Case Report: Intestinal Nodular Lymphoid Hyperplasia as First Manifestation of Activated PI3Kδ Syndrome Due to a Novel

PIK3CD activated PI3K-delta syndrome inborn error of immunity nodular lymphoid hyperplasia-GIT novel variant sirolimus

Journal

Frontiers in pediatrics
ISSN: 2296-2360
Titre abrégé: Front Pediatr
Pays: Switzerland
ID NLM: 101615492

Informations de publication

Date de publication:
2021
Historique:
received: 30 04 2021
accepted: 01 09 2021
entrez: 25 10 2021
pubmed: 26 10 2021
medline: 26 10 2021
Statut: epublish

Résumé

Nodular lymphoid hyperplasia (NLH) is a lymphoproliferative disease caused by non-clonal expansion of lymphoid cells in the gut mucosa. Little is known about the pathogenesis of NLH, which is often disregarded as an insignificant or para-physiologic phenomenon. We present the case of a girl with isolated diffuse NLH (extending from the stomach to the rectum) caused by activated PI3Kδ syndrome (APDS) due to the novel p.Glu525Gly variant in

Identifiants

pubmed: 34692603
doi: 10.3389/fped.2021.703056
pmc: PMC8528001
doi:

Types de publication

Case Reports

Langues

eng

Pagination

703056

Informations de copyright

Copyright © 2021 Marzollo, Bresolin, Colavito, Cani, Gaio, Bosa, Mescoli, Rossini, Barzaghi, Perilongo, Leon, Biffi and Cananzi.

Déclaration de conflit d'intérêts

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

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Auteurs

Antonio Marzollo (A)

Division of Pediatric Hematology, Oncology and Stem Cell Transplant, Padua University Hospital, Padua, Italy.
Fondazione Citta' della Speranza, Istituto di Ricerca Pediatrica, Padua, Italy.

Silvia Bresolin (S)

Division of Pediatric Hematology, Oncology and Stem Cell Transplant, Padua University Hospital, Padua, Italy.
Istituto di Ricerca Pediatrica, Citta' della Speranza, Padua, Italy.

Davide Colavito (D)

Research and Innovation (R and I Genetics) Srl, Padua, Italy.

Alice Cani (A)

Istituto di Ricerca Pediatrica, Citta' della Speranza, Padua, Italy.

Paola Gaio (P)

Unit of Pediatric Gastroenterology, Digestive Endoscopy, Hepatology and Care of the Child With Liver Transplantation, Department of Women's and Children's Health, University Hospital of Padua, Padua, Italy.

Luca Bosa (L)

Unit of Pediatric Gastroenterology, Digestive Endoscopy, Hepatology and Care of the Child With Liver Transplantation, Department of Women's and Children's Health, University Hospital of Padua, Padua, Italy.

Claudia Mescoli (C)

Unit of Surgical Pathology and Cytopathology, Department of Medicine (DIMED), University Hospital of Padua, Padua, Italy.

Linda Rossini (L)

Division of Pediatric Hematology, Oncology and Stem Cell Transplant, Padua University Hospital, Padua, Italy.

Federica Barzaghi (F)

Pediatric Immunohematology and Stem Cell Program, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) San Raffaele Scientific Institute, Milan, Italy.

Giorgio Perilongo (G)

Unit of Pediatric Gastroenterology, Digestive Endoscopy, Hepatology and Care of the Child With Liver Transplantation, Department of Women's and Children's Health, University Hospital of Padua, Padua, Italy.

Alberta Leon (A)

Research and Innovation (R and I Genetics) Srl, Padua, Italy.

Alessandra Biffi (A)

Division of Pediatric Hematology, Oncology and Stem Cell Transplant, Padua University Hospital, Padua, Italy.

Mara Cananzi (M)

Unit of Pediatric Gastroenterology, Digestive Endoscopy, Hepatology and Care of the Child With Liver Transplantation, Department of Women's and Children's Health, University Hospital of Padua, Padua, Italy.

Classifications MeSH