Imatinib Treatment Alone in Philadelphia-Positive Acute Lymphoblastic Leukemia: Is It Enough?

BCR-ABL fusion gene t(9;22)(q34;q11) occurs in only 3% of pediatric acute lymphoblastic leukemia (ALL) cases. Previously, less than 40% of Philadelphia-positive ALL patients were cured with intensive chemotherapy. The use of imatinib (340 mg/m2/day) added to an intensive chemotherapy regimen has improved the outcome in this population at 3 years to an event-free survival of 80%. Imatinib treatment alone was administered after remission induction chemotherapy to a patient with Philadelphia-positive ALL who presented with serious chemotherapy toxicity, so that intensive chemotherapy could not be maintained. This is the only patient in the literature who survived remission for more than 2.5 years with imatinib treatment only

Severe lupin bean intoxication: an anticholinergic toxidrome

Lupin beans are the yellow legume seeds of the genus Lupinus. They are traditionally eaten as a pickled snack in many countries. Bitter lupin beans have a high amount of alkaloids called lupanine. Because the alkaloid is responsible for anticholinergic toxidrome, the beans should pass a debittering process before consumption. Only few pediatric cases of lupin bean intoxication were reported. We present a 12-year-old boy who visited the emergency department for the unexpected onset of anticholinergic toxidrome

Pulmonary involvement in 3 SLE cases

WOS: 00029070310036

Inferior Petrosal Sinus Thrombosis in a Child due to Malposition of Central Venous Catheter: A Case Report

WOS: 000426011000013PubMed ID: 29080892Objective: To report a case of inferior petrosal sinus thrombosis associated with malposition of central venous catheterization (CVC). Clinical Presentation and Intervention: A 5-month-old boy was admitted to Sifa University Hospital because of pneumonia. When exophthalmos occurred in the right eye, he was referred to Ege University Hospital. Cranial magnetic resonance imaging and magnetic resonance venography confirmed that the catheter in the right inferior petrosal sinus caused the thrombosis. The catheter was extracted and anticoagulant treatment was started. Conclusion: In this case report, malpositioning of the CVC was the cause of the thrombosis. To minimize such complications, catheterization should be done with the supervision of an expert and postprocedure radiography should also be performed. (C) 2017 The Author(s) Published by S. Karger AG, Base

An Achondroplasic Case with Foramen Magnum Stenosis, Hydrocephaly, Cortical Atrophy, Respiratory Failure and Sympathetic Dysfunction

Background: Achondroplasia is a relatively frequent genetic disorder that may lead to limb weakness, motormental retardation, hydrocephaly, and respiratory disorders. In this pathology, foramen magnum stenosis and accompanying disorders like respiratory depression is well documented. Case Presentation: A 2.5 year-old child with the diagnosis of achondroplasia admitted to our clinic with severe respiratory depression, limb weakness, and motor mental retardation as well as sympathetic dysfunction. In radiologic evaluations, severe foramen magnum stenosis was detected. The patient was operated and posterior fossa decompression was accomplished to prevent compression of respiratory centers and neurons. Conclusion: This case is unique with the narrowest foramen magnum reported up to date and the sympathetic dysfunction which is not reported as a complication in achondroplasic patients. The authors review the relevant literature, focusing on the indications for cervicomedullary decompression in infants with achondroplasia

Trichosporon Asahii, Sepsis, and Secondary Hemophagocytic Lymphohistiocytosis in Children with Hematologic Malignancy

WOS: 000335379000011PubMed ID: 24383917Trichosporon asahii (T. asahii) is an uncommon fungal pathogen rarely seen in patients with hematologicmalignancies. Although appropriate therapy is started, infection with T. asahii usually leads to mortality. Here, we describe two patients developed severe T. asahii infection and secondary HLH. Despite rapid identification of T. asahii and negative blood cultures achieved by prompt initiation of treatment with voriconazole, fever and pancytopenia, persisted and both developed hepatosplenomegaly, and their clinical state worsened. Bone marrow aspiraton revealed hemophagocytosis. Elevated ferritin, triglyceride levels were seen. The first patient did not receive HLH directed therapy and died with multiple organ dysfunctions. Prompt diagnosis and treatment of secondary HLH led to rapid improvement in clinical and laboratory abnormalities in the second patient and kept her alive. We suggest that HLH may present as a secondary condition, accompanying a severe infection with T. asahii may, at least in part, contribute to high mortality rates in these cases

Soft tissue infection caused by Burkholderia cepacia in a child with polyarteritis nodosa

WOS: 000209048700018PubMed ID: 23692797Burkholderia cepacia belongs to a family of Burkholderia species previously described as Pseudomonas cepacia, especially in patients suffering from cystic fibrosis. There are also many studies about this agent in the last decade due to their life-threatening infections and ability to invade mucosal and cellular surfaces. Here, we report a case of soft tissue infection caused by B. cepacia in a child with an underlying condition of polyarteritis nodosa. Her complaints started at two months of age and she was on cyclosporine therapy. She was treated several times because of soft tissue infections especially in her extremities. The most common causative agents were Pseudomonas spp. and Escherichia coli, but recently, another soft tissue infection accompanied by fever and signs of sepsis had developed. All blood, urine and tissue (debrided from the necrotic area) specimens were incubated. Empirical antibiotherapy with clindamycin was started and cyclosporine therapy was discontinued. B. cepacia was grown in the tissue specimen culture and was only susceptible to carbapenems. Meropenem therapy was administered throughout 14 days with a daily dosage of 60 mg/kg, and she was treated successfully at least in this attack of soft tissue infection, which caused more severe sepsis and tissue damage than the previous infections with other agents

Severe aplastic anemia following liver transplantation in a patient with non-A-E fulminant hepatitis

WOS: 000293434100017PubMed ID: 21805427Aplastic anemia is a rare complication after liver transplantation. Its incidence has been estimated to be 0.007%. Aplastic anemia was observed in 23.2 - 33% of patients who underwent liver transplantation for fulminant non-A-E viral hepatitis. In this paper, we describe a child suffering from aplastic anemia who eventually died from sepsis after liver transplantation for non-A-E fulminant hepatic failure