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Amelo-Onycho-Hypohidrotic Syndrome

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Presentation

  • Her originality is to combine approaches both in mice and humans presenting these palate and teeth developmental defects as clinical phenotypical manifestations of rare diseases.[books.google.com]
  • The teeth that are present are frequently small and pointed.Hypohidrotic ectodermal dysplasia is associated with distinctive facial features including a prominent forehead, thick lips, and a flattened bridge of the nose.[icdlist.com]
  • This clinical report presents the diagnosis, treatment planning and prosthetic rehabilitation of a year-old female patient with AI ikparfaite with a group of dental anomalies.[avcilarescort.mobi]
  • Comprehensive examination of dento/oro/craniofacial anomalies Well-illustrated examples Presented in a compact, easy to use format More info Presents a clear diagnostic guide to dental defects and their developmental biology counterparts.[kriso.ee]
Nail Abnormality
  • Hypocalcified-hypoplastic enamel, onycholysis with subungual hyperkeratosis, and hypohidrosis, Amelo-onycho-hypohidrotic syndrome Overview A rare disorder characterized primarily by tooth and nail abnormalities and reduced sweating ability.[checkorphan.org]
  • In addition, nail abnormalities may be more striking that those associated with TDO-I, with the nails tending to be extremely thin and brittle, with upper layers that may be prone to splitting.[rarediseases.org]
  • Adult Syndrome This is another rare condition characterized by abnormal teeth development, tear duct obstruction, nail abnormalities, pigmentation anomalies, plus hand and foot abnormalities.[hxbenefit.com]
Yellow-Brown Discoloration of the Teeth
  • discoloration of the teeth 60 33 hallmark (90%) Very frequent (99-80%) HP:0006286 8 delayed eruption of teeth 60 33 frequent (33%) Frequent (79-30%) HP:0000684 9 dry skin 60 33 frequent (33%) Frequent (79-30%) HP:0000958 10 fine hair 60 33 frequent ([malacards.org]
  • discoloration of the teeth Yellow-brown discolored teeth 0006286 30%-79% of people have these symptoms Abnormality of dental morphology Abnormality of dental shape Abnormally shaped teeth Deformity of teeth Dental deformity Dental malformations Malformed[rarediseases.info.nih.gov]
Alopecia
  • […] malformations (Q80-Q89) Other congenital malformations of skin (Q82) Q82.4 Ectodermal dysplasia (anhidrotic) Code Version: 2019 ICD-10-CM Synonyms Acanthosis nigricans Ackerman syndrome Acrorenal field defect, ectodermal dysplasia, and lipoatrophic diabetes Alopecia[healthprovidersdata.com]
  • Synonyms Acanthosis nigricans Ackerman syndrome Acrorenal field defect, ectodermal dysplasia, and lipoatrophic diabetes Alopecia, onychodysplasia, hypohidrosis, deafness ectodermal dysplasia Amelo-onycho-hypohidrotic syndrome Anhidrotic ectodermal dysplasia[icdlist.com]
  • - anosmia - deafness - hypogonadism [ ] Alopecia antibody deficiency [ ] Alopecia - contractures - dwarfism - intellectual deficit [ ] Alopecia - deafness - hypogonadism [ ] Alopecia-epilepsy-oligophrenia syndrome, Moynahan type [ ] Alopecia - epilepsy[wikicure.fandom.com]
  • Full Causes List for Ectodermal dysplasia syndrome » Types of Ectodermal dysplasia syndrome Some types of this condition may include: 4 Types of Ectodermal dysplasia syndrome : Ackerman syndrome Acrofacial dysostosis , Weyers type Adams-Oliver syndrome Alopecia-contractures-dwarfism-intellectual[familydiagnosis.com]
  • […] antibody deficiency 3 Cases 700 Alopecia totalis 10.5 P * 701 Alopecia universalis 25.0 P * 1005 Alopecia-contractures- dwarfism-intellectual disability syndrome 5 Cases 1008 Alopecia-epilepsy-pyorrhea- intellectual disability syndrome 12 Cases 1014[azkurs.org]
Eczema
  • Additional features of this condition include thin, wrinkled, and dark-colored skin around the eyes; chronic skin problems such as eczema; and a bad-smelling discharge from the nose (ozena). [ Read More ][icdlist.com]
  • Clinical Features of Ectodermal dysplasia syndrome : Oral cleft Xerostomia Everted lower lip vermilion Sinusitis Low-set ears Chronic otitis media Conductive hearing impairment Wide nose Cataract Visual loss Photophobia Acanthosis nigricans Dry skin Eczema[familydiagnosis.com]
  • Lymphoma, Malignancy Opthalmologic No underlying causes Overdose / Toxicity Chronic vitamin A toxicity Psychiatric No underlying causes Pulmonary Obesity hypoventilation syndrome Renal / Electrolyte No underlying causes Rheum / Immune / Allergy Dermatitis, Eczema[wikidoc.org]
Normal Hair
  • hair Clinical features from OMIM: 104570 MalaCards organs/tissues related to Ameloonychohypohidrotic Syndrome: 42 Skin Search GEO for disease gene expression data for Ameloonychohypohidrotic Syndrome.[malacards.org]
Sparse to No Body Hair
  • Affected individuals tend to have sparse scalp and body hair (hypotrichosis). EDA-ID is also characterized by missing teeth (hypodontia) or teeth that are small and pointed.[icdlist.com]
Ulcer
  • Medical Encyclopedia) Milia (Medical Encyclopedia) Sebaceous cyst (Medical Encyclopedia) Seborrheic keratosis (Medical Encyclopedia) Skin lesion removal (Medical Encyclopedia) Skin lesion removal-aftercare (Medical Encyclopedia) Stasis dermatitis and ulcers[icdlist.com]
Irritability
  • Allergies, irritants, your genetic makeup, and certain diseases and immune system problems can cause rashes, hives, and other skin conditions. Many skin problems, such as acne, also affect your appearance.[icdlist.com]

