Family physician involvement in cancer care follow-up: the experience of a cohort of patients with lung cancer.

PURPOSE There has been little research describing the involvement of family physicians in the follow up of patients with cancer especially during the primary treatment phase We undertook a prospective longitudinal study of patients with lung cancer to assess their family physician s involvement in their follow up at the different phases of cancer METHODS In 5 hospitals in the province of Quebec Canada patients with a recent diagnosis of lung cancer were surveyed every 3 to 6 months whether they had metastasis or not, for a maximum of 18 months to assess aspects of their family physician s involvement in cancer care RESULTS Of the 395 participating patients 92% had a regular family physician but only 60% had been referred to a specialist by him/her or a colleague for the diagnosis of their lung cancer A majority of patients identified the oncology team or oncologists as mainly responsible for their cancer care throughout their cancer journey except at the advanced phase where a majority attributed this role to their family physician At baseline only 16% of patients perceived a shared care pattern between their family physician and oncologists but this pro portion increased with cancer progression Most patients would have liked their family physician to be more involved in all aspects of cancer care CONCLUSIONS Although patients perceive that the oncology team is the main party responsible for the follow up of their lung cancer they also wish their family physicians to be involved Better communication and collaboration between family physicians and the oncology team are needed to facilitate shared care in cancer follow up

Management of severe pediatric subglottic stenosis with glottic involvement.

OBJECTIVE: We sought to describe our experience in the management of complex glotto-subglottic stenosis in the pediatric age group. METHODS: Between 1978 and 2008, 33 children with glotto-subglottic stenosis underwent partial cricotracheal resection, and they form the focus of this study. They were compared with 67 children with isolated subglottic stenosis (no glottic involvement). The outcomes measured were need for revision open surgical intervention, delayed decannulation (>6 months), and operation-specific and overall decannulation rates. Fisher's exact test was used for comparison of outcomes. RESULTS: Results of preoperative evaluation showed Myer-Cotton grade III or IV stenosis in 32 (97%) patients and grade II stenosis in 1 patient. All patients with glotto-subglottic stenosis were treated with partial cricotracheal resection and simultaneous repair of the glottic pathology. Bilateral fixed vocal cords were seen in 19 (58%) of 33 patients, bilateral restricted abduction was seen in 7 (21%) of 33 patients, and unilateral fixed vocal cord was seen in 7 (21%) of 33 patients. Ten patients underwent single-stage partial cricotracheal resection with excision of interarytenoid scar tissue. The endotracheal tube was kept for a mean period of 7 days as a stent. Twenty-three patients underwent extended partial cricotracheal resection with LT-Mold (Bredam S.A., St. Sulpice, Switzerland) or T-tube stenting. The overall decannulation rate included 26 (79%) patients, and the operation-specific decannulation rate included 20 (61%) patients. CONCLUSIONS: Glotto-subglottic stenosis is a complex laryngeal injury associated with delayed decannulation and decreased overall and operation-specific decannulation rates when compared with those after subglottic stenosis without glottic involvement after partial cricotracheal resection.

Patients' preferences on information and involvement in decision making for gastrointestinal surgery.

BACKGROUND: The relationship between physicians and patients has undergone important changes, and the current emancipation of patients has led to a real partnership in medical decision making. The present study aimed to assess patients' preferences on different aspects of decision making during treatment and potential complications, as well as the amount and type of preoperative information wanted before visceral surgery. METHODS: This was a prospective non-randomized study based on a questionnaire given to 253 consecutive patients scheduled for elective gastrointestinal surgery. RESULTS: In considering surgical complications or treatment in the intensive care unit, 64 % of patients wished to take an active role in any medical decisions. The respective figures for cardiac resuscitation and treatment limitations were 89 and 60 %. As for information, 73, 77, and 47 % of patients wish detailed information, information on a potential ICU hospitalization, and knowledge of cardiac resuscitation, respectively. Elderly and low-educated patients were significantly less interested in shared medical decision making (p = 0.003 and 0.015), and in receiving information (p = 0.03 and 0.05). Similarly, involvement of the family in decision making was significantly less important to elderly and male patients (p = 0.05 and 0.03, respectively). Neither the type of operation (minor or major) nor the severity of disease (malignancies versus non-malignancies) was a significant factor for shared decision making, information, or family involvement. CONCLUSIONS: The vast majority of surgical patients clearly want to get adequate preoperative information about their disease and the planned treatment. They also consider it crucial to be involved in any kind of decision making for treatment and complications. For most patients, the family role is limited to supporting the treating physicians if the patient is unable to participate in decision making.

