Adjuvant Corticosteroid Therapy in Hepatosplenic Candidiasis-Related Iris

Candida infections are the most frequent infections in neutropenic patients. Hepatosplenic candidiasis (HSC) is a part of disseminated Candida infection that occurs most commonly in patients with hematologic malignancies treated with chemotherapy and requires protracted antifungal therapy. During invasive mycosis with rapid resolution of immunosuppression, immune reconstitution inflammatory syndrome (IRIS) which mimics treatment failure, drug toxicity or breakthrough infections may occur. Manifestation period, histopathologic findings and favorable effect of steroids to its inflammatory symptoms strongly suggest that HSC belongs to the invasive fungal infection induced IRIS. We present a child with B cell-acute lymphoblastic leukemia who developed HSC and addition of corticosteroid therapy to antifungal treatment achieved rapid resolution of the clinical symptoms and laboratory findings

Various clinical conditions can mimic Crimean-Congo hemorrhagic fever in pediatric patients in endemic regions

Summary: Crimean-Congo hemorrhagic fever (CCHF) is a tick-borne disease with high mortality. Many disorders can mimic CCHF. It is important to recognize the condition and to perform differential diagnosis in endemic countries. Twenty-one children aged 18 years or less with a preliminary diagnosis of CCHF were retrospectively evaluated. Real-time PCR and a confirmatory indirect immunofluorescence assay for negative results were performed. The diagnoses determined that 9 patients had (42.9%) CCHF; 7 patients had (33.3%) viral upper respiratory tract infections (URTI); 2 patients had (9.5%) brucellosis; 1 patients had (4.7%) periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome episode; 1 patient had (4.7%) cerebral palsy, diabetes insipidus, acute gastroenteritis, and hypernatremic dehydration; and 1 patient had (4.7%) cellulitis after a tick bite. The mean age of patients with CCHF was greater than that of the other patients (116.1 ± 53.6 vs. 94.1 ± 52.1 months, p = 0.02). Seventeen (81%) of the children included had a history of tick bites, 2 (9.5%) had a history of contact with a patient with CCHF, and 2 (9.5%) had no exposure, but were living in an endemic region. Three patients had an underlying disorder: cerebral palsy and diabetes insipidus, epilepsy, or PFAPA. All of the children experienced fever. Other frequent symptoms were malaise, diarrhea, vomiting, and abdominal pain, but none of these differed statistically between the patient groups. CCHF patients had a longer mean duration of symptoms (10.56 ± 1.42 vs. 6.75 ± 3.62 days, p = 0.008) and a longer mean length of hospitalization (8.00 ± 2.08 vs. 3.58 ± 1.56 days, p < 0.001) than the other patients. At laboratory examination, patients with CCHF had statistically significant lower leukocyte and platelet counts, more prolonged coagulation parameters, and greater AST, ALT, LDH, and CK levels than the other patients. No mortality or complications occurred in the study. Both infectious causes, such as URTI, cellulitis, and brucellosis, and non-infectious causes may resemble CCHF. Although they are not pathognomonic, some indicators, including a longer symptom duration and hospitalization, cytopenia, elevated liver enzymes, creatine kinase and prolonged coagulation parameters, were found to be in favor of CCHF. Keywords: Children, Crimean-Congo hemorrhagic fever, Differential diagnosi

The effect of serum vitamin D levels on anemia and iron parameters in children and review of the literature [Cocuklarda serum D vitamini duzeyinin anemi ve demir parametreleri uzerine etkisi ve literatur derlemesi]

