xml: <clinical_study>
    <!-- This xml conforms to an XML Schema at:
      https://clinicaltrials.gov/ct2/html/images/info/public.xsd -->
    <required_header>
      <download_date>ClinicalTrials.gov processed this data on September 20, 2023</download_date>
      <link_text>Link to the current ClinicalTrials.gov record.</link_text>
      <url>https://clinicaltrials.gov/ct2/show/NCT06014463</url>
    </required_header>
    <id_info>
      <org_study_id>GO 23/16</org_study_id>
      <nct_id>NCT06014463</nct_id>
    </id_info>
    <brief_title>Evaluation of Adult Patients With Immunodeficiency Within the Scope of the ICF</brief_title>
    <official_title>Evaluation of Adult Patients With Immunodeficiency Within the Scope of the International Classification of Functionality, Ability and Health</official_title>
    <sponsors>
      <lead_sponsor>
        <agency>Hacettepe University</agency>
        <agency_class>Other</agency_class>
      </lead_sponsor>
      <collaborator>
        <agency>Baskent University</agency>
        <agency_class>Other</agency_class>
      </collaborator>
    </sponsors>
    <source>Hacettepe University</source>
    <oversight_info>
      <has_dmc>No</has_dmc>
      <is_fda_regulated_drug>No</is_fda_regulated_drug>
      <is_fda_regulated_device>No</is_fda_regulated_device>
    </oversight_info>
    <brief_summary>
      <textblock>
        Primary immunodeficiency is a clinically, immunologically, and genetically heterogeneous&#xD;
        group of diseases that occur as a result of quantitative and/or qualitative deficiency of one&#xD;
        or more cellular and molecular components belonging to the immune system. This&#xD;
        classification, called the International Classification of Functioning, Disability and Health&#xD;
        ( ICF), constitutes a common language and perspective for the definition of health and&#xD;
        health-related conditions. This study aimed to evaluate the body structure function, activity&#xD;
        and participation limitations of adult immunodeficiency patients within the scope of ICF and&#xD;
        to compare body structure function, activity and participation limitations and compare with&#xD;
        healthy people.&#xD;
      </textblock>
    </brief_summary>
    <detailed_description>
      <textblock>
        Primary immunodeficiency is a clinically, immunologically and genetically heterogeneous group&#xD;
        of diseases that occur as a result of quantitative and/or qualitative deficiency of one or&#xD;
        more cellular and molecular components belonging to the immune system. The overall prevalence&#xD;
        of PID in the world is stated as 1/2,000-1/10,000 per live birth. In our country, the&#xD;
        national PID registration system has just been established. It is estimated that this rate is&#xD;
        higher in developing countries such as Turkey, where there is a lot of inbreeding. The&#xD;
        clinical appearance of PID is highly variable; however, most often it is characterized by an&#xD;
        increased predisposition to infection. Therefore, the disease manifests itself with&#xD;
        infections that are &quot;recurrent and difficult to respond to treatment&quot;. PID, which mostly&#xD;
        starts to give symptoms in childhood, are in the category of rare diseases. For this reason,&#xD;
        the probability of receiving a diagnosis in primary and second-line healthcare institutions&#xD;
        is very low. Since the course of the disease is poor in a significant part of PID, early&#xD;
        diagnosis and timely treatment are very important for reducing mortality, morbidity and&#xD;
        economic burden. This is possible with the level of social awareness and the high index of&#xD;
        suspicion of medical personnel. For the successful management of the disease, the patient&#xD;
        should be quickly referred to a clinical immunologist experienced in immunodeficiencies and&#xD;
        further tests should be performed. Fifteen years ago, the Jeffrey Modell Foundation (JMF)&#xD;
        identified clinical signs that arouse suspicion of PID in children and adults. This document&#xD;
        is a useful basis, but revision is required because it does not take into account the&#xD;
        autoimmune, inflammatory or malignant manifestations of PID, which may be the cause of the&#xD;
        main clinical presentation in some of the PID. Pulmonary complications are common in PID and&#xD;
        contribute significantly to the morbidity and mortality of patients. Recurrent respiratory&#xD;
        infections (more than 2 per year for children and within 3 years for adults) are one of the&#xD;
        10 warning signs of PİY recommended by the Jeffrey Modell Foundation and are often the cause&#xD;
        of death in adults with PID. Especially in PID, which is characterized by antibody&#xD;
        deficiency, frequent recurrent upper and lower respiratory tract infections lead to chronic&#xD;
        complications in tissues and organs. Bronchiectasis is the prominent one among these.&#xD;
