Comparative analysis of self-injurious behaviour topographies in young children with and without developmental delay.

BACKGROUND: The present study presents post hoc analyses of specific topographies of self-injurious behaviour (SIB) exhibited by young children with developmental delay (DD) and children with typical development (TD). We conducted these analyses to better understand similarities and differences between the groups from a developmental perspective. No previous study has compared the prevalence, severity and co-occurrence of specific topographies of SIB in young children. METHOD: The participants were parents of two groups of children one with DD (n = 49, mean age = 37.5 months) and one with TD (n = 49, mean age = 36.6 months). Individual items of the SIB subscale from the Repetitive Behaviour Scale-Revised were used in the analyses. RESULTS: Seven of the eight Repetitive Behaviour Scale-Revised SIB categories were reported for both groups. Children in the DD group were significantly more likely to engage in Hits Self against Surface or Object, Hits Self with Body Part, Inserts Finger or Object, Skin Picking and Bites Self. Parental ratings of severity were also significantly greater for the DD group for these five topographies. The DD group engaged in a significantly greater number of SIB topographies than the children in the TD group. Children in the TD group were more likely to exhibit a single SIB topography while the DD group were more likely to engage in two or more topographies. Topographies involving self-hitting were not only more frequent among the children in the DD group but also more likely to be rated as moderate or severe in nature. CONCLUSIONS: Compared with the TD group, the topographies of SIB exhibited by the DD group were more prevalent, more severe and co-occurred with greater frequency. Inclusion of a group of children with TD provided an important comparative context for the occurrence of SIB in children with DD.

Refinement of the Region for Split Hand/Foot Malformation 5 on 2q31.1.

Background: Deletions that encompass 2q31.1 have been proposed as a microdeletion syndrome with common clinical features, including intellectual disability/developmental delay, microcephaly, cleft palate, growth delay, and hand/foot anomalies. In addition, several genes within this region have been proposed as candidates for split hand-foot malformation 5 (SHFM5). Methods: To delineate the genotype-phenotype correlation between deletions of this region, we identified 14 individuals with deletions at 2q31.1 detected by microarray analysis for physical and developmental disabilities. Results: All subjects for whom detailed clinical records were available had neurological deficits of varying degree. Seven subjects with deletions encompassing the HOXD cluster had hand/foot anomalies of varying severity, including syndactyly, brachydactyly, and ectrodactyly. Of 7 subjects with deletions proximal to the HOXD cluster, 5 of which encompassed DLX1/DLX2, none had clinically significant hand/foot anomalies. In contrast to previous reports, the individuals in our study did not display a characteristic gestalt of dysmorphic facial features. Conclusion: The absence of hand/foot anomalies in any of the individuals with deletions of DLX1/DLX2 but not the HOXD cluster supports the hypothesis that haploinsufficiency of the HOXD cluster, rather than DLX1/DLX2, accounts for the skeletal abnormalities in subjects with 2q31.1 microdeletions.

Comparing the impacts of hiking, skiing and horse riding on trail and vegetation in different types of forest.

Nature-based tourism in protected areas has increased and diversified dramatically during the last decades. Different recreational activities have a range of impacts on natural environments. This paper reports results from a comparison of the impacts of hiking, cross-country skiing and horse riding on trail characteristics and vegetation in northern Finland. Widths and depths of existing trails, and vegetation on trails and in the neighbouring forests were monitored in two research sites during 2001 and 2002. Trail characteristics and vegetation were clearly related to the recreational activity, research site and forest type. Horse trails were as deep as hiking trails, even though the annual number of users was 150-fold higher on the hiking trails. Simultaneously, cross-country skiing had the least effect on trails due to the protective snow cover during winter. Hiking trail plots had little or no vegetation cover, horse riding trail plots had lower vegetation cover than forest plots, while skiing had no impact on total vegetation cover. On the other hand, on horse riding trails there were more forbs and grasses, many of which did not grow naturally in the forest. These species that were limited to riding trails may change the structure of adjacent plant communities in the long run. Therefore, the type of activities undertaken and the sensitivity of habitats to these activities should be a major consideration in the planning and management of nature-based tourism. Establishment of artificial structures, such as stairs, duckboards and trail cover, or complete closure of the site, may be the only way to protect the most sensitive or deteriorated sites.

