Füsun Dilara İçağasıoğlu

Crimean-Congo hemorrhagic fever disease due to tick bite with very long incubation periods.

BACKGROUND Crimean-Congo hemorrhagic fever (CCHF) is a zoonotic viral disease with a high mortality rate, and is one of the viral hemorrhagic fever syndromes. The average mortality rate of CCHF is 3-30%. Research indicates that the longest incubation period after a tick bite is 12 days in CCHF disease. However, in clinical practice, we encounter patients with CCHF as a result of tick bites with much longer incubation periods (max. 53 days) than those reported in the literature. We present herein CCHF cases presumably infected through tick bites and having incubation periods longer than the upper limit reported in the literature. METHODS We analyzed the cases of the 825 CCHF patients admitted to our hospital from 2007 to 2010 and found that 312 of them had undoubtedly been bitten by a tick. We searched the patient records for information on the incubation period and found that 12 patients had experienced an incubation period of over 12 days, which is the longest incubation period stated in the literature for patients definitely bitten by a tick. RESULTS A total of 12 patients (eight males and four females, with a mean age of 45 years) were recruited into this study. Five (41.7%) of the 12 patients had positive CCHF virus-specific IgM antibodies, three (25%) had a positive reverse transcription polymerase chain reaction test for CCHF virus, and four (33.3%) had positive results in both tests during the acute and/or convalescent phase of the disease. In these cases, the interval between tick bite and the onset of symptoms was a mean of 23.6 days (range 13-53 days). CONCLUSION Physicians serving in endemic regions should be aware of these longer incubation periods after a tick bite. It is suggested that they perform more follow-ups on clinically and serologically highly suspected patients than they currently do.

Can the mild clinical course of crimean–congo hemorrhagic fever in children be explained by cytokine responses?

Cytokines are possibly one of the factors responsible for death due to Crimean–Congo hemorrhagic fever (CCHF). This study aimed to determine the differences between the cytokine levels in children and adult patients with CCHF; the influence of cytokines; and the severity of the course of the disease, which seems to be milder in children. Thirty‐four children and 36 adult patients diagnosed with CCHF between 2010 and 2011 were included in this study. Diagnosis was performed by serology or by the polymerase chain reaction for CCHF virus. Levels of IFN‐γ, TNF‐α, IL‐1β, IL‐2, IL‐4, IL‐5, IL‐6, IL‐9, IL‐10, IL‐12 p70, IL‐13, IL‐17A, and IL‐22 were measured in all serum samples. Although the disease had a fatal course in three adult patients, there were no deaths in children. Statistically significant differences were not observed between the cytokine concentrations in the adults and children. No differences were detected between the serum cytokine levels in the children with moderate and those with a severe clinical course of the disease. In the adult patients with fatal outcome, significantly higher serum levels of IL‐2, IL‐5, IL‐9, IL‐12 p70, and IL‐13 were detected as compared to the cytokine levels in patients who survived the infection. No differences were detected between the serum levels of IFN‐γ, IL‐1β, IL‐17A, IL‐22, IL‐10, IL‐6, IL‐4, and TNF‐α in the patients who died and those who survived. Thus, the milder clinical course in children with CCHF cannot be explained by the cytokine network alone. The incomplete maturation of the immune system and timing and scale of immune responses could change the outcome dramatically. J Med. Virol. 85:1955–1959, 2013.

Cardiac findings in children with Crimean-Congo hemorrhagic fever

Summary Background Crimean-Congo hemorrhagic fever (CCHF) involves the multi-organ systems. The involvement of the heart in adult patients has been described previously. We investigated the electrocardiographic and echocardiographic findings of pediatric patients with CCHF. Material/Methods Patients younger than 16 years of age diagnosed with CCHF were enrolled in the study. The diagnosis of CCHF infection was based upon typical clinical and epidemiological findings and serological tests. All patients underwent a thorough cardiologic evaluation. A standard 12-lead electrocardiography and echocardiography were performed. Results Twenty-three consecutive patients who were hospitalized with diagnosis of CCHF were enrolled in the study (mean age: 12±2 years, 6 female). All electrocardiographic parameters were within normal ranges according to age. Seven patients (30%) had minimal (<1 cm) pericardial effusion. Fifteen (65%) patients had segmental wall motion abnormalities (hypokinesia). A second echocardiography revealed that all wall motion abnormalities had disappeared; the pericardial effusion persisted in only 2 of 7 patients (28%). Conclusions Cardiac involvement appears to be more frequent in children with CCHF disease than in adults, but it is slighter and almost totally reversible; however, the course of the disease in children is milder than it is in adults.

