小儿肾母细胞瘤化学治疗合并肝窦阻塞综合征4例临床分析

目的 总结小儿肾母细胞瘤化学治疗期间合并肝窦阻塞综合征(SOS)的临床特点,以提高对该病的认识.方法 对于2017年8月-2020年8月,在我院行化学治疗的4例肾母细胞瘤患儿合并SOS的临床表现、实验室检查、腹部超声检查、治疗及预后等情况进行回顾性分析.结果 4例患儿均出现腹胀、肝脏肿大、肝脏弹性值增大、脾脏大、腹水、...     

儿童肝窦阻塞综合征1例

6岁患儿主诉“腹部膨隆2个月”,表现为大量腹腔积液、间断右上腹痛。腹腔积液化验示漏出液、门脉高压性。CT示肝密度不均,肝静脉未显影,下腔静脉肝内段狭窄。病理示肝窦淤血、扩张,内见纤维素样物质。依据临床、CT表现及相关检查除外感染、代谢、免疫、肿瘤性疾病,结合患儿“一点红”服用史,诊断吡咯烷生物碱所致的肝窦阻塞综合征。予...     

儿童布加氏综合征2例报告

布加氏综合征,现又称肝-腔静脉阻塞综合征,多见于成人,在儿童极少见。本院儿科自1988年2月以来连续收治2例,报告如下。例1.男,9岁,因腹胀、腹壁及胸壁静脉曲张4个月,下肢水肿3个月,黄疸2个月入院。病后不发热,腹水量多,在院外抽腹水6次,每次约3000ml。查体:慢性病容,不能平卧,巩膜及皮肤中度黄染,胸壁及腹壁静脉曲张明显。腹壁静脉不以脐为中心,多分布在腹壁两侧,腰背部亦见静脉曲张。曲张静脉     

新生儿肝衰竭1例

患儿男,足月儿,28日龄,因发现皮肤黄染20 d、腹胀15 d入院。患儿生后1周起病,以皮肤黄染、肝脾大、大量腹水、肝功能异常进行性加重至肝衰竭、严重凝血功能障碍、血小板减少为主要表现。给予抗感染、限液利尿、保肝利胆、间断放腹水、换血,以及静脉注射免疫球蛋白、白蛋白、血浆等多种血制品治疗,病情无好转,入院第24天家属决定放弃治疗行临终关怀。患儿肝组织和父母血家系全外显子组测序未找到可以解释患儿肝衰竭的致病变异,最终尸体解剖肝组织病理提示先天性肝纤维化(congenital hepatic fibrosis, CHF)。鉴于CHF导致新生儿肝衰竭罕见,今后仍需对CHF病例的转归及其致病基因进一步研究。该文对新生儿肝衰竭的鉴别诊断进行重点描述,并介绍其多学科诊疗思路。[中国当代儿科杂志,2024,26 (2):213-218,V]     

放线菌素D治疗肾母细胞瘤致肝窦阻塞综合征1例并文献复习

目的了解肾母细胞瘤(WT)在辅助化疗中发生肝窦阻塞综合征/肝小静脉闭塞病(SOS/VOD)的情况。方法回顾性分析我科收治的1例Ⅱ期WT患儿,应用放线菌素D(Act-D)化疗后出现SOS/VOD的临床资料,并复习相关文献。结果 WT患儿术后化疗d5出现进行性血小板减少,化疗d9逐渐出现发热、肝大、腹水、黄疸,符合SOS/VOD的临床诊断,予暂停化疗、血浆置换、抗感染、对症支持等综合治疗,d22痊愈。结论 Act-D治疗WT患儿有一定几率引发SOS/VOD,早期出现孤立性血小板减少可作为SOS/VOD即将发生的警示。     

