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Riccardo A. Superina - One of the best experts on this subject based on the ideXlab platform.

  • metabolic profile of children with extrahepatic Portal Vein Obstruction undergoing meso rex bypass
    Journal of Surgical Research, 2018
    Co-Authors: Timothy B Lautz, Simon Eaton, Lisa Keys, Joy Ito, Mario Polo, Jonathan C K Wells, Agostino Pierro, Riccardo A. Superina
    Abstract:

    Abstract Background Extrahepatic Portal Vein Obstruction (EHPVO) in children is often associated with growth restriction, which improves after the restoration of Portal venous flow with a meso-Rex bypass, but the physiologic mechanism is unknown. The purpose of this study was to investigate the mechanism of growth delay in children with EHPVO by detailing the metabolic and nutritional profile before and after meso-Rex bypass. Methods Twenty consecutive children with EHPVO were prospectively studied before and 1 year after meso-Rex bypass. Caloric balance was determined by investigating caloric intake via a calorie count, total energy expenditure via a doubly labeled water isotope assay and stool caloric loss by bomb calorimetry. Laboratory markers of nutrition and growth hormone resistance were tested. Results Fifteen of the 20 children underwent successful meso-Rex bypass at a median age of 4.3 years. Prealbumin level was abnormally low (14.6 ± 3.0 mg/dL) at surgery but improved (17.0 ± 4.3 mg/dL) 1 year later (P = 0.026). Mean insulin-like growth factor 1 (IGF-1) level at baseline was 1.57 standard deviations below normal. IGF-1 levels increased from 88.3 ± 38.9 to 117.3 ± 54.5 ng/mL in the year after surgery (P = 0.047). Caloric intake divided by basal metabolic rate (1.90 ± 0.61), total energy expenditure (97.2 ± 15.0% of expected), and stool caloric losses (3.7 ± 1.8% of caloric intake) were all normal at baseline. Conclusions Children with EHPVO suffer from malnutrition and growth hormone resistance, which may explain their well-established finding of growth restriction. Prealbumin and IGF-1 levels improve after a successful meso-Rex bypass.

  • perioperative strategies and thrombophilia in children with extrahepatic Portal Vein Obstruction undergoing the meso rex bypass
    Journal of Gastrointestinal Surgery, 2013
    Co-Authors: Rukhmi Bhat, Riccardo A. Superina, Timothy B Lautz, Robert I Liem
    Abstract:

    Background/Purpose Extrahepatic Portal Vein Obstruction (EHPVO) is an important cause of chronic Portal hypertension in children. Although usually idiopathic in etiology, genetic and acquired thrombophilia have been implicated in EHPVO. Meso-Rex bypass is increasingly used to treat EHPVO in children.

  • advantages of the meso rex bypass compared with portosystemic shunts in the management of extrahepatic Portal Vein Obstruction in children
    Journal of The American College of Surgeons, 2013
    Co-Authors: Timothy B Lautz, Lisa Keys, Joy Ito, Joseph C Melvin, Riccardo A. Superina
    Abstract:

    Background Consequences of extrahepatic Portal Vein Obstruction (EHPVO) include variceal bleeding and hypersplenism due to Portal hypertension, as well as metabolic abnormalities secondary to impaired Portal venous circulation. The purpose of this study was to compare the effectiveness of meso-Rex bypass and portosystemic shunt (PSS) for reversing these symptoms in children with EHPVO. Study Design All children with idiopathic EHPVO evaluated for potential meso-Rex bypass at a single institution between 1997 and 2010 were reviewed. Portosystemic shunt was performed in patients with refractory Portal hypertension when meso-Rex bypass was not technically feasible. Outcomes of meso-Rex bypass and PSS were compared, including resolution of Portal hypertensive bleeding and hypersplenism, as well as changes in liver synthetic function, ammonia levels, and somatic growth. Results Sixty-five children with EHPVO underwent successful meso-Rex bypass, while 16 required PSS. Nearly all patients experienced complete relief of variceal bleeding after meso-Rex (96%) bypass and PSS (100%). The improvements in platelet count (+82.1±60.0 vs +32.4±56.3 thousand/μL; p=0.004), internal normalized ratio (−0.22±0.27 vs 0.01±0.14; p=0.022), and serum ammonia level (−26.8±36.8 vs +19.4±33.1μM/L; p=0.002) were greater after meso-Rex bypass than PSS. Among patients with below average (standard deviation z-score Conclusions Both meso-Rex bypass and PSS effectively relieve symptoms of Portal hypertensive bleeding in children with EHPVO, although the meso-Rex better relieves hypersplenism. By restoring normal Portal venous circulation, the meso-Rex bypass has additional metabolic benefits.

