An 8-year-old boy with diffuse cystic pulmonary tuberculosis: An intriguing story

Diffuse cystic lung disease due to pulmonary tuberculosis (TB) is rare. We describe an 8-year-old boy who presented with acute onset respiratory distress while on a compliant anti-tubercular treatment for a recently diagnosed pulmonary TB. On clinical examination, hypoxemia, clubbing, and features of the right-sided heart failure were observed. High-resolution computed tomography of the chest revealed extensive cystic lung parenchymal changes with ground glassing and consolidation, and echocardiography indicated the presence of pulmonary arterial hypertension. His treatment included supplemental oxygen, sildenafil, prednisolone, and anti-tubercular drugs. At the two-year follow-up, the patient showed complete clinical recovery and resolution of cysts on the chest X-ray.

Síndrome de Sjögren primario y enfermedad quística pulmonar: reporte de un caso / Primary Sjögren syndrome and pulmonary cysts disease: case report

El síndrome de Sjögren (SS) es una enfermedad inflamatoria, autoinmunitaria crónica de etiología desconocida, caracterizada por infiltración del tejido glandular y extraglandular por linfocitos y células plasmáticas. Presenta manifestaciones glandulares (xeroftalmia y xerostomía) y extraglandulares (50%); v.gr.: pulmonares (11%) ­las más comunes son la enfermedad intersticial linfocítica y la enfermedad quística pulmonar (25%)­; las manifestaciones extraglandulares preceden, muchas veces, a las manifestaciones glandulares; por este motivo su diagnóstico se confunde o se retrasa. Caso clínico: paciente de sexo femenino, 71 años de edad, con antecedentes de hipertensión arterial e hipotiroidismo. Hospitalizada por astenia, tos seca y disnea de medianos esfuerzos. Revisión de aparatos y sistemas: xeroftalmia y xerostomía. Exámenes complementarios: Rx tórax: opacidades difusas redondeadas en ambos campos pulmonares; tomografía simple de tórax: múltiples lesiones quísticas predominantes en los lóbulos inferiores y calcificaciones nodulares. Se amplían los estudios para determinar la etiología: exámenes inmunoquímicos: anti SSA/Ro 200 U/ml (< 20 UE/ ml), anti SSB/La 84,4U/ml (< 20 UE/ml), anticuerpos antinucleares 1/160 (≤ 1/40), factor reumatoideo 49,9UI/ml (< 20 UI/ml). Se sospecha síndrome de Sjogren y realiza sialografía de parótidas que evidencia obstrucción y procesos inflamatorios de los conductos parotídeos. Biopsia de labio inferior: mucosa revestida por epitelio escamoso, edema intercelular y exocitosis linfocitaria. El estroma contiene infiltrado linfomononuclear disperso característico del SS. Una vez confirmado el diagnóstico, se trata con corticoides y tratamiento específico del ojo y boca seca.

Context: Sjögren's syndrome (SS) is an inflammatory, chronic autoimmune disease of unknown etiology, which is characterized by infiltration of glandular and extraglandular tissue by lymphocytes and plasma cells. It presents glandu-lar manifestations (xerophthalmia and xerostomia), as extraglandular (50%). Within these, there are pulmonary manifestations (11%), the most common manifestation being lymphocytic interstitial disease followed by cystic lung dis-ease (25%). Its manifestations often precede the glandular manifestations so that the diagnosis of it is confused or delayed. Case presentation: A 71-year-old female patient with a history of high blood pressure and hypothyroidism. Hospitalized for presenting asthenia, dry cough and dyspnea to the medium efforts. In the review of all systems, she referred xerophthalmia and xerostomia. The following exams were carried out: Rx thor-ax: rounded diffuse opacities in both pulmonary fields, is complemented with simple chest Tomography: Multiple pre-dominant cystic lesions in lower lobes and nodular calcifications. It was decided to expand the studies to determine etiology and see if it is related to its xerophthalmia and xerostomia characteris-tic of SS. Immunochemical tests were performed. The results: anti-SSA / Ro 200 U / mL (< 20 EU / mL); anti SSB / 84.4U / mL (< 20 EU / mL); antinuclear antibodies with fine granular pattern 1/160 (≤ 1/40) Rheumatoid factor 49.9UI / mL (< 20 IU / mL). With the suspicion of Sjogren's syndrome, parotid sialog-raphy is performed, obstruction is evidenced and chronic inflammatory pro-cesses of parotid ducts. Lower lip biopsy: Mucosa lined by squamous epitheli-um with intercellular edema and exocytosis of small lymphocytes. The stroma contains mild scattered lymphomononuclear infiltrate characteristic of SS. Con-firmed diagnosis of SS managed with corticosteroids and specific treatment of eye and dry mouth.

