口腔颚裂的长期影响因类型而有不同


  【24drs.com】根据一项在挪威进行之近150万人的人口基础世代研究,在没有任何其它先天性异常下,口腔颚裂新生儿的预后大部份是根据颚裂类型而定。
  
  根据在线发表于9月26日JAMA小儿科期刊的研究报告,在将近30岁时,出生时有唇裂者、不论有无颚裂,死亡风险并未大幅增加,就算有,死亡率也只比无此症的对照组小幅增加。
  
  不过,天生单纯颚裂者就相当坎坷,医疗原因死亡风险是无此症之同龄者的3倍,他们在智力残疾、脑瘫和癫痫等状况的风险也大幅升高。
  
  挪威Bergen大学全球公共卫生暨初级卫生保健系Erik Berg医师等人写道,研究结果对于单独唇裂儿童的家长而言是好消息。相对的,从小对于[单纯颚裂婴幼儿]彻底检查其它潜在疾病是强烈建议的,以确保必要的介入和尽早治疗。
  
  作者们接著表示,他们进一步建议,这个信息应提供给遗传咨询者、颚裂病童的家长、以及参与这些孩童之治疗与追踪的健康照护工作者。
  
  华盛顿大学小儿科、西雅图儿童研究院临床和转化研究中心、西雅图儿童医院颅面中心的Carrie L. Heike医师与Kelly N. Evans医师在编辑评论中写道,目前的人口基础研究为强调连结大数据的潜力、确认相关终点提供了一个重要贡献,且激励持续进行研究,以进行最终目标是为了改善颚裂相关健康照护的质量研究。
  
  他们指出,探讨治疗和整体结果之关联、以及从病患的角度整合信息的进一步研究,将增加这类大规模、人口基础之定量方法,并对成人结果有更全面之理解。
  
  这次的研究中,研究者比较了1967-1992年出生时、单纯口腔颚裂的2,337名患者与1,413,819名没有口腔颚裂者的存活与健康结果, 追踪到2010年到他们18-43岁时。在颚裂组中, 695人只有唇裂、918人有唇裂和颚裂、724只有颚裂。
  
  Berg医师等人报告指出,结果显示,相对于对照组,只有唇裂者这组在青年时的发病率或死亡率病未显著增加。出生时有唇裂和颚裂者,智力残疾(相对风险[RR], 2.2;95%信赖区间[CI], 1.2 - 4.1)和脑瘫(RR, 2.6;95% CI, 1.1 - 6.2)风险中度增加,死亡风险未增加。
  
  不过,只有颚裂者更可能死于医疗原因(风险比3.4;95% CI, 2.1 - 5.7),他们的智力残疾(RR, 11.5;95% CI, 8.5 - 15.6)、焦虑异常(RR, 2.9;95% CI, 1.3 - 6.5)、自闭症(RR, 6.6;95% CI, 2.8 - 15.7)、严重学习障碍(RR, 10.6;95% CI, 5.5 - 20.2)、脑瘫(RR, 4.8;95% CI, 2.3 - 10.0)、癫痫(RR, 4.9;95% CI, 2.2 - 10.8)以及肌肉或骨骼疾病(RR, 2.7;95% CI, 1.4 - 5.4)之风险增加。
  
  资料来源:http://www.24drs.com/
  
  Native link:Long-term Effect of Oral Cleft Depends on Type

Long-term Effect of Oral Cleft Depends on Type

By Susan London
Medscape Medical News

The prognosis for infants born with oral clefts in the absence of any other congenital anomalies largely depends on the type of cleft, according to a population-based cohort study conducted among nearly 1.5 million individuals in Norway.

At approximately 30 years of age, individuals born with a cleft lip, with or without cleft palate, did not have an elevated risk for death and had fairly small increases, if any, in morbidity compared with unaffected counterparts, according to results reported in an article published online September 26 in JAMA Pediatrics.

However, those born with cleft palate alone had a much rockier course, with about three times the risk for death from medical causes relative to that seen in unaffected peers. They also had sharply higher risks for conditions such as intellectual disability, cerebral palsy, and epilepsy.

"The present results are good news for parents of children with isolated cleft lip," write Erik Berg, MD, from the Department of Global Public Health and Primary Care, University of Bergen, Norway, and colleagues. In contrast, "[t]horough screening for other underlying conditions in [infants with cleft palate alone] is highly recommended from a young age to ensure necessary interventions and treatment as early as possible."

The authors continue, "This information should be provided to genetic counselors, parents of children with clefts, and health care workers involved in the treatment or follow-up of these children," they further recommend.

"The current population-based study provides an important contribution, highlights the potential for linking large data sets, identifies relevant end points, and motivates the continued work to conduct quality research with an ultimate goal to improve cleft-related health care," write Carrie L. Heike, MD, and Kelly N. Evans, MD, both from the Craniofacial Center at Seattle Children's Hospital, the Center of Clinical and Translational Research at Seattle Children's Research Institute, and the Department of Pediatrics at the University of Washington, in an accompanying editorial.

"Further investigations to explore the association between treatments and holistic outcomes and to incorporate information from patient perspectives will augment such large scale, population-based quantitative approaches and allow for a more holistic understanding of adult outcomes," they maintain.

For the current study, the investigators compared survival and health outcomes between 2337 individuals with an isolated oral cleft and 1,413,819 patients without an oral cleft born between 1967 and 1992 and followed through 2010, when they were between 18 and 43 years old. In the cleft group, 695 individuals had cleft lip only, 918 had both cleft lip and palate, and 724 had cleft palate only.

Results showed that relative to the unaffected individuals, the group with cleft lip only did not have any significant increase in morbidity or mortality by young adulthood, Dr Berg and colleagues report. Individuals born with both cleft lip and cleft palate had moderately elevated risks for intellectual disability (relative risk [RR], 2.2; 95% confidence interval [CI], 1.2 - 4.1) and cerebral palsy (RR, 2.6; 95% CI, 1.1 - 6.2), and no increase in the risk for death.

However, individuals having cleft palate only were much more likely to have died from medical causes (hazard ratio, 3.4; 95% CI, 2.1 - 5.7). They also had elevated risks for intellectual disability (RR, 11.5; 95% CI, 8.5 - 15.6), anxiety disorders (RR, 2.9; 95% CI, 1.3 - 6.5), autism spectrum disorders (RR, 6.6; 95% CI, 2.8 - 15.7), severe learning disabilities (RR, 10.6; 95% CI, 5.5 - 20.2), cerebral palsy (RR, 4.8; 95% CI, 2.3 - 10.0), epilepsy (RR, 4.9; 95% CI, 2.2 - 10.8), and muscular or skeletal disorders (RR, 2.7; 95% CI, 1.4 - 5.4).

The investigators and editorialists have disclosed no relevant financial relationships.

JAMA Pediatrics. Published online September 26, 2016.

    
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