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Neonatal cyanosis, methaemoglobinaemia and haemolytic anaemia. 新生儿紫绀、甲基血红蛋白血症和溶血性贫血。
Pub Date : 1988-09-01 DOI: 10.1111/j.1651-2227.1988.tb10744.x
S Menahem
The early and correct diagnosis of t he cause of neonatal cyanosis is a medical emergency, particularly if t he infant develops increasing acidosis and low arterial oxygenation. Rarely acquired methaemoglobinaemia may be the cause which, once diagnosed, may readily b e treated with the infusion of the reducing agent methylene blue (1). Occasionally, however, the treatment itself may have consequences, namely haemolytic aneamia and/or cyanosis ( 2 4 ) . The former, if severe enough, may entail an exchange transfusion for increasing hyperbilirubinaemia. Two infants are described. They developed methhaemoglobinaemia from an inadvertent injection of prilocaine hydrochloride (Citanest). They responded well t o methylene blue, though one developed severe jaundice. Case I . A boy, delivered normally at term, with an Apgar of 8 at 1 min and 10 at 5 min. By 2 hours he had become pale and dusky and by 3 hours he was quite blue with mild tachypnoea. His pulse was normal, there were no murmurs and his chest was clinically clear. Blood gases done with the infant in air and 90% ambient oxygen revealed a normal pH and Paco, levels and a Pao2 of 55 and 236 mmHg, respectively. Chest X-rays and electrocardiograph were normal and the haemoglobin was 143 gll. The infant remained grey-blue despite oxygen, and a blood spot on filter paper took on a brown-chocolate colour. Methaemoglobinaemia was diagnosed clinically and subsequently confirmed by spectral absorption peaks constituting 39% of the haemoglobin. An echocardiogram showed a structurally normal heart. Four mg of diluted methylene blue (approximately 1 mg/kg-birthweight 3650 g) was given intravenously over 5 min with a dramatic resolution of the cyanosis over the next 15-30 min. The infant did well and was discharged with its mother. Careful examination of the infant’s scalp revealed a tiny abrasion which was thought to be due to a needle prick. It was considered that the baby had inadvertently been injected with prilocaine hydrochloride when the mother’s perineum was injected prior to the episiotomy. Case 2. A boy, Baby A was delivered normally at term with an Apgar of 10 at 1 min and at 5 min. Within an hour he was slightly dusky and was quite blue when seen at 2 hours. He was otherwise normal where examined. Blood gases taken with the infant in air and 90% ambient oxygen revealed a normal pH and Paco, levels and a Pao2 of 86 mmHg, and 372 mmHg, respectively. Chest X-ray and electrocardiograph were normal and a filter paper test was again suggestive of methaemoglobinaemia. Four mg of methylene blue was given slowly i.v. (birthweight 3860 g), the colour of the infant returned to normal within half an hour. By the next day, however, the infant had become increasingly jaundiced. His serum bilirubin measured 309 mmol/l on day 3, at which time phototherapy was commenced. No incompatibility was noted on a direct Coombs’ test and a glucose-6-phosphate dehydrogenase screen was negative. By the 4th day the serum bili
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引用次数: 4
Oral biotin treatment is effective for atopic dermatitis in children with low biotinidase activity. 口服生物素治疗对低生物素酶活性儿童的特应性皮炎有效。
Pub Date : 1988-09-01 DOI: 10.1111/j.1651-2227.1988.tb10748.x
Y Iikura, Y Odajima, T Nagakura, K Iinuma, K Hayakawa, J Oizumi
Clinical features of biotinidase deficiency in childhood are highlighted by various neurological and cultaneous manifestations (1, 2). The skin changes are indistinguishable from those of atopic dermatitis, however, they respond to biotin treatment (3) . We describe four patients with atopic dermatitis who were found to have low serum biotinidase activity and in whom oral biotin led to a dramatic improvement of the skin lesions.
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引用次数: 13
Brain atrophy with intracranial calcification following congenital HIV infection. 先天性HIV感染后脑萎缩伴颅内钙化。
Pub Date : 1988-09-01 DOI: 10.1111/j.1651-2227.1988.tb10752.x
P A Tovo, C Gabiano, S Favro-Paris, E Palomba, G Gajno

An 18-month-old girl with AIDS related complex following congenital HIV infection suffered since the first days of life from severe neurological disturbances. The extensive congenital brain atrophy and the diffuse intracranial calcification which were found were strongly reminiscent of intrauterine infection. Since the agents usually responsible for congenital infections were excluded an HIV embryopathy appears likely.

