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Prevalence and characteristics of hepatopulmonary syndrome in children with cirrhosis in southern Brazil

Ceza, Marília R.a; Garcia, Eduardob; Anselmi, Carlos E.b; Epifanio, Matiasa; Melere, Melina U.a; Ferreira, Cristina T.a; Steinhaus, Cintiaa; Coral, Gabriela P.b

European Journal of Gastroenterology & Hepatology: January 2019 - Volume 31 - Issue 1 - p 10–15
doi: 10.1097/MEG.0000000000001207
Original Articles: Hepatology

Background Hepatopulmonary syndrome (HPS) is defined as a triad characterized by arterial deoxygenation, intrapulmonary vascular dilatations (IPVDs), and liver disorder. The aims of this study were to assess the prevalence of HPS in children with cirrhosis, the clinical characteristics of patients with HPS, and the tests used for the diagnosis of IPVD.

Patients and methods This was a prospective, cross-sectional study of 40 children with cirrhosis (median age: 44 months). Investigations of HPS included arterial blood gas analysis, contrast-enhanced transthoracic echocardiography (CE-TTE), and perfusion lung scanning using technetium-99m-labeled macroaggregated albumin (99mTc-MMA). Patients’ clinical characteristics (age, etiology of cirrhosis, and severity of hepatopathy) were assessed. HPS was defined as liver disease; alveolar–arterial oxygen gradient of at least 15 mmHg and/or partial pressure of arterial oxygen less than 80 mmHg; and detection of IPVD by CE-TTE or 99mTc-MMA scanning. Statistical significance was indicated by a P value less than 0.05.

Results The prevalence of HPS was 42.5% (17/40). Eight patients had moderate HPS (47%) and two patients had severe HPS (12%). In bivariate analysis, biliary atresia (P=0.033) and median age (10 months; P=0.005) were associated with HPS. In multivariate analysis, only age remained statistically significant (prevalence ratio=0.99; 95% confidence interval=0.98–0.99; P=0.010). Sixteen patients with HPS had IPVD detected by CE-TTE (94.1%) and six patients had IPVD detected by 99mTc-MMA scanning (35.3%), with no significant agreement between these methods (κ=−0.12; P=0.163).

Conclusion HPS is a common complication of cirrhosis in children. A combination of clinical and imaging criteria should be used to diagnose HPS.

aDepartment of Pediatrics, Pediatric Gastroenterology and Liver Transplantation Unit, Hospital Santo Antônio

bIrmandade Santa Casa de Misericórdia de Porto Alegre, Porto Alegre, Brazil

Correspondence to Marília R. Ceza, MD, Department of Pediatrics, Pediatric Gastroenterology and Liver Transplantation Unit, Hospital Santo Antônio, Irmandade Santa Casa de Misericórdia de Porto Alegre, Avenida Independência, 155, CEP 90240-510 Porto Alegre, 90020-090 Rio Grande do Sul, Brazil Tel: +55 51 3214 8647; fax: +55 51 3214 8080; e-mail:

Received March 9, 2018

Accepted May 28, 2018

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