[Place of 3D custom-made implants after failure of modeling steno-chondro-plasties]. / Place des implants sur mesure 3D après échec des sterno-chondro-plasties modelantes.

Most common congenital malformation of the thorax, Pectus Excavatum affects about one in 500 people. Several surgical or medical techniques have been proposed. Some are followed by complications or insufficient results even though their constant functional value is highly controversial. Secondary surgery with a deep customized 3D elastomer implant, may be an elegant effective and safe solution compared to others; it allows a good aesthetic result expected by patients in the absence of any respiratory or cardio-vascular functional context.

The impact of chronic skin disease in adolescence and the need for specialist adolescent services.

Adolescence is a critical period in both physical and psychological development. Skin conditions are very common in this age group, and are known to impart a considerable psychological burden, with higher rates of both anxiety and depression in those with chronic skin disease. The Department of Health has identified the specialized needs of adolescents as they transition from paediatric to adult services as a key priority, with emphasis on providing 'developmentally appropriate healthcare'. However, in spite of this, there are very few dedicated transitional clinics in the UK with appropriate psychosocial support. We have demonstrated in our own clinical practices that a dedicated teenage and young adult dermatology clinic with embedded specialist psychological support can deliver effective dermatological care to young people with a variety of skin conditions. We call for a greater focus on achieving developmentally appropriate care for adolescents across the UK to address the unmet transitional and psychological care needs in this population.

Establishing and developing a Teenage and Young Adult dermatology clinic with embedded specialist psychological support.

Skin conditions are common in adolescence and impart considerable psychological burden. The Department of Health has identified the specialized needs of adolescents transitioning from paediatric to adult services as a priority, yet there are few dedicated transitional clinics in the UK providing appropriate psychosocial support. We have established a monthly Teenage and Young Adult (TYA) dermatology clinic dedicated to managing teenagers and young adults with skin disease alongside open-access psychological support. Demographic data and Teenagers' Quality of Life Index (T-QoL) measures were recorded for all patients in 2016. To evaluate patient experience, two online surveys were conducted. Statistically significant improvements in the T-QoL were recorded for patients with the most common skin condition (eczema) attending for repeat assessment by the psychologist. Patients reported high satisfaction rates in both patient experience surveys. These results demonstrate that specialized adolescent care both is well received and can improve outcomes for these patients.

Suicide in patients suffering from late-life anxiety disorders; a comparison with younger patients.

BACKGROUND: Anxiety disorders are assumed to increase suicide risk, although confounding by comorbid psychiatric disorders may be one explanation. This study describes the characteristics of older patients with an anxiety disorder who died by suicide in comparison to younger patients. METHOD: A 15-year national clinical survey of all suicides in the UK (n = 25,128). Among the 4,481 older patients who died by suicide (≥ 60 years), 209 (4.7%) suffered from a primary anxiety disorder, and 533 (11.9%) from a comorbid anxiety disorder. Characteristics of older (n = 209) and younger (n = 773) patients with a primary anxiety disorder were compared by logistic regression adjusted for sex and living arrangement. RESULTS: Compared to younger patients, older patients with a primary anxiety disorder were more often males and more often lived alone. Although 60% of older patients had a history of psychiatric admissions and 50% of deliberate self-harm, a history of self-harm, violence, and substance misuse was significantly less frequent compared to younger patients, whereas physical health problems and comorbid depressive illness were more common. Older patients were prescribed significantly more psychotropic drugs and received less psychotherapy compared to younger patients. CONCLUSION: Anxiety disorders are involved in one of every six older patients who died by suicide. Characteristics among patients who died by suicide show severe psychopathology, with a more prominent role for physical decline and social isolation compared to their younger counterparts. Moreover, treatment was less optimal in the elderly, suggesting ageism. These results shed light on the phenomenon of suicide in late-life anxiety disorder and suggest areas where prevention efforts might be focused.

Familial rare EGFR-mutant lung cancer syndrome: Review of literature and description of R776H family.

BACKGROUND: Interest in hereditary lung cancer is increasing, in particular germline mutations in the Epidermal Growth Factor Receptor (EGFR) gene. We review the current literature on this topic, discuss risk of developing lung cancer, treatment and screening options and describe a family of 3 sisters with lung cancer and their unaffected mother all with a rare EGFR germline mutation (EGFR p.R776H). METHODS: We searched PubMed, Medline, Embase, the Cochrane Library, Google Scholar and scanned reference lists of articles. Search terms included "EGFR germline" and "familial lung cancer" or "EGFR familial lung cancer". We also describe our experience of managing a family with rare germline EGFR mutant lung cancer. RESULTS: Although the numbers are small, the described cases in the literature show several similarities. The patients are younger and usually have no or light smoking history. 50% of the patients were treated with a tyrosine kinase inhibitor (TKIs) with OS over six months. CONCLUSION: Although rare, germline p.R776H EGFR lung cancer mutations are over-represented in light or never smoking female patients who often also possess an additional somatic EGFR mutation. Treatment with TKIs appears suitable but further research is needed into the appropriate screening regime for unaffected carriers or light/never smokers.

