Different features of lung involvement in Niemann-Pick disease and Gaucher disease

SummaryBackgroundNiemann-Pick disease (NPD) and Gaucher disease (GD) are well-known lysosomal storage diseases. Respiratory system involvement is an important cause of morbidity and mortality in patients with NPD and GD.ObjectivesWe tried to assess the clinical, radiological, and histological features of GD and NPD patients with lung involvement.MethodsWe reviewed medical history, physical examination, radiological, and histological data of 10 NPD and 7 GD patients.ResultsThe most common respiratory symptoms were recurrent lung infection and dyspnea. Although lung examination results in 6 NPD patients were normal, they had lung involvement; 3 patients were diagnosed as NPD directly via lung biopsy during investigation of recurrent lung infection or interstitial lung disease. All GD patients but 1 had respiratory system symptoms at the time of diagnosis. Hepatopulmonary syndrome was present in 4 GD patients. A ground-glass pattern and atelectasis were 2 important high-resolution computed tomography features in the NPD and GD patients. Flexible bronchoscopy and bronchoalveolar lavage were used for emergency extraction of bronchial casts in 1 NPD patient.ConclusionsLung involvement in NPD and GD patients should be included in the differential diagnosis of interstitial lung disease. Besides interstitial appearance on HRCT, atelectasis related to bronchial cast and bronchiectasis are other radiological findings in these group of patients. Analysis of bronchoalveolar fluid and lung biopsy provide very important clues for diagnosis. Hepatopulmonary syndrome is an important vascular complication observed in GD patients

Çocukluk Çağı Diffüz Parankimal Akciğer Hastalıkları: Tanı, Tedavi ve Izlem

Childhood diffuse parenchymal lung diseases are a group of heterogeneous disorders that have common histologic features with a chronic course and associated with high morbidity and mortality. To date, there is not an exact consensus about the terminology, classification, therapy and follow up of this disease because of its rarity and wide clinical spectrum. In this study, clinical features, laboratory, radiological and histopathological findings, therapy and outcome of 130 pediatric DPLD cases who were followed up at Hacettepe University Pediatric Pulmonology Department between 1974 and June 2012, are retrospectively evaluated. We also tried to classify these patients according to the last classification scheme recommended in the literature. All clinical and laboratory features and radiological findings at first admission, bronchoscopy and BAL findings, histopathological investigations of any organ biopsies, given therapies, therapy duration and outcome of all patients are recorded. Our childhood DPLD series is the largest one in the literature that’s reported from a single center. Sixteen distinct disease groups are discussed under the DPLD title. Fifteen patients, older than 2 years of age, with idiopathic interstitial pneumonia could not be classified in the last classification scheme recommended by Children’s Interstitial Lung Disease (chILD) Network. Also our patients with idiopathic pulmonary hemosiderosis