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1.
Liver Int ; 38(10): 1777-1784, 2018 10.
Artigo em Inglês | MEDLINE | ID: mdl-29325220

RESUMO

BACKGROUND & AIMS: There are no studies on acute kidney injury in paediatric acute-on-chronic liver failure. This study was planned with aim to describe the clinical presentation and outcome of acute kidney injury among paediatric acute-on-chronic liver failure patients. METHODS: Data of all children 1-18 years of age presenting with acute chronic liver failure (Asia pacific association for the study of the liver definition) was reviewed. Acute kidney injury was defined as per Kidney Diseases-Improving Global Outcomes guidelines. Poor outcome was defined as death or need for liver transplant within 3 months of development of acute kidney injury. RESULTS: A total of 84 children with acute-on-chronic liver failure were presented to us in the study period. Acute kidney injury developed in 22.6% of patients with acute-on-chronic liver failure. The median duration from acute-on-chronic liver failure to development of acute kidney injury was 4 weeks (Range: 2-10 weeks). The causes of acute kidney injury were hepatorenal syndrome (31.6%), sepsis (31.6%), nephrotoxic drugs (21%), dehydration (10.5%) and bile pigment related acute tubular necrosis in one patient. On univariate analysis, higher baseline bilirubin, higher international normalized ratio, higher paediatric end stage liver disease, presence of systemic inflammatory response syndrome and presence of spontaneous bacterial peritonitis had significant association with presence of acute kidney injury. On logistic regression analysis, presence of systemic inflammatory response syndrome (adjusted OR: 8.659, 95% CI: 2.18-34.37, P = .002) and higher baseline bilirubin (adjusted OR: 1.07, 95% CI: 1.008-1.135, P = .025) were independently associated with presence of acute kidney injury. Of the patients with acute kidney injury, 5(26.3%) survived with native liver, 10(52.6%) died and 4 (21.1%) underwent liver transplantation. CONCLUSION: Acute kidney injury developed in 22.6% of children with acute-on-chronic liver failure. Bilirubin more than 17.7 mg/dL and presence of systemic inflammatory response syndrome were high risk factors for acute kidney injury. Development of acute kidney injury in a child with acute-on-chronic liver failure suggests poor outcome and need for early intervention.


Assuntos
Injúria Renal Aguda/diagnóstico , Injúria Renal Aguda/etiologia , Insuficiência Hepática Crônica Agudizada/complicações , Adolescente , Criança , Pré-Escolar , Creatinina/sangue , Feminino , Síndrome Hepatorrenal/complicações , Humanos , Índia/epidemiologia , Lactente , Transplante de Fígado , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Estudos Retrospectivos , Fatores de Risco , Sepse/complicações , Análise de Sobrevida , Centros de Atenção Terciária
2.
J Pediatr Gastroenterol Nutr ; 67(4): e65-e72, 2018 10.
Artigo em Inglês | MEDLINE | ID: mdl-29901555

RESUMO

OBJECTIVES: There is limited literature on the spectrum of pediatric autoimmune liver disease (AILD, encompassing both autoimmune hepatitis/AIH and autoimmune sclerosing cholangitis [ASC]) in Asian populations and its diagnostic scores similarly require further validation. This work thus aimed to study the clinical characteristics, and to validate available diagnostic criteria in the local pediatric AILD cohort. METHODS: A review of all pediatric AILD cases, presenting over a 6-year (2011-2017) period was done, along with comparison of the available diagnostic scores: original (1999), simplified (2008) score, and new proposed (2017) score. RESULTS: A total of 85 subjects (AIH = 70 and ASC = 15) were diagnosed as having AILD. Majority of the cases in both groups presented with advanced hepatic disease (portal hypertension and/or hepatic decompensation). Overall 38 (44.7%) subjects had extrahepatic autoimmune disorders. Good outcome (survival with native liver with medically controllable disease), was seen in 80% AIH subjects, while poor outcome (death/need for liver transplantation or LT) was seen in 13% subjects, with similar results in the ASC cohort. All the 3 available scores had area under receiver operating characteristic (AUROC) curves exceeding 0.9 suggestive of excellent discrimination of AILD (to non-AILD patients), with no statistical difference between them (P >0.05). CONCLUSIONS: In Indian subcontinent, pediatric AILD subjects usually present with advanced hepatic disease, but may have a good outcome if timely therapy can be instituted. Associated autoimmune disorders should be carefully screened. There is no difference in the predictive value of the available diagnostic scores for pediatric AILD.


