Management of intractable pruritus in a child with cholestatic liver disorder

N. Adjei, Juliet Ankomah
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Abstract

 BackgroundChildhood cholestatic hepatobiliary disorders present with pruritus which is bothersome and difficult to manage. The pathogenesis of pruritus in cholestasis is unknown but several hypotheses have been proposed, including bile acid accumulation, increased opioidergic tone and elevations in lysophosphatidic acid levels. Pruritus can have a profound effect on the patient’s quality of life resulting in sleep deprivation and emotional disturbances. Treatment of the primary cause of cholestasis is important to address the pruritus. However, symptomatic treatment is often necessary especially in cases where the underlying hepatobiliary disease cannot be corrected. Case Description Currently, 19-month-old male was first seen at the gastroenterology clinic at age of 5 months. He was referred as a case of prolonged conjugated hyperbilirubinaemia. He was born at term with birth-weight (BW) of 2kg. He had a history of jaundice for 2 weeks. There was no pale stools or dark urine and has doubled his BW. On examination, he was jaundiced, had no dysmorphic features, but developed the typical facies of Alagille syndrome over time. Investigations showed conjugated hyperbilirubinemia with raised ALP, GGT and transaminases. A liver biopsy showed severe paucity of bile ducts in 12 portal areas. 2 weeks after the initial visit he presented with severe generalized itching associated with bleeding upon scratching, irritability and poor sleep. A presumptive diagnosis of cholestasis-associated pruritus was made after any possible dermatologic disorder associated with pruritus were ruled out. Ursodeoxycholic acid was started with no improvement even at a maximum dose of the medication. Phenobarbitone and rifampicin were added in a stepwise manner which improved pruritus, but there were challenges with obtaining rifampicin after 9 months of treatment hence cholestyramine was added to the ursodeoxycholic acid and phenobarbital, but the child’s pruritus worsened. Though clear instructions were given to the mother regarding administration of cholestyramine we thought the worsening of the child’s pruritus was still possibly due to chelation of the other medications by cholestyramine, hence it was suspended. An antihistamine, cetirizine was added to child’s treatment and his mother reported some improvement in pruritus but this was short-lived. Finally parenteral infusion of naloxone, an opioid antagonist, was administered over 24 hours with significant improvement in child’s symptoms like scratching, irritability and sleeplessness. Child is currently on oral ursodeoxycholic acid, natrelxone, phenobarbitone and cetirizine with continued remarkable response. Conclusion Pharmacologic treatment with recommended medications in a stepwise approach is essential for symptomatic relief of the pruritus.
胆汁淤积性肝病患儿顽固性瘙痒的治疗
背景:儿童胆汁淤积性肝胆疾病常伴有瘙痒,这是一种麻烦且难以治疗的疾病。胆汁淤积引起瘙痒的发病机制尚不清楚,但提出了几种假说,包括胆汁酸积聚、阿片能张力增加和溶血磷脂酸水平升高。瘙痒症会对患者的生活质量产生深远的影响,导致睡眠剥夺和情绪紊乱。治疗胆汁淤积的主要原因对解决瘙痒很重要。然而,对症治疗往往是必要的,特别是在潜在的肝胆疾病不能纠正的情况下。病例描述目前,19个月大的男性在5个月大时首次在胃肠病学诊所被发现。他被认为是一例长期结合性高胆红素血症。他足月出生,出生体重为2公斤。他有两周的黄疸病史。大便颜色没有变浅,尿液颜色也没有变深,体重增加了一倍。在检查中,他有黄疸,没有畸形特征,但随着时间的推移发展出典型的阿拉吉尔综合征。调查显示结合性高胆红素血症伴ALP、GGT和转氨酶升高。肝活检显示12个门静脉胆管严重缺乏。初次就诊后2周,患者出现严重全身瘙痒,伴抓挠出血、易怒和睡眠不佳。在排除任何可能与瘙痒相关的皮肤病后,推定诊断为胆汁淤积相关的瘙痒。开始使用熊去氧胆酸,即使在最大剂量下也没有改善。逐步加入苯巴比妥和利福平,瘙痒症状得到改善,但治疗9个月后难以获得利福平,于是在熊去氧胆酸和苯巴比妥的基础上加入消胆胺,但患儿瘙痒症状恶化。虽然已明确指示母亲服用胆甾胺,但我们认为孩子瘙痒的恶化仍然可能是由于胆甾胺与其他药物的螯合作用,因此暂停了治疗。一种抗组胺药西替利嗪被添加到孩子的治疗中,他的母亲报告瘙痒有所改善,但这是短暂的。最后静脉注射纳洛酮(一种阿片类拮抗剂)24小时后,孩子的抓伤、易怒和失眠等症状得到了显著改善。儿童目前口服熊去氧胆酸、纳曲酮、苯巴比妥和西替利嗪,疗效持续显著。结论采用推荐药物逐步进行药物治疗是缓解瘙痒症状的关键。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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