HOW TO FILL PROFORMA VERSION 6 ILD- India Registry: Indian Chest Society (Proforma Version 6) Center code Date: …………………..… Email ……….………………………… Name of the Consultant ………………………. Mobile no. ……………..…………….. Address for correspondence …………………….…………………….…………………………………. Particulars of the patients Patient Code number Age and sex Date of the birth Name and address of the patient will not be disclosed. The data of name and address will be noted in the investigators patient’s log. It will also be stored in the website and will be used by a computer program to stop enrolment of a single patient two times from the same center or from other center. The identity of the patient will not be revealed. Every patient will be give a code number. Patient code number : The code number of the patient will be given by the recruiting center by putting numerical number after center code number. If center code is AC then patient number may be AC 1, AC2, AC3 … Inclusion: Patients presenting with following Yes No Respiratory symptoms such as shortness of breath and cough Bilateral abnormalities in X ray/HRCT scan thorax No Yes Exclusion: Any Infectious or Malignant diseases Yes No On basis of inclusion and exclusion criteria a patient suspected to have ILD can be identified and thereafter workup of the patient can be done as follows: Proforma questionnaire based on patient’s history 1. Symptoms of patient Symptom Since when Any comment a) Cough March 2007 Every day, dry b) Dyspnoea Jan 2008 Grade 2 c) ______________________________________________________________________________ d) ______________________________________________________________________________ e) ______________________________________________________________________________ f) _______________________________________________________________________________ g) ______________________________________________________________________________ Please put all the symptoms (related or unrelated) in chronological order. Instead of duration it is better to write year of onset. Cough: Frequency ? (Do not include clearing of throat.)None (at all)/Rarely/Everyday/ Severe attacks that interfere with activity Dry (No expectoration)/Mucoid/Yellow-green/Green /Blood tinged/Blood Shortness of breath: Check the single number that describe the point at which S/he becomes short of breath: 0. Not troubled with breathlessness except with strenuous exercise 1. Short of breath when hurrying on the level or walking up a slight hill. 2. Walks slower than people of her/his age on the level because of breathlessness or S/he has to stop from breath when walking on normal pace on the level. 3. Stops for breath after walking about 100 yards or after a few minutes on the level. 4. Too breathless to leave the house or breathless on dressing or undressing. 2. Has the patient noticed any of the following? (put in respective column) Yes No Duration & Yes Remarks a) Weight loss b) Difficulty in swallowing No Duration & Remarks g) Bruising skin h) Hand ulcers c) Dry eyes or dry mouth i) Mouth ulcers Chest pain d) Rash or changes in skin j) e) Oedema on legs k) Joint pain f) Blood in urine l) Symptoms of Gastro-oesophagial reflux (GERD) : Yes No (If yes put ) i. Indigestion __________ ii. Heartburn __________ iii. Acid-sour taste ____ iv. Belching _ v. Bloating sensation ___ vi. Cough after meals ___ vii. Cough at night times/sleeping ___ Following symptoms may indicate, presence of below mentioned causes or results of ILD a)Weight loss –Almost all symptomatic patients with ILD specially in Rheumatoid arthritis, SLE , Wegener’s granulomatosis, Tuberculosis, Cryptogenic organising pneumonia, Pulmonary Langerhans cell histiocytosis, Malignancy, Allergic granulomatosis of Churg-Strauss. b) Difficulty in swallowing- Esophageal Dysmotility in Systemic Sclerosis c) Dry eyes or dry mouth- Sjogren’s syndrome, Sarcoidosis, Systemic Sclerosis d) Rash or changes in skin- Connective tissue diseases like SLE, R A, Sarcoidosis, Wegener’s granulomatosis,Raynaud’s phenomena, Behcet’s diasease; Maculopapular rash- drug induced, CTD, amyloidosis, gaucher’s disease; Telangictasia: scleroderma; Cutaneous vasculitis; CTD e) Oedema of legs- Congestive heart failure. f) Blood