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Article: Spectrum of renal lesions in infantile cholestasis.

Valamparampil, Joseph / Gupte, Girish L

2024 Volume 23, Issue 1, Page(s) e0150

Language	English
Publishing date	2024-04-15
Publishing country	United States
Document type	Journal Article
ZDB-ID	2657644-2
ISSN	2046-2484
ISSN	2046-2484
DOI	10.1097/CLD.0000000000000150
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Article ; Online: Chronic Liver Disease in Children: From Diagnosis to Liver Transplantation.

Gupte, Girish L / Srivastava, Anshu

Indian journal of pediatrics

2024 Volume 91, Issue 3, Page(s) 260–261

MeSH term(s)	Child ; Humans ; Infant ; Liver Transplantation ; Liver Diseases/diagnosis ; Liver Diseases/surgery ; Chronic Disease
Language	English
Publishing date	2024-02-07
Publishing country	India
Document type	Editorial
ZDB-ID	218231-2
ISSN	0973-7693 ; 0019-5456
ISSN (online)	0973-7693
ISSN	0019-5456
DOI	10.1007/s12098-024-05030-3
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Article ; Online: Chronic Liver Disease - What's New?

Effandie, Ezyana / Gupte, Girish L

Indian journal of pediatrics

2023 Volume 91, Issue 4, Page(s) 391–397

Abstract: Chronic liver disease (CLD) is a persistent public health burden, with over one billion cases reported worldwide. In most cases, the progression of CLD is slow and undulating with end-stage liver disease developing at variable time points depending on ... ...

Abstract	Chronic liver disease (CLD) is a persistent public health burden, with over one billion cases reported worldwide. In most cases, the progression of CLD is slow and undulating with end-stage liver disease developing at variable time points depending on the underlying etiology of the disease. The concept of reversibility or halting progression to end stage liver disease is recent and various medications are in the pipeline which influence the progression of CLD. Non-invasive tests for monitoring of CLD may have the potential to avoid the morbidity and mortality related to invasive procedures. However, their applicability and validation in pediatrics requires further development and a coordinated effort by large pediatric liver centres. Recent advances in metabolomics and modern molecular technologies have led to an understanding of the interaction between gut microbiome liver axis and gut dysbiosis contributing to liver diseases. In the future, modifying the gut microbiome has the potential to change the outcome and significantly reduce the morbidity associated with CLD. This article focuses on newer modalities and concepts in the management of CLD, which may help develop strategies to prevent its progression to end-stage liver disease and associated morbidity/mortality.
MeSH term(s)	Humans ; Child ; End Stage Liver Disease/etiology ; Liver Diseases ; Liver ; Forecasting ; Hepatomegaly ; Chronic Disease
Language	English
Publishing date	2023-09-22
Publishing country	India
Document type	Journal Article ; Review
ZDB-ID	218231-2
ISSN	0973-7693 ; 0019-5456
ISSN (online)	0973-7693
ISSN	0019-5456
DOI	10.1007/s12098-023-04819-y
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Article: Cystic fibrosis associated liver disease in children.

Valamparampil, Joseph J / Gupte, Girish L

World journal of hepatology

2021 Volume 13, Issue 11, Page(s) 1727–1742

Abstract: Cystic fibrosis (CF) is an autosomal recessive disorder caused by mutations in the CF transmembrane conductance regulator gene. CF liver disease develops in 5%-10% of patients with CF and is the third leading cause of death among patients with CF after ... ...

Abstract	Cystic fibrosis (CF) is an autosomal recessive disorder caused by mutations in the CF transmembrane conductance regulator gene. CF liver disease develops in 5%-10% of patients with CF and is the third leading cause of death among patients with CF after pulmonary disease or lung transplant complications. We review the pathogenesis, clinical presentations, complications, diagnostic evaluation, effect of medical therapies especially CF transmembrane conductance regulator modulators and liver transplantation in CF associated liver disease.
Language	English
Publishing date	2021-01-01
Publishing country	United States
Document type	Journal Article ; Review
ZDB-ID	2573703-X
ISSN	1948-5182
ISSN	1948-5182
DOI	10.4254/wjh.v13.i11.1727
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Article: Bridging Liver Transplantation in the Treatment of Intestinal Failure Associated Liver Disease in Infants-A Bridge Too Far?

Sharif, Abubakar / Sharif, Khalid / Mirza, Darius F / Gupte, Girish L

Children (Basel, Switzerland)

2022 Volume 9, Issue 5

Abstract: Infants with intestinal failure associated liver disease (IFALD) requiring liver and bowel transplant have a high mortality on the transplant waiting list due to the scarcity of the size-matched donor organs. Bridging liver transplantation has been used ... ...

