Preliminary report: Surgical outcomes following genitoplasty in children with moderate to severe genital atypia.

INTRODUCTION: Prior studies of outcomes following genitoplasty have reported high rates of surgical complications among children with atypical genitalia. Few studies have prospectively assessed outcomes after contemporary surgical approaches. OBJECTIVE: The current study reported the occurrence of early postoperative complications and of cosmetic outcomes (as rated by surgeons and parents) at 12 months following contemporary genitoplasty procedures in children born with atypical genitalia. STUDY DESIGN: This 11-site, prospective study included children aged ≤2 years, with Prader 3-5 or Quigley 3-6 external genitalia, with no prior genitoplasty and non-urogenital malformations at the time of enrollment. Genital appearance was rated on a 4-point Likert scale. Paired t-tests evaluated differences in cosmesis ratings. RESULTS: Out of 27 children, 10 were 46,XY patients with the following diagnoses: gonadal dysgenesis, PAIS or testosterone biosynthetic defect, severe hypospadias and microphallus, who were reared male. Sixteen 46,XX congenital adrenal hyperplasia patients were reared female and one child with sex chromosome mosaicism was reared male. Eleven children had masculinizing genitoplasty for penoscrotal or perineal hypospadias (one-stage, three; two-stage, eight). Among one-stage surgeries, one child had meatal stenosis (minor) and one developed both urinary retention (minor) and urethrocutaneous fistula (major) (Summary Figure). Among two-stage surgeries, three children developed a major complication: penoscrotal fistula, glans dehiscence or urethral dehiscence. Among 16 children who had feminizing genitoplasty, vaginoplasty was performed in all, clitoroplasty in nine, external genitoplasty in 13, urethroplasty in four, perineoplasty in five, and total urogenital sinus mobilization in two. Two children had minor complications: one had a UTI, and one had both a mucosal skin tag and vaginal mucosal polyp. Two additional children developed a major complication: vaginal stenosis. Cosmesis scores revealed sustained improvements from 6 months post-genitoplasty, as previously reported, with all scores reported as good or satisfied. DISCUSSION: In these preliminary data from a multi-site, observational study, parents and surgeons were equally satisfied with the cosmetic outcomes 12 months after genitoplasty. A small number of patients had major complications in both feminizing and masculinizing surgeries; two-stage hypospadias repair had the most major complications. Long-term follow-up of patients at post-puberty will provide a better assessment of outcomes in this population. CONCLUSION: In this cohort of children with moderate to severe atypical genitalia, preliminary data on both surgical and cosmetic outcomes were presented. Findings from this study, and from following these children in long-term studies, will help guide practitioners in their discussions with families about surgical management.

Prospective assessment of cosmesis before and after genital surgery.

INTRODUCTION: Little data exist about the surgical interventions taking place for children with disorders of sex development (DSD). Most studies that have evaluated cosmetic outcomes after genitoplasty have included retrospective ratings by a physician at a single center. OBJECTIVE: The present study aimed to: 1) describe frequency of sex assignment, and types of surgery performed in a cohort of patients with moderate-to-severe genital ambiguity; and 2) prospectively determine cosmesis ratings by parents and surgeons before and after genital surgery. STUDY DESIGN: This prospective, observational study included children aged <2 years of age, with no prior genitoplasty at the time of enrollment, moderate-to-severe genital atypia, and being treated at one of 11 children's hospitals in the United States of America (USA). Clinical information was collected, including type of surgery performed. Parents and the local pediatric urologist rated the cosmetic appearance of the child's genitalia prior to and 6 months after genitoplasty. RESULTS: Of the 37 children meeting eligibility criteria, 20 (54%) had a 46,XX karyotype, 15 (40%) had a 46,XY karyotype, and two (5%) had sex chromosome mosaicism. The most common diagnosis overall was congenital adrenal hyperplasia (54%). Thirty-five children had surgery; 21 received feminizing genitoplasty, and 14 had masculinizing genitoplasty. Two families decided against surgery. At baseline, 22 mothers (63%), 14 fathers (48%), and 35 surgeons (100%) stated that they were dissatisfied or very dissatisfied with the appearance of the child's genitalia. Surgeons rated the appearance of the genitalia significantly worse than mothers (P < 0.001) and fathers (P ≤ 0.001) at baseline. At the 6-month postoperative visit, cosmesis ratings improved significantly for all groups (P < 0.001 for all groups). Thirty-two mothers (94%), 26 fathers (92%), and 31 surgeons (88%) reported either a good outcome, or they were satisfied (see Summary Figure); there were no significant between-group differences in ratings. DISCUSSION: This multicenter, observational study showed surgical interventions being performed at DSD centers in the USA. While parent and surgeon ratings were discordant pre-operatively, they were generally concordant postoperatively. Satisfaction with postoperative cosmesis does not necessarily equate with satisfaction with the functional outcome later in life. CONCLUSION: In this cohort of children with genital atypia, the majority had surgery. Parents and surgeons all rated the appearance of the genitalia unfavorably before surgery, with surgeons giving worse ratings than parents. Cosmesis ratings improved significantly after surgery, with no between-group differences.

