Updated Treatment Recommendations Research Articles

BCR/ABL-Positive Chronic Myeloid Leukemia in Children: Current Treatment Approach.

The purpose of this review is to summarize the most updated treatment recommendations for pediatric CML, and to discuss current areas of investigation. There is new phase 1 data to support the safety of the non-ATP competitive tyrosine kinase inhibitor (TKI) asciminib in the pediatric cohort. Ongoing studies are investigating the role of treatment-free remission in children. Chronic phase CML in children is managed with lifelong TKI therapy; however, evidence of deeper remissions sustained with second-generation TKIs may permit shorter treatment courses. Use of more specific TKIs may mitigate some of the side effects specific to the pediatric cohort. Children with advanced phase CML should achieve a complete hematologic remission with use of a second-generation TKI prior to transplant to achieve the best outcome.

Surgical Treatment of Scaphoid Fractures: Recommendations for Management

Abstract Background: Several operative treatments exist for scaphoid fractures, varying by approach (e.g., ercutaneous, volar, or dorsal), implant type (e.g., screw or Kirschner wire), and bone raft choice (e.g., none, nonvascularized, or vascularized). Many previous systematic eviews and meta-analyses have investigated outcomes following different surgicalÚpproaches, the use of vascularized versus nonvascularized bone graft for scaphoidßracture nonunions, and treatment for specific fracture patterns. However, given the advancements n scaphoid fracture treatment in recent years, there is a need for updated treatment recommendations hat would be beneficial to hand surgeons. Purpose: We present a comprehensive review of the operative treatment of scaphoid fractures based on recent literature and propose a unified treatment algorithm for managing these fractures. Methods: The English-language literature was searched from 2002 to 2023 for high evidence level (e.g., randomized trials), review, and meta-analysis articles with the following search terms: “scaphoid, ”u8220“scaphoid” AND “nonunion, ” and “scaphoid” AND “malunion. ” Each article was creened by the authors to determine the scaphoid fracture scenario addressed and ubsequent treatment recommendations. The findings from article reviews were then rganized by scaphoid fracture types in this manuscript. Results: A total of 95 pertinent articles were ultimately selected and used as the basis for reviewing different scaphoid fracture scenarios. A treatment algorithm was then proposed based on literature review. Conclusion: This summary of the recent literature can guide hand surgeons in addressing scaphoidßractures. Future research in scaphoid fracture treatment, particularly for nonunions, would be most beneficial n the form of systematic review, meta-analysis, or multicenter prospective randomized clinical trials. Level of Evidence: IV

AMMI Canada Practice Point: Updated recommendations for treatment of adults with symptomatic COVID-19 in 2023-2024.

AMMI Canada Practice Point: Updated recommendations for treatment of adults with symptomatic COVID-19 in 2023-2024.

Lunch Symposium

Pulmonary arterial hypertension (PAH), defined as the presence of artery pulmonary artery pressure > 20 mmHg, pulmonary artery wedge pressure [Formula: see text]15 mmHg, and pulmonary vascular resistance (PVR) > 2Wood units based on expert consensus, is characterized by a progressive and sustained increase in PVR, which may lead to right heart failure and death. PAH is a well-known complication of connective tissue diseases (CTDs), such as systemic sclerosis, systemic lupus erythematosus, Sjögren’s syndrome, and other autoimmune conditions. In the past few years, tremendous progress in the understanding of PAH pathogenesis has been made, with various novel diagnostic and screening methods for the early detection of CTD-PAH proposed worldwide. This symposium would highlight the most updated 2022 ESC/ERS guideline in the disease management of PAH, including latest haemodynmaic definitions of PAH, new risk stratification approach at reassessment and updated PAH treatment recommendations. In addition to the guideline update, important discussion would cover the important diagnostic, and screening algorithm for early detection of CTD-PAH and clinical experiences in managing difficult CTD-PAH cases.

