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2.1
Monilethrix
Moniliform Hair Syndrome

Images

WIKIDATA, CC0 1.0

Presentation

Presentation[edit] Newborns are not born with Monilethrix; it usually develops within the first few months of life. The presentation may be of alopecia (baldness). Individuals vary in severity of symptoms. [en.wikipedia.org]

However, using trichogram analysis, pseudomoniletrix hair does not present with dystrophic constrictions but with flattened irregular beading. [orpha.net]

Author Affiliations †Present address, Department of Pediatrics, Johns Hopkins Hospital, Baltimore, Maryland. ‡Present address, Children's Memorial Hospital, Chicago, Illinois. Facebook Twitter LinkedIn Email Copy URL Permissions [nejm.org]

A similar history was present in mother, maternal aunts, and maternal grandfather. On cutaneous examination, hairs were sparse, short, blonde colored and dry. [ijpd.in]

It may remain un-presented until beyond adolescence. In children the hairshafts exhibit swellings or nodes with intervening constrictions. [hairscientists.org]

Gastrointestinal

  • Gagging

    In the Russian family, we found a G to A transition at the first base of codon 402, resulting in a lysine substitution (GAG to AAG), designated E402K. [ncbi.nlm.nih.gov]

Skin

  • Skin Patch

    At a glance It is a genetic disorder affecting the hair and occasionally the skin. Patches of alopecia may be present. It does not belong to the group of ectodermal dysplasias. [accessanesthesiology.mhmedical.com]

Musculoskeletal

  • Short Arm

    Each chromosome has a short arm designated “p” and a long arm designated “q”. Chromosomes are further sub-divided into many bands that are numbered. For example, “chromosome 12p13” refers to band 13 on the long arm of chromosome 12. [rarediseases.org]

Workup

There were no hematological abnormalities noted in routine workup. Her serum copper and ceruloplasmin levels were also normal. The examination of the parents and the elder brother was unremarkable. [ijpd.in]

Treatment

[…] result was obtained while treatment was continued with oral acitretin in a 7-year-old girl. [ncbi.nlm.nih.gov]

Before our consultation, she had received treatment with topical anti-fungal treatment (2.5% selenium sulfide shampoo) for six months. [cmaj.ca]

Treatments start at $20/month Find a hair loss plan that works for you. [ro.co]

Prognosis

Prognosis The course of the localized form of monilethrix can be favorable with a reduction of hypotrichosis with time. In general, hypotrichosis improves with age, although remissions have been noted during puberty or pregnancy. [orpha.net]

Differential Diagnosis Some other hair shaft anomalies merit consideration in the differential diagnosis of monilethrix, such as pseudo-monilethrix, congenital alopecia, trichorrhexis invaginata, and ectodermal dysplasia.[9] Prognosis The prognosis of [ncbi.nlm.nih.gov]

Sections Continuing Education Activity Introduction Etiology Epidemiology Pathophysiology History and Physical Evaluation Treatment / Management Differential Diagnosis Prognosis Complications Enhancing Healthcare Team Outcomes Review Questions References [pubmed.ncbi.nlm.nih.gov]

Etiology

In conclusion, our data strongly suggest that monilethrix is a disease of the hair cortex, whose etiology is interesting in that causative mutations seem to be restricted to type II hair keratins. [ncbi.nlm.nih.gov]

Etiology Four genes have been associated with monilethrix: KRT81, KRT83 and KRT86, coding for the type II hair keratins Hb1, Hb3 and Hb6, and are responsible for the autosomal dominant form of the disease. [orpha.net]

Sections Continuing Education Activity Introduction Etiology Epidemiology Pathophysiology History and Physical Evaluation Treatment / Management Differential Diagnosis Prognosis Complications Enhancing Healthcare Team Outcomes Review Questions References [pubmed.ncbi.nlm.nih.gov]

Picture Diseasesof the Skin and its Appendages Lethal Mutations Surviving by Mosaicism References Clinical Aspects Chapter 15Structural Anomalies of the Hair Shaft 6Diagnosis and Treatment Anomalies Without Increased Hair Fragility References Monilethrix Etiology [books.google.com]

Epidemiology

Summary Epidemiology The prevalence and incidence are not known. Clinical description Monilethrix has an early onset in infancy. [orpha.net]

Sections Continuing Education Activity Introduction Etiology Epidemiology Pathophysiology History and Physical Evaluation Treatment / Management Differential Diagnosis Prognosis Complications Enhancing Healthcare Team Outcomes Review Questions References [pubmed.ncbi.nlm.nih.gov]

Other treatment is symptomatic and supportive.[6] Epidemiology[edit] Currently, the frequency for this disease is unknown and affects both men and women. [en.wikipedia.org]

The suggestion is that the autosomal recessive monilethrix results from mutations in the desmoglein 4 gene (DSG4), the only desmoglein expressed in the keratinizing zone of the hair shaft cortex.[4] Epidemiology The prevalence and incidence are not known [ncbi.nlm.nih.gov]

Pathophysiology

Case Overview Monilethrix Member Rated 0 Patient case no. 10964 Date added 05 April 2011 Patient details Age Child Location Iraq Localisation Head / scalp Primary Lesions Alopecia Alopecia / non scarring Diffuse hair shaft disorder Pathophysiology genetic [dermquest.com]

Pathophysiology The exact mechanisms causing the periodic change in the diameter of the hair in monilethrix are still not clear. [ncbi.nlm.nih.gov]

Sections Continuing Education Activity Introduction Etiology Epidemiology Pathophysiology History and Physical Evaluation Treatment / Management Differential Diagnosis Prognosis Complications Enhancing Healthcare Team Outcomes Review Questions References [pubmed.ncbi.nlm.nih.gov]

Chapter 15Structural Anomalies of the Hair Shaft 6Diagnosis and Treatment Anomalies Without Increased Hair Fragility References Monilethrix Etiology and Pathogenesis Hypomelanotic Hair Disorders 2Modelsfor Cultivating Follicular Keratinocytes In Vitro Pathophysiological [books.google.com]

Prevention

Monilethrix treatments Can you prevent monilethrix? If you have any medical questions or concerns, please talk to your healthcare provider. [ro.co]

How can Monilethrix be Prevented? Currently, Monilethrix may not be preventable, since it is a genetic disorder. [dovemed.com]

[…] a Priority in 2019 Here's How the Government Shutdown Could Affect Your Health More Proof High-Fiber Diets Help Prevent Cancers, Heart Disease Foot Stools Move Human Stool Along Not found Sorry, the article you requested could not be found [drdeguzman.com]

Congenitally abnormal hair tends to have an increased susceptibility to weathering and cosmetic damage, which can prevent hair from growing to its maximal length. [ncbi.nlm.nih.gov]

The FDA acknowledged and endorsed the effectiveness of low level laser therapy (LLLT) to prevent baldness in women and men. LLLT is recommended by American and European professionals to stimulate growth and thickening of hair. [grivamax.com]

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