肾上腺皮质功能不全与代谢危象及猝死

肾上腺皮质功能不全是一类由原发或继发因素引起的肾上腺皮质激素分泌不足的疾病。由于其临床表现缺乏特异性,不易早期识别,可导致诊断延迟,若治疗不及时会引发肾上腺危象甚至发生猝死。由肾上腺皮质功能不全引起的患儿猝死可能与肾上腺危象导致的高钾血症直接相关。胃肠疾病及感染等是肾上腺危象的常见诱因。尽管其是一种可治疗的疾病,但若未进行适当的预防或延误治疗往往导致不必要的死亡。因此,需要制定更多措施来预防肾上腺危象,并确保在危象发生后采取恰当的急救方案,以减少猝死。     

儿童肾上腺危象的识别及急诊处理

肾上腺危象是指由各种原因导致肾上腺皮质激素分泌不足或缺如而引起的一系列临床症状，病情凶险，进展急剧，如不及时救治可致休克、昏迷、死亡，是小儿内科严重的急症之一。Marik等经荟萃分析指出，危重病患儿并发急性肾上腺皮质功能不全总的发生率约30％，而在败血症休克患儿则达到50％～60％。     

儿童肾上腺危象时的激素应用

肾上腺皮质主要分泌皮质醇和醛固酮，在维持血压、心肌张力和糖、盐平衡中起重要作用。肾上腺髓质则分泌应激激素，如肾上腺素和去甲肾上腺素，同样在心血管和神经系统的调节中起重要作用。我们通常讲的肾上腺危象指的是肾上腺皮质激素分泌绝对或相对不足而引起的有潜在生命威胁的急症。本文讨论儿童肾上腺危象的病因、高危因素、临床表现等，并重点强调了发生肾上腺危象时的激素应用及后续治疗，以提高对该病的认识和抢救成功率。     

儿童重症疾病时肾上腺的功能

目的　成人危重症及儿童感染性休克时肾上腺功能异常已得到阐述 ,未及时诊断与治疗者预后差。但有关其他儿童重症疾病时肾上腺的功能状态尚无报道。作者前瞻性地研究了危重患儿肾上腺功能异常的发生率。方法　以 13例小儿危重症评分≥ 10分和 (或 )血流动力学不稳定(应用血管活性药和 /或需要 2 0ml/kg液体扩容以保证血压稳定 )的患儿为研究对象。入选第 2天 7∶0 0～ 9∶0 0取血测皮质醇、促肾上腺皮质激素 (为基础值 ) ;静脉注射α 1,2 4 促肾上腺皮质激素 ,1h后再次取血检测上述指标。结果　皮质醇的基础值为 0 1～ 37 4 μg/dl [平均 (12 2± 9 6 ) μg/dl]。肾上腺功能不全的诊断标准为清晨皮质醇 <7μg/dl或注射ACTH后皮质醇 <18μg/dl。 13例中有 4例 (31% )肾上腺功能不全 ,其中皮质醇基础值 <7μg/dl的 3例的ACTH值均处于正常下限。发生与未发生肾上腺功能不全的患儿小儿危重评分 (14 7± 4 11、12 7± 2 6 5 ,P =0 37)与入院诊断无差异。结论　一些危重患儿在严重生理应激反应时不能产生足够的皮质醇 ,但对外源性ACTH反应良好。作者对危重症中下丘脑 垂体 肾上腺轴的作用进行了阐述 ,并认为可根据上述生化指标的异常来判断预后     

危重病肾上腺功能障碍的研究进展

相对肾上腺皮质功能不全(RAI)在危重病患儿中具有较高的发生率.下丘脑-垂体-肾上腺轴的机械性损伤、炎症细胞因子或有毒代谢产物和激素抵抗是发生RAI的原因.脓毒症患儿出现儿茶酚胺抵抗的难治性休克或充分容量复苏前提下,血管活性药物反应不良时应高度警惕RAI.促肾上腺皮质激素(ACTH)刺激试验是判断RAI的主要方法,在标准剂量ACTH(250 μg)刺激试验的基础上,研究发现小剂量ACTH(1 μg)刺激试验可能具有高敏感性和安全有效等特点.     

危重病肾上腺功能障碍的研究进展

There is high incidence of relative adrenal insufficiency (RAI) in critical children. The causes of adrenal insufficiency in patients with severe sepsis and septic shock are the mechanical injury of the hypothalamic-pituitary-adrenal axis, cytokines and other mediators of inflammatory or hormone resistance. There are many symptoms associated with adrenal insufficiency. Diagnosis is often suspected when these patients have hypotension refractory to fluid therapy and to vasoactive drugs. The corticotropin stimulation test is widely used as a method to identify adrenocortical hyporesponsiveness, but controversy exists as to the corticotropin dose to be used. The 250 μg dose is the standard dose. Low doses of corticotropin (1 μg) have recenfly been proposed,suggesting that they may have higher sensitivity and the characteristics of safe and effective.     

