Primary Care Provider Management of Congenital Hypothyroidism Identified Through Newborn Screening.

OBJECTIVE: To assess Primary Congenital Hypothyroidism (CH) management patterns and feasibility of providing long-term care for patients with CH identified through newborn screening by Primary Care Providers (PCPs) in California and Hawaii. STUDY DESIGN: A survey was mailed to all physicians (N=823) listed as the referral doctor for confirmed patients with CH identified through newborn screening programs in both states between 01/01/2009-12/31/2013. Information was collected on CH management patterns, barriers to providing care, and knowledge on CH treatment. Descriptive statistics and bivariate logistic regression results were reported. RESULTS: 206 PCPs completed the survey. Among these, 78% currently have patients with CH and 91% indicated willingness to provide long-term care to new patients with CH. Among PCPs currently caring for patients with CH, 17% managed CH by themselves with limited assistance from endocrinologists; 63% were involved in managing CH but endocrinologists played a larger role than PCPs; 19% were not involved in CH care. Only 49% of PCPs correctly answered questions regarding recommended follow-up frequencies and 23% knew the correct age for a trial off levothyroxine for suspected transient CH. Top two perceived barriers to providing long-term care included "need guidance or support from endocrinologists" (61%) and "not familiar with CH treatment guidelines" (28%). CONCLUSION: The majority of PCPs surveyed are willing to provide long-term care to patients with CH, but need support from endocrinologists and increased knowledge about current treatment guidelines.

Patterns of bone mineral acquisition in children with epidermolysis bullosa: a longitudinal study.

BACKGROUND: Reduced bone mass and fractures are known complications of generalized forms of epidermolysis bullosa (EB). However, the aetiology - inadequate bone acquisition, premature bone loss, or a combination - is unclear. OBJECTIVES: To determine patterns of bone mineral acquisition in children with EB and to identify clinical and laboratory correlates of change in areal bone mineral density (aBMD). METHODS: Seventeen subjects ≥ 6 years of age with generalized EB were studied at two visits at least 12 months apart with clinical and laboratory evaluations and dual energy X-ray absorptiometry scans of the lumbar spine. Wilcoxon signed-rank tests were used to determine if changes from baseline to follow-up were significant. Wilcoxon rank-sum tests were used to compare subjects with gains in aBMD Z-score with those who experienced no change or decreases to determine if baseline laboratory or clinical characteristics differed between the two groups. RESULTS: Subjects gained height and weight at follow-up, but there was no significant improvement in mean Z-scores for height, weight or body mass index. Laboratory values did not change significantly. Mean bone mineral content and aBMD of the lumbar spine increased significantly at follow-up, but mean aBMD Z-scores remained static. No differences in clinical characteristics or laboratory values were seen between subjects with increased aBMD Z-scores vs. those whose scores decreased or did not change. CONCLUSIONS: Low bone mass in children with generalized EB is due primarily to inadequate gains in aBMD. Interventions to improve overall health and to help build bone mass in this patient population are warranted.

Inadequate vitamin D status in adolescents with substantial bone mineral density loss during the use of depot medroxyprogesterone acetate injectable contraceptive: a pilot study.

STUDY OBJECTIVE: To examine vitamin D and parathormone (PTH) levels in adolescents who experienced substantial bone mineral density (BMD) loss during depot medroxyprogesterone acetate (DMPA) use. DESIGN: A non-randomized, multi-center study, during which DMPA was administered every 12 weeks and evaluation of lumbar spine and hip BMD by dual-energy X-ray absorptiometry (DXA) was conducted every 6 months. A blood sample for vitamin D and PTH measurements was obtained from adolescents who experienced >5% BMD loss. Vitamin D deficiency was defined as 25-hydroxyvitamin D (25OHD) level of <20 ng/mL, insufficiency as 25OHD level of 20-30 ng/mL, and sufficiency as 25OHD level of >30 ng/mL. RESULTS: Evaluation of vitamin D and PTH was carried out in 15 participants who experienced BMD loss of > or = 5% during DMPA use. At initiation of DMPA, participants had mean (+SE) age 17+1 years, gynecologic age 61+4 months, and body mass index 24+1.5 kg/m2. Racial/ethnic distribution was: Caucasian--7 girls, Hispanic--4 girls, African-American--3 girls, and other--1 girl. Six participants had BMD loss of >5% after 2 DMPA injections, five after 3 injections, one after 5 injections, one after 8 injections, one after 10 injections, and one after 13 injections. Only one girl (7%) had sufficient vitamin D. The other participants had vitamin D insufficiency (50%) or deficiency (43%). Participants' mean (+SE) PTH was 22+4 pg/mL (reference range 7-53 pg/mL), and mean (+SE) 1,25-dihydroxyvitamin D was 56+5 pg/mL (reference range 22-67 pg/mL). CONCLUSIONS: Inadequate vitamin D status was evident among the majority of female adolescents who experienced a substantial BMD loss while using DMPA.