一名18个月大的女婴患有先天性HIV感染后的艾滋病相关并发症,从出生的第一天起就遭受了严重的神经障碍。广泛的先天性脑萎缩和弥漫性颅内钙化是宫内感染的强烈提示。由于通常导致先天性感染的因素被排除在外,HIV胚胎病似乎很可能。
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引用次数: 8
Iatrogenic intraspinal epidermoid tumour. 医源性椎管内表皮样瘤。
Pub Date : 1988-09-01 DOI: 10.1111/j.1651-2227.1988.tb10746.x
V Ersbak, N Hobolth
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引用次数: 10
Low plasma levels of vitamin A and E during weight reduction. 减肥期间血浆维生素A和E水平低。
Pub Date : 1988-09-01 DOI: 10.1111/j.1651-2227.1988.tb10747.x
K Zwiauer, K Widhalm, G Brubacher
Current knowledge and the growing awareness of adverse consequences of human obesity to health has resulted in a frequent use of different weight reducing regimes within the last years. Much attention has been paid in the past to the effect of weight reduction on atherosclerotic risk factors and numerous reports have been published regarding protein and carbohydrate metabolism in obese subjects. Very few studies have evaluated the vitamin status of patients undergoing rapid and/or longterm weight reduction, particularly in grossly obese children and adolescents (1, 2).
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引用次数: 2
Venous thrombosis associated with lupus anticoagulant and anticardiolipin antibodies. 静脉血栓与狼疮抗凝血和抗心磷脂抗体相关。
Pub Date : 1988-09-01 DOI: 10.1111/j.1651-2227.1988.tb10750.x
P Pelkonen, O Simell, V Rasi, O Vaarala

We describe deep vein thrombosis associated with lupus anticoagulant and anticardiolipin antibodies in three children aged 10 to 14 years. One of them also had arterial thromboses. None of the patients had systemic lupus erythematosus when the thrombosis first occurred, but one fulfilled the criteria for systemic lupus erythematosus 3 years later. At presentation all had symptoms suggestive of pulmonary embolism and evidence of an autoimmune disease: Addison's disease in one, anti-DNA or antinuclear antibodies in all three, and a positive Coombs' test in two. Two of the three gave a false-positive test for syphilis. In the patient with systemic lupus erythematosus recurrent thrombocytopenia and severe haemolytic anaemia necessitated splenectomy. A child should be tested for lupus anticoagulant or anticardiolipin antibody if venous or arterial occlusion occurs without a known predisposing cause, or if there is pulmonary embolism or symptoms or laboratory findings suggestive of a connective tissue disease.

