Fiberoptic flexible bronchoscopy via the laryngeal mask airway in children.

The laryngeal mask airway (LMA) is a new device for controlling the airway during many procedures. Aside from its use in different kinds of surgical procedures, fiberoptic flexible bronchoscopy can also be performed easily with this mask in children under sedation. This procedure was performed via LMA in 36 children (aged 2-16 years) who suffered from different kinds of respiratory diseases and were seen at Hacettepe University Ihsan Dogramaci Children's Hospital, Pediatric Chest Disease Unit, during a seven-month period. The procedure was performed with success and no complications occurred. To the best of our knowledge, this is the first report from Turkey on flexible bronchoscopic evaluation via LMA in children with different kinds of respiratory diseases. We suggest that this technique can be used safely.

Overdiagnosis of pneumonia in children.

The aim of this study was to reevaluate patients diagnosed as pneumonia in our outpatient clinic in an effort to determine whether these patients really had pneumonia and needed antibiotic therapy. Over a 14-month period, 119 children who were diagnosed as pneumonia were prospectively evaluated. In order to find a relationship, specificity and sensitivity were defined. The diagnosis of pneumonia was ruled out in 48 of the 119 (40%) patient included. An incorrect diagnosis of pneumonia was made in 40% of cases and antibiotics were prescribed unnecessarily in 85%. Tachypnea, which had a specificity of 99% and sensitivity of 61%, was the most important criteria in diagnosing pneumonia. We believe that children who present with cough only do not always need antibiotic treatment. Tachypnea and auscultation findings are important criteria for diagnosing and treating pneumonia.

Screening for tuberculosis in a primary school in Ankara.

Tuberculosis is still an important health problem in developing countries. A screening program was conducted upon learning that one of the teachers of a primary school in Ankara was diagnosed to have active pulmonary tuberculosis. A total of 341 students in the same building with the index case were screened for tuberculosis. There were 109 students with positive tuberculin test reaction. A higher ratio of tuberculin test positivity among the students of the teacher with active tuberculosis versus students vaccinated with BCG one year previously according to the routine vaccination program was determined. Isoniazid prophylaxis was given to the students with positive tuberculin test. The study shows the importance of an urgent work-up of index cases and their environment to prevent the spread of tuberculosis.

Inhaled corticosteroids for maintenance treatment in childhood pulmonary sarcoidosis.

UNLABELLED: We present our experience with sequential oral and inhaled corticosteroid therapy in childhood pulmonary sarcoidosis. Fifteen children were followed-up for a mean of 7 y. Treatment consisted of oral prednisolone 2 mg/kg/d on initial diagnosis. After remission was reached, alternate day therapy with 1 mg/kg was continued. The dose was tapered to a maintenance dose which controlled the activity of the disease. When patients were free of symptoms and had no clinical and laboratory findings, inhaled corticosteriod treatment was started. Relapse treatment consisted of cessation of inhaled corticosteroids and start of oral corticosteroids at a dosage of 2 mg/kg/d and then a tapered dose. Five patients were given oral corticosteroids only. Nine patients were given inhaled steroids after oral corticosteroid therapy had been discontinued. Clinical and radiological remissions were achieved in every patient. CONCLUSION: Sequential oral and inhaled corticosteroid therapy may be an alternative treatment regimen for sarcoidosis in children.

Pulmonary involvement in a child with ligneous conjunctivitis and homozygous type I plasminogen deficiency.

Ligneous conjunctivitis (LC) is a rare disorder characterized by a chronic course of recurrent membranous lesions at conjunctivae. Pseudomembranes of other mucous membranes have been reported in patients with LC, but to the best of our knowledge, no case with alveolar involvement has been described. Here, we report a 2.5-year-old girl with LC who had tracheoaveolar involvement and homozygous type I plasminogen deficiency. Tracheal involvement was diagnosed by bronchoscopic biopsy and alveolar involvement with postmortem biopsy. She was shown to be homozygous for frameshift mutation in plasminogen exon 14 (Gly565ins-G) with molecular genetic examination of DNA which was obtained from parafin embedded postmortem lung tissue. Ligneous inflammation of the upper and lower respiratory tract must be considered in children with LC and recurrent respiratory tract problems.