Assuntos
Colangite Esclerosante/epidemiologia , Hepatite Autoimune/epidemiologia , Adolescente , Doenças Autoimunes/epidemiologia , Criança , Pré-Escolar , Feminino , Humanos , Índia/epidemiologia , Masculino , Estudos Prospectivos
3.
Eur J Pediatr ; 177(6): 827-834, 2018 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-29504044

RESUMO

There are no evidence-based recommendations on the ideal dose and regimen for supplementation of vitamin D in children with chronic liver disease (CLD). This study aimed to compare the safety and efficacy of weekly and stoss regimens for treatment of vitamin D deficiency in these children. Children between the ages of 1 to 18 years with CLD and hypovitaminosis D defined by 25-OH vitamin D (25(OH)D) < 30µg/l were included. They were randomized to receive either stoss regimen (600,000 IU on day 1) or weekly (60,000 IU weekly) regimen of vitamin D. The 25(OH)D levels at 3 and 6 months were compared in the two groups. A total of 210 suspected cases of CLD were assessed for eligibility. Of a total of 67 children satisfying the inclusion criteria, 33 and 34 were randomized to receive stoss and weekly regimen, respectively. Final analysis included 28 children in each group. Clinical rickets was seen in 25.4% of children with hypovitaminosis D. The rise in levels of 25(OH)D at 3 months was higher with weekly regimen (34.3 ± 30.7 µg/l) as compared to stoss regimen (17.2 ± 11.5 µg/l) (p = 0.009). Rise at 6 months as compared to baseline was significantly higher with weekly regimen (30.7 ± 24µg/l) as compared to stoss regimen (11 ± 8.4 µg/l) (p < 0.001). Normal levels of 25(OH)D at 6 months were achieved in 24/28 (85.7%) of those receiving weekly regimen and 9/28 (32.1%) of those receiving stoss regimen (p < 0.001). With stoss therapy, 25(OH)D increased at 3 months as compared to baseline but thereafter dropped significantly at 6 months (p = 0.008). CONCLUSION: Weekly regimen of vitamin D supplementation is more effective than stoss regimen for treatment of hypovitaminosis D in children with CLD. Once normal levels are achieved, child should be shifted to 60,000 IU per month as maintenance dose. What is Known: • Vitamin D deficiency is more common and severe in children with chronic liver diseases. • Currently used doses fail to achieve normal vitamin D levels in these children. What is New? • Weekly regimen of 60,000 IU of vitamin D3 is the most effective regimen for treating vitamin D deficiency in children with CLD. • Children with CLD should further receive maintenance dose of 60,000 IU every month.


Assuntos
Hepatopatias/complicações , Deficiência de Vitamina D/tratamento farmacológico , Vitamina D/administração & dosagem , Vitaminas/administração & dosagem , Adolescente , Criança , Pré-Escolar , Doença Crônica , Suplementos Nutricionais , Esquema de Medicação , Feminino , Seguimentos , Humanos , Lactente , Masculino , Estudos Prospectivos , Resultado do Tratamento , Vitamina D/uso terapêutico , Deficiência de Vitamina D/diagnóstico , Deficiência de Vitamina D/etiologia , Vitaminas/uso terapêutico
4.
Eur J Pediatr ; 177(8): 1261-1269, 2018 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-29868931