Evaluation of short-acting Beta-2-agonist prescriptions and associated clinical outcomes: Findings from the SABA use IN Asthma (SABINA) study in Asia

BackgroundThe extent of short-acting Beta-2-agonist (β2-agonist) (SABA) use across Asian countries is not well documented. As part of the SABA use IN Asthma (SABINA) III study, we assessed SABA prescriptions and clinical outcomes in patients with asthma from Asia. MethodsThis cross-sectional study recruited patients (aged ≥12 years) with asthma from 8 Asian countries. Data on disease characteristics and asthma treatments were collected using electronic case report forms. Patients were classified by practice type (primary or specialist care) and investigator-defined asthma severity (per Global Initiative for Asthma [GINA] 2017 recommendations). The association of SABA prescriptions with clinical outcomes was analyzed using multivariable regression models. ResultsOverall, 3066 patients were analyzed, with a mean (standard deviation) age of 51.8 (16.7) years; of these patients, 2116 (69%) were female, 2517 (82.1%) had moderate-to-severe asthma and 2498 (81.5%) and 559 (18.2%) were treated in specialist and primary care, respectively. In total, 1423 (46.4%) patients had partly controlled/uncontrolled asthma, with 1149 (37.5%) patients experiencing ≥1 severe asthma exacerbation in the previous year. Overall, 800 (26.7%) patients were prescribed ≥3 SABA canisters in the previous year, which is regarded as overprescription and was associated with a significantly decreased odds of at least partly controlled asthma and increased incidence rates of severe exacerbations (P < 0.01 for both associations). ConclusionThe findings from this cohort of predominantly specialist-treated patients with asthma indicate SABA overprescription in at least 1 in every 4 patients, and this overprescription is associated with poor clinical outcomes. These data highlight the need for adherence to recently updated asthma treatment recommendations in Asia.

Eosinophilic Granulomatosis with Polyangiitis: Latest Findings and Updated Treatment Recommendations.

Eosinophilic granulomatosis with polyangiitis (EGPA) causes necrotizing vasculitis and eosinophil-rich granulomatous inflammation in small- to medium-sized vessels, resulting in multiple organ damage. EGPA is classified as an antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, with myeloperoxidase-ANCA detected in approximately one-third of the patients. Conventional treatment of EGPA relies on systemic glucocorticoids (GCs) in combination with cyclophosphamide when poor prognostic factors are present; however, the dilemma between disease control and drug-related adverse effects has long been a challenge. Recent studies have revealed that the genetic background, pathophysiology, and clinical manifestations differ between ANCA-positive and ANCA-negative patients; however, mepolizumab, an interleukin (IL)-5 inhibitor, is effective in both groups, suggesting that the IL-5-eosinophil axis is deeply involved in the pathogenesis of both ANCA-positive and ANCA-negative EGPA. This review summarizes the latest knowledge on the pathophysiology of EGPA and focuses on the roles of eosinophils and ANCA. We then introduce the current treatment recommendations and accumulated evidence for mepolizumab on EGPA. Based on current unmet clinical needs, we discuss potential future therapeutic strategies for EGPA.

Treatment of Early Breast Cancer: The 18th St.Gallen International Breast Cancer Consensus Conference against the Background of Current German Treatment Recommendations.

This year's 18th St.Gallen (SG) consensus conference on the treatment of early breast cancer (SGBCC: St.Gallen International Breast Cancer Conference) focused on practice-oriented questions. The individual situation and risk-benefit assessment were discussed in great detail. As in previous years, a German working group of leading breast cancer experts presented the results of the international SGBCC2023 against the background of German treatment recommendations - especially the updated treatment recommendations of the Arbeitsgemeinschaft Gynäkologische Onkologie e. V. (AGO) - for everyday clinical practice in Germany. The German treatment recommendations of AGO are based on the current evidence. The comparison with the clinical approach in Germany has proven useful, as the SGBCC panel consists of experts from different countries and disciplines. That is why country-specific characteristics can be incorporated into the SGBCC recommendations.

Korean Medicine Clinical Practice Guideline Update for Temporomandibular Disorders: An Evidence-Based Approach.

Many updated clinical research results have been published since the Korean Medicine Clinical Practice Guidelines (KMCPGs) for temporomandibular disorders (TMDs) were published in 2018. Therefore, it is necessary to update the existing clinical practice guidelines (CPGs). This study presents updated recommendations for TMD treatment based on current research data published up to February 2020. The draft version of the level of evidence and grade of recommendation was determined through an assessment of the risk of bias and a meta-analysis of selected literature based on the Grading of Recommendations Assessment, Development, and Evaluation (GRADE). The final guidelines were derived using the Delphi method. Eighteen recommendations were derived for eight items of Korean medicine treatment for TMDs. Compared with previous Korean medicine CPGs for TMDs, the grades of seven recommendations, including acupuncture, pharmacopuncture, and Chuna manual therapy, were increased. The grades of the two recommendations have been changed. Six new recommendations were added to fully reflect clinical reality. Acupuncture, pharmacopuncture, and Chuna manual therapy are recommended for TMD patients in clinical practice. Concurrent conventional conservative therapy with Korean medicine or a combination of Korean medicines should be considered in clinical practice in patients with temporomandibular disorders.

