AN EVALUATION OF DYNAMIC PULMONARY FUNCTION TESTS IN SICKLE CELL ANAEMIA IN RAIPUR DISTRICT, CHHATTISGARH Karishma Singh*, D Sarkar**

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1 AN EVALUATION OF DYNAMIC PULMONARY FUNCTION TESTS IN SICKLE CELL ANAEMIA IN RAIPUR DISTRICT, CHHATTISGARH Karishma Singh*, D Sarkar** *Assisan Professor, **Associae Professor&Head Of Deparmen, Deparmen of Physiology, P. JNM Medical College,Raipur,Chhaisgarh, Absrac: Background and Objecives: Sickle cell anaemia is a life-long haemaological disorder characerized by red blood cells ha assume an abnormal, rigid, sickle shape. I is he mos widespread and numerically he mos imporan haemoglobinopahy in he world oday. The sickle cell disease (SCD) is commones monogenic disorder in India, affecs all he major organs of he body.impairmen of pulmonary funcion is a common complicaion of SCD. Hypoxaemia is he hallmark of pulmonary abnormaliy in SCD paiens of all age groups and is said o resul from he combined effecs of perfusion and diffusion defec. Hence presen sudy was carried ou o deermine he difference in lung volumes beween male and female sickle cell disease paiens ( ),sickle cell rai paiens(hb AS) and compare wih normal conrols ( HbAA)non sicklers. Mehod: A cross secional sudy was done in 50 cases of SCD (22& 28) and age and sex mached normal 50 HbAA conrols. From he various measured pulmonary funcion es( PFT )parameers Forced Vial Capaciy(FVC),Forced Expiraory Volume in 1 sec(fev1), Peak Expiraory Flow Rae(PEFR),Forced Expiraory Flow during 25%-75% of Expiraion(FEF25%-75%),Forced Expiraory Volume Raio(FEV1/FVC) were seleced for he sudy. The daa colleced was subjeced o saisical analysis involving compuaion of Mean, Sandard deviaion, Independen T es. Resul: The resuls revealed ha subjecs wih SCD had significanly lower mean FVC, FEV1, PEFR, FEF25-75% and FEV1/FVC compared o he conrols. The lung funcion indices were lower in females han males of he sickle cell paiens. This work also showed ha he difference in FEV1, PEFR, FEF25%-75%,FEV1/FVC beween and was saisically significan bu he difference in FVC beween and urned ou o be saisically non-significan. Conclusion: The resul of his work suggesed ha Pulmonary funcion es differs significanly in subjecs wih SCD compared wih mached conrols of a similar age and gender. I is indicaive of mixed paern (boh resricive and obsrucive) lung impairmen in sickle cell anaemia. Key Words: Pulmonary Funcion Tes, Sickle Cell Trai, Sickle Cell Disease,Forced Vial Capaciy,Forced Expiraory Volume in 1 sec, Peak Expiraory Flow Rae, Forced Mid Expiraory Flow 25%-75%, Forced Expiraory Volume Raio. Auhor for correspondence: Dr. Karishma Singh,M.D, Assisan Professor, Deparmen of Physiology, P.J.N.M Medical College, Raipur, Chhaisgarh, India. E- mail: karishma_s22@yahoo.com Inroducion: Sickle cell anaemia is an uncompensaed haemolyic anaemia in which a markedly shorened overall RBC survival is insufficienly balanced by increased eryhropoiesis o mainain he normal levels of oal RBCs and Hemoglobin concenraion. I is mos widespread and imporan haemoglobinopahy in he world oday. The sickle cell gene was described in India in he ribal populaion in he souh[1] bu furher repors indicaed he high prevalence among many disrics of Orissa, Chhaisgarh, Madhya Pradesh, Maharashra and no only affecing he ribal bu also non-ribal & many oher backward cases [2].Chhaisgarh has he larges ribal populaion in he counry and i lies in he sickle cell bel[2,6]. The sickle cell muaion resuls from a single nucleoide change GAG->GTG in he 6h codon of exon 1 of bea globin chain[3]. Consequenly, he normal Gluamic acid of bea 6 is replaced by Valine, hus leading o he formaion of sickle Hb (HbS) causing a complex disease involving muliple organs. The inheriance of SCD obeys he principle of Mendelian inheriance[4].when one paren is heerozygous for he sickle cell gene & oher is normal, he offspring would have an equal chance of having eiher sickle cell rai AS or a normal AA genoype. If boh have AS here is a 1 in 2 chance of offspring having AS& 1in 4 chance of having normalaa or SCD SS. The clinical course of SCD is puncuaed by complicaions or crisis. Impairmen of pulmonary In J Basic Appl Physiol., 4(1),

