6 research outputs found

    Diagnosis and management of Silver–Russell syndrome: first international consensus statement

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    This Consensus Statement summarizes recommendations for clinical diagnosis, investigation and management of patients with Silver–Russell syndrome (SRS), an imprinting disorder that causes prenatal and postnatal growth retardation. Considerable overlap exists between the care of individuals born small for gestational age and those with SRS. However, many specific management issues exist and evidence from controlled trials remains limited. SRS is primarily a clinical diagnosis; however, molecular testing enables confirmation of the clinical diagnosis and defines the subtype. A 'normal' result from a molecular test does not exclude the diagnosis of SRS. The management of children with SRS requires an experienced, multidisciplinary approach. Specific issues include growth failure, severe feeding difficulties, gastrointestinal problems, hypoglycaemia, body asymmetry, scoliosis, motor and speech delay and psychosocial challenges. An early emphasis on adequate nutritional status is important, with awareness that rapid postnatal weight gain might lead to subsequent increased risk of metabolic disorders. The benefits of treating patients with SRS with growth hormone include improved body composition, motor development and appetite, reduced risk of hypoglycaemia and increased height. Clinicians should be aware of possible premature adrenarche, fairly early and rapid central puberty and insulin resistance. Treatment with gonadotropin-releasing hormone analogues can delay progression of central puberty and preserve adult height potential. Long-term follow up is essential to determine the natural history and optimal management in adulthood

    Prevalence of otologic complaints in patients with temporomandibular disorder

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    The prevalence and rank of order of 4 otologic complaints in 200 temporomandibular disorder (TMD) patients, as well as the relationship between the complaints and TMD subgroups, were investigated and compared with an asymptomatic control group. No subjective otologic complaints were reported by 45 (22.5%) TMD patients; the remaining 155 (77.5%) patients had at least 1 otologic complaint. Otalgia, tinnitus, vertigo, and hearing loss were reported by 63.6%, 59.1%, 50%, and 36.4%, respectively, of the subjects with myofascial pain and dysfunction; by 46.1%, 44.2%, 32.5%, and 22% of the patients with internal derangement; and by 62.5%, 45.8%, 41.6%, and 20.8% of the patients with both myofascial pain and dysfunction and internal derangement. However, the incidence of otalgia (8%), tinnitus (26%), vertigo (14%), and hearing loss (14%) was found to be lower for the control group. Statistically, the control group had fewer otologic complaints. Patients in the TMD groups had high incidences of otologic complaints compared with the control subjects without TMD signs or symptoms. Aural symptoms in patients with internal derangement or myofascial pain and dysfunction, or their combination, were nonspecific

    Diagnosis and management of Silver–Russell syndrome: first international consensus statement

    No full text
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