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Biomedical subjects

N Kiper

Publications and source records attributed to N Kiper.

At least 19 recordsLinked to original sources
PubMed2006

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). AIM: To evaluate the effects of a macrolide antibiotic [clarithromycin (CAM)] on the process of inflammation [by measuring IL-8, TNF-alpha, IL-10 levels and cell profiles in bronchoalveolar lavage (BAL) fluid], pulmonary function and sputum production in children with steady-state bronchiectasis, secondary to causes other than CF or primary immunodeficiencies. METHODS: Seventeen patients randomized to the treatment group received CAM and supportive therapies for 3 months and 17 patients in the control group were given supportive therapies only. RESULTS: Compared with the control group, the treatment group showed a significant decrease in IL-8 levels, total cell count, neutrophil ratios in BAL fluid and daily sputum production at the end of the third month. There was also a significant increase in the treatment group's BAL fluid macrophage ratios. The differences in pulmonary function test parameters were not significant. CONCLUSION: Use of CAM in children with steady-state bronchiectasis results in laboratory improvement by reducing the inflammatory processes in the lungs. No corresponding clinical improvement could be shown but although this is possible with long-term use, trial validation is necessary.

Adolescent↗
PubMed2005

Congenital hydrocephalus as a rare association with ligneous conjunctivitis and type I plasminogen deficiency.

Severe type I plasminogen deficiency is the underlying cause of ligneous conjunctivitis (LC). Furthermore, pseudomembranes may also be found on other mucous membranes (gastrointestinal tract, bronchial system, genital tract). In very rare cases, congenital hydrocephalus has been associated with the more severe forms of the disease and may even precede LC. The pathophysiological mechanism is unclear at present. It is advisable to look for plasminogen deficiency in patients with congenital hydrocephalus, because obstruction of ventriculoperitoneal shunts is possible when such a condition is overlooked. Here, we report a case of LC with hydrocephalus. This report reemphasizes the association of LC with hydrocephalus which is not well known.

Cerebral Cortex↗
PubMed2002

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

UNLABELLED: 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 beta-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 beta-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-alpha (TNF-alpha) in children with CF and in children with BE than in normal subjects. After 6 mo of beta-carotene supplementation, the plasma levels of beta-carotene and vitamin E increased and the plasma levels of TNF-alpha and malondialdehyde decreased in both groups. CONCLUSION: Potent antioxidants, beta-carotene and vitamin E are deficient in patients with CF and in patients with BE, and they are more susceptible to oxidative damage. These patients may benefit from beta-carotene supplementation.

Adolescent↗
PubMed2001

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.

Conjunctivitis↗
PubMed2001

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.

Adolescent↗
PubMed2001

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.

Albendazole↗
PubMed2001

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.

Administration, Inhalation↗
PubMed2000

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.

Adrenal Glands↗
PubMed1999

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.

Adolescent↗
PubMed1998

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 Doğramaci 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.

Adolescent↗
PubMed1998

Effectiveness of short-course, intermittent chemotherapy for tuberculosis in young infants aged less than 6 months.

In order to determine the efficacy of short-course intermittent chemotherapy for tuberculosis in young infants less than 6 months of age, 15 newly diagnosed infants were treated with isoniazid (10-15 mg/kg) and rifampin (10-15 mg/kg), with/without streptomycin (30 mg/kg), daily for 15 days followed by similar doses of isoniazid and rifampin twice a week for another 8.5 months. The follow-up period ranged from 1 to 10 years. No patient demonstrated relapse following the termination of the treatment. These results suggest that short-course, intermittent treatment may be safe and effective for treating tuberculosis in young infants.

Antitubercular Agents↗
PubMed1996

Auditory nerve-brainstem responses in cystic fibrosis patients.

We conducted a study in 30 children with cystic fibrosis (CF) to determine if their hearing had been affected. Their ages ranged between 2 to 17 years and they were studied by means of complete audiologic evaluation and brainstem evoked response audiometry (BERA). Twenty of them had received some ototoxic drugs for variable periods. The results showed that there was no significant correlation between CF and BERA findings. The findings indicated that, except a few middle ear problems such as serous otitis media, CF did not affect hearing status of the CF children per se.

Adolescent↗
PubMed1996

Clinical status, ocular surface changes and tear ferning in patients with cystic fibrosis.

Twenty-three cystic fibrosis patients and 20 controls were examined for ocular surface changes and tear fluid ferning characteristics. The patients were also evaluated systemically and given numerical scores according to Schwachman's scoring system. Frequency of blepharitis, fluorescein staining, and the Schirmer's test values did not differ between patients and controls. Patients with cystic fibrosis had a higher frequency of altered ferning pattern that corresponded to the disease severity as measured by the Schwachman score (r = -0.48, p < 0.05). Although altered ferning pattern was frequent, the rate of type I ferning was also high (30%) in patients with cystic fibrosis. We conclude that the ferning test should not be used as an aid in diagnosis of cystic fibrosis but it may be used as an indicator of clinical status during follow-up.

Adolescent↗
PubMed1995

Alpha-mannosidosis: the first Turkish case.

We describe a 10 month old boy with alpha-mannosidosis who presented with recurrent bronchopneumonia and diarrhea. Facial coarsening, deafness, hepatosplenomegaly, umbilical hernia, pectus carinatum and widespread Mongolian spots were distinguishing features. He also had mild skeletal deformities grouped together as 'dysostosis multiplex', and vacuolated lymphocytes on peripheral blood smear. These findings coupled with an abnormal urinary oligosaccharide pattern led to the suspicion of a lysosomal storage disease in the patient which proved to be alpha-mannosidosis. An exceptionally low level of alpha-mannosidase activity was subsequently found in serum and cultured skin fibroblasts. The patient's brother, who had died at the age of 10 months, had similar features. To the best of our knowledge, this is the first case reported from Turkey.

Humans↗