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  1. Article ; Online: Midodrine reduces new-onset acute kidney injury and hyponatremia in children with cirrhosis and ascites awaiting liver transplantation: Results from an open-label RCT.

    Ashritha, A / Lal, Bikrant B / Khanna, Rajeev / Sood, Vikrant / Sood, Arun K / Alam, Seema

    Journal of pediatric gastroenterology and nutrition

    2023  Volume 78, Issue 2, Page(s) 350–359

    Abstract: Objectives: Midodrine, an oral α-1-adrenergic receptor agonist, counters arterial hypovolemia and reduces complications in adult patients with cirrhosis. This randomized controlled trial (RCT) aimed to assess the efficacy and safety of midodrine in ... ...

    Abstract Objectives: Midodrine, an oral α-1-adrenergic receptor agonist, counters arterial hypovolemia and reduces complications in adult patients with cirrhosis. This randomized controlled trial (RCT) aimed to assess the efficacy and safety of midodrine in preventing complications and improving survival in children with cirrhosis and ascites who are awaiting liver transplantation (LT).
    Methods: This open-label RCT conducted from January 2022 to May 2023 included children under 18 years with cirrhosis and ascites. Patients were randomized to receive either midodrine plus standard medical therapies (SMTs) or SMT alone. The primary outcome measure was the incidence of cirrhosis-related complications within 6 months.
    Results: Thirty-five subjects were enrolled and randomized. Patients in the midodrine arm had a lower incidence of new-onset acute kidney injury (AKI) compared with the SMT arm (11.1% vs. 41.2%). Patients in the midodrine arm showed a decline in serum creatinine and improvement in glomerular filtration rate, whereas no changes were observed in the SMT arm. There was a lower incidence of new-onset hyponatremia in the midodrine arm (20% vs. 56%). Midodrine led to reduction in plasma rennin activity (PRA) and improvement in systemic hemodynamics. There was no difference in the rate of resolution of ascites, recurrence of ascites, requirement of therapeutic paracentesis, cumulative albumin infusion requirement, episodes of spontaneous bacterial peritonitis, and hepatic encephalopathy between the two arms.
    Conclusion: Midodrine, when added to SMT, was effective in reducing the incidence of new-onset AKI and hyponatremia in pediatric cirrhotics awaiting LT. It also improved systemic hemodynamics and showed a trend towards reducing PRA.
    MeSH term(s) Adult ; Humans ; Child ; Adolescent ; Midodrine/therapeutic use ; Liver Transplantation/adverse effects ; Ascites/drug therapy ; Ascites/etiology ; Hyponatremia/complications ; Hyponatremia/drug therapy ; Treatment Outcome ; Liver Cirrhosis/complications ; Liver Cirrhosis/surgery ; Acute Kidney Injury/etiology ; Acute Kidney Injury/prevention & control
    Chemical Substances Midodrine (6YE7PBM15H)
    Language English
    Publishing date 2023-12-11
    Publishing country United States
    Document type Randomized Controlled Trial ; Journal Article
    ZDB-ID 603201-1
    ISSN 1536-4801 ; 0277-2116
    ISSN (online) 1536-4801
    ISSN 0277-2116
    DOI 10.1002/jpn3.12077
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: Percutaneous Cholecystocholangiography-A Tool to Conclusively Exclude Biliary Atresia.

    Ashritha, A / Gautam, Vipul / Lal, Bikrant Bihari / Mukund, Amar / Vijay, Priti / Khanna, Rajeev / Sood, Vikrant / Alam, Seema

    Indian journal of pediatrics

    2022  Volume 89, Issue 11, Page(s) 1144–1147

    Abstract: The objective of this study was to evaluate the feasibility, safety, and diagnostic accuracy of percutaneous cholecystocholangiography (PCC) in cases of conjugated hyperbilirubinemia in which biliary atresia (BA) could not be diagnosed or ruled out based ...

