Helping children with disabilities: disability registers

This article discusses health registers. It describes what they are, why they are required, and the problems that might be encountered setting one up. It discusses the type of data held and how this data is captured and handled.     

Psychosocial aspects of disability in children

General psychosocial issues must be considered in facilitating the development of children with chronic illnesses and disabilities. A coping model and the developmental stages can be used as a framework to regard the impact of disabling conditions on children and the interventions that encourage psychosocial growth. Health professionals are encouraged to recognize the key role that they can play in promoting effective coping and growth for disabled children and members of their families and to incorporate psychosocial interventions into their practice.     

Psychopathology in children with intellectual disability

Recent advances are reviewed in understanding the heightened prevalence of psychopathology and maladaptive behavior among children with intellectual disability. Researchers have traditionally emphasized measurement and prevalence issues, using either psychiatric assessments or rating scales to identify the prevalence of various problems in children with intellectual disability. Yet the time is ripe to shift directions, and identify more precisely why children are at increased risk for psychopathology to begin with. Although several "biopsycho-social" hypotheses are reviewed, a particularly promising line of work links psychopathology to genetic intellectual disability syndromes. Psychiatric vulnerabilities in several syndromes are reviewed, as are the advantages of phenotypic work for understanding psychopathology among children with intellectual disability more generally.     

Rhinosinusitis in children: the role of surgery

Indications and nature of surgery for chronic rhinosinusitis (CRS) have yet to be elucidated in children. After review of the literature and based on their experience, the authors suggest guidelines for the treatment of CRS in children. They suggest grouping children with rhinosinusitis into two groups: those with complicated acute rhinosinusitis and those with CRS. For the first group, the authors suggest an early surgical intervention because of the potential serious consequences and sometimes irreversible damage. For the second group, most agree that maximal medical management should be the first line of treatment with antibiotics, nasal lavage and as a last resort surgery. It is important to realize that surgery should be considered in these cases if medical treatment fails. Once surgery is recommended, the kind of surgery then becomes an issue between adenoidectomy, endoscopic sinus surgery or a combination of the two depending on the age and other conditions.     

Cecal volvulus in children with mental disability

Two cases of cecal volvulus in children with mental disability are described. Case 1: a 3-year-old girl with trisomy 18 was admitted with abdominal pain and vomiting. She had received left lateral segmentectomy 6 months earlier because of hepatoblastoma. Release of the cecal volvulus followed by the fixation of the cecum and ascending colon to the right retroperitonium was performed. Case 2: a 15-month-old boy with Cornelia de Lange syndrome who had undergone a standard Nissen’s fundoplication. On the sixth postoperative day, progressive abdominal distention developed. Abdominal color Doppler ultrasonography from the right rear side revealed a clockwise-twisted ileocecal artery and vein and a dilated colon with tapering configuration. On the ninth postoperative day, emergent release of the cecal volvulus followed by ascending colostomy through a perforation site was performed. Approximately 40 children with cecal volvulus have so far been reported, of whom 13 are mentally disabled. We speculated that in the cases reported here, distention of the intestine accompanying the mental disability and the previous surgery contributed to the development of cecal volvulus in addition to the prerequisite of abnormal mobility of the cecum. Pediatric surgeons should consider the cecal volvulus as a cause of intestinal obstruction in mentally disabled children.     

Heart transplantation in children with intellectual disability: An analysis of the UNOS database

Heart transplantation in children with intellectual disability (ID) is an issue of debate due to the shortage of available donor organs. We sought to perform the first large‐scale retrospective cohort study describing the prevalence and outcomes of heart transplantation in this population. The United Network of Organ Sharing database was queried from 2008 to 2015 for pediatric patients (age <19 years) receiving first, isolated heart transplant. Recipients were divided into three subgroups: definite ID, probable ID, and no ID. The chi‐square test was used to compare patients’ baseline characteristics. Kaplan‐Meier and Cox proportional hazard regression analyses were used to estimate the association between ID and death‐censored graft failure and patient survival. Over the study period, 565 pediatric patients with definite (131) or probable (434) ID received first heart transplant, accounting for 22.4% of all first pediatric heart transplants (n=2524). Recipients with definite ID did not significantly differ from those without ID in terms of gender, ethnicity, ischemia time, severity of pretransplant condition (waitlist status, mechanical ventilation, inotrope dependence, ECMO, VAD, PVRI, infection prior to transplant), or incidents of acute rejection within the first year. ID was associated with prolonged waitlist time (P<.001). Graft and patient survival at 3 years was equivalent between children with and without ID (P=.811 and .578, respectively). We conclude that intellectual disability is prevalent in children receiving heart transplants, with 22.4% of recipients over the study period having definite or probable ID. ID does not appear to negatively affect transplantation outcomes. Future studies are needed to assess long‐term outcomes of transplantation in this population.     

Disparities in the prevalence of disability between black and white children

OBJECTIVES: To examine disparities in the prevalence of the limitation of activity caused by chronic conditions or disability for black and white non-Hispanic children and to examine trends over time in the prevalence of disability. DESIGN: We analyzed data on 419,843 children (22,758 with a disability) younger than 18 years included in 14 annual editions of the National Health Interview Survey spanning the period 1979-2000. SETTING: Noninstitutionalized population in the United States. INTERVENTIONS: None. MAIN OUTCOME MEASURE: Prevalence of disability. RESULTS: The prevalence of disability increased markedly for both black and white children between 1979 and 2000. Bivariate analysis demonstrated racial differences that fluctuated through time, but persisted through 2000, with black children experiencing a higher prevalence of disability than white children. Multivariate analyses conducted on the 1999-2000 data indicated that the black-white difference in disability prevalence could be explained entirely by differences in poverty status. CONCLUSION: Black children have higher rates of disability primarily owing to their increased exposure to poverty.