婴儿及儿童期门静脉高压症的外科处理

自1949年至1972年7月,某儿童医院共收治59例门脉高压并发食管静脉曲张患儿。肝外型47例,其中13例(28％)有新生儿脐炎史,3例(6％)有新生儿腹泻及发烧史,其余2/3病例无明显原因可查。肝外型组中均无脐静脉插管史。肝内型12例,其中10例继发于肝硬化,2例为肝脏纤维化。全组病例多在生后1年内出现症状,主要为肝脾肿大及腹水,以腹水为首发症状者11例(19％)。在2～6岁之前,消化道出血并非主要症状。外科处理:38例患儿作了60次手术,其中姑息性手术36次,根治性手术24次。     

肝窦阻塞综合征12例临床分析

目的 总结肝窦阻塞综合征(SOS)的临床特点,以提高对该病的认识。方法 回顾性分析12 例SOS患儿的临床表现、实验室检查、影像学表现、治疗及预后等情况。结果 12 例患儿中,8 例患儿原发病为白血病,4 例为造血干细胞移植患儿。临床表现主要有腹胀(4例)、肝肿大伴触痛(9例)、脾大(6例)、体重增加(10例)。12 例患儿均有不同程度的肝损害;凝血功能检测中活化部分凝血活酶时间延长7 例;7 例患儿行血D-二聚体检查,其中4 例升高。血常规检测中,4 例白细胞、粒细胞均降低;9 例有不同程度的血小板降低。8 例患儿行彩色多普勒超声检查,可见弥漫性肝肿大、实质非均质改变,肝静脉变细、显示不清或胸腹水、脾大。2 例行肝活检,示肝窦内细胞浸润或肝窦扩张。治疗以对症支持为主,均预后良好。结论 白血病及干细胞移植患儿治疗后如果发生肝肿大、体重突然增加、肝功能异常、凝血功能异常等表现,需警惕SOS 的发生。     

小儿Budd-Chiari综合症并肝功能衰竭1例

1病例资料患儿,男,3岁,因“腹胀、少尿17d”入院。体查:神志淡漠,腹膨隆,呈蛙腹,肝脾肋下未触及,叩诊呈浊音,肠鸣音弱,移动性浊音( )。B超显示:肝、脾肿大,大量腹腔积液,左肾轻度积水。MRI检查显示:肝段下腔静脉(HIVC)部分狭窄,肝静脉(HV)显示不清。血管造影显示:HIVC部分狭窄和HV右支完全阻塞,确诊为Budd-Chiari综合症(BCS)。入院后予中心吸氧,低盐低脂、高蛋白、高热量半流饮食,补充白蛋白,利尿及纠正水电解质、酸碱平衡紊乱等,家长放弃治疗,随访2个月后因肝功能衰竭,病情恶化而死亡。2讨论BCS为HV和/或HIVC部分或完全阻塞引…     

以溶血性贫血为首发表现的肝豆状核变性伴鱼鳞癣1例报告

目的探讨以溶血性贫血为首发表现的肝豆状核变性(HLD)的临床特点及HLD与鱼鳞癣之间的关系。方法回顾分析1例以溶血性贫血为首发表现的肝豆状核变性伴鱼鳞癣患儿的临床表现、发病经过及诊断和治疗过程。结果患儿,女,10岁,临床以乏力、黄疸为首发症状,入院5 d后出现腹胀,进行性加重。网织红细胞8.5%,Coombs试验阴性,角膜出现K-F环,全腹CT平扫示胸腔及腹腔积液,肝脏异常物质沉积,脾大。ATP 7 B及TGM 1基因突变,均位于第14对染色体上。确诊肝豆状核变性合并鱼鳞癣。给予驱铜、阻止铜吸收及口服维A酸等对症支持治疗,病情缓解。结论以溶血性贫血首发的HLD,且合并鱼鳞癣极其少见。     