Hepatitis C Guidance 2023 Update: AASLD-IDSA Recommendations for Testing, Managing, and Treating Hepatitis C Virus Infection.

The Infectious Diseases Society of America and the American Association for the Study of Liver Diseases have collaboratively developed evidence-based guidance regarding the diagnosis, management, and treatment of hepatitis C virus (HCV) infection since 2013. A panel of clinicians and investigators with extensive infectious diseases or hepatology expertise specific to HCV infection periodically review evidence from the field and update existing recommendations or introduce new recommendations as evidence warrants. This update focuses on changes to the guidance since the previous 2020 published update, including ongoing emphasis on recommended universal screening; management recommendations for incomplete treatment adherence; expanded eligibility for simplified chronic HCV infection treatment in adults with minimal monitoring; updated treatment and retreatment recommendations for children as young as 3 years old; management and treatment recommendations in the transplantation setting; and screening, treatment, and management recommendations for unique and key populations.

An update on multimodal management of craniopharyngioma in children.

Craniopharyngioma (CP) represent 1.2-4.6% of all intracranial tumors in children and carry a significant morbidity due to their lesional intimacy with structures involved in neurological, visual, and endocrinological functions. Variable treatment modalities being available, including surgery, radiation therapy, alternative surgeries, and intracystic therapies or combinations of them, their common goal is to reduce immediate and long-term morbidity while preserving these functions. Multiple attempts have been made to re-evaluate surgical and irradiation strategies in order to optimize their complication and morbidity profile. However, despite significant advances in "function sparing" approaches, such as limited surgery and improved technologies of radiation therapies, achieving interdisciplinary consensus on the optimal treatment algorithm remains a challenge. Furthermore, there remains a significant span of improvement given the number of specialties involved as well as the complex and chronic nature of CP disease. This perspective article aims to summarize recent changes and knowledge gains in the field of pediatric CP, outlining updated treatment recommendations, a concept of integrative interdisciplinary care and the implication of novel potential diagnostic tools. A comprehensive update on the multimodal treatment of pediatric CP is presented, focusing on "function-preserving" therapies and their implications.

COPD: what GPNs need to know from the latest GOLD update

Every year, the Global Initiative for Obstructive Lung Disease publishes its update on COPD. Beverley Bostock provides an overview of what the latest publication means for general practice nurses The latest guidance from the Global Initiative for Obstructive Lung Disease (GOLD) on chronic obstructive pulmonary disease (COPD) will have an impact on the management of patients in general practice. The definition of COPD has been updated as well as the recommended approach to diagnosis. Updated treatment recommendations act as an important reminder for primary care clinicians to implement interventions that are relevant in the community setting and to refer on for more specialised interventions in secondary care as appropriate. The replacement of the ABCD assessment tool with the ABE approach underlines the significant impact that exacerbations can have in the management of COPD, independent of the symptom burden. Updated sections on exacerbations remind clinicians of the importance of considering comorbid or new diagnoses as the cause of any symptoms.

Ups and downs of hypertension control in Canada: critical factors and lessons learned.

ABSTRACTAs the leading risk for death, population control of increased blood pressure represents a major challenge for all countries of the Americas. In the early 1990’s, Canada had a hypertension control rate of 13%. The control rate increased to 68% in 2010, accompanied by a sharp decline in cardiovascular disease. The unprecedented improvement in hypertension control started around the year 2000 when a comprehensive program to implement annually updated hypertension treatment recommendations started. The program included a comprehensive monitoring system for hypertension control. After 2011, there was a marked decrease in emphasis on implementation and evaluation and the hypertension control rate declined, driven by a reduction in control in women from 69% to 49%. A coalition of health and scientific organizations formed in 2011 with a priority to develop advocacy positions for dietary policies to prevent and control hypertension. By 2015, the positions were adopted by most federal political parties, but implementation has been slow.This manuscript reviews key success factors and learnings. Some key success factors included having broad representation on the program steering committee, multidisciplinary engagement with substantive primary care involvement, unbiased up to date credible recommendations, development and active adaptation of education resources based on field experience, extensive implementation of primary care resources, annual review of the program and hypertension indicators and developing and emphasizing the few interventions important for hypertension control. Learnings included the need for having strong national and provincial government engagement and support, and retaining primary care organizations and clinicians in the implementation and evaluation.