and pulmonary alveolar microlithiasis stayed out of this classification. A newly defined congenital lung disease, diffuse chondroid malformation of the lung, was not defined in the available classification scheme. These results indicate that a new classification system involving all these patients is required. Our patients’ mean age at diagnosis was 7,7 years. The most frequent symptoms were cough and dyspnea in all disease groups. The diagnosis of patients were delayed between median 5,5 months and 3 years in all groups; and these patients were treated as if they had recurrent pneumonia, tuberculosis and asthma. As the presenting clinical manifestations are non-specific, every child, with a history of normal birth, who has signs and symptoms suggesting DPLD for more than 3 months should be investigated in terms of DPLD.Çocukluk çağı diffüz parankimal akciğer hastalıkları, ortak histolojik bulguları olan, çoğunlukla kronik seyreden ve yüksek morbidite ve mortaliteye sahip, solunum sistemine ait özellik gösteren bir hastalıklar grubudur. Çok nadir görülen hastalıklar olduğundan halen hastalığın sınıflandırılması, tanı, tedavi ve izlemi konusunda belirlenmiş protokoller yoktur. Bu çalışmada, Hacettepe Üniversitesi Çocuk Göğüs Hastalıkları Bilim Dalı’nda, kurulduğu 1974 yılından Haziran 2012’ye kadar izlenmiş olan 130 pediatrik DPAH vakasının klinik özellikleri, laboratuvar, radyolojik ve histopatolojik bulguları, tedavi ve izlem sonuçları retrospektif olarak değerlendirilmiştir. Ayrıca bu hastalar literatürdeki mevcut önerilere göre sınıflandırılmaya çalışılmıştır. Tüm hastaların ilk başvurudaki klinik ve laboratuvar özellikleri, radyolojik bulguları, yapılmışsa bronkoskopi ve BAL sonuçları, tanı için herhangi bir organdan alınan biyopsilerinin histopatolojik inceleme sonuçları, verilen tedaviler, tedavi süreleri, tedaviye yanıt, izlem süreleri, izlemde gelişen ek bulgular kaydedilmiştir. Çocukluk çağı DPAH serimiz, literatürde tek merkezden bildirilen en büyük hasta serisidir. Çalışmamızda DPAH başlığı altında 16 ayrı hastalık grubu tartışılmıştır. Çalışmamızdaki hastaları literatürde en son önerilen Children’s Interstitial Lung Disease (chILD) Network sınıflandırmasına göre sınıflandırdığımızda 2 yaşın üzerindeki hastalarımızdan 15 idiyopatik interstisiyel pnömonili hastamız herhangi bir gruba oturtulamamıştır. Ayrıca, idiyopatik pulmoner hemosiderozisli hastalarımız ve PAM’lı hastalarımız sınıflandırma dışında kalmıştır. Literatürde yeni tanımlanan “akciğerlerin diffüz kondroid malformasyonu” tanısı alan hastamız da mevcut sınıflamada tanımlanmamıştır. Bu sonuçlar, tüm bu hastaları kapsayabilecek yeni bir sınıflandırma sistemine ihtiyaç olduğunu göstermektedir. Hastalarımızın ortalama tanı yaşı 7,7 yıldır. Tüm hastalık gruplarında en sık karşılaşılan semptomlar öksürük ve dispnedir. Tüm gruplarda hastaların tanıları ortanca 5,5 ay ile 3 yıl arasında değişen sürelerde gecikmiştir. Bu süre içinde hastalar sıklıkla tekrarlayan akciğer enfeksiyonları, tüberküloz ve astım gibi tanılarla izlenmişlerdir. Hastalık bulguları non-spesifik olduğundan, normal doğum öyküsü olan ve 3 aydan uzun süreli DPAH düşündüren semptom ve bulguları olan her çocuğun bu açıdan araştırılması önerilir