Psychiatric in-patient care and suicide in England, 1997 to 2008: a longitudinal study.

BACKGROUND: Psychiatric in-patients are at high risk of suicide. Recent reductions in bed numbers in many countries may have affected this risk but few studies have specifically investigated temporal trends. We aimed to explore trends in psychiatric in-patient suicide over time. METHOD: A prospective study of all patients admitted to National Health Service (NHS) in-patient psychiatric care in England (1997-2008). Suicide rates were determined using National Confidential Inquiry and Hospital Episode Statistics (HES) data. RESULTS: Over the study period there were 1942 psychiatric in-patient suicides. Between the first 2 years of the study (1997, 1998) and the last 2 years (2007, 2008) the rate of in-patient suicide fell by nearly one-third from 2.45 to 1.68 per 100,000 bed days. This fall in rate was observed for males and females, across ethnicities and diagnoses. It was most marked for patients aged 15-44 years. Rates also fell for the most common suicide methods, particularly suicide by hanging on the ward (a 59% reduction). Although the number of post-discharge suicides fell, the rate of post-discharge suicide may have increased by 19%. The number of suicide deaths in those under the care of crisis resolution/home treatment teams has increased in recent years to approximately 160 annually. CONCLUSIONS: The rate of suicide among psychiatric in-patients in England has fallen considerably. Possible explanations include falling general population rates, changes in the at-risk population or improved in-patient safety. However, a transfer of risk to the period after discharge or other clinical settings such as crisis resolution teams cannot be ruled out.

A racial bias test with tertiary cancer centre employees: why anti-racist measures are required for First Nations Australians cancer care equity.

Objective To examine implicit bias in employees at a cancer centre using an Australian race (Aboriginal-white) Implicit Association Test (IAT), in an attempt to understand a potential factor for inequitable outcomes of First Nations Australians cancer patients. Methods All employees at an Australian cancer centre were invited to take part in a web-based, cross-sectional study using an Australian race IAT. The results were analysed using Welch t-tests, linear regression and ANOVA. Results Overall, 538/2871 participants (19%) completed the IAT between January and June 2020. The mean IAT was 0.147 (s.d. 0.43, P < 0.001, 95% CI 0.11-0.18), and 60% had a preference for white over First Nations Australians. There was no significant mean difference in IAT scores between sub-groups of gender, age or clinical/non-clinical employees. 21% of employees (95% CI 17.65-24.53) had moderate to strong preference for white over First Nations Australians, compared to 7.1% with moderate to strong preference for First Nations over white Australians (95% CI 5.01-9.09). Conclusions Inequitable cancer survival for First Nations patients has been well established and cancer is now the leading cause of mortality. This paper documents the presence of racial bias in employees at one cancer centre. We argue that this cannot be understood outside the history of colonialism and its effects on First Nations Australians, healthcare workers and our society. Further research is required to evaluate measures of racism, its effect on health care, and how to eliminate it.

Horner's syndrome in a patient presenting with a spontaneous pneumothorax.

Spontaneous pneumothoraces are a common thoracic problem presenting to an Accident and Emergency (A&E) department. The symptoms and signs are well described and a chest x-ray examination is usually diagnostic. However the neurological signs, specifically a Horner's syndrome on the ipsilateral side, are not widely recognised. This case illustrates the association and emphasises that when assessing a patient with a suspected spontaneous pneumothorax, an ipsilateral Horner's syndrome supports the clinical diagnosis. Further, its presence makes a tensioning pneumothorax, or as in this case a pneumothorax with significant collapse and apical adhesions, more likely. No previous case reporting the association has had the opportunity for thorascopic assessment and demonstration of likely cause.

The role of a video-assisted thoracic approach for rib fixation.