in urine- Glomerulonephritis in Goodpasture’s syndrome, SLE, Wegener’s granulomatosis, Sarcoidosis g)Bruising skin –use of Steroid, vasculitis h)Hand ulcers - Systemic sclerosis, Vasculitis i) Mouth ulcers – SLE, Wegener’s granulomatosis , HIV, Behcet’s diasease j) Chest pain –Pleuritis in Wegener’s granulomatosis ,SLE ,RA k) Joint pain- Connective tissue disease like SLE ,RA , Ankylosing Spondylitis, Wegener’s granulomatosis, Sjogren’s syndrome. l) GERD – occurs in Systemic Sclerosis. Can be a cause of Aspiration pneumonia and chronic cough 3. Has any doctor ever told him (past or known medical history) (put in respective column) Never a) Tuberculosis b) Extra pulmonary Tuberculosis c) Hypertension d) Diabetes e) Coronary heart disease f) Heart failure g) Thyroid disease h) Stroke (CVA) i) Seizure j) Hepatitis A/ B/C k) Hapatitis, other/unknown l) Kidney disease m) Anemia n) Eye inflammation o) Parasites (worms) p) Pneumothorax q) Pleural effusion r) Pneumonia s) Asthma t) Bronchitis u) Sinus disease v) Pulmonary hypertension Before ILD Treatment After ILD treatment Never w)Pulmonary embolism x) Sleep apnoea y) Lung Cancer z) GERD aa) Hiatal hernia ab) Bleeding disorder ac) Rheumatologic disease /collagen vascular disease ad) Vasculitis ae) Raynaud’s phenomenon af) Rheumatoid arthritis, ag) Lupus ah) Scleroderma ai) Mixed connective tissue disease aj) Sjogren’s Syndrome ak) Wegener’s, al) Polymyositis or dermatomyositis am) Bechet’s disease an) Ankylosing spondylitis. ao) overlapping ap) unspecified/ unclear Others i) ii) Before ILD treatment After ILD treatment Various diseases mentioned here are associated with specific type of ILD or starts with complication of therapy of ILD. Therefore please go through the list carefully and indicate the onset of the disease before initiation of treatment of ILD or after it. Common associations are as follows: a) Tuberculosis- associated with bronchocentric granulomatosis. Can cause lung fibrosis and restrictive pattern in PFT b) Extra pulmonary Tuberculosis c) Hypertension – occurs in Systemic Sclerosis, neurofibromatosis, diffuse alveolar hemorrage d) Diabetes-Patients more prone to get tuberculosis, more severe pneumonia and thus complications. Conversely steroids may precipitate diabetes. e) Coronary heart disease-RA, SLE f) Heart failure –Infiltrative diseases like Sarcoidosis , Systemic Sclerosis , Ankylosing Spondylitis, Symptoms and CT picture ( in lying down position ) may mimic ILD. g) Thyroid disease- Autoimmune diseases. Infiltrative disease like Systemic Sclerosis may cause hypothyroidism as well as ILD h) Stroke(CVA) – Vasculitis. Prone for Aspiration pneumonia i) Seizures (Prone for Aspiration pneumonia) - Tuberous Sclerosis, Neurofibromatosis, Gaucher’s disease. Prone for Aspiration pneumonia. j)Hepatitis A/B/C –Chronic active hepatitis causes ILD k) Hepatitis Other unknown l) Kidney diseases-Amylodosis, Goodpasture’s syndrome, Wegener’s granulomatosis, Connective tissue diseases,Sarcoidosis m) Anaemia-Connective tissue diseases ,Gauchers Disease, Cytotoxic drugs like Cyclophosphamide used in treatment of ILD n) Eye inflammation - Sjogren’s syndrome, Wegener’s granulomatosis, Ankylosing Spondylitis, sarcoidosis o) Parasites –Eosinophilic Pneumonias p) Pneumothorax- Systemic sclerosis, RA, Pulmonary Langerhans cell histiocytosis, Pulmonary lymphangioleiomyomatosis. q) Pleural effusion- SLE, RA, Tuberculosis, Pulmonary lymphangioleiomyomatosis. r) Pneumonia- Acute interstitial pneumonia, ARDS. s) Asthma- Eosinophilic pneumonia. t) Bronchitis- Sjogren’s syndrome, inhalation of toxic fumes, organic dust. u) Sinus disease- Wegener’s granulomatosis, Allergic granulomatosis of Churg-Strauss, Sarcoidosis. v) Pulmonary Hypertension- SLE, RA, Systemic sclerosis, Sarcoidosis. w) Pulmonary embolism- chronic PE causes pulmonary hypertension and presents as dyspnoea and hypoxemia. x) Sleep apnoea- causes hypoxemia and eventually pulmonary hypertension. y) Lung cancer- Associated with Asbestosis, Inhalation of smoke of tobacco, polycyclic hydrocarbons, biomass fuel z) GERD- Systemic sclerosis, can cause aspiration pneumonia, common cause of chronic cough. aa) Hiatal hernia-cause of GERD ab) Bleeding