Evidence map›Paper›PMID 38854322›Full record

ArticleCureus2024

Wilson Disease in Children in the Eastern Region of Morocco: Analysis of 24 Cases.

Maria Rkain, Massilia Bouhmidi, Amal Hamamı, Aziza Elouali, Siham Chariba, Imane Kamaoui, Imane Skiker, Abdeladim Babakhouya

Abstract read
In one paragraph

Article in Cureus, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.

0numbers the graph read from it
0cells of the map it votes in
2citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

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Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

2 citing papers in PubMed.

  1. Review
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4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

8 authors.

Maria RkainDepartment of Pediatrics, University Hospital Mohammed VI, Faculty of Medicine and Pharmacy, Mohammed First University, Oujda, MAR.
Massilia BouhmidiDepartment of Pediatrics, University Hospital Mohammed VI, Faculty of Medicine and Pharmacy, Mohammed First University, Oujda, MAR.
Amal HamamıDepartment of Pediatrics, University Hospital Mohammed VI, Faculty of Medicine and Pharmacy, Mohammed First University, Oujda, MAR.
Aziza EloualiDepartment of Pediatrics, University Hospital Mohammed VI, Faculty of Medicine and Pharmacy, Mohammed First University, Oujda, MAR.
Siham CharibaDepartment of Ophthalmology, University Hospital Mohammed VI, Faculty of Medicine and Pharmacy, Mohammed First University, Oujda, MAR.
Imane KamaouiDepartment of Radiology, University Hospital Mohammed VI, Faculty of Medicine and Pharmacy, Mohammed First University, Oujda, MAR.
Imane SkikerDepartment of Radiology, University Hospital Mohammed VI, Faculty of Medicine and Pharmacy, Mohammed First University, Oujda, MAR.
Abdeladim BabakhouyaDepartment of Pediatrics, University Hospital Mohammed VI, Faculty of Medicine and Pharmacy, Mohammed First University, Oujda, MAR.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Wilson's disease (WD), or "hepato-lenticular degeneration," is a rare genetic disorder of autosomal recessive inheritance causing toxic tissue accumulation of copper, mainly in the liver, brain, and cornea. Its phenotypic and genotypic heterogeneity characterizes it. This study aimed to clarify the clinical features and spectrum of Wilson's disease in children from the eastern region of Morocco and to study the evolutionary profile and survival in this population while discussing and highlighting the various diagnostic and therapeutic difficulties encountered in the management of WD in our context. This retrospective study encompassed 24 children diagnosed with Wilson's disease, selected from the gastroenterology-hepatology and pediatric nutrition units at Mohamed VI University Hospital in Oujda, Morocco, over a span of nine years, from January 2015 to November 2023. Our series results show 14 boys and 10 girls; the median age of discovery was 11 years, with extremes ranging from 18 months to 15 years. The consanguinity was found in 13 patients. Clinically, the edemato-ascitic syndrome was noted in 14 patients with an alteration of the general state; icterus was found in 13 patients; signs of portal hypertension were present in six patients; and neurological signs in seven cases. Skin manifestations occurred in three cases, and arthralgia in three cases. Six children were diagnosed on the occasion of a family screening. Biologically, hepatic cytolysis was found in 20 patients, with signs of hepatocellular failure in 15 cases. Hemolytic anemia was present in nine patients. Ceruloplasminemia was decreased in 21 patients and cupremia in 19 patients. Cupruria was increased in 22 cases. The Kayser-Fleicher ring was found in 10 cases. Abdominal ultrasound showed ascites in 16 patients, hepatomegaly in 1, splenomegaly in two cases, hepatosplenomegaly in five cases, and cirrhosis in two. MRI showed signal abnormalities in 11 patients. Therapeutically, D-penicillamine was initially introduced in 18 patients and zinc acetate in 6 patients. The evolution was favorable for 15 patients still followed up in the department. Three patients died of hepatocellular failure, and two died of hepatic encephalopathy. Four patients were lost to follow-up.

Indexed as

atp7b mutationscentral nervous systemchildcopperd-penicillaminehepatopathykayser-fleischer ringscreening.wilson

Identifiers

PMID38854322
PMCPMC11162554

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.