Treatment failure of gentamicin in pediatric patients with oropharyngeal tularemia

Summary Background Tularemia is a zoonotic infection, and the causative agent is Francisella tularensis. A first-line therapy for treating tularemia is aminoglycosides (streptomycin or, more commonly, gentamicin), and treatment duration is typically 7 to 10 days, with longer courses for more severe cases. Material/Methods We evaluated 11 patients retrospectively. Failure of the therapy was defined by persistent or recurrent fever, increased size or appearance of new lymphadenopathies and persistence of the constitutional syndrome with elevation of the levels of the proteins associated with the acute phase of infection. Results We observed fluctuating size of lymph nodes of 4 patients who were on the 7th day of empirical therapy. The therapy was switched to streptomycin alone and continued for 14 days. The other 7 patients, who had no complications, were on cefazolin and gentamycin therapy until the serologic diagnosis. Then we evaluated them again and observed that none of their lymph nodes regressed. We also switched their therapy to 14 days of streptomycin. After the 14 days on streptomycin therapy, we observed all the lymph nodes had recovered or regressed. During a follow-up 3 weeks later, we observed that all their lymph nodes had regressed to the clinically non-significant dimensions (<1 cm). Conclusions All patients were first treated with gentamicin, but were than given streptomycin after failure of gentamicin. This treatment was successful in all patients. The results of our study suggest that streptomycin is an effective choice of first-line treatment for pediatric oropharyngeal tularemia patients.

Elevated chemokine levels during adult but not pediatric Crimean–Congo hemorrhagic fever

BACKGROUND Crimean-Congo hemorrhagic fever (CCHF) is a tick-borne viral zoonosis. Clinical reports indicate the severity of CCHF is milder in children than adults. The chemokines are important chemo-attractant mediators of the host immune system. OBJECTIVES The main aim of the study was to identify whether or not there were any differences in chemokine levels between the pediatric and adult patients and control groups, and whether there was any correlation with disease severity. STUDY DESIGN The serum levels of select chemokines including chemokine (C-C) ligand 2 (CCL2), CCL3, CCL4, chemokine (C-X-C) ligand 8 (CXCL8), CXCL9, and granulocyte-colony stimulating factor (G-CSF) in 29 adult and 32 pediatric CCHF patients and in 35 healthy children and 40 healthy adult control groups were studied by flow cytometric bead immunoassay method. RESULTS Great variability was detected in the serum levels of the chemokines for both the adult and pediatric patients and controls. With the exception of G-CSF, the median serum levels of CCL2, CCL3, CCL4, CXCL8, and CXCL9 were found to be significantly higher in the adult patients compared to adult controls (2364.7 vs. 761 pg/ml; 714.1 vs. 75.2 pg/ml; 88.6 vs. 25.5 pg/ml; 217.9 vs. 18.3 pg/ml; 875 vs. 352.2 pg/ml, respectively, p < 0.0001 for all comparisons). Among the chemokines the median CCL4 and G-CSF levels were significantly higher in the pediatric patients compared to pediatric controls (40.3 vs. 7.1 pg/ml, p < 0.0001; 0.1 vs. 0.1 pg/ml, p = 0.049, respectively). CONCLUSION The results of this study showed prominent chemokine raising in adult CCHF patients compared to children CCHF patients.

Evaluation of Serum Perforin, Caspase-3, sFasL, and M-30 Levels as Apoptotic Markers in Children with Crimean-Congo Hemorrhagic Fever

Background: Apoptosis is a main regulator in responses of cellular immunity throughout systemic viral infections. Perforin, soluble Fas ligand, caspase-3 and caspase-cleaved cytokeratin-18 (M-30) are mediators of apoptosis. The aim of this study is the evaluation of Crimean-Congo hemorrhagic fever (CCHF) disease changes in the levels of these apoptotic markers and the relation of these changes with disease severity. Methods: Forty-nine hospitalized children with CCHF and 36 healthy controls were enrolled in this prospective study. The CCHF patients were classified into 2 groups based on disease severity (severe group and nonsevere group). Demographic characteristics and clinical and laboratory findings of all patients were recorded on admission. Results: Serum perforin, caspase-3 and soluble Fas ligand levels were found to be significantly higher both in the severe and nonsevere CCHF groups than the healthy control group (P < 0.05), but there was no significant difference in these apoptotic markers between severe and nonsevere CCHF groups (P > 0.05). In addition, serum M-30 levels did not differ significantly among all groups (P > 0.05). There was a positive correlation between serum values for perforin, caspase-3 and M-30 and the disease’s severity criteria such as aspartate aminotransferase and/or alanine aminotransferase. The serum levels of all these markers were negatively correlated with disease severity criteria such as the platelet count. Conclusions: In this study, we concluded that the interactions of cytolytic granules containing perforin and caspase cascade and Fas-FasL may play an important role in the pathogenesis of CCHF in children.

