Lethargy in a patient with cirrhosis.
Lethargy in a patient with cirrhosis.
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肝硬化患者嗜睡。
DOI：
10.1136/pgmj.73.857.177
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发表时间：
1997
影响因子：
5.1
通讯作者：
K. Goh
中科院分区：
文献类型：
--
作者：
S. Chuah;N. W. Wong;K. Goh
1996年1月12日入院，1983年12月，一位53岁的中国女性出现一年的全身瘙痒病史。她的家族中的许多成员，世代相传，都患有自身免疫性甲状腺疾病。在检查中，她有双侧黄褐斑，但没有慢性肝病的斑点，也没有黄疸。她的肝脏可以触摸到，但她既没有脾肿大，也没有腹水。血清胆红素7例/L，总碱性磷酸酶710IU/l，天冬氨酸氨基转移酶98IU/l，丙氨酸转氨酶108IU/l，白蛋白39g/L，血沉110 mm/h，总胆固醇6.1 mm/dl，血清免疫球蛋白升高至404 mg/dl(正常范围：100~206)，但均在正常范围内。抗核因子和抗平滑肌抗体均为阴性，抗线粒体抗体均为阳性。进行肝脏活检(图1、2)。她开始服用逐渐增加的青霉胺，并在短短六个月多一点的时间里达到了每天600毫克的最大剂量。由于她有很强的自身免疫性甲状腺疾病家族史，她的甲状腺状况被检查了。临床和生化检查甲状腺正常，但抗微粒子抗体和抗甲状腺球蛋白抗体滴度分别为1/1600和1/20。大约在发病一年后，她开始服用氯苯乙胺，因为她的瘙痒持续存在。她的血清胆红素目前已升至30imol/l，但她的碱性磷酸酶、天冬氨酸转氨酶和丙氨酸转氨酶分别为313、115和108IU/l。六个月后，她的瘙痒症状有了显著的改善，但她注意到在六周的时间里，她的双上肢逐渐出现无力，视力模糊。这些症状在临近傍晚时趋于恶化。她抱怨向左看时出现复视，但这在正式测试中并不存在。医生做出了诊断，并对她进行了医疗治疗。她的虚弱逐渐好转，不到一年，她就停止了治疗。在最初诊断的四年后，她出现了嗜睡、心悸和体重在10个月内减轻了约8公斤，尽管食欲正常。1990年1月，她抱怨眼睛发炎。熊去氧胆酸是在她首次出现的五年后开始的。她的血清胆红素为25，uMol/L，总碱性磷酸酶441IU/l，天冬氨酸氨基转移酶114IU/l，丙氨酸转氨酶130IU/l，白蛋白36g/L，总胆固醇10.6mmoL/L。
Accepted 12 January 1996 In December 1983, a 53-year old Chinese woman presented with a one-year history of generalised pruritus. Various members of her family, extending over four generations, had autoimmunne thyroid disorders. On examination, she had bilateral xanthelasmata but no stigmata of chronic liver disease nor jaundice. Her liver was just palpable but she had neither splenomegaly nor ascites. Serum bilirubin was 7 jumol/l, total alkaline phosphatase 710 IU/1, aspartate transaminase 98 IU/1, alanine transaminase 108 IU/1 and albumin 39 g/l. Erythrocyte sedimentation rate was 110 mm/h and her total cholesterol was 6.1 mmol/l. Serum IgM level was elevated at 404 mg/dl (normal range: 100 206) but IgG and IgA levels were both within normal limits. Antinuclear factor and anti-smooth muscle antibody were both negative but antimitochondrial antibody was positive. A liver biopsy was done (figures 1, 2). She was started on a gradually increasing dose of penicillamine and attained a maximum dose of 600 mg/day in just over six months. Because of her strong family history of autoimmune thyroid disorders, her thyroid status was checked. She was clinically and biochemically euthyroid but antimicrosomal antibody and antithyroglobulin were detected at titres of 1 in 1600 and 1 in 20, respectively. About a year after the onset of illness, she was started on cholestyramine as her itching had persisted. Her serum bilirubin had by now risen to 30 imol/ but her alkaline phosphatase, aspartate transaminase and alanine transaminase were 313, 115 and > 108 IU/1, respectively. Six months later, her pruritus had improved remarkably but she noticed gradual onset weakness in both upper limbs over a six-week period and blurring of vision. These symptoms tended to worsen towards the evening. She complained of diplopia on looking to the left but this was not present on formal testing. A diagnosis was made and she was treated medically. Her weakness gradually improved and within a year, she was off treatment. Four years after the initial diagnosis she presented with lethargy, palpitations and weight loss of about 8 kg in 10 months despite a normal appetite. In January 1990 she complained of eye irritation. Ursodeoxycholic acid was started five years after her initial presentation. It has been 12 years since she first presented and she remains relatively well with a serum bilirubin of 25 ,umol/l, total alkaline phosphatase 441 IU/1, aspartate transaminase 114 IU/1, alanine transaminase 130 IU/1, albumin 36 g/l and a total cholesterol of 10.6 mmol/l.