Anemia, vitamin D and iron deficiency remains a major public health problem all over the world. Vitamin D is known for its crucial role in bone and mineral metabolism and is increasingly recognized to have extra-skeletal effects on cell proliferation and differentiation, immune function and anti-inflammatory effects. In addition, vitamin D is thought to have effect on iron metabolism and erythropoiesis. The aim of this research was to determine the effect of serum 25(OH) vitamin D (25D) on anemia and iron deficiency in childhood. A hundred and five patients aged between 1-18 years who were admitted Pediatric Hematology-Oncology and Pediatric Endocrinology out-patient clinic between January 2014 and November 2015 in Erzurum Regional and Research Hospital were enrolled in the study. The patients who had signs of infection/inflammation, chronic disease and thalassemia trait were excluded from the study. Data of patients, including gender, age, complete blood count, serum levels of 25D, iron, iron binding capacity and ferritin were retrospectively reviewed. In our study, the prevelance of anemia (11.1%, 27.8%, 11.9%, respectively, p=0.2), the prevelance of iron deficiency (33.3%, 38.9%, 33.3%, respectively, p=0.9), level of serum iron (67.7±35.3 µgr/dL, 63.2±31.9 µgr/dL, 67.5±33.7 µgr/dL, respectively, p=0.8), level of serum ferritin (26±18.9 mg/L, 24±16.4 mg/L, 22.5±18.1 mg/L, respectively, p=0.4) and index of transferrin saturation (26.7±19.1%, 22.5±13.2%, 25.5±19.7, respectively, p=0.7) was not different between 25D deficient, insufficient and normal groups. Our study has not shown an association between anemia, iron deficiency and vitamin D. We think that vitamin D has effect on iron metabolism and erythropoiesis through inflammation pathways and hepsidin. Further studies is needed to evaluate the relation between vitamin D and anemia/iron metabolism. [Med-Science 2016; 5(3.000): 821-5

Successful Off-Label Use of Recombinant Factor VIIa and Coil Embolization in an Adolescent with Massive Hemoptysis Due to Invasive Pulmonary Aspergillosis

Invasive fungal infections have turned out to be a significant cause of morbidity and mortality in pediatric patients with malignant disorders. Massive hemoptysis, a rare complication of invasive pulmonary aspergillosis, may threaten the lives of patients, usually during the resolution of neutropenia. In this report, we describe a patient with massive hemoptysis due to invasive pulmonary aspergillosis whose bleeding was controlled successfully with off-label use of recombinant factor VIIa and subsequent coil embolization of the right pulmonary artery

Assessment of physicians' knowledge in transfusion medicine in eastern part of Turkey

Transfusion of blood and blood components is one of the most common medical procedures in the developed world. Knowledge of physicians about blood transfusion is the most important determinant of their approach towards blood transfusion. In this study, we aimed to assess clinicians' basic knowledge regarding transfusion medicine through a questionnaire in a regional hospital. One hundred and one physicians including general practitioners, resident physicians, and specialists participated in the study. The participants were tested with a questionnaire consisting of 20 questions about transfusion medicine. Their scores were compared with regard to their departments, age and experience in medicine. The mean age of study population was 33.1±6 years. Of the total, 70 (69.3%) were male and the average year in medical practice was 7.3±5.8 years. Overall, 52% of the questions were correctly answered. The proportion of correct answers to the questions about basic knowledge, clinical use of blood, and transfusion reactions were as; 52.7%, 54.7%, and 47.3%, respectively. Thirty-four (33.7%) participants scored higher than 60 points, out of 100 points. The average score of total knowledge of participants about transfusion medicine was 52±10. The total knowledge scores of groups including internal departments, surgical departments, and emergency medicine were 55.7±10.2, 51.5±8.3, and 46±10.3, respectively and the differences between three groups were significant (p=0.001) . Also, a positive correlation has been shown between age, experience in medicine and total awareness score in transfusion medicine. The study yielded low/moderate levels of knowledge about transfusion medicine and it was concluded that additional education in transfusion medicine is necessary in all specialties and at each phases of medical practice. [Med-Science 2017; 6(2.000): 208-12

