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Journal of Emergency Medicine 02/2012; 43(1):e65-6. · 1.31 Impact Factor
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ABSTRACT: In Henoch-Schönlein purpura (HSP), involvement of the ileum and ascending colon with vasculitis can mimic appendicitis and cause unnecessary appendicectomy. A 13-year-old boy presented with signs of HSP and abdominal pain. He was treated with prednisolone (2 mg/kg/day) for 10 days, but there was no improvement. At laparotomy he had acute suppurative appendicitis. A 12-year-old girl presented with HSP associated with abdominal pain and bloody diarrhoea and at laparotomy was also found to have suppurative appendicitis. Both patients had vasculitic areas in the ileum. In HSP, although suppurative appendicitis is rare, it should always be considered and appropriate investigations, including ultrasonography, undertaken.
Paediatrics and international child health. 01/2012; 32(4):244-5.
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ABSTRACT: Wolfram syndrome, also named "DIDMOAD" (diabetes insipidus, diabetes mellitus, optic atrophy, and deafness), is an inherited association of juvenile-onset diabetes mellitus and optic atrophy as key diagnostic criteria. Renal tract abnormalities and neurodegenerative disorder may occur in the third and fourth decade. The wolframin gene, WFS1, associated with this syndrome, is located on chromosome 4p16.1. Many mutations have been described since the identification of WFS1 as the cause of Wolfram syndrome. We identified a new homozygous WFS1 mutation (c.1532T>C; p.Leu511Pro) causing Wolfram syndrome in a large inbred Turkish family. The patients showed early onset of IDDM, diabetes insipidus, optic atrophy, sensorineural hearing impairment and very rapid progression to renal failure before age 12 in three females. Ectopic expression of the wolframin mutant in HEK cells results in greatly reduced levels of protein expression compared to wild-type wolframin, strongly supporting that this mutation is disease-causing. The mutation showed perfect segregation with disease in the family, characterized by early and severe clinical manifestations.
European journal of medical genetics 01/2012; 55(1):37-42. · 1.57 Impact Factor
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ABSTRACT: Brucellosis is a multisystem disease with a broad spectrum of clinical manifestations; hematological abnormalities ranging from fulminant as of disseminated intravascular coagulation (DIC) to anaemia, leucopoenia, thrombocytopenia, and clotting disorders. In this report, we present DIC in a case of brucellosis because of rare presentation. A 3-year-old boy was admitted with the complaints of continuous fever, vomiting, abdominal pain, and bruise on leg. He looked pale and his physical examination revealed purpuric skin lesions on both legs. A mild hepatosplenomegaly was noted on palpation. Laboratory tests showed hematocrit 21%, hemoglobin 7 g/dL, platelet count 20,000/mm(3), prothrombin time 19 seconds, activated partial thromboplastin time 48 seconds, plasma fibrinogen level 20 mg/dL, and D-dimer 8 µg/mL. Serum antibrucella titration agglutination test was found to be 1 of 1280 positive. Blood cultures performed on specimens obtained at the time of admission yielded Brucella melitensis. The clinical and laboratory findings were consistent with DIC.
Clinical and Applied Thrombosis/Hemostasis 11/2011; 17(6):E10-2. · 1.33 Impact Factor
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ABSTRACT: In the current study, clinical and laboratory findings and treatment modalities of children with acute and chronic immune thrombocytopenic purpura (ITP) were evaluated retrospectively. Our purpose was to determine clinical outcome of children with ITP and their responses to different treatment regimes. Total of 260 children with ITP were enrolled in the study. The mean age of patients was 76.8 ± 48.1 months. The therapy responses of high-dose methylprednisolone (HDMP; n = 134), standard dose methylprednisolone (n = 32), and intravenous immunoglobulin (IVIG; n = 15) treatments were similar. Two (0.8%) of the 260 ITP patients had intracranial bleeding. None of the patients died due to hemorrhage. Of all the patients with ITP, 191 (73.5%) completely resolved within 6 months after initiation of the disease and therefore they were diagnosed as acute ITP; 69 patients (26.5%) had progressed into chronic ITP. The therapy responses of HDMP, standard dose methylprednisolone, and IVIG treatments are similar.
