Double patella. A case of duplication in the coronal plane

A case of duplication of the patella in the coronal plane is reported. Previously reported cases of double patella have shown sagittal or vertical duplication, and some have been associated with multiple epiphyseal dysplasia. In our case, excision of one patella and realignment of the extensor mechanism relieved symptoms of giving-way.     

Evaluation of effects of rofecoxib on platelet function in an in vitro model of thrombosis with circulating human blood

BACKGROUND: Cyclooxygenase (COX)-2-selective non-steroidal anti-inflammatory drugs have been used for anti-inflammatory therapy. However, it has also been described that they may increase risk of cardiovascular events. OBJECTIVES: To study the effects of COX2 inhibitor rofecoxib on platelet function using in vitro tests. Results were compared with those obtained in a parallel experiment with acetyl salicylic acid (ASA). METHODS: Studies of platelet aggregation, using different agonists, were performed by a turbidimetric method. Adhesive and cohesive function of platelets were analyzed by perfusion techniques, treated blood was exposed to thrombogenic surfaces and platelet interaction was morphometrically evaluated. RESULTS: Twenty-five micro M of rofecoxib induced a prolonged lag time and a reduction in the percentage of aggregation when arachidonic acid, ADP or collagen were used as agonists. In perfusion studies with parallel chamber rofecoxib 50 microM and ASA 500 microM reduced overall platelet interaction with the collagen surface (17.4 +/- 3.7, P < 0.05; vs. 32.1 +/- 2.6%P < 0.05 and 17.9 +/- 2.4, vs. 31.9 +/- 3.24, P < 0.05, respectively). In studies performed on annular chambers, 25 micro M of rofecoxib reduced platelet interaction; values of the thrombus and covered surface were 17.4 +/- 4.5%; P < 0.05 and 21.1 +/- 4.1%; P < 0.05, respectively, vs. 30.4 +/- 7.5% and 33.5 +/- 6.5 in the control. ASA did also impair thrombus formation but differences did not reach the levels of statistical significance. Moreover, rofecoxib but not ASA reduced significantly thrombus height and thrombus area (7.4 +/- 0.5 microM; P < 0.005 and 96.0 +/- 21.2 microM(2); P < 0.05 vs. control 11.2 +/- 0.9 microM and 220.0 +/- 47.7 microM(2), respectively). CONCLUSION: We conclude that under our experimental conditions, rofecoxib diminished platelet aggregation induced by different agonists and inhibited platelet-mediated thrombogenesis in an in vitro model of thrombosis.     

Clinical assessment of a flow-through fluorometric blood gas monitor

We performed an observational study to evaluate a flow-through fluorometric instrument (Gas-STAT) that continuously measures the carbon dioxide tension (PCO ₂), oxygen tension (PO ₂), and pH of blood in the cardiopulmonary bypass circuit. Setup and calibration of the instrument typically required 20 minutes. During bypass, 129 blood samples were drawn from 16 patients for comparison with conventional measurements obtained with a blood gas machine. Data for each variable, within each sensor, were analyzed by linear regression. The ranges of the standard errors of the estimate were 0.7 to 4.2 mm Hg forPCO ₂, 18.3 to 78.7 mm Hg for the highPO ₂ range, 1.4 to 7.1 mm Hg for the lowPO ₂ range, and 0.008 to 0.049 for pH. The regression lines differed from the identity line (P<0.05) in at least one variable in most patients, and large deviations from the line of identity in both slope and intercept were common. Among 58 sensors evaluated, failures occurred in 5 (2.9%) of the 174 optodes, and minor leakage occurred in 2 (3.4%) of the flow-through cells. We conclude that although this flow-through fluorometric instrument is an adequate monitor of trends in blood gases during cardiopulmonary bypass, it is not accurate enough to supplant conventional laboratory measurements.Supported in part by grant HL-30881 from the National Institutes of Health.Presented in part at the National Meeting of the American Association for Clinical Chemistry, Atlanta, GA, July 1985, and at the Annual Meeting of the American Society of Anesthesiologists, San Francisco, CA, Oct 1985.The authors thank Dr Brenda Townes for supervising the bypass outcome study of which this was a component; our surgical colleagues, Drs Margaret Allen, Peter McKeown, and Gregory Misbach for allowing us to study their patients; cardiopulmonary perfusionists Gary Tarter, David Anderson, Roland Alberto, and Debora Bley; and the technologists of the Department of Laboratory Medicine.Gas-STAT and disposables were provided by Cardiovascular Devices, Inc.     