  • outcomes of percutaneous interventions for managing stenosis after meso rex bypass for extrahepatic Portal Vein Obstruction
    Journal of Vascular and Interventional Radiology, 2012
    Co-Authors: Timothy B Lautz, Stanley Kim, James S Donaldson, Riccardo A. Superina
    Abstract:

    Abstract Purpose To determine outcomes in children with extrahepatic Portal Vein Obstruction who underwent percutaneous intervention for anastomotic stenosis after meso-Rex bypass. Materials and Methods Eighty-six patients who underwent meso-Rex bypass between 1997 and 2009 were retrospectively reviewed, and 15 who underwent transhepatic Portal venography for graft stenosis were identified. Technical success and clinical outcomes were analyzed. Results Nine of 15 patients (60%) with anastomotic stenosis were successfully treated by endovascular techniques and remain symptom-free with patent shunts, three (20%) underwent technically successful interventions but currently have recurrent stenosis, and three (20%) underwent failed attempts at percutaneous intervention. All three in whom percutaneous intervention failed required surgical revision, including two with near-occlusive lesions that prevented traversal of the stenosis and one who developed postprocedure shunt thrombosis. In total, patients underwent 27 endovascular procedures, including 17 balloon venoplasties, four cutting balloon venoplasties, five stent placements, and one balloon venoplasty with thrombolysis. The mean pressure gradient across the stenosis decreased from 11 mm Hg ± 3 to 5 mm Hg ± 3 ( P P = .002) and ammonia levels decreased (from 40 μmol/L ± 11 to 24 μmol/L ± 13; P = .05) after intervention. Patients were followed for a median duration of 39 months after the last intervention. Conclusions Percutaneous interventions allow for long-term Vein graft patency and clinical resolution of symptoms in the majority of patients with anastomotic stenosis after meso-Rex bypass.

  • growth impairment in children with extrahepatic Portal Vein Obstruction is improved by mesenterico left Portal Vein bypass
    Journal of Pediatric Surgery, 2009
    Co-Authors: Timothy B Lautz, Lisa Keys, Shikha S Sundaram, Peter F Whitington, Riccardo A. Superina
    Abstract:

    Abstract Background Extrahepatic Portal Vein Obstruction (EHPVO) has been associated with growth impairment in children. We hypothesized that growth parameters improve after reversal of Portal hypertension and restoration of mesenteric venous blood flow to the liver by the mesenterico-left Portal Vein bypass (MLPVB). Methods A retrospective review of 45 children with idiopathic EHPVO who underwent MLPVB between 1997 and 2007 and had follow-up data for analysis was carried out. Growth was assessed using SD scores ( z scores) for height, weight, and body mass index (BMI) at the time of operation and at early (5-12 months) and late (13-24 months) follow-up. Results The mean height and weight of children with EHPVO was significantly lower than the general population before surgery. Mean BMI was also lower, although statistically insignificant. All parameters increased significantly after MLPVB as follows: height from −0.42 before surgery to −0.12 ( P = .027) at 5 to 12 months and −0.14 ( P = .026) at 13 to 24 months; weight from −0.49 before surgery to 0.03 ( P P P = .001) at 5 to 12 months and 0.48 ( P Conclusion Restoration of Portal blood flow to the liver by MLPVB improves growth in children with EHPVO.

Shiv Kumar Sarin - One of the best experts on this subject based on the ideXlab platform.

