新生儿急性呼吸窘迫综合征的发病机制及诊断治疗进展

急性呼吸窘迫综合征是各种致病因素引起的急性、进行性、炎性肺损伤过程.新生儿急性呼吸窘迫综合征的病死率高达30％ ～ 60％.近年来随着对该病发病机制的深入研究,人们在其诊断、治疗方面也提出了很多新的观点.该文着重就新生儿急性呼吸窘迫综合征的发病机制、诊断及治疗的进展作一综述.     

急性肺损伤发病机制研究进展

急性肺损伤/急性呼吸窘迫综合征是指非心源性的各种内外因素导致的急性、进行性、缺氧性呼吸功能不全或呼吸衰竭;肺损伤的病因多种多样,发病机制非常复杂,且尚不明确.该文对其目前相关病因及发病机制研究进行综述.     

Acute respiratory distress syndrome in children with acute iron poisoning: the role of intravenous desferrioxamine

The acute respiratory distress syndrome is a rare but potentially fatal complication of severe acute iron poisoning and its pathogenesis has been linked with direct and indirect iron toxicity as well as the use of the chelator drug desferrioxamine. We report a case of severe acute respiratory decompensation in a child treated according to the current protocol for chelation therapy and discuss its implications. Conclusion We believe that the efficacy and safety of chelation therapy in severe acute iron poisoning may be improved by targeting the initial high levels of readily chelatable serum iron with adequate doses of desferrioxamine without prolonging its use unnecessarily. Received: 18 December 1998 / Accepted: 2 June 1999     

Acute disseminated encephalomyelitis – a diagnosis to consider

The case history of an 11-year-old boy with post-infectious acute disseminated encephalomyelitis (ADEM) is presented. ADEM is a diagnosis that is increasingly being made with the advent of modern radiological techniques. However, the syndrome cannot be thought of as a discrete clinical entity as it is triggered by a wide range of factors including infections and vaccinations. Furthermore, other pathological processes may give the same radiological appearances. The aetiology, pathophysiology and management of the syndrome are discussed. Conclusion Acute disseminated encephalomyelitis is a radiological diagnosis that has many precipitants. Thought must be applied in developing a differential diagnosis to allow the possibility of targeting treatment at the underlying cause. Received: 10 May 1999 / Accepted: 4 January 2000     

Simultaneous occurrence of the haemolytic uraemic syndrome and acute post-infectious glomerulonephritis

We report on two children, a 12-year-old boy and a 6-year-old girl, with simultaneous occurrence of clinical and laboratory features consistent with both diarrhoea-negative haemolytic uraemic syndrome (D-HUS) and acute post-infectious glomerulonephritis (APGN). Both presented with acute renal insufficiency, hypertension and oedema. Laboratory evaluation revealed micro-angiopathic anaemia with burr cells, thrombocytopenia, elevated lactic dehydrogenase and low complement C3. Urinalysis showed marked proteinuria and haematuria. Renal biopsy was characteristic of APGN, but not of HUS. The outcome was good in both children. Conclusion The simultaneous occurrence of diarrhoea-negative haemolytic uraemic syndrome and acute post-infectious glomerulonephritis is rare. The outcome is generally good as is expected in the latter condition in contrast to the former. Received: 10 August 2000 / Accepted: 20 September 2000     

糖皮质激素治疗儿童急性肺损伤/急性呼吸窘迫综合征的研究进展

儿童急性肺损伤/急性呼吸窘迫综合征(ALI/ARDS)是许多肺内疾病和(或)肺外疾病所致的严重应激反应和(或)严重炎症反应而导致的肺组织内皮和上皮受损引起的急性呼吸衰竭,病死率很高.糖皮质激素是目前临床使用最为广泛的抗纤维化和抗炎症药物,但由于其疗效差异较大,故在治疗ALI/ARDS中一直存在争议.近年研究表明其作用与糖皮质激素受体数量及亲和力有密切关系.     

