Orthopedic and orthodontic management in a patient with DiGeorge Syndrome and Familial Mediterranean Fever: A case report

Aim The aim of this study is to report the case of the orthopedic and orthodontic treatment in a young patient affected by DiGeorge Syndrome and Familial Mediterranean Fever. Case report An 8‐year‐old boy with dysmorphic facial features was brought to our observation. Anamnesis revealed signs of fet...

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Published in:Special care in dentistry 2019-05, Vol.39 (3), p.340-347
Main Authors: Cazzolla, Angela Pia, Lacaita, Maria Grazia, Lacarbonara, Vitantonio, Zhurakivska, Khrystyna, Franco, Annarosa, Gissi, Irene, Testa, Nunzio Francesco, Marzo, Giuseppe, Muzio, Lorenzo
Format: Article
Language:English
Subjects:
25-Hydroxyvitamin D
Amyloidosis
Antihypertensives
Arthralgia
Blood
Brain
Calcitriol
Case reports
Colchicine
DiGeorge syndrome
DNA sequencing
Dura mater
Erythropoietin
Familial Mediterranean Fever
Fetuses
Hematoma
Hyperparathyroidism
Immunosuppressive agents
Low protein diet
Meninges
Nephritis
Nutrient deficiency
Orthodontics
Orthopedics
orthopedic‐orthodontic treatment
Polyserositis
Pyrin protein
Rehabilitation
Thymic hypoplasia
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Summary:Aim The aim of this study is to report the case of the orthopedic and orthodontic treatment in a young patient affected by DiGeorge Syndrome and Familial Mediterranean Fever. Case report An 8‐year‐old boy with dysmorphic facial features was brought to our observation. Anamnesis revealed signs of fetal respiratory distress, previous surgically removed subdural hematoma, recurrent episodes of fever, arthralgia, polyserositis, hepatosplenomegaly, chronic interstitial nephritis with hypertension, microprotenuria, normocytic anemia, hyperparathyroidism and secondary amyloidosis. DNA sequencing identified microdeletions on 22q11.2 and MEFV mutation. The patient was in treatment with immunosuppressive agents, colchicine, antihypertensive therapy, calcitriol, erythropoietin, and low‐protein diet. An intraoral and extraoral examination, as well as radiographic and model analysis, were performed in order to define an accurate diagnosis and a proper rehabilitation planning. An orthopedic‐orthodontic treatment was performed and satisfactory final results obtained. Conclusions Literature does not describe cases of patients having DiGeorge syndrome associated to Familial Mediterranean Fever undergoing orthodontic and orthopedic treatment. In this patient an early started treatment with a timely management of orthopedic and orthodontic forces allowed to reach positive and stable results.
ISSN:0275-1879
1754-4505
DOI:10.1111/scd.12381