Treatment

  • Provides practical help with treatment planning, guiding the reader through the process of safe and effective decision-making.[books.google.com]
  • Outpatient treatment clinics for alcoholism and drug addiction, Rehabilitation centers, outpatient (medical treatment), Respiratory therapy clinics.[webhealthnetwork.com]
  • Treatment - Ameloonychohypohidrotic syndrome Not supplied. Resources - Ameloonychohypohidrotic syndrome[checkorphan.org]
  • You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments.[rarediseases.info.nih.gov]

Prognosis

  • Prognosis - Ameloonychohypohidrotic syndrome Not supplied. Treatment - Ameloonychohypohidrotic syndrome Not supplied. Resources - Ameloonychohypohidrotic syndrome[checkorphan.org]
  • Pediatric dentists should be aware of this pathology as early diagnosis provided by the oral symptoms leads to a better renal prognosis. 1. Paine ML, White SN, Luo W, Fong H, Sarikaya M, Snead ML.[contempclindent.org]

Etiology

  • They are organised into groups, and further divided into clinical, etiological or histopathological sub-types.[orpha.net]

Pathophysiology

  • Pereira PC, Miranda DM, Oliveira EA, Silva AC: Molecular pathophysiology of renal tubular acidosis. Curr Genomics 2009;10:51–59. MacGibbon D: Generalized enamel hypoplasia and renal dysfunction. Aust Dent J 1972;17:61–63.[karger.com]
  • Molecular pathophysiology of renal tubular acidosis. Curr Genomics 2009;10:51-9. 9. Lubinsky M, Angle C, Marsh PW, Witkop CJ Jr.[contempclindent.org]

Prevention

  • Careful direct laryngoscopy is required to prevent damage to fragile teeth. Hypohidrosis may result in temperature disturbances, and atropine should be used carefully.[accessanesthesiology.mhmedical.com]
  • Prevention - Ameloonychohypohidrotic syndrome Not supplied. Diagnosis - Ameloonychohypohidrotic syndrome In cases of diffuse thickening of the skin, a thyroid profile with T 3 , T 4 , and TSH should be done. This should also identify hypothyroidism.[checkorphan.org]
  • Your skin Holds body fluids in, preventing dehydration Keeps harmful microbes out, preventing infections Helps you feel things like heat, cold, and pain Keeps your body temperature even Makes vitamin D when the sun shines on it Anything that irritates[icdlist.com]
  • The dentist then applies the sodium hypochlorite for 10 minutes, cleans the teeth, and applies a sealant to prevent new stains.[teethokay.com]

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