AIM:To study the management and outcome of children with extrahepatic portal vein obstruction(EHPVO) in a whole country population.METHODS:A nationwide multicenter retrospective case series of children with EHPVO was ...AIM:To study the management and outcome of children with extrahepatic portal vein obstruction(EHPVO) in a whole country population.METHODS:A nationwide multicenter retrospective case series of children with EHPVO was conducted.Data on demographics,radiographic studies,laboratory workup,endoscopic and surgical procedures,growth and development,were extracted from the patients' charts.Characteristics of clinical presentation,etiology of EHPVO,management and outcome were analyzed.RESULTS:Thirty patients,13 males and 17 females,19(63.3%) Israeli and 11(36.7%) Palestinians,were included in the analysis.Age at presentation was 4.8± 4.6 years,and mean follow-up was 4.9±4.3 years.Associated anomalies were found in 4 patients.The incidence of EHPVO in Israeli children aged 0-14 years was 0.72/million.Risk factors for EHPVO were detected in 13(43.3%)patients,including 9 patients(30%) with perinatal risk factors,and 4 patients(13.3%) with prothrombotic states:two had low levels of protein S and C,one had lupus anticoagulant,and one was homozygous for methyltetrahydrofolate reductase mutations.In 56.6% of patients,no predisposing factors were found.The most common presenting symptoms were an incidental finding of splenomegaly(43.3%),and upper gastrointestinal bleeding(40%).No differences were found between Israeli and Palestinian children with regard to age at presentation,etiology and clinical symptoms.Bleeding occurred in 18 patients(60%),at a median age of 3 years.Sclerotherapy or esophageal banding was performed in 20 patients.No sclerotherapy complications were reported.Portosystemic shunts were performed in 11 patients(36.6%),at a median age of 11(range 3-17)years:splenorenal in 9,mesocaval in 1,and a meso-Rex shunt in 1 patient.One patient underwent splenectomy due to severe pancytopenia.Patients were followed up for a median of 3(range 0.5-15)years.One patient died aged 3 years due to mucopolysaccharidase deficiency type Ⅲ.None of the patients died due to gastrointestinal bleeding.CONCLUSION:EHPVO is a rare disorder.The etiological factors are still mostly unknown,and the endoscopic and surgical treatment options ensure a good long-term prognosis.展开更多
Inflammatory bowel diseases(IBD), including Crohn's disease(CD) and ulcerative colitis, are chronic relapsing and remitting diseases of the bowel, with an unknown etiology and appear to involve interaction between...Inflammatory bowel diseases(IBD), including Crohn's disease(CD) and ulcerative colitis, are chronic relapsing and remitting diseases of the bowel, with an unknown etiology and appear to involve interaction between genetic susceptibility, environmental factors and the immune system. Although our knowledge and understanding of the pathogenesis and causes of IBD have improved significantly, the incidence in the pediatric population is still rising. In the last decade more drugs and treatment option have become available including 5-aminosalicylate,antibiotics, corticosteroids, immunomodulators and biological agents. Before the use of anti-tumor necrosis factor(TNF)-α became available to patients with IBD, the risk for surgery within five years of diagnosis was very high, however, with anti-TNF-α treatment the risk of surgery has decreased significantly. In the pediatric population a remission in disease can be achieved by exclusive enteral nutrition. Exclusive enteral nutrition also has an important role in the improvement of nutritional status and maintained growth. In this review we summarize the current therapeutic treatments in CD. The progress in the treatment options and the development of new drugs has led to optimized tactics for achieving the primary clinical goals of therapy- induction and maintenance of remission while improving the patient's growth and overall well-being.展开更多
文摘AIM:To study the management and outcome of children with extrahepatic portal vein obstruction(EHPVO) in a whole country population.METHODS:A nationwide multicenter retrospective case series of children with EHPVO was conducted.Data on demographics,radiographic studies,laboratory workup,endoscopic and surgical procedures,growth and development,were extracted from the patients' charts.Characteristics of clinical presentation,etiology of EHPVO,management and outcome were analyzed.RESULTS:Thirty patients,13 males and 17 females,19(63.3%) Israeli and 11(36.7%) Palestinians,were included in the analysis.Age at presentation was 4.8± 4.6 years,and mean follow-up was 4.9±4.3 years.Associated anomalies were found in 4 patients.The incidence of EHPVO in Israeli children aged 0-14 years was 0.72/million.Risk factors for EHPVO were detected in 13(43.3%)patients,including 9 patients(30%) with perinatal risk factors,and 4 patients(13.3%) with prothrombotic states:two had low levels of protein S and C,one had lupus anticoagulant,and one was homozygous for methyltetrahydrofolate reductase mutations.In 56.6% of patients,no predisposing factors were found.The most common presenting symptoms were an incidental finding of splenomegaly(43.3%),and upper gastrointestinal bleeding(40%).No differences were found between Israeli and Palestinian children with regard to age at presentation,etiology and clinical symptoms.Bleeding occurred in 18 patients(60%),at a median age of 3 years.Sclerotherapy or esophageal banding was performed in 20 patients.No sclerotherapy complications were reported.Portosystemic shunts were performed in 11 patients(36.6%),at a median age of 11(range 3-17)years:splenorenal in 9,mesocaval in 1,and a meso-Rex shunt in 1 patient.One patient underwent splenectomy due to severe pancytopenia.Patients were followed up for a median of 3(range 0.5-15)years.One patient died aged 3 years due to mucopolysaccharidase deficiency type Ⅲ.None of the patients died due to gastrointestinal bleeding.CONCLUSION:EHPVO is a rare disorder.The etiological factors are still mostly unknown,and the endoscopic and surgical treatment options ensure a good long-term prognosis.
文摘Inflammatory bowel diseases(IBD), including Crohn's disease(CD) and ulcerative colitis, are chronic relapsing and remitting diseases of the bowel, with an unknown etiology and appear to involve interaction between genetic susceptibility, environmental factors and the immune system. Although our knowledge and understanding of the pathogenesis and causes of IBD have improved significantly, the incidence in the pediatric population is still rising. In the last decade more drugs and treatment option have become available including 5-aminosalicylate,antibiotics, corticosteroids, immunomodulators and biological agents. Before the use of anti-tumor necrosis factor(TNF)-α became available to patients with IBD, the risk for surgery within five years of diagnosis was very high, however, with anti-TNF-α treatment the risk of surgery has decreased significantly. In the pediatric population a remission in disease can be achieved by exclusive enteral nutrition. Exclusive enteral nutrition also has an important role in the improvement of nutritional status and maintained growth. In this review we summarize the current therapeutic treatments in CD. The progress in the treatment options and the development of new drugs has led to optimized tactics for achieving the primary clinical goals of therapy- induction and maintenance of remission while improving the patient's growth and overall well-being.