Çocukluk çağı tüberkülozu ile bitmeyen savaş: Daha az ilaç ve daha kısa sürede tedavi edilebilir mi?

Giriş: Etkili tedavi rejimleriyle mortalitesi tüm dünyada azalmış olsa da tüberküloz halen global bir sağlık problemidir. Tedaviye uyumsuzluk, ilaç direnci, tedavi başarısızlığı ve relaps tüberküloz tedavisi sırasında sık karşılaşılan problemlerdir. İntermittan tedavi rejimlerinin, özellikle kaynakları sınırlı bölgelerde, tedavi maliyetini ve tedavinin yan etkilerini azalttığı, hastanın tedaviye uyumunu artırdığı bilinmektedir; ayrıca çocuk hastalarda da her gün verilen tedavi kadar etkili olduğu gösterilmiştir. Bu çalışmada, hastanemizde pulmoner ve ekstrapulmoner tüberküloz tanısı almış çocuk hastalara iki ilaçla verilen 6 ve 9 aylık intermittan tüberküloz tedavi rejimlerinin sonuçları karşılaştırılmıştır. Materyal ve Metod: Hastanemizde 1986 ile 2001 yılları arasında akciğer, lenf nodu, iskelet sistemi, plevral, abdominal, miliyer, perikardiyal, deri ve böbrek tüberkülozu nedeniyle intermittan antitüberküloz tedavi alan 115 hasta retrospektif olarak değerlendirilmiştir. Elli bir hastaya 15 gün süreyle izoniazid ve rifampisin tedavisi sonrası 9 ay süreyle aynı ilaçlarla ve aynı dozlarda haftada iki gün intermittan tedavi verilmiştir. Altmış dört hastaya ise aynı tedavi rejimi 6 ay süreyle uygulanmıştır. Bulgular: Tedavinin ilk ayında 1. gruptaki (9 aylık intermittan tedavi) pulmoner tüberkülozlu hastaların %75'inde, 2. gruptaki (6 aylık intermittan tedavi) pulmoner tüberkülozlu hastaların %79'unda klinik düzelme gözlendi. Pulmoner tüberkülozlu hastalarda ilk 6 ayda, grup 1'de %81'inde, grup 2'de %86'sında radyolojik düzelme görüldü. Klinik ve radyolojik düzelme zamanları karşılaştırıldığında iki grup arasında anlamlı bir farklılık saptanmadı (p 0.05). Benzer sonuçlar ekstrapulmoner tüberkülozlu hastalarda da gözlendi (p 0.05). Hastalar 7 ay ile 15 yıl arasında takip edildi. Hiçbir olguda erken relaps gözlenmedi. Dokuz aylık intermittan tedavi alan (grup 1) 4 hastada, tedavi kesildikten iki yıl sonra geç relaps görüldü. Sonuç:Çocukluk çağı pulmoner ve menenjit dışı ekstrapulmoner tüberkülozda, iki ilaçla 6 aylık intermittan tedavi, 9 aylık intermittan tedavi kadar etkilidir.Introduction: Tuberculosis is still a global health problem all over the world despite its mortality has been decreased with effective treatment regimens. Poor treatment adherence, acquired drug resistance, treatment failure and relapse are the major problems during the course of the tuberculosis treatment. Intermittent regimens have the advantages of reducing the side effects and the cost of the therapy and increasing the adherence, especially in resource-limited areas; and have been documented to be as effective as daily regimen in the paediatric population. In this study, we compared the results of 6-month and 9-month intermittent-therapy regimens with two drugs, given to the children with pulmonary and extrapulmonary tuberculosis at our hospital. Materials and Methods: One hundred and fifteen patients with pulmonary and extrapulmonary tuberculosis other than meningitis, who had been given intermittent anti-tuberculosis therapy between 1986 and 2001, were evaluated retrospectively. Fifty one patients were given isoniazid and rifampin daily for 15 days, followed by the same drugs and doses twice weekly for a total of 9-months. Also, 64 patients were treated with the same regimen for a total of 6-months. Results: Clinical recovery was observed in 75% and 79% of pulmonary tuberculosis patients at the first month of therapy in group 1 (9-month group) and group 2 (6-month group), respectively. Radiological recovery was noted between 0-6 months in 81% of the patients in group 1 and 86% of the patients in group 2. According to the clinical and radiological recovery times, no significant difference was detected between the two groups (p< 0.05). Similar results had been observed in extrapulmonary tuberculosis (p< 0.05). Follow-up periods ranged from 7 months to 15 years. There was no case of early relapse. Late relapse was noted in 4 patients, who had been received 9-month therapy (group 1). Conclusion: Six-month intermittent therapy with two drugs is as efficacious as 9-month intermittent-therapy in childhood pulmonary and extrapulmonary tuberculosis, other than meningitis

[Atypical presentation in hydatid disease: hemoptysis]. Hidatik Hastalikta Atipik Prezentasyon: Hemoptizi.

Hydatid disease is an infectious disease caused by the larval stage of Echinococcus granulosus. It constitutes a major public health problem worldwide. This disease may present in several atypical forms, causing difficulty in diagnosis. In this article it is stressed that the diagnosis of hydatid disease should be kept in mind in patients presenting with hemoptysis, although this presentation is very rare

COVID-19 pandemic and the global perspective of Turkish Thoracic Society

It has been more than 3 months now since the first case of COVID-19 was reported in Turkey. Globally, the number of confirmed cases and deaths reached 9,653,048 and 491,128 respectively, as reported by 216 countries by June 27, 2020. Turkey had 1,396 new cases, 194,511 total cases, and 5,065 deaths by the same date. From the first case until today, the Turkish Thoracic Society (TTS) has been very proactive in educating doctors, increasing public awareness, undertaking academic studies, and assisting with public health policies. In the present report, social, academic, and management perspectives of the pandemic are presented under appropriate subtitles. During this critical public health crisis, TTS has once again demonstrated its readiness and constructive stance by supporting public health, healthcare workers, and the environment. This review summarizes the perspective of TTS on each aspect of the COVID-19 pandemic and casts light on its contributions

Respiratory functions in the follow-up of children with COVID-19 infection

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