  • extrahepatic Portal Vein Obstruction asian and global perspective
    2018
    Co-Authors: Rakhi Maiwall, Shiv Kumar Sarin
    Abstract:

    Portal Vein thrombosis refers to either thrombotic or non-thrombotic occlusion of the Portal Vein as well as cavernoma formation. It is known to exist in association with cirrhosis or malignancy or without any associated condition i.e. non-malignant and non-cirrhotic Portal Vein thrombosis. Extra Hepatic Portal Venous Obstruction (EHPVO) refers to the development of Portal cavernoma in the absence of associated liver disease and is the commonest cause of non-cirrhotic Portal hypertension in Asia. The main pathophysiological basis of PVT is based on the “Virchow’s triad” which comprises of venous stasis, hypercoagulability and endothelial injury. Recently, an expert consensus has proposed a new classification system for PVT based on the site, clinical presentation (acute or chronic), degree of occlusion i.e. (partial or complete) and extent (extension into the splenic or superior mesenteric Veins), symptomatic or asymptomatic as well as based on the type of underlying liver disease. Anticoagulation remains the mainstay of management in acute PVT and should be administered life-long in patients with an underlying proven thrombophilia. Transjugular intrahepatic portosystemic shunt even though challenging result in recanalization in a significant proportion of treated patients. Prevention of PVT with anticoagulants in compensated cirrhotics has been suggested to prevent disease progression and improve survival. Thrombectomy and thrombolysis can be done in selected symptomatic cases with acute PVT. Shunt surgery or meso-Rex bypass operation are the definitive treatment options in patients with EHPVO who have complications of Portal hypertension.

  • liver and spleen stiffness in patients with extrahepatic Portal Vein Obstruction
    Radiology, 2012
    Co-Authors: P Sharma, Barjesh Chander Sharma, Smruti Ranjan Mishra, Manoj Kumar, Shiv Kumar Sarin
    Abstract:

    PURPOSE: To evaluate liver stiffness (LS) and spleen stiffness (SS) in patients with extrahepatic Portal Vein Obstruction (EHPVO). MATERIALS AND METHODS: Institutional research board approval and informed consent were obtained. LS and SS were measured in 65 consecutive patients with EHPVO. Patients underwent endoscopy, liver biopsy, liver function tests, abdominal ultrasonography, a detailed history, and examination. LS and SS measurements were also obtained in 50 age-matched healthy control subjects. Comparisons were made by using the Student t test, Mann-Whitney test for quantitative data, and χ(2) or Fisher exact test for qualitative data. RESULTS: Sixty-five patients with EHPVO (with a bleed, n = 45; without a bleed, n = 20; mean age, 25.4 years ± 10.7 [standard deviation]; 29 men, 36 women) were enrolled. Twenty-two (34%) had hypersplenism. LS (P = .001) and SS (P = .01) were higher in patients with EHPVO (6.7 kPa ± 2.3 and 51.7 kPa ± 21.5, respectively) than in control subjects (4.6 kPa ± 0.7 and 16.0 kPa ± 3.0, respectively). Patients who had a bleed had higher SS than did those without a bleed (60.4 kPa ± 5.4 vs 30.3 kPa ± 14.2, P = .01). There was no significant difference in age (26.7 years ± 10.4 vs 22.5 years ± 9.8, P = .8) and median duration of disease (4.5 years [range, 1-26 years] vs 6.0 years [range, 1-22 years], P = .23) in patients with a bleed versus those without. With a cutoff of 5.9 kPa for LS, sensitivity and specificity for detection of a variceal bleed were 67% and 75%, respectively. An SS cutoff of 42.8 kPa yielded sensitivity and specificity of 88% and 94%, respectively. CONCLUSION: LS and SS were higher in patients with EHPVO than in control subjects, and patients with a history of a bleed had a higher SS than did those without a bleed.

  • liver and spleen stiffness in patients with extrahepatic Portal Vein Obstruction
    Radiology, 2012
    Co-Authors: P Sharma, Barjesh Chander Sharma, Smruti Ranjan Mishra, Manoj Kumar, Shiv Kumar Sarin
    Abstract:

    Spleen stiffness measurement is useful in patients with extrahepatic Portal Vein Obstruction and can be used to discriminate patients with a bleed from those without a bleed with high sensitivity and specificity.

