Are adult patients with Laron syndrome osteopenic? A comparison between dual-energy X-ray absorptiometry and volumetric bone densities

Severe short stature resulting from a deficiency in IGF-I is a prominent feature of Laron syndrome (LS). Although low bone mineral density (BMD) has been noted in LS patients examined by dual energy x-ray absorptiometry (DEXA), this technique does not take volume into account and may therefore underestimate the true bone density in patients with small bones. The aim of the present study was to evaluate the BMD yielded by DEXA in our LS patients using estimated volumetric values. Volumetric density was calculated with the following formulas: bone mineral apparent density (BMAD) = bone mineral content (BMC)/(area)(3/2) for the lumbar spine and BMAD = BMC/area(2) for the femoral neck. The study sample included 12 patients (mean age, 43.9 yr; mean height, 123.7 cm). Findings were compared with 10 osteopenic subjects without developmental abnormalities (mean age, 56 yr; mean height, 164.8 cm) and 10 healthy control subjects matched for sex and age to the LS patients (mean height, 165.5 cm). BMAD in the LS group was 0.201 +/- 0.02 g/cm(3) at the lumbar spine and 0.201 +/- 0.04 g/cm(3) at the femoral neck; corresponding values for the osteopenic group were 0.130 +/- 0.01 and 0.140 +/- 0.01 g/cm(3), and for the controls, 0.178 +/- 0.03 and 0.192 +/- 0.02 g/cm(3). Although areal BMD was significantly lower in the LS and osteopenic subjects compared with controls (P < 0.02) at both the lumbar spine and femoral neck, BMAD was low (P < 0.01) in the osteopenic group only. In conclusion, DEXA does not seem to be a reliable measure of osteoporosis in patients with LS.     

Intussusception in children: can we rely on screening sonography performed by junior residents?

Background Ultrasonography is an important tool in the screening and diagnosis of patients with suspected intussusception.Objective To retrospectively evaluate the accuracy and performance of junior residents and compare it to that of senior residents and staff radiologists.Materials and methods Between January 1999 and February 2003, 151 patients with suspected intussusception underwent screening US. The mean age of the patients was 13.8 months. Patients were divided into three groups according to examiner: staff radiologist, senior resident or junior resident.Results Sixty-five patients had both US and air enema. Forty-four patients had a positive US result; 37 (84%) were true positive and 7 (16%) were false positive. Twenty-one patients had a negative US result; 18 (86%) were true negative and 3 (14%) were false negative. Eighty-six patients underwent screening US only and were then kept under observation in the emergency room. They were all diagnosed as having a non-surgical condition. The total accuracy rate was 93%, sensitivity was 84%, specificity was 97%, positive predictive value was 93% and negative predictive value was 94%. Accuracy rate, sensitivity and specificity were 92%, 85% and 98% for staff radiologists, 94%, 75% and 96% for senior residents and 95%, 83% and 97% for junior residents, respectively.Conclusions Junior residents perform as well as staff radiologists in screening US for suspected intussusception and have gained both the respect and confidence of the paediatricians.     

Assessment of cartilage repair after chondrocyte transplantation with a fibrin-hyaluronan matrix--correlation of morphological MRI, biochemical T2 mapping and clinical outcome

Objective

To evaluate change over time of clinical scores, morphological MRI of cartilage appearance and quantitative T2 values after implantation with BioCart™II, a second generation matrix-assisted implantation system.

Methods

Thirty-one patients were recruited 6–49 months post surgery for cartilage defect in the femoral condyle. Subjects underwent MRI (morphological and T2-mapping sequences) and completed the International Knee Documentation Committee (IKDC) questionnaire. MRI scans were scored using the MR Observation of Cartilage Repair Tissue (MOCART) system and cartilage T2-mapping values were registered.Analysis included correlation of IKDC scores, MOCART and T2 evaluation with each other, with implant age and with previous surgical intervention history.

Results

IKDC score significantly correlated with MOCART score (r = −0.39, p = 0.031), inversely correlated with previous interventions (r = −0.39, p = 0.034) and was significantly higher in patients with longer follow-up time (p = 0.0028).MOCART score was slight, but not significantly higher in patients with longer term implants (p = 0.199).T2 values were significantly lower in patients with longer duration implants (p < 0.001). This trend was repeated in patients with previous interventions, although to a lesser extent.

Conclusions

Significant improvement with time from BioCart™II implantation can be expected by IKDC scoring and MRI T2-mapping values. Patients with previous knee operations can also benefit from this procedure.     

