Balloon Dilatation of the Pulmonary Valve in a Patient with Pulmonary Atresia and Intact Ventricular Septum Using a Commercially Available Radiofrequency Catheter

Using a commercially available 5F deflectable radiofrequency catheter, we have succeeded in percutaneous valvotomy of an imperforate pulmonary valve and consecutive balloon dilatation in a baby with pulmonary atresia and intact ventricular septum. After the procedure, right ventricular systolic pressure fell from 125 mmHg to 65 mmHg, and right ventriculography demonstrated anterograde blood flow into the pulmonary arteries. There were no major complications. Doppler echocardiography at 1 year after the procedure demonstrated a pressure gradient across the pulmonary valve of 20 mmHg with mild pulmonary and tricuspid regurgitations.     

Response to nitric oxide in term and preterm infants

The response to three levels (10 ppm, 20 ppm and 40 ppm) of nitric oxide (NO) was assessed in 30 infants, median gestational age 30 (range 24–42) weeks. All the infants required an inspired oxygen concentration of more than 0.5, despite receiving surfactant where appropriate. All but one infant had a positive response to NO (median reduction in the oxygenation index (OI) was 33%, range −9%–90%), but only 20 infants showed a greater than 20% reduction in the OI. There was no obvious relationship of the optimum NO level (i.e. that associated with the maximum reduction in OI) and either diagnosis (congenital diaphragmatic hernia, meconium aspiration syndrome, respiratory distress syndrome, pulmonary interstitial emphysema (PIE), hydrops and sepsis) or maturity, except that five of six infants with PIE responded best to 40 ppm, as did eight of nine infants less than 28 weeks gestational age. We conclude NO dosage should be individualized and NO levels up to 40 ppm should be considered in very immature infants. Received: 15 March 1996 / Accepted: 5 February 1997     

Malignant fibrous histiocytoma of the lung in a child

We describe a 12-year-old patient with a primary pulmonary mass in the left upper lung. The diagnosis of inflammatory pseudotumour was suspected preoperatively. After pathological examination and complete clinical evaluation, a diagnosis of malignant primary pulmonary fibrous histiocytoma was established. This is a very uncommon primary neoplasm of the lung and to our knoweledge only five paediatric cases have been reported. Because of the rarity of these sarcomas and histological similarities to benign inflammatory pseudotumour, care must be taken to avoid confusion between the two disorders particularly in intra-operative frozen sections. Conclusion Primary malignant fibrous histiocytoma of the lung is an uncommon tumour that should be considered in the differential diagnosis of pulmonary neoplasms of childhood. The histological diagnosis can be difficult due to the similarities with␣inflammatory pseudotumour. Received: 29 May 1996 / Accepted: 21 July 1996     

Coarctation of the Left Pulmonary Artery: Effects on the Pulmonary Vasculature of Infants

At autopsy, two infants had unsuspected coarctation of the left pulmonary artery (CoLPA), which was produced by an extension of ductal tissue into the wall of the left pulmonary artery. The first case, a 4-month-old girl, also had a ventricular septal defect and an anomalous branching pattern of the innominate arterial trunk. Pulmonary arterial hypertensive changes were noted in the right lung. In contrast, the left lung showed thin-walled pulmonary arteries. The second case, a term female newborn, had exhibited severe unexplained respiratory distress since birth. Histologic sections of the right lung showed dilated pulmonary arteries with thinned media, whereas the left lung showed a persistent fetal arterial pattern. It is believed that the peripheral pulmonary arterial changes are age-dependent and associated with asymmetric blood flow between the right and left pulmonary arteries. CoLPA is a rare pulmonary artery defect, and early diagnosis of this abnormality is important.     

Tetralogy of Fallot with Absent Pulmonary Valve and Bronchial Compression: Treatment with Endobronchial Stents

Absent pulmonary valve syndrome (APVS) is a rare congenital cardiac lesion. The lesion includes ventricular septal defect, overriding aorta, and absence of the pulmonary valve, with resultant pulmonary incompetence. It has been suggested that the pulmonary incompetence induces intrauterine dilatation of the pulmonary artery, which leads to tracheobronchial compression. One of the presenting features in infants with APVS is severe airway obstruction, which may be difficult to manage. We report an infant who benefited from bilateral endobronchial endoscopic stent placement.     

