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Rosselli-Gulienetti Syndrome
Rosselli Gulienetti Syndrome

Presentation

The present clinical report illustrates an alternative solution by integrating a small retentive component into a maxillary complete denture. [go.gale.com]

Onychodystrophy may be present. [orpha.net]

Jaw & Teeth

  • Abnormal Teeth

    Clinical description Zlotogora-Ogur syndrome is a congenital disorder characterized by sparse and twisted hair ( pili torti ) and absent or sparse eyebrows, hypohidrosis, dry skin, palmoplantar keratoderma, abnormal teeth (delayed eruption, microdontia [orpha.net]

Skin

  • Sparse, Abnormal Hair

    progressive sparse, abnormal hair Head And Neck Ears: anteverted ears Skeletal Feet: syndactyly, cutaneous Skin Nails Hair Nails: onychodysplasia may be present [malacards.org]

Psychiatrical

  • Anger

    France : Aix-en-Provence, Ajaccio, Alsace, Angers, Annecy, Antibes, Aquitaine, Arles, Armagnac, Auch, Auvergne, Avignon, Avoriaz, Bastia, Bayonne, Beaune, Besançon, Biarritz, Bonifacio, Bordeaux, Boulogne-sur-Mer, Briançon, Brittany, Burgundy, Cabourg [maria-online.com]

Neurologic

  • Excitement

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    […] orthostatic tachycardia syndrome Potter's syndrome Poult Enteritis Mortality Syndrome Powell Chandra Saal Syndrome Powell Venencie Gordon syndrome Ppm-X Syndrome Prader-Willi syndrome + Prader-Willi-Like Syndrome Prata Libéral Gonçalves Syndrome Pre-Excitation [rgd.mcw.edu]

Treatment

The prosthetic approach is an age specific treatment involving the fabrication of partial or complete dentures initially. [thefreelibrary.com]

Health Conditions Therapies Allergies General Information Symptoms and Causes Prevention and Treatment References Rosselli-Gulienetti syndrome (RGS) is a form of ectodermal dysplasia, one of a group of syndromes deriving from abnormalities of the ectodermal [wellness.com]

Treatment[edit] There is no specific treatment or cure for individuals affected with this type of syndrome, though some of the abnormal physical features may be surgically correctable.[4] Epidemiology[edit] About 35 cases have been described in medical [en.wikipedia.org]

Prognosis

[…] can rarely be a cause of death fingernails and toenails may be absent, thick, abnormally shaped, discoloured, ridged, slow growing, or brittle lack of breast development absent fingers or toes recurrent infections missing ears and hearing difficulties Prognosis [pacs.de]

Diagnosis - Zlotogora syndrome Prognosis - Zlotogora syndrome The long-term prognosis for people with this syndrome is largely unknown because there are so few cases. [checkorphan.org]

The patient had recently undergone extraction of remaining poor prognosis teeth in the upper and lower arches. [thefreelibrary.com]

Prognosis This syndrome is rare and has only recently been delineated, so it is difficult to find any reliable information on long-term outlook. Patients who are adequately managed seem to do well in the medium term. [patient.info]

Etiology

Etiology Zlotogora-Ogur syndrome is caused by mutations in the gene PVRL1 (11q23-q24) which encodes nectin-1, the principal receptor used by alpha-herpesviruses to mediate entry into human cells. [orpha.net]

The authors concluded that WNT10A is a major gene in the etiology of isolated hypodontia. [ncbi.nlm.nih.gov]

Epidemiology

Summary Epidemiology The prevalence is unknown but to date, less than 50 cases have been described in the literature. The disorder is frequent on Margarita Island due to a founder effect. [orpha.net]

Treatment[edit] There is no specific treatment or cure for individuals affected with this type of syndrome, though some of the abnormal physical features may be surgically correctable.[4] Epidemiology[edit] About 35 cases have been described in medical [en.wikipedia.org]

Epidemiology ED is rare with an estimated prevalence of 1:17,000. It can occur in any race but is most commonly seen in Caucasians. [pacs.de]

Prevention

The presence of an oronasal fistula presents a challenge to maxillary complete denture fabrication because leakage of air from the nasal cavity through the fistula prevents the formation of an adequate border seal and further absence of an anterior portion [go.gale.com]

If symptoms are managed and complications prevented, people with RGS can lead a long and healthy life. This should prevent or delay tooth loss and enhances early replacement of missing teeth for the preservation of function and esthetics. [thefreelibrary.com]

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