Developmental screenings in rural settings: a comparison of the child development review and the Denver II Developmental Screening Test.

Screening results for the Child Development Review (CDR) and the Denver II Developmental Screening Test (Denver II) were compared in two locations: the Cheyenne River Reservation in South Dakota and Sioux Falls, S.D. Seventy-three white, Native American and other minority children, half originating from the reservation and half from Sioux Falls, were randomly assigned to take one developmental screening test. A chi-square analysis indicated a significant difference in results across tests. Specifically, more CDR than Denver II subjects passed the screening and more Denver II than CDR subjects failed the screening. This pattern held for subjects living on, but not off, the reservation. Thus, for Native American, white and other minority children living on the Cheyenne River Reservation, the CDR may be undersensitive and/or the Denver II oversensitive to suspect presentations. Medical practitioners are advised to use these instruments with caution in rural settings.

Reliability and validity of the Gillette Functional Assessment Questionnaire as an outcome measure in children with walking disabilities.

A 10-level, parent-report walking scale encompassing a range of walking abilities from nonambulatory to ambulatory in all community settings and terrains was developed at Gillette Children's Specialty Healthcare (GCSH) as part of the Gillette Functional Assessment Questionnaire (FAQ). The reliability and validity of the walking-scale portion of the FAQ were tested on a group of individuals seen in the Motion Analysis Laboratory at GCSH between May 1996 and January 1997. A complete data set on 41 individuals with neuromuscular conditions represented the community ambulation levels (6-10) of the walking scale. Good test-retest reliability among parents and good interrater reliability between parents and community caregivers was demonstrated. Content and concurrent validity were also high, as assessed by correlation to standardized functional outcome measures, energy expenditure, and gait-analysis information. A reliable and valid scale specific to the task of walking such as the FAQ can assist clinicians in documenting functional change in children with chronic neuromuscular conditions.

Effectiveness of methylphenidate in Native American children with fetal alcohol syndrome and attention deficit/hyperactivity disorder: a controlled pilot study.

This pilot study was designed to assess the short-term effectiveness and side effects of methylphenidate in treating symptoms of attention deficit hyperactivity disorder (ADHD) in Native American children (5 to 12 years old) with documented fetal alcohol syndrome (FAS) or partial fetal alcohol syndrome. Using strict criteria for the diagnosis of FAS and ADHD, a randomized double-blind cross-over study of two placebos and a fixed dose of methylphenidate was completed in 4 Native American children in a residential school. Each treatment condition lasted 5 days, and daily observational outcome measures, the Conners Parent Rating Scale (CPRS-48), and the Conners Teacher Rating Scale (CTRS-39), were employed. Methylphenidate significantly improved scores of the Hyperactivity Index Scale on the CPRS-48 and the CTRS-39 but not the Daydreaming-Attention score on the CTRS-39. Side effects were similar to those traditionally found in other populations. The promising preliminary results suggest that a more definitive study of methylphenidate in Native and non-Native children with FAS and ADHD is warranted.

Trazodone is only slightly faster than fluoxetine in relieving insomnia in adolescents with depressive disorders.

This retrospective chart review examined the relative effectiveness of fluoxetine and trazodone in relieving insomnia associated with depressive disorders in adolescents (aged 13-17 years). We reviewed the hospital charts of consecutively admitted adolescents with a depressive disorder and insomnia, who received one of three treatments: fluoxetine (20 +/- 2.2 mg), trazodone (71 +/- 32 mg), or a fluoxetine-trazodone combination (fluoxetine 29 +/- 2.2 mg, trazodone 68 +/- 29 mg). Each treatment was examined in 20 patients. Insomnia was defined as a change in sleep patterns characterized by decreased total sleep time that was sufficient to cause clinical concern, and insomnia resolution was defined as sleep starting by midnight and lasting 6 hours. Mean time to resolution of insomnia was significantly faster in adolescents treated with trazodone rather than fluoxetine (2.5 vs. 5.1 days, p < 0.05). Trazodone seemed to save only about 3 days and insomnia resolved in all subjects by the 11th day of antidepressant treatment. Median time to insomnia resolution was 2 days (range 1-5 days) in the trazodone group and 4 days (range 1-11 days) in the fluoxetine group. This difference between trazodone and fluoxetine, although statistically significant, was generally not clinically significant in the management of insomnia associated with depressive disorders in adolescents. The resolution of insomnia was not faster for treatment with a combination of fluoxetine and trazodone in comparison to fluoxetine monotherapy. Insomnia resolution was slightly later in older children. These clinical findings await confirmation by a controlled study. Both drugs seemed effective in ameliorating sleep symptoms in this sample, although it is likely that they produced these changes by different mechanisms.