disorder-Hermansky- Pudlak syndrome ac) Rheumatologic diseases/ Collagen vascular disease - cause ILD ad)Vasculitis- occurs in diaeases like wegener’s granulomatosis, allergic granulomatosis of Churg and Strauss, SLE, RA, Sarcoidosis etc. ae) Raynaud’s phenomena - Systemic sclerosis, SLE, RA, Dermatomyositis, polymyositis, IPF. af) Rheumatoid arthritis, ag) Lupus ah) Scleroderma ai) Mixed connective tissue disease aj) Sjogren’s Syndrome ak) Wegener’s al) Polymyositis or dermatomyositis am) Bechet’s disease an) Ankylosing spondylitis. ao) Overlapping ap) Unspecified/ unclear Others Domestic environmental factors Duration & Remarks 4. Conditions of his/her house Yes No a) Dusts (visible) ----------------------------------------------------------------Yes No b) Molds(visible) ----------------------------------------------------------------Yes No c) Air conditioner --------------------------------------------------------------No Yes d) Cooler --------------------------------------------------------------------------No Yes e) Birds in home(caged) (Include pigeons, parrot, hen, crow mourning No dove) Yes f) Any changes in house/housing conditions in recent past ---------g) IF YES to changes in housing conditions, how many days, months or years before the cough and/or breathing /chest problems ___________________ a) Dusts (visible) – organic dust :hypersensitivity pneumonitis like Bagassosis, Farmers lungs. Inorganic dust like silica causes ILD b) Molds(visible) – hypersensitivity pneumonitis c) Air conditioner- hypersensitivity pneumonitis ( Air conditioner Lung) d ) Cooler- hypersensitivity pneumonitis (Humidifier lung) e) Birds- hypersensitivity pneumonitis(Birds fancier’s lung) f) Changes in house/housing conditions may cause hypersensitivity pneumonitis due to molds g) To classify them into acute, subacute and chronic hypersensitivity pneumonitis. Duration & Remarks 5. Does the current or past home have/had following Yes No a) Open cooking on fire wood or cow dung -----------------------------Yes No a) Cooking on kerosene stove -----------------------------------------------Yes No b) Cooking on coal -------------------------------------------------------------No c) Cooking with LPG gas ----------------------------------------------------Yes a) Open cooking on fire wood or cow dung-Fumes of acrolein and other aldehydes can cause mucosal irritation and decrease in lung function. To look association with ILD b) Acid fumes cause bronchitis. c) Cooking on coal- inhalation of SO2,NO2, cause chronic bronchitis d) Cooking with LPG gas- to know the frequency of LPG users 6. Previous residential locations (Please list all locations lived for atleast 6 months.) Duration & Remarks a) Urban (District headquarter or higher) ---------------------------Yes No b) Sub urban (Tehsil HQ) ------------------------------------------------------Yes No a) Rural-village (Panchayat HQ or lower) ---------------------------------Yes No Others _____________________________________________________________________________ People living in urban areas are more exposed to vehicular pollution while rural patients are more exposed to molds and many other organic dusts. 7. Work environmental factors : Working outside house No Yes 8. Occupational history: Please include all occupations worked : (include dust, metal, paint, fine particles, etc) List any exposures that you feel might be related to cough/breathing /chest problems and /or not listed above? (see appendix A) Occupation Years worked [from-to] Exposures _________________________ ______________________ ___________________ _________________________ ______________________ ___________________ _________________________ ______________________ ___________________ _________________________ ______________________ ___________________ _________________________ ______________________ ___________________ 9. Exposed to visibly smoky or dusty air? No Yes 10. Use of protective masks while working No Yes Work environmental factors: Any exposure to organic or inorganic dust should be mentioned. Duration of exposure is important for ILD eg. Asbestosis develop after ten years of exposure and Coal Workers Pneumoconiosis develops after 15 to 20 years of exposure. Stone workers are prone to develop silicosis while pipe fitters may develop asbestosis. Hypersensitivity pneumonitis may develop in farmers and carpenters.. Mask may protect from inhaling dust 11. Ever taken substance of abuse such as cannabis, opium or smoked ganza, charas etc? 12. Inhaled tobacco use? (If yes put ) Never Current Ever Tobacco use: Bidi Cigarette Hookah/ Chillum Chewing tobacco Alcohol Yes Ganza, charas Starting age Stopping age Quantity/Day Opiates may cause lung fibrosis. Tobacco smoke may cause chronic bronchitis, fibrosis. 