Congenital aplasia of parathyroid glands and thymus.

Congenital aplasia of parathyroid glands and thymus.
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先天性甲状旁腺和胸腺发育不全。

DOI:
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发表时间:
1967
影响因子:
5.2
通讯作者:
andH . E. Zoethout
andH . E. Zoethout
中科院分区:
医学2区
文献类型:
--
作者:
J. Huber;P. Cholnoky;andH . E. Zoethout

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先天性甲状旁腺缺失可能与先天性胸腺缺失有关(Lobdell,1959),这并不奇怪,因为这两种结构都起源于第3和第4鳃囊。同样,第3和第4鳃动脉的血管异常也与胸腺异常有关,包括胸腺发育不全(卡梅隆,1965)。据描述,一名患者患有胸腺发育不全、甲状旁腺缺失和先天性心脏病。这个案例的有趣之处在于,这种“自然实验”可能会影响人体的免疫系统。4100g儿童病例报告,正常妊娠后剖腹产,出生后因持续性重度紫绀入院。临床、心电图和放射学检查结果证实诊断为重度肺动脉瓣狭窄,可能为肺动脉闭锁,在3个月大时通过心血管造影证实,当时的结果与法洛四联症伴重度漏斗部狭窄一致。
Congenital absence oftheparathyroid glands may beassociated withcongenital absence ofthethymus (Lobdell, 1959).Thisisnotsurprising asboth structures arise together fromthe3rdand4th branchial pouches. Inthesameway,vascular anomalies ofthe3rdand4thbranchial arteries are foundtobeassociated withabnormalities ofthe thymus, including thymic aplasia (Cameron, 1965). A patient isdescribed inwhomthymic aplasia, absence oftheparathyroid glands, andcongenital heart disease werepresent. Theinterest ofthis case lies inthelight this 'experiment ofnature' mayshed ontheimmunological systems ofthebody. CaseReport Amalechild of4100g.,delivered bycaesarean section after normal pregnancy, wasadmitted afewhoursafter birth because ofpersistent severe cyanosis present from birth. Clinical, ECG,andradiological findings suggesteda diagnosis ofsevere pulmonary stenosis, possibly pulmonary atresia, andthis wasconfirmed attheageof3 monthsbyangiocardiography, whenthefindings were consistent withFallot's tetralogy withsevere infundibular