Evidence map›Paper›PMID 39633313›Full record

ArticleBMC pediatrics2024

Clinical spectrum and genetic variation of six patients with methylmalonic aciduria (MMA); a report from Iran.

Zahra Beyzaei, Hossein Moravej, Mohammad Hadi Imanieh, Sorour Inaloo, Bita Geramizadeh

Abstract read
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Article in BMC pediatrics, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers.

0numbers the graph read from it
0cells of the map it votes in
4citing papers in PubMed
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1 · What the graph read from it

What it found

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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

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3 · Its place in the literature

Who cites it

4 citing papers in PubMed.

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4 · The record

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5 · Who and what money

Authors and funding

5 authors.

Zahra BeyzaeiShiraz Transplant Research Center (STRC), Shiraz University of Medical Sciences, Shiraz, Iran.
Hossein MoravejDepartment of Pediatric Endocrinology, Shiraz University of Medical Sciences, Shiraz, Iran.
Mohammad Hadi ImaniehGastroenterology and Hepatology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.
Sorour InalooNeuroscience Research Centers, Shiraz University of Medical Sciences, Shiraz, Iran.
Bita GeramizadehShiraz Transplant Research Center (STRC), Shiraz University of Medical Sciences, Shiraz, Iran. geramib@gmail.com.

Funding

Shiraz Transplant Research Center, Shiraz University of Medical Sciences 28294
6 · The paper itself

Abstract

objectiveMethylmalonic acidemia (MMAs) is known as a severe, complex, and lethal disorder of methylmalonate and cobalamin. The patients with MMA may have developmental, neurological, and metabolic disorders such as liver disease. Here, we aim to evaluate 6 Iranian patients suspected to MMA disorder. STUDY

designWe will provide genetic results, biochemical analysis and treatment for these patients. Liquid chromatography-tandem mass spectrometry (LC-MS/MS) and variant screening in probands by whole exome sequencing (WES) were performed.

resultsA total of six homozygous variants were identified, including five previously identified variants and one novel variant, in the two MMA-causing genes as follows: c.577G > C, c.290 + 69G > T, c.662T > A, c.290 + 69G > T of MMAB, and c.100dupA, c.394 C > T of MMACHC. Sanger sequencing confirmed the identified variants. Additionally, metabolomics data analysis reliably identified elevated C3 and MMA levels, as well as abnormalities in the amino acid profile, indicating the presence of pathogenic variants.

conclusionsOur findings expand the global spectrum of genotypes in MMA. While WES, combined with metabolomics and biochemical analysis, offers valuable insights for accurate diagnosis and subtyping of MMA, it is most beneficial in complex cases where clinical findings are unclear.

Indexed as

Amino Acid Metabolism, Inborn ErrorsChildChild, PreschoolChromatography, LiquidExome SequencingFemaleGenetic VariationHumansInfantInfant, NewbornIranMaleMethylmalonic AcidMutationTandem Mass SpectrometryMethylmalonic AcidMethylmalonic acidemiaNovel mutationVitamin B12/hydroxocobalaminWhole exome sequencing

Identifiers

PMID39633313
PMCPMC11616211

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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.