2017 Cilt 15 Sayı 1

Permanent URI for this collectionhttps://hdl.handle.net/11452/8954

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    Correctable cause of dilated cardiomyopathy in an infant with heart failure: ALCAPA syndrome
    (Uludağ Üniversitesi, 2015-08-19) Güvenç, Osman; Saygı, Murat; Öztürk, Erkut; Güzeltaş, Alper
    Anomalous origin of the left coronary artery arising from pulmonary artery ALCAPA syndrome is a rare congenital heart disease seen in children. If untreated, it may lead to congestive heart failure, dilated cardiomyopathy (DCM), ischemic and arrhythmic complications may lead to patient’s death. ALCAPA is diagnosed with echocardiography; in the patients of suspected diagnosis, computerized tomography, magnetic resonance imaging and cardiac catheterization are used for further testing. Surgically correctable ALCAPA syndrome must be considered as etiology of DCM in children. In this report, we presented the case of an infant that was referred to our center with the diagnosis of DCM, who was echocardiographically diagnosed with ALCAPA syndrome and successfully treated with surgery, as well as a review of recent literature.
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    The features of anaphylaxis cases followed in the pediatric allergy clinic
    (Uludağ Üniversitesi, 2015-05-19) Doğru, Mahmut; Bostancı, İlknur; Özmen, Serap; Giniş, Tayfur; Şenol, Handan Duman
    Introduction: Anaphylaxis is a severe hypersensitivity reaction that can be lifethreatening. The frequency of anaphylaxis varies among the societies. In this study, the demographic characteristics, clinical course, triggering agents and treatment approaches of the patients who were diagnosed with anaphylaxis in our pediatric allergy clinic were evaluated. Materials and Methods: The medical records of children who received a diagnosis of anaphylaxis between 2010 and 2012 were retrospectively evaluated. Results: Between 2010 and 2012, 39.371 patients were admitted to our outpatient clinic. Sixty-six patients (49 male, 17 female) with a mean age of 8.9±5.3 years were evaluated as anaphylaxis. In 37 of the cases, anaphylactic attack occurred at home. Dermatological symptoms were the most frequent complaints. A probable cause of anaphylaxis was identified in all the patients except for five of them (92.4%). Food was the cause of anaphylaxis in 25 of the cases, followed by hymenoptera sting in 22 patients and drugs in 14 patients. The most common causes of anaphylaxis according to age groups were food, hymenoptera sting and drugs (0-3 years, 4-14 years and over the age of 14, respectively). Antihistamines were applied to all patients. The other medications that were applied were corticosteroid, oxygen, intravenous fluids, adrenaline (21/66), and salbutamol. Conclusions: The common cause of anaphylaxis in children is food allergens. However, the frequency decreases by age and other causes are more common than food. In our country, usage of adrenaline for the treatment of anaphylaxis is low.
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    Retrospective evaluation of patients diagnosed with osteogenesis imperfecta
    (Uludağ Üniversitesi, 2016-08-14) Aslan, Mustafa Törehan; Eren, Erdal; Sağlam, Halil; Tarım, Ömer; Tıp Fakültesi; Çocuk Sağlığı ve Hastalıkları Ana Bilim Dalı; Endokrinoloji Bilim Dalı
    Introduction: Osteogenesis imperfecta (OI) is a hereditary disease that impairs the quality of life by frequent bone fractures. The objective of our study is to retrospectively evaluate patients diagnosed with OI and to come up with helpful data that will assist developing new diagnosis and treatment protocols. Materials and Methods: Twenty-eight cases with OI who were followed-up in our clinic were retrospectively evaluated. Clinical classification of OI was done. Age, sex, and oxologic data were evaluated. Height, weight and body mass index (BMI) data was given as standard deviation score (SDS). Family history of fracture and consanguineous marriage was sought. Blue sclera and presence of deformity was evaluated on physical examination. Results: Out of the 28 cases in our study, 14 (50%) were boys, 14 (50%) were girls, and mean age was 7.48±5.09 years. Mean age of diagnosis was 25.59±39.59 months. Ten cases (47.6%) had OI, and 7 cases (25%) had consanguineous marriage in their family history. The cases were separated into autosomal dominant 4 clinical types according to Sillence classification as follows; 13 cases (46.4%) type 1, 10 cases (35.7%) type 3, and 5 cases (17.9%) type 4. The mean average basal dual energy X-ray absorptiometry Z score, mean height SDS, mean weight SDS and BMI SDS significantly increased for the cases after treatment (p<0.001). Conclusions: Treatment of OI with pamidronate was observed to increase bone mineral density, decrease number of fractures and pain, and improve the patient’s quality of life with inreasing mobility. Pamidronate is one of the most effective treatments of OI until a more effective treatment is found. On the other hand, since the long-term side effect of pamidronate on bones is not well-known, we think that randomised controlled studies still need to be done to determine the optimal time, interval and dose for bisphosphonate use.