66% to 75% of the IPF patients are smokers. Strong association of smoking is also present in PLCH, DIP, Goodpasture’s syndrome, Respiratory bronchiolitis and pulmonary alveolar proteinosis. No a. b. c. d. e. f. g. h. i. j. k. Name of medicines patient has taken in the PAST Starting date Stopping date l. m. n. o. p. q. r. s. t. u. v. Medication history – Many drugs can cause ILD such as amiodarone, acyclovir, bleomycin, busulphan, methotrexate, chlorambucil, melphalan, methysergide, nitrofurantoin, sulphonamides, aminoglycosides, opiates, hypnotics, mitomycin C, gold, polymyxins. Drugs used in treatment of ILD can also predispose many conditions such as diabetes, increased occurrence of infections etc. 13. b) How many courses of antibiotics were taken in last one year? _______________ 14. Family medical history (biological): grandparents, parents, brothers, sisters, aunts, uncles, first cousins, or children have any of the following lung disease? Yes a) Asthma --------------------------------------------------------------------------------Yes b) Chronic Obstructive Pulmonary disease (COPD) -------------------------------Yes c) Sarcoidosis ----------------------------------------------------------------------------Yes b) Bronchiectasis ------------------------------------------------------------------------Yes c) Pulmonary fibrosis (ILD/IPF) -----------------------------------------------------Yes d) Hypersensitivity pneumonitis ----------------------------------------------------Yes Relation No No No No No No No e) Rheumatologic disease /collagen vascular disease ( i. rheumatoid arthritis, ii. lupus, iii. scleroderma, iv. mixed connective tissue disease, v. Sjogren’s Syndrome, vi. Wegener’s, vii. Polymyositis or dermatomyositis , viii. Bechet’s disease, ix. Ankylosing spondylitis x. Overlapping xi. Unspecified/unclear_ Asthma is genetically transmitted. Familial inheritance also exists in Sarcoidosis, rheumatological diseases, Tuberous sclerosis, neurofibromatosis, Niemann-Pick disease, Gaucher’s disease, HermanskyPudlak syndrome. Familial lung fibrosis may present as different patterns of interstitial pneumonia. Moreover, more than one family member can be affected if residing in close proximity to mines or other areas of dust exposure. Disease Status Route Stopping date Dose Starting date Route Name of medicine patient is taking PRESENTLY Dose 13. a) Medication history: Please include all medicines patient has taken especially those given in Appendix E 15. Physical examination: Pulse ___________ BP _________________ JVP ___________________ Cynosis _______________ Height __________ Weight ____________ BMI _____________________ Face/ Head_______ Eye/ ENT ____________ Skin __________________ Joints __________Clubbing ____________ Bibasillar crackles _______Wheeze _______________ P2 heart) _______ Pallor _______________ Icterus ________________ Lymphademopathy ____ Others _________________________________ Physical examinationFace & skin- Rashes, ulcers in SLE, RA, Systemic sclerosis, Sjogren’s syndrome, Behcet’s disease, Sarcoidosis. Prolonged intake of steroids causes moon facies and easy bruising on skin. Joints – Arthritis and arthralgia in connective tissue diseases, Wegener’s granulomatosis, Sjogren’s syndrome. Clubbing-IPF, Primary biliary cirrhosis, Inflammatory bowel diseases. Bibasillar crackles- most common auscultatory finding in ILD. May also occur in CHF. Pallor- Connective tissue diseases, Gaucher’s disease, result of cytotoxic drugs. Icterus- Hepatitis(infective or drug induced), cirrhosis. Lymphadenopathy- Tuberculosis, HIV, Sarcoidosis, lymphoma, lymphocyatic interstitial pneumonia Salivary gland