Clinical and Applied Thrombosis/Hemostasis 11/2011; 17(6):E30-5. · 1.33 Impact Factor
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ABSTRACT: Hematologic abnormalities of mild anemia and leucopenia have been frequently associated with acute brucellosis, but thrombocytopenia are less frequently seen. In the present study, we documented 5 (2.6%) isolated thrombocytopenic patients with the manifestations of brucellosis observed during the course of active infection. Five (2.6%) patients, 4 boys and 1 girl, with ages ranging from 2 to 14 years, had isolated thrombocytopenic at diagnosis. In 5 (2.6%) patients, platelet counts ranged from 39,000 to 120,000/mm(3). Tube agglutination tests for brucellosis were positive for all patients (1/160-1/1280). All patients recovered completely, and their thrombocytopenia returned to normal by 2 to 4 weeks after antibiotic treatment of brucellosis. In our study, we present 5 patients with Brucella-induced thrombocytopenia mimicking idiopathic thrombocytopenic purpura to emphasize the isolated thrombocytopenia and the resolution of thrombocyte counts following treatment of brucellosis.
Clinical and Applied Thrombosis/Hemostasis 11/2011; 17(6):E36-8. · 1.33 Impact Factor
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ABSTRACT: Ethylenediaminetetraacetic acid (EDTA)-dependent pseudothrombocytopenia (PTCP) is the phenomenon of a spurious low platelet count due to antiplatelet antibodies that cause platelet clumping in blood anticoagulated with EDTA. The aggregation of platelets in EDTA-dependent PTCP is usually prevented by other anticoagulants, such as sodium citrate or heparin. EDTA-dependent PTCP has never been associated with hemorrhagic diathesis or platelet dysfunction. In this article, a 10-year-old boy with EDTA- and heparin-dependent PTCP is presented because of rare presentation. We report that EDTA and heparin can induce platelet clumping, and thus spuriously low platelet counts. However, aggregation of platelets was not detected in blood samples with sodium citrate, and platelet count was normal.
Clinical and Applied Thrombosis/Hemostasis 10/2011; 17(5):494-6. · 1.33 Impact Factor
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ABSTRACT: Brucellosis produces a variety of nonspecific hematologic abnormalities. Hematologic complications of mild anemia and leukopenia have been frequently associated with acute brucellosis, but pancytopenia is less frequently seen. In this study, records of children with brucellosis aged under or equal to 16 years, admitted to Yuzuncu Yil University Hospital between 2004 and 2010, were analyzed retrospectively. Over this time period, 187 patients with brucellosis were diagnosed. Twenty-five (13.3%) of 187 patients had pancytopenia during admission to hospital. The diagnosis of brucellosis was confirmed by standard tube agglutination test in all patients; titers were 1:320 in 1 patient and 1:1280 in 24 patients. Blood culture was positive for Brucella melitensis in 3 patients (12%). Fever was the most common manifestation, followed by malaise, anorexia, sweating, and weight loss. Fever and splenomegaly were the common signs in most patients. In addition, arthritis was observed in 5 patients, and epistaxis, headache, and abdominal pain were observed in 3 patients. The common bone marrow aspiration findings consisted of increased megakaryocytes and hyperplasia of erythroid series, with a shift to the left of the granulocytic series. Histiocytic hyperplasia was observed in the bone marrow smear of 2 patients. Mild hemophagocytosis was observed in the bone marrow of 3 patients. All patients recovered completely, and their peripheral blood counts returned to normal by 2 to 6 weeks after antibiotic treatment of brucellosis. In conclusion, the authors would like to emphasize that brucellosis should be considered in the differential diagnosis of children with pancytopenia.
Pediatric Hematology and Oncology 04/2011; 28(3):203-8. · 0.89 Impact Factor
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ABSTRACT: Initial presentation of Non-Hodgkin Lymphoma (NHL) as primary pleural lymphoma is extremely rare. We report a case with NHL, who was initially presented with pleural effusion and pleural thickening. Our patients at first received intensive treatment of broad spectrum antibiotics for 10 days with no change in the clinical status, and then were diagnosed with T-lymphoblastic lymphoma with the examination of pleural fluid by using cytologic and flow cytometric methods, and treated with LMT89 group B treatment protocol. Our case points out the necessity for considering the NHL within the differential diagnosis of any pediatric patient presenting with sterile pleural effusion and pleural thickening who does not respond to antimicrobial therapy.
Journal of Pediatric Hematology/Oncology 03/2011; 33(5):e192-4. · 1.16 Impact Factor
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ABSTRACT: Primary germ cell tumors may spread to the lung, liver, brain, and bone hematogenously. However, spinal metastasis is extremely rare. A case with yolk sac tumor (YST) and cauda equina metastasis was presented in a 2-year-old boy. Two months prior to admisssion, he underwent left radical orchiectomy for testicular YST. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed spinal and cauda equina involvement. This is the first report of metastasis of testicular YST to cauda equina in a child.