Abstract	Infants with intestinal failure associated liver disease (IFALD) requiring liver and bowel transplant have a high mortality on the transplant waiting list due to the scarcity of the size-matched donor organs. Bridging liver transplantation has been used to allow the children to grow to a reasonable size so that a combined liver and small bowel transplant could be performed in the future. We report on two children with irreversible intestinal failure (ultra-short bowel syndrome secondary to gastroschisis and microvillous inclusion disease) with IFALD who underwent bridging liver transplantation at our institution. Both patients made a good recovery from their initial surgery. One patient died 6 months following surgery from generalized sepsis, and the other patient survived in good condition to undergo a combined liver and small bowel transplant but died a few days post-transplant. In the current era of scarcity of donor organs, this raises an ethical dilemma for the team involved regarding appropriate utilisation of a scarce resource.
Language	English
Publishing date	2022-05-10
Publishing country	Switzerland
Document type	Journal Article
ZDB-ID	2732685-8
ISSN	2227-9067
ISSN	2227-9067
DOI	10.3390/children9050699
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Article ; Online: Congenital Myotonic Dystrophy with Combined Heterozygous ATP8B1/ABCB4 Mutation Leading to Progressive Cholestasis and Liver Failure.

Chiou, Fang Kuan / Rizvi, Hina / Quinlivan, Ros / Gupte, Girish L

JPGN reports

2021 Volume 2, Issue 4, Page(s) e121

Abstract: Myotonic dystrophy (MyoD) is an inherited genetic disorder caused by the expansion of a CTG trinucleotide repeat in the dystrophia myotonica protein kinase gene. It manifests as a multisystem disease affecting not only skeletal muscles, but also heart, ... ...

Abstract	Myotonic dystrophy (MyoD) is an inherited genetic disorder caused by the expansion of a CTG trinucleotide repeat in the dystrophia myotonica protein kinase gene. It manifests as a multisystem disease affecting not only skeletal muscles, but also heart, lung, eye, gastrointestinal tract, central nervous system, and endocrine system. However, MyoD is rarely associated with a progressive liver disorder. We report a case of congenital MyoD with combined heterozygous ATP8B1/ABCB4 mutation who developed chronic, progressive low gamma-glutamyltransferase cholestatic liver disease at early infancy, and eventually underwent successful liver transplantation.
Language	English
Publishing date	2021-10-25
Publishing country	United States
Document type	Case Reports
ISSN	2691-171X
ISSN (online)	2691-171X
DOI	10.1097/PG9.0000000000000121
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Article ; Online: Hypogammaglobulinemia and bacterial infections following pediatric post-transplant lymphoproliferative disorder in the rituximab era.

Chiou, Fang Kuan / Beath, Sue V / Patel, Mitul / Gupte, Girish L

Pediatric transplantation

2019 Volume 23, Issue 6, Page(s) e13519

Abstract: Introduction: Treatment of PTLD using immune-depleting agents such as RTX may be associated with increased risk of infections. The aim of this report was to describe the incidence of hypogammaglobulinemia and bacterial infections in children with PTLD ... ...