Congenital disorder of glycosylation type Id (CDG Id): phenotypic, biochemical and molecular characterization of a new patient.

Congenital disorders of glycosylation (CDG) are a family of multisystem inherited disorders caused by defects in the biosynthesis of N- or O-glycans. Among the many different subtypes of CDG, the defect of a mannosyltransferase encoded by the human ALG3 gene (chromosome 3q27) is known to cause CDG Id. Six patients with CDG Id have been described in the literature so far. We further delineate the clinical, biochemical, neuroradiological and molecular features of CDG Id by reporting an additional patient bearing a novel missense mutation in the ALG3 gene. All patients with CDG Id display a slowly progressive encephalopathy with microcephaly, severe psychomotor retardation and epileptic seizures. They also share some typical dysmorphic features but they do not present the multisystem involvement observed in other CDG syndromes or any biological marker abnormalities. Unusually marked osteopenia is a feature in some patients and may remain undiagnosed until revealed by pathological fractures. Serum transferrin screening for CDG should be extended to all patients with encephalopathy of unknown origin, even in the absence of multisystem involvement.

Food-dependent Cushing's syndrome: possible involvement of leptin in cortisol hypersecretion.

Stimulation ofcortisol secretion by food intake has been implicated in the pathogenesis of some cases of ACTH-independent Cushing's syndrome, via an aberrant response of the adrenal glands to gastric inhibitory polypeptide (GIP). We report here a novel case of food-dependent Cushing's syndrome in a patient with bilateral macronodular adrenal hyperplasia. In this patient we were able to confirm a paradoxical stimulation of cortisol secretion by GIP in vivo as well as in vitro on dispersed tumor adrenal cells obtained at surgery. In addition to GIP, in vitro stimulation of these cultured tumor adrenal cells with leptin, the secreted product of the adipocyte, induced cortisol secretion. By comparison, no such stimulation was observed in vitro in adrenal cells obtained from another patient with bilateral macronodular adrenal hyperplasia and Cushing's syndrome that did not depend on food intake, in tumor cells obtained from a solitary cortisol-secreting adrenal adenoma, and in normal human adrenocortical cells. These results demonstrate that as in previously described cases of food-dependent Cushing's syndrome, GIP stimulated cortisol secretion from the adrenals of the patient reported here. Therefore, they indicate that such a paradoxical response probably represents the hallmark of this rare condition. In addition, they suggest that leptin, which normally inhibits stimulated cortisol secretion in humans, participated in cortisol hypersecretion in this case. Further studies in other cases of food-dependent Cushing's syndrome, however, will be necessary to better ascertain the pathophysiological significance of this finding.

Involvement of family physicians in structured programs for chronic diseases or multi-morbidity in Switzerland.

The increasing prevalence of chronic diseases and multi-morbidity represents challenges for health systems worldwide. In that perspective, the current organization of healthcare delivery, fragmentation of care, limited use of evidence-based guidelines and patients'insufficient empowerment are some reasons explaining the current limited effectiveness of the management of chronically ill patients. Based on theoretical models such as the Chronic Care Model (CCM), initiatives targeting improvements in the care of patients with chronic diseases have been implemented worldwide since more than a decade. Their development in Switzerland, a health system where more than half of practices are still single handed [6], is only recent and infrequent. Structured programs for patients with chronic diseases or multimorbidity usually propose patient-centered interventions and consider an integrative multidisciplinary approach. Currently, little is known on the existence of such programs and on the role of family physicians (FPs)within these programs, in Switzerland. The objective of this study was to identify and describe current structured programs targeting chronic diseases or multi-morbidity in Switzerland. This may help in examining innovative approaches that are only developed locally but would deserve wider interest for further implementation. We conducted a telephone-based survey between June and November 2013 and contacted systematically key institutions, informants and stakeholders nationwide and in the 26 cantons...