  • systemic and pulmonary hemodynamics in patients with extrahepatic Portal Vein Obstruction is similar to compensated cirrhotic patients
    Hepatology International, 2009
    Co-Authors: Sanjeev Kumar Jha, Barjesh Chander Sharma, Ashish Kumar, Shiv Kumar Sarin
    Abstract:

    Patients with cirrhosis and Portal hypertension exhibit a hyperdynamic circulation manifesting as increased cardiac output, heart rate and plasma volume; and decreased arterial blood pressure, systemic vascular resistance, and pulmonary vascular resistance. It is believed that these changes are related to both hepatocellular dysfunction and Portal hypertension. However, the role of Portal hypertension per se in producing these changes in circulation has not been clear. Extrahepatic Portal Vein Obstruction (EHPVO), a vascular disorder of the liver characterized by cavernomatous transformation of the main Portal Vein, is an excellent model to study the role of Portal hypertension per se in producing these changes because there is no hepatic dysfunction in EHPVO. The main aim of our study was, therefore, to evaluate alterations of systemic and pulmonary vascular systems in patients with EHPVO and compare them with patients with compensated cirrhosis. Consecutive patients of EHPVO, 15 years or older, and past variceal bleeders were studied. For comparison, consecutive patients with compensated cirrhosis and history of variceal bleed, matched for variceal status, and body surface area were included. The hemodynamic studies included the measurements of cardiac index (by Fick’s oxygen method), and systemic and pulmonary vascular resistance indices. Fifteen patients of EHPVO and same number of controls (compensated cirrhotics) were included in the study. The baseline parameters in the two groups were comparable. Both EHPVO patients and cirrhotics had similar values in all the measured systemic and pulmonary hemodynamic parameters. The median (range) cardiac index in EHPVO was 3.8 (2.3–7.7) l min−1 m−2, whereas it was 4.4 (2.8–8.9) l min−1 m−2 in cirrhosis (P = 0.468). The median (range) systemic vascular resistance index in EHPVO was 1,835 (806–3400) dyne s cm−5 m−2, which was similar to that in cirrhotic patients (1,800 [668–3022], P = 0.520). Similarly, the values of median (range) pulmonary vascular resistance index were comparable in the two groups (71 [42–332] vs. 79 [18–428], P = 0.885). A subgroup analysis was done for 8 patients of EHPVO and 8 age-matched compensated cirrhotic patients, which also revealed similar values of cardiac index, cardiac output, systemic vascular resistance index, systemic vascular resistance, pulmonary vascular resistance index, and pulmonary vascular resistance in the two groups. EHPVO patients have hyperdynamic circulation manifested by high cardiac index and low systemic and pulmonary vascular resistance indices. These hemodynamic changes are comparable with compensated cirrhotic patients who have similar grade of Portal hypertension. This suggests a predominant role of Portal hypertension per se in the genesis of systemic and pulmonary hemodynamic alterations.

  • natural history of minimal hepatic encephalopathy in patients with extrahepatic Portal Vein Obstruction
    The American Journal of Gastroenterology, 2009
    Co-Authors: P Sharma, Barjesh Chander Sharma, Vinod Puri, Shiv Kumar Sarin
    Abstract:

    Natural History of Minimal Hepatic Encephalopathy in Patients With Extrahepatic Portal Vein Obstruction

Rakesh K. Gupta - One of the best experts on this subject based on the ideXlab platform.

  • evaluation of cognitivity proinflammatory cytokines and brain magnetic resonance imaging in minimal hepatic encephalopathy induced by cirrhosis and extrahepatic Portal Vein Obstruction
    Journal of Gastroenterology and Hepatology, 2016
    Co-Authors: Santosh K. Yadav, Michael A. Thomas, Vivek A. Saraswat, Amit Goel, Ena Wang, Francesco M Marincola, Mohammad Haris, Rakesh K. Gupta
    Abstract:

    Minimal hepatic encephalopathy (MHE) is the mildest form of hepatic encephalopathy (HE) and is characterized by deficits in neurocognitive performance without any clinical symptoms of HE. In the current study, we aim to evaluate and compare the neurocognitive, biochemical, and brain magnetic resonance (MR) imaging changes between patients with cirrhotic MHE and extrahepatic Portal Vein Obstruction (EHPVO) MHE.Thirty-three cirrhotic and 14 EHPVO patients were diagnosed with MHE and were included in the analysis along with 24 normal healthy volunteers. All subjects underwent MR imaging including diffusion tensor imaging and proton MR spectroscopy (1 H-MRS) followed by cognitive assessments, critical flicker frequency (CFF) measurements, quantification of blood ammonia, and serum proinflammatory cytokine levels.We observed abnormal neurocognitive functions and CFF measurements in both cirrhotic MHE and EHPVO MHE patients as compared with controls. Significantly increased blood ammonia, serum proinflammatory cytokines (IL-6, TNF-α) level, mean diffusivity in multiple brain sites, 1 H-MRS derived glutamate/glutamine (Glx)/creatine (Cr), and significantly decreased 1 H-MRS derived myo-inositol/Cr were observed in both cirrhotic MHE and EHPVO MHE compared with those of controls. Choline/Cr level was significantly decreased in cirrhotic MHE as compared with controls and EHPVO MHE.Cirrhotic MHE showed more severe changes on mean diffusivity in multiple brain sites and inflammation as compared with EHPVO MHE. This study confirms that there are significant difference in neurocognitive, biochemical, and MR profile between cirrhotic MHE and EHPVO MHE, which may help to understand the pathophysiologies of these two types of MHE and may contribute to improve their clinical managements.

  • evaluation of cognitivity proinflammatory cytokines and brain magnetic resonance imaging in minimal hepatic encephalopathy induced by cirrhosis and extrahepatic Portal Vein Obstruction
    Journal of Gastroenterology and Hepatology, 2016
    Co-Authors: Santosh K. Yadav, Michael A. Thomas, Vivek A. Saraswat, Amit Goel, Ena Wang, Francesco M Marincola, Mohammad Haris, Rakesh K. Gupta
    Abstract:

    bs_bs_banner doi:10.1111/jgh.13427 H E PAT O L O G Y Evaluation of cognitivity, proinflammatory cytokines, and brain magnetic resonance imaging in minimal hepatic encephalopathy induced by cirrhosis and extrahepatic Portal Vein Obstruction Santosh K Yadav,* Amit Goel, † Vivek A Saraswat, † Michael A Thomas, ‡ Ena Wang,* Francesco M Marincola,* Mohammad Haris* and Rakesh K Gupta § *Research Branch, Sidra Medical and Research Center, Doha, Qatar; † Department of Gastroenterology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, § Department of Radiology, Fortis Memorial Research Institute, Gurgaon, Haryana, India; and ‡ Department of Radiological Sciences, David Geffen School of Medicine at University of California at Los Angeles, Los Angeles, California, USA Key words cirrhosis, extrahepatic Portal Vein Obstruction, magnetic resonance imaging, mean diffusivity, minimal hepatic encephalopathy. Accepted for publication 20 April 2016. Correspondence Santosh K. Yadav, Research Branch, Sidra Medical and Research Center, P.O. Box 26999, Doha, Qatar. Email: syadav@sidra.org, santoshyadav20076@gmail.com Declaration of conflict of interest: All listed authors disclose no conflicts of interest. Abstract Background and Aims: Minimal hepatic encephalopathy (MHE) is the mildest form of hepatic encephalopathy (HE) and is characterized by deficits in neurocognitive perfor- mance without any clinical symptoms of HE. In the current study, we aim to evaluate and compare the neurocognitive, biochemical, and brain magnetic resonance (MR) imag- ing changes between patients with cirrhotic MHE and extrahepatic Portal Vein Obstruction (EHPVO) MHE. Methods: Thirty-three cirrhotic and 14 EHPVO patients were diagnosed with MHE and were included in the analysis along with 24 normal healthy volunteers. All subjects underwent MR imaging including diffusion tensor imaging and proton MR spectroscopy ( 1 H-MRS) followed by cognitive assessments, critical flicker frequency (CFF) measure- ments, quantification of blood ammonia, and serum proinflammatory cytokine levels. Results: We observed abnormal neurocognitive functions and CFF measurements in both cirrhotic MHE and EHPVO MHE patients as compared with controls. Significantly in- creased blood ammonia, serum proinflammatory cytokines (IL-6, TNF-α) level, mean dif- fusivity in multiple brain sites, 1 H-MRS derived glutamate/glutamine (Glx)/creatine (Cr), and significantly decreased 1 H-MRS derived myo-inositol/Cr were observed in both cir- rhotic MHE and EHPVO MHE compared with those of controls. Choline/Cr level was sig- nificantly decreased in cirrhotic MHE as compared with controls and EHPVO MHE. Conclusions: Cirrhotic MHE showed more severe changes on mean diffusivity in multiple brain sites and inflammation as compared with EHPVO MHE. This study confirms that there are significant difference in neurocognitive, biochemical, and MR profile between cirrhotic MHE and EHPVO MHE, which may help to understand the pathophysiologies of these two types of MHE and may contribute to improve their clinical managements. Introduction Liver cirrhosis is a chronic disease, commonly caused by hepatitis B, hepatitis C, alcoholism, and fatty liver and associated with asci- tes, poor quality of life, increased work disability, and risk of infection, 1–4 consequently development of hepatic encephalopathy (HE). HE is a potentially reversible wide range of neuropsychiatric symptoms, associated with liver failures and extrahepatic Portal Vein Obstruction (EHPVO). 5–8 It is considered that increased blood ammonia and proinflammatory cytokine levels are key factors involved in pathogenesis of HE. 9,10 Minimal hepatic encephalopa- thy (MHE) is the mildest form of HE, which represents quantifi- able neuropsychological manifestations without any clinical symptoms of HE. 11 MHE is frequently diagnosed in cirrhotic patients, and recently it has also been reported in EHPVO patients. 6,7 EHPVO is a condition associated with an Obstruction of the main Portal Vein with or without Obstruction to its tribu- taries, without liver cirrhosis or malignancy. 12 MHE is considered clinically relevant because of many reasons; in particular, it pre- dicts the development of cerebral edema and overt HE. 13 It is observed that hyperammonemia and inflammation are synergisti- cally responsible for symptoms of HE in cirrhotic patients; 10,14 however, in EHPVO, patients’ role of these factors are not well studied. 15 It is reported that cirrhotic patients with MHE have a characteristic proton magnetic resonance spectroscopy ( 1 H-MRS) profile such as increased glutamine/glutamate (Glx), decreased myo-inositol (mIns) and choline (Cho), 16–18 and increased mean diffusivity on diffusion tensor imaging (DTI), 19 whereas EHPVO patients with MHE showed increased Glx, decreased mIns and no change in Cho, while on DTI significantly increased mean Journal of Gastroenterology and Hepatology 31 (2016) 1986–1994 © 2016 Journal of Gastroenterology and Hepatology Foundation and John Wiley & Sons Australia, Ltd