Health-related quality of life in college students with and without childhood-onset asthma.

OBJECTIVE: The current study investigated whether differences existed in health-related quality of life between individuals who self-identified as having childhood-onset asthma and individuals without a chronic illness. Additionally, the relationship between perceived illness intrusiveness and illness uncertainty to health-related quality of life was explored. METHODS: College undergraduates at least 18 years of age who self-identified as having childhood asthma were randomly matched by age and gender to healthy control participants. Participants completed a demographic form, the Mishel Uncertainty in Illness Scale-Community Form, the Illness Intrusiveness Scale, and the SF-36 Health Survey, a measure of health-related quality of life. RESULTS: Participants with asthma had significantly lower scores on the total and mental health-related quality of life scales than did healthy control subjects. There were no significant differences between self-identified participants with asthma and matched healthy control subjects on physical health-related quality of life scales. Illness intrusiveness was not related to either the physical (e.g., physical functioning, general health) or mental health-related quality of life. Higher levels of illness uncertainty were significantly related to higher levels of mental health-related quality of life (e.g., vitality, mental health). In addition, participants with asthma scored significantly lower than healthy controls on the social functioning and role-emotional subscales. CONCLUSION: The current study adds to the extant literature by examining the relationships between illness intrusiveness, illness uncertainty, and health-related quality of life among a young adult population. College students with asthma appear to be at risk for diminished quality of life compared to a healthy comparison group. Further examination of various domains of health-related quality of life among older adolescents and young adults with childhood asthma is needed.

An experimental examination of learned helplessness in older adolescents and young adults with long-standing asthma.

OBJECTIVE: To examine the effects of experimentally induced learned helplessness in older adolescents and young adults with long-standing asthma. METHODS: Thirty-nine participants (18-24 years of age) with histories of long-standing asthma (AS) and an age-matched healthy cohort (HC) (N = 94) received either contingent or noncontingent feedback on an experimental task. Participants' anagram-solving performance was assessed following the experimental procedure. Participants also completed a measure of depression and pretest-posttest measures of mood, expectancy, and attributions related to experimental task performance. RESULTS: The AS participants demonstrated significantly greater problem-solving deficits following response-noncontingent feedback, compared to the HC group. Further, whereas both AS and HC participants made more internal performance attributions when given response-contingent feedback, only AS participants demonstrated a pattern of increased internal attributions (i.e., self-focus) following response-noncontingent failure. In addition, 21% of AS participants met DSM-IV criteria for major depression, compared to only 5% of the HC group. CONCLUSIONS: Individuals with long-standing asthma may be at increased risk for depression and for learned helplessness deficits, specifically impaired problem solving, in response to environmental noncontingency. Results are discussed in terms of both learned helplessness theory and perseverative self-focus conceptualizations of depression. The implications for both short- and long-term management of pediatric asthma are also discussed.

Transactional patterns of child, mother, and father adjustment in insulin-dependent diabetes mellitus: a prospective study.

Utilized both interview and self-report methods to examine transactional patterns of child, mother, and father adjustment in a sample of children and adolescents with insulin-dependent diabetes mellitus (IDDM). Overall, levels of child and parental adjustment were relatively stable over the 1-year study period. Regression analyses revealed that increases in fathers', but not mothers', distress over time contributed significant incremental variance to poorer subsequent children's adjustment, after controlling for demographic (age, gender, and SES) and disease parameters (illness duration and metabolic control). Decline in fathers' adjustment was a significant predictor of better mothers' adjustment at follow-up; child adjustment was not significantly associated with mothers' adjustment. Variations in both children's and mothers' adjustment made significant, independent contributions to predicting subsequent fathers' adjustment. Findings illustrate the transactional nature of relationships that exist in families of children with IDDM and underscore the importance of family systems or biobehavioral family treatment approaches in the clinical management of children with chronic illnesses.

Illness uncertainty, attributional style, and psychological adjustment in older adolescents and young adults with asthma.

