利,手术成功,于术后10天顺利出院。结论：达芬奇机器人单孔肾上腺肿瘤切除术具有创伤小、出血少、疼痛减轻、术后恢复快等优势,值得推广。     

胸内巨大淋巴结增生症的诊断与外科治疗预后研究(附16例分析)

目的回顾总结胸内巨大淋巴结增生症(CD)的临床特点、诊断和外科治疗效果。方法分析1982~2009年收治的16例胸内巨大淋巴结增生症患者,男女各8例,年龄19~52岁[(35.37±10.00)岁],均行手术治疗,另有2例分别行术前、术后放疗。随访10~318个月。结果术前确诊1例。局限性巨大淋巴结增生症13例(HV型11例,PC型1例,Mix型1例),多中心性巨大淋巴结增生症3例(PC型1例,Mix型2例),所有病例术后均生存,其中13例局限型和2例多中心型CD术后无复发。1例多中心Mix型患者于术后第4年、第9年各复发1次,均再次手术治疗。结论胸内巨大淋巴结增生症的诊断主要依靠病理学,胸内局限型CD和部分多中心型CD经彻底手术可获治愈;部分胸内多中心型CD手术治疗效果不佳,应辅助放射或其他综合治疗手段,但预后仍较差。     

Well-differentiated hepatocellular carcinoma: findings of US, CT, and MR imaging

Background: To elucidate the imaging characteristics of well-differentiated hepatocellular carcinomas (HCCs) on ultrasonography (US), computed tomography (CT), and magnetic resonance (MR) imaging. Methods: Ultrasonograms, CTs, and MR images of 18 histopathologically proven well-differentiated HCCs in 15 patients were reviewed. The findings of these images were correlated with histopathologic findings. Results: On US, seven tumors were depicted as a hyperechoic area and eight as a hypoechoic area. Three tumors were not visualized. On precontrast CT, four tumors were depicted as a low-density area, but 14 were not visualized. On conventional contrast-enhanced CT, 12 tumors were depicted as a low-density area but six were not visualized. On T1-weighted MR images, 10 tumors had high signal intensity and two had low signal intensity. Six tumors were not visualized. On T2-weighted MR images, five tumors had high signal intensity and two had low signal intensity. Eleven tumors were not visualized. Tumors with fatty change and/or clear cell formation were frequently hyperechoic on US and hyperintense on T1-weighted MR images. Conclusions: Well-differentiated HCCs show different findings on US, CT, and MR imaging. Therefore, reliable diagnosis of well-differentiated HCCs by these imaging techniques may be difficult. Received: 29 April 1998/Revision accepted: 15 July 1998     

Contrast-enhanced ultrasound (CEUS) appearances of an adrenal phaeochromocytoma in a child with Von Hippel-Lindau disease

A phaeochromocytoma is a rare catecholamine-secreting tumour arising from the chromaffin cells. We describe a case of a child with Von Hippel-Lindau disease, with an adrenal phaeochromocytoma who presented with severe dilated cardiomyopathy driven by secondary hypertension. Contrast-enhanced ultrasound findings are described and compared with both magnetic resonance imaging and computed tomography imaging.