N. Kiper

Long-term clinical course of patients with idiopathic pulmonary hemosiderosis (1979-1994): prolonged survival with low-dose corticosteroid therapy.

Idiopathic pulmonary hemosiderosis (IPH) is a rare disease of unknown etiology characterized by recurrent episodes of pulmonary symptoms such as cough, hemoptysis, and dyspnea. Our study consisted of 23 patients: 12 males and 11 females with IPH. The diagnosis was based on history, presence of anemia, and characteristic chest X‐ray, and was confirmed by showing macrophages laden with hemosiderin in gastric washings or bronchoalveolar lavage and/or open lung biopsy. All but one patient were diagnosed in our department between 1979–1994.

Pulmonary thromboembolism in childhood: A single-center experience from Turkey

OBJECTIVEnThis study was designed to evaluate the clinical characteristics, acquired and congenital risk factors, treatment strategies, and long-term outcome in pediatric pulmonary thromboembolism (PTE) cases followed in our center in Turkey.nnnSUBJECTSnOf the total 470 pediatric patients with thrombosis referred to our center, 16 (3.4%) had PTE. The mean age of the children with PTE was 10.3 +/- 6.8 years (range: 1.5-20.0, median: 10.5), and 12 (75.0%) were boys.nnnRESULTSnThe mean follow-up period was 28.9 +/- 21.0 months (range: 3-66, median: 22). During the follow-up period, recurrence was observed in three children (18.8%). The mean time from the appearance of symptoms to accurate diagnosis was 6.4 +/- 4.0 days (range: 2-10). Six patients (37.5%) were initially diagnosed as having pneumonia. After they were hospitalized and showed no clinical improvement with broad-spectrum antibiotic treatment, the accurate diagnosis of PTE was established. Of these 16 patients with PTE, 8 (50%) had associated thrombosis and 6 (37.5%) had congenital heart diseases. Infections including septic arthritis and osteomyelitis (n = 1), cytomegalovirus infection (n = 1), and infective endocarditis (n = 2) were detected in our patient group. In addition, two patients had a central venous line and one patient had obesity associated with malignancy. Other underlying diseases included thalassemia major, Behçet disease, antiphospholipid antibody syndrome, and autoimmune lymphoproliferative disorder in one patient each. Factor V G1691A heterozygous mutation was detected in two children, and methylene tetrahydrofolate reductase C677T homozygous mutation was detected in one child. A high level of factor VIII was the most common (8/16, 50%) laboratory risk factor in our patient group, and 12 children (75.0%) had a high D-dimer level. Among 16 children with PTE, one child had one, three children had two, five children had three, three children had four, and four children had five laboratory and/or clinical risk factors. Therefore, all children with PTE had at least one laboratory and/or clinical risk factor that facilitated development of thrombosis. In addition, according to the risk assessment for persistence or recurrence of venous thrombosis in children conducted by Manco-Johnson, 12 children (75%) with PTE in the present study had high-risk criteria.nnnCONCLUSIONnWhen a child with thrombosis at any site of the body develops unexpected respiratory symptoms or pneumonia unresponsive to antibiotic treatment, imaging studies should be performed for diagnosis of PTE. Furthermore, thrombotic children with high-risk criteria should be followed closely for the development of PTE.

Antioxidant effect of β-carotene in cystic fibrosis and bronchiectasis: clinical and laboratory parameters of a pilot study

The carotenoids are potent antioxidants with the ability to quench singlet oxygen and other toxic oxygen species. The aim of this pilot study was to investigate the protective effect of β‐carotene on oxidant system in patients with cystic fibrosis (CF) and in patients with bronchiectasis (BE) caused by a reason other than CF. Eighteen children with CF and 15 children with BE followed in the Pediatric Chest Disease Unit of Hacettepe University, and 15 healthy children participated in the study. Compared with the controls, significantly lower plasma levels of β‐carotene were found in the CF group and significantly lower plasma levels of vitamin E in the CF and BE groups. The standardization of carotenoid levels for total cholesterol did not significantly attenuate these differences. In addition, there were significantly higher levels of malondialdehyde (a marker of lipid peroxidation) and tumour necrosis factor‐α (TNF‐α) in children with CF and in children with BE than in normal subjects. After 6 mo of β‐carotene supplementation, the plasma levels of β‐carotene and vitamin E increased and the plasma levels of TNF‐α and malondialdehyde decreased in both groups.

Predominance of hospital-associated MRSA among cystic fibrosis patients in a Turkish reference cystic fibrosis centre

Abstract Methicillin-resistant Staphylococcus aureus (MRSA) strains are the major causative agents of numerous hospital- and community-acquired infections. Increasing prevalence of MRSA in cystic fibrosis (CF) populations is reported all over the world. Although there are papers reporting the prevalence and genetic backgrounds of MRSA isolates from different settings in Turkey, there is no information regarding the situation in the CF community. This study was conducted to characterize the MRSA strains recovered from CF patients followed-up at a Turkish reference CF centre. Microbiological testing of isolates was performed via conventional microbiological techniques. Molecular characterization of MRSA isolates was carried out by SCCmec typing by multiplex PCR and PVL gene determination. Among a total of 604 CF patients included in the study, 325 patients were found to harbour S. aureus (53·8%). Of those 325 patients, 24 were positive for MRSA during their follow-up (7·4%). Thirty-two MRSA isolates from these patients were chosen for further assessment of molecular characteristics. Twenty-six MRSA isolates exhibited a pattern like SCCmec type III (81·2%) and six consecutive MRSA isolates of a single patient revealed SCCmec type IV (18·7%). Our findings definitely support the need for further surveillance studies for CF-MRSA strains and highlight the need for infection control measures in the setting of CF centres.