Carbonated drinks and alcohol consumption may exacerbate hypocalcemia. Surveillance: Evaluation for nasal speech quality after language emergence antibody studies to assess seroconversion reevaluate immune status in childhood before administration of live vaccines annual complete blood count and differential serum ionized calcium every three to six months in infancy, every five years through childhood, every one to two years thereafter, preoperatively and postoperatively, and regularly during pregnancy TSH and free T4 annually ophthalmologic evaluation between age one and three years or as indicated audiology evaluation in infancy, at preschool age, and in school age children developmental assessments annually annual clinical surveillance for scoliosis dental examination every six months.Īgents/circumstances to avoid: Infants with lymphocyte abnormalities should not be immunized with live vaccines (e.g., oral polio, MMR). 83.2 ).Treatment of manifestations: Cardiac anomalies are treated as recommended by cardiologist surgical repair for palate anomalies as recommended by otolaryngologist feeding issues are treated with modification of spoon placement standard treatment for gastroesophageal reflux and gastrointestinal dysmotility immune deficiency requires aggressive treatment of infections rarely, prophylactic antibiotics, IVIG therapy, or thymic transplantation are required irradiated blood products are recommended until normalization of the immune system can be confirmed treatment of autoimmune disease as per immunologist calcium supplementation and referral to an endocrinologist and nephrologist because of increased risk of renal calculi if long-term supplementation is required standard treatment for growth hormone deficiency standard treatment for ocular anomalies hearing aids may be helpful for hearing loss occupational, physical, and speech therapy with introduction of sign language by age one year, educational and behavioral therapy support and treatment for psychiatric disease as indicated activity restriction as recommended by an orthopedist for cervical spine anomalies surgery and treatment as recommended by a nephrologist for renal anomalies routine dental treatment with consideration of sealants. The three-vessel and trachea view shows reduced diameter or unidentifiable aorta, with pronounced asymmetry between the aortic arch and pulmonary artery ( Fig. The pulmonary artery is disproportionally bigger than the aortic root. There is disproportion of the sizes of the outflow tracts. This sign is less evident in type B IAA because there is frequently a large malaligned VSD that balances right and left flow. The right ventricle appears larger than the left, and greater right ventricular flow is identified with color Doppler. Ventricular disproportion can be identified on the four-chamber view, especially in IAA type A and C. The key finding that raises suspicion of IAA is the disproportion of the left and right ventricles ( Fig. Most patients (90%) have an associated ventricular septal defect (VSD). Proximal interruption, between the innominate and left carotid artery (5% to 10%). The interruption is between the left carotid and left subclavian artery (50% to 60%). The interruption is distal to the left subclavian artery (30% to 40%) this form is more frequent in cases with associated truncus arteriosus. Depending on the level of the interrupted site, three types of IAA have been described, as follows : The etiology is unknown, although it has been related to genetic and chromosomal abnormalities, especially 22q11 microdeletion. IAA is due to a lack of connection between the proximal and distal portion of the aortic arch. IAA has a high prevalence of associated anomalies, including:Įxtracardiac abnormalities: central nervous system, urinary, and gastrointestinal malformations are the most frequently associated (present in 30% of cases).Ĭhromosomal abnormalities: type B IAA has a high association (60% to 70%) with 22q11 microdeletion.Ĭardiac anomalies: associated cardiac anomalies are common, especially truncus arteriosus (10% to 20%), left ventricular outflow tract obstruction (10% to 16%), aortic valvular stenosis (10%), aortopulmonary window, double-outlet right ventricle (5%), and transposition of great arteries (4% to 6%). IAA accounts for 0.1% of all prenatally detected congenital heart diseases (CHDs) and 0.2% to 1% of postnatal CHDs, affecting 1 : 300,000 newborns. IAA is characterized by the lack of continuity between the ascending and descending aorta.
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