Deniz Anadol

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.

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.

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.

Inhaled corticosteroids for maintenance treatment in childhood pulmonary sarcoidosis.

We present our experience with sequential oral and inhaled corticosteroid therapy in childhood pulmonary sarcoidosis. Fifteen children were followed‐up for a mean of 7y. Treatment consisted of oral prednisolone 2mg/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.

Chemical pneumonia caused by glutaraldehyde

reactive chemical properties.1 It has bactericidal, sporicidal and fungicidal properties, and is used as a cold sterilizing agent especially for medical, surgical and dental equipment. Its vapors are irritant to skin, eye, nose and lungs. As far as we know there have been no reports that it can cause pneumonia. Here we report a child with chemical pneumonia as a result from glutaraldehyde who presented with acute respiratory failure and was successfully treated with noninvasive mechanical ventilation via a nasal mask.

Phenylketonuria and cystic fibrosis in the same patient

Classical phenylketonuria (PKU, McKusick 261600), resulting from a deficiency of the enzyme phenylalanine hydroxylase, is transmitted as an autosomal recessive trait. Decreased or absent activity of this enzyme results in the accumulation of phenylalanine and some metabolites of phenylalanine normally present in minor amounts, such as phenylpyruvic acid. 1 Cystic fibrosis (CF, McKusick 219700) is an autosomal recessive disease caused by mutations in a gene located on the long arm of chromosome 7. The gene product is the 1480 amino acid cystic fibrosis transmembrane conductance regulator (CFTR), a protein that normally regulates and participates in the transport of electrolytes across epithelial cell membranes and probably across intracellular membranes as well. 2 The probability of the presence of these two inborn errors in the same patient must be low and it has not been described in the literature before to the best of our knowledge. We hereby describe a 2-month-old boy with PKU and CF.