Acute myeloid leukemia in TAR syndrome

TAR syndrome is a rare inherited.autosomal recessive disorder with a mortality rate of 30–40% mainly as a result of haemorrhage, in the first year of life. Most of the infants recover from the effects of thrombocytopenia and associated haematological complications with the modern medical care. Very rarely, the outcome is fatal, with the occurrence of acute leukemias.     

Clinical features of acute kidney injury in patients with Kawasaki disease

Although acute kidney injury (AKI) is a common complication in hospitalized children, AKI has rarely been reported in patients with Kawasaki disease (KD). Herein, we review the clinical trajectories of AKI in patients with KD. A total of 39 patients with KD who developed AKI have been reported in 28 publications as case reports. The causes of AKI include prerenal AKI associated with acute heart failure (AHF), intrinsic AKI caused by tubulointerstitial nephritis (TIN), acute nephritic syndrome (ANS), hemolytic uremic syndrome (HUS), immune complex-mediated nephropathy, rhabdomyolysis, and KD shock syndrome (KDSS). Six of the 39 patients (15.4%) underwent renal replacement therapy. While AHF and multiple organ dysfunction syndrome developed in 41% and 68% of KD patients with AKI, respectively, all patients recovered without any renal sequelae. Although the precise pathogenic mechanism underlying the development of AKI in patients with KD is unknown, several possible mechanisms have been proposed, including T-cell-mediated immunologic abnormalities for TIN, renal and glomerular endothelial injury resulting from vasculitis for HUS, immune complex-mediated kidney injury for immune complex-mediated nephropathy and ASN, and capillary leak and an increased release of cytokines with myocardial dysfunction for KDSS.     

他汀类药物的研究进展及在急性呼吸窘迫综合征中的作用

急性呼吸窘迫综合征是儿童常见重症,威胁儿童的生命健康.其病理机制为肺部组织广泛的炎症反应.他汀类药物已广泛应用于心脑血管疾病的防治,目前研究发现他汀类药物具有抗炎及调节免疫的作用.本文将对他汀类药物的作用机制、在急性肺损伤及急性呼吸窘迫综合征中的作用机制和应用,以及他汀类药物的有效性和安全性进行综述.     

SIGNIFICANCE OF MONOSOMY 7 IN MYELOID LEUKEMIAS IN CHILDREN

Loss of chromosome 7 material, either as complete loss of one chromosome-monosomy 7 (-7)- or as deletion of the long arm (7q-), is the most common cytogenetic abnormality in childhood myelodysplastic syndrome (MDS) and juvenile myelomonocytic leukemia (JMML), seen in approximately 30% of the cases. Only 4-5% of childhood acute myeloid leukemia (AML) cases show -7/7q-. Monosomy 7 in children has often been considered to represent a distinct hematologic disorder described as the monosomy 7 syndrome. Loss of chromosome 7 occurs in a heterogeneous group of myeloid disorders and recent data give no support to the concept of monosomy 7 as a distinct syndrome. Only patients with a diagnosis of JMML fit what previously has been referred to as the monosomy 7 syndrome. There are no major clinical differences between JMML with and without -7. Unlike what has been found in adults, monosomy 7 is not a poor prognostic factor in childhood MDS. However, -7 in AML is associated with a very poor prognosis. AML with 7q-, in contrast, has an outcome similar to AML with normal karyotype. Stem cell transplantation is the treatment of choice and should be performed as early as possible.     

儿童急性呼吸窘迫综合征肺复张策略的研究进展

近年来肺复张策略因可打开肺泡,减少肺泡萎陷所致的肺损伤,改善肺顺应性,提高动脉氧分压与氧合指数,减少肺内分流而成为急性呼吸窘迫综合征机械通气治疗手段之一.本文就肺复张在儿童急性呼吸窘迫综合征的应用做一综述.     

应激性心肌病

应激性心肌病(TTS)是一种急性且大部分可逆的心肌病。虽然儿茶酚胺升高与TTS有关,但详细的致病机制尚不完全清楚。根据左室壁运动异常的分布,临床分成不同亚型。最终诊断需要心脏血管造影、超声心动图以及心脏磁共振。治疗建议多来自于观察性研究,而非多中心随机对照研究。对重症患者,机械循环辅助是必要的支持措施,同时应避免使用儿...     

