Unusual case of simultaneous presentation of plasma cell myeloma, chronic myelogenous leukemia, and a jak2 positive myeloproliferative disorder.

Background. Multiple articles discuss the rare incidence and potential causes of second hematologic disorders arising after treatment of Chronic Myelogenous Leukemia (CML), leading to the theory of imatinib, the current treatment regimen for CML, as a possible trigger for the development of secondary neoplasms. Our case eliminates the possibility of imatinib as the sole cause since our patient received a diagnosis of simultaneous plasma cell myeloma, CML, and a Jak2 mutation positive myeloproliferative disorder (MPD) arising de novo, prior to any treatment. We will further investigate into alternative theories as potential causes for multiple hematopathologic disorders. Case Report. There are currently no reported cases with the diagnosis of simultaneous plasma cell myeloma, chronic myelogenous leukemia, and Jak2 positive myeloproliferative disorder. We present a case of a 77-year-old male who was discovered to have these three concurring hematopathologic diagnoses. Our review of the literature includes a look at potential associations linking the three coexisting hematologic entities. Conclusion. The mechanism resulting in simultaneous malignancies is most likely multifactorial and potentially includes factors specific to the host, continuous stimulation of the immune system, previous chemotherapy or radiation, a potential common pluripotent stem cell, or, lastly, preexisting myeloma which may increase the susceptibility of additional malignancies.

¿Hay un modelo experimental animal adecuado para el estudio del hombro? / Is there an adequate experimental animal model for the study of the shoulder?

Objetivo: Describir el esqueleto de la articulación del hombro en diferentes especies para establecer un modelo experimental de la patología del hombro. Material y metodología: Obtuvimos hombros de diferentes especies: perro (beagle), cerdo (mini-pig), conejo de Nueva Zelanda, liebre, cordero y oveja adulta. Extrajimos las articulaciones escápulo-humerales de ambos lados. Efectuamos una radiografía en cuatro proyecciones (antero-posterior, axial y dos oblicuas) y un TAC con reconstrucción tridimensional. Posteriormente disecamos las piezas para analizar el espacio subacromial y la cavidad glenoidea. Resultados: Ninguna de las especies analizadas posee un acromion desarrollado ni coracoides para definir un espacio subacromial. El cerdo presenta una cápsula posterior más desarrollada que la anterior y un m. subescapular desarrollado y el m. bíceps presenta un tendón en su porción larga pequeño y extraarticular con una corredera bicipital profunda. La oveja presenta un labrum superior desarrollado, con un tendón de la porción larga del m. bíceps en una corredera poco profunda. Conclusión: No hemos encontrado un modelo experimental adecuado para estudiar la patología del espacio subacromial. La oveja y el cerdo pueden ser buenos modelos para estudiar la patología de la glenoides (AU)

Objective: To describe the skeleton of the shoulder joint in different species to establish an experimental model of shoulder pathology. Material and methods: Shoulders were obtained from different species: dog (beagle), pig (minipig), New Zealand rabbit, hare, lamb and adult sheep. The scapulohumeral joints from both sides were extracted. X-ray images were obtained in 4 projections (anteroposterior, axial and two oblique) and a CT scan with 3D reconstruction. We then dissected the pieces to analyze the subacromial space and glenoid cavity. Results: None of the species analyzed possesses a developed acromion or coracoid to define a subacromial space. The pig has a more developed posterior than anterior capsule and a developed subscapularis muscle and the biceps muscle has a tendon on its extraarticular small long portion with a deep bicipital groove. The sheep has a developed superior labrum, with a tendon of the long portion of the biceps muscle in a shallow groove. Conclusion: We did not find an adequate experimental model to study the pathology of the subacromial space. The sheep and the pig may be good model to study the pathology of the glenoid (AU)

Hemiartroplastia humeral asociada a reconstrucción biológica glenoidea en pacientes jóvenes con artrosis gleno-humeral / Humeral hemiarthroplasty associated with a biologic glenoid repair in young patients

El tratamiento de la artrosis gleno-humeral en pacientes jóvenes y activos constituye un desafío para todo cirujano ortopédico. La reconstrucción biológica glenoidea surge como una alternativa a la artroplastia total de hombro en aquellos pacientes con artrosis gleno-humeral, presentando un alivio sintomático similar a la artroplastia total de hombro pero sin las complicaciones del componente protésico glenoideo. El objetivo de este artículo es la presentación de la técnica quirúrgica de reconstrucción bilógica glenoidea empleando injerto de fascia lata autólogo (AU)