Serum adiponectin, leptin, and interleukin 6 levels as adipocytokines in children with febrile seizures The role of adipose tissue in febrile seizures

Proinflammatory and anti-inflammatory cytokines have an important role in the pathogenesis of febrile seizures (FS). Adipocytokines like interleukin 6 (IL-6), leptin, and adiponectin released from adipose tissue play a role in inflammation. This study aimed to assess the probable role of adipose tissue in children with FS. We measured serum IL-6, leptin, and adiponectin levels and evaluated clinical and laboratory findings in children with FS (n = 32) and compared the results with the values of children of the same age with febrile illness without seizures (febrile control, FC; n = 26) and healthy control group (HC; n = 29). The serum levels of white blood cells, C-reactive protein, IL-6, leptin, and adiponectin were found to be significantly higher, while serum hemoglobin (Hb) levels were found to be significantly lower in FS and FC groups than in the HC group (p < 0.001). When we compared the FS with the FC group, the serum Hb levels were significantly lower in the FS group than those in the FC group (p = 0.001). There was no significant difference between the FS and FC group with regard to the serum levels of these adipocytokines (p > 0.05). Our data showed that elevated levels of these adipocytokines as acute phase reactants in FS and FC groups did not contribute to the development of FS.

Assessment of 17 Pediatric Cases With Colchicine Poisoning in a 2-Year Period.

AimThe aim of the study is to discuss clinical effects, treatments, and outcomes of pediatric colchicine poisoning. MethodThis study was designed as an observational case series study. The medical records of children aged between 0 and 18 years, who were hospitalized for colchicine poisoning at the Department of Pediatric Intensive Care Unit, Cumhuriyet University Faculty of Medicine, between January 2010 and January 2012, were retrospectively evaluated. ResultsWe presented 17 children with colchicine poisoning. The mean (SD, range) age of patients was 71.5 (69.19, 18–204) months. The period to apply to the hospital after taking the medications was 7.3 hours (7.97, 30 minutes-26 hours) on average. The use of colchicine was due to diagnosis of Familial Mediterranean fever (FMF) in the families of 8 patients, diagnosis of Behçet disease in 1 patients father, diagnosis of Behçet disease in 1 patient herself, and diagnosis of FMF in 6 patients themselves. Thirteen patients had taken colchicine at the dose of less than 0.5 mg/kg known as subtoxic and 1 patient had taken colchicine at the dose of greater than 0.8 mg/kg, and doses taken by 3 patients were not known. Fourteen patients (82.4%) had involuntary drug intake. Fifty percent of them were symptomatic at the moment of application and all had gastrointestinal complaints. All patients were observed in intensive care unit upon first admission and received supportive care. One of patients showed total alopecia, one showed leucocytosis, and another one showed acute abdomen picture. None of the patients showed mortality. ConclusionsMortality of colchicine toxicity is high and quick assessment is absolutely required. In regions where FMF is common and the use of colchicine is high, clinicians should pay attention to symptoms and findings related to colchicine intoxication and keep them in mind in differential diagnosis.

Three Cases of Walker Warburg Syndrome

DOI: 10.4328/JCAM.833 Received: 07.11.2011 Accepted: 08.11.2011 Printed: 01.07.2014 J Clin Anal Med 2014;5(4): 338-40 Corresponding Author: Adnan Ayvaz, Cumhuriyet Universitesi, Tip Fakultesi Pediatrik Noroloji, 58040 Sivas, Turkiye. GSM: +905339218131 T.: +90 3462581182 / +90 3462581179 E-Mail: ayvaz@ttmail.com Ozet Walker Warburg Sendromu (WWS) otozomal resesif gecis gosteren goz, beyin ve kaslarin etkilendigi nadir bir dogumsal muskuler distrofi tipidir. Bu yazida Cumhuriyet Universitesi Cocuk Sagligi ve Hastaliklari kliniginde takip edilen uc WWS olgusu sunulmustur. Olgularimizdan biri bilgilerimize gore rapor edilmis en uzun yasayan olgudur.

Fallot Pentalojisi’nin eşlik ettiği Dandy-Walker varyasyonu: Olgu sunumu

Ozet Dandy-Walker varyasyonu, 4. ventrikul ve sisterna magna arasindaki devamlilikla sonuclanan vermisin parsiyel agenezisi ile karakterize santral sinir sisteminin nadir bir anomalisidir. Literaturde ilk kez 1988’de Kohyama ve arkadaslari tarafindan bildirilen Dandy-Walker varyasyonu ve Fallot Tetralojisi birlikteligi daha sonraki yillarda birkac vaka sunumu seklinde bildirilmistir. Biz bu yazida, Dandy-Walker varyasyonu ve Fallot Pentalojisi olan 2 gunluk bir erkek hastayi bildiriyoruz. Anahtar sozcukler: Dandy-Walker varyasyonu, Fallot Pentalojisi Abstract Dandy-Walker variant is a rare anomaly of the central nervous system that is characterized by partial agenesis of vermis and results in persistence of the 4. ventricule and cisterna magna. Firstly, in 1988 Kohyama et al. reported the coexistence of Dandy-Walker variant and tetralogy of Fallot. Afterwards, it is reported as several case reports in the literature. In this case report, we report a 2 days-old male patient with Dandy-Walker variant and pentalogy of Fallot. Keywords: Dandy-Walker variant, pentalogy of Fallot