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Expanding the Utilization of Metabolic Livers for Domino Transplantation: Successful Domino Liver Transplant from a Patient with Propionic Acidemia [Meeting Abstract]
Ovchinsky, Nadia; Cunningham, Ryan M.; Kogan-Liberman, Debora; Bellemare, Sarah; Kinkhabwala, Milan; Levy, Paul; Jan, Dominique
ISI:000385493803206
ISSN: 0270-9139
CID: 5416672
Pyogenic Liver Abscess Masquerading as a Hepatic Neoplasm [Case Report]
Taylor, Sarah; Lobritto, Steven; Orellana, Katherine; Novak, Inna; Jan, Dominique; Martinez, Mercedes; Ovchinsky, Nadia
PMID: 24345844
ISSN: 1536-4801
CID: 5416472
Improved tolerance for enteral nutrition after serial transverse enteroplasty (STEP) in infants and children with short bowel syndrome--a seven-year single-center experience
Oh, Pilyung S; Fingeret, Abbey L; Shah, Manan Y; Ventura, Kara A; Brodlie, Susan; Ovchinsky, Nadia; Martinez, Mercedes; Lobritto, Steven J; Cowles, Robert A
BACKGROUND:Serial transverse enteroplasty (STEP) was designed to lengthen and taper the small intestine in patients with short bowel syndrome (SBS) and dilated small bowel. We hypothesized that tolerance for enteral nutrition (EN) improves after STEP. METHODS:Patients who underwent STEP between March 2004 and January 2011 were identified. Candidates for STEP had radiographic evidence of dilated small bowel and either failed to advance EN or demonstrated deterioration in tolerance for EN. Clinical and nutritional data were analyzed pre- and post-STEP. EN was defined as the percentage of calories administered enterally. Statistical analysis employed the signed rank test with significance assumed when p<0.05. RESULTS:Twenty STEPs were performed at a median age of 13.7 months. Median pre-STEP bowel length was 30 cm with a median increase in bowel length of 42%. Five patients achieved enteral autonomy at a median of 6.5 months post-STEP. EN increased in 75%, while 25% exhibited unchanged or decreased EN post-STEP. In aggregate, median EN tolerance increased from 22% at one month pre-STEP to 61% at six months post-STEP (p=0.003). CONCLUSIONS:The STEP is an effective adjunct in the treatment of patients with intestinal failure. While enteral autonomy is eventually possible in some patients, improved enteral tolerance can be achieved in a majority of cases.
PMID: 25475799
ISSN: 1531-5037
CID: 5416482
The use of bi-planar tissue expanders to augment abdominal domain in a pediatric intestinal transplant recipient [Case Report]
Weiner, Joshua; Wu, June; Martinez, Mercedes; Lobritto, Steven; Ovchinsky, Nadia; Rohde, Christine; Griesemer, Adam; Kato, Tomoaki
Intestinal transplantation is a well-accepted treatment for SBS. However, patients with SBS often have decreased abdominal capacity, which makes size-matching of donor organs more difficult, thus decreasing organ availability. Reported approaches for addressing this problem include surgically reducing the graft size, leaving an open abdomen for a prolonged period, and cotransplanting rectus fascia as a non-vascularized allograft. Each approach has significant disadvantages. There has been one previous report of tissue expanders used intra-abdominally and two reports of subcutaneous use to increase intra-abdominal capacity prior to transplantation. We report the first use of bi-planar expander placement for this purpose. In our case, a two-yr-old male child with SBS due to malrotation was treated with tissue expanders 10Â months prior to intestinal transplantation, thus allowing transplantation of a larger graft with the ability to close the abdomen safely. There were no complications, and the patient is now doing well and tolerating diet off PN. The use of tissue expanders prior to intestinal transplantation is a promising approach for such patients and avoids the morbidity associated with other approaches. This approach requires a multidisciplinary effort by gastroenterology, transplant surgery, and plastic surgery teams.