PURPOSE: Rib fixation remains a contentious issue in the current practice of orthopaedic, trauma and thoracic surgeons. Whilst rib fractures are undoubtedly associated with high levels of morbidity and mortality, the optimal surgical approach has not yet been fully elucidated in prospective trials and the volume of procedures performed remains low. METHODS: We evaluated 21 consecutive patients who underwent surgical rib fixation either via a standard thoracotomy approach or following the introduction of a video-assisted technique with minimal thoracic incisions. RESULTS: The average age of patients undergoing rib fixation was 47 and the median length of post-operative stay was 4 days. More than 70 % of patients were found to have concurrent haemothoraces, and 19 % had significant injuries to underlying intra-thoracic structures requiring repair. One patient returned to theatre for persistent blood loss; however, there were no other immediate complications or mortalities. CONCLUSIONS: We discuss the involvement of thoracic surgeons, early assessment of the thoracic cavity with video assistance and optimal peri-operative management with particular reference to cases which demonstrate recent changes in our practice.

Investigations of the reliability of observational gait analysis for the assessment of lameness in horses.

The objectives of this study were to assess the reliability of a numerical rating scale (NRS) and a verbal rating scale (VRS) for the assessment of lameness in horses and to determine whether they can be used interchangeably. Sixteen independent observers graded the severity of lameness in 20 videotaped horses, and the agreement between and within observers, correlation and bias were determined for each scale. The observers agreed with each other in 56 per cent of the observations with the NRS and in 60 per cent of the observations with the VRS, and the associated Kendall coefficient of concordance was high. Similar trends were evident in the agreement between two observations by each observer. The correlation between and within observers was high for both scales. There were no significant differences (bias) among the observers' mean scores when using either scale. There was a significant correlation between the lameness scores attributed when using the two scales, but the differences between the scores when plotted against their overall mean were unacceptable for clinical purposes. The results indicate that the NRS and VRS are only moderately reliable when used to assess lameness severity in the horse, and that they should not be used interchangeably.

The Kinetics of N-Ethylmaleimide Inhibition of a Vacuolar H+-ATPase and Determination of Nucleotide Dissociation Constants.

All eukaryotic vacuolar (V-type) ATPases share the property of being inhibited by low concentrations (1-2 [mu]M) if N-ethylmaleimide (NEM). This distinguishes them from P-type ATPases, which are inhibited by higher concentrations of NEM (0.1-1 mM), and F-type ATPases, which are virtually resistant to inhibition by NEM. Using tonoplast vesicles from Beta vulgaris we have determined the kinetics of NEM inactivation of the V-type ATPase to be pseudo-first order. The concentration dependence of the reaction indicates interaction with a single class of inhibitory site with a rate constant of 4.1 x 104 M-1 min-1. Nucleotides protect against inactivation with an efficacy that agrees with their capacity to act as enzyme substrates. The dissociation constant for MgATP has been determined from protection experiments to be 0.44 mM, which is close to the observed Km for hydrolysis (0.39 mM). Likewise, the dissociation constant for protection by MgADP (127 [mu]M) is close to its inhibition constant as a competitive inhibitor (110 [mu]M). Taken together, these findings suggest that NEM inactivation is associated with nucleotide protectable exposure of a single cysteine residue on the catalytic subunit and confirm the utility of this residue for the determination of ligand dissociation constants through protection of maleimide inhibition.

A case of co-existing paraganglioma and thymoma.

BACKGROUND: Head and neck paragangliomas are rare tumours and can arise as a part of inherited syndromes. Their association with thymic tumour is not well known. CASE DESCRIPTION: This report describes a female patient who presented with right sided neck paragangliomas. The histology of the tumour was consistent with paraganlioma. Few years later her MRI scan of the chest revealed presence of an anterior mediastinal mass that corresponded to the location of the thymus. Review of her previous scans showed that the mass was present all along and had gradually increased in size. Patient developed symptoms including fatigue, dyspnoea, migratory polyarthritis, Raynaud's phenomenon and erythema nodosum. She had sternotomy and excision of mediastinal mass. The histology was consistent with cortical thymoma (WHO type B2) and she had radiotherapy. After treatment her constitutional symptoms improved. Her paraganglioma susceptibility genes are negative. DISCUSSION AND EVALUATION: To our knowledge this is only the second case report in the literature of coexistence of carotid body tumour and thymoma. The first case reported was bilateral carotid body tumour, thyroid gland adenoma and thymoma. This case also highlights the importance of long term surveillance, multidisciplinary management and being aware of associated pathologies in patients with isolated paraganglioma.

Food and nutrient intake of Seventh-day Adventist women.

Dietary intake data were collected on Seventh-day Adventist vegetarian and Methodist omnivorous postmenopausal women living in the Los Angeles area: Both groups met the Recommended Dietary Allowances except for zinc, folate, vitamin B-6, and vitamin E. Vegetarians' diets had a somewhat higher nutrient density for folate, thiamin, vitamin C, and vitamin A; lower total fat, saturated fatty acids, and cholesterol; and higher dietary fiber. No correlation between dietary intake and bone mineral content was observed. These findings are compared with those in a study on Oregon vegetarian women.