Treatment of osteoarthritis of the glenohumeral joint in young and active patients represents a therapeutic challenge for orthopaedic surgeons. Biologic glenoid resurfacing was developed as an alternative to total shoulder arthroplasty for those patients with glenohumeral arthritis providing pain relief similar to total shoulder arthroplasty without the risk of polyethylene wear. The purpose of this article is to present an alternative surgical procedure of biologic glenoid resurfacing employing autologous fascia lata (AU)

Atrapamiento o choque femoroacetabular / Femoroacetabular impingement

Introducción. El atrapamiento o choque femoroacetabular es una patología conocida y publicada recientemente en la comunidad médica. Diagnóstico. Se caracteriza por dolor en los movimientos de flexión-rotación interna-aducción de la cadera que provoca alteraciones degenerativas en el cartílago articular y en el complejo cápsulo-labral. Si bien existen diferentes combinaciones en cuanto a su mecanismo lesional, también concurrirán diferentes alteraciones femoroacetabulares, que se enuncian en esta actualización. Su diagnóstico se basará en la clínica, radiología, ­con signos radiológicos patognomónicos­, tomografía axial computarizada y resonancia magnética nuclear. En algunos casos será necesaria la artrorresonancia. Tratamiento. Aunque existen ya otras vías entre el tratamiento conservador y la artroplastia de cadera, como es la artroscopia en los estadios iniciales, con posible reparación del labrum acetabular, la osteoplastia femoroacetabular posiblemente sea hoy por hoy, en adultos jóvenes activos, el tratamiento de elección cuando comienza el proceso degenerativo. Se reserva la artroplastia de superficie para pacientes con grado avanzado de deterioro articular y con ciertas expectativas de actividad física, aunque ya sean de menor intensidad. Siempre quedará como último recurso la artroplastia total de cadera. Conclusiones. Si bien estas técnicas quirúrgicas de reciente introducción proporcionan resultados muy alentadores, nunca hay que olvidar que existen casos en los que concurren diversas alteraciones con desviación femoral, acetabular o combinadas. En estos casos, y con anterioridad a la desestructuración de la articulación, las osteotomías correctoras tendrán su lugar

Introduction. Femoroacetabular impingement is a condition that has only recently become familiar in medical circles and been the subject of published reports. Diagnosis. There is pain on hip flexion-internal rotation-and adduction. These movements cause degenerative alterations of the joint cartilage and the capsule-labrum complex. Though the causes of disorders of this joint are different lesions, there are also different femoroacetabular alterations that are the underlying cause. Diagnosis is based on clinical examination, X-rays -characteristic radiological signs-CT and MRI scans. In some cases MR arthrography may be necessary. Treatment. Nowadays, there are many alternatives that range from conservative treatment to total hip replacement. One is arthroscopy in the initial stages, with the possibility of labrum repair. However, it is possible that femoroacetabular osteoplasty is currently, in young active adults, the treatment of choice when joint degeneration begins. Resurfacing arthroplasty is for patients with an advanced degree of joint deterioration who wish to continue physical activity, but of a less strenuous kind. Total hip replacement will always be a last resort. Conclusions. Although these recently introduced surgical techniques have very encouraging outcomes, we must always remember that there are cases in which several alterations are present with femoral, acetabular or combined disorders. In these cases, before taking down the joint, corrective osteotomies are advisable

Comparison of family history measures used to identify high risk of coronary heart disease.

We examined 15 published continuous family history measures (scores) as well as two new formulations in terms of several desirable properties. We applied the scores to sample pedigrees and found that some systematically increase with family size. In contrast to aggregate scores, non-aggregate scores are sensitive to the age, sex, and covariate status of individual relatives but are unstable when the families are small. We also applied these scores to our own population case-control data, characterised by a high proportion of missing and false-negative responses. In these small families, all scores provided significant discrimination between CHD cases and controls beyond the usual categorical definition of positive family history, but appeared no better than detailed categorical definitions or even simple counts. Our new formulations offer no solution to the problems of few data; most scores apply asymptotic approximations to differences between observed and expected number of affected relatives and are not suited to small families. All scores would be improved by ruling out families with only one affected relative, as is being done in the NHLBI Family Heart Study. We recommend that researchers, when using a family history measure, consider the number of informative families and other characteristics of their data prior to choosing any particular formulation.

Correction for biases in a population-based study of family history and coronary heart disease. The Newcastle Family History Study I.

