身患罕见基因病 With a rare genetic disease

16岁男孩 只有两颗“獠牙” 16 years old boy only two stars"tusks"

　　每个人都有牙齿，但对16岁的张成(化名)来说，从小到大，他只有上颚部分有两颗牙，看上去更像长着一对獠牙，这让他很自卑又易怒。12月18日，在母亲的陪同下，他来到三军医大西南医院求助。经医生检查，发现张成患有一种罕见的基因病。

Everyone has teeth,But for 16 years old r. vishny(alias)for,size,He is only part of the palate has two teeth,Looks more like a pair of long tusks,This makes him very self-abased and irritability.12 months and days,In the mother, accompanied by,He came to China three military hospital for help.The doctor check,R. vishny found with a rare genetic disease.

从小到大只长了两颗牙 Since the childhood got only two teeth

　　张成是贵州铜仁地区思南县人，从生下来就似乎得了一种怪病，身上毛发稀少，面部发育也不好。更奇怪的是，别人家的孩子都长牙吃东西了，他却一直长不出牙齿，最后只在上颚部分冒出两颗比较突出且对称的牙齿，看起来就像一对獠牙。

R. vishny is guizhou tongren area SiNaXian people,From birth seems to got a strange disease,Body hair rare,Facial development well.More strange is,Other people's children are long tooth eat,He has been a long teeth,Only the last part in palate from two a more prominent and symmetric teeth,Look like a pair of tusks.

　　“儿子小时候得过几次重病，差点没挺过去。”张成的母亲袁进珍说，她与丈夫都很正常，不明白儿子怎么会这样，心里一直觉得亏欠。

"When the son had several severe illnesses,Almost didn't quite in the past."R. vishny mother YuanJin Jane said,She and her husband are very normal,Don't understand son how,The heart has been think owes.

　　袁进珍也带儿子辗转去了不少医院检查，医生都说这个孩子的病很复杂，如果要进行治疗，不仅要好几万元，而且要等他成年以后才适合做手术。

YuanJin Jane also take my son to the hospital after a check,The doctor said that the child's illness was very complicated,If you want to treatment,Not only to several ten thousand yuan,And to wait for his adult before surgery.

　　但让袁进珍担忧的是，随着年龄的增大，张成的性格越来越孤僻。特别是上学后，“我最害怕的就是老师给我打电话，说他在学校打架了。”袁进珍说，很多同学甚至是路人，稍微多看他两眼，有时都会让他愤怒。

But let YuanJin Jane concern is,With the increase of age,R. vishny character more and more withdrawn.Especially after school,"I fear most is the teacher gave me a call,Said he in the school into a fight."YuanJin Jane said,Many students even passers-by,A little bit see his eyes,Sometimes will let him angry.

是一种遗传性综合性疾病 Is a genetic comprehensive disease

　　在母亲陪同下，张成来到西南医院口腔颌面外科就诊。经医生检查，张成被确诊为无汗型外胚层发育不良。

In accompanied by mother,R. vishny came to southwest hospital oral cavity jaw face surgery clinic.The doctor check,R. vishny are identified as no sweat type ectoderm dysplasia.

　　“这是一种遗传性综合性疾病，多以X染色体连锁隐性方式遗传。”该院口腔颌面外科汪昆副教授说，这个病可能是母亲遗传给他的，但他母亲只是作为基因携带者，并无病状。

"This is a kind of hereditary comprehensive disease,With the X chromosome chain recessive genetic way."Oral cavity jaw face surgery hospital WangKun associate professor said,This disease may be passed to his mother,But his mother just as gene carriers,And no symptom.

　　据介绍，该病的发病率不到十万分之一，是一种较为罕见和严重的基因病，多见于男性。如果患上这种病，其乳牙或恒牙会先天性缺失，还可能伴随泪腺发育障碍、视光敏感、视力下降、听力障碍、唇腭裂、发音困难、身材矮小、乳房发育不良等症状。以西南医院为例，每年都会遇见一两例这样的患者，但像张成这样严重的，非常少见。

According to introducing,The incidence of the disease is less than one over one hundred thousand,Is a kind of relatively rare and serious genetic disease,More common in men.If suffering from the disease,The baby teeth or permanent teeth will be congenital deficiencies,May also with lachrymal gland development obstacle/Visual light sensitive/Vision loss/Hearing impairment/Cleft lip and palate/Pronunciation difficulties/Short stature/The symptom such as breast dysplasia.In southwest hospital as an example,Each year, met a two cases of such patients,But like r. vishny so serious,Very rare.

　　“以前的研究不充分，口腔种植牙技术也相对落后，很多医生都认为有这个病的患者，最好通过保守治疗，直到成年以后才适合进行种植牙安装、面部矫正。”汪昆说，但现在，临床多认为应早期介入进行手术，不仅有利于身体发育，对患者心理健康也有帮助。

"Previous research insufficiency,Oral dental implant technology is relatively backward,Many doctors think there is the disease of the patients,Best by conservative treatment,Until adulthood, just for a dental implant installation/Facial correction."WangKun said,But now,Clinical thought that should be early intervention for surgery,Is not only beneficial to body development,Mental health of patients may also help.

　　为此，汪昆为其拟定了种植牙方案，分批为张成安装种植牙，并进行面部矫正。预计10天后，张成将装上一部分牙齿。(记者 李珩)

therefore,WangKun worked for its growing dental plan,Batch for r. vishny installation dental implants,And facial correction.After ten days is expected to,R. vishny will be installed on the part of the teeth.(Reporter LiHang)