Review of the current literature on H syndrome treatment.

Autor: Saleh Anaraki K; General Medicine, Shahid Beheshti University of Medical Sciences, Tehran, Iran., Khosravi S; General Medicine, Student Research Center, School of Medicine, Iran University of Medical Sciences (IUMS), Tehran, Iran.; Universal Scientific Education and Research Network (USERN), Tehran, Iran., Behrangi E; Department of Dermatology, Rasool Akram Medical Complex Clinical Research Development Center (RCRDC), School of Medicine, Iran University of Medical Sciences, Tehran, Iran.; Skin and Stem Cell Research Center, Tehran University of Medical Sciences, Tehran, Iran., Sadeghzadeh-Bazargan A; Department of Dermatology, Rasool Akram Medical Complex Clinical Research Development Center (RCRDC), School of Medicine, Iran University of Medical Sciences, Tehran, Iran.; Skin and Stem Cell Research Center, Tehran University of Medical Sciences, Tehran, Iran., Goodarzi A; Department of Dermatology, Rasool Akram Medical Complex Clinical Research Development Center (RCRDC), School of Medicine, Iran University of Medical Sciences, Tehran, Iran.; Skin and Stem Cell Research Center, Tehran University of Medical Sciences, Tehran, Iran.
Jazyk: angličtina
Zdroj: Journal of family medicine and primary care [J Family Med Prim Care] 2022 Mar; Vol. 11 (3), pp. 857-860. Date of Electronic Publication: 2022 Mar 10.
DOI: 10.4103/jfmpc.jfmpc_1435_21
Abstrakt: H syndrome is a systemic inherited autosomal recessive histiocytosis, with characteristic cutaneous findings accompanying systemic manifestations and a most common genetic mutation (OMIM 612391) as SLC29A3. The term "H Syndrome" is representative of presentation with hyperpigmentation, hypertrichosis, hepatosplenomegaly, heart anomalies, hearing loss, hypogonadism, low height, and, occasionally, hyperglycemia. H syndrome is new and growing entity in medicine. This syndrome is not specific to a region or a nationality. There are very few treatment experiences on H Syndrome patients and most of them are unsatisfactory apart from hypertrichosis, which is able to treat almost permanently by hair removal lasers. Latest findings suggest that there is possibility of prevention of short stature or other cutaneous or systemic complications in this syndrome with earlier diagnosis and treatment. We searched Medline, Scopus, Web of Sciences, and Google Scholar, up to now and reviewed previous published papers with emphasis on treatment methods and its effects on certain common symptoms.
Competing Interests: There are no conflicts of interest.
(Copyright: © 2022 Journal of Family Medicine and Primary Care.)
Databáze: MEDLINE
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