PMCID:4367952
PMID: 25041331
ISSN: 1399-3046
CID: 5150932
Histological abnormalities in children with nonalcoholic fatty liver disease and normal or mildly elevated alanine aminotransferase levels
Molleston, Jean P; Schwimmer, Jeffrey B; Yates, Katherine P; Murray, Karen F; Cummings, Oscar W; Lavine, Joel E; Brunt, Elizabeth M; Scheimann, Ann O; Unalp-Arida, Aynur; Abrams, Stephanie H; Fairly, Leanel Angeli; Hawkins, Carol; Stager, Margaret; Mohan, Parvathi; DeVore, Stephanie; Kohli, Rohit; Lake, Kathleen; Xanthakos, Stavra; Lavine, Joel E; Mencin, Ali; Ovchinsky, Nadia; Byam, Elizabeth; Cummings, Oscar W; Klipsch, Ann; Molleston, Jean P; Subbarao, Girish; Devadason, Caroline; Pfeifer, Kimberly; Scheimann, Ann; Torbenson, Michael; Kerkar, Nanda; Suchy, Frederick; Dunne, Katherine; Fishbein, Mark H; Jacques, Katie; Quinn, Ann; Riazi, Cindy; Whitington, Peter F; Barlow, Sarah; Derdoy, Jose; King, Debra; Morris, Andrea; Siegner, Joan; Stewart, Susan; Neuschwander-Tetri, Brent A; Thompson, Judy; Behling, Cynthia; Collins, Jennifer; Durelle, Janis; Morgan, Anya; Rose, Steven; Schwimmer, Jeffrey B; Sirlin, Claude; Stein, Tanya; Ferrell, Linda D; Filipowski, Danuta; Fleck, Shannon; Langlois, Camille; Rosenthal, Philip; Young, Melissa; Rich, Deana; Murray, Karen; Yeh, Matthew; Boyett, Sherry; Contos, Melissa J; Fuchs, Michael; Jones, Amy; Luketic, Velimir A C; Puri, Puneet; Sandhu, Bimalijit; Sanyal, Arun J; Sargeant, Carol; Noble, Kimberly; White, Melanie; Brunt, Elizabeth M; Kleiner, David E; Grave, Gilman D; Doo, Edward C; Hoofnagle, Jay H; Robuck, Patricia R; Sherker, Averell; Belt, Patricia; Clark, Jeanne M; Donithan, Michele; Isaacson, Milana; May, Kevin P; Miriel, Laura; Sternberg, Alice; Tonascia, James; Ünalp-Arida, Aynur; Van Natta, Mark; Vaughn, Ivana; Wilson, Laura; Yates, Katherine
OBJECTIVE:To investigate the histological spectrum of nonalcoholic fatty liver disease (NAFLD) in children with normal, mildly elevated (26-50 U/L boys, 23-44 U/L girls), or elevated (>50 U/L in boys, >44 U/L in girls) serum alanine aminotransferase (ALT) levels. STUDY DESIGN/METHODS:The Nonalcoholic Steatohepatitis Clinical Research Network enrolls children aged 5-18 years with NAFLD. We analyzed baseline clinical and histological data from 91 children with suspected NAFLD and normal or mildly elevated ALT and liver biopsy analysis within 180 days of ALT measurement, and compared them with data from 392 children with elevated ALT. RESULTS:Seventeen of the 91 children with suspected NAFLD (19%) had a normal ALT level, and 74 (81%) had a mildly elevated ALT level. Overall, 45% of the biopsy specimens analyzed had steatosis ≥33%, 22% had grade ≥2 lobular inflammation, 81% had portal inflammation, 29% had ballooned hepatocytes, 35% had "suspicious/borderline" steatohepatitis, 8% had definite nonalcoholic steatohepatitis, 34% had an NAFLD activity score ≥4, and 46% had fibrosis (38% mild/moderate and 8% bridging/cirrhosis). Marked steatosis (50% vs 24%) and fibrosis (54% vs 12%) were significantly more common in the patients with mildly elevated ALT compared with those with normal ALT, with no difference in ballooning, inflammation, or NAFLD activity score ≥4 between the 2 groups. Fibrosis stage 3/4 was seen in none of the children with normal ALT, in 9% of those with mildly elevated ALT, and in 15% of those with elevated ALT. CONCLUSION/CONCLUSIONS:Liver biopsy specimens from children with NAFLD with normal or mildly elevated ALT levels show significant histological abnormalities, including advanced fibrosis in children with mildly elevated ALT. Thus, measurement of ALT may underestimate liver injury in NAFLD. The use of appropriate ALT cutoff levels can help identify children at risk for more severe disease.