Alendronate for osteoporosis in men with androgen-repleted hypogonadism

Male hypogonadism is associated with low bone mineral density (BMD) and an increased risk of fractures. Testosterone replacement therapy improves BMD in young hypogonadal men. This effect is milder in older patients, who are at greater risk for fractures. We studied the effects of alendronate or placebo on BMD in 22 osteoporotic men, 29–69 years of age (mean, 50.2±11.2 years) with long-standing hypogonadism, receiving standard testosterone replacement treatment. Alendronate 10 mg daily ( n =11) increased lumbar-spine BMD by 6.0 and 8.4% at 6 and 12 months, respectively, compared with –0.5% at 6 months and +3.3% at 12 months in the placebo group ( n =11; P <0.005). Alendronate also increased mean femoral-neck BMD by 1.9% after 1 year, compared to a 1.4% decrease with placebo ( P <0.005), and increased the total body bone mineral content by 4.4%, compared to a 0.6% decrease with placebo ( P =0.07). After 6 months alendronate suppressed urinary deoxypyridinoline by 50% ( P <0.005), compared to a 24% decrease in the placebo group. Both the alendronate and placebo groups continued with alendronate 70 mg once weekly for the following 2 years. Lumbar-spine BMD during this open-label study phase did not change significantly in the group originally treated with alendronate, but continued to increase in the placebo-alendronate group by 5.4, 6.5, and 6.2% after 18 (6 months of alendronate), 24 and 36 months, respectively ( P <0.05). Femoral-neck BMD continued to increase in both groups receiving active therapy; in the alendronate-alendronate group by 3.7, 2.7, and 5.2% after 18, 24, and 36 months, respectively ( P =0.01), and in the placebo-alendronate group by 0.7 and 1.9% at 24 (first 12 months of alendronate) and 36 months, respectively ( P <0.05). Our results support the long-term administration of alendronate along with testosterone replacement to men with hypogonadism-induced osteoporosis.     

Community-orientated primary health care. The responsibility of the team for the health of the total population

Community-orientated primary health care (COPHC) is presented as a framework for the delivery of primary health care based on the epidemiologically assessed needs of the population. It requires that the health team and the responsible agencies or institutions take upon themselves responsibility for the provision of care in relation to these measured needs of all those persons entitled to the service. This epidemiological diagnosis of community health conditions will include both the measurement of the distribution of health and disease states in the community as well as the possible causes for this distribution--this is in order to plan possible intervention adequately. It is concluded that COPHC is to the advantage of the community, the health team and the health care system.     

Update on research and future directions of the OMERACT MRI inflammatory arthritis group

The OMERACT Magnetic Resonance Imaging (MRI) Task Force has developed and evolved the psoriatic arthritis MRI score (PsAMRIS) over the last few years, and at OMERACT 10, presented longitudinal evaluation by multiple readers, using PsA datasets obtained from extremity MRI magnets. Further evaluation of this score will require more PsA imaging datasets. As well, due to improved image resolution since the development of the original rheumatoid arthritis MRI scoring system (RAMRIS), the Task Force has worked on semiquantitative assessment of joint space narrowing, and developed a reliable method as a potential RAMRIS addendum, although responsiveness will need to be evaluated. One of the strengths of MRI is the ability to detect subclinical synovitis, so the group worked on obtaining low disease activity/clinical remission datasets from a number of international centers and presented cross-sectional findings. Subsequent longitudinal evaluation of this unique resource will be a major continuing focus for the group.     

Glial M6B stabilizes the axonal membrane at peripheral nodes of Ranvier

Glycoprotein M6B and the closely related proteolipid protein regulate oligodendrocyte myelination in the central nervous system, but their role in the peripheral nervous system is less clear. Here we report that M6B is located at nodes of Ranvier in peripheral nerves where it stabilizes the nodal axolemma. We show that M6B is co‐localized and associates with gliomedin at Schwann cell microvilli that are attached to the nodes. Developmental analysis of sciatic nerves, as well as of myelinating Schwann cells/dorsal root ganglion neurons cultures, revealed that M6B is already present at heminodes, which are considered the precursors of mature nodes of Ranvier. However, in contrast to gliomedin, which accumulates at heminodes with or prior to Na⁺ channels, we often detected Na⁺ channel clusters at heminodes without any associated M6B, indicating that it is not required for initial channel clustering. Consistently, nodal cell adhesion molecules (NF186, NrCAM), ion channels (Nav1.2 and Kv7.2), cytoskeletal proteins (AnkG and βIV spectrin), and microvilli components (pERM, syndecan3, gliomedin), are all present at both heminodes and mature nodes of Ranvier in Gpm6b null mice. Using transmission electron microscopy, we show that the absence of M6B results in progressive appearance of nodal protrusions of the nodal axolemma, that are often accompanied by the presence of enlarged mitochondria. Our results reveal that M6B is a Schwann cell microvilli component that preserves the structural integrity of peripheral nodes of Ranvier.