Pulmonary Physiology |

Patterns and Prevalence of Pulmonary Funtion Test (PFT) Abnormalities in Children and Adolescents With Sickle Cell Disease (SCD) FREE TO VIEW

Ameer Rasheed*, MD; Viswanath Vasudevan, MD; Farhad Arjomand, MD
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The Brooklyn Hospital Center, Brooklyn, NY

Chest. 2012;142(4_MeetingAbstracts):799A. doi:10.1378/chest.1389720
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SESSION TYPE: Physiology/PFTs/ Rehabilitation Posters

PRESENTED ON: Wednesday, October 24, 2012 at 01:30 PM - 02:30 PM

PURPOSE: Cardiopulmonary complications such as acute chest syndrome (ACS) and vasoocclusion induced pulmonary infarction are prevalent in SCD. These result in pulmonary fibrosis, pulmonary hypertension, and high output cardiac failure. Abnormal PFTs have been reported in adults (upto 90%) with SCD. These include Restriction (fibrosis from repeated ACS), obstruction or mixed obstructive-restrictive physiology and isolated reduction in DLCO. It is important for the clinicians to recognize SCD related PFT abnormalities to avoid errors in diagnosis and misguided therapy. The aim of the study is to report prevalence and patterns of abnormal PFT finding in children and adolescents.

METHODS: 30 patients were included with median of 14 years (range: 10-19). 15 were males and 15 were females. Patients were subclassified into five groups: obstructive, restrictive, mixed obstructive/restrictive physiology, isolated low DLCO, or normal. ATS/ERS 2005 interpretation strategy was used to diagnose restriction obstruction based on NHANES-II reference values. The DLCO were not corrected for Hg or alveolar volume.

RESULTS: Normal PFTs were present in 22/30 (73%). Obstructive physiology was noted in 3, Restrictive physiology in 4 (all with reduced VC but normal TLC). DLCO was normal in all except one though all were anemic with a median Hb of 9 (4.9-11.1 mg/dl). Chest X-Ray was available in 17 patients; normal in 3 and abnormal in 14 (82%); Cardiomegaly was noted in 8 (57%); Prominent Pulmonary Artery (Pulmonary Hypertension) in 6 (43%); 2 patient had both cardiomegaly and prominent vascular marking.

CONCLUSIONS: Unlike adult patients, most children with SCD had normal PFT including normal DLCO despite severe anemia. The low incidence of PFT is abnormality is attributed to fewer episode of sickle cell crises, vasoocclusive or ACS. The normal DLCO is explained by high blood volume from high cardiac output in chronic anemia.

CLINICAL IMPLICATIONS: Majority of Children and Adolescents have normal PFT’s. DLCO could be normal even in the presence of severe anemia due to high cardiac output state. Longitudinal PFT’s in children are helpful in monitoring the progression of SCD.

DISCLOSURE: The following authors have nothing to disclose: Ameer Rasheed, Viswanath Vasudevan, Farhad Arjomand

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The Brooklyn Hospital Center, Brooklyn, NY




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