scholarly journals Care pathway for fetal joint contractures, Fetal Akinesia Deformation Sequence and Arthrogryposis Multiplex Congenita.

Author(s):  
Jill K. Tjon ◽  
Maria B. Tan-Sindhunata ◽  
Marianna Bugiani ◽  
Melinda M.E.H. Witbreuk ◽  
Johannes A. van der Sluijs ◽  
...  

Introduction The majority of arthrogryposis multiplex congenita (AMC) and lethal forms of AMC such as fetal akinesia deformation sequence (FADS) cases are missed prenatally. We have demonstrated the additional value of fetal motor assessment and evaluation in a multidisciplinary team for the period 2007-2016. An applied care pathway for fetuses presenting with joint contracture(s) in one anatomic region (e.g. talipes equinovares), more than one body part with non-progressive contractures and motility (AMC) and with deterioration over time (FADS). Methods The multidisciplinary team of Amsterdam University Medical Centre Expertise Centre FADS and AMC developed the care pathway. Additional tools are provided including a motor assessment by ultrasound examination and postmortem assessment form. Results An eight-step care pathway is presented with a proposed timing for prenatal sonographic examination, genetic examinations, multidisciplinary meetings, prenatal and postnatal counselling of the parents by specialist also treating after birth, follow-up of prenatal and postnatal findings with counselling for future pregnancies. Discussion/conclusion The scheduled serial structural and motor sonograpahic assessment together with follow-up examinations and genetic analysis, should be tailored per prenatal centre per available resources. The multidisciplinary care pathway may pave the way to increase detection rate and diagnosis of isolated contracture(s), talipes equinovares with underlying genetic causes and the rare phenotypes AMC/FADS and prompt treatment after birth within expertise teams.

2019 ◽  
Vol 39 (3) ◽  
pp. 219-231 ◽  
Author(s):  
Jill K. Tjon ◽  
Gita M. Tan-Sindhunata ◽  
Marianna Bugiani ◽  
Melinda M. Witbreuk ◽  
Johannes A. van der Sluijs ◽  
...  

Author(s):  
C. J. Koppel ◽  
B. W. Driesen ◽  
R. J. de Winter ◽  
A. E. van den Bosch ◽  
R. van Kimmenade ◽  
...  

Abstract Background Current guidelines on coronary anomalies are primarily based on expert consensus and a limited number of trials. A gold standard for diagnosis and a consensus on the treatment strategy in this patient group are lacking, especially for patients with an anomalous origin of a coronary artery from the opposite sinus of Valsalva (ACAOS) with an interarterial course. Aim To provide evidence-substantiated recommendations for diagnostic work-up, treatment and follow-up of patients with anomalous coronary arteries. Methods A clinical care pathway for patients with ACAOS was established by six Dutch centres. Prospectively included patients undergo work-up according to protocol using computed tomography (CT) angiography, ischaemia detection, echocardiography and coronary angiography with intracoronary measurements to assess anatomical and physiological characteristics of the ACAOS. Surgical and functional follow-up results are evaluated by CT angiography, ischaemia detection and a quality-of-life questionnaire. Patient inclusion for the first multicentre study on coronary anomalies in the Netherlands started in 2020 and will continue for at least 3 years with a minimum of 2 years of follow-up. For patients with a right or left coronary artery originating from the pulmonary artery and coronary arteriovenous fistulas a registry is maintained. Results Primary outcomes are: (cardiac) death, myocardial ischaemia attributable to the ACAOS, re-intervention after surgery and intervention after initially conservative treatment. The influence of work-up examinations on treatment choice is also evaluated. Conclusions Structural evidence for the appropriate management of patients with coronary anomalies, especially (interarterial) ACAOS, is lacking. By means of a structured care pathway in a multicentre setting, we aim to provide an evidence-based strategy for the diagnostic evaluation and treatment of this patient group.


Nutrients ◽  
2021 ◽  
Vol 13 (8) ◽  
pp. 2635
Author(s):  
Koen Huysentruyt ◽  
Kim Brunet-Wood ◽  
Robert Bandsma ◽  
Leah Gramlich ◽  
Bonnie Fleming-Carroll ◽  
...  

