Caregiver disclosure of common early childhood pediatric urologic surgeries.

BACKGROUND: Certain pediatric urologic diagnoses can have serious long-term adverse health outcomes. As a result, it is important for a child to be aware of their diagnosis and a prior surgery. When children have surgery prior to the age of memory formation, it is incumbent upon their caregiver to disclose this surgery. When and how to disclose this information and even if this occurs, is not clear. OBJECTIVE: We developed a survey to assess caregiver plans to disclose early childhood pediatric urologic surgery and evaluate for predictors of disclosure and resources needed. METHODS: A questionnaire was distributed to caregivers of male children ≤4 years old undergoing single stage repair of hypospadias, inguinal hernia, chordee, or cryptorchidism as part of an IRB approved research study. These surgeries were chosen due to being outpatient surgeries with potential long-term complications and impact. The age criteria was chosen due to likely being before patient memory formation and thus reliance on caregiver disclosure of prior surgery. Surveys were collected the day of surgery and contained information on caregiver demographics, validated health literacy screening, and plans to disclose surgery. RESULTS: 120 survey responses were collected (Summary Table). The majority of caregivers responded affirmatively to planning to disclose their child's surgery (108; 90%). There was no impact of caregiver age, gender, race, marital status, education level, health literacy, or personal surgical history on plans to disclose surgery (p ≥ 0.05). Plan to disclose was also not different across urologic surgery type. Race was significantly associated with being "concerned or nervous about disclosing the surgery to the patient". The median patient age for planned disclosure was 10 years (IQR: 7-13). Only 17 respondents (14%) stated they received any information about how to discuss this surgery with the patient, however 83 (69%) felt this information would be helpful. CONCLUSIONS: Our study suggests that most caregivers plan to discuss early childhood urologic surgeries with children, however want further guidance in how to talk to their child. While no specific surgery or demographic factor was found to be significantly associated with plans to disclose surgery, it is concerning that one in ten patients will potentially never learn about impactful surgery they had as a child. There is an opportunity for us to better counsel our patients' families about surgical disclosure and fill this gap with quality improvement efforts.

Cognitive ability predicts degree of genetic abnormality in participants with 18q deletions.

One of the most common chromosomal deletions is a loss of genetic material from the long arm of chromosome 18. Most individuals with this condition exhibit mental retardation (68%), yet previous attempts to link cognitive status to deletion size have not shown an association, possibly because cases with additional genetic abnormalities were included. We studied 46 participants ranging from 3 to 35 years of age who had a pure genetic abnormality by excluding those with mosaicism or complex genetic rearrangements. Our patients had terminal deletions ranging from a proximal breakpoint at 18q21.1 (greater genetic abnormality, larger deletion size) to a more distal breakpoint at 18q23 characterized with molecular genetic techniques. Cognitive ability, assessed with the age-appropriate measure (Bayley, 1993 , Differential Ability Scale, Wechsler Scales), ranged from IQ = 49 to 113, with a predominance of mild and moderate mental retardation. Using multivariate regression, deletion size breakpoint rank order was predicted by cognitive ability, age, and adaptive behavior (Vineland Adaptive Behavior Scales), accounting for 36% of the variance in deletion size. However, lower cognitive ability (beta = .34, p = .032) and younger age (beta = .296, p = .024) predicted a larger deletion size, but adaptive behavior (beta = .225, p = .15) did not. An additional multivariate regression showed that cognitive ability and age together accounted for 33% of the variance in deletion size, whereas univariate regression showed that cognitive ability accounted for 26% of the variance and age accounted for 11% of the variance. These findings suggest that degree of cognitive impairment is associated with genetic abnormality when a large sample of individuals with "pure" deletions of genetic material from chromosome 18 is examined.

Growth hormone benefits children with 18q deletions.

Most individuals with constitutional deletions of chromosome 18q have developmental delays, dysmyelination of the brain, and growth failure due to growth hormone deficiency. We monitored the effects of growth hormone treatment by evaluating 23 individuals for changes in growth, nonverbal intelligence quotient (nIQ), and quantitative brain MRI changes. Over an average of 37 months, the treated group of 13 children had an average nIQ increase of 17 points, an increase in height standard deviation score of 1.7, and significant change in T1 relaxation times in the caudate and frontal white matter. Cognitive changes of this magnitude are clinically significant and are anticipated to have an effect on the long-term outcomes for the treated individuals.

Who enrolls in observational end of life research? Report from the cultural variations in approaches to end of life study.

BACKGROUND: Response bias in end of life research is important though not well understood. OBJECTIVES: To compare consenting advanced cancer patient participants and refusers in observational end of life research. We hypothesized that, compared with refusers, consenters would: 1) have a stronger belief in personal gain from research participation, 2) be more satisfied with their medical care, 3) be more satisfied with support from family and friends, and 4) be in less physical discomfort compared with refusers. METHODS: Sixty eight patients consented to enroll in the 'parent' observational study. Thirty six patients refused to participate. Reasons for refusal were recorded verbatim and coded using qualitative techniques. Both patient consenters and patient refusers were asked the same questions regarding personal gain, satisfaction with medical care, family and social support, and physical discomfort. RESULTS: Consenters believed that they had more to gain from participation in research compared with refusers (p = 0.04). Consenters felt that aches or pain were more of a problem for them compared with refusers (p < 0.001). Both satisfaction with medical care and with support from family and friends were similar between consenters and refusers. CONCLUSIONS: Consenting study participants in observational research at end of life believe they have more to gain from study participation than do refusers. Contrary to our hypothesis, consenting participants were those who were experiencing greater physical discomfort compared to refusers.

"He is everything": religion's role in the lives of immigrant youth.

Using emergent theme analysis of qualitative interview data in combination with quantitative survey data, the role of religion in the lives of immigrant youth was explored. Latino, Haitian, and Chinese teenagers described, in their own rich words, the significance of religion to them; their responses are reflected in themes that point to the potential protective role of religion for some immigrant groups.