ArticlePostepy dermatologii i alergologii2018
High frequency of primary hereditary ichthyoses in the North-East region of Cairo, Egypt.
Article in Postepy dermatologii i alergologii, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 3 papers.
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Who cites it
3 citing papers in PubMed.
- Advances in the treatment of autosomal recessive congenital ichthyosis, a look towards the repositioning of drugs.Frontiers in pharmacology · 2023Review
- Proposal for a 6-step approach for differential diagnosis of neonatal erythroderma.Journal of the European Academy of Dermatology and Venereology : JEADV · 2022Review
- Novel Homozygous Mutations in the GenesGenes · 2021Article
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3 authors.
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Abstract
introductionInherited ichthyoses are caused by mutations in various genes important for keratinocyte differentiation and epidermal barrier function. Although ichthyoses are rare disorders, they require costly long-term medical management, and thus there is a need for efficient preventive and therapeutic strategies.
aimWe performed a retrospective study to determine the frequency, types, clinical presentation and associated genomic errors of primary hereditary ichthyoses in Egyptian patients and their relatives consulting the Genetics Clinic, Pediatric Hospital, Ain Shams University. MATERIAL AND
methodsThe outpatient log books of patients between January 2000 and December 2014 were reviewed, and diagnosis of new patients was confirmed through examination by a dermatologist. All epidemiologic, demographic, and clinical data were extracted and recorded in especially designed data collection forms.
resultsThe occurrence rate of primary hereditary ichthyoses in our study was 25.7% of genodermatosis patients attending the genetics clinics and 1 per 2359 patients attending the Pediatric Hospital. The commonest type of ichthyosis in our study was Lamellar ichthyosis (38%), followed by congenital ichthyosiform erythroderma (26.8%). Consanguineous marriage was reported among the parents of 79% of patients and positive family history was reported in 72% of patients.
conclusionsTo the best of our knowledge, this preliminary study is the first report on the clinico-epidemiological features of primary hereditary ichthyoses in Egypt. The high rate of prenatal consanguinity among parents of our patients may account for the high frequency of these genodermatoses in Egypt. This highlights the importance of genetic counselling and prenatal diagnosis in Egypt.
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