Long term outcome of cystic fibrosis patients with multisystem evaluation

  INTRODUCTION: Cystic fibrosis is a chronic disease with multiple organ involvement and chiefly results in chronic respiratory infections, pancreatic insufficiency and associated complications. The age at diagnosis, clinical presentation, rate of disease progression and prognosis is variable among patients. This study is designed to evaluate the behavior of disease to provide epidemiologic data for early recognition and proper management. MATERIAL AND METHODS: The study was designed as an active surveillance of 192 patients diagnosed with cystic fibrosis in a tertiary lung disease centre between 2008 and 2015. The diagnosis of cystic fibrosis was established in all patients accordingly to conventional criteria, including two positive sweat chloride tests and clinical signs and symptoms. Demographic, clinical and laboratory data were obtained from these patients in each hospitalization and also every follow-up visit and carefully evaluated for complications of this chronic disease. RESULTS: The majority of patients showed positive culture for Pseudomonas aeroginosa. Bronchiectasis was the most prevalent finding in chest CT scan. 44.3% of patients had been treated for allergic bronchopulmonary aspergillosis and all had sinus disease. Increased pulmonary artery pressure was observed in 40% of patients with cystic fibrosis. 33 patients died which consisted 17.1% of all the patients.The mean age of mortaliy was 18.15 year. CONCLUSIONS: The clinical outcome of cystic fibrosis is variable in different countries which may reflect environmental influences and the role of early diagnosis on long term outcomes. However, the role of early diagnosis in long-term outcomes of the disease can not be ignored.

Kompleksowa ocena długoterminowa chorych na mukowiscydozę

WSTĘP: Mukowiscydoza jest wielonarządową chorobą przewlekłą, której głównymi następstwami są infekcje układu oddechowego, niewydolność trzustki i inne pokrewne powikłania. Wiek w czasie rozpoznania, przebieg kliniczny, tempo progresji i rokowanie są różne u różnych chorych. Celem prezentowanego badania była ocena przebiegu choroby i wpływu uwarunkowań epidemiologicznych na wczesne rozpoznanie i właściwe leczenie. MATERIAŁ I METODY: Przeprowadzono aktywną ocenę prospektywną 192 chorych na mukowiscydozę, leczonych w ośrodku referencyjnym w latach 2008–2015. U wszystkich chorych rozpoznanie mukowiscydozy postawiono na podstawie obowiązujących kryteriów, w tym stwierdzenia dwóch dodatnich wyników potowego testu chlorkowego oraz obecności typowych objawów klinicznych. Dane demograficzne, kliniczne i laboratoryjne uzyskiwano od pacjentów podczas kolejnych hospitalizacji i wizyt kontrolnych. Dane te były szczegółowo oceniane pod kątem powikłań choroby podstawowej. WYNIKI: U większości chorych stwierdzono dodatni wynik hodowli plwociny w kierunku Pseudomonas aeroginosa. Rozstrzenia oskrzeli były najczęściej występującym objawem w badaniu tomograficznym klatki piersiowej. W badanej grupie 44,3% chorych było leczonych z powodu alergicznej aspergilozy oskrzelowo-płucnej a u wszystkich chorych stwierdzono chorobę zatok przynosowych. Podwyższone ciśnienie w tętnicy płucnej stwierdzono u 40% chorych na mukowiscydozę. Zmarło 33 chorych, co stanowi 17,1% badanej grupy. Średni czas przeżycia wynosił 18,15 roku. WNIOSKI: Wyniki leczenia chorych na mukowiscydozę są różne w różnych krajach, co może odzwierciedlać wpływy środowiska, a także znaczenie wczesnego rozpoznania dla odległego rokowania. Znaczenie wczesnego rozpoznania dla odległych wyników leczenia nie powinno być lekceważone.WSTĘP: Mukowiscydoza jest wielonarządową chorobą przewlekłą, której głównymi następstwami są infekcje układu oddechowego, niewydolność trzustki i inne pokrewne powikłania. Wiek w czasie rozpoznania, przebieg kliniczny, tempo progresji i rokowanie są różne u różnych chorych. Celem prezentowanego badania była ocena przebiegu choroby i wpływu uwarunkowań epidemiologicznych na wczesne rozpoznanie i właściwe leczenie. MATERIAŁ I METODY: Przeprowadzono aktywną ocenę prospektywną 192 chorych na mukowiscydozę, leczonych w ośrodku referencyjnym w latach 2008–2015. U wszystkich chorych rozpoznanie mukowiscydozy postawiono na podstawie obowiązujących kryteriów, w tym stwierdzenia dwóch dodatnich wyników potowego testu chlorkowego oraz obecności typowych objawów klinicznych. Dane demograficzne, kliniczne i laboratoryjne uzyskiwano od pacjentów podczas kolejnych hospitalizacji i wizyt kontrolnych. Dane te były szczegółowo oceniane pod kątem powikłań choroby podstawowej. WYNIKI: U większości chorych stwierdzono dodatni wynik hodowli plwociny w kierunku Pseudomonas aeroginosa. Rozstrzenia oskrzeli były najczęściej występującym objawem w badaniu tomograficznym klatki piersiowej. W badanej grupie 44,3% chorych było leczonych z powodu alergicznej aspergilozy oskrzelowo-płucnej a u wszystkich chorych stwierdzono chorobę zatok przynosowych. Podwyższone ciśnienie w tętnicy płucnej stwierdzono u 40% chorych na mukowiscydozę. Zmarło 33 chorych, co stanowi 17,1% badanej grupy. Średni czas przeżycia wynosił 18,15 roku. WNIOSKI: Wyniki leczenia chorych na mukowiscydozę są różne w różnych krajach, co może odzwierciedlać wpływy środowiska, a także znaczenie wczesnego rozpoznania dla odległego rokowania. Znaczenie wczesnego rozpoznania dla odległych wyników leczenia nie powinno być lekceważone

