ABSTRACT
AIMS: To examine whether the learning difficulties seen in a proportion of children with DGS are secondary to cardiac pathology and treatment, or a feature of the DGS phenotype. METHODS: Cohort study of all patients with DGS and coexisting cardiac lesions within a region. Ten children with 22q11 deletion were assigned two controls each, matched for age, sex, cardiac lesion, and preoperative hemodynamic status but without DGS. The neurodevelopmental status was evaluated with the Ruth Griffiths test for babies and young children. RESULTS: Children with the 22q11 deletion showed a wide range of developmental quotient (DQ; mean 71, 95% CI 47 to 95) and subscale scores, but these as a group were significantly lower than those of the control group (DQ 113, 95% CI 108 to 118). Four of the DGS children had DQs below 60. Hypocalcaemia, prolonged postoperative ventilation, and abnormal neurology perioperatively were associated with a low DQ. CONCLUSIONS: A proportion of children with DGS have a very poor developmental outcome following cardiac surgery. This outcome is not attributable to the cardiac condition and its treatment alone, but represents either a pre-existing component of the syndrome or an interaction between the syndrome and its treatment.
Subject(s)
Central Nervous System Diseases/etiology , DiGeorge Syndrome/complications , Heart Defects, Congenital/complications , Case-Control Studies , Chromosomes, Human, Pair 22 , Cohort Studies , DiGeorge Syndrome/surgery , Gene Deletion , Heart Defects, Congenital/surgery , Humans , Hypocalcemia/complications , Infant , Infant, Newborn , Phenotype , Postoperative Complications , Treatment OutcomeABSTRACT
Intravenous acyclovir had a significant effect on the resolution of the skin rash in patients with acute zoster, but the 5-day course of therapy was not, in itself, sufficient to treat coexisting ocular involvement. In an open study, topical acyclovir was found to control herpes zoster kerato-uveitis, without recurrences and in a shorter time than if steroids alone were used. The use of steroids in combination with acyclovir led to prolonged treatment and high recurrence rates. A comparative trial of topical acyclovir versus steroids in the treatment of acute herpes zoster kerato-uveitis showed significant differences in favour of acyclovir in terms of the time to resolution of corneal epithelial disease, total treatment duration and the numbers of patients having a recurrence of infection. The reductions in treatment duration and recurrence rate would be expected to result in a reduced incidence of ocular damage and visual loss in acyclovir treated patients.