Leg length,sitting height,and body proportions references for achondroplasia: New tools for monitoring growth

Achondroplasia is the most common form of inherited disproportionate short stature. We report leg length, sitting height, and body proportion curves for achondroplasia. Seven centile format of sitting height, leg length, sitting height/leg length ratio, sitting height/height ratio, and head circumference/height ratio were estimated by the LMS method. The Q‐test was applied to assess the goodness of fit. For comparison, centiles of sitting height and leg length were graphed using Argentine national growth references for achondroplasia and non‐achondroplasia populations. The sample consisted of 342 children with achondroplasia (171 males, 171 females) aged 0–18 years. The median (interquartile range) number of measurements per child was 6 (3, 12) for sitting height and 8 (3, 13) for head circumference. Median leg length increased from 14 cm at age 1 week to 44 and 40 cm (males and females, respectively) in achondroplasia adolescents which is 3.5 cm shorter than non‐achondroplasia children at age 1 week and, 38 cm shorter at adolescence. Median sitting height increased from 34 cm at birth to 86 and 81 in adolescents’ boys and girls respectively, only 5 cm shorter than non‐achondroplasia children. Sitting height/leg length decreased from 2.61 at birth to approximately 1.90 at adolescent. Median head circumference/height ratio decreased from 0.79 at birth to 0.46 at 18 years in both sexes. Growth of lower limbs is affected early in life and becomes more noticeable throughout childhood. The disharmonic growth between the less affected trunk and the severely affected limbs determine body disproportion in achondroplasia.

     

A bio‐artificial renal epithelial cell system conveys survival advantage in a porcine model of septic shock

Renal cell therapy using the hollow fiber based renal assist device (RAD) improved survival time in an animal model of septic shock (SS) through the amelioration of cardiac and vascular dysfunction. Safety and ability of the RAD to improve clinical outcomes was demonstrated in a Phase II clinical trial, in which patients had high prevalence of sepsis. Even with these promising results, clinical delivery of cell therapy is hampered by manufacturing hurdles, including cell sourcing, large‐scale device manufacture, storage and delivery. To address these limitations, the bioartificial renal epithelial cell system (BRECS) was developed. The BRECS contains human renal tubule epithelial cells derived from adult progenitor cells using enhanced propagation techniques. Cells were seeded onto trabeculated disks of niobium‐coated carbon, held within cryopreservable, perfusable, injection‐moulded polycarbonate housing. The study objective was to evaluate the BRECS in a porcine model of SS to establish conservation of efficacy after necessary cell sourcing and design modifications; a pre‐clinical requirement to move back into clinical trials. SS was incited by peritoneal injection of E. coli simultaneous to insertion of BRECS (n=10) or control (n=15), into the ultrafiltrate biofeedback component of an extracorporeal circuit. Comparable to RAD, prolonged survival of the BRECS cohort was conveyed through stabilization of cardiac output and vascular leak. In conclusion, the demonstration of conserved efficacy with BRECS therapy in a porcine SS model represents a crucial step toward returning renal cell therapy to the clinical setting, initially targeting ICU patients with acute kidney injury requiring continuous renal replacement therapy. Copyright © 2014 John Wiley & Sons, Ltd.     

Implication of long-distance regulation of the HOXA cluster in a patient with postaxial polydactyly

Apparently balanced chromosomal inversions may lead to disruption of developmentally important genes at the breakpoints of the inversion, causing congenital malformations. Characterization of such inversions may therefore lead to new insights in human development. Here, we report on a de novo inversion of chromosome 7 (p15.2q36.3) in a patient with postaxial polysyndactyly. The breakpoints do not disrupt likely candidate genes for the limb phenotype observed in the patient. However, on the p-arm the breakpoint separates the HOXA cluster from a gene desert containing several conserved noncoding elements, suggesting that a disruption of a cis-regulatory circuit of the HOXA cluster could be the underlying cause of the phenotype in this patient.     