  • Encephalopathy assessment in children with extra-hepatic Portal Vein Obstruction with MR, psychometry and critical flicker frequency.
    Journal of hepatology, 2010
    Co-Authors: Santosh K. Yadav, Anshu Srivastava, Arti Srivastava, Michael A. Thomas, Jaya Agarwal, Chandra M. Pandey, Richa Lal, Surender Kumar Yachha, Vivek A. Saraswat, Rakesh K. Gupta
    Abstract:

    Background & Aims Mild cognitive and psychomotor deficit has been reported in patients with extra-hepatic Portal Vein Obstruction. This prospective study was done to ascertain the presence of minimal hepatic encephalopathy by neuropsychological testing and its correlation with diffusion tensor imaging derived metrics, T1 signal intensity, brain metabolites in 1 H magnetic resonance spectroscopy, blood ammonia and critical flicker frequency in patients with extra-hepatic Portal Vein Obstruction. Methods Neuropsychological tests, critical flicker frequency, blood ammonia, diffusion tensor imaging, T1 signal intensity and 1 H magnetic resonance spectroscopy were determined in 22 extra-hepatic Portal Vein Obstruction and 17 healthy children. Bonferroni multiple comparison post hoc analysis was done to compare controls with patient groups. Results Based on neuropsychological tests, 7/22 patients had minimal hepatic encephalopathy, and significantly increased Glx/Cr ratio, blood ammonia, mean diffusivity and globus pallidus T1 signal intensity with decreased critical flicker frequency in comparison to controls and in those without minimal hepatic encephalopathy. Cho/Cr, mI/Cr ratio and fractional anisotropy were unchanged in patient groups compared to controls. A significant inverse correlation of neuropsychological test with mean diffusivity, Glx/Cr ratio and blood ammonia and a positive correlation among mean diffusivity, blood ammonia and Glx/Cr ratio was seen. Conclusions Extra-hepatic Portal Vein Obstruction is a true hyperammonia model with porto-systemic shunting and normal liver functions that results in minimal hepatic encephalopathy in one-third of these children. Hyperammonia results in generalized low grade cerebral edema and cognitive decline as evidenced by increased Glx/Cr ratio, mean diffusivity values and abnormal neuropsychological tests.