In this paper, the authors report on the design of a population-based case-control study of family history as a risk factor for coronary heart disease (CHD). They studied the characteristics of subjects who completed a detailed family history questionnaire in 1992-1994 as well as the accuracy of recall of family history in order to quantify both selection and recall biases. Coronary disease cases were enrolled through the Newcastle MONICA Project (Monitoring Trends and Determinants in Cardiovascular Disease), which registered all suspected heart attacks and sudden cardiac deaths in the Lower Hunter region of New South Wales, Australia, between August 1984 and March 1994. Controls were selected at random from the New South Wales electoral roll. The response rate was 76% in cases and 62% in controls; the major factor associated with participation in the study was perceived family history of CHD, more so in the control series than in the case series. Accuracy was determined by comparing information obtained from the proband with that recorded on death certificates. In first-degree relatives, sensitivity of CHD recall was 85% (95% confidence interval (CI) 74-92%) in cases and 95% (95% CI 84-99%) in controls, while specificity was 59% (95% CI 49-69%) and 74% (95% CI 65-82%), respectively. The net bias in both selection and recall is toward the null and hence the comparisons provide a conservative estimate of risk of CHD associated with a positive family history.

Risk associated with various definitions of family history of coronary heart disease. The Newcastle Family History Study II.

The authors carried out a population-based case-control study to estimate the risk of an acute coronary disease event associated with various definitions of a family history of coronary heart disease (CHD). A detailed family history questionnaire was completed by 403 cases and 236 controls in Newcastle, New South Wales, Australia from 1992 to 1994. Odds ratios of an acute coronary disease event adjusted for proband age and sex ranged from 2.7 (95% confidence interval (CI) 1.8-4.1) for the simplest definition (one or more first-degree relatives with CHD at any age) to 5.4 (95% CI 1.7-16.8) for the most stringent definition (two or more first-degree relatives with CHD before age 55 years). In a series of nested models, the authors examined the improvement in model fit as each component of the detailed family history was added. Additional information was provided by accounting for "don't know" responses, the number of affected relatives, the age of the affected relative, and whether the first-degree relative was a sibling rather than a parent. The results were similar when the data were analyzed as a cohort design with proband disease status as the exposure variable. The authors suggest that, to facilitate preventive efforts in a population, more detailed family history definitions should be used to better target high risk subjects.

Gender differences and other determinants of the rise in plasma homocysteine after L-methionine loading.

The methionine load test is widely used to identify subjects with mild homocysteinemia. We studied factors influencing the rise in plasma homocysteine (tHcy) levels following oral methionine loading, after taking into account determinants of the basal level. The study population comprised 522 persons (mean age 53, range 17 to 95; 63% male; 60% with coronary disease). tHcy was measured fasting and 4 h after administration of L-methionine (dose 4 g/m2). The percentage rise in tHcy was greater in females, at younger ages, and with lower serum creatinine. Basal tHcy was lower in those with high levels of serum folate, red cell folate and serum B12; they also showed a greater percentage rise with loading. After adjustment for age, creatinine and serum vitamins the percent rise was greater in females (mean 230%, S.D. 92) than in males (mean 172%, S.D. 71, P < 0.0001). About 1/4 of this effect was explained by the methionine dose, but important independent gender effects remained. When the results of the loading test were compared with an overall reference range, females were systematically labelled as 'high' but comparison with age and gender-specific reference ranges normalised the sex distribution. Our data show that a single L-methionine loading protocol for both sexes is not appropriate. Gender-specific reference ranges should be used to define post-load homocysteinemia and previous studies which have identified a high proportion of females with this trait probably reflect the need for gender specific reference ranges.

Fasting and post-methionine homocyst(e)ine levels in a healthy Australian population.

BACKGROUND: Plasma homocyst(e)ine (H[e]) is frequently measured in patients with occlusive vascular disease, but levels vary between populations and between laboratories. AIMS: We sought to derive reference values for an Australian population over a wide age range. METHODS: We measured plasma H(e) in the fasting state and after methionine loading in 116 volunteers selected at random from the Hunter districts of the New South Wales electoral roll and in 49 apparently healthy, active subjects recruited from the region's lawn bowling clubs. Reference ranges were derived for both sexes, in three age strata. We collaborated with two international laboratories in standardising our results. RESULTS: Mean fasting H(e) levels were approximately 2.7 mumol/L higher in men than women, at all ages (95% CI for the difference, 1.2 to 4.1 mumol/L). Levels increased with age (approximately 1.0 mumol/L/decade, 95% CI 0.5 to 1.5) and were correlated with serum creatine (r = 0.48), serum folate (r = -0.30), red cell folate (r = -0.25) and serum B12 (r = -0.31) (all p < 0.001). These results are similar to those reported in other populations. CONCLUSIONS: We have defined reference ranges for a typical Australian population, following careful laboratory standardisation. H(e) levels must be interpreted with regard to age, sex, renal function and vitamin B12 and folate status.