Breast cancer seeding associated with core needle biopsies: A systematic review

Background

Preoperative diagnosis has become the standard in breast cancer (BC) management. Recently, ultrasound guided core needle biopsy (CNB) and stereotactic needle core biopsy have replaced fine needle aspiration cytology. Epithelial cell displacement (DE) occurs frequently after core needle biopsy (CNB) for breast cancer diagnosis.

Aim

Systematically review (between 1900 and 2008) the clinical significance of DE after CNB in BC patients, and associated risk factors (delay between biopsy and surgery, needle passes, duration of the procedure, tumor size, histological type, tumor grade, margins, type of surgery, and of adjuvant treatment).

Materials and methods

We selected 15 studies: 9 assessed the rate of DE after CNB and 6 the impact of CNB on outcome endpoints.

Results

We found 3 prospective and 12 retrospective studies. However these had numerous biases such as insufficient power, confounding factors, selection of cases and controls, surrogate endpoints, heterogeneity of measured displacement. Malignant DE on surgical specimens occurred in 22% of the patients. A short interval between CNB and surgical excision increased the risk of detecting displaced cells. No increase in local recurrence was reported after CNB. Contradictory results were found in terms of sentinel node metastases. Only one study evaluated overall survival data and reported no worse survival in patients with preoperative CNB.

Conclusion

Although data are limited, no increased morbidity has been associated with iatrogenic seeding after CNB.     

Personality dimensions in obsessive-compulsive disorder: relation to clinical variables

Research on the relationship between personality factors and obsessive-compulsive disorder (OCD) has proved difficult to interpret due to conceptual problems including a lack of consensus on the model of personality employed as a framework as well as a failure to consider the clinical heterogeneity of the disorder. The aim of this study was to examine the dimensional personality profile associated with OCD and to determine whether any relationship exists between personality factors and clinical variables in a sample of 60 OCD outpatients who were administered Cloninger's Temperament and Character Inventory (TCI). The Yale-Brown Obsessive-Compulsive Scale (Y-BOCS), the Hamilton Depression Rating Scale (HDRS) and the Y-BOCS symptom checklist were used to assess the severity of obsessive-compulsive and depressive symptoms and the presence of the main OCD symptom dimensions. OCD patients showed significantly higher scores in harm avoidance and lower scores in novelty-seeking, self-directedness and cooperativeness than healthy subjects. These results remained unchanged when only pure OCD patients without comorbid psychiatric conditions were considered. Comorbid depressive symptoms and hoarding obsessions and compulsions were significantly associated with high harm avoidance scores. These results support the existence of a dimensional personality profile associated with OCD and characterized by high harm avoidance and low novelty-seeking, self-directedness and cooperativeness scores, but also emphasize the importance of considering the influence of comorbid clinical conditions or symptom subtypes in addressing the role of personality factors in OCD.     

Vestibular examination of children with alterations in balance (I): clinical and instrumental examination methods

This is a review of the normal methods for vestibular examination, both clinical and instrumental, to evaluate the vestibular system. Emphasis is given to the examination sequence and objectives in each method.     

Similar cytokine profiles in response to infection with respiratory syncytial virus type a and type B in the upper respiratory tract in infants

Human respiratory syncytial virus (RSV) is the leading viral cause of severe respiratory illness in infants and young children worldwide. RSV isolates can be divided into 2 subgroups, type A and type B. Here, we compare for the first time the nasal profiles of 27 immune mediators in response to both viral subtypes in 14 children infected with RSV/A, 8 children infected with RSV/B, 11 children coinfected with RSV/A plus other respiratory viruses, and finally, 27 control children, all <2 years old. Our results evidence that children's infection with both RSV subtypes induces very similar profiles of immune mediators in the upper respiratory tract, characterized by the elevation of Th1 and Th2 cytokines, chemokines and growth factors. Interestingly, no major differences in the profiles of the immune mediators were found between the children infected exclusively with RSV/A and those infected with RSV/A plus other respiratory viruses.