    Abstract The objective of this study was to evaluate the feasibility, safety, and diagnostic accuracy of percutaneous cholecystocholangiography (PCC) in cases of conjugated hyperbilirubinemia in which biliary atresia (BA) could not be diagnosed or ruled out based on clinical, radiological, and histopathological findings. This was a retrospective, chart review of all cholestatic infants who underwent PCC within the last 5 y. PCC was performed via the transhepatic route using 23-g needle. The patency of both the proximal and distal biliary trees was assessed. PCC was technically feasible in 12/13 (92.3%) of infants without any procedure-related complications. PCC demonstrated proximal and distal biliary patencyin 7/12 (58.3%) infants, thereby avoiding unnecessary laparotomy in them. PCC failed to demonstrate biliary patency in 5 infants; of which, 4 were confirmed as cases of BA on laparotomy. PCC can correctly differentiate BA from non-BA cases of conjugated hyperbilirubinemia preoperatively, reducing the negative laparotomy rates.
    MeSH term(s) Biliary Atresia/diagnostic imaging ; Biliary Atresia/surgery ; Cholangiography ; Cholestasis/complications ; Cholestasis/diagnostic imaging ; Cholestasis/surgery ; Humans ; Hyperbilirubinemia/complications ; Hyperbilirubinemia/surgery ; Infant ; Portoenterostomy, Hepatic ; Retrospective Studies
    Language English
    Publishing date 2022-09-20
    Publishing country India
    Document type Journal Article
    ZDB-ID 218231-2
    ISSN 0973-7693 ; 0019-5456
    ISSN (online) 0973-7693
    ISSN 0019-5456
    DOI 10.1007/s12098-022-04354-2
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  3. Article: Study of Adherence to Medication in Pediatric Liver Diseases ("SAMPLD" Study) in Indian Children.

    Suchismita, Arya / Ashritha, A / Sood, Vikrant / Lal, Bikrant B / Khanna, Rajeev / Kumar, Guresh / Alam, Seema

    Journal of clinical and experimental hepatology

    2022  Volume 13, Issue 1, Page(s) 22–30

    Abstract: Background/objectives: Adherence to medication(s) is an essential component of holistic management in any chronic disease including in post liver transplant (LT) patients. Thus, this study aimed to assess adherence to medications in Indian pediatric ... ...

    Abstract Background/objectives: Adherence to medication(s) is an essential component of holistic management in any chronic disease including in post liver transplant (LT) patients. Thus, this study aimed to assess adherence to medications in Indian pediatric liver disease patients (including post LT recipients) and to identify variables affecting its occurrence.
    Methods: A cross-sectional study was conducted among pediatric (<18 years of age) subjects with Wilson disease (WD) and autoimmune liver disease (AILD) along with post LT recipients from May 2021 to October 2021. Structured tools using prevalidated questionnaires (
    Results: A total of 152 children were included in the study (WD 39.5%, AILD 32.9%, and post LT 27.6%). Prevalence of missed and late dose nonadherence (at a cut-off of ≥20%) was 12.5% and 16.4%, respectively. Older age (odd's ratio/O.R 1.185), stay in a rural area (O.R 5.08), and barriers like
    Conclusions: Overall, nonadherence was seen in 12-16%, i.e., around one-sixth of the patients, with least nonadherence seen in post LT recipients (0-2.4%). Older age of the patient, rural place of stay and personal barriers like hard to remember/forgetfulness and bad medication taste were identified as factors independently leading to nonadherence.
    Language English
    Publishing date 2022-10-17
    Publishing country India
    Document type Journal Article
    ISSN 0973-6883
    ISSN 0973-6883
    DOI 10.1016/j.jceh.2022.10.006
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  4. Article ; Online: Evaluation of Bone Mineral Density in Children With Hemophilia: An Observational Case-Control Study.

    Ashritha, A / Delhi Kumar, C G / Sahoo, Jayaprakash / Nalini, P

    Journal of pediatric hematology/oncology

    2019  Volume 41, Issue 7, Page(s) 511–514

    Abstract: Objectives: The objectives of this study were to study bone mineral content (BMC), bone mineral density (BMD), vitamin D level, and bone turnover markers in children with hemophilia and compare it with their normal counterparts.: Design: This was an ... ...