        According to the European Society of Immunodeficiencies, bronchiectasis develops in ~23% of&#xD;
        patients with NDI (one-third of all PID patients) . It has been shown that the general state&#xD;
        of health, psychosocial well-being level and quality of life are low in patients with PI who&#xD;
        have developed chronic lung disease, and it has been found that their daily activities are&#xD;
        significantly limited.&#xD;
  &#xD;
        This classification, called the International Classification of Functioning, Disability and&#xD;
        Health (ICF), was developed in 2000 to create a common language and perspective for the&#xD;
        definition of health and health-related conditions. Objectives of the ICF classification:&#xD;
  &#xD;
          -  To create a scientifically based approach to determine health and health-related&#xD;
             conditions&#xD;
  &#xD;
          -  To facilitate and increase communication between all health professionals and&#xD;
             individuals in different professional groups and to ensure the formation of a common&#xD;
             language Dec&#xD;
  &#xD;
          -  To ensure the determination of health-related applications&#xD;
  &#xD;
          -  Coding health-related factors with a specific system ICF provides the functional status&#xD;
             of individuals and the identification of problems that they may encounter in daily life&#xD;
             from a biopsychosocial point of view.&#xD;
  &#xD;
        ICF-CY evaluates health-related situations with a total of 1685 items in 4 sections. The fact&#xD;
        that it has so much substance constitutes a disadvantage in terms of its use. For this&#xD;
        reason, with the ICF Core Sets Project study in 2003, disease-specific core sets were created&#xD;
        by selecting categories that are specific to diseases and can fully identify the patient.&#xD;
        Core sets, just like ICF, treat the patient holistically. Each set has 2 separate forms in&#xD;
        the form of short form and long form. There are 5 core sets that can evaluate cardiopulmonary&#xD;
        system disorders. Cardiopulmonary disorders, obesity, diabetes mellitus, COPD, chronic&#xD;
        ischemic heart problems are the core sets. In a study conducted on 40 pediatric patients with&#xD;
        and without cystic fibrosis, it was shown that the ICF model is applicable and useful in&#xD;
        evaluating body function and structure disorders such as November muscle weakness, postural&#xD;
        anomalies, and limitations in activity and participation in daily life, and in creating a&#xD;
        treatment plan. a study conducted on 24 lung transplant patients showed that the ICF model is&#xD;
        practical and applicable in the clinic for evaluating activity and participation restrictions&#xD;
        such as decreased exercise capacity, body structure and function disorders such as postural&#xD;
        disorders, physical activity in daily life, balance disorder, decreased quality of life.&#xD;
  &#xD;
        However, there are no studies evaluating the body structure, function, activity and&#xD;
        participation of immunodeficiency patients within the scope of ICF. Our study has the feature&#xD;
        of being the first study in which immunodeficiency patients were evaluated within the scope&#xD;
        of ICF. With this study, the investigators believe that a holistic assessment conducted&#xD;
        within the framework of ICF in immunodeficiency patients will have a positive impact on the&#xD;
        clinical decision-making process of cardiopulmonary rehabilitation professionals. This study&#xD;
        aims; to evaluate the body structure function, activity and participation limitations of&#xD;
        adult immunodeficiency patients within the scope of ICF and to compare with healthy people. A&#xD;
        second goal is to evaluate the relationships between body structure, function, activity and&#xD;
        participation limitations in adult immune deficiency patients.&#xD;
      </textblock>
    </detailed_description>
    <overall_status>Recruiting</overall_status>
    <start_date type="Actual">March 15, 2023</start_date>
    <completion_date type="Anticipated">April 2024</completion_date>
    <primary_completion_date type="Anticipated">January 2024</primary_completion_date>
    <study_type>Observational</study_type>
    <has_expanded_access>No</has_expanded_access>
    <study_design_info>
      <observational_model>Other</observational_model>
      <time_perspective>Prospective</time_perspective>
    </study_design_info>
    <primary_outcome>
      <measure>Evaluation of hand grip strength</measure>
      <time_frame>1 day</time_frame>
      <description>The participants will be measured in an unsupported sitting position with his back upright, while the elbow is in flexion (the person's arm is adjacent to the torso, in a neutral position, and the elbow is in a 90° flexion, with the wrist neutral). The measurements for the dominant and non-dominant sides will be repeated three times. The average of the three measurements for each side will be taken and recorded in Newton (N).</description>
