scholarly journals Classification of malnutrition in cystic fibrosis: implications for evaluating and benchmarking clinical practice performance

2008 ◽  
Vol 88 (1) ◽  
pp. 161-166 ◽  
Author(s):  
HuiChuan J Lai ◽  
Suzanne M Shoff
Keyword(s):  
Cystic Fibrosis ◽  
Clinical Practice ◽  
Practice Performance

Case report: a child with cystic fibrosis and phenylketonuria

2020 ◽  
Vol 1 (2) ◽  
pp. 38-44
Author(s):  
Irina V. Vakhlova ◽  
Anastasia D. Kazachina ◽  
Olga A. Beglyanina
Keyword(s):  
Cystic Fibrosis ◽  
Case Report ◽  
Clinical Practice ◽  
Neonatal Screening ◽  
Genetic Disorders ◽  
Epileptiform Activity ◽  
Autism Spectrum ◽  
Speech Development ◽  
Inherited Disorders ◽  
Congenital Diseases

Background. In the international clinical practice there have been occasional reports of phenylketonuria (PKU) and cystic fibrosis (CF) found simultaneously in the same patient. Both PKU and CF are the inherited disorders characterized by autosomal recessive type of inheritance. Currently the combination of two or more inherited disorders in one patient is considered to be a clinical rarity.Case description. This is a clinical case of two genetic disorders, CF and PKU, combined in a 5-year old patient who had been followed up since birth. Owing to implementation of neonatal screening for inherited and congenital diseases into clinical practice, during the first month of life the infant was diagnosed with CF (diagnostically significant elevation of immunoreactive trypsin [IRT] at the initial [163.2 ng/mL] and repeat testing on day 21 of life [138.7 ng/mL]) and PKU (phenylalanine [PA] level 15.9 mg/dL). Both disorders have been confirmed by genetic tests, i.e., homozygous DelF508 mutation was found in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, and P281L mutation in the phenylalanine hydroxylase (PAH) gene was also present in homozygous state. Child’s parents strictly adhered to dietary and treatment recommendations. By the age of 5 years the child developed symptoms of neurological disorder and disorder of the respiratory system, cognitive impairment and delay in speech development, subclinical epileptiform activity with high risk of epilepsy, and chronic inflammation of the respiratory tract.Conclusion. This case report demonstrates the important role of neonatal screening in early diagnosis and timely start of therapy, and underscores the importance of continuous medication in such genetic disorders as CF and PKU. On the whole, such approach brings about a relative preservation of functioning of the most affected organs and systems. By the age of 5 years the child does not form bronchiectases, shows no signs of chronic hypoxia, nutritional deficiency or pronounced neurologic deficit, and is at low risk for the development of autism spectrum disorder. At the same time, the larger scale and longer-term observations are required in order to make the unequivocal conclusions about the prognosis of these diseases under conditions of modern-day medical follow-up.

Diverse Dimensions of the Cotard’s Syndrome - an Approach Towards a Clear Classification of the Phenomenon

2009 ◽  
Vol 24 (S1) ◽  
pp. 1-1
Author(s):  
A. Nagy ◽  
V. Voros ◽  
T. Tenyi
Keyword(s):  
Clinical Practice ◽  
Diagnostic Category ◽  
Treatment Strategies ◽  
Differential Diagnostics ◽  
Dimensional System ◽  
Central Feature ◽  
Scientific Databases ◽  
Search Terms ◽  
The One

Aim:The authors present the Cotard's syndrome, a rare psychiatric condition, pointing out the latest results in terms of psychoneurology and classification of the phenomenon. The central feature of the syndrome is a nihilistic delusion, in which the patient denies his or her own existence and that of the external world.Method:We searched electronic scientific databases using the appropriate search terms; relevant articles were carefully reviewed. We also present three cases from our clinical practice.Results:After the overview of the latest biological and neuropsychological findings, the terminology, the nosology, the classification and the differential diagnostics are discussed. To sum up with useful information for the clinical practice, the possible treatment strategies, the course and the prognosis of the disease are also presented.Conclusions:The reported cases together with the reviewed literature suggest that a dimensional system of classifying Cotard's syndrome is preferable. At the one end of the spectrum is the presence of the pure nihilistic delusions, appearing as a symptom of an underlying psychiatric or neurological condition. The full-blown, classical syndrome as a diagnostic category forms the other end of the spectrum. The presented theoretical and practical aspects give a lead on deeper understanding, easier recognition and more adequate therapy of the Cotard's syndrome.