Growth of asthmatic children is slower during than before treatment with inhaled glucocorticoids

Reports on the influence of inhaled glucocorticoids on growth have been controversial. We studied the growth of prepubertal asthmatic children prior to and during glucocorticoid therapy. We collected retrospectively the notes of 201 asthmatic children aged 1–11 years receiving inhaled beclomethasone dipropionate or budesonide. We calculated their height and height velocity standard deviation scores (HSDS and HVSDS, respectively) before the treatment and up to 5 years during the treatment and compared those with the growth of healthy peers. The dose of the medication was calculated and the severity of asthma was assessed. The asthmatic children grew similarly to their healthy peers before treatment with inhaled glucocorticoids: the mean HSDS was +0.02 and the mean HVSDS +0.01 for boys and -0.16 and +0.13 for girls, respectively. Growth retardation took place soon after the start of the treatment, the most profound decrease in the growth velocity (the change in the mean HVSDS from +0.05 to -0.88) occurring during the first year of treatment. The growth-retarding effect of inhaled glucocorticoids was not dose dependent. In the covariance analysis the increasing severity of asthma had a significant interaction with repeated measurements, showing more growth retardation along with more severe asthma, especially during long-term treatment. Asthma per se does not impair growth, but inhaled glucocorticoids may do so. Careful monitoring of the growth of all asthmatic children receiving inhaled glucocorticoids is necessary because the growth-retarding effect of the medication is not dose dependent. Individual sensitivity might explain the differences seen in the growth patterns of children receiving inhaled glucocorticoids.     

Impairments, disabilities and handicaps in children and adolescents with epilepsy

The patterns of motor and sensory impairments, disabilities and handicap were assessed in 217 children and adolescents with epilepsy. Motor and sensory dysfunctions were found to be common even in children without major additional neurodisorders such as cerebral palsy and mental retardation. Handicap was most severe in the dimensions of physical independence and orientation and increased significantly with duration of epilepsy. It was more severe when the onset of seizures was early and when secondary generalized seizures were present. Handicap was significantly reduced after epilepsy surgery.     

低出生体重儿血锌值及其动态观察

为了比较５２例低出生体重儿血锌值，生后每月随访外周血锌至９个月，将调查对象分为３组，根据简易ＡＡＳ测定方法，将经过特殊处理的标本采用ＳＡＳ／７２７原子吸收分光光度计测定血锌值。结果：低出生体重儿脐血红细胞锌。外周血锌值均低于正常足月儿；低出生体重儿母亲血红细胞锌值低于正常足月儿母亲。对低出生体重儿９个月的随访表明，其外周血锌值仍处在低水平。提示：锌能影响胎儿宫内生长，低出生体重儿应补充足量的锌。     

内皮素与新生儿败血性休克的研究

为了探讨内皮素在新生儿败血性休克中的作用，应用体外培养及放射免疫测定法，观察内毒素对人脐静脉血内皮细胞分泌内皮素的影响及山莨菪碱（６５４－２）的可能作用，并测定３２例败血症及２１例败血性休克新生儿血浆内皮素含量的变化。结果：（１）内毒素能刺激人脐静脉内皮细胞内皮素的分泌，呈时间及剂量依赖性；（２）６５４－２在体外能抑制内毒素刺激的内皮素的合成；（３）新生儿败血症及败血性休克时血浆内皮素水平明显增高，并与疾病严重程度及预后有关。提示：内皮素参与新生儿败血性休克的病理生理过程，为６５４－２治疗败血性休克提供了新的理论依据。     

异丙酚的剂量与镇静和呼吸抑制作用之间的关系

研究不同剂量的异丙酚的量效关系。方法:选择50例甲状腺腺瘤切除病人,随机分为5组,Ⅰ组,0.5mg/kg、Ⅱ组0.75mg/kg、Ⅲ组1.0mg/kg、Ⅳ组1.5mg/kg、V组2.0mg/kg.采用Star呼吸机监测呼吸功能指标,同时观察其神志变化,应用Probit法分析该药量效关系。结果:Ⅱ、IV、V组随着剂量增加,潮气量降低的幅度和呼吸抑制持续时间明显增加,以给药后1～3分钟潮气量减少最为明显(P<0.01),浅睡状态ED_(50)为0.98mg/kg.深睡状态ED_(50)为1.26mg/kg,呼吸抑制ED_(50)为1.1mg/kg。结论:静注0.8～1mg/kg异丙酚可以产生适用于非全麻病人的镇静、催眠作用,而对呼吸抑制的作用较小。