AI Article Synopsis
- Acute intermittent porphyria (AIP) is a genetic disorder that affects heme production in the liver and can lead to severe abdominal pain attacks that are hard to manage for some patients.* -
- A 20-year-old woman with recurrent AIP attacks did not improve with standard treatments, but showed significant relief after receiving a year-long treatment with gonadotropin-releasing hormone analogues and estrogen.* -
- The treatment resulted in the woman not experiencing any acute attacks and achieving long-term remission, suggesting this combination could be effective for menstrual-related AIP cases.*
Article Abstract
Background: Acute intermittent porphyria (AIP) is an autosomal dominant hepatic porphyria characterized by a partial deficiency of hydroxymethylbilane synthase involved in heme biosynthesis. It is difficult for all patients to achieve complete control of AIP episodes.
Method: We report on a 20-year-old female woman who suffered from recurrent abdominal pain and was diagnosed as "acute intermittent porphyria". She failed to respond to conventional symptomatic treatment and subsequently was treated with gonadotropin-releasing hormone analogues (GnRH) combined with estrogen for one year.
Result: The case did not experience acute attacks and obtained long-term clinical remission to date.
Conclusions: GnRH combined with estrogen, one of the treatment options for menstrual-associated AIP, might induce long-term remission.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.7754/Clin.Lab.2022.211218 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Objectives: Investigate the consequences of the histological progression of metabolically associated steatohepatitis (MASH) and fibrosis on long-term survival after bariatric surgery.
Methods: From 1994 to 2021, 3028 patients at the University Hospital of Lille were prospectively included. Baseline liver biopsies were systematically performed with proposed follow-up biopsies 1 year after surgery, mainly in MASH patients.
Associations between momentary mental states and concurrent social functioning after remission from first episode psychosis: A HAMLETT ecological momentary assessment study.
J Psychiatr Res
December 2024
University of Groningen, University Medical Center Groningen, University Center for Psychiatry, Hanzeplein 1, PO Box 30.001, 9700 GZ, Groningen, the Netherlands.
Background: Symptom severity and social functioning are important outcomes after first episode psychosis (FEP), yet current evidence about associations between them is inconsistent and lacks (subclinical) momentary insights.
Methods: The current Ecological Momentary Assessment (EMA) study was conducted in 58 people in remission from FEP, as part of the HAMLETT (Handling Antipsychotic Medication: Long-term Evaluation of Targeted Treatment) trial. At baseline, participants were prompted to report momentary mental states and social context 10x/day for eight consecutive days, including psychotic experiences (PEs), motivation/drive and negative affect, that may indicate proxies of (subclinical) psychotic, negative and general affective symptoms, respectively.
Epstein-Barr virus-associated lymphoproliferative disease during remission after induction therapy with dasatinib in Philadelphia chromosome-positive acute lymphoblastic leukemia: a case report.
Ann Hematol
December 2024
Division of Hematology, Respiratory Medicine and Oncology, Department of Internal Medicine, Faculty of Medicine, Saga University, Saga, Japan.
Dasatinib, a second-generation tyrosine kinase inhibitor, has been reported to have immunomodulatory effects. Epstein-Barr virus (EBV)-associated lymphoproliferative disorders (EBV-LPD) occur in immunocompromised patients, such as those receiving methotrexate or other immunosuppressive drugs or after allogenic transplantation. EBV-LPD is also reported to be a rare side effect in patients receiving long-term dasatinib or imatinib.
View Article and Find Full Text PDFBackground: This study aimed to evaluate the response to therapy and outcome with long-term daily mycophenolate mofetil (MMF) and high-dose alternate-day steroids (HADS) in children with dense deposit disease (DDD).
Methods: Children with DDD who received long-term MMF (1200 mg/m/day) and HADS (1.5-2 mg/kg AD) with slow tapering were retrospectively evaluated for their clinico-pathological presentation, response to therapy (complete, partial, no remission) and outcome (patient and renal survival).
Post-transplant relapse in pediatric acute lymphoblastic leukemia in the era of CAR-T cell therapy. A multicenter analysis of Grupo Español de Trasplante Hematopoyetico y Terapia Celular (GETH-TC) Pediatric Committee.
Cytotherapy
December 2024
Pediatric Hematology Oncology, Hospital Vall´de Hebrón, Barcelona, Spain.
Background: The management of relapsed acute lymphoblastic leukemia (ALL) after hematopoietic stem cell transplantation (HSCT) has evolved significantly. Initially, treatment options were limited to palliative care, salvage chemotherapy, and second HSCT. Currently, the focus has shifted to innovative immunotherapies, particularly CAR T-cell therapy.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!