72 weeks post-partum follow-up of dolutegravir versus efavirenz initiated in late pregnancy (DolPHIN-2): an open-label, randomised controlled study

Late initiation of antiretrovirals in pregnancy is associated with increased risk of perinatal transmission and higher infant mortality. We report the final 72-week postpartum results for efficacy and safety of dolutegravir-based compared with efavirenz-based regimens in mothers and infants. DolPHIN-2 was a randomised, open-label trial. Pregnant women in South Africa and Uganda aged at least 18 years, with untreated but confirmed HIV infection and an estimated gestation of at least 28 weeks, initiating antiretroviral therapy in third trimester were eligible for inclusion. Eligible women were randomly assigned (1:1) to receive either dolutegravir-based (50 mg dolutegravir, 300 mg tenofovir disoproxil fumarate, and either 200 mg emtricitabine in South Africa or 300 mg lamivudine in Uganda) or efavirenz-based (fixed dose combination 600 mg tenofovir disoproxil fumarate plus either emtricitabine in South Africa or lamivudine in Uganda) therapy. The primary efficacy outcome was the time to a viral load of less than 50 copies per mL measured at 6, 12, 24, 48, and 72 weeks postpartum with a Cox model adjusting for viral load and CD4 cell count. Safety endpoints were summarised by the number of women and infants with events. This trial is registered with ClinicalTrials.gov, NCT03249181. Between Jan 23 and Aug 15, 2018, 280 women were screened for inclusion, of whom 268 (96%) women were randomly assigned: 133 (50%) to the efavirenz group and 135 (50%) to the dolutegravir group. 250 (93%; 125 [50%] in the efavirenz group and 125 [50%] in the dolutegravir group) women were included in the intention-to-treat analysis of efficacy. Median time to viral load of less than 50 copies per mL was 4·1 weeks (IQR 4·0-5·1) in the dolutegravir group compared with 12·1 weeks (10·7-13·3) in the efavirenz group (adjusted hazard ratio [HR] 1·93 [95% CI 1·5-2·5]). At 72 weeks postpartum, 116 (93%) mothers in the dolutegravir group and 114 (91%) in the efavirenz group had a viral load of less than 50 copies per mL. Of 57 (21%) mothers with a severe adverse event, three (2%) in the dolutegravir group and five (4%) in the efavirenz group were related to the drug (dolutegravir drug-related events were one woman each with suicidal ideation, suicide attempt, herpes zoster meningitis; efavirenz drug-related events were one woman each with suicide attempt and liver cirrhosis, and three people with drug-induced liver injury). Of 136 (56%) infants in whom severe adverse events were recorded, none were related to the study drugs. In addition to the three infant HIV infections detected at birth in the dolutegravir group that have been previously reported, an additional transmission in the efavirenz group occurred during breastfeeding despite optimal maternal viral suppression and serial negative infant tests in the first year of life. Dolutegravir was safe and well tolerated, supporting updated WHO treatment recommendations in pregnant and breastfeeding women. Infant HIV transmissions can occur during breastfeeding despite persistently undetectable maternal viral load highlighting the need for continued infant testing. Unitaid.

Current Perspectives on Nucleos(t)ide Analogue Therapy for the Long-Term Treatment of Hepatitis B Virus.