The child with attention deficit hyperactivity disorder and learning disability.

The following case is presented to demonstrate the difficulties associated with treatment of Attention Deficit Hyperactivity Disorder (ADHD) when it is comorbid with a learning disability (LD). These two disorders exist simultaneously in about 30% of those diagnosed with ADHD. It is often difficult to separate the effects of one condition from those of the other. Researchers and clinicians often struggle with questions concerning the origin of inattentive symptoms. A child with LD may have trouble attending if placed in a classroom setting not designed to deal with issues related to LD, while a child with attention deficit may not be progressing as well as expected because of problems maintaining attention. This case highlights the need for a multi-faceted approach to the treatment of ADHD, as well as, the need for continued evaluation of the effectiveness of the treatment plan instituted.

The Objective Structured Clinical Examination (OSCE) in the clinical clerkship: an overview.

The Objective Structured Clinical Examination (OSCE) for student assessment is well established, with an extensive body of research documenting that this is a valid means to assess clinical skills that are fundamental to the practice of medicine. The OSCE consists of a circuit of stations which tests a range of skills and learning to assess undergraduate medical students. A well-constructed OSCE provides important information about candidate performance and the quality of training. It is used at the University of South Dakota School of Medicine (USDSM) in assessment of third year medical students during their Obstetrics Clerkship, and as a teaching tool in the Pediatric Clerkship. On August 10, 1996, the USDSM administered an OSCE for the first time to third year medical students. The purpose of this article is to present state of the art information about setting up OSCE based on our recent experience and to provide practical examples of OSCE questions which can be addressed in the clinical setting. The narrative, references and examples give guidelines for the preparation of OSCE testing. The OSCE provided a standardized way of assessing clinical competence. Both students and faculty were very satisfied with the examination, and felt that the material tested was relevant and appropriate. The OSCE process does serve to identify areas of weakness in the curriculum and/or teaching methods, and thus can serve as a mechanism to improve educational effectiveness.

Clonidine: a practical guide for usage in children.

Clonidine, oral and patch, has been used in adults for the treatment of hypertension and Tourette's Syndrome. Recently clinicians have begun to employ it in several oft label usages in children, especially behavioral syndromes. This article is a guide to its usage in children and includes discussion of its indications, contraindications, pre-treatment laboratory studies, dosing and drug interactions.

Comparison of Sprotte and Quincke needles with respect to post dural puncture headache and backache.

OBJECTIVE: The objective of this study was to compare 24-gauge Sprotte and 25-gauge Quincke needles with respect to post dural puncture headache and backache. METHODS: Three hundred ASA Physical Status I or II patients scheduled for minor orthopedic or urologic operations under spinal anesthesia were chosen for this randomized, prospective study at a university hospital and a city hospital. Anesthetic technique, intravenous fluids, and postoperative pain therapy were standardized. Patients were randomly divided into three equal groups. Spinal anesthesia was performed with either a 24-gauge Sprotte needle or a 25-gauge Quincke needle with the cutting bevel parallel or perpendicular to the dural fibers. RESULTS: Anesthesia could not be performed in three cases with the Sprotte needle and in one case with the Quincke needle. The most common complications were post dural puncture backache (18.0%), post dural puncture headache (8.2%), and non-postural headache (6.7%). No major complications occurred. The Quincke needle with bevel perpendicular to the dural fibers caused a 17.9% incidence of post dural puncture headache. The Quincke with bevel parallel to the dural fibers and the Sprotte needles caused similar post dural puncture headache rates (4.5% and 2.4%, respectively). Other factors associated with post dural puncture headache were young age, early ambulation, and sedation during spinal anesthesia. There were no significant differences between needles in the incidence of post dural puncture backache. CONCLUSION: Our data indicate that Quincke needles should not be used with the needle bevel inserted perpendicular to the dural fibers. The Sprotte needle does not solve the problem of post dural puncture headache and backache.