Examined psychological adjustment in a college sample of older adolescents and young adults (n = 49) with histories of childhood asthma. A substantial number of subjects evidenced clinically significant levels of overall distress. In addition, greater perceived asthma uncertainty and increased stable attributions for negative events were significantly associated with poorer psychological adjustment after controlling for demographic and disease variables. Further analyses revealed a moderating influence of uncertainty on attribution-adjustment relationships. These findings provide initial support for a cognitive diathesis-stress view of adjustment in long-standing asthma. Results also support a growing body of evidence suggesting that the focus of efforts to enhance adjustment to asthma need to be expanded beyond childhood and early adolescence.

Results of a clinical trial of the holmium:YAG laser in disc decompression utilizing a side-firing fiber: a two-year follow-up.

BACKGROUND AND OBJECTIVE: Laser-assisted disc decompression (LADD) is an operative technique for the treatment of symptomatic, nonsequestered herniated nucleus pulposus that has failed to respond to conservative treatment. The current study reports 2-year follow-up data. STUDY DESIGN MATERIALS AND METHODS: Patients were evaluated by an independent interviewer postoperatively, and at 1 week, 3 months, 6 months, 1 year, and 2 years. Ratings were based upon the modified Macnab criteria. All patients evidenced primarily leg pain with or without back pain that had failed a minimum of 6 weeks of conservative treatment. Patients with lateral recess or central stenosis, sequestered discs, or predominantly scar tissue from a previous discectomy were not considered candidates for LADD. RESULTS: Utilizing postoperative follow-up at 2 years, a surgical success rate of 86.9% was achieved. For patients requiring an additional LADD procedure, results at 6-month follow-up yielded a surgical success rate of 80%. CONCLUSION: LADD appears to be a viable treatment modality for symptomatic, nonsequestered lumbar disc herniation recalcitrant to conservative treatment. LADD may represent a more cost-effective and safer alternative to traditional surgical procedures.

Laser assisted disc decompression: an alternative treatment modality in the Medicare population.

The current study evaluated the efficacy of LADD within the Medicare population, and examined the influence of coexisting medical conditions and previous surgical intervention on surgical outcome. Patients who met inclusion criteria underwent laser-assisted disc decompression (LADD) using the Holmium: YAG Laser with Sidefire laser fiber. All patients evidenced primarily leg pain, with or without back pain, which had failed a minimum of six weeks conservative treatment. Post-operative follow-up at one year yielded a surgical success rate of 80%. Surgical outcome was not related to coexisting medical conditions or previous surgical intervention on the lumbar spine. Findings of the current study suggest that LADD offers a viable alternative treatment modality for lumbar disc disease within the Medicare population.

Percutaneous laser disc decompression with the holmium: YAG laser.

This article discusses the evolution of the percutaneous laser disc decompression (PLDD) method using a holmium:YAG laser. Advantages of using this wavelength and several techniques for its use in PLDD are reviewed. The article also discusses the current devices and delivery systems available for this application.

Child and maternal adaptation to cystic fibrosis and insulin-dependent diabetes mellitus: differential patterns across disease states.

Investigated the relationship between maternal and child emotional adaptation both across and within samples of children with cystic fibrosis (CF) and insulin-dependent diabetes mellitus (IDDM). Higher levels of maternal depression were associated with increased depression in children with IDDM. In addition, increased illness severity and greater length of time since diagnosis were related to increased depression in children with IDDM. Whereas maternal depression was related to decreased trait anxiety for children in the CF group, neither maternal anxiety or depression were associated with child depression or state anxiety. Empirical and clinical implications of a disease-specific approach to studying chronic disease in children are discussed.

Laser-assisted disc decompression: a clinical trial of the holmium:YAG laser with side-firing fiber.

In order to evaluate the efficacy of laser-assisted disc decompression (LADD) as an alternative treatment modality for nonsequestered herniated nucleus pulposus of the lumbar spine, we used the Holmium:YAG laser with Sidefire laser fiber on 223 consecutive patients who met inclusion criteria. Patients were evaluated by an independent interviewer postoperatively, and at 1 week, 3 months, 6 months, and 1 year. Ratings were based upon the modified Macnab criteria. All patients presented with leg pain with or without back pain that had failed a minimum of 6 weeks conservative treatment (e.g., rest, NSAIDs, physical therapy, epidural steroid injections). Patients were not considered for LADD if the primary source of neurologic findings was a result of scar tissue from a previous discectomy, lateral recess or central stenosis, or sequestered discs. At 1 year postoperative follow-up, the surgical success rate was 84%. For patients requiring an additional LADD procedure, results at 6 month follow-up yielded surgical success rates of 92.3% and 90% for additional level and index level LADD, respectively. LADD appears to be a viable treatment modality for nonsequestered herniated nucleus pulposus recalcitrant to conservative treatment. LADD may represent a more cost-effective and safer treatment alternative to traditional invasive surgical procedures and other percutaneous methods.

Survey of pediatricians' management practices for recurrent abdominal pain.