    </primary_outcome>
    <secondary_outcome>
      <measure>Evaluation of body composition:</measure>
      <time_frame>1 day</time_frame>
      <description>The bioelectric impedance analysis (XIAOMI MI 2 Body Analysis Scale)</description>
    </secondary_outcome>
    <secondary_outcome>
      <measure>Comorbidity Assessment</measure>
      <time_frame>1 day</time_frame>
      <description>The Charlson Comorbidity Index developed by Charlson et al. will be used to determine the short-term mortality risk of patients with concomitant disease conditions. DM 1 point without myocardial infarction, congestive heart failure, peripheral vascular disease, cerebrovascular disease, dementia, chronic pulmonary disease, connective tissue disorder, peptic ulcer disease, mild liver disease and end-organ damage on the scale; hemiplegia, moderate and severe kidney disease, DM with end-organ damage, non-metastatic tumor, leukemia, lymphoma and myeloma are 2 points; 3 points for moderate or severe liver disease; and metastatic solid tumor or Acquired Immune Deficiency Syndrome (Acquired Immune Deficiency Syndrome -AIDS) is 6 points. The total score, on the other hand, will be calculated by adding up the equivalent scores of diseases.</description>
    </secondary_outcome>
    <secondary_outcome>
      <measure>Evaluation of Sleep Quality</measure>
      <time_frame>1 day</time_frame>
      <description>The Pittsburgh Sleep Quality Index (PSQI), which has Turkish validity and reliability and evaluates an individual's sleep quality for the last one month, contains a total of 24 questions. 19 Of these questions are answered by the patient himself, he participates in the scoring. 5 questions are answered by the patient's spouse or roommate and are used only for clinical information and do not participate in scoring. Each question is evaluated with a score between 0-3.Dec. The total PSQI score takes a value between 0-21.Dec. In the end, 7 components are calculated. These; subjective sleep quality is sleep latency, sleep duration, habitual sleep efficiency, sleep disorder, sleeping medication use, daytime dysfunction. The total PSQI score is obtained by summing the scores of all these components. Those with a total score of 5 and below 5 are rated as having &quot;good&quot; sleep quality, and those with a total score of over 5 are rated as &quot;poor&quot; sleep quality.</description>
    </secondary_outcome>
    <secondary_outcome>
      <measure>Fatigue Assessment</measure>
      <time_frame>1 day</time_frame>
      <description>The severity of fatigue in the daily life of the patients was evaluated with the Fatigue Severity Scale (FSS), which has validity and reliability in Turkish. Patients can apply this scale, which has nine items, on their own. The patient is informed about how to fill out the scale, but patients are not guided for questions and statements that they have difficulty understanding. Patients are asked to choose a score from 1 to 7, which indicates how much they agree with each statement on the scale. Statements related to scoring; 1. I Strongly Disagree, 2. Disagree, 3. I Tend to Disagree, 4: I am Undecided, 5: I Tend to Agree, 6: I Agree, 7: I Strongly Agree. Scores of 4 and above given to the statements indicate that fatigue is severe.</description>
    </secondary_outcome>
    <secondary_outcome>
      <measure>Posture Evaluation</measure>
      <time_frame>1 day</time_frame>
      <description>The posture analysis form developed by Corbin and his colleagues was used to determine the postural disorders of the patients. The analysis will be evaluated in two different ways, posterior and lateral. According to the scoring of the results of the posture analyses according to their severity, they will be rated as 0: none, 1: light, 2: moderate and 3: severe. According to the total lateral and posterior posture score, the total score varies between 0-12. Dec.</description>
    </secondary_outcome>
    <secondary_outcome>
      <measure>Evaluation of Quality of Life</measure>
      <time_frame>1 day</time_frame>
      <description>To evaluate the quality of life, Koçyiğit et al. the SF-36 quality of life scale, which has been validated by our country, will be applied. SF-36 Quality of Life consists of eight subgroups: physical function, physical role restriction, emotional role restriction, body pain, social function, mental health, vitality, general health and contains a total of 36 questions. Scoring is made between 0-100 for each group. The lowest score indicates the worst state of health. The last four weeks of the patients' conditions will be taken into account in the evaluation.</description>
    </secondary_outcome>
    <secondary_outcome>
      <measure>Evaluation of Daily Living Activities</measure>
      <time_frame>1 day</time_frame>