RESUMO

We studied the etiological spectrum, clinicolaboratory and histological profile, and outcome of infants and children under 18 years of age presenting between December 2010 and May 2016 with histological evidence of paucity of intralobular bile ducts (PILBD, bile ducts to portal tract ratio < 0.6) Post-transplant PILBD was excluded. Of 632 pediatric liver biopsies screened, 70 had PILBD-44 were infants. PILBD was classified histologically into destructive (n = 50) and non-destructive PILBD (n = 20). Presentations were jaundice (98%), organomegaly (94%), pale stools (50%), and pruritus (43%). Infants had more cholestasis but less fibrosis on histology. Overall, 29 required liver transplantation (LT) for portal hypertension (n = 26), decompensation (n = 25), growth failure (n = 20), intractable pruritus (n = 5), and recurrent cholangitis (n = 2). Destructive PILBD has an odds for poor outcome (decompensation or need for LT within 1 year) of 1.53 (95% CI = 1.15-2.04). On binary logistic regression analysis, poor outcome was related to advanced fibrosis on liver biopsy [Exp (B) = 5.46, 95% CI = 1.56-19.04]. CONCLUSION: PILBD was present in 11% of pediatric liver biopsies and has a varied etiological spectrum. Destructive PILBD has poor outcome. Need for LT is guided by the presence of advanced fibrosis. What is Known: • Natural history of syndromic ductal paucity (Alagille syndrome) is complex. • Duct loss is commonly seen with late presentation of biliary atresia. What is New: • The study classifies the etiological spectrum of ductal paucity histologically into destructive and non-destructive. • Destructive duct loss carries poor prognosis regardless of the etiology of liver disease with subsequent need for liver transplantation.


Assuntos
Doenças dos Ductos Biliares/congênito , Doenças dos Ductos Biliares/diagnóstico , Ductos Biliares Intra-Hepáticos/anormalidades , Adolescente , Doenças dos Ductos Biliares/etiologia , Doenças dos Ductos Biliares/terapia , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Modelos Logísticos , Masculino , Prognóstico , Estudos Retrospectivos
5.
Liver Int ; 37(10): 1508-1514, 2017 10.
Artigo em Inglês | MEDLINE | ID: mdl-28111909

RESUMO

BACKGROUND & AIMS: The outcome of paediatric acute liver failure largely depends on age and aetiology. The aim of this work was to study the aetiological spectrum and outcome of the paediatric acute liver failure cases. METHODS: This prospective observational study included all children (<18 years age) fulfilling paediatric acute liver failure study group definition. Aetiological evaluation was done and predictive factors for poor outcome (death or liver transplantation) were analysed. RESULTS: There were 109 children in total. The commonest aetiology was viral infections (50, 45.8%) followed by metabolic liver diseases (14, 13.2%) and drug-induced liver injury (12, 11%). Viral, indeterminate and drug-induced liver injury group were older in age, had higher international normalized ratio and alanine transaminases in comparison with those with metabolic liver diseases and other aetiologies (P<.05). At 90 days from presentation, 52 (47.7%) children survived with native liver. On multivariate analysis, jaundice to encephalopathy interval >7 days (adjusted OR: 9.16, 95% CI: 1.55-53) and higher paediatric/model for end-stage liver disease scores at 72 hours (adjusted OR: 1.2, 95% CI: 1.08-1.32) were associated with poor outcome. CONCLUSION: Viral infections, indeterminate and drug-induced liver injury-related paediatric acute liver failure usually present in older children with higher international normalized ratio and alanine transaminases. Jaundice to encephalopathy interval >7 days and paediatric/model for end stage liver disease score >24 at 72 hours are associated with poor outcome.


Assuntos
Falência Hepática Aguda/etiologia , Adolescente , Fatores Etários , Doença Hepática Induzida por Substâncias e Drogas/etiologia , Criança , Pré-Escolar , Feminino , Encefalopatia Hepática/etiologia , Humanos , Índia , Lactente , Recém-Nascido , Icterícia/etiologia , Falência Hepática Aguda/diagnóstico , Falência Hepática Aguda/mortalidade , Falência Hepática Aguda/cirurgia , Transplante de Fígado , Masculino , Análise Multivariada , Razão de Chances , Valor Preditivo dos Testes , Estudos Prospectivos , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento , Viroses/complicações
6.
J Pediatr Gastroenterol Nutr ; 64(5): 748-753, 2017 05.
Artigo em Inglês | MEDLINE | ID: mdl-28437324