Fetal and pediatric pathology 02/2011; 30(3):150-5. · 0.36 Impact Factor
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ABSTRACT: Four children with vincristine (VCR)-induced neuropathy are being reported. All cases were followed with the diagnosis of acute lymphoblastic leukemia. Two were boys aged between 2 and 13 year. Electromyographic examination consisted of sensoriomotor polyneuropathy with axonal involvement in three patients. In another patient, it consisted of motor axonal polyneuropathy. In all patients, pyridoxine and pyridostigmine were successfully used in the treatment of VCR-induced neuropathy. They recovered completely with this drug combination. Recovering period of symptoms was between 1-2 week.
The Indian Journal of Pediatrics 06/2010; 77(6):681-3. · 0.52 Impact Factor
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ABSTRACT: Although hypertension is a well-documented risk factor for cardiovascular diseases, including myocardial infarction and stroke, in adults, only recently has knowledge about childhood hypertension increased significantly.
To determine the incidence of asymptomatic hypertension in school-age children.
Children in primary school were chosen with a randomized sampling method. During measurement of blood pressure, a calm, comfortable setting was provided. Blood pressure measurements were performed by only 1 researcher. For accurate measurement of blood pressure, recommended standards were followed.
A total of 1,963 children were included in the study. The incidence of systolic hypertension and diastolic hypertension were 7% and 2%, respectively. Obesity was present in 10.5% girls with hypertension and 13.9% of boys with hypertension.
Our findings indicate that hypertension is an important health problem in children, and its prevalence is quite high. Blood pressure measurements must be a part of routine clinical examinations. Further studies should be performed in high-risk populations to prevent hypertension and to establish methods of early diagnosis and treatment in children.
Journal of Nippon Medical School 06/2010; 77(3):160-5.
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ABSTRACT: Immune thrombocytopenic purpura (ITP) of childhood is a common hematologic disorder. Immune thrombocytopenic purpura is characterized by increased destruction of antibody-coated platelets in the reticuloendothelial system. In the majority of children with acute ITP, thrombocytopenia occurs within 1 to 3 weeks after an infectious disease. Immune thrombocytopenic purpura may also occur after rubella, rubeola, chickenpox, or live virus vaccination. Here we report 2 cases with acute ITP that were developed after honeybee and insect bite.
Clinical and Applied Thrombosis/Hemostasis 05/2010; 17(4):408-9. · 1.33 Impact Factor
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ABSTRACT: Thrombotic thrombocytopenic purpura (TTP) is characterized by disseminated thrombotic occlusions located in the microcirculation and a syndrome of microangiopathic hemolytic anemia, thrombocytopenia, fever, and renal and neurologic abnormalities. Thrombotic thrombocytopenic purpura is encountered in a variety of clinical situations such as viral, bacterial, and mycobacterial infections, autoimmune disorders, drug reactions, connective tissue disease, and solid tumors. In this report, we present TTP in a case of brucellosis because of rare presentation. A 7-year-old girl was admitted with the complaints of headache, fever, hematuria, malaise, jaundice, epistaxis, and purpura. Her physical examination revealed conjunctival pallor, scleral icterus, petechial-purpuric skin lesions on both legs, and confusion. Laboratory tests showed hematocrit 14%; hemoglobin 4.8 g/dL; platelet count 6000/mm(3), and reticulocytosis 6%. Peripheral blood smear revealed fragmented red blood cells and a complete absence of platelets. The clinical and laboratory findings were consistent with TTP. Serum antibrucella titration agglutination test was found to be 1/1280 positive.
Clinical and Applied Thrombosis/Hemostasis 03/2010; 17(3):245-7. · 1.33 Impact Factor
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ABSTRACT: A 3-day-old boy was admitted for right preauricular swelling. Examination showed a toxic looking neonate with poor neonatal reflexes. There was erythema and swelling of 4 x 3 cm at the level of the right cheek. Laboratory investigation pointed to a bacterial infection. With parenteral antibiotics, the lesion resolved completely.
European Journal of Pediatrics 01/2010; · 1.88 Impact Factor
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ABSTRACT: Langerhans cell histiocytosis (LCH) is an uncommon clinically heterogeneous disorder characterized by the proliferation and accumulation of Langerhans cells with local infiltration of tissues and organ destruction. LCH takes many clinical forms, affecting different systems and different sites in the same system with variable outcomes. Bone, skin, lymph node, pituitary, liver, lung, bone marrow and spleen involvement can be seen in patients with LCH. Involvement of the perianal site is rare. In this article, a 16-month-old boy with multiple organ involvement including skin, liver, lung, and bone is presented. Aside from these systemic involvements, he also had a simple anal fistula. According to our best knowledge, this case of LCH with anal fistula is only the second to be reported in childhood. We would like to emphasize that LCH may be associated with anal fistula; therefore, we suggest that patients with LCH should be examined for this condition.
The Kurume Medical Journal 01/2009; 56(3-4):79-83.