The relative contributions of lean tissue mass and fat mass to bone density in young women.

Although obesity is associated with increased risk of many chronic diseases including cardiovascular disease, diabetes, hypertension, and cancer, there is little evidence to suggest that obesity increases risk of osteoporosis. In fact, both weight and body mass index (BMI) are positive predictors of bone mass in adults, suggesting that those who are overweight or obese may be at lower risk of osteoporosis. However, recent evidence suggests that in children and adolescents, obesity may be associated with lower rather than higher bone mass. To understand the relation of fat mass to bone mass, we examined data gathered from an ethnically diverse group of 921 young women, aged 20-25 years (317 African Americans, 154 Asians, 322 Caucasians, and 128 Latinas) to determine how fat mass (FM) as well as lean tissue mass (LTM) is associated with bone mass. Bone mass, FM, and LTM were measured using dual energy X-ray absorptiometry (GE Lunar Corp, Madison, WI). Bone mass was expressed as bone mineral density (BMD; g/cm2) and bone mineral apparent density (BMAD; g/cm3) for the spine and femoral neck, and as BMD and bone mineral content (BMC; g) for the whole body. Regression techniques were used to examine the following: (1) in separate equations, the associations of LTM and FM with each bone mass parameter; and (2) in the same equation, the independent contributions of LTM and FM to bone mass. LTM and FM were positively correlated with BMD at all skeletal sites. When the contributions of FM and LTM were examined simultaneously, both FM and LTM continued to be positively associated with bone mass parameters but the effect of FM was noted to be smaller than that of LTM. We conclude that in young women, LTM has a greater effect than fat mass on bone density per kg of tissue mass.

The state of the state mental hospital at the turn of the century.

There is a core of services whose specific content varies from place to place that still appears to belong to the state mental hospital.

Calcaneus ultrasound measurements in a convenience sample of healthy youth.

We examined age-related changes in quantitative ultrasound of the calcaneus in 311 healthy males and females ages 6.6-20 yr using the Lunar Achilles ultrasound device. This equipment has been adapted for pediatric use with the provision of shims designed to properly position smaller feet relative to the transducers. Broadband ultrasound attenuation (BUA) (decibels/megahertz), speed of sound (SOS) (meters/second), and stiffness index (SI) (percent) increased across the age range until a plateau was reached at 16-18 yr. BUA increased by 40%, SOS by 4%, and SI by 80% across this age range. There was no gender difference in age-related gains. Age, weight, height, and hours of weight-bearing physical activity were all significantly associated with BUA, SOS, and SI. After controlling for age and weight, hours of weight-bearing physical activity showed little to no additional effect on these parameters. Short-term in vivo precision using this device was similar in children to that observed in adults in our laboratory; coefficients of variation for between-day measurements were 1.8, 0.6, and 3.2% for BUA, SOS, and SI, respectively. These data support the feasibility of using the Lunar Achilles in evaluating pediatric bone mass. The ability of this technique to discriminate between osteopenic and normal children remains to be determined.

Some perspectives on deinstitutionalization.