RESUMO

OBJECTIVES: Noncirrhotic portal fibrosis (NCPF) has been classically described as a disease of young to middle age with limited literature regarding its occurrence, onset, or clinical presentation in children. We hereby present a series of 19 patients diagnosed and managed as NCPF in pediatric age group. METHODS: A retrospective review of all the patients presenting to the pediatric hepatology department (age <18 years) and diagnosed as NCPF was done and data were evaluated. RESULTS: A total of 19 patients were diagnosed as NCPF with median age at onset of symptoms and diagnosis as 10 years and 13.8 years respectively. Majority presented with left upper quadrant discomfort or mass. Laboratory parameters showed hypersplenism in majority with preserved liver synthetic functions. Median values for hepatic venous pressure gradient and liver stiffness measurement were 13.5 mmHg and 10.6 kPa, respectively. Classical hepatic histopathological features seen were maintained lobular architecture, atretic portal tracts, approximation of portal-portal and portal-central areas, and aberrant peripheral portal channels. During follow-up, majority of the patients did not show disease progression. CONCLUSIONS: NCPF is not an uncommon entity in pediatric population with age of onset in early second decade. Hepatic histopathology must be used to exclude cirrhosis and to confirm the diagnosis. Hepatic venous pressure gradient and liver stiffness measurement values, in some cases, may overlap with those in patients with cirrhosis and may not be diagnostic in isolation. Any patient presenting with evidence of portal hypertension with preserved hepatic functions, irrespective of the age, should be evaluated for possible NCPF.


Assuntos
Hipertensão Portal/diagnóstico , Hipertensão Portal/patologia , Sistema Porta/patologia , Adolescente , Criança , Pré-Escolar , Progressão da Doença , Feminino , Fibrose , Seguimentos , Humanos , Fígado/patologia , Masculino , Estudos Retrospectivos
7.
J Pediatr Gastroenterol Nutr ; 64(6): 869-875, 2017 06.
Artigo em Inglês | MEDLINE | ID: mdl-28045774

RESUMO

OBJECTIVES: Fatty acid oxidation defects (FAODs) may underlie or modify the course of acute liver failure (ALF). Overall significance of carnitine/acylcarnitine and amino acid profile in ALF is similarly undetermined. Thus, this study was undertaken to study the abnormalities in carnitine/acylcarnitine and amino acid profile in ALF. METHODS: A prospective study was performed including all patients with ALF, and detailed evaluation including metabolic testing was done. RESULTS: A total of 55 patients (33 pediatric and 22 adult patients) were included in the study. Three patients (a 1-year 6-month-old child, a 13-year-old adolescent, and a 21-year-old adult, ie, 5.5% of all) were identified for the study with underlying metabolic etiology, that is, carnitine palmitoyl transferase-1 deficiency, based on the abnormal carnitine/acylcarnitine profile. Almost three-fourths of patients (78%) had evidence of serum hyperaminoacidemia. Thirty-one patients (56%) had evidence of abnormal carnitine/acylcarnitine profile with predominant abnormality being low free carnitine (C0). Higher levels of serum tyrosine (P = 0.002) and lower levels of serum C0 (P = 0.032) in children and higher levels of serum phenyalanine (P = 0.047) in adults predicted poor outcome (death/liver transplant) on univariate analysis. CONCLUSIONS: FAODs are not uncommon in ALF with a suggested prevalence of approximately 5.5%. FAODs can cause ALF or modify the natural course of ALF caused by other etiologies. Serum hyperaminoacidemia and low serum free carnitine may predict poor outcome in patients with acute liver failure.


Assuntos
Aminoácidos/sangue , Carnitina O-Palmitoiltransferase/deficiência , Carnitina/análogos & derivados , Carnitina/sangue , Hipoglicemia/complicações , Erros Inatos do Metabolismo Lipídico/complicações , Falência Hepática Aguda/etiologia , Adolescente , Adulto , Biomarcadores/sangue , Carnitina O-Palmitoiltransferase/sangue , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Hipoglicemia/sangue , Hipoglicemia/diagnóstico , Lactente , Erros Inatos do Metabolismo Lipídico/sangue , Erros Inatos do Metabolismo Lipídico/diagnóstico , Falência Hepática Aguda/sangue , Falência Hepática Aguda/mortalidade , Falência Hepática Aguda/cirurgia , Transplante de Fígado , Masculino , Gravidade do Paciente , Estudos Prospectivos
8.
J Pediatr Gastroenterol Nutr ; 63(4): 400-5, 2016 10.
Artigo em Inglês | MEDLINE | ID: mdl-26967824