      <description>To evaluate daily living activities, Işık et al. The Lawton Instrumental Daily Living Activities (IADL) scale, which has been made a validity study for our country, will be applied. The Lawton Instrumental Daily Living Activities (IADL) scale consists of eight categories: the ability to use a phone, shopping, food preparation, household chores, laundry, transportation method, responsibility for using one's medicines and the ability to manage financial affairs. The responses given to each of the eight categories on the scale are coded as 0 (can't or partially can) or 1 (can), and the responses are summed. The total score varies between 0 (low-functioning, dependent) and 8 December (high-functioning, independent).</description>
    </secondary_outcome>
    <secondary_outcome>
      <measure>Evaluation of Physical Activity</measure>
      <time_frame>7 day</time_frame>
      <description>The International Physical Activity Questionnaire (IPAQ) long form, which has validity and reliability in Turkish, will be used to determine the level of physical activity by questioning the activities within the last 7 days. It includes the sum of the duration (how many minutes per day) and frequency (how many days in the last 7 days) to calculate the total score. The total score can be calculated with two different evaluations. The first includes special scoring (work, transportation, home gardening, leisure time), and the second includes activity-specific scoring (walking, moderate-intensity activity, vigorous activity). In activity-specific scoring, walking, moderate violent activity and violent activity under their heading of the sections are Decoupled by the sum between them. With these calculations, a value is found in MET-minutes. A MET-minute is calculated by multiplying the MET score by the minute of the activity performed.</description>
    </secondary_outcome>
    <secondary_outcome>
      <measure>Exercise Capacity Assessment</measure>
      <time_frame>1 day</time_frame>
      <description>It is a simple, applicable alternative test to 6 DYT that evaluates exercise capacity, whose reliability has been proven in various lung diseases such as Cystic Fibrosis and COPD. The patient, whose height is 44 cm, who is sitting in a chair without armbands and has a place to recline, is asked to cross his arms to chest level. The patient, who is standing upright with his hips and knees coming to full flexion, sits down again. the number of repetitions of the patient who does sit-up for 1 minute will be recorded. In the interpretation of the test, the reference values determined by age will be taken as a basis and expressed as a percentage of the expected values. In addition, heart rate, oxygen saturation, dyspnea and leg fatigue values will be recorded before and after the test.</description>
    </secondary_outcome>
    <secondary_outcome>
      <measure>Evaluation of ICF Parameters</measure>
      <time_frame>1 day</time_frame>
      <description>ICF belongs to the international classification &quot;family&quot; developed by the World Health Organization (WHO) for application in many areas of health. It uses a standard, common language that enables worldwide communication related to health and medical care in various disciplines and scientific fields. In order to evaluate disorders, activity limitations and limitations in participation, the subparagraphs of area b (body functions), area s (body structures), area d (activity and participation level) and area e (environmental and personal factors) will be comprehensively scanned to select items related to the classification area. Scoring is done separately for each item in the body structures (s), body functions (b), activity and participation (d), environmental and personal factors section. In this study, a Decrement between 0-4 will be made for each item in all sections. Items or sections with a higher score indicate more affect/disorder.</description>
    </secondary_outcome>
    <number_of_groups>2</number_of_groups>
    <enrollment type="Anticipated">36</enrollment>
    <condition>Primary Immune Deficiency</condition>
    <condition>Exercise Capacity</condition>
    <condition>Physical Activity</condition>
    <condition>Quality of Life</condition>
    <arm_group>
      <arm_group_label>Patients with Primary Immune Deficiency</arm_group_label>
      <description>Patients between the ages of 18-65 years who have been diagnosed with primary immunodeficiency</description>
    </arm_group>
    <arm_group>
      <arm_group_label>Healthy Controls</arm_group_label>
      <description>Age and sex matched healthy controls between the ages of 18-65 years</description>
    </arm_group>
    <eligibility>
      <study_pop>
        <textblock>
          Patients between the ages of 18-65 who have been diagnosed with immunodeficiency at Başkent&#xD;
          University Hospital Immunology Department and Gülhane Hospital Immunology Department and&#xD;
          who are willing to participate in the research and 18-65 patients who are diagnosed with&#xD;
          immunodeficiency will be accepted for the study.&#xD;
  &#xD;