Radiologic Manifestation of Pulmonary Tuberculosis in Children Admitted in Pediatric Ward-Massih Daneshvari Hospital: A 5-Year Retrospective Study

"nDespite the extensive preventive and therapeutic measures present against tuberculosis (TB), this disease still remains as one of the important causes of mortality and morbidity in the world. Considering the high incidence of TB in children, rareness of its' clinical features and complexity of bacteriologic diagnosis in this age group paraclinical studies, especially radiologic evaluations, is useful for reaching a final diagnosis. This 5 year study was conducted in National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Massih Daneshvari Hospital, Tehran, Iran. This retrospective study was conducted on 70 children (43 (61%) female and 27 (38.5%) male) aged between 5 months to 15 years old during a five year period (from 2001-2006) in pediatric ward. It was performed on children who were confirmed to have TB by various clinical, bacteriologic and radiologic features and tuberculin skin test. We studied the radiologic features of pulmonary TB in these children. Right lung involvement was observed in 65%, left lung 23% and bilateral involvement was detected in 12%. Also middle and superior lobes were the most common lobes affected. The commonest radiographic feature was hilar (mediastinal) lymphadenopathy; 70% detected on chest x-ray (CXR) and 85% on CTscan. Lymph nodes on right side were affected more; 25% were calcified. Also nodular infiltration of lung parenchyma was observed in 35% of CXRS and 61% of CTscans. This was followed by patchy consolidation detected in 25% and 35% of CXRs and CTscans respectively. We also observed that children <3yr. of age had the highest lymph node involvement but the least parenchymal lesions as compared to older children. It is concluded that primary TB is the most common form of pulmonary TB in children. This could be in the form of hilar lymphadenopathy with or without lung parenchymal involvement. Also radiologic features could provide valuable information in regard to diagnosis, treatment and follow-up of pulmonary TB in children

Extra Pulmonary Tuberculosis in Children: Two Years Study

Tuberculosis (TB) is an important health problem in developing countries, with varying clinical presentations depending on the organs/systems involved. To study the spectrum of clinical and paraclinical aspects of extra pulmonary TB in children suffering from pulmonary TB. This study has been carried out on 65 children with tuberculosis, admitted in TB wards of National Research Institute of Tuberculosis and Lung Disease (N.R.I.T.L.D) during 2004-2006. All patients were investigated according to specific diagnostic criteria including; history of contact with TB patient, clinical manifestations, radiological findings, tuberculin test and bacteriologic or pathologic results and after confirmation, treatment was administered. Out of 65 cases, 14 had different types of extra pulmonary tuberculosis, and data concerning following factors were studied: age, gender, race, site of involvement, bacteriology, pathology, ADA (ascitic fluid), PCR (tissue specimens), history of close contact, HIV tests (ELISA), tuberculin test, radiological findings, and immunological studies (in disseminated TB). Of 14 cases, 8 were girls and 6 were boys with mean age of 8.75±4.2. Nine patients were Iranian and 5 were Afghan. History of close contact was detected in 4 cases. Type of involvement was: 5 cervical adenitis, 3 osteoaticular disease, 2 peritonitis and 2 disseminated form of tuberculosis, one pericarditis, one renal tuberculosis. Radiological findings showed 4 pulmonary disease and 3 osteoarticular involvement. Tuberculin skin tests greater than 15 mm observed in 5 cases, 9 patients had 0-5 mm induration. 4 cases had concomitant pulmonary and extra pulmonary involvement. Positive AFB in gastric lavage was recognized in 4 cases, in which 3 showed positive cultures for MTB. Pathological examinations in 10 cases revealed granuloma with caseation compatible with tuberculosis, five in lymphadenopaty, three in osteoarticular, two in abdominal tuberculosis. According to this study, 20% of patients had extra pulmonary involvement, which is comparable to other reports (20-25%) and TB lymphadenitis is the most common from of presentation

The Correlation of Brody High Resolution Computed Tomography Scoring System with Clinical Status and Pulmonary Function Test in Patients with Cystic Fibrosis

Background: To reduce the mortality and morbidity rates of cystic fibrosis (CF) patients, and to have an effective clinical management, it is important to monitor the progression of the disease. The aim of this study was to evaluate the progression of lung disease in CF patients by means of assessing the correlation of the CT scoring system with clinical status and pulmonary function test at the Pediatric Pulmonary Ward of Masih Daneshvari Hospital in 2008. Methods: Pulmonary high resolution computed tomography (HRCT) was performed in 23 CF patients using the Brody's scoring system. Morphologic signs as well as the extent and severity of each sign were scored, and the total score was calculated. The correlation of HRCT scores (total score as well as the score for each parameter) with Shwachman Kuczycki scoring system and pulmonary function test were examined. Results: The study included 9 female and 14 male patients with an age range of 5-23 years (mean: 13.42 years). Bronchiectasis (100%) and peribronchial wall thickening (100%) were the most frequent CT abnormalities. Mucus plugging, air trapping and parenchymal involvements were respectively seen in 95.7%, 91.3% and 47.8% of patients. The overall CT score for all patients was 57.6±24.2 (means±SD). The results of pulmonary function test showed a restrictive pattern; however, in 5.3% of the patients PFT was normal. The overall Shwachman-Kulczycki score was 53.48±13.8. There was a significantly (P=0.015) negative correlation between the total CT score and Shwachman-Kulczycki score; however, there was no significant correlation between total CT score and the results of PFT (P=0.481). Conclusion: The Brody's scoring system for high resolution computed tomography seems to be a sensitive and efficient method to evaluate the progression of CF, and can be more reliable when we combine the CT scores with clinical parameter