Surveyed general pediatricians (N = 116) regarding six content areas: (a) diagnostic procedures utilized during the initial evaluation sequence of a child with recurrent abdominal pain (RAP); (b) factors that influence decisions to order additional evaluations; (c) management practices following negative evaluation results; (d) factors influencing decisions about consultation with or referral to a mental health professional; (e) attitudes about the etiology of RAP; and (f) the frequency in which pediatricians encountered children with RAP and referred them to mental health services. Results of this survey are presented and discussed from a biopsychosocial perspective. Limitations of the survey, implications of the results for integrating mental health professionals into the evaluation and treatment process of RAP patients, and directions for future research are discussed.

Changing medical students' attitudes about AIDS.

Medical students may have negative attitudes about persons with the acquired immunodeficiency syndrome (AIDS) as well as concerns about occupational infection with human immunodeficiency virus. We conducted a brief intervention to assess and modify attitudes of first-year students. The intervention was presented to small groups of students by peers, faculty, and a person with AIDS. Assessments of attitudes and knowledge were conducted one week prior to the intervention (pretest), and one (posttest 1) and 47 weeks (posttest 2) afterwards. Females had more positive attitudes about AIDS on pretest and posttest 1. Attitude scores improved significantly from pretest to posttest 1 but returned close to baseline by posttest 2. Knowledge scores were high on pretest and did not change significantly. Attitude scores were significantly correlated with knowledge, and with students' opinions regarding obligation to care for AIDS patients. Acquaintance with a homosexual was highly correlated with both scores. This intervention may serve as a model in improving students' attitudes about AIDS.

Risk and resistance factors in the adaptation of mothers of children with cystic fibrosis.

Examined 35 mothers of children with cystic fibrosis (CF) to assess the relationship of risk and resistance factors to level of psychological adjustment. Dimensions of maternal adaptation, disease severity, family adaptability and cohesion, family life stress, and intrapersonal coping style were assessed. As a group, mothers evidenced significant levels of general psychological distress, and appear to constitute an at-risk population. Multiple regression analyses indicated higher levels of maternal distress were associated with increased levels of stressful family life events and an escape-avoidance coping style. Disease severity, family financial resources, and family adaptability and cohesion did not significantly contribute to the regression model.

Developing a program to assist Turner's syndrome patients and families.

Turner's Syndrome [TS] is a chromosomal disorder that affects one in 2500 women. It results in an array of physical difficulties, including short stature, lack of secondary sexual development and cognitive problems. Little research exists to document the psychosocial problems and needs of individuals with TS and their families. The current literature and the results of a regionally based needs assessment are reviewed to guide program development, with emphasis on the emotional and informational needs of these families. Suggestions are provided for strategic early communication and information sharing, development of skill-specific support groups, family networks and family therapy.

Hate revisited: power, envy, and greed in the rehabilitation setting.

Hate has been recognized as an important psychological element in the rehabilitation setting. Hate emanates from a variety of sources, including the patient, his family, and staff; it can also stem from various systems influences and is perpetuated by power, envy, and greed. This article examines several systems factors that contribute to hate and conflict in the rehabilitation setting. It is argued that the growth and rapid expansion of rehabilitative services, the incorporation of rehabilitation enterprises along with new career opportunities, and quests for power potentially contribute to the enhancement of hate. Ultimately, this hate can interfere with the quality of care and outcomes in rehabilitation if it is not recognized and addressed.

Childhood depression: peer reactions to depression and life stress.

The present study investigated children's responses to a peer's childhood depression. Younger children in third and fourth grade and older children in fifth and sixth grade were exposed to one of four films. The four films portrayed a female peer who was either depressed or not depressed and who had experienced numerous recent life stresses or no recent life stress. Overall, children rated the depressed peers as less likeable and attractive, as engaging in fewer positive current and future behaviors, and as needing therapy more than a nondepressed peer. There was a tendency to rate the depressed peer with high life stress more positively than the depressed peer with low life stress; this tendency decreased with age. Girls rated all of the peers and especially the stressed peers more positively than did the boys. The results are discussed in terms of the implications of children's social interaction for the initiation or maintenance of childhood depression.

Cognitive problem-solving and life event correlates of depressive symptoms in children.

This study examined the relationship between a number of cognitive problem-solving and life event variables and depressive symptoms in a sample of non-referred grade school children. The results indicated that higher levels of depressive symptoms were associated with an external locus of control, increased levels of objective and subjective life stress, and lower performance levels on an impersonal problem-solving task. Level of depressive symptoms was also found to be inversely related to socioeconomic status as measured by father's occupation. Contrary to prediction, no consistent relationship was found between depression and interpersonal problem-solving ability.