先天性心脏病患儿体外循环术后肾上腺皮质功能不全的影响因素

目的 研究小儿体外循环（CPB）术后血皮质醇的变化规律，探讨可能导致术后肾上腺皮质功能不全（A1）的危险因素。方法 收集2006年3～10月本院行心内直视手术治疗的先天性心脏病患儿80例。记录术前及术中可能的相关危险因素，放射免疫分析法测定围术期血浆皮质醇水平，化学发光法测定围术期血浆ACTH质量浓度。结果 术后即刻及术后6h血浆皮质醇浓度较术前明显升高（P〈0．05）。术后24、48、72h血浆皮质醇水平明显回落，较术前略降低，但无统计学差异。本组Al发生率为41％。A1患儿年龄、经皮氧饱和度[Sp（O2）]、CPB时间和主动脉阻断时间与其他患儿比较有显著差异（P〈0.05）。年龄、CPB时间为独立因素。结论 先天性心脏病小儿CPB术后血浆皮质醇水平术后早期升高，术后24h即明显回落，部分患儿术后出现Al。年龄、CPB时间是导致Al的高危因素。     

呼吸衰竭早产儿肾上腺皮质功能的研究

目的 研究呼吸衰竭早产儿肾上腺皮质功能的变化,肾上腺皮质功能不全的发生率及其与病情和预后的关系.方法 研究对象为住院早产儿43例(研究组33例,对照组10例),分别检测清晨血清基础皮质醇浓度及小剂量促肾上腺皮质激素(ACTH)刺激试验后血清皮质醇峰值.结果 需机械通气的呼吸衰竭早产儿血清基础皮质醇浓度[(114.6 ±76.1)μg/L]和小剂量ACTH刺激试验后血清皮质醇峰值[(232.5±74.6)μg/L]比非机械通气早产儿[(182.1 ±86.8)、(312.1 ±93.8)μg/L]低;呼吸衰竭早产儿合并肾上腺皮质功能不全的发生率较高(21.21%);肾上腺皮质功能不全早产儿中无死亡及慢性肺疾病(CLD)病例.结论 呼吸衰竭早产儿具有较高肾上腺皮质功能不全的发生率;需机械通气的呼吸衰竭早产儿肾上腺皮质的合成分泌功能较差;未发现肾上腺皮质功能不全与病死率和CLD的发生相关     

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??Adrenal crisis??AC?? is the term of a syndrome which has been used to describe a group of critical symptoms in rather unclear words for decades. It has brought a poor concordance on the data of mortality??morbidity and treatment among researches??and it has also hindered researchers to reach consensus till 2015??when the adrenal crisis criteria was proposed by a British physician. While it is still not perfect??it does give physicians a reference in clinical practice. The author considers it a meaningful step forward. Adrenal insufficiency could be turned into adrenal crisis with any stress. Being aware of the threat of AC and educating people of its symptoms and treatment are essential for both the patients and their care givers. CAH is the most common disease for AC in pediatrics and the therapy should be further researched because the morbidity of AC has increased since the regime of short-acting tablets and small doses. Perfect evidence is still absent at present.     

抗高血压药物在小儿高血压危象紧急处理时的临床应用

小儿高血压危象属儿科相对罕见的急症,具有较高的致残率和致死率,需及时、安全、有效的治疗以缓解临床症状,减轻脏器损害,提高生存质量并降低病死率.虽然许多抗高血压药物在小儿高血压危象的治疗中被应用了数十年,但该类药物的临床研究资料多来源于成人,小儿高血压危象中应用的随机对照研究较少,且多为小样本研究,科学性及说服力不足,故临床上小儿高血压危象的药物选择仍然是一大难题.本文旨在结合文献对抗高血压药物在小儿高血压危象中的应用进行简介.     

Rare adrenal tumors in children

Apart from neuroblastomas, adrenal tumors are exceedingly rare in children and young adults. In this age group, the vast majority of patients present with clinical signs associated with excess hormone production. The most common tumor to arise from the adrenal cortex is an adrenocortical carcinoma (ACC). Similar to the situation in adults, this tumor is frequently diagnosed at a late stage and carries a very poor prognosis. ACCs require extensive/aggressive local resection followed by mitotane chemotherapy. A multidisciplinary approach is essential, and these children should be referred to units that have previous experience in managing ACCs. International registries are an invaluable source for evidence-based care, and such collaborations should be further developed in the future.Pheochromocytomas are derived from the adrenal medulla and present with symptoms caused by high secretion of catecholamines. At least one-third of these children will be found to carry genetic mutations, most commonly the RET gene (MEN2 syndrome) or the VHL gene.Open radical adrenalectomy should be offered to children with adrenocortical cancers. For all other cases, laparoscopic adrenalectomy is the treatment of choice. It is possible that the retroperitoneoscopic approach will gain increasing favor. The role of robotic adrenalectomy remains controversial.     

