Early Vocal Development in Tuberous Sclerosis Complex.

BACKGROUND: Our goal was to assess for the first time early vocalizations as precursors to speech in audio-video recordings of infants with tuberous sclerosis complex (TSC). METHODS: We randomly selected 40 infants with TSC from the TSC Autism Center of Excellence Research Network dataset. Using human observers, we analyzed 74 audio-video recordings within a flexible software-based coding environment. During the recordings, infants were engaged in developmental testing. We determined syllables per minute (volubility), the number of consonant-vowel combinations, such as 'ba' (canonical babbling), and the canonical babbling ratio (canonical syllables/total syllables) and compared the data with two groups of typically developing (TD) infants. One comparison group's data had come from a laboratory setting, while the other's had come from all-day Language Environment Analysis recordings at home. RESULTS: Compared with TD infants in laboratory and all-day Language Environment Analysis recordings, entry into the canonical babbling stage was delayed in the majority of infants with TSC, and the canonical babbling ratio was low (TD mean = 0.346, SE = 0.19; TSC mean = 0.117, SE = 0.023). Volubility level in infants with TSC was less than half that of TD infants (TD mean = 9.82, SE = 5.78; TSC mean = 3.99, SE = 2.16). CONCLUSIONS: Entry into the canonical stage and other precursors of speech development were delayed in infants with TSC and may signal poor language and developmental outcomes. Future studies are planned to assess prediction of language and developmental outcomes using these measures in a larger sample and in more precisely comparable recording circumstances.

Panuveitis in Sweet syndrome.

Ocular manifestations of Sweet syndrome, or acute febrile neutrophilic dermatosis, are usually limited to the anterior segment. We report the case of a patient with bilateral panuveitis and retinal vasculitis associated with Sweet syndrome. A 45-year-old Asian female with an undiagnosed febrile illness with rash presented with bilateral panuveitis with haemorrhagic occlusive retinal vasculitis. Skin biopsy confirmed Sweet Syndrome. Intraocular inflammation resolved with a combination of topical and systemic corticosteroids as well as intravenous cyclophosphamide, with resulting permanent severe right visual impairment. Although an uncommon condition, Sweet syndrome should be considered in any febrile patient with skin lesions and uveitis.

Informal caregiving for elders in Sweden: an analysis of current policy developments.

In Sweden, care of elderly people is a public responsibility. There are comprehensive public policies and programs providing health care, social services, pensions, and other forms of social insurance. Even so, families are still the major providers of care for older people. In the 1990s, the family was "rediscovered" regarding eldercare in Sweden. New policies and legislative changes were promoted to support family caregivers. The development of services and support for caregivers at the municipal level has been stimulated through the use of national grants. As a result, family caregivers have received more recognition and are now more visible. However, the "Swedish model" of publicly financed services and universal care has difficulty addressing caregivers. Reductions in institutional care and cutbacks in public services have had negative repercussions for caregivers and may explain why research shows that family caregiving is expanding. At the same time, a growing "caregivers movement" is lobbying local and national governments to provide more easily accessible, flexible, and tailored support. In 2009, the Swedish Parliament passed a new law that states: "Municipalities are obliged to offer support to persons caring for people with chronic illnesses, elderly people, or people with functional disabilities." The question is whether the new legislation represents a paradigm shift from a welfare system focused on the individual to a more family-oriented system. If so, what are the driving forces, motives, and consequences of this development for the different stakeholders? This will be the starting point for a policy analysis of current developments in family caregiving of elderly people in Sweden.

Self-management of health-behaviors among older and younger workers with chronic illness.

OBJECTIVE: To examine the self-management of health behaviors carried out by older (aged 50-69 years) and younger workers (aged 20-49 years) with a chronic illness. METHODS: Questionnaire data was collected from 759 employees with a diagnosed chronic illness. Four categories of self-managing health behaviors were examined: using prescribed medication, monitoring and responding to symptoms, managing an appropriate diet and exercising. RESULTS: The majority of participants (56-97%) reported being advised to carry out health behaviors at home and at work. Controlling for confounding factors, medication use was associated with younger and older workers. Managing an appropriate diet was associated with younger workers with asthma, musculoskeletal pain or diabetes. Exercising was associated with younger workers with asthma and with older workers with heart disease, arthritis and rheumatism or diabetes. CONCLUSIONS: The findings indicate that there are differences in diet and exercise activities among younger and older workers. PRACTICE IMPLICATIONS: To increase self-management in health behaviors at work, improved communication and understanding between the different health professions and the patient/employee is required so that different tailored approaches can be effectively targeted both by age and within the context of the working environment, to those managing asthma, heart disease, diabetes and arthritis and rheumatism.

Presumed CMV associated necrotizing retinopathy in a non-HIV immunocompromised host.

Herpetic necrotizing retinitis is caused by the herpesvirus group of viruses, including cytomegalovirus (CMV). Reports defining causative viruses have shown CMV to be a rare cause of acute retinal necrosis. In fact, there is only one report in the literature of CMV associated with progressive outer retinal necrosis, which is most commonly associated with Varicella zoster virus. A case is presented of CMV-related necrotizing retinopathy concurrent with subtle signs of herpetic retinitis in an immunocompromised patient, highlighting the spectrum of response of herpetic retinopathies.