          The snowball sampling method will be used for our control group. We plan to contact the&#xD;
          patient's relatives and then contact someone else in the same way. At the same time, we&#xD;
          plan to prepare a text explaining each method and reach people through social media.&#xD;
        </textblock>
      </study_pop>
      <sampling_method>Probability Sample</sampling_method>
      <criteria>
        <textblock>
          Criteria for Inclusion in the Adult Immunodeficiency Group&#xD;
  &#xD;
            1. to be between the ages of 18-65 years old.&#xD;
  &#xD;
            2. to be volunteer to participate in research&#xD;
  &#xD;
            3. to have been diagnosed with Primary Immunodeficiency&#xD;
  &#xD;
            4. to be taking immunoglobulin replacement therapy&#xD;
  &#xD;
            5. to have no problems in reading and/or understanding scales and being able to cooperate&#xD;
               with tests&#xD;
  &#xD;
          Criteria for exclusion of the Adult Immunodeficiency Group:&#xD;
  &#xD;
            1. The presence of active infection&#xD;
  &#xD;
            2. Musculoskeletal and neurological disease that may affect exercise performance&#xD;
               symptomatic heart disease&#xD;
  &#xD;
            3. To have a neurological disease or other clinical diagnosis that may affect cognitive&#xD;
               status&#xD;
  &#xD;
          Criteria for inclusion in the control group:&#xD;
  &#xD;
            1. to be between the ages of 18-65 years old&#xD;
  &#xD;
            2. to give informed consent&#xD;
  &#xD;
            3. to have no problems in reading and/or understanding scales and being able to cooperate&#xD;
               with tests&#xD;
  &#xD;
          Criteria for exclusion of the Control Group:&#xD;
  &#xD;
            1. to have any orthopedic or neuromuscular disorder that will interfere with walking or&#xD;
               exercise performance,&#xD;
  &#xD;
            2. to have any chronic disease or to have psychiatric disorders or mental influences that&#xD;
               may prevent cooperation or compliance with exercise tests.&#xD;
        </textblock>
      </criteria>
      <gender>All</gender>
      <minimum_age>18 Years</minimum_age>
      <maximum_age>65 Years</maximum_age>
      <healthy_volunteers>Accepts Healthy Volunteers</healthy_volunteers>
    </eligibility>
    <overall_official>
      <last_name>Ebru Calik-Kutukcu, PhD</last_name>
      <role>Principal Investigator</role>
      <affiliation>Hacettepe University</affiliation>
    </overall_official>
    <overall_contact>
      <last_name>Ebru Calik Kutukcu, PhD</last_name>
      <phone>+905442850087</phone>
      <email>ecalik85@gmail.com</email>
    </overall_contact>
    <overall_contact_backup>
      <last_name>Ecran Cinkavuk</last_name>
      <phone>+90 542 743 35 95</phone>
      <email>ecranbar@gmail.com</email>
    </overall_contact_backup>
    <location>
      <facility>
        <name>Ebru Çalık Kutukcu</name>
        <address>
          <city>Ankara</city>
          <state>Hacettepe Unıversity</state>
          <zip>06100</zip>
          <country>Turkey</country>
        </address>
      </facility>
      <status>Recruiting</status>
      <contact>
        <last_name>Ebru Calik Kutukcu, Phd</last_name>
        <phone>0903123051576</phone>
        <phone_ext>195</phone_ext>
        <email>ebrucalk85@hotmail.com</email>
      </contact>
    </location>
    <location_countries>
      <country>Turkey</country>
    </location_countries>
    <verification_date>August 2023</verification_date>
    <study_first_submitted>August 18, 2023</study_first_submitted>
    <study_first_submitted_qc>August 23, 2023</study_first_submitted_qc>
    <study_first_posted type="Actual">August 28, 2023</study_first_posted>
    <last_update_submitted>August 23, 2023</last_update_submitted>
    <last_update_submitted_qc>August 23, 2023</last_update_submitted_qc>
    <last_update_posted type="Actual">August 28, 2023</last_update_posted>
    <responsible_party>
      <responsible_party_type>Principal Investigator</responsible_party_type>
      <investigator_affiliation>Hacettepe University</investigator_affiliation>
      <investigator_full_name>Ebru Calik Kutukcu</investigator_full_name>
      <investigator_title>Associate Professor</investigator_title>
    </responsible_party>
    <keyword>Primary Immune Deficiency</keyword>
    <keyword>ICF</keyword>
    <keyword>Functional Performance</keyword>
    <condition_browse>
      <!-- CAUTION:  The following MeSH terms are assigned with an imperfect algorithm            -->
      <mesh_term>Primary Immunodeficiency Diseases</mesh_term>
      <mesh_term>Immunologic Deficiency Syndromes</mesh_term>
    </condition_browse>
    <patient_data>
      <sharing_ipd>No</sharing_ipd>
    </patient_data>
    <!-- Results have not yet been posted for this study                                          -->
  </clinical_study>
  
  
text: download date: ClinicalTrials.gov processed this data on September 20, 2023
  link text: Link to the current ClinicalTrials.gov record.
