The Usefulness of Distal Splenorenal Shunt in Children with Portal Hypertension for the Treatment of Severe Thrombocytopenia and Leukopenia

Moon, Sun-Young; Jung, Sung Eun; Ha, Jong Won; Park, Kwi Won; Seo, Jeong Kee; Kim, Woo Ki

doi:10.1007/s00268-007-9356-0

Cited by 21 publications

(19 citation statements)

References 28 publications

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“…Although deficiencies in endothelial prostacyclin synthase [6], excess circulating endothelin-1 [7], and PA platelet aggregates [8] have been documented in POPH patients, the molecular mechanisms driving the pathogenesis of the disease remain unclear. One of the primary reasons that the pathophysiological mechanisms directing POPH is still unknown is that animal, and in particular, mouse models of disease have not been developed.…”

Section: Introductionmentioning

confidence: 99%

Pathological Changes in Pulmonary Circulation in Carbon Tetrachloride (ccl4)-Induced Cirrhotic Mice

et al. 2014

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RationaleLack of an experimental model of portopulmonary hypertension (POPH) has been a major obstacle in understanding of pathophysiological mechanisms underlying the disease.ObjectiveWe investigated the effects of CCl4-mediated cirrhosis on the pulmonary vasculature, as an initial step towards an improved understanding of POPH.Methods And ResultsMale C57BL/6 mice received intraperitoneal injection of either sterile olive oil or CCl4 3 times/week for 12 weeks. Cirrhosis and portal hypertension were confirmed by evidence of bridging fibrosis and nodule formation in CCl4-treated liver determined by trichrome/picrosirius red staining and an increase in spleen weight/body weight ratio, respectively. Staining for the oxidative stress marker, 4-hydroxynonenal (4-HNE), was strong in the liver but was absent in the lung, suggesting that CCl4 did not directly induce oxidative injury in the lung. Pulmonary acceleration time (PAT) and the ratio of PAT/pulmonary ejection time (PET) measured by echocardiography were significantly decreased in cirrhotic mice. Increase in right ventricle (RV) weight/body weight as well as in the weight ratio of RV/(left ventricle + septum) further demonstrated the presence of pathological changes in the pulmonary circulation in these mice. Histological examination revealed that lungs of cirrhotic mice have excessive accumulation of perivascular collagen and thickening of the media of the pulmonary artery.ConclusionCollectively, our data demonstrate that chronic CCl4 treatment induces pathological changes in pulmonary circulation in cirrhotic mice. We propose that this murine cirrhotic model provides an exceptional tool for future studies of the molecular mechanisms mediating pulmonary vascular diseases associated with cirrhosis and for evaluation of novel therapeutic interventions.

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Section: Introductionmentioning

confidence: 99%

Pathological Changes in Pulmonary Circulation in Carbon Tetrachloride (ccl4)-Induced Cirrhotic Mice

et al. 2014

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“…Previously Moon et al [25] reported the result of distal spleno-renal shunt (DSRS, Warren shunt) in 15 patients, showing DSRS is an effective and reliable procedure for children with portal hypertension and is still useful for selected pediatric patients. DSRS can be used as a bridge to LT if PHT is secondary to intrahepatic problems.…”

Section: Phtmentioning

confidence: 99%

Pediatric Vascular Surgery Review with a 30-Year-Experience in a Tertiary Referral Center

Min

Cho

Kim

et al. 2017

VSI

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Pediatric vascular disease is rare, and remains a big challenge to vascular surgeons. In contrast to adults, surgery for pediatric vascular disease is complicated by issues related to small size, future growth, and availability of suitable vascular conduit. During the last 30 years, 131 major vascular operations were performed in a tertiary referral center, Seoul National University Hospital, including aortoiliac aneurysm, acute or chronic arterial occlusion, renovascular hypertension, portal venous hypertension, trauma, tumor invasion to major abdominal vessels, and others. Herein we review on the important pediatric vascular diseases and share our clinical experiences on these rare diseases.

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“…Moreover, for these complications endoscopic treatments are clearly unsuitable [51]. In those cases, or when hemorrhagic episodes are refractory to other treatments, surgery becomes the only option [52]. …”

Section: Treatmentmentioning

confidence: 99%

Etiology and Management of Hemorrhagic Complications of Portal Hypertension in Children

Costaguta¹,

Álvarez

2012

International Journal of Hepatology

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Portal hypertension in children represents a particular diagnostic and management challenge for several reasons: (1) treatment outcomes should be evaluated in relationship with a long-life expectancy, (2) pediatric patients with portal hypertension constitute an heterogeneous population, both in terms of individual characteristics and diversity of liver diseases; making comparison between treatment outcomes very difficult, (3) application of techniques and procedures developed in adult patients (v.gr. TIPS) face size limitations in small children, and (4) absence of data from well-controlled trials in children forces pediatric specialists to adapt results obtained from adult cohorts suffering from diseases such as HCV and alcoholic cirrhosis. Despite those limitations, substantial progress in the treatment of children with portal hypertension has been achieved in recent years, with better outcomes and survival. Two main factors influence our therapeutic decision: age of the patient and etiology of the liver disease. Therefore, diagnosis and treatment of complications of portal hypertension in children need to be described taking such factors into consideration. This paper summarizes current knowledge and expert opinion.

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The Usefulness of Distal Splenorenal Shunt in Children with Portal Hypertension for the Treatment of Severe Thrombocytopenia and Leukopenia

Abstract: DSRS is an effective and reliable procedure for children with PH and is still useful for selected pediatric patients.

Cited by 21 publications

References 28 publications

Pathological Changes in Pulmonary Circulation in Carbon Tetrachloride (ccl4)-Induced Cirrhotic Mice

Pathological Changes in Pulmonary Circulation in Carbon Tetrachloride (ccl4)-Induced Cirrhotic Mice

Pediatric Vascular Surgery Review with a 30-Year-Experience in a Tertiary Referral Center

Etiology and Management of Hemorrhagic Complications of Portal Hypertension in Children

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