BETA-GLOBIN GENE MUTATIONS IN TURKISH CHILDREN WITH BETA-THALASSEMIA: RESULTS FROM A SINGLE CENTER STUDY

Introduction: The beta thalassemias are common genetic disorders in Turkey and in this retrospective study our aim was to evaluate β-globin chain mutations and the phenotypic severity of β-thalassemia patients followed-up in our hospital, a tertiary center which serves patients from all regions of Turkey. Materials and Methods: 106 pediatric patients were analysed for β-globin gene mutations by using DNA analysis. Patients were classified as having β-thalassemia major or β-thalassemia intermedia based on age at diagnosis, transfusion frequency and lowest hemoglobin concentration in between transfusions. Results: There were 106 patients (52.8% female and 47.2% male) with a mean age of 11.2±5 years (1.6 – 22.3 years). Eighty-four (79.2%) patients had β-thalassemia major, whereas the remaining 22 patients (20.8%) were identified as having β-thalassemia intermedia. Overall, 18 different mutations were detected on 212 alleles. The most frequently encountered mutation was IVS I.110 (G>A) (35.3%), followed by Codon 8 del-AA (10.4%), IVS II.1 (G>A) (8%), IVS I.1 (G>A) (7.5%), Codon 39 (C>T) (7.1%) and Codon 5 (-CT) (6.6%), which made up 79.4% of observed mutations. According to present results, IVS I.110 (G>AA) was the most frequent mutation observed in this study, as in other results from Turkey. Evaluation of β-thalassemia mutations in 106 patients with 212 alleles, revealed the presence of homozygous mutation in 85 patients (80.2%) and compound heterozygous mutation in 21 patients (19.8%). The mutations detected in patients with homozygous mutation were IVS I.110 (G>A) (38.8%), Codon 8 del –AA (11.8%), IVS II.1 (G>A) (8.2%) and IVS I.1 (G>A) (8.2%). Observed mutations in the compound heterozygotes were Codon 39 (C>T)/Codon 41-42 (-CTTT) (14.3%), IVS I.110 (G>A)/Codon 39(C>T) (14.3%), IVS I.110 (G>A)/Codon 44(-C) (14.3%), and IVS II.745 (C>G)/ 5’UTR + 22 (G>A) (9.5%). Conclusion: Our hospital is a tertiary referral center that provides care to patients from all over the country, and thus the distribution of mutations observed in the current study is significant in term of representing that of the country as a whole

Evaluation of Endocrine Late Complications in Childhood Acute Lymphoblastic Leukemia Survivors: A Report of a Single-Center Experience and Review of the Literature

Objective: Improvement in long-term survival in patients with acute lymphoblastic leukemia (ALL) in childhood has led to the need for monitorization of treatment-related morbidity and mortality. In the current study, we aimed to evaluate endocrine side effects of treatment in ALL survivors who were in remission for at least 2 years. Materials and Methods: Sixty patients diagnosed with ALL, who were in remission for at least 2 years, were cross-sectionally evaluated for long-term endocrine complications. Results: The median age of the patients at the time of diagnosis, at the time of chemotherapy completion, and at the time of the study was 5 years (minimum-maximum: 1.7-13), 8 years (minimummaximum: 4.25-16), and 11.7 years (minimum-maximum: 7-22), respectively, and median follow-up time was 4 years (minimummaximum: 2-10.1). At least one complication was observed in 81.6% of patients. Vitamin D insufficiency/deficiency (46.6%), overweight/ obesity (33.3%), and dyslipidemia (23.3%) were the three most frequent endocrine complications. Other complications seen in our patients were hyperparathyroidism secondary to vitamin D deficiency (15%), insulin resistance (11.7%), hypertension (8.3%), short stature (6.7%), thyroid function abnormality (5%), precocious puberty (3.3%), and decreased bone mineral density (1.7%). There were no statistically significant correlations between endocrine complications and age, sex, and radiotherapy, except vitamin D insufficiency/deficiency, which was significantly more frequent in pubertal ALL survivors compared to prepubertal ALL survivors (57.5% and 25%, respectively, p=0.011). Conclusion: A high frequency of endocrine complications was observed in the current study. The high frequency of late effects necessitates long-term surveillance of this population to better understand the incidence of late-occurring events and the defining of high-risk features that can facilitate developing intervention strategies for early detection and prevention