Diagnosis and treatment of cystic lung disease

Cystic lung disease (CLD) is a group of lung disorders characterized by the presence of multiple cysts, defined as air-filled lucencies or low-attenuating areas, bordered by a thin wall (usually < 2 mm). The recognition of CLDs has increased with the widespread use of computed tomography. This article addresses the mechanisms of cyst formation and the diagnostic approaches to CLDs. A number of assessment methods that can be used to confirm CLDs are discussed, including high-resolution computed tomography, pathologic approaches, and genetic/serologic markers, together with treatment modalities, including new therapeutic drugs currently being evaluated. The CLDs covered by this review are lymphangioleiomyomatosis, pulmonary Langerhans cell histiocytosis, Birt-Hogg-Dube syndrome, lymphocytic interstitial pneumonia/follicular bronchiolitis, and amyloidosis.

Diagnostic value of MR imaging in congenital cystic lung disease:comparative study with ultrasound / 实用放射学杂志

Objective To investigate the value of MRI in diagnosis of congenital cystic lung disease.Methods The MRI was per-formed in 105 fetuses with congenital cystic lung disease.Subjects were classified into two groups including group A (the gestational age ranged from 12-20 w)and group B (the gestational age>20 w).The MRI and ultrasonography characteristics,as well as path-ologic diagnosis were compared.Results Among 105 cases,there were 80 cases of congenital cystic adenomatoid malformation (CCAM)(including 23 cases of typeⅠ,38 cases of typeⅡ and 1 9 cases of typeⅢ),18 cases of bronchopulmonary sequestration (BPS),5 cases of congenital lobar emphysema,1 case of bronchogenic cyst,and 1 case was BPS with CCAM.In group A,MRI can diagnose the majority of the anomalies (41/45),ultrasonography can diagnose anomalies of 39 cases.There was no significant differ-ence of diagnostic accuracy between the two methods (P >0.05).In group B,compared with ultrasonography,MRI could diagnose more cases accurately (58 vs 52),with statistical significance (P 20 w compared with ultra-sonography.

Analysis of reports on orphan lung diseases in Korean children / 소아과

PURPOSE: Orphan lung diseases are defined as lung diseases with a prevalence of 1 or less in 2,000 individuals. Despite an increase in the numbers of patients with such diseases, few studies on Korean children have appeared. To obtain epidemiologic and demographic data on these diseases, we systematically reviewed reports on pediatric orphan lung diseases in Korea over the last 50 years. METHODS: We reviewed 223 articles that have appeared since 1958 on orphan lung diseases in Korean children. These articles described a total of 519 patients aged between 0 and 18 years. We classified patients by year of publication, diagnosis, geographic region, and journal. RESULTS: Of 519 patients, 401 had congenital cystic lung diseases and 66 had bronchiolitis obliterans. About 80% of patients were described in reports published in three journals, Pediatric Allergy and Respiratory Disease (Korea), the Korean Journal of Pediatrics, and the Korean Journal of Thoracic and Cardiovascular Surgery, in which papers on 157 (30.2%), 138 (26.6%), and 111 (21.4%) patients appeared, respectively. The frequency of publication of case reports has increased since 1990. Of the 519 patients, 401 (77.3%) were from Seoul/Gyeonggi-do and 72 (13.9%) from Busan/Gyeongsangnam-do. CONCLUSION: The prevalence of pediatric orphan lung disease has increased since 1990, and some provinces of Korea have a higher incidence of these diseases than do others. Studies exploring the incidence of pediatric orphan lung diseases in Korea are needed for effective disease management.

Síndrome de Sjögren primario con neumonía intersticial linfocítica y enfermedad quística pulmonar / Primary Sjögren's syndrome with lymphocitic interstitial pneumonia and cystic pulmonary disease

El síndrome de Sjögren primario (SSP) es una exocrinopatía autoinmune crónica caracterizada por infiltración linfocítica de tejido glandular y extraglandular; el compromiso pulmonar es heterogéneo; el objetivo del presente estudio es informar el primer caso en Colombia de SSP con neumonía intersticial linfocítica y enfermedad quística pulmonar, plantear diagnósticos diferenciales, abordaje diagnóstico y alternativas terapéuticas.

The primary Sjögren's syndrome (PSS) is a chronic autoimmune exocrinopathy characterized by lymphocytic infiltration of glandular and extraglandular tissue, the lung involvement is heterogeneous; the aim of this study is report the first case in Colombia of PSS with lymphocytic intersticial pneumonia and cystic pulmonary disease discuss differential diagnosis and the initial diagnostic and therapeutic work up.

A case of localized persistent interstitial pulmonary emphysema

Interstitial pulmonary emphysema is a well-documented complication of assisted mechanical ventilation in premature infants with respiratory distress syndrome. Localized persistent interstitial pulmonary emphysema (LPIPE) confined to a single lobe was incidentally presented in a 4-day-old female infant. This patient was a normal full-term baby with no respiratory distress symptom and no experience of assisted mechanical ventilation. Chest radiograph showed radiolucent area in right lower lobe zone, which needed differential diagnosis from other congenital lesions such as congenital cystic adenomatoid malformation and congenital lobar emphysema. CT scan showed irregular-shaped air cystic spaces and pathologically, cystic walls primarily consisted of compressed lung parenchyma and loose connective tissue intermittently lined by multinucleated foreign body giant cells.