RESUMO

OBJECTIVES: The aim of the study was to assess the prevalence, profile, outcome, and predictive factors of pediatric acute-on-chronic liver failure (ACLF). METHODS: All children 3 months to 18 years satisfying the Asia Pacific Association for the Study of Liver Diseases definition of ACLF were included. Data were both extracted from records (January 2011 to December 2014) and prospectively collected (January to October 2015). Successful outcome was defined as survival with native liver at 90 days, whereas poor outcome included those who died or received liver transplantation. RESULTS: Of the 499 children with chronic liver disease (CLD), 56 (11.2%) presented as ACLF, with a mean age of 9.35 (±4.39) years. Wilson disease and autoimmune hepatitis were the commonest underlying CLDs accounting for 24 (42.8%) and 18 (32.1%) cases, respectively. The most frequent events precipitating ACLF were a flare up of the underlying disease in 27 (48.2%) and acute viral hepatitis in 17 (30%). Poor outcome occurred in 22 (39.3%) children: 17 (30.4%) died and 5 (8.9%) received liver transplantation. Poor outcome was associated with grades 3 to 4 hepatic encephalopathy, bilirubin ≥17.5, international normalized ratio ≥3.5, and presence of 2 or more organ failures. On multivariate analysis, a Chronic Liver Failure-Sequential Organ Failure Assessment score ≥10 best predicted mortality (odds ratio 20.45, 95% confidence interval 3.9-106.7). CONCLUSIONS: ACLF is present in 11.2% of childhood CLD, with a 90-day native liver survival of 61%. A Chronic Liver Failure-Sequential Organ Failure Assessment score of ≥10 best predicts mortality at day 90.


Assuntos
Insuficiência Hepática Crônica Agudizada , Insuficiência Hepática Crônica Agudizada/diagnóstico , Insuficiência Hepática Crônica Agudizada/epidemiologia , Insuficiência Hepática Crônica Agudizada/etiologia , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Índia/epidemiologia , Lactente , Estimativa de Kaplan-Meier , Masculino , Análise Multivariada , Prevalência , Prognóstico , Fatores de Risco
9.
Indian J Plast Surg ; 47(2): 242-8, 2014 May.
Artigo em Inglês | MEDLINE | ID: mdl-25190922

RESUMO

BACKGROUND: Blood volume measurement though important in management of critically ill-patients is not routinely estimated in clinical practice owing to labour intensive, intricate and time consuming nature of existing methods. AIMS: The aim was to compare blood volume estimations using trivalent chromium [(51)Cr(III)] and standard Evans blue dye (EBD) method in New Zealand white rabbit models and establish correction-factor (CF). MATERIALS AND METHODS: Blood volume estimation in 33 rabbits was carried out using EBD method and concentration determined using spectrophotometric assay followed by blood volume estimation using direct injection of (51)Cr(III). Twenty out of 33 rabbits were used to find CF by dividing blood volume estimation using EBD with blood volume estimation using (51)Cr(III). CF is validated in 13 rabbits by multiplying it with blood volume estimation values obtained using (51)Cr(III). RESULTS: The mean circulating blood volume of 33 rabbits using EBD was 142.02 ± 22.77 ml or 65.76 ± 9.31 ml/kg and using (51)Cr(III) was estimated to be 195.66 ± 47.30 ml or 89.81 ± 17.88 ml/kg. The CF was found to be 0.77. The mean blood volume of 13 rabbits measured using EBD was 139.54 ± 27.19 ml or 66.33 ± 8.26 ml/kg and using (51)Cr(III) with CF was 152.73 ± 46.25 ml or 71.87 ± 13.81 ml/kg (P = 0.11). CONCLUSIONS: The estimation of blood volume using (51)Cr(III) was comparable to standard EBD method using CF. With further research in this direction, we envisage human blood volume estimation using (51)Cr(III) to find its application in acute clinical settings.