enlarged: sarcoidosis, lymphocyatic interstitial pneumonia Hepatosplenomegaly: Sarcoidosis,CTD, amyloidosis, lymphocyatic interstitial pneumonia JVP- Cor pulmonale, CHF 16. Investigations (Pulmonary evaluation): A. Spirometry, lung volumes and diffusing capacity (Indian predicted value) Predicted Actual Percentage Post Reversibility% FEV1 FVC FEV1/FVC PEFR Total lung capacity RV DLCO (corrected for hb) KCO B. ABG (while breathing air at sea level ) pH _____ pO2 _____ pCO2 _____ HCO3 _____ C. 6 minute walk test: Test performed while breathing air Yes No Test performed with supplemental Oxygen If Yes No Yes,……….l/min Distance covered in 6 minutes _____ Baseline SpO2 _____ SpO2 immediately after walk ……. Reason for stopping before 6 minutes : breathless …….Fatigue….leg /joint pain ……….. Borg scale dyspnea : Rest ……… At the end of testing …….. D. Fiberoptic Bronchoscopy (When relevant) date_________ Yes No Fiberoptic Bronchoscopy Findings/diagnosis f) Consistent with: alveolar hemorrhage……, proteinosis…, Purulent secretions/infection… Others………. ii) Smear for acid fast bacilli, cultures for M Tb /Myc species _____________ iii) Transbronchial biopsy: specific diagnosis - granuloma ____; malignancy _____infection iv) Consistent with: Sarcoidosis .. Hypersensitivity pneumonitis …, Lymphangiectatic carcinoma…. E. Open /surgical /thoracoscopic lung biopsy: (If performed) Yes No date _________ i)Right lung (upper lobe/middle lobe/lowerlobe) __________Left Lung(upper lobe /lingula/lower lobe ii) Pathology (surgical lung biopsy) Date____________ Yes No UIP Consistent : definite probable possible definitely not Per OFFICIAL DOCUMENT –ATS-ERS-JRS-ALAT (Raghu et al AJRCCM, March 15 2011) F. Chest Xray Recent: bilateral shadows present Chest Xray, old: bilateral shadows present Yes No Yes No date _________ date _________ G. HRCT : 1mm cuts Yes No ;Prone & supine views Yes No , Expiratory views Yes Report: Date of HRCT : _____________________________________________________________________________________ _____________________________________________________________________________________ UIP: Yes No High resolution computed tomography criteria for UIP pattern in Appendix C. Yes No H. Sinus CT (Optional) ___ SINUSITIS INVESTIGATIONS Spirometry and lung volumes- most common presentation in ILD is restrictive pattern with decreased TLC, FRC and RV. FEV1 and FVC are also decreased but FEV1/FVC is normal or increased. Obstructive pattern may also be seen particularly in LAM and Tuberous sclerosis. It also has prognostic value in IPF and NSIP. Diffusion capacity- reduced in ILD. ABG- may be normal or may show hypoxemia and respiratory alkalosis particularly during exercise as revealed after 6MWT. Fiberoptic Bronchoscopy- Bronchoalveolar lavage(BAL) findingsPulmonary alveolar proteinosis: BAL yields a cloudy, effluent that contains large amounts of periodic acid-Schiff–positive lipoproteinaceous material, as evidenced by light microscopy. 5% or more CD1a-positive cells in the BAL fluid is highly specific for the diagnosis of pulmonary Langerhan’s cell histiocytosis. BAL finding diagnose Pulmonary hemorrhage, berylliosis, hypersensitivity pneumonitis, and some pneumoconioses. BAL cell profiles are not diagnostic in idiopathic interstitial pneumonias. Excess of neutrophils: the proportions of which correspond to the extent of reticular change on HRCT No Excess eosinophils (more than 20%): eosinophilic lung disease Lymphocytosis above 15%: NSIP, COP, hypersensitivity pneumonitis, sarcoidosis or other granulomatous lung diseases Surgical lung biopsy- may be needed in cases of IPF, Sarcoidosis, hypersensitivity pneumonitis, asbestosis, lymphangitic carcinoma, PLCH. CXRay – most common presentation is bibasilar reticular pattern. Nodular or mixed pattern are also seen. HRCT – Both inspiratory + expiratory and supine + prone films are required. Slice thickness of 1 to 1.5 mm is required. If clinical features and chest radiograph suggest hilar enlargement, then contrast is also required to delineate mediastinal structures. Sinus CT- may be useful in wegener’s granulomatosis, sarcoidosis. 