PMID: 24360992
ISSN: 1097-6833
CID: 5417092
Longitudinal assessment of high blood pressure in children with nonalcoholic fatty liver disease
Schwimmer, Jeffrey B; Zepeda, Anne; Newton, Kimberly P; Xanthakos, Stavra A; Behling, Cynthia; Hallinan, Erin K; Donithan, Michele; Tonascia, James; Abrams, Stephanie H; Barlow, Sarah; Himes, Ryan; Krisnamurthy, Rajesh; Maldonado, Leanel; Morris, Beverly; Bernstein, Kimberlee; Cecil, Kim; DeVore, Stephanie; Kohli, Rohit; Lake, Kathleen; Podberesky, Daniel; Slaughter, Crystal; Xanthakos, Stavra; Behr, Gerald; Lavine, Joel E; Mencin, Ali; Ovchinsky, Nadia; Reynoso, Elena; Alazraki, Adina; Cleeton, Rebecca; Karpen, Saul; Raviele, Nicholas; Vos, Miriam; Byam, Elizabeth; Cummings, Oscar W; Fleming, Cynthia; Klipsch, Ann; Molleston, Jean P; Sandrasegaran, Kumar; Subbarao, Girish; Pfeifer, Kimberly; Scheimann, Ann; Torbenson, Michael; Arnon, Ronen; Boyd, Mariel; Amsden, Katie; Fishbein, Mark H; Kirwan, Elizabeth; Mohammad, Saeed; Quinn, Ann; Rigsby, Cynthia; Whitington, Peter F; Derdoy, Jose; Jain, Ajay; King, Debra; Osmack, Pat; Siegner, Joan; Stewart, Susan; Romo, Dana; Angeles, Jorge; Arroyo, Sandra; Awai, Hannah I; Behling, Cynthia; Bross, Craig; Collins, Jennifer; Durelle, Janis; Middleton, Michael; Newton, Kimberly; Paiz, Melissa; Schwimmer, Jeffrey B; Sirlin, Claude; Ugalde-Nicalo, Patricia; Zepeda, Anne; Aouizerat, Bradley; Ferrell, Linda D; Fleck, Shannon; Gill, Ryan; Langlois, Camille; Perito, Emily Rothbaum; Rosenthal, Philip; Tsai, Patrika; Cooper, Kara; Horslen, Simon; Hsu, Evelyn; Murray, Karen; Otto, Randolph; Rich, Deana; Yeh, Matthew; Young, Melissa; Brunt, Elizabeth M; Fowler, Kathryn; Kleiner, David E; Grave, Gilman D; Doo, Edward C; Hoofnagle, Jay H; Robuck, Patricia R; Sherker, Averell; Belt, Patricia; Clark, Jeanne M; Hallinan, Erin; Donithan, Michele; Isaacson, Milana; May, Kevin P; Miriel, Laura; Sternberg, Alice; Tonascia, James; Ünalp-Arida, Aynur; Van Natta, Mark; Vaughn, Ivana; Wilson, Laura; Yates, Katherine
OBJECTIVE:Nonalcoholic fatty liver disease (NAFLD) affects 9.6% of children and may put these children at elevated risk of high blood pressure and subsequent cardiovascular morbidity and mortality. Therefore, we sought to determine the prevalence of and risk factors for high blood pressure in children with NAFLD. METHODS:Cohort study performed by the NIDDK NASH Clinical Research Network. There were 484 children with NAFLD ages 2 to 17 at enrollment; 382 children were assessed both at enrollment and 48 weeks afterwards. The main outcomes were high blood pressure at baseline and persistent high blood pressure at both baseline and 48 weeks. RESULTS:Prevalence of high blood pressure at baseline was 35.8% and prevalence of persistent high blood pressure was 21.4%. Children with high blood pressure were significantly more likely to have worse steatosis than children without high blood pressure (mild 19.8% vs. 34.2%, moderate 35.0% vs. 30.7%, severe 45.2% vs. 35.1%; P = 0.003). Higher body mass index, low-density lipoprotein, and uric acid were independent risk factors for high blood pressure (Odds Ratios: 1.10 per kg/m2, 1.09 per 10 mg/dL, 1.25 per mg/dL, respectively). Compared to boys, girls with NAFLD were significantly more likely to have persistent high blood pressure (28.4% vs.18.9%; P = 0.05). CONCLUSIONS:In conclusion, NAFLD is a common clinical problem that places children at substantial risk for high blood pressure, which may often go undiagnosed. Thus blood pressure evaluation, control, and monitoring should be an integral component of the clinical management of children with NAFLD.