Alagille 综合征合并肝细胞癌1 例报告并文献复习

目的探讨儿童Alagille综合征合并肝细胞癌的临床表现、影像学检查、治疗及预后。方法分析1例Alagille综合征合并肝细胞癌患儿的临床表现、辅助检查、诊断,并复习相关文献。结果患儿,女,6岁,因反复皮肤黄染入院,曾有心脏手术史。入院后查体有特殊面容(前额突出、眼眶深陷、尖下颌、鼻前端肥大)。血生化提示存在肝内胆汁淤积,甲胎蛋白升高;影像学腹部B超示肝内弥漫性多发实性占位,肝脏磁共振提示肝大,肝脏多发占位。Jagged 1基因检测为c.1205delC,杂合型。结论儿童期Alagllie综合征合并肝细胞癌极为罕见,早期诊断和长期随访对治疗及预后具有积极意义。     

Congenital Budd-Chiari Syndrome

Budd-Chiari syndrome, due to thrombotic occlusion of hepatic veins or ostia, is unusual in infants. Review of the literature reveals no documented case symptomatic at birth. We describe a male infant with ascites present at birth. A liver biopsy at 7 weeks of age showed sinusoidal dilatation, but the diagnosis of Budd-Chiari syndrome was made only at autopsy, 4 months later. The cause was not determined although maternal drug abuse was suspected.     

Combined Esophageal and Pyloric Atresia without Tracheoesophageal Fistula in Down's Syndrome

This is the first report of a newborn infant with Down's syndrome associated with combined congenital pyloric and esophageal atresias without a tracheoesophageal fistula (Gross A type). A mother was noted to have marked abdominal distension and hydramios was suspected at 28 weeks gestation. Ultrasonographic findings of her abdomen demonstrated that the fetus was suspected of having congenital duodenal and esophageal atresia, from the large doubleecho-free spaces occupying the greater part of the abdomen. Chromosome analysis of the amniotic fluid showed a karyotype of 21 trisomy. The newborn baby had clinical characteristics of Down's syndrome and severe abdominal distension after birth. At 19 days after birth, autopsy revealed pyloric and esophageal atresias without trache oesophageal fistula. We conclude that ultrasonographic examination is useful for the prenatal diagnosis of a patient with gastrointestinal obstruction combined with esophageal atresias, without trache oesophageal fistula.     

Posttraumatic hepatic vein thrombosis (Budd-Chiari syndrome) in a child

The Budd-Chiari syndrome (BCS) results from hepatic venous outflow obstruction and is characterized by massive ascites and painful hepatomegaly. It is a rare condition, particularly in children, and is often fatal without surgical intervention. Although several conditions resulting in a hypercoagulable state appear to predispose individuals to developing the disorder in adulthood, the etiology in children is often obscure. We present the case of a 6-year-old girl treated nonoperatively for blunt abdominal trauma and hemoperitoneum. Two weeks following her injury, she developed an acute BCS with rapid liver failure, ascites, and abdominal pain. The diagnosis of hepatic vein thrombosis was confirmed radiographically and the patient underwent a C-type mesocaval shunt procedure. The etiology, diagnosis, and surgical management of BCS in children and a review of the literature are discussed.     

Neonatal total parenteral nutrition ascites from liver erosion by umbilical vein catheters

Background. A preterm infant was found to have total parenteral nutrition (TPN) ascites after infusion through a low umbilical vein catheter (UVC). Objective. To evaluate the clinical and imaging findings of neonates with TPN ascites after infusion through UVCs. Materials and methods. Eight patients with TPN ascites were identified over three years. Charts were abstracted for clinical data. Plain-film, ultrasound (US), and contrast studies through the UVCs were examined to determine UVC placement, presence of liver injury, and confirmation of intraperitoneal extravasation from the UVC. Results. All eight patients with TPN ascites presented with hypotension and abdominal distension. All had UVCs overlying the liver on plain film. Catheters were in place a mean of 8.9 days prior to TPN extravasation. US in four patients showed hepatic parenchymal damage around the UVC tip. Contrast studies in six patients showed intraperitoneal spill. Conclusion. While low UVC placement may sometimes be clinically unavoidable, TPN administered through abnormally positioned UVCs is not without risk. Received: 26 February 1998 Accepted: 29 June 1998     