10.
J Pediatr Gastroenterol Nutr ; 57(2): 161-6, 2013 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-23518487

RESUMO

BACKGROUND AND OBJECTIVE: Congenital hepatic fibrosis (CHF) and Caroli syndrome are frequently associated with renal cystic diseases. They have a variable clinical course, and the natural history is not well defined despite molecular advances. Our study describes the clinical manifestations and long-term outcome in children with this disorder. METHODS: A retrospective case review of children with CHF at a single centre diagnosed on the basis of clinical features, radiological and endoscopic evidence of portal hypertension (PHT), and compatible histopathological findings. Children were categorised based on hepatic phenotype-group 1 (Caroli syndrome) and group 2 (CHF). Hepatobiliary as well as renal manifestations were recorded at presentation, and their evolution followed up until transplant or last follow-up. RESULTS: There were 40 children (22 boys) with a median age of 1.3 years at clinical presentation. Fourteen of 40 (35%) children presented in the neonatal period with primarily renal disease, of whom 11 (78%) had Caroli syndrome (P = 0.02). Significant PHT with oesophageal varices was seen in 86%, with no difference in the incidence of gastrointestinal bleeding and varices between Caroli syndrome and CHF. Cholangitis developed in 10 of 40 (25%) and was more common in the Caroli syndrome group (P = 0.009). A higher proportion of children with Caroli syndrome developed chronic kidney disease (CKD) stage 3 and above as compared with CHF (85% vs 42%; P = 0.007). Twelve of 21 (57%) and 8 of 19 (42%) children in the Caroli syndrome and CHF groups required either combined liver-kidney or isolated liver transplant, with the most common indication for renal transplantation being end-stage renal disease (CKD5d) with or without advanced PHT or cholangitis. All 14 (100%) children with neonatal presentation developed CKD5d and required combined liver-kidney transplant before 14 years of age, whereas 77% of children presenting beyond the neonatal period survived without liver-kidney transplant (P < 0.001). Neonatal presentation was the best predictor of the need for transplant. CONCLUSIONS: Caroli syndrome is more likely to present in the neonatal period and these patients are more likely to develop CKD5d. CKD stage 3 or above with recurrent cholangitis is more common in Caroli syndrome presenting beyond the neonatal period and adds to the significant morbidity in these patients. Children presenting in the neonatal period have a more severe phenotype and should be considered early for combined liver-kidney transplant.


Assuntos
Doença de Caroli , Doenças Genéticas Inatas , Hipertensão Portal/etiologia , Falência Renal Crônica/etiologia , Rim/patologia , Cirrose Hepática , Fígado/patologia , Rim Policístico Autossômico Recessivo , Adolescente , Doença de Caroli/complicações , Doença de Caroli/epidemiologia , Doença de Caroli/patologia , Doença de Caroli/cirurgia , Criança , Pré-Escolar , Colangite/epidemiologia , Colangite/etiologia , Colangite/genética , Varizes Esofágicas e Gástricas/epidemiologia , Varizes Esofágicas e Gástricas/etiologia , Varizes Esofágicas e Gástricas/genética , Feminino , Doenças Genéticas Inatas/complicações , Doenças Genéticas Inatas/epidemiologia , Doenças Genéticas Inatas/patologia , Doenças Genéticas Inatas/cirurgia , Humanos , Hipertensão Portal/epidemiologia , Hipertensão Portal/genética , Lactente , Recém-Nascido , Falência Renal Crônica/epidemiologia , Falência Renal Crônica/genética , Transplante de Rim , Cirrose Hepática/complicações , Cirrose Hepática/epidemiologia , Cirrose Hepática/patologia , Cirrose Hepática/cirurgia , Transplante de Fígado , Masculino , Fenótipo , Rim Policístico Autossômico Recessivo/complicações , Rim Policístico Autossômico Recessivo/epidemiologia , Rim Policístico Autossômico Recessivo/patologia , Rim Policístico Autossômico Recessivo/cirurgia , Prevalência , Insuficiência Renal Crônica/epidemiologia , Insuficiência Renal Crônica/etiologia , Estudos Retrospectivos
11.
Langmuir ; 28(3): 1824-32, 2012 Jan 24.
Artigo em Inglês | MEDLINE | ID: mdl-22168522