17. Laboratory tests (relevant): a) CBC: Hb___ TLC_____Platelets___ MCV ___ DLC: N___ L___ E___ M___ B___ b) B Sugar F ___ PP ___ c) Renal : Urea __________ Creatinine __________ d) Liver function tests: Bilirubin _____ SGOT_____ SGPT ___ Hepatitis Bs __Ab__, Hepatitis C__ Ab__ e) Urinalysis: Protein _____ Sugar _____ Cast _____ Pus cells _____ f) Sputum: AFB_____ Fungus _____ g) Collagen vascular disease profile: ANA + -, RF + - , dsDNA + - , Sm + - , RNP + - , Scl 70 + - , SSA + -, SSB + - , CPK + - , Anti Jo-1 + - , ANCA + - ACE _____ EBV titer + Collagen vascular disease profile relevant in workup of ILD? Test Disease Anti nuclear antibody SLE Anti double stranded DNA (ds-DNA) Anti Smith (Sm) RF Non specific Rheumatoid arthritis Anti Ribonucleprotein (RNP) Mixed connective tissue disorder Cytoplasmic antinuclear antibody (c-ANCA) Wegner’s granulomatosis Perinuclear antinuclear antibody (p-ANCA) Microscopic polyangiitis Churg strauss Angiotensin converting enzyme (ACE) Sarcoidosis Anti topoisomerase Systemic sclerosis Anti SSA (Ro) SLE Anti SSB (La) Sjogren’s syndrome h) HIV + i) Lymphocyte transformation testing (metals) ________________________________________ j) Mantoux test (PPD) Optional: k) Echocardiogram (Optional): Date EF __________________ Wall asymmetry _____________________ Estimated systolic PA pressure ____________________ RV dilation/thickness ________________ Besides CBC showing anemia or pancytopenia (Gaucher’s disease, marrow suppression by drugs), RFT and urine examination may show nephritis or nephritic syndrome. LFT should be done for hepatitis, cirrhosis. Peripheral eosinophilia occur in eosinophilic pneumonia, Churg Strauss syndrome and drug reactions. Hypercalcemia occurs in sarcoidosis. ANCA and Anti Basement membrane antibodies are useful in vasculitis. Scl70 in systemic sclerosis and raised CPK in polymyositis and dermatomyositis. ECG may show enlargement of RA,RV. Cardiac echocardiography relevant in work up of DPLD? Cardiac echocardiography is relevant because – Cor pulmonale due to chronic hypoxia Concomitant heart disease 18. Consultant’s impression /opinion _____________________________________________________________________________________ _____________________________________________________________________________________ _____________________________________________________________________________________ A) Suspected ILD diagnosis : Yes No a. Idiopathic Interstitial Pneumonias (IIP) i. UIP ii. NSIP iii.COP iv. LIP v. RB-ILD b. IPF c. Pulmonary fibrosis of unknown cause other than IIP d. Occupational ILD e. Granulomatous diseases eg. Sarcoidosis f. Hypersensitivity pneumonitis g. ILD secondary to collagen vascular disease h. familial ILD i. Other rare ILD given in appendix D) B) Suspect active infection (Active TB or pneumonia) ____________________________________________ C) Other coexisting lung disease ______________________________________________________________ D) Comorbidities ___________________________________________________________________________ 19. Follow up of the patient: Date A. Symptoms: Improved Same Deteriorated B. New symptoms /finding/diagnosis ____________________________________________________________________________________ ____________________________________________________________________________________ C. Investigations: FEV1 FVC FEV1/FVC Predicted Actual Percentage ____________________________________________________________________________________ D. Treatment prescribed ____________________________________________________________________________________ ____________________________________________________________________________________ Date A. Symptoms: Improved Same Deteriorated B. New symptoms /finding/diagnosis ____________________________________________________________________________________ ____________________________________________________________________________________ C. Investigations: FEV1 FVC FEV1/FVC Predicted Actual Percentage ____________________________________________________________________________________ D. Treatment prescribed ____________________________________________________________________________________ ____________________________________________________________________________________
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