Background: Disease-associated malnutrition (DAM) is common in hospitalized children. This survey aimed to assess current in-hospital practices for clinical care of pediatric DAM in Canada. Methods: An electronic survey was sent to all 15 tertiary pediatric hospitals in Canada and addressed all pillars of malnutrition care: screening, assessment, treatment, monitoring and follow-up. Results: Responses of 120 health care professionals were used from all 15 hospitals; 57.5% were medical doctors (MDs), 26.7% registered dietitians (RDs) and 15.8% nurses (RNs). An overarching protocol for prevention, detection and intervention of pediatric malnutrition was present or “a work in progress”, according to 9.6% of respondents. Routine nutritional screening on admission was sometimes or always performed, according to 58.8%, although the modality differed among hospitals and profession. For children with poor nutritional status, lack of nutritional follow-up after discharge was reported by 48.5%. Conclusions: The presence of a standardized protocol for the clinical assessment and management of DAM is uncommon in pediatric tertiary care hospitals in Canada. Routine nutritional screening upon admission has not been widely adopted. Moreover, ongoing nutritional care of malnourished children after discharge seems cumbersome. These findings call for the adoption and implementation of a uniform clinical care pathway for malnutrition among pediatric hospitals.


2021 ◽  
Vol 2 (1) ◽  
pp. 75-86
Author(s):  
Maria Irene Bellini ◽  
Vito Cantisani ◽  
Augusto Lauro ◽  
Vito D’Andrea

Living kidney donation represents the best treatment for end stage renal disease patients, with the potentiality to pre-emptively address kidney failure and significantly expand the organ pool. Unfortunately, there is still limited knowledge about this underutilized resource. The present review aims to describe the general principles for the establishment, organization, and oversight of a successful living kidney transplantation program, highlighting recommendation for good practice and the work up of donor selection, in view of potential short- and long-terms risks, as well as the additional value of kidney paired exchange programs. The need for donor registries is also discussed, as well as the importance of lifelong follow up.


Injury ◽  
2021 ◽  
Author(s):  
Elvira R. Flikweert ◽  
Klaus W. Wendt ◽  
Ronald L. Diercks ◽  
Gerbrand J. Izaks ◽  
Roy Stewart ◽  
...  

2010 ◽  
Vol 24 (2) ◽  
pp. 77-82 ◽  
Author(s):  
Tomohiro Ishikita ◽  
Noboru Oriuchi ◽  
Tetsuya Higuchi ◽  
Go Miyashita ◽  
Yukiko Arisaka ◽  
...  

Medicine ◽  
2015 ◽  
Vol 94 (41) ◽  
pp. e1731
Author(s):  
Ming-Hsien Tsai ◽  
Yu-Weil Fang ◽  
Li Hui Wang ◽  
Xiang Gin You ◽  
Jyh-Gang Leu

2021 ◽  
Vol 12 (4) ◽  
pp. 21
Author(s):  
Virna Ribeiro Feitosa Cestari ◽  
Lorena C. De Souza ◽  
Raquel S. Florêncio ◽  
Maria G.V. Sobral ◽  
Vera L.M.P. Pessoa ◽  
...  

Objective: To understand the expectations of the professionals about the construction and use of an educational and follow-up application to care.Methods: Phenomenological and qualitative study. Convenience and purposive sampling were carried out and in-depth individual interviews with 35 professionals from the multidisciplinary team, between September and October 2020 in Brazil. All interviews were audio-recorded and data analyzed using the hermeneutic circle. The COREQ checklist was employed to report on the current study.Results: Two main units of meaning emerged: (a) The care of the person who lives with heart failure; and (b) The care of the person with heart failure intermediated by an application. Care for the person with the disease brings together elements related to the identification of demands and understanding of their surroundings, with guidance and use of technologies.Conclusions: The professionals were favorable to the development of an application and considered it beneficial. The use of it, would allow the approximation between patients and their family and the multidisciplinary team; respect the patient’s needs and overcome the precariousness of the health system.


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