The hepatitis B virus (HBV) infection remains a global public health problem. This review presents updated recommendations for the optimal current treatment of choice with nucleos(t)ide analogues (NA). Current clinical practice guidelines on the management of chronic hepatitis B (CHB) by the Asian Pacific Association for the Study of the Liver, the European Association for the Study of the Liver and the American Association for the Study of Liver Diseases have been considered. Patients with chronic HBV infection are at increased risk of liver disease progression to cirrhosis and hepatocellular carcinoma (HCC) development. The main goal of therapy is to improve survival preventing disease progression and HCC. The induction of long-term suppression of HBV replication represents the main endpoint of current treatment strategies, while hepatitis B surface antigen (HBsAg) loss is the optimal endpoint. The typical indication for treatment requires elevated HBV desoxyribonucleic acid (DNA), elevated alanine aminotransferase and/or at least moderate histological lesions, while all cirrhotic patients with detectable HBV DNA should be treated. The long-term administration of a potent NA with high barrier to resistance, ie, entecavir, tenofovir disoproxil fumarate or tenofovir alafenamide, represents the treatment of choice. However, HBsAg seroclearance is anecdotal with NA. Treated patients should be monitored for therapy response, adherence, risk of disease progression, and risk of HCC development. This review aims to assess the evolving trends on the potent NA and the new perspectives on finite therapy.

Group for Research and Assessment of Psoriasis and Psoriatic Arthritis (GRAPPA): updated treatment recommendations for psoriatic arthritis 2021.

Since the second version of the Group for Research and Assessment of Psoriasis and Psoriatic Arthritis (GRAPPA) treatment recommendations were published in 2015, therapeutic options for psoriatic arthritis (PsA) have advanced considerably. This work reviews the literature since the previous recommendations (data published 2013–2020, including conference presentations between 2017 and 2020) and reports high-quality, evidence-based, domain-focused recommendations for medication selection in PsA developed by GRAPPA clinicians and patient research partners. The overarching principles for the management of adults with PsA were updated by consensus. Principles considering biosimilars and tapering of therapy were added, and the research agenda was revised. Literature searches covered treatments for the key domains of PsA: peripheral arthritis, axial disease, enthesitis, dactylitis, and skin and nail psoriasis; additional searches were performed for PsA-related conditions (uveitis and inflammatory bowel disease) and comorbidities. Individual subcommittees used a GRADE-informed approach, taking into account the quality of evidence for therapies, to generate recommendations for each of these domains, which were incorporated into an overall schema. Choice of therapy for an individual should ideally address all disease domains active in that patient, supporting shared decision-making. As safety issues often affect potential therapeutic choices, additional consideration was given to relevant comorbidities. These GRAPPA treatment recommendations provide up-to-date, evidence-based guidance on PsA management for clinicians and people with PsA.

Quo vadis reactive arthritis?

We provide an overview of recent articles which describe new thinking regarding HLA-B27-associated reactive arthritis (ReA), including those additional infection-related arthritides triggered by microbes that often are grouped under the term ReA. With the advent and continuation of the pandemic, an increasing number of cases and case series of post-COVID-19 arthritis have been reported and classified as ReA. Further, arthritis after COVID-19 vaccination is a new entity included within the spectrum of ReA. New causative microorganisms identified in case reports include Clostridium difficile, Mycoplasma pneumoniae, Giardia lamblia, Leptospira , and babesiosis. SARS-CoV-2 is emerging as a significant etiologic agent for apparent ReA. It is now clear that comprehensive clinical and laboratory investigations, synovial fluid analyses, and close follow-up of patients all are essential to differentiate ReA from diseases that may present with similar clinical attributes. Further, and importantly, additional research is required to define the wide diversity in causative agents, epidemiology, and rare case presentations of these arthritides. Finally, new classification and diagnostic criteria, and updated treatment recommendations, are essential to the advancement of our understanding of ReA.

Treatment Guidelines for Rheumatoid Arthritis

Rheumatoid arthritis (RA) is a chronic systemic autoimmune disease with synovitis and extra-articular systemic involvement. Chronic RA treatment is challenging and represents a major health burden worldwide. Recent insights regarding RA pathogenesis have led to novel therapeutic agents, especially biologics. Furthermore, accumulating experience and new clinical studies have helped to inform updated recommendations for treatment of RA. Recently, treatment guidelines from the American College of Rheumatology were released. Here, we review these guidelines and their application to daily practice.

[Update on diastolic heart failure].

