Nural Kiper

Effects of claritromycin on inflammatory parameters and clinical conditions in children with bronchiectasis.

Background:  The effects of the macrolides cannot be ascribed to their antibacterial action alone. Their immunoregulatory and anti‐inflammatory functions are significant too. They are frequently used in the treatment of diffuse panbronchiolitis and cystic fibrosis (CF).

High resolution CT in children with cystic fibrosis: correlation with pulmonary functions and radiographic scores

OBJECTIVE To compare the high resolution CT (HRCT) scores of the Bhalla system with pulmonary function tests and radiographic and clinical points of the Shwachman-Kulczycki clinical scoring system. METHODS HRCT of the chest was obtained in 40 children to assess the role of HRCT in evaluating bronchopulmonary pathology in children with cystic fibrosis (CF). The HRCT severity scores of the Bhalla system were compared with chest radiographic and clinical points of the Shwachman-Kulczycki scoring system and pulmonary function tests. Only 14 of the patients older than 6 years cooperated with spirometry. RESULTS HRCT scores correlated well with radiographic points (r = 0.80, P<0.0001) and clinical points (r=0.67. P<0.0001) of the Shwachman-Kulczycki system, FVC (r = 0.71 P = 0.004) and FEV1 (r = 0.66, P = 0.01). Although radiographic points correlated significantly with FVC (r = 0.61, P = 0.02) and FEV1 (r = 0.56, P = 0.04), HRCT provides a more precise scoring than the chest X-ray. CONCLUSION The HRCT scoring system may provide a sensitive method of monitoring pulmonary disease status and may replace the radiographic scoring in the Shwachman-Kulczycki system. It may be helpful especially in follow-up of small children too young to cooperate with spirometry.

Treatment of Hydatid Disease

Hydatid disease caused by Echinococcus granulosus presents medical, veterinary and economic problems worldwide. Hydatidosis can be treated by medical, surgical and percutaneous modalities. Benzoimidazole carbamates are effective against E. granulosus. Although mebendazole, the first benzoimidazole used, has some beneficial effects on the disease in selected patients, it has also been associated with treatment failure in some cases, perhaps because of its poor absorption. Albendazole, a more recently developed benzoimidazole, is more effective than mebendazole. Praziquantel, an isoquinoline derivative, has recently shown value in the treatment of human echinococcal disease and its use in combination with albendazole is recommended in some patients. Ultrasound guided cyst puncture is another choice of treatment which has been used successfully in selected patients, although anaphylactic and allergic reactions due to spillage of the cyst contents have occurred. Surgical therapy in echinococcal hydatid disease is indicated for large cysts with multiple daughter cysts, superficially located single liver cysts which have a risk of rupture, complicated cysts such as those accompanied by infection, compression or obstruction, and cysts located in vital organs or which are exerting pressure on adjacent vital organs. However, surgical therapy carries high risk of mortality, morbidity or recurrence. Therefore, medical therapy may be an alternative option in uncomplicated cysts and in patients at high risk from surgery. The adjuvant use of drugs with surgery and percutaneous treatment can also be recommended for some patients.

Postinfectious Bronchiolitis obliterans in Children: Clinical and Radiological Profile and Prognostic Factors

Background: Postinfectious bronchiolitis obliterans (BO) designates a clinical syndrome of chronic airflow obstruction associated with inflammatory changes in the small airways. Objectives: The aim of this study was to determine the etiology, clinical and radiological features and define prognostic factors of postinfectious BO in children. Methods: We undertook a review of the medical records of 20 children with postinfectious BO, who were followed up between January 1994 and August 2001. Results: Postinfectious BO was diagnosed in 11 boys and 9 girls with a median age of 21.5 months (6–69 months) who were followed up for a median of 23 months. Cough and wheezing persisted since the initial lung infection in all patients. Postinfectious BO was diagnosed in an average of 6 months (1–42 months) after the acute illness. Adenoviruses were the most common etiologic agents identified serologically. The diagnoses of postinfectious BO was made by thoracic high-resolution computed tomography and clinical features. Corticosteroid therapy was used in 17 patients and supportive treatment was applied in all patients. Age at initial lung infection, gender, time of starting corticosteroid treatment, presence of bronchiectasis or atelectasis and etiologic agents identified on presentation did not predict severity in our study. Conclusions: Despite the prominent role of inflammation in the pathogenesis of postinfectious BO, the use of anti-inflammatory agents remains controversial. We could not identify any prognostic factors related to disease severity. In order to minimize associated complications, patients with postinfectious BO should be closely followed up and receive meticulous lung care.

