患有胶原性皮肤病的新生儿

Elsita Lisnawati, Fitriana Yusiyanti Dewi, Wuri Puspita Rani
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引用次数: 0

摘要

Collodion Baby(胶版婴儿)是一个描述性术语,指新生儿的整个身体表面都被一层淡黄色的硬膜包裹着,就像一张坚实而半透明的羊皮纸。这是一种罕见的疾病,通常发生在出生体重不足的早产儿身上。由于基因突变,患儿的蛋白质和脂肪代谢可能存在缺陷。处理方法包括使用润肤剂、保持体液和电解质平衡以及克服感染。病例报告 :据报告,一名新生儿因患有柯洛迪翁症和出生体重过轻而从 Puskesmas 转诊到 Soeselo 医院急诊室。全身皮肤呈黄色,如半透明的厚羊皮纸,有一些糜烂的裂口,双眼有糜烂。患者被安置在温度为 30-32 摄氏度的保温箱中,接受润肤治疗,在皮肤裂口和糜烂处局部使用抗生素,预防性使用抗生素,外翻时使用抗生素药膏。讨论:胶原婴儿有可能出现严重并发症,包括:感染风险、高钠血症性脱水、体液和电解质失衡,以及表皮脂质紊乱和蛋白质平衡失调导致的体温不稳定,造成经表皮失水(TEWL)增加,其原因是负责表皮平衡的转谷氨酰胺酶 1-TGM1、ALOXE3 或 ALOX12B、ABCA12、HIPAL4/鱼鳞素、ABHD5 基因发生突变。结论角膜塑形镜宝宝是一种严重损伤角膜的疾病。为了减少并发症,必须进行适当和全面的治疗,以降低发病率和死亡率。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Newborns with Collodion Skin Disorders
Collodion Baby is a descriptive term for newborns with the entire surface of the body wrapped in a rigid yellowish membrane that resembles solid and translucent parchment paper. A rare disorder that often occurs in preterm babies with low birth weight. Collodion babies can have defects in protein and fat metabolism due to genetic mutations. Management with emollients, fluid and electrolyte balance and overcoming infection. Case Report : Reported newborn came to the emergency room of Soeselo Hospital referral from Puskesmas with Collodion Baby and low birth weight. Yellowish skin is obtained throughout the body such as translucent thick parchment, fissures with some erosion and ecropions in both eyes. The patient is placed in an incubator with a temperature of 30-32o C, emollient therapy, topical antibiotics on fissures and erosions, prophylactic antibiotics and antibiotic creams for ectropion. Discussion : Collodion baby is a condition at risk of serious complications including: risk of infection, hypernatremic dehydration, fluid and electrolyte imbalances and temperature instability caused by epidermal lipid disorders and protein homeostasis, resulting in increased transepidermal water loss (TEWL) caused by mutations in the transglutaminase 1-TGM1, ALOXE3 or ALOX12B, ABCA12, HIPAL4/ichthyin, ABHD5 genes responsible for epidermal homeostasis. Conclusion: Collodion Baby is a disorder with severe corneal damage. The importance of proper and comprehensive management with the aim of minimizing complications can reduce morbidity and mortality.
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