The role of type-B Natriüretic peptid (NT-proBNP) in the diagnosis and follow up of Thalassemic Cardiomyopathy

Talasemi majorlu (TM) hastalar genellikle hayatın ilk 6 ayından sonra kan transfüzyonuna gereksinim duyarlar. Üç dört haftada bir yapılan kan transfüzyonları ve gastrointestinal sistemden artan demir emilimi nedeniyle hastalarda hemakromatozis gelişir. Fazla demir kalpte ileti sistemi ve myokardium olmak üzere tüm dokularda birikerek progresif fonksiyon bozukluğu oluşturur. TM hastalarda en sık ölüm nedeni olan kardiyak komplikasyonların erken tespiti önemlidir. Bu çalışmanın amacı düzenli transfüzyon alan TM hastalarında kardiyak etkilenmeyi EKO ve NT-proBNP düzeyleri ile erken dönemde saptayabilmek, diyastolik disfonksiyon tespit edilen hastalarda enalapril ile tedaviye başlamak ve tedavi boyunca EKO parametreleri ve NT-proBNP düzeylerindeki değişiklikleri izleyerek NT-proBNP nin talasemik kardiyomyopati tanı ve izleminde markır olup olamayacağını belirlemektir. Çalışmaya 43 TM hastası ile başlandı. Diyastolik disfonksiyon tespit edilen 23 hasta çalışma grubunu oluşturdu. Bu hastalara 5 mg/gün enalapril tedavisi başlandı. Tedavinin 6, 12 ve 18. ayında EKO tekrarlandı. Çalışma grubunda 0, 6, 12 ve 18. aylarda, sağlıklı kontrol grubu ve kardiyak disfonksiyonu olmayan TM hastalarından ise başlangıçta bir kez serum NT-proBNP düzeyi çalışıldı. Kardiyak disfonksiyonu belirlemede EKO parametreleri ile serum ferritin, pretransfüzyonel hemoglobin ve NT-proBNP düzeyleri arasındaki ilişkiye bakıldı. Serum NT-proBNP düzeyleri kontrol grubunda ortalama 51,8±23,2 pg/ml, kardiyak disfonksiyon olmayan TM hasta grubunda 112,6±85,7 pg/ml, çalışma grubunda ise 143,5±98,3 pg/ml bulundu. Enalapril tedavisinin 6. 12. ve 18. ayında serum NT-proBNP düzeyleri sırasıyla 90,4±63,4 pg/ml, 65,5±51,0 pg/ml, 107,1±62,3 pg/ml saptandı. Çalışma grubunda pretransfüzyonel hemoglobin düzeyleri ile NT-proBNP düzeyleri arasında korelasyon saptanmazken, NT-proBNP ve ferritin düzeylerinin 6. ve 12. ay düzeyleri arasında pozitif korelasyon saptandı. EKO parametrelerinden 12. ay mitral E/A oranı ve triküspit E dalgası arasında pozitif yönde, aort ve pulmoner ejeksiyon zamanları arasında negatif yönde korelasyon saptandı. Onsekizinci ay değerlendirmesinde triküspit E ve mitral A dalgası arasında negatif, sağ izovolümik relaksasyon zamanı arasında ise pozitif korelasyon saptandı. Serum NT-proBNP'nin talasemik kardiyopati tanı ve izleminde kullanışlı bir markır olduğu sonucuna varıldı.Patients with thalassemia major (TM) usually require blood transfusion in the first 6 months of life. Patients develop haemochromatosis develop due to blood transfusions performed one in 2-3 weeks and the increased ferritin absorbation from the gastrointestinal system. Exceeding iron precipitates in every tissue with the cardiac conduction system and the myocard have the lead. Establisihing the cardiac complications which are the most reason of death in TM patients is important. The aim of this study is to determine the cardiac influence in TM patients recieving regular transfusion early with ECO and NT-proBNP levels, to start enalapril treatment to patients whom we defined diastolic dysfunction and determining whether NT-proNBP is a marker in the diagnosis and treatment of thalassemic cardiomyopathy by watching the changes in ECO parameters and NT-proBNP levels during the treatment. The study started with 43 TM patients. 23 patients in whom diastolic dysfunstion is established has constituted the study group. These patients were started enalapril treatment of 5 mg/day. ECO was repeated in the 6th, 12th and 18th months of therapy. In the study group serum NT-proBNP levels were measured in months 0th, 6th, 12th and 18th whereas in the healthy control group and in TM patients without serum NT-proBNP levels were measured only once at the start up. When defining cardiac dysfunction, the association between ECO parameters and serum ferritin, pretransfusional hemoglobin and NT-proBNP levels were checked. Serum NT-proBNP levels were 51,8±23,2 pg/ml in the control group, 112,6±85,7 pg/ml in TM patients without cardiac dysfunction and 143,5±98,3 pg/ml in the study group. In the 6th, 12th and 18th months of enalapril treatment, serum NT-proBNP levels were 90,4±63,4 pg/ml, 65,5±51 pg/ml and 107,1±62,3 pg/ml, respectively. A positive correlation between NT-proBNP levels and the 6th and 12th month measures of ferritin levels whereas no correlation was found between pretransfusional hemoglobin levels and NT-proBNP levels. A positive correlation between an ECO parameter which is 12th month mitral E/A rate and tricuspidal E wave, and a negative correlation was found between aortic and pulmoner ejection rates. In the 18th month evaluation a negative correlation between tricuspidal E wave and mitral A wave and a positive correlation between tricuspidal E wave and right isovolumic relaxation time was established. It was figured out that serum NT-proBNP is a useful marker in the diagnosis anf follow up of thalassemic cardiomyopathy