我们描述深静脉血栓与狼疮抗凝血剂和抗心磷脂抗体相关的三个儿童年龄10至14岁。其中一人还患有动脉血栓。患者首次发生血栓时均无系统性红斑狼疮,但有1例患者3年后符合系统性红斑狼疮的诊断标准。在就诊时,所有人都有提示肺栓塞的症状和自身免疫性疾病的证据:1人患有艾迪生氏病,3人都有抗dna或抗核抗体,2人的库姆斯试验呈阳性。其中两人的梅毒检测呈假阳性。系统性红斑狼疮患者复发性血小板减少和严重溶血性贫血需要脾切除术。如果静脉或动脉闭塞没有已知的易感原因,或者有肺栓塞或结缔组织疾病的症状或实验室结果,儿童应检测狼疮抗凝血剂或抗心磷脂抗体。
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引用次数: 43
A double-blind clinical trial on diphenhydramine in pertussis. 苯海拉明治疗百日咳的双盲临床试验。
Pub Date : 1988-07-01 DOI: 10.1111/j.1651-2227.1988.tb10716.x
A Danzon, J Lacroix, C Infante-Rivard, L Chicoine
Patients were prospectively included in the study if they were younger than 12 months of age, if nurses noted characteristic whooping spells, and if the cases were not treated with steroids. Randomization and allocation of treatment followed a double-blind pattern, according to a list determined by the ParkeDavis Company from a table of random numbers. The hospital pharmacy was responsible for the assignment of patients to the treatment groups. The following baseline data were measured: age at entry, previous vaccination for pertussis, interval between the first paroxysm and hospitalization, concomitant administration of erythromycin, and presence of an opacity on chest radiography. Since patients were included in the trial at different stages of their disease, the number of fits during the 24 hours preceding the introduction of drug or placebo was measured as an indicator of the severity of disease at time zero. Systematic laboratory tests included white blood cell count, lymphocyte count, glycemia, chest radiography, viral serology, and three nasopharyngeal cultures for B. pertussis. Paired serum specimen, obtained at least two-weeks apart, were tested for complement fixing antibody to adenovirus, influenza, parainfluenza and respiratory syncitial virus. Appearance, smell, and taste of active and placebo syrups were similar. Patients in the experimental group received 5 mg/kg/day of the active drug in three doses. The outcome was the frequency of paroxysms between the 25th and the 48th hour after initiation of treatment. Monitoring was similar for all patients. Coughs were monitored around the clock with a system of microphones set up in each room. All patients were fed with frequent small meals. None received sedatives or humidity. The average number of fits per day was compared with a Student's t-test. A dummy regression analysis was used to test the hypothesis that the slopes for the compared groups were parallel (no interaction between prognostic factors and treatment). Having accepted that the slopes were parallel, the mean number of fits per day was compared in the two groups, adjusting for baseline data and the number of fits during the 24 hours preceding entry into the trial. Results were considered as significant for p<0.05. The smallest detectable difference between the two groups, given the sample size, was estimated using a formula suggested by Lachin (1). Informed consent was obtained from a parent or a guardian for each patient. The study was approved by the Ethics Committee of Sainte-Justine Hospital.
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引用次数: 11
Longitudinal analysis of infantile growth in children with celiac disease. 乳糜泻患儿婴儿生长的纵向分析。
Pub Date : 1988-07-01 DOI: 10.1111/j.1651-2227.1988.tb10693.x
J Karlberg, J I Henter, E Tassin, B S Lindblad

The aim of the present study was to describe linear growth of infants with celiac disease, using the "ICP-growth model". Supine length during the first three years of life was studied longitudinally in 63 infants with diagnosed celiac disease. "Undisturbed" linear growth was seen during the first six postnatal months followed by reduced growth during the second half of the first year. After 1.5 years of age a pattern of catch-up growth was observed, leading to an average attained length at 3 years of age similar to that of the controls. According to the "ICP-growth model", normal linear growth can mathematically be represented during the first 3 years of life by an Infancy component with the addition of a Childhood component, the latter acting from the second half of the first postnatal year. The onset of the Childhood component (assumed to represent the age at which growth hormone begins to influence linear growth significantly) was delayed by about an average of 3 months, which is in agreement with the observed reduction in gain during the second half of the first year of life. Children suffering from celiac disease and with "late" onset of the Childhood component were shorter at 1, 2 and 3 years of age than those with "normal" onset. The results of this investigation show that ICP-based growth charts are helpful in detecting and monitoring growth for children with celiac disease, and indicate a possible mechanism whereby malabsorption (and perhaps secondary malnutrition) leads to reduced growth velocity.