Homozygous and compound-heterozygous type I plasminogen deficiency is a common cause of ligneous conjunctivitis.

Severe type I plasminogen deficiency has been recently linked to ligneous conjunctivitis, a rare and uncommon form of chronic conjunctivitis. In this study, eight unrelated ligneous conjunctivitis patients living in different parts of the world were examined. All affected subjects from which plasma was available displayed absent or markedly reduced plasminogen antigen and plasminogen functional activity. Molecular genetic studies of seven patients identified a Lys19-->Glu mutation in two boys in a homozygous state, and in two girls in a compound-heterozygous state in which the second plasminogen gene carried a missense (Arg134-->Lys) and a nonsense mutation (Cys133--> Stop), respectively. A fifth patient was shown to be homozygous for a frameshift mutation in plasminogen exon 14 (Gly565ins-G). In two unrelated subjects with ligneous conjunctivitis no mutations in the plasminogen gene were identified. Our results suggest that the Lys19-->Glu mutation is the most prevalent mutation in the plasminogen gene of patients with ligneous conjunctivitis.

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.

Allergic bronchopulmonary aspergillosis in two patients with cystic fibrosis.

Allergic bronchopulmonary aspergillosis (ABPA) is hypersensitivity to Aspergillus fumigatus which manifests as episodic wheezing, usually refractory to bronchodilator therapy, with fixed and transient pulmonary infiltrates, central bronchiectasis, blood eosinophilia, elevated serum IgE level, immediate skin reactivity to an A. fumigatus antigen and precipitating antibodies to A. fumigatus. It is an unusual complication of asthma and cystic fibrosis (CF). We present two cystic fibrosis patients with ABPA treated successfully with prednisone and, in Case 1 also with itraconazole. The physician should be alert to the possibility of ABPA whenever CF patients present with the new infiltrates, high serum total IgE and other positive parameters of A. fumigatus sensitization. Treatment with systemic steroids should be started in order to prevent irreversible lung damage.

Triple A syndrome mimicking cystic fibrosis.

We report a 2-8/12 year-old male who presented with symptoms resembling cystic fibrosis (failure to thrive, developmental delay and recurrent diarrhea) and had elevated sweat chloride concentration. Mucosal hyperpigmentation led to the diagnosis of adrenal insufficiency which was ultimately shown to be a component of triple A syndrome (achalasia, alacrima, adrenal insufficiency). Elevated sweat chloride concentration normalized after initiation of adrenal replacement therapy. We suggest that non-CF conditions causing elevated sweat chloride concentration should be considered in patients with atypical findings or who do not have objective evidence of pulmonary or exocrine pancreatic disease.

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. There was a history of multiple blood transfusions for anemia in 10 patients. Consanguinity between parents was noted in 11 patients. Severe pallor, cough, hemoptysis, and hepatomegaly were the most common findings on physical examination. All but 2 patients had hypochromic microcytic anemia of varying severity. In 12 children, moderate reticulocytosis was noted. Corticosteroids were administered with doses ranging from 5 mg every other day to 2 mg/kg/day depending on the severity of the episodes (duration of disease from 2-14 years). It is our impression that patients with IPH, benefit from long-term steroid treatment which in turn results in a milder course. Long-term low-dose steroid treatment appeared to prevent crises and assured a prolonged survival.

Intermittent chemotherapy for miliary tuberculosis in children.

Miliary tuberculosis is a severe manifestation of tuberculosis. Six children aged between two months and 10 years with the diagnosis of miliary tuberculosis were treated with intermittent antituberculous therapy for six, nine or 12 months. All the patients showed clearance of both clinical and radiological symptoms; there was no drug toxicity or resistance and no relapses were seen in the follow-up period ranging from nine months to nine years. Intermittent therapy is safe and effective in miliary tuberculosis and it may be an alternative therapy because of its minimal toxicity and lower cost.

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 Dogramaci Children's 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. When evaluating the results of therapy, the relapse rate was higher in surgically treated patients than medically treated patients. We conclude that medical treatment of childhood hydatidosis is best, except in cases with complications such as infection, parenchymal compression or obstruction of airways, bile duct or viscera; all of these are indications for surgical therapy.