scholarly journals Recent Development toward the Next Clinical Practice of Primary Aldosteronism: A Literature Review

Biomedicines ◽  
2021 ◽  
Vol 9 (3) ◽  
pp. 310
Author(s):  
Yuta Tezuka ◽  
Yuto Yamazaki ◽  
Yasuhiro Nakamura ◽  
Hironobu Sasano ◽  
Fumitoshi Satoh
Keyword(s):  
Clinical Practice ◽  
Primary Aldosteronism ◽  
Diagnostic Procedures ◽  
Secondary Hypertension ◽  
Refractory Hypertension ◽  
Diagnostic Biomarkers ◽  
Histopathological Classification ◽  
Subtype Differentiation ◽  
Work Up

For the last seven decades, primary aldosteronism (PA) has been gradually recognized as a leading cause of secondary hypertension harboring increased risks of cardiovascular incidents compared to essential hypertension. Clinically, PA consists of two major subtypes, surgically curable and uncurable phenotypes, determined as unilateral or bilateral PA by adrenal venous sampling. In order to further optimize the treatment, surgery or medications, diagnostic procedures from screening to subtype differentiation is indispensable, while in the general clinical practice, the work-up rate is extremely low even in the patients with refractory hypertension because of the time-consuming and labor-intensive nature of the procedures. Therefore, a novel tool to simplify the diagnostic flow has been recently in enormous demand. In this review, we focus on recent progress in the following clinically important topics of PA: prevalence of PA and its subtypes, newly revealed histopathological classification of aldosterone-producing lesions, novel diagnostic biomarkers and prediction scores. More effective strategy to diagnose PA based on better understanding of its epidemiology and pathology should lead to early detection of PA and could decrease the cardiovascular and renal complications of the patients.

You’ve heard about outcome measures, so how do you use them? Integrating clinically relevant outcome measures in orthotic management of stroke

2012 ◽  
Vol 37 (1) ◽  
pp. 30-42 ◽  
Author(s):  
Christopher Robinson ◽  
Stefania Fatone
Keyword(s):  
Clinical Practice ◽  
Outcome Measures ◽  
Careful Consideration ◽  
International Classification Of Functioning ◽  
Common Goal ◽  
Disability And Health ◽  
Healthcare Environment ◽  
Relevant Outcome ◽  
Selection Of

In today’s healthcare environment it is increasingly important to be able to quantify the amount of change associated with a given intervention; this can be accomplished using one or more appropriate outcome measures. However, the selection and integration of outcome measures within clinical practice requires careful consideration. This includes identification of the measure construct which can be assisted by the International Classification of Functioning, Disability, and Health; selection of outcome measures based on need, appropriateness and feasibility; and careful use in regular clinical practice including data collection, analysis and re-assessment of the process. We describe this process, focusing on orthotic management of stroke, in particular the improvement of mobility as a common goal. Clinical relevance The growing emphasis on improved documentation of patient care and outcomes requires that clinicians integrate clinically relevant outcome measures into their practice. We suggest a process to assist clinicians integrate outcome measures into clinical practice with a particular emphasis on the orthotic management of stroke.

scholarly journals Assessment of the Non-Cystic Fibrosis Bronchiectasis Severity: The FACED Score vs the Bronchiectasis Severity Index

2015 ◽  
Vol 9 (1) ◽  
pp. 46-51 ◽  
Author(s):  
J Minov ◽  
J Karadzinska-Bislimovska ◽  
K Vasilevska ◽  
S Stoleski ◽  
D Mijakoski
Keyword(s):  
Body Mass Index ◽  
Cystic Fibrosis ◽  
Clinical Practice ◽  
Observational Study ◽  
Body Mass ◽  
Severity Index ◽  
Sufficient Power ◽  
Chronic Colonization ◽  
The Mean