2 funcion characerised by airway obsrucion, resricive lung disease, abnormal diffusion capaciy& hypoxaemia[5] is a common complicaion of SCD. Hence he presen sudy was underaken o evaluae he PFT in cases of SCA visiing PT.JNM MEDICAL COLLEGE and DR BRAM HOSPITAL, RAIPUR, CHHATTISGARH for invesigaions. Maerial and Mehods: Afer aking ehical clearance from scienific commiee of P.J.N.M Medical College, Raipur, Chhaisgarh, he presen cross-secional sudy was conduced in he Dep. of PHYSIOLOGY & CENTRE FOR GENETIC DISEASE & MOLECULAR BIOLOGY in he Dep. of BIOCHEMISTRY a P.J.N.M.MEDICAL COLLEGE, RAIPUR, C.G from A oal of 50 cases of SCD (22 & 28 ) from years of age were seleced. Inclusion Crieria: 1. All he subjecs were non-smokers and none had received blood ransfusions wihin he pas 3 monhs. 2. Normal healhy non-sicklers were seleced as conrols. 3. Consen o paricipae in he sudy. Exclusion Crieria: 1. Subjecs wih hisory of malignancy, pulmonary Tuberculosis, Diabees. Mellius, Hyperension, chronic lung disease, acue ches syndrome and respiraory infecion 2weeks prior o spiromery were excluded from he sudy. 2. Paiens wih Hb <8gm/dl were no included in sudy because of compensaed anaemia. 3. Smokers Sickling Tes: The subjecs were evaluaed for sickling by Sickling Tes (Sodium Mea bisulphide Slide Tes) & posiive resuls were confirmed for rai AS or disease SS by Cellulose Aceae(Hb)Elecrophoresis. Subjecs were divided ino 3 groups. Group A: Homozygous () sickle cell disease- Included 22 paiens Group B: Heerozygous ()-sickle cell rai Included 28 paiens Group C: Normal Conrols (HbAA)-Included 50 subjecs of mached age and sex group. Spiromery (Lung Funcion Tes): Deail clinical hisory of he subjecs was aken. Afer aking informed consen, PFT (spiromery) was performed using compuerized Spiromeer HELIOS-501(RMS, Chandigarh, INDIA).All he ess were done a he same ime of he day o avoid diurnal variaion wihou any igh clohing which subsanially resrics full ches and abdominal expansion. The whole procedure was explained and demonsraed o he subjecs hen spiromery performed. They were allowed o do enough pracice, as lung volume depends on subjec making a maximal volunary effor. The subjec was made o si holding he elecronic spiromeer in fron wih he mouh piece of Spiromeer a he level of his lips. The nosrils were closed wih nose clip. The subjec was hen asked o ake maximum deepes possible inspiraion and hold i, and hen close lips around he mouh piece so as o avoid escape of any air and expire forcefully and as fas and long as much as possible ino he mouh piece. By doing his of FVC and is componens were obained. The Lung funcion es parameers were inerpreed according o guidelines for measuremen of respiraory funcion of American Thoracic Sociey and he Associaion of Respiraory Technicians and Physiologiss. Gas volumes and flow were correced o Body Temperaure and Pressure Sandards (BTPS) auomaically by he insrumen.50 subjecs were compared wih 50 normal conrols of mached age and sex groups. Heigh &weigh were also measured. Saisical analysis: The resul were expressed mean ± sandard deviaion and Independen Sample T es as was applied o calculae he level of significance. A p- of 0.05 or less was considered Saisically significan. Resul: Following observaions were made from he sudy of PFT in 50 normal conrols, 28 and 22 cases. In HbAA group-maximum number of subjecs were in he age group years in boh he sexes. In group- Maximum number of cases were in he age group years in boh sexes. In J Basic Appl Physiol., 4(1),

3 In group- Maximum number of cases were in he age group years in boh sexes. Table 1 shows ha he Mean s of FVC, FEV1, PEFR,FEF25%-75% and FEV1/FVC were less in and han heir normal mached conrols in boh he sexes. Also FVC, FEV1, PEFR, FEF25%-75% and FEV1/FVC in were less han group in boh he sexes. Table 2 shows ha Mean s of FVC,FEV1,PEFR,FEF25%-75% and FEV1/FVC were found o be significanly lower han normal conrols, bu he difference in FVC beween and urned ou o be saisically nonsignifican whereas he difference in PEFR, FEF25%- 75%, FEV1/FVC beween and was saisically significan. Table: 1 Sudy variables of PFTs (FVC, FEV1,PEFR, FEF25%-75%, FEF/FVC ) beween males and females of Normal Conrol groups and Sickle Cell Paiens FVC FEV1 PEFR FEF25-75% FEV1/FVC CATEGORY SEX MEAN±SD MEAN±SD MEAN± SD MEAN±SD MEAN±SD (n=33) (n=17) (n=17) (n=11) (n=12) (n=10) 3.66± ± ± ± ± ± ± ± ± ± ± ± ± ± ± ± ± ± ± ± ± ± ± ± ± ± ± ± ± ±15.90 In J Basic Appl Physiol., 4(1),