Frequency of hematological findings associated with severe plasma vitamin B12 deficiency in infants and adolescents

WOS: 000342857400016PubMed ID: 24779301Background: The aim of this study was to determine the hematological status of severe vitamin B12 deficiency in infants and adolescents. Methods: This study involved 95 infants and 117 adolescents with severe plasma vitamin B12 deficiency (< 120 pg/mL) and normal plasma folate and ferritin. Infants were aged between one and 24 months. Adolescents were aged between 11 and 17 years. Results: Macrocytic anemia was associated with nine (9.5%) out of 95 infants with severe vitamin B12 deficiency. Neutropenia was found in 16 (16.8%) out of 95 infants with severe vitamin B12 deficiency. Thrombocytopenia was not found in 95 infants with severe vitamin B12 deficiency. Macrocytic anemia was found in two (1.7%) out of 117 adolescents with a severe vitamin B12 deficiency. Neutropenia was associated in one (0.8%) out of 117 adolescents with severe vitamin B12 deficiency. Thrombocytopenia was not found in 117 adolescents with severe vitamin B12 deficiency. Conclusions: Low clinical or hematological findings for B12 deficiency in infants and adolescents living in regions at risk, such as those with low consumption of meat and other animal products warrant the measurement of vitamin B12 level

Enteric Duplication Cyst Located at the Posterior Tongue: A Rare Case Report and Review of the Literature

The lingual localization of an enteric duplication is extremely rare but may present with respiratory and feeding problems that require emergency intervention. A7-month-old boy was brought to our clinic with feeding difficulties and tongue swelling. Physical examination showed a cystic lesion located near the left side of the tongue base that caused tongue protrusion to the contralateral side. During surgery, a 3-cm diameter opaque thick-walled cyst was found to be very closely adherent to the base of tongue, which was excised in its entirety. Following surgery, the patient fed during the early postoperative period and no complications were observed other than hypersalivation. On histological examination, a cystic lesion lined with intestinal mucosa and goblet cells was found. We present the rare case of a duplication cyst of the posterior tongue, with a literature review