Although nonadherence to prescribed therapies is widespread, it is particularly problematic with highly active antiretroviral therapy for HIV infection. This review of >50 studies in the area of pediatric HIV infection revealed varying methods for assessing antiretroviral adherence with a wide range of estimates of adherence. Correlates of adherence could be grouped as those relating to the medication, the patient, and the caregiver/family, with many conflicting findings and a lack of theory guiding the research. Only 8 studies, mainly small feasibility or pilot investigations, evaluated highly active antiretroviral therapy adherence interventions in pediatric populations. We conclude with specific recommendations for assessment and clinical management of adherence and discuss directions for future research in this area. KeywordsHIV/AIDS; adherence; compliance; interventions; pediatric Adherence to therapy, or the extent to which a patient's behaviors coincide with medical advice mutually negotiated between the health professional and the patient, is a universal HHS Public Access Author Manuscript Author ManuscriptAuthor ManuscriptAuthor Manuscript challenge with all illnesses and in all age groups. 1 Each year in the United States, 500 000 physicians write 1.8 billion prescriptions involving 55 000 pharmacies. 2 However, many of these prescribed medications are never taken, with rates of nonadherence ranging from 15% to 93%. 3 Among persons with chronic illnesses, nonadherence is especially problematic, occurring in up to 82% of cases. [4][5][6] The effects of nonadherence range from individual disability (eg, unrelieved pain) to global threat (eg, development of treatment-resistant bacteria or viruses). The yearly monetary costs of nonadherence exceed $100 billion. 7Adherence is particularly critical with highly active antiretroviral therapy (HAART) in the treatment of pediatric HIV infection. The data on HAART for pediatric HIV infection, although scarcer than for adults, suggest that medication adherence is a strong predictor of therapeutic impact. 8,9 For example, Wiener et al 10 observed that among children with an HIV-1 RNA viral load (VL) < 10 000, 75% had taken 100% of their medication doses in the previous week, whereas among those with a VL of ≥10 000, only 36% reported taking all of their medication.Despite the benefits of HAART in treating pediatric HIV infection [11][12][13][14][15] and the adverse consequences of nonadherence, adherence is reportedly suboptimal among children. [16][17][18][19][20] It is likely thwarted by multiple barriers 21,22 and complicated because, unlike with many other chronic illnesses, most children who are born with HIV in the United States are ethnic/racial minorities who live in chronic poverty with limited resources and face discrimination, family disruption, substance abuse, and the stressors of life in the inner city. 23 In addition, stigma is greater for HIV/AIDS than other chronic illnesses, which often leads caregivers to conceal the child's diagnosis a...

ObjectiveTo assess brain morphometry in a sample of patients with juvenile-onset Huntington disease (JOHD) and several mouse models of Huntington disease (HD) that likely represent the human JOHD phenotype.MethodsDespite sharing the mutation in the Huntingtin gene, adult-onset HD characteristically presents as a hyperkinetic motor disorder, while JOHD typically presents as a hypokinetic motor disease. The University of Iowa Kids-JHD program enrolls individuals 5 to 25 years of age who have already received the clinical diagnosis. A total of 19 children with juvenile HD (JHD) (mean CAG = 72) were studied. Patients with JHD were compared to healthy controls (n = 234) using a cross-sectional study design. Volumetric data from structural MRI was compared between groups. In addition, we used the same procedure to evaluate brain morphology of R6/2, zQ175, HdhQ250 HD mice models.ResultsParticipants with JHD had substantially reduced intracranial volumes. After controlling for the small intracranial volume size, the volumes of subcortical regions (caudate, putamen, globus pallidus, and thalamus) and of cortical white matter were significantly decreased in patients with JHD. However, the cerebellum was proportionately enlarged in the JHD sample. The cerebral cortex was largely unaffected. Likewise, HD mice had a lower volume of striatum and a higher volume of cerebellum, mirroring the human MRI results.ConclusionsThe primary pathology of JOHD extends beyond changes in the striatal volume. Brain morphology in both mice and human patients with JHD shows proportional cerebellar enlargement. This pattern of brain changes may explain the unique picture of hypokinetic motor symptoms in JHD, which is not seen in the hyperkinetic chorea-like phenotype of adult-onset HD.

Despite limitations, the study supports that there are symptoms in the JHD population that are not considered classic, however, are common and significant for patients and caregivers.

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