Heterogeneity of nemaline myopathy. A follow-up study of 13 cases.

The marked heterogeneity of nemaline myopathy is again shown in the present series of 13 patients. Most children have a long-standing, mild, and slowly progressing proximal myopathy. Two brothers with extreme weakness died during the neonatal period of respiratory failure representing the X-linked variant. One adult with proximal weakness was also diagnosed as having nemaline myopathy. An unusual course was observed in 2 infants who initially had moderate weakness but subsequently developed severe generalized weakness including respiratory muscles. This led to irreversible respiratory failure requiring continuous ventilatory support for as long as 9 and 15 years, respectively. Although uncommon, the possibility of an imminent respiratory failure in initially weak infants should also be taken into account within the clinical spectrum of nemaline myopathy.

Rehabilitation of communication impairment in dystonia musculorum deformans.

Augmentative and alternative communication (AAC) aids were used in three young, intellectually normal patients with dystonia musculorum deformans (DMD) who had severe speech and writing impediments. These aids included speech therapy, communication boards, and voice synthesizers for verbal communication and typewriters, memowriters, and computer software and printers for written communication. At times customized accessing was needed which required specific adaptive modifications. Implementation of the AAC aids system was determined effective for DMD patients in view of the intellect-sparing nature of the disorder. Improvement was hampered by the progressive nature of the disease and by the emotional stress of accepting the long-term use of AAC. Correct and early diagnosis of communication impediments are crucial for the appropriate AAC aids prescription and implementation. An AAC protocol is suggested to meet the special communication needs of DMD patients.

Factors explaining the referral of the elderly to home nursing or home help.

Factors explaining the referral of the elderly to home nursing or home help or both were studied in a population (n = 1358) aged 60 years or over and living in the commune of Ahtäri, a semi-industrialized community in middle-western Finland, using social and health care registers and questionnaires in collecting data. The clients of home nursing and of combined home nursing and home help were predominantly women, whereas men were in the majority among those referred to home help. A stepwise regression analysis showed old age, living alone, low self-perceived health, low education and few visiting contacts to explain home nursing or home help among men. Among women, the most important variables explaining home nursing or home help in the regression model were old age, low self-perceived health, low standard of housing, high amount of symptoms and lowered functional capacity. The poor availability of social support is more important a factor explaining the referral to home care among elderly men than among women. On the other hand, low health status and poor functional capacity, as well as poor availability of physical support are more important factors among women than men.

The disabled young adult: ready to leave home?

The disabled young adult strives to be independent when preparing to leave home and family. This article provides a checklist that can help the disabled young adult and his/her family to make this transition. In some cases, a transitional rehabilitation program may also be of help. The family physician who is familiar with the issues that pertain to adolescent development can play a central part in addressing patient and family problems that present at this time.

Vaccinations and the physically handicapped child.

The vaccination status of 98 physically handicapped children was examined to identify factors associated with an inadequate vaccination status. Of the 98 children, 57 had cerebral palsy, 14 had myelomeningocele, 3 had muscular dystrophy and 24 had myelomeningocele, 3 had muscular dystrophy and 24 had other motor disabilities. According to the available vaccination records, only 17 children had received all the recommended injections on schedule; 26 had missed at least one injection, and 3 of them had never been vaccinated. The overall rate of vaccination in our study group (63%) was lower than expected. The children with moderate to severe limitation of function due to cerebral palsy were significantly less likely (P less than 0.05) than those with less severe limitations to have received a basic series of vaccinations. Health departments must ensure that physically handicapped children receive the preventive health measures viewed normal and appropriate for other children.