Introduction : Non-cystic fibrosis bronchiectasis (NCFB) is a multidimensional disease, and no single isolated parameter is proved to have sufficient power for any overall determination of its severity and prognosis. Objective : To compare the results of the assessment of the NCFB severity with respect to its prognosis in the same patients by two different validated scores, i.e. the FACED score and the Bronchiectasis Severity Index (BSI). Methods : An observational study including 37 patients with NCFB (16 males and 21 female aged 46 to 76 years) was performed. All patients underwent evaluation of the variables incorporated in the FACED score (FEV1 % predicted, age, chronic colonization by Pseudomaonas aeruginosa, radiological extent of the disease, and dyspnea) and in the BSI (age, body mass index, FEV1 % predicted, hospitalization and exacerbations in previous year, dyspnea, chronic colonization by Pseudomaonas aeruginosa and other microrganisms, and radiological extent of the disease). Results : According to the value of the derived overall FACED score we found 17 patients (45.9%) with mild bronchiectasis, 14 patients (37.8%) with moderate bronchiectasis and 6 patients (16.2%) with severe bronchiectasis. The mean derived FACED score was 3.4 ± 1.3. In addition, according to the value of the derived overall BSI score, the frequency of patients with low, intermediate and high BSI score was 16 patients (43,2%), 14 patients (37.8%) and 7 patients (18.9%), respectively. The mean derived BSI score was 6.4 ± 2.5. Conclusion : We found similar results by the assessment of the NCFB severity in regard to its prognosis by both the FACED score and the BSI. Further studies determining how these scores may impact clinical practice are needed.

scholarly journals A New Classification for Hemorrhoidal Disease: The Creation of the “BPRST” Staging and Its Application in Clinical Practice

2020 ◽  
Vol 36 (4) ◽  
pp. 249-255 ◽  
Author(s):  
Carlos Walter Sobrado Júnior ◽  
Carlos de Almeida Obregon ◽  
Afonso Henrique da Silva e Sousa Júnior ◽  
Lucas Faraco Sobrado ◽  
Sérgio Carlos Nahas ◽  
...  
Keyword(s):  
Clinical Practice ◽  
Strong Association ◽  
Management Strategies ◽  
Hemorrhoidal Disease ◽  
New Classification ◽  
The Past ◽  
Internal Hemorrhoids ◽  
New Treatment ◽  
Treatment Alternatives

Purpose: Present an updated classification for symptomatic hemorrhoids, which not only guides the treatment of internal hemorrhoids but also the treatment of external components. In addition, this new classification includes new treatment alternatives created over the last few years.Methods: Throughout the past 7 years, the authors developed a method to classify patients with symptomatic hemorrhoids. This study, besides presenting this classification proposal, also retrospectively analyzed 149 consecutive patients treated between March 2011 and November 2013 and aimed to evaluate the association between the management adopted with Goligher classification and our proposed BPRST classification.Results: Both classifications had a statistically significant association with the adopted management strategies. However, the BPRST classification tended to have fewer management discrepancies when each stage of disease was individually analyzed.Conclusion: Although there is much disagreement about how the classification of hemorrhoidal disease should be updated, it is accepted that some kind of revision is needed. The BPRST method showed a strong association with the management that should be adopted for each stage of the disease. Further studies are needed for its validation, but the current results are encouraging.

scholarly journals Antiarrhythmic drugs: mechanisms of action, clinical effects, indications for use

2019 ◽  
Vol 26 (2) ◽  
pp. 76-90
Author(s):  
G. M. Solovyan ◽  
T. V. Mikhalieva
Keyword(s):  
Clinical Practice ◽  
Cardiac Arrhythmias ◽  
Antiarrhythmic Drugs ◽  
Mechanisms Of Action ◽  
Current Evidence ◽  
Clinical Effects ◽  
Trigger Activity ◽  
Impulse Formation ◽  
Indications For Use

The lecture is devoted to one of the most difficult problems of modern cardiology – the use of antiarrhythmic therapy in clinical practice. The basic mechanisms of arrhythmias, aspects of their onset, maintenance and termination are briefly described. The current evidence on the electrophysiological mechanisms of cardiac arrhythmias – re-entry, abnormal impulse formation, and trigger activity – is presented. The article contains information about the remodeling of ion channels properties. The Sicilian gambit is analyzed, in which the mechanisms of arrhythmias are compared to the mechanisms of anti-arrhythmic action of drugs. Classification of anti-arrhythmic drugs, their mechanisms of action, indications and contraindications, side effects, and interaction with other drugs are presented.

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