4 Table: 2 Comparison of FVC,FEV1, PEFR, FEF25-75% and FEV1/FVC in differen sudy group CATEGORY FVC FEV1 PEFR FEF25-75% FEV1/FVC 3.32± ± ± ± ± ± ± ± ± ± ± ± ± ± ± ± ± ± ± ± ± ± ± ± ± (p>.05) 2.82± ± ± ± ± p <.05 is saisically significan, p >.05 is saisically no significan Discussion: Wih improved supporive care he median age of survival has risen o 42 years for men and 48 years for women[8].as survival ino adulhood has become more common in subjecs wih SCD, here has been an increased incidence of chronic organ failure. Lung is among he major organs involved in SCD[5,7]&pulmonary manifesaions are he leading cause of morbidiy and moraliy in paiens wih SCD[8,9]. Pulmonary funcion ess are useful in assessing he funcional saus of he respiraory sysem boh in physiological and pahological condiions. Spiromery is also he gold sandard for he diagnosis, assessmen, and monioring of COPD. Sickle Cell Trai were found o be more common han Sickle Cell Disease. This is consisen wih BEET,LEHMAN[1] who in heir sudies recorded he higher frequency of SCT as compared o SCD.This migh be due o deah of SCD paiens before he age of sample populaion. FEMI-PEARSE e al[10] repored decreased vial capaciy in paiens wih SCD bu no airway obsrucion. In his sudy FVC, FEV1, FEV1/FVC,FEF 25%-75%,PEFR were reduced in SCD paiens especially afer he age of 20 years. This can be due o changes in lung compliance developed afer repeaed infecions& embolic episodes. MILLER& SERJEANT[11] observed significan reducion in FEV1&FVC of SCD paiens as compared o normal prediced s. This is comparable o he sudy conduced by PIANOSI e al [12] & he presen sudy.powars e al [5]repored 28 cases of Sickle Cell Chronic Lung Disease wih progressive deerioraion of pulmonary funcions in each sage of SCCLD.SCCLD is a prime conribuor o moraliy in young adul paiens wih SCD. In presen sudy, here was a progressive decrease in FVC,FEV1, FEV1/FVC,PEFR andfef25%-75%wih advancing age (afer 20 years) in boh sexes of SCD paiens. This is comparable o he sudy done by PLATT e al[8], MILLER&SERJEANT[11],PIANOSI e In J Basic Appl Physiol., 4(1),

5 al[12]and BOWEN e al[13]. PIANOSI e al[12] observed eiher a resricive or an obsrucive paern of PFTS in a small group of children wih SCD.They used FEF 25%-75% as an indicaor of small airway obsrucion.this is comparable wih he presen sudy and he sudy done by Koumbourlis e al[14]. BOWEN e al [13] repored highly significan reducion in PEFR in children wih muiple episodes of acue ches syndrome.the abnormal s for PEFR are likely o resul from he changes in he lung compliance. This is consisen wih presen sudy. Conclusion: By his sudy i is concluded ha sicklers show a significan reducion in PFT s compared o non sicklers indicaive of boh obsrucive and resricive paern of lung diseases.in obsrucive pulmonary disorder,he hallmark is reducion in FEV1,PEFR,FEF25%-75% and FEV1/FVC%.Also,in resricive impairmen,hallmark is reducion in FVC so i is mixed pulmonary impairmen in sickle cell anaemia.this will help in diagnosis,reamen and prevenion of Sickle Cell Chronic Lung Disease. Acknowledgmen: Sincere Thanks o all he paricipans of his sudy for heir cooperaion. Auhor would also like o hank MRS NEERJA SINGH M.SC Bioechnology,CRC, TATA MEMORIAL HOSPITAL, MUMBAI for her suppor, ime and energy. 7. Houp HM,Moore GW,Bauer TW,e al.the lung in SCD.Ches 1982;81: Pla OS,Brambilla DJ,Rosse WF,e al.moraliy in SCD.Life expecancy & risk facors for early deah.n Engl J Med 1994;330: Thomas AN,Serjean GR.Causes of deah in SCD in Jamaica.BMJ 1982;285: Femi-Pearse,e al.pulmonary funcion sudies in SCD.J Appl Physiol 1970;28: Miller GJ,Serjean GR.An assessmen of lung volumes &gas ransfer in sickle anemia.thorax 1971;26: Pianosi P,e al. Pulmonary funcion abnormaliies in childhood SCD.J Pediaric 1993;122: Bowen EF,e al.peak expiraory flow rae and acue ches syndrome in homozygous SCD.Arch.Dis Child 1990;65: Koumbourlis AC,Zar HJ,e al.prevalence and reversibiliy of lower airway obsrucion in children wih cell disease.pediaric 2001;138: Disclosure: No conflics of ineres, financial, or oherwise are declared by auhors References: 1. Lehman H,Cubush MC.Sickle cell rai in Souhern India.Br Med J 1952; i: KarBC,Devis,Dash KC,DasM.The sickle cell gene is widespread in India.Trans.Roy Soc.Trop.Med.Hyg. 1987;81: Ingram VM.Gene Muaions in human Hb: he chemical difference beween normal & sickle Hb. Naure 1957; 180: Huck JG.Sickle cell anemia.john Hopk Hosp.Bull 1923;34: PowarsD,Weidman JA,e al.sickle cell chronic lung disease:prior morbidiy& risk of pulmonary failure.med 1988;67: Ghage SG,Pradhan PK,Agrawals.Hb in Kurmi communiy of MP,a preliminary repor.ind J Med Res 1977;66: In J Basic Appl Physiol., 4(1),