  url: https://clinicaltrials.gov/ct2/show/NCT06014463
  org study id: GO 23/16
  nct id: NCT06014463
  lead sponsor: 
  collaborator: 
  has dmc: No
  is fda regulated drug: No
  is fda regulated device: No
  Primary immunodeficiency is a clinically, immunologically, and genetically heterogeneous
        group of diseases that occur as a result of quantitative and/or qualitative deficiency of one
        or more cellular and molecular components belonging to the immune system. This
        classification, called the International Classification of Functioning, Disability and Health
        ( ICF), constitutes a common language and perspective for the definition of health and
        health-related conditions. This study aimed to evaluate the body structure function, activity
        and participation limitations of adult immunodeficiency patients within the scope of ICF and
        to compare body structure function, activity and participation limitations and compare with
        healthy people.
  Primary immunodeficiency is a clinically, immunologically and genetically heterogeneous group
        of diseases that occur as a result of quantitative and/or qualitative deficiency of one or
        more cellular and molecular components belonging to the immune system. The overall prevalence
        of PID in the world is stated as 1/2,000-1/10,000 per live birth. In our country, the
        national PID registration system has just been established. It is estimated that this rate is
        higher in developing countries such as Turkey, where there is a lot of inbreeding. The
        clinical appearance of PID is highly variable; however, most often it is characterized by an
        increased predisposition to infection. Therefore, the disease manifests itself with
        infections that are "recurrent and difficult to respond to treatment". PID, which mostly
        starts to give symptoms in childhood, are in the category of rare diseases. For this reason,
        the probability of receiving a diagnosis in primary and second-line healthcare institutions
        is very low. Since the course of the disease is poor in a significant part of PID, early
        diagnosis and timely treatment are very important for reducing mortality, morbidity and
        economic burden. This is possible with the level of social awareness and the high index of
        suspicion of medical personnel. For the successful management of the disease, the patient
        should be quickly referred to a clinical immunologist experienced in immunodeficiencies and
        further tests should be performed. Fifteen years ago, the Jeffrey Modell Foundation (JMF)
        identified clinical signs that arouse suspicion of PID in children and adults. This document
        is a useful basis, but revision is required because it does not take into account the
        autoimmune, inflammatory or malignant manifestations of PID, which may be the cause of the
        main clinical presentation in some of the PID. Pulmonary complications are common in PID and
        contribute significantly to the morbidity and mortality of patients. Recurrent respiratory
        infections (more than 2 per year for children and within 3 years for adults) are one of the
        10 warning signs of PİY recommended by the Jeffrey Modell Foundation and are often the cause
        of death in adults with PID. Especially in PID, which is characterized by antibody
        deficiency, frequent recurrent upper and lower respiratory tract infections lead to chronic
        complications in tissues and organs. Bronchiectasis is the prominent one among these.
        According to the European Society of Immunodeficiencies, bronchiectasis develops in ~23% of
        patients with NDI (one-third of all PID patients) . It has been shown that the general state
        of health, psychosocial well-being level and quality of life are low in patients with PI who
        have developed chronic lung disease, and it has been found that their daily activities are
        significantly limited.
  
        This classification, called the International Classification of Functioning, Disability and
        Health (ICF), was developed in 2000 to create a common language and perspective for the
        definition of health and health-related conditions. Objectives of the ICF classification:
  
          -  To create a scientifically based approach to determine health and health-related
             conditions
  
          -  To facilitate and increase communication between all health professionals and
             individuals in different professional groups and to ensure the formation of a common
             language Dec
  
          -  To ensure the determination of health-related applications
  
          -  Coding health-related factors with a specific system ICF provides the functional status
             of individuals and the identification of problems that they may encounter in daily life
             from a biopsychosocial point of view.
  
        ICF-CY evaluates health-related situations with a total of 1685 items in 4 sections. The fact
        that it has so much substance constitutes a disadvantage in terms of its use. For this
        reason, with the ICF Core Sets Project study in 2003, disease-specific core sets were created
        by selecting categories that are specific to diseases and can fully identify the patient.
        Core sets, just like ICF, treat the patient holistically. Each set has 2 separate forms in
        the form of short form and long form. There are 5 core sets that can evaluate cardiopulmonary
        system disorders. Cardiopulmonary disorders, obesity, diabetes mellitus, COPD, chronic
        ischemic heart problems are the core sets. In a study conducted on 40 pediatric patients with
        and without cystic fibrosis, it was shown that the ICF model is applicable and useful in
        evaluating body function and structure disorders such as November muscle weakness, postural
        anomalies, and limitations in activity and participation in daily life, and in creating a
        treatment plan. a study conducted on 24 lung transplant patients showed that the ICF model is
        practical and applicable in the clinic for evaluating activity and participation restrictions
        such as decreased exercise capacity, body structure and function disorders such as postural
        disorders, physical activity in daily life, balance disorder, decreased quality of life.