Patients' preferences on information and involvement in decision-making in gastrointestinal surgery

Contexte et but de l'étude: La relation médecin-patient a subi d'importants changements et l'actuelle émancipation des patients a conduit à un véritable partenariat dans la prise de décisions thérapeutiques. Notre étude a pour but de déterminer les préférences des patients pour différents aspects de la prise de décisions au cours d'un traitement et de ses potentielles complications, de même que la quantité et le type d'information souhaitée avant une intervention chirurgicale digestive. Patients et méthodes : Il s'agit d'une étude prospective non-randomisée basée sur un questionnaire donné lors de la consultation préopératoire à 254 patients consécutifs prévus pour une chirurgie gastro-intestinale élective. Résultats : Pour les potentielles complications chirurgicales et la possibilité d'un séjour aux soins intensifs, 64% des patients souhaitent participer activement aux décisions médicales, et respectivement 89% et 60% des patients aimeraient discuter d'une éventuelle réanimation cardio-pulmonaire et de limitations au traitement. Respectivement 73%, 77% et 47% des patients ont souhaité une information très détaillée, une infoimation pour une possible hospitalisation en soins intensifs ou une éventuelle réanimation cardiaque. Les patients âgés ou avec un niveau de formation bas étaient significativement moins intéressés à une prise de décision partagée (p=0.003 et 0.015) et à une information complète (p=0.03 et 0.05), De plus, l'implication des familles dans les prises de décision n'était favorisée que si le patient est en coma (74%), et significativement moins importante chez les personnes âgées et de sexe masculin (p=0.04 et 0.03 respectivement). Ni le type de chirurgie prévue (majeure ou mineure) ni la sévérité de la pathologie (cancer ou non) ne furent des facteurs statistiquement significatifs pour un désir plus élevé de partager la prise de décision, pour plus d'information ou pour impliquer d'avantage la famille. Conclusions : Notre étude démontre que la majorité des patients chirurgicaux souhaitent recevoir une information préopératoire complète concernant leur maladie et le traitement planifié. Ils considèrent également comme crucial d'être impliqués dans les prises de décisions thérapeutiques pour le traitement et pour les possibles complications. Le rôle de la famille est limité aux situations ou le patient n'est plus en mesure de participer aux décisions.

Oral implant rehabilitation in a patient with Moebius syndrome

Introduction: Moebius syndrome is a rare congenital disorder characterized by unilateral or bilateral involvement of the sixth and seventh cranial nerves, resulting in a lack of facial expression and eye movements. These patients suffer a series of oral manifestations that may complicate their dental treatment, such as facial and tongue muscle weakness, uncontrolled salivation secondary to defi cient lip sealing, micrognathia, microstomia, bifi d uvula, gothic and fi ssured palate, fi ssured tongue, and glossoptosis. The underlying etiology remains unclear, though vascular problems during embryogenesis appear to be involved. Clinical case: We report the case of a woman with Moebius syndrome and total edentulism. Eight years ago she underwent complete oral rehabilitation with the placement of two implants in each dental arch. Discussion: Moebius syndrome has still an unknown etiology, although it is related to disorders during pregnancy. This kind of patient can be rehabilitated using oral implants.

Ventricularisation of the left atrium: a rare cause of left ventricular dysfunction in a patient with mitral valve prolapse