Abstract	Introduction: Treatment of PTLD using immune-depleting agents such as RTX may be associated with increased risk of infections. The aim of this report was to describe the incidence of hypogammaglobulinemia and bacterial infections in children with PTLD after SOT at a single center since the introduction of RTX. Methods: A retrospective review was conducted over a study period of 2000-2016 in pediatric patients diagnosed with biopsy-proven PTLD based on the WHO histologic criteria. Hypogammaglobulinemia was defined by serum IgG <4 g/L; CPBI was defined by clinically significant infection by an identified pathogenic bacteria isolated from a normally sterile body site. Results: Twenty-eight patients were included, comprising 16 LTx and 12 ITx patients, and 17 patients received RTX therapy. Total of 31 episodes of CPBI occurred in 16 patients. Incidence of CPBI was 31.4 infections per 100 patient-years in RTX-treated patients, as compared to 8.4 infections per 100 patient-years in non-RTX-treated patients (P < 0.001). Hypogammaglobulinemia was significantly more prevalent after 6 months (P = 0.001) and 2 years (P = 0.005) in RTX-treated patients, as compared to none in the group that did not receive RTX. Hypogammaglobulinemia (P = 0.047), ITx (P = 0.027), and monomorphic PTLD (P = 0.024) were significantly associated with recurrent (≥2) CPBI and/or CPBI-related deaths within the first year post-PTLD. Conclusion: While RTX is an effective treatment for PTLD, hypogammaglobulinemia can persist for up to 2 years following RTX therapy, which may be associated with the higher cumulative rates of CPBI observed in RTX-treated patients.
MeSH term(s)	Agammaglobulinemia/complications ; Bacterial Infections/complications ; Biopsy ; Child ; Child, Preschool ; Follow-Up Studies ; Humans ; Immunoglobulin G ; Immunosuppressive Agents/adverse effects ; Immunosuppressive Agents/therapeutic use ; Incidence ; Infant ; Lymphoproliferative Disorders/etiology ; Organ Transplantation/adverse effects ; Pediatrics/methods ; Postoperative Complications/etiology ; Postoperative Complications/microbiology ; Remission Induction ; Retrospective Studies ; Risk Factors ; Rituximab/adverse effects ; Rituximab/therapeutic use
Chemical Substances	Immunoglobulin G ; Immunosuppressive Agents ; Rituximab (4F4X42SYQ6)
Language	English
Publishing date	2019-06-17
Publishing country	Denmark
Document type	Journal Article
ZDB-ID	1390284-2
ISSN	1399-3046 ; 1397-3142
ISSN (online)	1399-3046
ISSN	1397-3142
DOI	10.1111/petr.13519
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Article ; Online: Principles of immunisation in children with solid organ transplant.

Kelgeri, Chayarani / Kelly, Dominic F / Brant, Alexandra / Patel, Mitul / Gupte, Girish L

Archives of disease in childhood

2020 Volume 106, Issue 3, Page(s) 219–223

Abstract: Vaccine-preventable diseases (VPD) are a significant risk to paediatric solid organ transplant (SOT) recipients on lifelong immunosuppressive therapy. Children progressing to end-stage organ dysfunction are unable to mount a robust immune response. Hence, ...

Abstract	Vaccine-preventable diseases (VPD) are a significant risk to paediatric solid organ transplant (SOT) recipients on lifelong immunosuppressive therapy. Children progressing to end-stage organ dysfunction are unable to mount a robust immune response. Hence, it is important to plan vaccination early in the course of disease, especially if a child is anticipated to be a SOT candidate. Vaccine recommendations need to be individualised in this population based on vaccine history and serology. Catch-up or accelerated schedules may be used to complete vaccinations before transplant. Post-transplant, immunisation is recommenced in consultation with the transplant team taking into context the time since transplant and the intensity of the immunosuppressive regime. Inactivated vaccines are safe post-transplant but postexposure prophylaxis may still be required in children with inadequate immunity to VPD. Specific vaccines may be advised for SOT recipients travelling abroad (in consultation with a travel clinic) or those entering high-risk professions. Additionally, the vaccination status of all household members and close contacts should be reviewed and optimised, offering additional protection to the transplant recipient.
MeSH term(s)	Adolescent ; Child ; Child, Preschool ; Family Characteristics ; Humans ; Immunization/methods ; Immunization/standards ; Immunosuppressive Agents/adverse effects ; Organ Transplantation/adverse effects ; Organ Transplantation/standards ; Post-Exposure Prophylaxis/methods ; Transplant Recipients/education ; Transplantation Immunology/immunology ; Travel ; Vaccine-Preventable Diseases/complications ; Vaccine-Preventable Diseases/epidemiology ; Vaccine-Preventable Diseases/immunology ; Vaccines/standards ; Vaccines/therapeutic use
Chemical Substances	Immunosuppressive Agents ; Vaccines
Language	English
Publishing date	2020-09-16
Publishing country	England
Document type	Journal Article ; Review
ZDB-ID	524-1
ISSN	1468-2044 ; 0003-9888 ; 1359-2998
ISSN (online)	1468-2044
ISSN	0003-9888 ; 1359-2998
DOI	10.1136/archdischild-2020-319822
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Article ; Online: Addressing extreme size mismatch in pediatric intestinal transplantation: Outcomes of intestinal length reduction.

Hann, Angus / Gupte, Girish L / Pathanki, Adithya / Coelho, Maria / Beath, Sue / Hartley, Jane / Kelly, Deirdre / De Ville De Goyet, Jean / Oo, Ye H / Hartog, Hermien / Perera, Thamara P R / Sharif, Khalid / Mirza, Darius F

Pediatric transplantation

2023 Volume 27, Issue 5, Page(s) e14528

Abstract: Background: Bench liver reduction, with or without intestinal length reduction (LR) (coupled with delayed closure and abdominal wall prostheses), has been a strategy adopted by our program for small children due to the limited availability of size- ... ...