  
        However, there are no studies evaluating the body structure, function, activity and
        participation of immunodeficiency patients within the scope of ICF. Our study has the feature
        of being the first study in which immunodeficiency patients were evaluated within the scope
        of ICF. With this study, the investigators believe that a holistic assessment conducted
        within the framework of ICF in immunodeficiency patients will have a positive impact on the
        clinical decision-making process of cardiopulmonary rehabilitation professionals. This study
        aims; to evaluate the body structure function, activity and participation limitations of
        adult immunodeficiency patients within the scope of ICF and to compare with healthy people. A
        second goal is to evaluate the relationships between body structure, function, activity and
        participation limitations in adult immune deficiency patients.
  observational model: Other
  time perspective: Prospective
  measure: Evaluation of hand grip strength
  time frame: 1 day
  description: The participants will be measured in an unsupported sitting position with his back upright, while the elbow is in flexion (the person's arm is adjacent to the torso, in a neutral position, and the elbow is in a 90° flexion, with the wrist neutral). The measurements for the dominant and non-dominant sides will be repeated three times. The average of the three measurements for each side will be taken and recorded in Newton (N).
  measure: Evaluation of body composition:
  time frame: 1 day
  description: The bioelectric impedance analysis (XIAOMI MI 2 Body Analysis Scale)
  measure: Comorbidity Assessment
  time frame: 1 day
  description: The Charlson Comorbidity Index developed by Charlson et al. will be used to determine the short-term mortality risk of patients with concomitant disease conditions. DM 1 point without myocardial infarction, congestive heart failure, peripheral vascular disease, cerebrovascular disease, dementia, chronic pulmonary disease, connective tissue disorder, peptic ulcer disease, mild liver disease and end-organ damage on the scale; hemiplegia, moderate and severe kidney disease, DM with end-organ damage, non-metastatic tumor, leukemia, lymphoma and myeloma are 2 points; 3 points for moderate or severe liver disease; and metastatic solid tumor or Acquired Immune Deficiency Syndrome (Acquired Immune Deficiency Syndrome -AIDS) is 6 points. The total score, on the other hand, will be calculated by adding up the equivalent scores of diseases.
  measure: Evaluation of Sleep Quality
  time frame: 1 day
  description: The Pittsburgh Sleep Quality Index (PSQI), which has Turkish validity and reliability and evaluates an individual's sleep quality for the last one month, contains a total of 24 questions. 19 Of these questions are answered by the patient himself, he participates in the scoring. 5 questions are answered by the patient's spouse or roommate and are used only for clinical information and do not participate in scoring. Each question is evaluated with a score between 0-3.Dec. The total PSQI score takes a value between 0-21.Dec. In the end, 7 components are calculated. These; subjective sleep quality is sleep latency, sleep duration, habitual sleep efficiency, sleep disorder, sleeping medication use, daytime dysfunction. The total PSQI score is obtained by summing the scores of all these components. Those with a total score of 5 and below 5 are rated as having "good" sleep quality, and those with a total score of over 5 are rated as "poor" sleep quality.
  measure: Fatigue Assessment
  time frame: 1 day
  description: The severity of fatigue in the daily life of the patients was evaluated with the Fatigue Severity Scale (FSS), which has validity and reliability in Turkish. Patients can apply this scale, which has nine items, on their own. The patient is informed about how to fill out the scale, but patients are not guided for questions and statements that they have difficulty understanding. Patients are asked to choose a score from 1 to 7, which indicates how much they agree with each statement on the scale. Statements related to scoring; 1. I Strongly Disagree, 2. Disagree, 3. I Tend to Disagree, 4: I am Undecided, 5: I Tend to Agree, 6: I Agree, 7: I Strongly Agree. Scores of 4 and above given to the statements indicate that fatigue is severe.
  measure: Posture Evaluation
  time frame: 1 day
  description: The posture analysis form developed by Corbin and his colleagues was used to determine the postural disorders of the patients. The analysis will be evaluated in two different ways, posterior and lateral. According to the scoring of the results of the posture analyses according to their severity, they will be rated as 0: none, 1: light, 2: moderate and 3: severe. According to the total lateral and posterior posture score, the total score varies between 0-12. Dec.