Reproducibility of brachial ultrasonography and flow-mediated dilatation (FMD) for assessing endothelial function.

BACKGROUND: High-resolution brachial artery ultrasonography is used to study vasodilator response induced by physiologic reactive hyperaemia. We examined the reproducibility of measuring flow-mediated dilatation (FMD) on two occasions. AIMS: To determine the degree of variability of this technique in our vascular laboratory for the design of clinical research studies. METHODS: Nineteen subjects were studied on two separate occasions using an Acuson 128 ultrasound device and a 7.0 MHz linear array transducer. Reactive hyperaemia was induced in the brachial artery by inflation and release of a blood pressure cuff. Nitrate-induced dilatation was assessed in 11 of the 19 subjects. Measurements were made by two observers blinded to subject details. RESULTS: The 11 subjects given sublingual GTN during the first ultrasound study had a mean nitrate-induced dilatation of 20.7% (sd 9.6). The mean vessel diameter of 3.78 mm (sd 0.7) at rest and 3.89 mm (sd 0.7) during reactive hyperaemia yielded a mean FMD of only 3.0% (sd 2.7). The mean difference in FMD within-observers was 0.13% (sd 2.07), between-observers 0.06% (sd 2.17) and between-studies was 0.57% (sd 6.83). CONCLUSIONS: The reproducibility of FMD measured by brachial artery ultrasound was poor and likely to render the measurements inaccurate for clinical research in our hands. Between-study variation contributed the largest proportion of total study variability. We suggest that investigators using this technique conduct their own careful reproducibility studies in order to avoid the misinterpretation of 'negative' studies.

[Women's hearts are hard to break. Coronary heart diseases--is the woman a special case!?]. / Women's hearts are hard to break. Koronare Herzkrankheit--Speziallfall Frau!?

1. Recommendations and decisions concerning treatment should primarily be based on the absolute evaluation of risk and risk reduction and not on calculation of a relative risk situation. 2. The therapeutic benefit in women at low risk should be balanced against possible physical and psychic side effects and submitted to a cost/benefit analysis. 3. The fact that coronary heart disease is rare in young women must not at all be reason for a less consequent treatment and prevention in women with established coronary heart disease.

Assay of plasma homocysteine: light sensitivity of the fluorescent 7-benzo-2-oxa-1, 3-diazole-4-sulfonic acid derivative, and use of appropriate calibrators.

The recognition of homocysteine as a vascular risk factor has led to increased clinical interest in assaying plasma homocysteine concentrations. Our aim was to improve the reliability of a widely used assay based on HPLC of the fluorescent 7-benzo-2-oxa-1, 3-diazole-4-sulfonic acid (SBD) derivative. We found that SBD derivatives of homocysteine, cysteine, and N-acetylhomocysteine were highly unstable in light but essentially stable in the dark for several hours at either 0 degree C or 25 degrees C. As our primary calibrator, we chose homocystine added to human serum for more consistent results than homocysteine or homocystine in an aqueous buffer. N-acetylcysteine was effective as an internal recovery standard. We observed a previously unreported peak with a prolonged elution time in some plasma samples from subjects who had ingested methionine. Our findings suggest improvements in this and other assay procedures for plasma homocysteine.

Cardiovascular findings in self-reported healthy elderly. The Elite Seniors Study.

BACKGROUND: A descriptive study of self-reported healthy elderly in Newcastle was undertaken for an adequate understanding of the normal characteristics of the elderly. AIMS: We sought to determine the frequency of abnormal clinical, laboratory and echocardiographic findings in a self-reported healthy elderly population. METHODS: Volunteers aged greater than 75 years were recruited from active members of the region's bowling clubs. Forty-five subjects with no self-reported vascular or cardiac disease underwent clinical, laboratory and echocardiography assessment. RESULTS: Minor cardiovascular abnormalities were frequently detected. Echocardiography correspondingly found a high frequency of haemodynamically insignificant degenerative valvular lesions. Doppler left ventricular filling pattern of abnormal relaxation was a universal finding. CONCLUSION: There is a high prevalence of age-related echo-Doppler abnormalities in the self-reported healthy elderly with important implications for the interpretation of echo-Doppler studies.