PMCID:4242611
PMID: 25419656
ISSN: 1932-6203
CID: 5417122
Low Rate of Recurrent Primary Sclerosing Cholangitis in Pediatric Orthotopic Liver Transplant Recipients [Meeting Abstract]
Taylor, Sarah; Lobritto, Steven J.; Martinez, Mercedes; Vittorio, Jennifer; Griesemer, Adam; Kato, Tomoaki; Emond, Jean C.; Ovchinsky, Nadia
ISI:000344483804165
ISSN: 0270-9139
CID: 5397382
Success of meso-Rex bypass in the management of extrahepatic portal vein obstruction in children [Meeting Abstract]
Wehrman, Andrew; Ovchinsky, Nadia; Griesemer, Adam; Lobritto, Steven J.; Martinez, Mercedes; Kato, Tomoaki; Emond, Jean C.
ISI:000344483802144
ISSN: 0270-9139
CID: 5416662
Estimation of fish and omega-3 fatty acid intake in pediatric nonalcoholic fatty liver disease
St-Jules, David E; Watters, Corilee A; Brunt, Elizabeth M; Wilkens, Lynne R; Novotny, Rachel; Belt, Patricia; Lavine, Joel E; [Ovchinsky, Nadia]
AIMS/OBJECTIVE:Fish and omega-3 fatty acids are reported to be beneficial in pediatric nonalcoholic fatty liver disease (NAFLD), but no studies have assessed their relation to histological severity. The objectives of this study were to evaluate the dietary intake of fish and omega-3 fatty acids in children with biopsy-proven NAFLD, and examine their association with serological and histological indicators of disease. METHODS:This was a cross-sectional analysis of 223 children (6-18 years) who participated in the Treatment of Nonalcoholic Fatty Liver Disease in Children trial or the NAFLD Database study conducted by the Nonalcoholic Steatohepatitis Clinical Research Network. The distribution of fish and omega-3 fatty acid intake was determined from responses to the Block Brief 2000 Food Frequency Questionnaire, and analyzed for associations with serum alanine aminotransferase, histological features of fatty liver disease, and diagnosis of steatohepatitis after adjusting for demographic, anthropometric, and dietary variables. RESULTS:The minority of subjects consumed the recommended 8 ounces of fish per week (22/223 [10%]) and 200 mg of long-chain omega-3 fatty acids per day (12/223 [5%]). Lack of fish and long-chain omega-3 fatty acid intake was associated with greater portal (P = 0.03 and P = 0.10, respectively) and lobular inflammation (P = 0.09 and P = 0.004, respectively) after controlling for potential confounders. CONCLUSIONS:Fish and omega-3 fatty acid intake was insufficient in children with NAFLD, which may increase susceptibility to hepatic inflammation. Patients with pediatric NAFLD should be encouraged to consume the recommended amount of fish per week.
PMCID:3864540
PMID: 24177784
ISSN: 1536-4801
CID: 5417082
Vitamin E and changes in serum alanine aminotransferase levels in patients with non-alcoholic steatohepatitis
Hoofnagle, J H; Van Natta, M L; Kleiner, D E; Clark, J M; Kowdley, K V; Loomba, R; Neuschwander-Tetri, B A; Sanyal, A J; Tonascia, J; Abrams, Stephanie H; Angeli Fairly, Leanel; Brandt, Patricia; Bringman, Diane; Dasarathy, Jaividhya; Hawkins, Carol; Liu, Yao-Chang; Rogers, Nicholette; Stager, Margaret; Whitwell, Judy; McCullough, Arthur J; Dasarathy, Srinivasan; Pagadala, Mangesh; Sargent, Ruth; Yerian, Lisa; Zein, Claudia; Merriman, Raphael; Nguyen, Anthony; Mohan, Parvathi; Nair, Kavita; DeVore, Stephanie; Kohli, Rohit; Lake, Kathleen; Xanthakos, Stavra; Cosme, Yohaime; Lavine, Joel E; Mencin, Ali; Ovchinsky, Nadia; Abdelmalek, Manal F; Buie, Stephanie; Diehl, Anna Mae; Gottfried, Marcia; Guy, Cynthia; Hanna, Meryt; Kigongo, Christopher; Killenberg, Paul; Kwan, Samantha; Pan, Yi-Ping; Piercy, Dawn; Smith, Melissa; Srivastava, Savita; Byam, Elizabeth; Chalasani, Naga; Cummings, Oscar