Nine cases of childhood adrenal tumour presenting with hypertension and a review of the literature

AIM: To describe the clinical features, treatment and outcome of children adrenal tumors presenting with hypertension. METHODS: The records of nine children under 16 years of age with adrenal tumours presenting with hypertension were analysed. Details were recorded for family history, clinical presentation, biochemistry, imaging, histology, treatment and outcome. RESULTS: Abdominal mass was palpable only in one patient at diagnosis. Besides hypertension-associated symptoms, Cushing's syndrome was the common presentation form (n = 4). Abdominal computed topography showed adrenal mass in all patients. Tumours were completely resected for each patient. The median tumour weight was 73 g (11-530 g) and the size ranged from 1.5 x 1.5 to 12 x 14 cm2. Pheochromocytoma (n = 2), adrenocortical adenoma (n = 3), adrenocortical carcinoma (n = 1), neuroblastoma (n = 2) and ganglioneuromas (n = 1) were found. In one case, adrenal pheochromocytoma first occurred and non-functioning islet cell tumour successively occurred at pancreas. A better status is common at a median follow-up time of 3.5 years. CONCLUSIONS: Childhood adrenal tumours presented with hypertension showed an atypical course, variable presentation. We report a unique case of adrenal pheochromocytoma followed by the occurrence of non-functioning islet cell tumour. Reversal of hypertension by surgery is crucial. Imaging techniques are important to detect adrenal tumours.     

儿童肾上腺肿瘤104例临床分析

目的分析儿童肾上腺肿瘤的临床特征,提高对儿童肾上腺肿瘤诊断和处理的认识。方法回顾分析1991—2002年12年间中山大学附属第一医院收治的104例儿童肾上腺肿瘤的临床特点、诊断和病理的关系。结果发病年龄0～14岁,男63例,女41例。神经母细胞瘤75例;肾上腺皮质肿瘤22例,其中癌及腺瘤各11例;嗜铬细胞瘤2例;其他5例。神经母细胞瘤患儿以发热、腹痛、腹部包块为主要表现,尿香草杏仁酸(VMA)可不升高;肾上腺皮质肿瘤患儿中外周性性早熟的临床及内分泌激素改变多于库欣综合征表现。血乳酸脱氢酶(LDH)水平对鉴别良/恶性肿瘤有提示意义。结论儿童肾上腺肿瘤以神经母细胞瘤及肾上腺皮质肿瘤最为常见,临床表现异于成人,应结合临床及影像学特征综合诊断。     

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各种应激均可使正常的肾上腺分泌皮质醇增多,可较平时增高2~7倍。严重应激状态下血皮质醇水平可高于1mg/L,以适应机体的需要。凡有原发或继发的、急性或慢性的肾上腺皮质功能减退时,就不能产生正常量的皮质醇,应激时更不能相应地增加皮质醇的分泌,因此产生一系列肾上腺皮质激素缺乏的急性临床表现,包括高热,胃肠紊乱,循环虚脱,神志淡漠、萎靡或躁动不安,谵妄甚至昏迷,称为肾上腺危象。强烈的刺激促使机体出现以交感-肾上腺髓质和下丘脑-垂体-肾上腺皮质功能增强为主要特征的一系列神经-内分泌反应,并由此引起机体各种机能和代谢,甚至结构上…     

Diagnosis and management of children with adrenal insufficiency

Adrenal insufficiency (AI) is a rare diagnosis. Symptoms may be vague, easily overlooked or attributed to more common childhood diagnoses. Early diagnosis and treatment is essential as untreated AI may present as adrenal crisis, a potentially fatal condition. Adrenal insufficiency may result from lesions at all levels of the hypothalamic pituitary adrenal axis. Accurate diagnosis is essential as the treatment and prognosis of AI differs according to the site and nature of the lesion. Cortisol is secreted in a circadian manner. Cortisol concentrations rise from the early hours of the morning, peak 30 minutes after waking before falling throughout the day and overnight. Childhood AI is treated with hydrocortisone, which has a short half-life, requiring three to four daily doses. Modified release formulations, which achieve a more physiological cortisol profile and require less frequent dosing, are in development and may become available to children. During periods of illness and injury, children with AI are at risk of adrenal crisis. It is essential that parents and carers are educated in the management of ‘sick days’, can recognise symptoms of adrenal crisis and seek medical advice appropriately. In turn, it is essential that medical staff know how to manage an adrenal crisis in children and young people. In this article, we will briefly review the physiology of the hypothalamic pituitary adrenal axis, causes of AI, options for treatment, methods of monitoring for the adequacy of treatment, and finally the treatment of children with AI during illness and surgery.