Case: A 11 yo girl with Marfan syndrome was referred to cardiac MR (CMR) to measure the size of her thoracic aorta. She had a typical phenotype with arachnodactyly, abnormally long arms, and was tall and slim (156 cm, 28 kg, body mass index 11,5 kg/m2). She complained of no symptoms. Cardiac auscultation revealed a prominent mid-systolic click and an end-systolic murmur at the apex. A recent echocardiogram showed a moderately dilated left ventricle with normal function and a mitral valve prolapse with moderate mitral valve regurgitation. CMR showed a dilatation of the aortic root (38 mm, Z-score 8.9) and a severe prolapse of the mitral valve with regurgitation. The ventricular cavity was moderately dilated (116 ml/m2) and its contraction was hyperdynamic (stroke volume (SV): 97 ml; LVEF 72%, with the LV volumes measured by modified Simpson method from the apex to the mitral annulus). In this patient however, the mitral prolapse was characterized by a severe backward movement of the valve toward the left atrium (LA) in systole and the dyskinetic movement of the atrioventricular plane caused a ventricularisation of a part of the LA in systole (Figure). This resulted in a significant reduction of LVEF: more than ¼ of the apparent SV was displaced backwards into the ventricularized LA volume, reducing the effective LVEF to 51% (effective SV 69ml). Moreover, by flow measurement, the SV across the ascending aorta was 30 ml (cardiac index 2.0 l/min/m2) allowing the calculation of a regurgitant fraction across the mitral valve of 56%, which was diagnostic for a severe mitral valve insufficiency. Conclusion: This case illustrates the phenomenon of a ventricularisation of the LA where the severe prolapse gives the illusion of a higher attachement of the mitral leaflets within the atrial wall. Besides the severe mitral regurgitation, this paradoxical backwards movement of the valve causes an intraventricular unloading during systole reducing the apparent LVEF of 72% to an effective LVEF of only 51%. In addition, forward flow fraction is only 22% after accounting for the regurgitant volume, as well. This combined involvement of the mitral valve could explain the discrepancy between a low output state and an apparently hyperdynamic LV contraction. Due to its ability to precisely measure flows and volumes, CMR is particularly suited to detect this phenomenon and to quantify its impact on the LV pump function.

Patient-centered Care in Maternity Services: A Critical Appraisal and Synthesis of the Literature.

BACKGROUND: Patient-centered care (PCC) has been recognized as a marker of quality in health service delivery. In policy documents, PCC is often used interchangeably with other models of care. There is a wide literature about PCC, but there is a lack of evidence about which model is the most appropriate for maternity services specifically. AIM: We sought to identify and critically appraise the literature to identify which definition of PCC is most relevant for maternity services. METHODS: The four-step approach used to identify definitions of PCC was to 1) search electronic databases using key terms (1995-2011), 2) cross-reference key papers, 3) search of specific journals, and 4) search the grey literature. Four papers and two books met our inclusion criteria. ANALYSIS: A four-criteria critical appraisal tool developed for the review was used to appraise the papers and books. MAIN RESULTS: From the six identified definitions, the Shaller's definition met the majority of the four criteria outlined and seems to be the most relevant to maternity services because it includes physiologic conditions as well as pathology, psychological aspects, a nonmedical approach to care, the greater involvement of family and friends, and strategies to implement PCC. CONCLUSION: This review highlights Shaller's definitions of PCC as the one that would be the most inclusive of all women using maternity services. Future research should concentrate on evaluating programs that support PCC in maternity services, and testing/validating this model of care.

Magnetic resonance imaging and BMB score in the evaluation of bone involvement in Gaucher’s disease patients

AbstractObjective:To evaluate by magnetic resonance imaging changes in bone marrow of patients undergoing treatment for type I Gaucher’s disease.Materials and Methods:Descriptive, cross-sectional study of Gaucher’s disease patients submitted to 3 T magnetic resonance imaging of femurs and lumbar spine. The images were blindly reviewed and the findings were classified according to the semiquantitative bone marrow burden (BMB) scoring system.Results:All of the seven evaluated patients (three men and four women) presented signs of bone marrow infiltration. Osteonecrosis of the femoral head was found in three patients, Erlenmeyer flask deformity in five, and no patient had vertebral body collapse. The mean BMB score was 11, ranging from 9 to 14.Conclusion:Magnetic resonance imaging is currently the method of choice for assessing bone involvement in Gaucher’s disease in adults due to its high sensitivity to detect both focal and diffuse bone marrow changes, and the BMB score is a simplified method for semiquantitative analysis, without depending on advanced sequences or sophisticated hardware, allowing for the classification of the disease extent and assisting in the treatment monitoring.