    Abstract Objectives: The objectives of this study were to study bone mineral content (BMC), bone mineral density (BMD), vitamin D level, and bone turnover markers in children with hemophilia and compare it with their normal counterparts.
    Design: This was an observational case-control study.
    Setting: This study was conducted in our tertiary care institute during the period spanning from September 2016 to June 2018.
    Materials and methods: A total of 38 children with hemophilia 2 to 18 years of age and 38 age-matched and sex-matched healthy controls were included. Children with symptomatic hypocalcemia and those receiving drugs affecting BMC and BMD were excluded. Dual-energy x-ray absorptiometry was performed to estimate BMC and BMD. 25-Hydroxyvitamin D [25(OH)D], intact parathormone, osteocalcin, calcium, phosphate and alkaline phosphatase, and spot urine pyrilinks-D/creatinine ratio were estimated in them.
    Results: BMC and BMD in cases was lower than that in controls (P<0.05). Prevalence of low BMC was seen in 22 (58%) and low BMD in 8 (21%) of cases. All controls had normal BMC and BMD for age. The prevalence of low vitamin D level (<20 ng/mL) was seen in 36 (95%) among cases and in 25 (65%) among controls (P<0.001). Serum phosphorus was lower, and serum alkaline phosphatase was higher in cases than in controls (P<0.05).
    Conclusion: BMC, BMD, and vitamin D in hemophiliacs were lower than in the general population.
    MeSH term(s) Bone Density/physiology ; Case-Control Studies ; Child ; Child, Preschool ; Female ; Hemophilia A/complications ; Humans ; Male
    Language English
    Publishing date 2019-07-23
    Publishing country United States
    Document type Journal Article ; Observational Study ; Research Support, Non-U.S. Gov't
    ZDB-ID 1231152-2
    ISSN 1536-3678 ; 1077-4114 ; 0192-8562
    ISSN (online) 1536-3678
    ISSN 1077-4114 ; 0192-8562
    DOI 10.1097/MPH.0000000000001554
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  5. Article ; Online: Genotype correlates with clinical course and outcome of children with tight junction protein 2 (TJP2) deficiency-related cholestasis.

    Lal, Bikrant Bihari / Alam, Seema / Sibal, Anupam / Kumar, Karunesh / Hosaagrahara Ramakrishna, Somashekara / Shah, Vaibhav / Dheivamani, Nirmala / Bavdekar, Ashish / Nagral, Aabha / Wadhwa, Nishant / Maria, Arjun / Shah, Aashay / Shah, Ira / Nalwalla, Zahabiya / Snehavardhan, Pandey / Srikanth, K P / Gupta, Subhash / Sivaramakrishnan, Viswanathan M / Waikar, Yogesh /
    Suchismita, Arya / Ashritha, A / Sood, Vikrant / Khanna, Rajeev

    Hepatology (Baltimore, Md.)

    2024  

    Abstract: Background and aims: The study aimed to describe the clinical course and outcomes, and analyze the genotype-phenotype correlation in patients with tight junction protein 2 (TJP2) deficiency.: Approach and results: Data from all children with chronic ... ...

    Abstract Background and aims: The study aimed to describe the clinical course and outcomes, and analyze the genotype-phenotype correlation in patients with tight junction protein 2 (TJP2) deficiency.
    Approach and results: Data from all children with chronic cholestasis and either homozygous or compound heterozygous mutations in TJP2 were extracted and analyzed. The patients were categorized into 3 genotypes: TJP2-A (missense mutations on both alleles), TJP2-B (missense mutation on one allele and a predicted protein-truncating mutation [PPTM] on the other), and TJP2-C (PPTMs on both alleles). A total of 278 cases of genetic intrahepatic cholestasis were studied, with TJP2 deficiency accounting for 44 cases (15.8%). Of these, 29 were homozygous and 15 were compound heterozygous variants of TJP2 . TJP2-A genotype was identified in 21 (47.7%), TJP2-B in 7 cases (15.9%), and TJP2-C in 16 cases (36.4%), respectively. Patients with the TJP2-C genotype were more likely to experience early infantile cholestasis (87.5% vs. 53.5%, p =0.033), less likely to clear jaundice (12.5% vs. 52.2%, p =0.037), more likely to develop ascites, and had higher serum bile acids. Patients with the TJP2-C genotype were more likely to die or require liver transplantation (native liver survival: 12.5% vs. 78.6%, p <0.001), with a median age at death/liver transplantation of 2.5 years. Cox regression analysis revealed that TJP2-C mutations ( p =0.003) and failure to resolve jaundice ( p =0.049) were independent predictors of poor outcomes.
    Conclusions: Patients with the TJP2-C genotype carrying PPTMs in both alleles had a rapidly progressive course, leading to early decompensation and death if they did not receive timely liver transplantation.
    Language English
    Publishing date 2024-03-06
    Publishing country United States
    Document type Journal Article
    ZDB-ID 604603-4
    ISSN 1527-3350 ; 0270-9139
    ISSN (online) 1527-3350
    ISSN 0270-9139
    DOI 10.1097/HEP.0000000000000828
    Database MEDical Literature Analysis and Retrieval System OnLINE

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