The authors discuss what can be learned from our experience with deinstitutionalization. The deinstitutionalization of mentally ill persons has three components: the release of these individuals from hospitals into the community, their diversion from hospital admission, and the development of alternative community services. The greatest problems have been in creating adequate and accessible community resources. Where community services have been available and comprehensive, most persons with severe mental illness have significantly benefited. On the other hand, there have been unintended consequences of deinstitutionalization-a new generation of uninstitutionalized persons who have severe mental illness, who are homeless, or who have been criminalized and who present significant challenges to service systems. Among the lessons learned from deinstitutionalization are that successful deinstitutionalization involves more than simply changing the locus of care; that service planning must be tailored to the needs of each individual; that hospital care must be available for those who need it; that services must be culturally relevant; that severely mentally ill persons must be involved in their service planning; that service systems must not be restricted by preconceived ideology; and that continuity of care must be achieved.

Acquisition of optimal bone mass in childhood and adolescence.

Peak bone mass (PBM), which is achieved by early adulthood, is a key determinant of the lifetime risk of osteoporosis. Because the foundation for skeletal health is established so early in life, osteoporosis prevention begins by optimizing gains in bone mineral throughout childhood and adolescence. Heritable factors account for an estimated 60-80% of the variability in PBM, with diet, physical activity and hormonal status serving as important modifiers of bone accrual. Recent pediatric studies have clarified the tempo and magnitude of gains in bone mineral and the modulating effects of diet, activity and sex steroids. The challenge lies in designing effective means to reverse trends of decreased calcium consumption, increased sodium intake and diminished physical activity among children and adolescents. Equally important is raising the awareness of health care providers to recognize children at risk for suboptimal acquisition of PBM.

Assessment of bone mineralization following renal transplantation in children: limitations of DXA and the confounding effects of delayed growth and development.

Pediatric renal transplantation recipients have numerous risk factors for decreased bone mass, including the underlying renal disease, nutritional deficits, decreased physical activity, inflammation and exposure to steroid therapy. The assessment of bone mineralization in children following renal transplantation is fraught with difficulty. Dual energy x-ray absorptiometry (DXA) is the most commonly employed tool to assess bone mineralization. However, DXA has important limitations in children and in individuals with renal disease. This brief review will examine the expected gains in bone size and bone mass during growth and the mechanisms by which renal failure and steroid therapy interrupt these process. In addition, the limitations of DXA for detecting impaired bone mineralization in children with renal disease are reviewed and alternative approaches explored.

Dual energy X-ray absorptiometry (DEXA) measurements of bone density and body composition: promise and pitfalls.

Dual energy X-ray absorptiometry (DEXA) is widely viewed as the preferred method to assess pediatric bone mineral content because of its speed, precision, and minimal radiation exposure, and the availability of pediatric reference data. DEXA can also be used to estimate body composition precisely with minimal patient cooperation. Accurate interpretation of DEXA data in children requires consideration of bone size, pubertal stage, skeletal maturation, ethnicity and body composition. Bone mineral content may be underestimated in smaller children and overestimated in larger ones. Corrections for skeletal age or sexual maturity may also be needed in children with advanced or delayed growth. Errors in body composition measurement occur because body fat and fat-free mass are not distributed uniformly. In addition, fat mass present adjacent to bone will influence the measurement of bone mineral content. In conclusion, DEXA is a valuable tool for assessing pediatric bone health, but accurate interpretation of densitometry results requires recognition of a myriad of pitfalls.

Mechanobiology of femoral neck structure during adolescence.

Understanding femoral neck structure may be critical to preventing fractures at this site. We examined the correlates of changes in the femoral neck during adolescence. Dual energy x-ray absorptiometry measurements of proximal femora were made in 101 Caucasian youths (ages 9 to 26 years). Relationships were examined between developmental parameters (age, pubertal stage, height, body mass, lean mass, and fat mass) and femoral structure (bone mineral content, bone mineral density, neck width, cross-sectional area, and cross-sectional strength). Lean body mass was the best predictor of femoral neck structure, explaining 53-87 percent of the variance, and was independent of gender. Body mass only explained 51-79 percent of the variance. Previously we found body mass to be the strongest predictor of femoral mid-diaphyseal cross-sectional properties. These findings suggest that trabecular bone of the femoral neck may be more responsive to its mechanical environment than the cortical diaphysis. In addition, lean body mass may be a more reliable predictor of muscle loading than body mass.