Timothy B Lautz - One of the best experts on this subject based on the ideXlab platform.

  • metabolic profile of children with extrahepatic Portal Vein Obstruction undergoing meso rex bypass
    Journal of Surgical Research, 2018
    Co-Authors: Timothy B Lautz, Simon Eaton, Lisa Keys, Joy Ito, Mario Polo, Jonathan C K Wells, Agostino Pierro, Riccardo A. Superina
    Abstract:

    Abstract Background Extrahepatic Portal Vein Obstruction (EHPVO) in children is often associated with growth restriction, which improves after the restoration of Portal venous flow with a meso-Rex bypass, but the physiologic mechanism is unknown. The purpose of this study was to investigate the mechanism of growth delay in children with EHPVO by detailing the metabolic and nutritional profile before and after meso-Rex bypass. Methods Twenty consecutive children with EHPVO were prospectively studied before and 1 year after meso-Rex bypass. Caloric balance was determined by investigating caloric intake via a calorie count, total energy expenditure via a doubly labeled water isotope assay and stool caloric loss by bomb calorimetry. Laboratory markers of nutrition and growth hormone resistance were tested. Results Fifteen of the 20 children underwent successful meso-Rex bypass at a median age of 4.3 years. Prealbumin level was abnormally low (14.6 ± 3.0 mg/dL) at surgery but improved (17.0 ± 4.3 mg/dL) 1 year later (P = 0.026). Mean insulin-like growth factor 1 (IGF-1) level at baseline was 1.57 standard deviations below normal. IGF-1 levels increased from 88.3 ± 38.9 to 117.3 ± 54.5 ng/mL in the year after surgery (P = 0.047). Caloric intake divided by basal metabolic rate (1.90 ± 0.61), total energy expenditure (97.2 ± 15.0% of expected), and stool caloric losses (3.7 ± 1.8% of caloric intake) were all normal at baseline. Conclusions Children with EHPVO suffer from malnutrition and growth hormone resistance, which may explain their well-established finding of growth restriction. Prealbumin and IGF-1 levels improve after a successful meso-Rex bypass.

  • perioperative strategies and thrombophilia in children with extrahepatic Portal Vein Obstruction undergoing the meso rex bypass
    Journal of Gastrointestinal Surgery, 2013
    Co-Authors: Rukhmi Bhat, Riccardo A. Superina, Timothy B Lautz, Robert I Liem
    Abstract:

    Background/Purpose Extrahepatic Portal Vein Obstruction (EHPVO) is an important cause of chronic Portal hypertension in children. Although usually idiopathic in etiology, genetic and acquired thrombophilia have been implicated in EHPVO. Meso-Rex bypass is increasingly used to treat EHPVO in children.

  • advantages of the meso rex bypass compared with portosystemic shunts in the management of extrahepatic Portal Vein Obstruction in children
    Journal of The American College of Surgeons, 2013
    Co-Authors: Timothy B Lautz, Lisa Keys, Joy Ito, Joseph C Melvin, Riccardo A. Superina
    Abstract:

    Background Consequences of extrahepatic Portal Vein Obstruction (EHPVO) include variceal bleeding and hypersplenism due to Portal hypertension, as well as metabolic abnormalities secondary to impaired Portal venous circulation. The purpose of this study was to compare the effectiveness of meso-Rex bypass and portosystemic shunt (PSS) for reversing these symptoms in children with EHPVO. Study Design All children with idiopathic EHPVO evaluated for potential meso-Rex bypass at a single institution between 1997 and 2010 were reviewed. Portosystemic shunt was performed in patients with refractory Portal hypertension when meso-Rex bypass was not technically feasible. Outcomes of meso-Rex bypass and PSS were compared, including resolution of Portal hypertensive bleeding and hypersplenism, as well as changes in liver synthetic function, ammonia levels, and somatic growth. Results Sixty-five children with EHPVO underwent successful meso-Rex bypass, while 16 required PSS. Nearly all patients experienced complete relief of variceal bleeding after meso-Rex (96%) bypass and PSS (100%). The improvements in platelet count (+82.1±60.0 vs +32.4±56.3 thousand/μL; p=0.004), internal normalized ratio (−0.22±0.27 vs 0.01±0.14; p=0.022), and serum ammonia level (−26.8±36.8 vs +19.4±33.1μM/L; p=0.002) were greater after meso-Rex bypass than PSS. Among patients with below average (standard deviation z-score Conclusions Both meso-Rex bypass and PSS effectively relieve symptoms of Portal hypertensive bleeding in children with EHPVO, although the meso-Rex better relieves hypersplenism. By restoring normal Portal venous circulation, the meso-Rex bypass has additional metabolic benefits.

  • outcomes of percutaneous interventions for managing stenosis after meso rex bypass for extrahepatic Portal Vein Obstruction
    Journal of Vascular and Interventional Radiology, 2012
    Co-Authors: Timothy B Lautz, Stanley Kim, James S Donaldson, Riccardo A. Superina
    Abstract:

    Abstract Purpose To determine outcomes in children with extrahepatic Portal Vein Obstruction who underwent percutaneous intervention for anastomotic stenosis after meso-Rex bypass. Materials and Methods Eighty-six patients who underwent meso-Rex bypass between 1997 and 2009 were retrospectively reviewed, and 15 who underwent transhepatic Portal venography for graft stenosis were identified. Technical success and clinical outcomes were analyzed. Results Nine of 15 patients (60%) with anastomotic stenosis were successfully treated by endovascular techniques and remain symptom-free with patent shunts, three (20%) underwent technically successful interventions but currently have recurrent stenosis, and three (20%) underwent failed attempts at percutaneous intervention. All three in whom percutaneous intervention failed required surgical revision, including two with near-occlusive lesions that prevented traversal of the stenosis and one who developed postprocedure shunt thrombosis. In total, patients underwent 27 endovascular procedures, including 17 balloon venoplasties, four cutting balloon venoplasties, five stent placements, and one balloon venoplasty with thrombolysis. The mean pressure gradient across the stenosis decreased from 11 mm Hg ± 3 to 5 mm Hg ± 3 ( P P = .002) and ammonia levels decreased (from 40 μmol/L ± 11 to 24 μmol/L ± 13; P = .05) after intervention. Patients were followed for a median duration of 39 months after the last intervention. Conclusions Percutaneous interventions allow for long-term Vein graft patency and clinical resolution of symptoms in the majority of patients with anastomotic stenosis after meso-Rex bypass.

  • growth impairment in children with extrahepatic Portal Vein Obstruction is improved by mesenterico left Portal Vein bypass
    Journal of Pediatric Surgery, 2009
    Co-Authors: Timothy B Lautz, Lisa Keys, Shikha S Sundaram, Peter F Whitington, Riccardo A. Superina
    Abstract:

    Abstract Background Extrahepatic Portal Vein Obstruction (EHPVO) has been associated with growth impairment in children. We hypothesized that growth parameters improve after reversal of Portal hypertension and restoration of mesenteric venous blood flow to the liver by the mesenterico-left Portal Vein bypass (MLPVB). Methods A retrospective review of 45 children with idiopathic EHPVO who underwent MLPVB between 1997 and 2007 and had follow-up data for analysis was carried out. Growth was assessed using SD scores ( z scores) for height, weight, and body mass index (BMI) at the time of operation and at early (5-12 months) and late (13-24 months) follow-up. Results The mean height and weight of children with EHPVO was significantly lower than the general population before surgery. Mean BMI was also lower, although statistically insignificant. All parameters increased significantly after MLPVB as follows: height from −0.42 before surgery to −0.12 ( P = .027) at 5 to 12 months and −0.14 ( P = .026) at 13 to 24 months; weight from −0.49 before surgery to 0.03 ( P P P = .001) at 5 to 12 months and 0.48 ( P Conclusion Restoration of Portal blood flow to the liver by MLPVB improves growth in children with EHPVO.

Arun Kumar Gupta - One of the best experts on this subject based on the ideXlab platform.