  measure: Evaluation of Quality of Life
  time frame: 1 day
  description: To evaluate the quality of life, Koçyiğit et al. the SF-36 quality of life scale, which has been validated by our country, will be applied. SF-36 Quality of Life consists of eight subgroups: physical function, physical role restriction, emotional role restriction, body pain, social function, mental health, vitality, general health and contains a total of 36 questions. Scoring is made between 0-100 for each group. The lowest score indicates the worst state of health. The last four weeks of the patients' conditions will be taken into account in the evaluation.
  measure: Evaluation of Daily Living Activities
  time frame: 1 day
  description: To evaluate daily living activities, Işık et al. The Lawton Instrumental Daily Living Activities (IADL) scale, which has been made a validity study for our country, will be applied. The Lawton Instrumental Daily Living Activities (IADL) scale consists of eight categories: the ability to use a phone, shopping, food preparation, household chores, laundry, transportation method, responsibility for using one's medicines and the ability to manage financial affairs. The responses given to each of the eight categories on the scale are coded as 0 (can't or partially can) or 1 (can), and the responses are summed. The total score varies between 0 (low-functioning, dependent) and 8 December (high-functioning, independent).
  measure: Evaluation of Physical Activity
  time frame: 7 day
  description: The International Physical Activity Questionnaire (IPAQ) long form, which has validity and reliability in Turkish, will be used to determine the level of physical activity by questioning the activities within the last 7 days. It includes the sum of the duration (how many minutes per day) and frequency (how many days in the last 7 days) to calculate the total score. The total score can be calculated with two different evaluations. The first includes special scoring (work, transportation, home gardening, leisure time), and the second includes activity-specific scoring (walking, moderate-intensity activity, vigorous activity). In activity-specific scoring, walking, moderate violent activity and violent activity under their heading of the sections are Decoupled by the sum between them. With these calculations, a value is found in MET-minutes. A MET-minute is calculated by multiplying the MET score by the minute of the activity performed.
  measure: Exercise Capacity Assessment
  time frame: 1 day
  description: It is a simple, applicable alternative test to 6 DYT that evaluates exercise capacity, whose reliability has been proven in various lung diseases such as Cystic Fibrosis and COPD. The patient, whose height is 44 cm, who is sitting in a chair without armbands and has a place to recline, is asked to cross his arms to chest level. The patient, who is standing upright with his hips and knees coming to full flexion, sits down again. the number of repetitions of the patient who does sit-up for 1 minute will be recorded. In the interpretation of the test, the reference values determined by age will be taken as a basis and expressed as a percentage of the expected values. In addition, heart rate, oxygen saturation, dyspnea and leg fatigue values will be recorded before and after the test.
  measure: Evaluation of ICF Parameters
  time frame: 1 day
  description: ICF belongs to the international classification "family" developed by the World Health Organization (WHO) for application in many areas of health. It uses a standard, common language that enables worldwide communication related to health and medical care in various disciplines and scientific fields. In order to evaluate disorders, activity limitations and limitations in participation, the subparagraphs of area b (body functions), area s (body structures), area d (activity and participation level) and area e (environmental and personal factors) will be comprehensively scanned to select items related to the classification area. Scoring is done separately for each item in the body structures (s), body functions (b), activity and participation (d), environmental and personal factors section. In this study, a Decrement between 0-4 will be made for each item in all sections. Items or sections with a higher score indicate more affect/disorder.
  arm group label: Patients with Primary Immune Deficiency
  description: Patients between the ages of 18-65 years who have been diagnosed with primary immunodeficiency
  arm group label: Healthy Controls
  description: Age and sex matched healthy controls between the ages of 18-65 years
  study pop: 
  sampling method: Probability Sample
  criteria: 
  gender: All
  minimum age: 18 Years
  maximum age: 65 Years
  healthy volunteers: Accepts Healthy Volunteers
  last name: Ebru Calik-Kutukcu, PhD
  role: Principal Investigator
  affiliation: Hacettepe University
  last name: Ebru Calik Kutukcu, PhD
  phone: +905442850087
  email: ecalik85@gmail.com
  last name: Ecran Cinkavuk
  phone: +90 542 743 35 95
  email: ecranbar@gmail.com
  facility: 
  status: Recruiting
  contact: 
  country: Turkey
  responsible party type: Principal Investigator
  investigator affiliation: Hacettepe University
  investigator full name: Ebru Calik Kutukcu
  investigator title: Associate Professor
  mesh term: Primary Immunodeficiency Diseases
  mesh term: Immunologic Deficiency Syndromes
  sharing ipd: No