Pathogenesis of the prune-belly syndrome: a functional urethral obstruction caused by prostatic hypoplasia

Abdominal muscle deficiency, urinary tract abnormalities, and cryptorchidism are the three major features of the prune-belly syndrome, also referred to as triad syndrome or Eagle-Barrett syndrome. The etiology is unclear and the pathogenesis a subject of continuing debate. Clinical and pathologic experience with seven cases of prune-belly syndrome is reviewed. Findings indicate that the urogenital anomalies can be attributed to a functional urethral obstruction which in turn is the result of prostatic hypoplasia. The histology of the abdominal wall is that of atrophy-ie, the degeneration of already formed muscle--and not of primitive muscle. This observation supports the theory that the abdominal muscle hypoplasia is a nonspecific lesion, resulting from fetal abdominal distension of various causes. Transient fetal ascites may be an important feature of the prune-belly syndrome.     

Early graft failure due to a veno-occlusive disease after a pediatric living donor liver transplantation

A 10-month-old boy with biliary atresia after Kasai procedure underwent a living donor liver transplantation (LDLT). Five days after the LDLT, high fever and increased ascites followed by poor bile drainage was accompanied by elevation of serum liver enzymes. Liver biopsy showed occlusion of the central veins by fibro-edematous endothelium and submassive necrosis of the parenchyma. Veno-occlusive disease (VOD) was suspected, and re-LDLT was urgently performed because of deterioration of hepatic failure. There are few cases of VOD after liver transplantation and this is the first one in an infant after LDLT.     

Chylous ascites: total parenteral nutrition as primary therapeutic modality

A female infant with Down syndrome and congenital chylous ascites presented at birth with respiratory distress secondary to marked abdominal distension. Total parenteral nutrition (TPN) and paracentesis were the primary therapeutic modality. On hyperalimentation, however, ascites initially recurred, requiring additional paracenteses to improve respiratory distress. The chylous ascites, lymphopenia and hypoalbuminemia were relieved after 10 weeks of TPN administration. We recommend a long-term course (10 weeks) of TPN before an exploratory laparatomy and possible surgical intervention are considered.     

Chylous ascites in infants and children: a case report and literature review

Successful treatment of an infant with chylous ascites secondary to "congenital leaky lymphatics" is described, followed by a literature review of cases of pediatric chylous ascites. The infant was placed on home total parenteral nutrition for 10 weeks, during which time the chylous ascites resolved and did not recur with the introduction of a normal diet. Forty cases of pediatric chylous ascites have been reported since 1960, with an age range from birth to 18 years. The presenting signs and symptoms were secondary to abdominal distension in over 80% of cases. The chylous ascites was secondary to trauma, which included child abuse (19%), obstruction (27%), or lymphatic abnormalities (54%). Most studies described were not helpful in making the diagnosis, except for lymphangiography and exploratory laparotomy. Many patients were treated with a high-protein, low-fat diet, with or without medium-chain triglycerides, with variable success. Total parenteral nutrition (TPN) was the primary therapeutic modality in five patients, and in all but one of the cases there was complete resolution of the chylous ascites over 3 to 10 weeks with no significant side effects. Total parenteral nutrition, including home TPN, is a safe and effective therapeutic modality for some cases of chylous ascites.     

Respiratory Distress and Abdominal Distension in an 8-Month-Old

An 8-month-old presented to a community hospital with respiratory distress, progressive abdominal distension, and emesis in the setting of fever and significant irritability. His illness course eventually progressed to requiring intubation and bedside exploratory laparotomy. He was subsequently diagnosed with abdominal compartment syndrome secondary to methicillin-resistant Staphylococcus aureus sepsis. The differential for abdominal distension in this age group is extensive, and the eventual diagnosis offers valuable teaching points in the management of the sick infant with abdominal distension.