本研究的目的是用“icp -生长模型”描述患有乳糜泻的婴儿的线性生长。对63名确诊为乳糜泻的婴儿进行了3岁前仰卧位长度的纵向研究。“未受干扰”的线性生长在出生后的前六个月,随后在第一年的下半年生长减少。1.5岁后,观察到一种追赶生长模式,导致3岁时的平均达到长度与对照组相似。根据“icp -生长模型”,正常的线性生长在生命的前3年可以用一个婴儿期组成部分加上一个儿童期组成部分来表示,后者从出生后第一年的下半年开始。儿童期部分(假定代表生长激素开始显著影响线性生长的年龄)的开始时间平均推迟了约3个月,这与观察到的一岁后半段体重减少的情况一致。患有乳糜泻和儿童期“晚”发病的儿童在1岁、2岁和3岁时比“正常”发病的儿童要短。本研究结果表明,以icp为基础的生长图表有助于检测和监测乳糜泻儿童的生长,并指出了吸收不良(可能是继发性营养不良)导致生长速度降低的可能机制。
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引用次数: 25
Plasma lipid and apolipoprotein levels in children hereditarily predisposed to coronary heart disease. 冠心病遗传易感性儿童的血脂和载脂蛋白水平
Pub Date : 1988-07-01 DOI: 10.1111/j.1651-2227.1988.tb10700.x
N Perova, H Aingorn, V Metelskaya, T Dorofeeva, N Belokonj

Plasma lipids and apoproteins A-I, A-II and B levels were measured in 125 children whose fathers had coronary atherosclerosis (paternal coronary heart disease group), in 172 children with blood pressure lability and in 154 children selected at random from a representative subsample (reference). The paternal coronary heart disease group had, compared with the reference group, lower levels of high density lipoprotein cholesterol and apoprotein A-I, higher levels of low density lipoprotein cholesterol, triglycerides and apoprotein B, and also a higher ratio of apoprotein B to apoprotein A-I. The paternal coronary heart disease group subjects with blood pressure lability had the most pronounced changes in the lipoprotein spectrum, which were similar to atherogenic changes in the lipoprotein profile, characteristic of patients with coronary atherosclerosis.

在125名父亲患有冠状动脉粥样硬化的儿童(父亲冠心病组)、172名血压不稳定的儿童和从代表性子样本(参考)中随机选择的154名儿童中,测量了血浆脂质和载脂蛋白a - i、a - ii和B水平。与参照组相比,父亲冠心病组的高密度脂蛋白胆固醇和载脂蛋白a - i水平较低,低密度脂蛋白胆固醇、甘油三酯和载脂蛋白B水平较高,载脂蛋白B与载脂蛋白a - i的比例也较高。父系冠心病组血压不稳定受试者的脂蛋白谱变化最为明显,与冠状动脉粥样硬化患者的特征——脂蛋白谱的动脉粥样硬化变化相似。
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引用次数: 15
Pulmonary resection for localized bronchiectasis in cystic fibrosis. Report of three cases and review of the literature. 肺切除术治疗囊性纤维化伴局限性支气管扩张。三例病例报告及文献复习。
Pub Date : 1988-07-01 DOI: 10.1111/j.1651-2227.1988.tb10702.x
G Steinkamp, H von der Hardt, H J Zimmermann

Three children with cystic fibrosis and localized bronchiectasis were treated by surgical removal of the affected lobe, after attempts to clear the obstructed bronchus by medical therapy and bronchoscopy with suction and lavage had been unsuccessful. Three to six years after surgery, the results are excellent. Pulmonary disease is mild in all patients, shown by chest radiographs and pulmonary function tests. A review of 102 cases reported in the literature demonstrates that pulmonary surgery is effective and can be performed safely in selected patients.

3例囊性纤维化和局限性支气管扩张的儿童在尝试通过药物治疗和支气管镜吸洗清除阻塞的支气管失败后,通过手术切除受影响的肺叶。手术后三到六年,效果很好。胸部x线片和肺功能检查显示,所有患者均为轻度肺部疾病。对102例文献报道的病例的回顾表明,肺手术是有效的,可以在选定的患者中安全进行。
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引用次数: 15
期刊
Acta paediatrica Scandinavica
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