Hydatid disease in childhood: A retrospective analysis of 376 cases

During a 20‐year period, 376 children with hydatid disease were treated at Hacettepe University Ihsan Dog˘ramaci Childrens Hospital. There were 223 males and 153 females with a mean age of 8.9 ± 0.1 years. Hydatid cysts were localized in the lungs in 222 patients, in the liver in 56 patients, and in other organs in the remaining patients. Cough, fever, and abdominal pain were the most common symptoms. One hundred eight patients had medical, 182 patients had surgical, 73 patients had medical and surgical, and 4 patients had medical and percutaneous drainage treatment as the initial therapy.

Conservative Treatment of Empyema in Children

Seventy-two children with no underlying diseases were treated for empyema. Radical surgical approaches like decortication were necessary for only 3 children. In 66 children tube drainage was applied. Staphylococcus aureus was cultured or was shown in Grams stain in 32 (44%) and children with this microorganism had longer duration of tube drainage (p < 0.05). The period for normalization of chest X-ray was positively related with the age of the patient (p < 0.05). In 60 of 72 children, chest X-ray was normal after 3 months. Follow-up of the patients 18 months after the infection revealed that pulmonary radiograms were normal in all cases and pulmonary function tests were within normal limits in all of the tested children (n = 25). It is emphasized that avoiding major surgical approaches must be encouraged in childhood empyema.

European protocols for the diagnosis and initial treatment of interstitial lung disease in children

Interstitial lung disease in children (chILD) is rare, and most centres will only see a few cases/year. There are numerous possible underlying diagnoses, with specific and non-specific treatment possibilities. The chILD-EU collaboration has brought together centres from across Europe to advance understanding of these considerations, and as part of this process, has created standard operating procedures and protocols for the investigation of chILD. Where established consensus documents exist already, for example, for the performance of bronchoalveolar lavage and processing of lung biopsies, these have been adopted. This manuscript reports our proposals for a staged investigation of chILD, starting from when the condition is suspected to defining the diagnosis, using pathways dependent on the clinical condition and the degree of illness of the child. These include the performance of genetic testing, echocardiography, high-resolution CT, bronchoscopy when appropriate and the definitive investigation of lung biopsy, in order to establish a precise diagnosis. Since no randomised controlled trials of treatment have ever been performed, we also report a Delphi consensus process to try to harmonise treatment protocols such as the use of intravenous and oral corticosteroids, and add-on therapies such as hydroxychloroquine and azithromycin. The aim is not to dictate to clinicians when a therapeutic trial should be performed, but to offer the possibility to collaborators of having a unified approach when a decision to treat has been made.

Long-term results of disodium etidronate treatment in pulmonary alveolar microlithiasis.

Pulmonary alveolar microlithiasis (PAM) is a rare disease with alveolar microliths mainly composed of calcium phosphate. The gene responsible for the disease is SLC34A2, which encodes a type‐IIb sodium phosphate cotransporter, has been described recently. Treatment of this disease is not clearly defined. Disodium etidronate is a member of bisphonates and it has been administered in these patients due to its inhibitory effect on the precipitation of hydroxyapatite microcrystals. Here, clinical and radiological improvement of two patients with PAM who were treated with disodium etidronate for 9 and 11 years, respectively, are presented. The pathogenetic mechanism of this treatment on the genetic basis of disease is discussed. Pediatr Pulmonol. 2010; 45:514–517.

Gorham-Stout Syndrome with chylothorax: successful remission by interferon alpha-2b.

Gorham–Stout syndrome is a rare disease and most often recognized in children and young adults. Chylothorax is a serious complication of Gorham–Stout syndrome. The treatment of either Gorham–Stout syndrome or chylothorax is still a dilemma. We described a 9‐year‐old girl with Gorham–Stout syndrome and chylothorax who was admitted to our hospital because of dyspnea, orthopnea, and bone pain, and treated with interferon alpha‐2b. Pediatr Pulmonol. 2009; 44:613–615.

Clinical features and treatment approaches in cystic fibrosis with pseudo-Bartter syndrome.

Abstract Introduction: Infants with cystic fibrosis (CF) are prone to develop episodes of hyponatraemic, hypochloraemic dehydration with metabolic alkalosis, which are biochemical hallmarks of the pseudo-Bartter syndrome (PB). Method: We reviewed the clinical and laboratory features and treatment aproaches of 29 children with CF and PB who were under follow-up in our institution from May 1992 to November 2003. Results: Of 241 patients with CF, PB was detected in 29 (12%) with a median age of 4 months at the time of the first attack. Most patients experienced vomiting, loss of appetite and dehydration during episodes of PB. All patients were managed with intravenous fluids and sodium chloride solutions. During follow-up, 12/29 cases required hospital admission for recurrent PB attacks. The oldest age at the time of the last attack was 48 months. Conclusions: CF should be considered in the differential diagnosis of metabolic alkalosis in young children. Vomiting and loss of appetite are important warning signs of possible PB in CF patients, particularly before 4 years of age. To prevent serious complications, it is crucial that parents and physicians recognise PB as early as possible.