W; Ghabril, Marwan; Klipsch, Ann; Molleston, Jean P; Ragozzino, Linda; Subbarao, Girish; Tandra, Sweta; Vuppalanchi, Raj; Devadason, Caroline; Pfeifer, Kimberly; Scheimann, Ann; Torbenson, Michael; Kerkar, Nanda; Narayanappa, Sreevidya; Suchy, Frederick; Dunne, Katherine; Fishbein, Mark H; Jacques, Katie; Quinn, Ann; Riazi, Cindy; Whitington, Peter F; Barlow, Sarah; Derdoy, Jose; King, Debra; Morris, Andrea; Siegner, Joan; Stewart, Susan; Neuschwander-Tetri, Brent A; Thompson, Judy; Behling, Cynthia; Collins, Jennifer; Durelle, Janis; Hassanein, Tarek; Loomba, Rohit; Morgan, Anya; Rose, Steven; Patton, Heather; Schwimmer, Jeffrey B; Sirlin, Claude; Stein, Tanya; Aouizerat, Bradley; Bambha, Kiran; Bass, Marissa; Bass, Nathan M; Ferrell, Linda D; Filipowski, Danuta; Fleck, Shannon; Gu, Bo; Hameed, Bilal; Langlois, Camille; Pabst, Mark; Rosenthal, Monique; Rosenthal, Philip; Coffey, Melissa; Galdzicka, Sarah; Murray, Karen; Yeh, Matthew; Boyett, Sherry; Contos, Melissa J; Fuchs, Michael; Jones, Amy; Luketic, Velimir A C; Puri, Puneet; Sandhu, Bimalijit; Sanyal, Arun J; Sargeant, Carol; Noble, Kimberly; White, Melanie; Ackermann, Sarah; Kowdley, Kris V; Park, Jane; Pierce, Tracey; Mooney, Jody; Nelson, James; Shaw, Cheryl; Stead, Alice; Wang, Chia; Brunt, Elizabeth M; Kleiner, David E; Grave, Gilman D; Doo, Edward C; Hoofnagle, Jay H; Robuck, Patricia R; Sherker, Averell; Belt, Patricia; Brancati, Frederick L; Clark, Jeanne M; Colvin, Ryan; Donithan, Michele; Green, Mika; Hollick, Rosemary; Isaacson, Milana; Jin, Wana K; Lydecker, Alison; Mann, Pamela; May, Kevin P; Miriel, Laura; Sternberg, Alice; Tonascia, James; Ünalp-Arida, Aynur; Van Natta, Mark; Vaughn, Ivana; Wilson, Laura; Yates, Katherine
BACKGROUND:Non-alcoholic steatohepatitis (NASH) is a common cause of serum alanine aminotransferase (ALT) elevations and chronic liver disease, but it is unclear how well ALT elevations reflect the liver injury. AIM/OBJECTIVE:To assess how well changes in ALT elevations reflect improvements in liver histology in response to vitamin E therapy. METHODS:The vitamin E and placebo arms of the Pioglitazone vs. Vitamin E vs. Placebo in Non-alcoholic Steatohepatitis (PIVENS) trial were reassessed for associations among changes in ALT levels, body weight and liver histology. An ALT response was defined as a decrease to ≤40 U/L and by ≥30% of baseline. Liver biopsies taken before and after treatment were scored for non-alcoholic fatty liver disease activity (NAS) and fibrosis. RESULTS:ALT responses were more frequent among vitamin E (48%) than placebo (16%) recipients (P < 0.001). Among vitamin E recipients, ALT responses were associated with decreases in NAS (P < 0.001), but not fibrosis scores (P = 0.34), whereas among placebo recipients, ALT responses were associated with significant decreases in both (P < 0.05). Weight loss (≥2 kg) was also associated with ALT response (P < 0.001), improvements in NAS (P < 0.001) and fibrosis (P < 0.02), but vitamin E had an added effect both with and without weight loss. Weight gain (≥2 kg) was associated with lack of ALT response and worsening NAS and fibrosis scores in patients not on vitamin E. CONCLUSIONS:Decrease of ALT levels to normal in patients with NASH is usually associated with improved histological activity. Management should stress the value of weight loss and strongly discourage weight gain. Vitamin E can improve both ALT levels and histology with and without weight loss. CLINICAL TRIAL NUMBER/BACKGROUND:NCT00063622.
PMCID:3775262
PMID: 23718573
ISSN: 1365-2036
CID: 5417112