Two Unusual Cases of Congenital Rubella Syndrome Associated with Acute Myelocytic Leukemia or chilaiditi's Syndrome

In the Ryukyu Islands of Japan, an extensive epidemic of rubella occurred from the end of 1964 until the beginning of 1965. Approximately 400 children with congenital rubella syndrome were born in 1965 [1]. Two boys with unusual associated problems are reported in this paper. One case was complicated with leukemia and the other with Chilaiditi's syndrome.     

Tubulo-interstitial nephritis and uveitis syndrome associated with mastitis: a case report

A new case of tubulo-interstitial nephritis and uveitis (TINU) syndrome in a 14-y-old girl is described. In this patient unilateral mastitis was an associated feature. The aetiology of this rare syndrome, the prognosis for which is usually good, is still unknown. The most common theory supports a cell-mediated immune response induced by infectious/antigenic stimuli. CONCLUSION: The presence of acute unilateral mastitis in this patient may represent an additional localization in TINU syndrome.     

高频振荡通气在小儿急性呼吸窘迫综合征的研究进展

小儿急性呼吸窘迫综合征是小儿常见危重症,病死率较高.高频振荡通气具有肺保护、改善氧合和减轻肺部炎症反应等作用,被认为是治疗小儿急性呼吸窘迫综合征的一种理想通气模式.该文就国内外关于高频振荡通气在小儿急性呼吸窘迫综合征的应用进展及地位做一综述.     

Leucocytoclastic vasculitis in infant

Acute hemorrhagic edema of infancy is a laucocytoclastic vasculitis characterized by a triad of fever, large purpuric skin lesions and edema. The presentation is often dramatic. The manifestations are often misinterpreted as a septicemic illness. We report the occurrence of this syndrome in a 11/2-year-old-child, highlighting some of the diagnostic and therapeutic problems encountered.     

急性呼吸窘迫综合征机械通气策略的新进展

急性呼吸窘迫综合征（ARDS）机械通气的目标是保证足够的氧合和最小的呼吸机相关肺损伤。无创机械通气应慎用于ARDS。小潮气量通气的肺保护性通气策略能降低ARDS死亡率,以驱动压为导向设置潮气量更合理。建议根据静态压力-容积曲线采用低位转折点法来确定理想的呼气末正压（PEEP）,不支持常规使用高水平PEEP。俯卧位通气、高频振荡通气和体外膜氧合技术可用于拯救重症ARDS。机械通气中保持ARDS患者自主呼吸很重要,可采用双相气道正压通气、压力支持通气和神经电活动辅助通气等辅助通气模式。不推荐补充外源性肺泡表面活性物质、吸入一氧化氮或支气管扩张剂、气道压力释放通气和部分液体通气。     

糖皮质激素在急性呼吸窘迫综合征中的应用

持久的全身和肺部炎症反应是急性呼吸窘迫综合征（acute respiratory distress syn-drome,ARDS）使用糖皮质激素（简称激素）治疗的基本依据,但目前ARDS患者使用激素的剂量、时间、疗程和效果仍然存在争议.大剂量、短疗程激素疗效欠佳,甚至增加病死率.现阶段认为相对中长疗程（2周～1个月）、小剂量或替代剂量（如甲泼尼龙1 ～2 mg/kg）激素,可降低ARDS病死率,缩短住ICU时间和呼吸机使用时间,并减轻肺纤维化.儿童ARDS的激素治疗仍缺少多中心、随机对照研究.     

Reyes’s syndrome in Delhi

Reye’s syndrome was diagnosed in about 15% of cases of encephalopathy syndrome admitted in hospital. Hypoglycemia, considered a hallmark of the disease, was an uncommon observation particularly in patients beyond one year of age (8.5%). Transaminases were more useful than hypoglycemia for suspecting this syndrome (raised in 80% of cases). Progression of the disease was very rapid. The existing management is very unsatisfactory. 11 out of 12 cases died. A high order of suspicion in a patient who presents with vomiting, hepatomegaly and sensorial obtundation is proposed for early diagnosis.