RESUMO

We present the results of Monte Carlo simulations of the adsorption of single-component ethane and ethylene and of equimolar mixtures of these two gases on bundles of closed, single-walled carbon nanotubes. Two types of nanotube bundles were used in the simulations: homogeneous (i.e., those in which all the nanotubes have identical diameters) and heterogeneous (those in which nanotubes of different diameters are allowed). We found that at the same pressure and temperature more ethane than ethylene adsorbs on the bundles over the entire range of pressures and temperatures explored. The simulation results for the equimolar mixtures show that the pressure at which maximum separation is attained is a very sensitive function of the diameter of the nanotubes present in the bundles. Simulations using heterogeneous bundles yield better agreement with single-component experimental data for isotherms and isosteric heats than those obtained from simulations using homogeneous bundles. Possible applications of nanotubes in gas separation are discussed. We explored the effect of the diameter of the nanotubes on the separation ability of these sorbents, both for the internal and for the external sites. We found that substrate selectivity is a decreasing function of temperature.

13.
J Pediatr Gastroenterol Nutr ; 53(5): 478-9, 2011 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-21701407

RESUMO

6-Thioguanine (6-TG) therapy in childhood acute lymphoblastic leukaemia results in chronic hepatotoxicity and portal hypertension. We report follow-up data in a cohort of 10 children with acute lymphoblastic leukaemia and 6-TG-related hepatotoxicity described initially in 2006. Clinically significant portal hypertension was present in the majority of patients several years after cessation of 6-TG treatment. These data reflect the natural history of noncirrhotic portal hypertension and emphasises the need to incorporate ongoing surveillance in the transition arrangement to adult services in this select group of patients.


Assuntos
Doença Hepática Crônica Induzida por Substâncias e Drogas/patologia , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamento farmacológico , Tioguanina/administração & dosagem , Tioguanina/efeitos adversos , Adolescente , Criança , Estudos de Coortes , Feminino , Seguimentos , Humanos , Hipertensão Portal/induzido quimicamente , Hipertensão Portal/patologia , Masculino , Adulto Jovem
17.
MethodsX ; 6: 1068-1071, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31193381

RESUMO

Though often used in cardiac intensive monitoring set up, simultaneous evaluation of several variables like CVP, PCWP, SVV and hTEE for fluid volume resuscitation (especially when capillary permeability is major problem than cardiac performance) is a major challenge in many ICU setups. Therefore, repetitive determination of blood volume by trivalent chromium [51Cr (III)] as a direct single variable method may be a near ideal method during fluid volume resuscitation in cases where capillary permeability is a major problem (e.g. Burns). Hence, in the present article the repeatability and reliability of 51Cr (III) method in New Zealand white rabbits was explored. Mean blood volume values of initial measurement were 195.66 ± 47.30 ml or 89.81 ± 17.88 ml/kg body weight. Repeated mean blood volume values, measured after 1 h, was 181.98 ± 53.16 ml or 83.68 ± 22.09 ml/kg body weight. The average difference between the initial and repeated measurements was 10.93 ml (95% CI -3.33, 25.19 ml), which is not statistically significant (P = 0.128). The method using 51Cr (III) for repeat blood volume measurements after sixty minutes in rabbits is a reliable method. •Rapid•Repeatable•Reliable.

18.
Indian Pediatr ; 55(7): 561-567, 2018 07 15.
Artigo em Inglês | MEDLINE | ID: mdl-30129536

RESUMO

OBJECTIVE: To find association of pediatric NAFLD with metabolic risk factors, and Patatin-like phospholipase domain-containing protein 3 (PNPLA3) gene polymorphism. DESIGN: Cross-sectional study. SETTING: Pediatric Hepatology unit of a tertiary care hospital. PARTICIPANTS: Overweight/obese children (<18 years) with (69 patients) or without (30 patients) NAFLD (ultrasonography based), and their parents. INTERVENTION: Metabolic screening, PNPLA3 gene polymorphism, and transient elastography. OUTCOME MEASURE: Association of pediatric NAFLD with parental metabolic risk factors and PNPLA3 gene polymorphism. RESULTS: In the NAFLD group, there was high parental incidence of metabolic diseases, fatty liver (80%) and low high-density lipoproteins levels (84%). Family history of NAFLD (in any parent), higher alanine aminotransferase levels and higher total cholesterol levels in the child independently predicted possibility of NAFLD, but similar results could not be replicated for PNPLA3 gene polymorphism. Controlled attenuation parameter measurement (by transient elastography) had high sensitivity and specificity to diagnose steatosis. CONCLUSIONS: There is high familial incidence of metabolic diseases in children with NAFLD. Controlled attenuation parameter can be useful as a non-invasive modality to screen fatty liver in children.


