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Lipoid proteinosis. 脂蛋白沉积。
Pub Date : 2020-02-10 DOI: 10.32388/21g3ua
J. Keipert
The first symptom of this condition is usually a hoarse voice, which is due to deposits in the vocal cords. In infancy the hoarseness is expressed as a weak cry. The voice abnormalities persist throughout life and can ultimately cause difficulty speaking or complete loss of speech. Involvement of the throat, tonsils, and lips can result in breathing problems and upper respiratory tract infections. Deposits in the tongue can result in a thick and shortened tongue. They can also thicken the band of tissue that connects the tongue to the bottom of the mouth (frenulum), making it difficult to extend the tongue. The tongue may also have a smooth appearance due to damage to the taste buds.
这种情况的第一个症状通常是声音嘶哑,这是由于声带中的沉积物引起的。在婴儿时期,声音嘶哑表现为微弱的哭声。声音异常在一生中持续存在,最终可能导致说话困难或完全丧失语言能力。累及喉咙、扁桃体和嘴唇会导致呼吸问题和上呼吸道感染。舌头上的沉积物会导致舌头变厚和变短。它们还会使连接舌头和口腔底部的组织带(系带)变厚,使舌头难以伸展。舌头也可能由于味蕾受损而呈现光滑的外观。
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引用次数: 0
Infant sleeping position and cot death. 婴儿睡姿与婴儿床死亡
Pub Date : 1989-12-01
S Tonkin, I Hassall
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引用次数: 0
Breast feeding and HIV infection. 母乳喂养与艾滋病毒感染。
Pub Date : 1989-10-01
J Biddulph
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引用次数: 0
The Australian College of Paediatrics and the Paediatric Research Society of Australia. Abstracts of papers presented at the annual scientific meeting. May 1989. 澳大利亚儿科学院和澳大利亚儿科研究协会。在年度科学会议上发表的论文摘要。1989年5月。
Pub Date : 1989-10-01
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引用次数: 0
A cautionary note regarding the ACP policy statement on alcohol consumption in relation to pregnancy. 关于与怀孕有关的酒精消费的ACP政策声明的警告说明。
Pub Date : 1989-10-01
A H Lipson, W S Webster
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引用次数: 0
Stabilization and fixation of percutaneous central venous catheters. 经皮中心静脉导管的稳定和固定。
Pub Date : 1989-08-01
G J Reynolds
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引用次数: 0
Arousal deficit: mechanism of the sudden infant death syndrome? 唤醒缺陷:婴儿猝死综合征的机制?
Pub Date : 1989-08-01
N M Newman, J A Trinder, K A Phillips, K Jordan, J Cruickshank

Polygraphic tracings of 13 normal infants were recorded in a morning sleep at 1 and 2 weeks of age and 1, 2, 3, 4, and 6 months of age. A vibrotactile stimulus graded at 25, 50 and 100 Hz (frequency) and amplitudes of 1, 2 and 3 mm (intensity) was used, each combination being applied twice at 30 s intervals to the hand of the sleeping infant during active sleep (AS) and quiet sleep (QS). The results were analysed as percentages of failure to arouse (FTA) in relation to the number of stimulus trials, the criteria for FTA being the absence of a response in heart or respiratory rate, electroencephalogram, or chin electromyogram. The percentages of FTA from QS did not change significantly from 1 week to 6 months of age, irrespective of frequency or intensity. The percentages of FTA from AS fell sharply and significantly from 1 week to 2 months of age (P less than 0.001). At 3 months of age there was a significant increase followed by a significant decrease at 4 months of age, both changes showing a significant difference at P less than 0.05. Apart from the first week of age, the numbers of FTA from QS were greater than from AS for all stimulus trials. It is concluded that there is an arousal deficit in QS from 1 week to 6 months of age and the temporary deficit in AS at 3 months of age could explain the peak incidence of SIDS at this time.

对13名正常婴儿在1、2周龄和1、2、3、4、6个月大时的晨间睡眠进行多路描记。使用频率分别为25、50和100 Hz,强度分别为1、2和3 mm的触觉振动刺激,每隔30秒对处于活跃睡眠(AS)和安静睡眠(QS)的婴儿的手施加两次刺激。结果被分析为未唤起的百分比(FTA)与刺激试验次数的关系,FTA的标准是在心脏或呼吸频率、脑电图或下巴肌电图中没有反应。从1周龄到6个月龄,无论频率或强度如何,QS的FTA百分比没有显著变化。从1周龄到2月龄,AS的FTA百分比急剧下降且显著(P < 0.001)。3月龄显著升高,4月龄显著降低,差异均有统计学意义,P < 0.05。除第1周龄外,在所有刺激试验中,来自QS的FTA数量均大于来自AS的FTA数量。综上所述,婴儿在1周至6月龄时存在唤醒缺陷,而在3月龄时存在暂时的AS缺陷可以解释婴儿在这一时期发生SIDS的高峰。
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引用次数: 0
Coarctation of the aorta and lower limb blood pressure. 主动脉缩窄和下肢血压升高。
Pub Date : 1989-08-01
P B Colditz, R Glover, S G Cooper
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引用次数: 0
Traumatic rupture of the posterior urethra. 外伤性后尿道破裂。
Pub Date : 1989-06-01
S W Beasley, H L Tan, J H Kelly
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引用次数: 0
Urinary calculi in children in Western Australia: 1972-86. 西澳大利亚州儿童尿路结石:1972-86。
Pub Date : 1989-04-01
T W Jones, T R Henderson

Records of all children presenting with urinary calculi in the period 1972-86 were reviewed in order to detail clinical features, laboratory and radiographic findings and treatment. Of a total of 85 children, 59 were Aboriginal and 26 were Caucasian. The features of urolithiasis differed between these groups. In the Aboriginal patients, calculi consisted mainly of uric acid and urates. Important clinical characteristics of this group included a young age at presentation (median = 2.1 years) and frequent presentation with failure to thrive. Calculi were commonly located in the upper urinary tract and most required surgical removal. Documented sequelae included renal scarring and hypertension. Caucasian children presented at a later age (median = 10.5 years), frequently with abdominal pain, and most calculi were associated with an underlying urological or metabolic abnormality.

本文回顾了1972- 1986年间所有泌尿系结石患儿的临床特征、实验室和影像学检查结果以及治疗方法。在总共85名儿童中,59名是土著居民,26名是高加索人。尿石症的特征在这两组之间有所不同。在原住民患者中,结石主要由尿酸和尿酸盐组成。该组的重要临床特征包括发病年龄小(中位年龄为2.1岁)和频繁出现发育不全。结石常见于上尿路,多数需要手术切除。记录的后遗症包括肾瘢痕和高血压。白种人患儿发病年龄较晚(中位年龄10.5岁),常伴有腹痛,多数结石伴有潜在的泌尿系统或代谢异常。
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引用次数: 0
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Australian paediatric journal
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