Immunodeficiency in Children With Severe Craniofacial Anomalies

Gc, Miller; B, Say

doi:10.1097/00007611-198201000-00005

Cited by 4 publications

(3 citation statements)

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“…The association of facial birth defects in context with immunodeficiency [58] has been suggested in previous studies. A case report of two brothers, with unusual facial features including micrognathia along with other skeletal developmental problems, reported the presence of chemotactic defect and transient hypogammaglobulinemia [59].…”

Section: Association Between Skeletal Malocclusion and Other Medical mentioning

confidence: 99%

Skeletal Malocclusion: A Developmental Disorder With a Life-Long Morbidity

Joshi

Hamdan

Fakhouri³

2014

J Clin Med Res

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The likelihood of birth defects in orofacial tissues is high due to the structural and developmental complexity of the face and the susceptibility to intrinsic and extrinsic perturbations. Skeletal malocclusion is caused by the distortion of the proper mandibular and/or maxillary growth during fetal development. Patients with skeletal malocclusion may suffer from dental deformities, bruxism, teeth crowding, trismus, mastication difficulties, breathing obstruction and digestion disturbance if the problem is left untreated. In this review, we focused on skeletal malocclusion that affects 27.9% of the US population with different severity levels. We summarized the prevalence of class I, II and III of malocclusion in different ethnic groups and discussed the most frequent medical disorders associated with skeletal malocclusion. Dental anomalies that lead to malocclusion such as tooth agenesis, crowding, missing teeth and abnormal tooth size are not addressed in this review. We propose a modified version of malocclusion classification for research purposes to exhibit a clear distinction between skeletal vs. dental malocclusion in comparison to Angle’s classification. In addition, we performed a cross-sectional analysis on orthodontic (malocclusion) data through the BigMouth Dental Data Repository to calculate potential association between malocclusion with other medical conditions. In conclusion, this review emphasizes the need to identify genetic and environmental factors that cause or contribute risk to skeletal malocclusion and the possible association with other medical conditions to improve assessment, prognosis and therapeutic approaches.

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Section: Association Between Skeletal Malocclusion and Other Medical mentioning

confidence: 99%

Skeletal Malocclusion: A Developmental Disorder With a Life-Long Morbidity

Joshi

Hamdan

Fakhouri³

2014

J Clin Med Res

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“…There are early case reports on concomitant occurrence of idiopathic hypoparathyroidism and chronic Candida infections, suggesting that cellular immunity may be compromised, at least in some cases of this rare condition [37]. As CHD and typical facial stigmata were found to contribute to the syndrome in most cases reported later, the two patients included in this report with hypoparathyroidism, typical facies and immunologic disturbances but without CHD fit well into the broad spectrum of DGS [5,6,9,10,14,29]. Congenital absence of the thymus and parathyroid glands were the only features of DiGeorge's original description [15].…”

Section: Discussionmentioning

confidence: 63%

“…Other features such as cellular immunodeficiency have been reported at least for the Treacher-Collins syndrome [11,16,18,28,29]. Other features such as cellular immunodeficiency have been reported at least for the Treacher-Collins syndrome [11,16,18,28,29].…”

Section: Discussionmentioning

confidence: 95%

The DiGeorge syndrome

et al. 1988

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This study describes clinical signs and symptoms in 16 patients with the DiGeorge syndrome (DGS). Diagnosed on the basis of typical facial stigmata, a broad spectrum of severity is seen with respect to congenital heart disease, hypoparathyroidism and immunologic parameters. A simple index of severity is introduced that clearly differentiates complete forms of the syndrome (cDGS) with poor prognosis from partial forms of the syndrome (pDGS). Of 13 pDGS patients, 12 are still living; 8 underwent corrective heart surgery without infectious complications. Moderate to severe mental retardation is seen in all pDGS patients. Due to the lack of thymus function, immunodeficiency is a result of cDGS, whereas immunoregulatory disturbances (hypergammaglobulinaemia, high titres of specific antibody production) prevail in pDGS patients.

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2007

Taybi and Lachman's Radiology of Syndromes, Metabolic Disorders and Skeletal Dysplasias

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Immunodeficiency in Children With Severe Craniofacial Anomalies

Cited by 4 publications

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Skeletal Malocclusion: A Developmental Disorder With a Life-Long Morbidity

Skeletal Malocclusion: A Developmental Disorder With a Life-Long Morbidity

The DiGeorge syndrome

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