Use of hormone replacement therapy to reduce the risk of osteopenia in adolescent girls with anorexia nervosa.

PURPOSE: To assess how commonly hormone replacement therapy (HRT) and other measures are prescribed for the treatment of osteopenia in children and adolescents with anorexia nervosa (AN). METHODS: A self-administered questionnaire was distributed and completed by allopathic and osteopathic physician members of the Society for Adolescent Medicine at its 1998 annual meeting. The questionnaire was also mailed and E-mailed between March 1998 and February 1999. Descriptive statistics included percentages and measures of central tendency. RESULTS: The questionnaire was completed by 394 of the 1029 physicians surveyed (38.3%). Of the 268 respondents who treated patients with AN under the age of 18 years, 77.6% prescribed HRT. The decision to prescribe HRT was influenced by patient's age but not by bone mineral status. Among those who prescribed HRT, additional therapies included increased caloric intake (89.4%), weight gain (82.2%), increased calcium intake (84.1%), a change in exercise patterns (59.1%), and vitamin D supplementation (37.0%). Only 59 (22.0%) did not use HRT as a treatment modality. One-third of nonprescribers cited the lack of evidence of efficacy of HRT in preventing osteopenia. More recent medical graduates were less likely to prescribe HRT. CONCLUSIONS: This survey suggests that practitioners caring for adolescent females with AN commonly prescribe HRT for the treatment of osteopenia despite the paucity of evidence demonstrating that it effectively prevents or reverses bone loss associated with this disorder.

Psychosocial rehabilitation and psychiatry in the treatment of schizophrenia--what are the boundaries?

OBJECTIVE: To examine the relationship between psychosocial rehabilitation and psychiatry with particular emphasis upon treatment of people who suffer from schizophrenia. METHOD: Current literature is examined in an effort to identify prevailing premises and myths held by each discipline as it relates to the other. RESULTS: A working definition of psychosocial rehabilitation is proposed, and nine interrelated concepts that constitute the belief system of that discipline are identified. CONCLUSION: Rehabilitation's biopsychosocial perspective provides a strong basis for joint enterprise with psychiatry in the care of people with schizophrenia. However, members of both disciplines must pursue active measures in order to effect co-operative treatment interventions.

Bone mineral acquisition in healthy Asian, Hispanic, black, and Caucasian youth: a longitudinal study.

Ethnic and gender differences in bone mineral acquisition were examined in a longitudinal study of 423 healthy Asian, black, Hispanic, and white males and females (aged 9-25 yr). Bone mass of the spine, femoral neck, total hip, and whole body was measured annually for up to 4 yr by dual energy x-ray absorptiometry. Age-adjusted mean bone mineral curves for areal (BMD) and volumetric (BMAD) bone mineral density were compared for the 4 ethnic groups. Consistent differences in areal and volumetric bone density were observed only between black and nonblack subjects. Among females, blacks had greater mean levels of BMD and BMAD at all skeletal sites. Differences among Asians, Hispanics, and white females were significant for femoral neck BMD, whole body BMD, and whole body bone mineral content/height ratio, for which Asians had significantly lower values; femoral neck BMAD in Asian and white females was lower than that in Hispanics. Like the females, black males had consistently greater mean values than nonblacks for all BMD and BMAD measurements. A few differences were also observed among nonblack male subjects. Whites had greater mean total hip BMD, whole body BMD, and whole body bone mineral content/height ratio than Asian and Hispanic males; Hispanics had lower spine BMD than white and Asian males. The tempo of gains in BMD varied by gender and skeletal site. In females, total hip, spine, and whole body BMD reached a plateau at 14.1, 15.7, and 16.4 yr, respectively. For males, gains in BMD leveled off at 15.7 yr for total hip and at age 17.6 yr for spine and whole body. Black and Asian females and Asian males tended to reach a plateau in BMD earlier than the other ethnic groups. The use of gender- and ethnic-specific standards is recommended when interpreting pediatric bone densitometry data.

The state of the state mental hospital at the turn of the century.

There is a core of services whose specific content varies from place to place that still appears to belong to the state mental hospital.