Dietary zinc intake of low-income pregnant women of Mexican descent.

Zinc intakes of low-income pregnant women of Mexican descent were estimated by the use of 24-hr dietary recalls. Recalls were obtained during the first two trimesters for 344 women and again during the third trimester of pregnancy for 279 of the same women. The daily mean zinc intake was calculated as 9.4 +/- 3.8 mg during the first two trimesters and as 10.0 +/- 4.3 mg during the third trimester. For about 85% of the women, the reported intakes were below two-thirds of the Recommended Dietary Allowance for zinc. In a subsample of the women, no significant correlation was shown between low dietary zinc intakes and low serum zinc levels during either early or late pregnancy. Zinc and protein intakes were highly correlated in both early and late pregnancy (r = 0.83 and 0.89, respectively). Diets that provided 90 to 100 g of protein (about 125% of the Recommended Dietary Allowance) provided a mean of 13.6 g of zinc (67% of the Recommended Dietary Allowance).

Apoenzyme activities of erythrocyte transketolase, glutathione reductase, and glutamic-pyruvic transaminase during pregnancy.

The erythrocyte apoenzyme activities of transketolase, glutathione reductase, and glutamic-pyruvic transaminase were determined in 236 pregnant women during the first or second trimester and again during the third trimester. There were no differences in erythrocyte glutathione reductase and erythrocyte glutamic-pyruvic transaminase activities during these two periods. In contrast, erythrocyte transketolase decreased significantly in the third trimester. No statistically significant correlations were found between levels of activity for the various enzymes and dietary intakes of protein, vitamins or calories. The percent of subjects with low erythrocyte transketolase activity (a value one standard deviation or more below the mean initial value) increased significantly during the third trimester. The percent of subjects with low erythrocyte glutamic-pyruvic transaminase activity was significantly reduced during the third trimester although the mean apoenzyme level did not change. Vitamin deficiencies as measured by enzyme stimulation tests tended to occur less frequently among subjects with low enzyme activities but in no instance was there a statistically significant difference. Hence, no association could be found between apoenzyme activity and the incidence of vitamin deficiencies.

Biochemical assessment of the nutritional status of low-income pregnant women of Mexican descent.

Biochemical parameters of nutritional status were investigated in 300 women of Mexican descent during the first and second trimesters of pregnancy. Blood samples were obtained from the women during clinic visits, and measurements were made of serum iron, hemoglobin, hematocrit, serum protein, and transferrin saturation. Additionally, the nutritional status of seven vitamins was determined either by direct assay of the vitamin levels in blood or by measurement of erythrocyte enzyme stimulation. Thiamin and riboflavin were also determined in causal urine samples. Very few women, 8% or less, were classified as being low or deficient in hemoglobin, serum protein, iron, transferrin saturation, vitamin C, carotene, vitamin A, or vitamin B12. Thirty-one percent had low or deficient hematocrit values according to the guidelines used. Folic acid was the most prevalent vitamin deficiency, with 69% of the women having low or deficient serum levels. Based on the erythrocyte enzyme stimulation tests, 22% of the women were low or deficient in thiamin, 29% were low or deficient in riboflavin, and 9% were deficient in pyridoxine. None of the women had a low urinary excretion of thiamin, but 8% had excretion values of riboflavin below the acceptable level. Fewer deficiencies of thiamin and serum folic acid were observed in women taking vitamin and mineral supplements than in those who were not.

Vitamin B-6 nutriture and plasma diamine oxidase activity in pregnant Hispanic teenagers.

Vitamin B-6 status was assessed by measuring erythrocyte glutamic-pyruvic transaminase (EGPT) indices in 122 pregnant Hispanic teenagers. Seventeen percent were vitamin B-6 deficient (EGPT indices greater than 1.25) at the initial interview (first or second trimester). A daily supplement of 5 mg vitamin B-6, beginning at initial interview, did not reduce prevalence of vitamin B-6 deficiency at final interview (third trimester). No association was found between EGPT indices greater than 1.25 and the outcome of pregnancy. The activity of diamine oxidase (DAO), a vitamin B-6-dependent enzyme produced by the placental decidua, was measured in maternal plasma. At initial and final interviews, plasma-DAO activity was increased by in vitro addition of pyridoxal-5'-phosphate. The activity in early pregnancy was positively associated with dietary vitamin B-6 intake and was lower in teenagers with EGPT indices greater than 1.25 at the final interview. Findings suggest that plasma-DAO activity is influenced by vitamin B-6 status.