ABSTRACT
La enfermedad pulmonar interstical crónica es frecuente en lactantes y comprende un grupo heterogéno de enfermedades con patrón histológico semejante. Se presenta el caso de una lactante que inició su sintomatología en el período neonatal, con neumonías y atelectasias repetidas, asociadas a transtornos de la deglución con aspiración hacia la vía aérea. El estudio radiológico de tórax demostró infiltrados intersticiales bilaterales persistentes. La biopsia pulmonar fue compatible con una neumonía crónica interstical histiocitaria. Se trató con oxigenoterapia permanente, corticoides inhalatorios, broncodilatadores, kinesiterapia respiratoria, prednisona oral, que luego se cambió a cloroquina, la cual se mantiene hasta la fecha (tiempo de admini9stración 34 meses). La evolución clínica y radiológica ha sido favorable, lográndose la suspensión de la oxigenoterapia luego de un año de uso. Después del primer año de tratamiento no ha presentado neumonías y no han aparecido efectos secundarios por el uso prolongado de cloroquina. Su desarrollo pondoestatural no ha sido adecuado ya que es portadora de una genopatía aún no precisada, sin relación con la enfermedad pulmonar
Subject(s)
Humans , Infant , Female , Lung Diseases, Interstitial/complications , Lung Diseases, Interstitial/diagnosis , Beclomethasone/therapeutic use , Chloroquine/therapeutic use , Cytomegalovirus Infections/etiology , Gastrostomy , Lung Diseases, Interstitial/therapy , Oxygen Inhalation Therapy , Physical Therapy Specialty , Prednisone/therapeutic use , Deglutition Disorders/surgery , Deglutition Disorders/etiologyABSTRACT
Background: Primary ciliary dyskinesia is characterized by a congenital alteration of the ciliary ultrastructure and function. As a consequence, their respiratory tract sweeping action is lost and recurrent respiratory infections ensue. Aim: To analyze a clinical series of patients with primary ciliary dyskinesia, their clinical and laboratory features. Patients and methods: a retrospective review of patients with primary ciliary dyskinesia seen a university hospital, between 1994 and 1998. Bronchial biopsies were obtained with 3.6 mm diameter Olympus fibrobronchoscope, using a cayman type forceps. Ultrastructural alterations of respiratory tract ciliated cells were recorded. Results: six patients (four male) aged 9 months to 13 years old were reviewed. Three patients had situs inversus. All had repeated bouts of obstructive bronchitis and pneumonia, five had sinusitis, four atelectasis, three recurrent otitis and three had bronchiectasis. Cystic fibrosis and immunological alterations were ruled out in five children. Ultrastructural analysis revealed absence of dynein arms in three cases, absence of the internal dynein arm in one, additional peripheral microtubules and absence of dynein arms in one case. Conclusions: primary ciliary dyskinesia must be considered in the differential diagnosis of recurrent respiratory infections. Ultrastructural analysis of ciliary structure can be done in bronchial biopsies obtained through bronchoscopy