Clinicopathological Correlation of Portal Hypertension in Children and Management Strategies

Asmita Mahajan, R. Ghildiyal, Prachi S. Karnik
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引用次数: 4

Abstract

We studied 51 children with clinical setting of portal hypertension over a period of one and half years. Out of these, 27 (52.94%) were males and 24 (47.05%) were females. All the cases were subjected to biochemical, diagnostic, radiological investigations and liver biopsy. Portal hypertension was diagnosed by demonstrating on USG abdomen/ Doppler, the presence of collaterals of portal vein and esophageal varices on endoscopy. Treatment was initiated depending upon clinical presentation. The most common presenting complaints were hematemesis and malena (35.3%) followed by their combination with abdominal distension (19.6%). 86.3% patients had anemia mostly due to upper GI bleed. 35.3% patients had leucopenia while 47.1% had thrombocytopenia. Only 18 (i.e. 35.3%) had Hypersplenism. Maximum patients who bled more than twice had a mild (41.2%) or severe (29.4%) derangement of prothrombin time. The etiology of portal hypertension turned out to be extra- hepatic portal vein obstruction in 86.3% cases. There were 2 cases each (3.9% each) of Wilson’s disease and Budd-Chiari syndrome. One (2%) was secondary to chronic liver disease due to hepatitis C infection, one was extra- hepatic biliary atresia and one was autoimmune hepatitis. 9.8% cases required drugs in the form of somatostatin drip and all these required a packed cell transfusion as well. 23 patients (45.1%) required only blood transfusion without a somatostatin infusion. Those with severe acute bleeding usually were given somatostatin infusion. The variceal size at presentation was a very important predictor of the morbidity and outcome.
儿童门静脉高压症的临床病理相关性及治疗策略
我们在一年半的时间里研究了51名临床表现为门静脉高压症的儿童。其中,男性27例(52.94%),女性24例(47.05%)。所有病例均行生化、诊断、影像学检查及肝活检。通过USG腹部/多普勒显示门静脉高压,内窥镜显示门静脉侧支和食管静脉曲张的存在。根据临床表现开始治疗。最常见的主诉是呕血和小便(35.3%),其次是合并腹胀(19.6%)。86.3%的患者出现贫血,以上消化道出血为主。35.3%的患者有白细胞减少,47.1%的患者有血小板减少。只有18例(即35.3%)患有脾功能亢进。最多出血两次以上的患者有轻度(41.2%)或重度(29.4%)凝血酶原时间紊乱。门静脉高压症的病因为肝外门静脉阻塞,占86.3%。Wilson病和Budd-Chiari综合征各2例,各占3.9%。1例(2%)继发于丙型肝炎感染引起的慢性肝病,1例为肝外胆道闭锁,1例为自身免疫性肝炎。9.8%的病例需要以生长抑素滴注的形式使用药物,所有这些病例都需要填充细胞输注。23例患者(45.1%)只需要输血而不需要注射生长抑素。严重急性出血患者通常给予生长抑素输注。发病时的静脉曲张大小是预测发病率和预后的重要指标。
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