Personal Liberty in Psychiatric Care - Towards Service User Involvement

This study explores personal liberty in psychiatric care from a service user involvement perspective. The data were collected in four phases during the period 2000-2006 in psychiatric settings in Finland. Firstly, patient satisfaction and factors associated with user involvement were studied (n = 313). Secondly, patients’ experiences of deprivation of their liberty were explored (n = 51). Thirdly, an overview on patients’ options for lodging complaints was conducted, and all complaints (n = 4645) lodged in Finland from 2000 to 2004 were examined. Fourthly, the effects of different patient education methods on inpatients’ experiences of deprivation of liberty were tested (n = 311). It emerged that patients were quite satisfied, but reported dissatisfaction in restrictions, compulsory care and information dissemination. Patients experienced restrictions on leaving the ward and on communication, confiscation of property and coercive measures as deprivation of liberty. Patients’ experienced these interventions to be negative. In Finland, the patient complaint process is complicated and not easily accessible. In general, patient complaints increased considerably in Finland during the study period. In psychiatric care the number of complaints was quite stable and complaints led more seldom to consequences. An Internet-based patient education system was equivalent with traditional education and treatment as usual in supporting personal liberty during hospital care. This dissertation provides new information about the realization of patients' rights in psychiatric care. In order to improve patients' involvement, systematic methods to increase personal liberty during care need to be developed, the procedures for patients lodging complaints should be simplified, and patients' access to information needs to be ensured using multiple methods.

Montreal classification of patient operated for Crohn's disease and identification of surgical recurrence predictors

OBJECTIVE: to evaluate Crohn's disease recurrence and its possible predictors in patients undergoing surgical treatment. METHODS: We conducted a retrospective study with Crohn's disease (CD) patients undergoing surgical treatment between January 1992 and January 2012, and regularly monitored at the Bowel Clinic of the Hospital das Clínicas of the UFMG. RESULTS: we evaluated 125 patients, 50.4% female, with a mean age of 46.12 years, the majority (63.2%) diagnosed between 17 and 40 years of age. The ileum was involved in 58.4%, whereas stenotic behavior was observed in 44.8%, and penetrating, in 45.6%. We observed perianal disease in 26.4% of cases. The follow-up average was 152.40 months. Surgical relapse occurred in 29.6%, with a median time of 68 months from the first operation. CONCLUSION: The ileocolic location, penetrating behavior and perianal involvement (L3B3p) were associated with increased risk of surgical recurrence.

Measurement of epidermal thickness in a patient with psoriasis by computer-supported image analysis

The aim of the present study was to measure full epidermal thickness, stratum corneum thickness, rete length, dermal papilla widening and suprapapillary epidermal thickness in psoriasis patients using a light microscope and computer-supported image analysis. The data obtained were analyzed in terms of patient age, type of psoriasis, total body surface area involvement, scalp and nail involvement, duration of psoriasis, and family history of the disease. The study was conducted on 64 patients and 57 controls whose skin biopsies were examined by light microscopy. The acquired microscopic images were transferred to a computer and measurements were made using image analysis. The skin biopsies, taken from different body areas, were examined for different parameters such as epidermal, corneal and suprapapillary epidermal thickness. The most prominent increase in thickness was detected in the palmar region. Corneal thickness was more pronounced in patients with scalp involvement than in patients without scalp involvement (t = -2.651, P = 0.008). The most prominent increase in rete length was observed in the knees (median: 491 µm, t = 10.117, P = 0.000). The difference in rete length between patients with a positive and a negative family history was significant (t = -3.334, P = 0.03), being 27% greater in psoriasis patients without a family history. The differences in dermal papilla distances among patients were very small. We conclude that microscope-supported thickness measurements provide objective results.

Craniometaphyseal Dysplasia With Severe Craniofacial Involvement Shows Homozygosity at 6q21-22.1 Locus

Craniotubular dysplasias (CTD) are a heterogeneous group of genetic disorders of skeletal development, whose clinical and etiological classification is still much debated. One of the most common form is the autosomal dominant craniometaphyseal dysplasia (CMD) which is associated with mutation in the ANKH gene. In the literature a few families are reported with CMD phenotype that suggest an autosomal recessive (AR) pattern of inheritance. A candidate locus at 6q21-22 has been mapped in a large inbred Brazilian family, but the gene of the recessive form is still unknown. Our data on a female patient with CMD phenotype, born from healthy first degree cousins and displaying homozygosity for polymorphic markers at the 6q21-22 locus, further support the existence of an AR CMD, expanding its clinical spectrum to a more severe phenotype. (C) 2011 Wiley-Liss, Inc.