In August 2021, an update of the European Society of Cardiology-Heart Failure Association guideline for the diagnosis and treatment of heart failure was released. To review the changes implied by current guidelines regarding the diagnosis and treatment of patients with heart failure and preserved left ventricular ejection fraction (HFpEF). The diagnosis of HFpEF requires the combined presence of clinical signs, left ventricular ejection fraction ≥ 50%, elevated natriuretic peptides, and elevated left ventricular filling pressure. If the diagnosis remains equivocal, astress test is recommended. The targeted identification and treatment of comorbid conditions is key for aholistic therapeutic approach to HFpEF. Diuretics are recommended in congested patients with HFpEF in order to alleviate signs and symptoms. The treatment of diabetic patients with heart failure should include asodium glucose co-transporter‑2 (SGLT2) inhibitor. All patients with HFpEF should be enrolled in amultidisciplinary heart failure management program aiming to improve self-care strategies and offer participation in an exercise program. It was recently shown for the first time in arandomized trial that hard clinical endpoints could be reduced in patients with HFpEF using the SGLT2 inhibitor empagliflozin. It is expected that this finding will become part of updated treatment recommendations in the near future. Although challenging, the early diagnosis of HFpEF is key to averting the poor prognosis associated with this frequent condition. Multidisciplinary care and innovative pharmacologic and non-pharmacologic therapies, however, can improve quality of life, exercise tolerance, and prognosis.

Treatment outcomes in women with idiopathic generalized epilepsy.

ObjectivesTo evaluate the changes in prescription patterns in the treatment of idiopathic generalized epilepsy (IGE) due to updated treatment recommendations and to assess seizure outcomes of valproate compared to other antiseizure medications (ASMs), with emphasis on women with epilepsy (WWE).Materials and MethodsRecords of IGE patients treated at Tampere University Hospital between 1 January 2009 and 31 December 2018 were retrospectively inspected. Data were analysed for two subgroups based on age and sex. Seizure control with reference to the efficacy of different ASMs and their combinations was examined for each subgroup.ResultsThe study compiled 263 subjects (166 females and 97 males). Of all patients, 72.6% remained seizure free. There was no difference in seizure control between sexes (OR 1.25, p = .48). Males used valproate more often than females while females used lamotrigine and levetiracetam more often than males. Lamotrigine and levetiracetam were used especially as monotherapy in WWE, and mostly as part of combination therapy in males. Valproate alternatives were found as effective as valproate when used in monotherapy in adults. Valproate remained the most used ASM in the paediatric subgroup.ConclusionsThe use of valproate has decreased in daily clinical use with the simultaneous increased use of alternative ASMs compared to our previous study. Decreasing use of valproate in WWE did not increase the risk of seizure recurrence; therefore, valproate alternatives could be considered as first‐line ASMs for WWE. Overall, IGE patients demonstrated good clinical outcomes with valproate or other broad‐spectrum ASMs as monotherapy.

P050 Consensus based practice guidelines for the management and treatment of Juvenile familial Mediterranean fever: the Egyptian College of Paediatric Rheumatology initiative

Abstract Background Familial Mediterranean fever (FMF) is the most common monogenic auto-inflammatory disease, with the highest prevalence amongst Mediterranean countries including Egypt (where there is high carrier rate of MEFV gene), characterized by recurrent attacks of fever and polyserositis. Mutations in the MEFV gene encoding pyrin has been associated with the disease, which causes exaggeration of the inflammatory response through uncontrolled interleukin production. Issuing updated treatment recommendations are vital for the treating healthcare professionals to get well acquainted with its diagnosis &amp; treatment. To produce consensus-based recommendations to guide the early diagnosis, management and follow-up of patients with FMF. Methods The Patient/Population, Intervention, Comparison, and Outcomes (PICO) questions were developed and refined by the core team. A qualitative synthesis of scientific evidence based on systematic review and clinical experience was conducted to compile evidence for the diagnosis and management of FMF. A consensus process was conducted among the expert panel to generate the final recommendations and grade their strength. 3 rounds of Delphi process were carried out. Results Following 3 Delphi rounds, recommendations were developed for: early diagnosis, who to treat, treatment targets, genetic testing and its interpretation in association with clinical presentation, treatment of FMF and dealing with acute attacks, monitoring of management, identify treatment response, systemic affection, persistent attacks or inflammation, resistant cases, protracted symptoms, as well as remission status. Algorithm for patients’ diagnosis and management is provided. The final document comprises 12 recommendations, each presented with its degree of agreement (0–10), Level of agreement, grade of recommendation and rationale. The degree of agreement was &amp;gt;7/10 in all instances. Conclusion This guideline provides comprehensive approach to the accurate diagnosis and effective management/monitoring of FMF. It also represents a model for the incorporation of medical genetics practice into the more traditional domains of general medicine.