Assuntos
Predisposição Genética para Doença , Lipase/genética , Anamnese , Proteínas de Membrana/genética , Doenças Metabólicas/genética , Hepatopatia Gordurosa não Alcoólica/genética , Polimorfismo de Nucleotídeo Único , Adolescente , Criança , Estudos Transversais , Feminino , Marcadores Genéticos , Humanos , Masculino , Doenças Metabólicas/diagnóstico , Análise Multivariada , Hepatopatia Gordurosa não Alcoólica/diagnóstico , Pais , Estudos Prospectivos , Fatores de Risco
19.
Indian J Gastroenterol ; 37(4): 326-334, 2018 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-30168073

RESUMO

BACKGROUND: Our aim was to evaluate the efficacy and safety of sequential therapy using pegylated interferon (Peg-IFN) and nucleos(t)ide analogue (NA) for treatment of children in immunoactive (IA) and immunotolerant (IT) phases of chronic hepatitis B. METHODS: It was a prospective observational study where those willing for sequential therapy were allocated to group 1 (sequential therapy) and others to group 2 (standard therapy). Sequential therapy included 8 weeks of NA followed by 44 weeks of combination of NA and Peg-IFN. In group 2, IA children received NA monotherapy, and IT children received no therapy. HBe seroconversion, HBs seroconversion, and loss of HBV DNA were the major outcome measures. RESULTS: A total of 61 children (36 IA and 25 IT) were included in the analysis. Among the IA children, 17 received sequential therapy and 19 received standard therapy; whereas, among the IT children, 12 received sequential therapy and 13 did not receive any therapy. In IA phase, sequential therapy led to higher HBe seroconversion (64.7% vs. 21.05%, p = 0.017) and higher virological clearance (94.12% vs. 52.63%, p = 0.008). In IT children, there was no benefit of treatment with sequential therapy over observation alone. Baseline ALT > 100 IU/L predicted response to therapy with 100% sensitivity, 89.5% specificity, and LR+ of 9.52. CONCLUSION: Sequential therapy leads to higher HBe seroconversion and virological response in children in IA phase. Children with baseline ALT > 100 IU/mL are more likely to respond to sequential therapy. There appears to be no role of sequential therapy in children in IT phase.


Assuntos
Antivirais/administração & dosagem , Antígenos E da Hepatite B/imunologia , Hepatite B Crônica/tratamento farmacológico , Hepatite B Crônica/imunologia , Interferon-alfa/administração & dosagem , Polietilenoglicóis/administração & dosagem , Adolescente , Criança , Pré-Escolar , Quimioterapia Combinada , Feminino , Anticorpos Anti-Hepatite B/sangue , Humanos , Interferon alfa-2 , Lamivudina/administração & dosagem , Masculino , Nucleosídeos/administração & dosagem , Estudos Prospectivos , Proteínas Recombinantes/administração & dosagem , Soroconversão , Tenofovir/administração & dosagem
20.
Indian J Gastroenterol ; 37(3): 266-270, 2018 May.
Artigo em Inglês | MEDLINE | ID: mdl-29744756

RESUMO

The concept of irreversibility of cirrhosis has been challenged in the recent past with literature in this regard, albeit still scarce, now being accumulated across all age groups, etiologies, and geographical regions. This small series of nine pediatric cases elegantly recapitulates the concept of regression of hepatic fibrosis/cirrhosis and paves way for further detailed studies to enable development of therapeutic anti-fibrotic modalities in future.


Assuntos
Cirrose Hepática/patologia , Hepatopatias/patologia , Fígado/patologia , Adolescente , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Fibrose , Humanos , Lactente , Cirrose Hepática/etiologia , Hepatopatias/etiologia , Masculino
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