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患者,男,50岁。头手足增粗增厚15年。经常气促、胸闷、心悸逐渐加剧1年,突然呼吸困难,全身发绀,头晕、昏厥约半小时入院。进院后查体中又出现昏厥一次,约2分钟,经侧卧位后症状自行缓解。查体T36℃,P90次/分,R20次/分,Bp16/11kPa,皮肤粗糙,头脸部皮皱深,额骨、颧骨、眼眶上嵴增粗宽,下颌宽大,耳鼻长大,舌唇粗厚,牙齿疏松,口语音低沉,颈段粗短,甲状腺不大。胸背宽阔,双肺呼吸音清晰,心界向双侧扩大,心尖区闻及第一心音增强,舒张期隆隆样杂音,第二音分裂,第二音后可闻及扑落样低调心音,腹平坦,腹壁软,肝右肋缘下约3cm,质中等,表现光滑,无触压痛,脾未扪及,肠音正常,脊椎粗宽,背部呈伛偻样后凸,外生殖器正常,胸片示左心房增
Patient, male, 50 years old. Head and foot thickening thickened 15 years. Often shortness of breath, chest tightness, heart palpitations gradually aggravate 1 year, sudden breathing difficulties, systemic cyanosis, dizziness, fainting about half an hour admitted. After admission to the body there syncope again, about 2 minutes, the symptoms after the lateral position to ease themselves. Examination T36 ℃, P90 beats / min, R20 beats / min, Bp16 / 11kPa, rough skin, face skin deep wrinkles, frontal bone, cheekbones, orbital crest thickening, wide mandibular, ear and nose growth, tongue Lips thick, loose teeth, voice low voice, stubby neck, thyroid is not large. Chest and back wide, clear breath sounds of both lungs, the heart bound to bilateral enlargement, apex area of the heart and the first heart sound enhancement, diastolic rumbling noise, the second sound split, can be heard after the second tone and flapping down low-key heart sounds , Abdominal flat, abdominal soft, right under the right rib margin about 3cm, medium quality, smooth performance, no tenderness, the spleen is not palpable, normal bowel sounds, wide spine, back was 伛 偻 like kyphosis, external genitalia normal, chest Film shows the left atrium increased