Cohort Study: Central Venous Catheter-Related Complications In Children With Hematologic Diseases At A Single Center

Objective: This study aims to document and analyze the central venous catheter (CVC)-related complications in children with hematological diseases who were treated within a single institution. Materials and Methods: A retrospective investigation was conducted in 106 pediatric patients in whom 203 CVCs were inserted. A total of 175 catheter-related complications occurred in 5 years. Results: The rates of clinical catheter infections, local catheter infections, venous thromboembolism, bleeding, and mechanical complications were 2.6, 1.1, 0.2, 0.2, and 0.2 per 1000 catheter days. Methicillin-resistant Staphylococcus epidermidis was the predominant infectious organism in blood and catheter cultures. The children with leukemia had a significantly higher frequency of clinical catheter infections (p=0.046). The children who underwent bone marrow transplantation had a significantly lower frequency of clinical catheter infections (p=0.043) and higher frequency of local catheter infections (p=0.003). The children with implanted catheters had a significantly lower frequency of clinical catheter infections (p=0.048). The children with thrombocytopenia had significantly fewer local catheter infections and significantly more clinical catheter infections and catheter-related bleeding (respectively p=0.001, p=0.042, and p=0.024). Conclusion: Leukemia, bone marrow transplantation, and thrombocytopenia are risk factors for CVC-associated complications. The relatively higher number of interventions performed via permanent catheters may be responsible for the significantly increased incidence of systemic infections and mechanical injury.PubMedWoSScopu

Structural And Functional Analysis Of Perforin Mutations In Association With Clinical Data Of Familial Hemophagocytic Lymphohistiocytosis Type 2 (Fhl2) Patients

Perforin plays a key role in the immune system via pore formation at the target cell membrane in the elimination of virus-infected and transformed cells. A vast number of observed mutations in perforin impair this mechanism resulting in a rare but fatal disease, familial hemophagocytic lymphohistiocytosis type 2 (FHL2). Here we report a comprehensive in silico structural analysis of a collection of 76 missense perforin mutations based on a proposed pore model. In our model, perforin monomers oligomerize having cyclic symmetry in consistent with previously found experimental constraints yet having flexibility in the size of the pore and the number of monomers involved. Clusters of the mutations on the model map to three distinct functional regions of the perforin. Calculated stability (free energy) changes show that the mutations mainly destabilize the protein structure, interestingly however, A91V polymorphism, leads to a more stable one. Structural characteristics of mutations help explain the severe functional consequences on perforin deficient patients. Our study provides a structural approach to the mutation effects on the perforin oligomerization and impaired cytotoxic function in FHL2 patients.Wo

Serum Erythropoietin Levels In Pediatric Hematologic Disorders And Impact Of Recombinant Human Erythropoietin Use

Objective: In anemic patients, the correlation between serum erythropoietin (sEpo) level and the severity of anemia has been reported previously. However. in different anemia groups, different sEpo levels are measured in patients with similar hemoglobin levels and the etiology of this situation could not be explained. Methods: We evaluated hemoglobin and sEpo levels in 31 iron deficiency anemia, 26 Fanconi anemia (FA), 21 thallasemia intermedia (TI), 15 acute lymphoblastic leukemia (ALL) patients at presentation and 12 healthy controls. Results: In all disease groups, an inverse linear correlation was shown between hemoglobin and logarhytmic sEpo level. The covariance analyses according to corrected hemoglobin levels exhibited the highest sEpo level in FA, followed by ALL, TI and iron deficiency anemia, sequentialy. Conclusion: There was no statisticaly significant difference of sEpo levels in FA patients in terms of androgen treatment and this finding supports that androgen affects erythropoisis directly, and has no effect on erythropoietin. The results indicate that there is no erythropoietin deficiency in the anemia of these patients and the admnistration of exogenous erythropoietin offers no clinical benefit. (Turk J Hematol 2009; 26: 72-6)Wo

Hypertrichosis: The Possible Side Effect Of Cyclosporin In An Infant With Hemophagocytic Lymphohistiocytosis Receiving Hlh-2004 Chemotherapy Protocol

Hemophagocytic lymphohistiocytosis is a life-threatening condition of severe hyperinflammation that results from an uncontrolled proliferation of activated lymphocytes and histiocytes secreting high amounts of inflammatory cytokines. The immediate treatment strategies include immune suppressive therapy such as corticosteroid, etoposide and cyclosporin A. Herein, we present a 13-month-old infant who developed severe hypertrichosis after the administration of HLH-2004 treatment protocol. We discuss the various hypotheses regarding the causal relationship between cyclosporin A and hypertrichosis, emphasizing the importance of patient follow up. (Turk J Hematol 2009; 26: 154-6)Wo

Pulmonary thromboembolism in childhood: a single-center experience from Turkey.

Wo

Sağlıklı Çocuklarda Gelişen Varisella Enfeksiyonu Sırasında Bağışıklık Sisteminde Geçici Baskılanma

Objective: Varicella is a common childhood infection and has a number of complications in the unvaccinated population. Perforin, found in natural killer cells, is important for the killing of virally infected cells. For this reason, the am of this study was to determine natural killer cell count and activity, perforin expression, and Fas and soluble Fas ligand (sFas-L) levels in immunocompetent children with varicella infection and define any possible relations between the levels and varicella complications. Material and Methods: Forty children were analyzed at diagnosis and on the 15th day of varicella infection. There was a significant difference in hemoglobin levels and leukocyte and platelet counts between days 0 and 15. Results: Thirteen (32%) patients were found to be lymphopenic, Natural killer cell count and activity were significantly higher on day 15 when compared to values at diagnosis. The Fas-mediated apoptotic pathway was found to be active in acute varicella infection because Fas and sFas-L levels at diagnosis were higher than values on day 15. Conclusion: These findings suggest that the Fas and Fas-L apoptotic pathway is active during the acute phase of the viral infection and that it becomes inactive by day 15, paralleling the hematologic recovery. (Turk J Hematol 2009; 26: 12-6)Wo

Hemophagocytic Syndrome and Acute Liver Failure Associated with Ethylene Glycol Ingestion: A Case Report

The authors describe a case of accidental ethylene glycol poisoning in an 18-month-old boy who developed hemophagocytic syndrome (HPS). Ethylene glycol is a common substance in various antifreeze preparations. Acute ethylene glycol intoxication is a medical emergency that, if not diagnosed correctly and treated aggressively, will lead to serious neurological, cardiopulmonary, and renal dysfunction, and may result in death. The taking of a detailed history, physical examination, and laboratory testing are essential for diagnosis. To the best of the authors' knowledge this is the first case in the literature of a subject who developed HPS after ethylene glycol intoxication.Wo

Purpura fulminans as the presenting manifestation in a patient with juvenile SLE

We present a 12-year-old girl with systemic lupus erythematosus and associated antiphospholipid syndrome who developed an unusual manifestation of purpura fulminans in an accelerated fashion. The patient improved after prompt treatment with anticoagulants, aggressive immunosuppressive drugs and plasmapheresis. This is the first pediatric case of purpura fulminans due to secondary antiphospholipid syndrome of systemic lupus erythematosus. We suggest that SLE patients with lupus anticoagulant should be followed closely for similar complications