Abstract	Background: Bench liver reduction, with or without intestinal length reduction (LR) (coupled with delayed closure and abdominal wall prostheses), has been a strategy adopted by our program for small children due to the limited availability of size-matched donors. This report describes the short, medium, and long-term outcomes of this graft reduction strategy. Methods: A single-center, retrospective analysis of children that underwent intestinal transplantation (April 1993 to December 2020) was performed. Patients were grouped according to whether they received an intestinal graft of full length (FL) or following LR. Results: Overall, 105 intestinal transplants were performed. The LR group (n = 10) was younger (14.5 months vs. 40.0 months, p = .012) and smaller (8.7 kg vs. 13.0 kg, p = .032) compared to the FL group (n = 95). Similar abdominal closure rates were achieved after LR, without any increase in abdominal compartment syndrome (1/10 vs. 7/95, p = .806). The 90-day graft and patient survival were similar (9/10, 90% vs. 83/95, 86%; p = .810). Medium and long-term graft survival at 1 year (8/10, 80% vs. 65/90, 71%; p = .599), and 5 years (5/10, 50% vs. 42/84, 50%; p = 1.00) was similar. Conclusion: LR of intestinal grafts appears to be a safe strategy for infants and small children requiring intestinal transplantation. This technique should be considered in the situation of significant size mismatch of intestine containing grafts.
MeSH term(s)	Infant ; Child ; Humans ; Liver Transplantation/methods ; Retrospective Studies ; Intestines/transplantation ; Liver ; Tissue Donors ; Graft Survival
Language	English
Publishing date	2023-06-19
Publishing country	Denmark
Document type	Journal Article
ZDB-ID	1390284-2
ISSN	1399-3046 ; 1397-3142
ISSN (online)	1399-3046
ISSN	1397-3142
DOI	10.1111/petr.14528
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Article ; Online: Central Line in Long-term Parenteral Nutrition in Children: A European Survey.

Hojsak, Iva / Lacaille, Florence / Gupte, Girish L / Köglmeier, Jutta

Journal of pediatric gastroenterology and nutrition

2018 Volume 67, Issue 3, Page(s) 409–413

Abstract: Background and aims: The guidelines for the insertion and maintenance of the central venous catheter (CVC) in children on long-term parenteral nutrition (PN) were published 12 years ago and studies evaluating the outcomes are limited. Therefore, the aim ...

Abstract	Background and aims: The guidelines for the insertion and maintenance of the central venous catheter (CVC) in children on long-term parenteral nutrition (PN) were published 12 years ago and studies evaluating the outcomes are limited. Therefore, the aim of the present study was to perform a survey about criteria for CVC insertion and maintenance in intestinal failure/rehabilitation centers treating children on home PN. Methods: An online cross-sectional survey based on previous European Society of Paediatric Gastroenterology, Hepatology and Nutrition PN guidelines was distributed electronically to the members of the European Society of Paediatric Gastroenterology, Hepatology and Nutrition networking group, Network for Intestinal Failure and Transplantation in Europe and tertiary pediatric gastroenterology centers in Europe. Results: Overall, 55 responses from 49 centers in 18 European countries and Israel were collected. The majority of respondents were from the United Kingdom (10, 19%), followed by Germany (7, 13%) and France (6, 11%). Eleven centers (21%) cared for >30 patients, 8 (15%) centers between 20 and 30 patients, 18 (34%) centers between 10 and 20 patients, and 16 (30%) <10 patients on home PN. There was a high variability in the majority of answers to the cross-sectional survey. Conclusions: CVC insertion and maintenance in children on home PN varies largely amongst centers in Europe. These differences could be at least partially explained by the lack of updated guidelines and limited evidence. There is an urgent need for collaborative research to make recommendations about the best possible practice.
MeSH term(s)	Central Venous Catheters ; Child ; Child, Preschool ; Cross-Sectional Studies ; Europe ; Female ; Guidelines as Topic ; Humans ; Long-Term Care ; Male ; Parenteral Nutrition, Home/instrumentation ; Parenteral Nutrition, Home/methods ; Surveys and Questionnaires
Language	English
Publishing date	2018-06-26
Publishing country	United States
Document type	Journal Article ; Multicenter Study
ZDB-ID	603201-1
ISSN	1536-4801 ; 0277-2116
ISSN (online)	1536-4801
ISSN	0277-2116
DOI	10.1097/MPG.0000000000002064
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