title: A case of primary ciliary dyskinesia with pulmonary arterial hypertension responding to oral sildenafil.
abstract: Primary ciliary dyskinesia (PCD) is an autosomal recessive heterogeneous group of conditions with variable clinical findings like recurrent respiratory tract infections, bronchiectasis, situs inversus, singly or in various combinations. Development of Pulmonary arterial hypertension can be a late complication of this disease. Here we present a case of PCD with recurrent respiratory tract infections, bronchiectasis and severe PAH, who responded to treatment with Oxygen, IV broad spectrum antibiotics and oral sildenafil.
authors.affiliation: Department of cardiology, ICVS, SSKMH, Kolkata.
authors.forename: Goutam
authors.initials: G
authors.lastname: Datta
authors.forename: Dipankar Ghosh
authors.initials: DG
authors.lastname: Dastidar
authors.forename: Somnath
authors.initials: S
authors.lastname: Kundu
authors.forename: Brita
authors.initials: B
authors.lastname: Datta
authors.forename: Nandita Ghosh
authors.initials: NG
authors.lastname: Dastidar
authors.forename: Amal
authors.initials: A
authors.lastname: Bannerjee
mesh_terms: D000328:Adult; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D001987:Bronchiectasis / Q000150:complications / Q000000981:diagnostic imaging*; D002316:Cardiotonic Agents / Q000627:therapeutic use; D002925:Ciliary Motility Disorders / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy*; D004071:Digitalis Glycosides / Q000627:therapeutic use; D065627:Familial Primary Pulmonary Hypertension; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications* / Q000188:drug therapy; D008297:Male; D010102:Oxygen Inhalation Therapy; D010879:Piperazines / Q000627:therapeutic use*; D011687:Purines / Q000627:therapeutic use; D012008:Recurrence; D012141:Respiratory Tract Infections / Q000175:diagnosis / Q000628:therapy; D000068677:Sildenafil Citrate; D013450:Sulfones / Q000627:therapeutic use*; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D014665:Vasodilator Agents / Q000627:therapeutic use*
chemical_list: D000900:Anti-Bacterial Agents; D002316:Cardiotonic Agents; D004071:Digitalis Glycosides; D010879:Piperazines; D011687:Purines; D013450:Sulfones; D014665:Vasodilator Agents; D000068677:Sildenafil Citrate