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Sample records for acute childhood myositis

  1. Benign childhood acute myositis: clinical and laboratory findings of 15 cases

    Saltık, Sema; Sürücü, Murat; Özdemir, Öner

    2012-01-01

    Aim: In this study; clinical and laboratory findings of 15 cases with benign childhood acute myositis are presented to look over pathognomonic findings of the disease Material and Method: Fifteen typical cases with benign childhood acute myositis referred to our Pediatric Neurology Clinic because of inability to walk from 15th of January to 15th of March 2011 were enrolled into this study Eighty percent of cases were male and their mean age was 6 3 years Guillian Barre rsquo;s syndrome was th...

  2. Benign childhood acute myositis: clinical and laboratory findings of 15 cases

    Saltık, Sema; Sürücü, Murat; Özdemir, Öner

    2011-01-01

    Aim: In this study; clinical and laboratory findings of 15 cases with benign childhood acute myositis are presented to look over pathognomonic findings of the disease Material and Method: Fifteen typical cases with benign childhood acute myositis referred to our Pediatric Neurology Clinic because of inability to walk from 15th of January to 15th of March 2011 were enrolled into this study Eighty percent of cases were male and their mean age was 6 3 years Guillian Barre rsquo;s syndrome was th...

  3. Acute dengue myositis with rhabdomyolysis and acute renal failure

    Acharya Sourya

    2010-01-01

    Full Text Available Dengue is an acute mosquito-borne infection caused by dengue viruses from the genus flavivirus. Neurologic complications have been attributed chiefly to metabolic alterations and to focal and sometimes massive intracranial haemorrhages, but anecdotal cases and limited case series have indicated the possibility of viral CNS and skeletal muscle invasion causing encephalitis and myositis. We present a case of a 40-year-old male who presented with severe dengue myositis resulting in quadriparesis, respiratory failure and acute renal failure with red urine. His elevated serum creatine kinase (CK, serum and urine myoglobin levels justified rhabdomyolysis as the cause of acute renal failure. A muscle biopsy revealed inflammatory myositis. He required ventilator support for respiratory failure and was treated conservatively. This case highlights the severe and persistent muscle involvement in dengue which is a rarity.

  4. Orbital hemorrhage and eyelid ecchymosis in acute orbital myositis.

    Reifler, D M; Leder, D; Rexford, T

    1989-02-15

    We examined two patients with acute orbital myositis associated with orbital hemorrhage and eyelid ecchymosis. Both patients were young women (aged 22 and 30 years) who had painful proptosis, diplopia, and computed tomographic evidence of single extraocular muscle involvement with spillover of inflammatory edema into the adjacent orbital fat. Patient 1 showed contralateral preseptal eyelid inflammation and did not suffer an orbital hemorrhage until after an episode of vomiting. In Patient 2, the diagnosis of occult orbital varix was initially considered but an orbital exploration and a biopsy specimen showed no vascular anomaly. Both patients were treated successfully with high-dose systemic corticosteroids. Some cases of idiopathic orbital inflammation may be related to preexisting vascular anomalies or orbital phlebitis. PMID:2913803

  5. Acute hepatitis and myositis associated with Erythema infectiosum by Parvovirus B19 in an adolescent

    Koliou, Maria; Karaoli, Evaggelia; Soteriades, Elpidoforos S; Pavlides, Sylvie; Bashiardes, Stavros; Christodoulou, Christina

    2014-01-01

    Background: Erythema infectiosum is the most common clinical manifestation of Parvovirus B19 infection although it has also been associated with rheumatologic diseases and various types of systemic vasculitides. Acute hepatitis and benign myositis however are rarely reported in association with Parvovirus B19 infection. Case presentation: Here we report a 14-year old male, who developed acute hepatitis and benign myositis associated with erythema infectiosum following Parvovirus B19 infection...

  6. Acute hepatitis and myositis associated with Erythema infectiosum by Parvovirus B19 in an adolescent

    Koliou, Maria; Karaoli, Evaggelia; Soteriades, Elpidoforos S; Pavlides, Sylvie; Bashiardes, Stavros; Christodoulou, Christina

    2014-01-01

    Background Erythema infectiosum is the most common clinical manifestation of Parvovirus B19 infection although it has also been associated with rheumatologic diseases and various types of systemic vasculitides. Acute hepatitis and benign myositis however are rarely reported in association with Parvovirus B19 infection. Case presentation Here we report a 14-year old male, who developed acute hepatitis and benign myositis associated with erythema infectiosum following Parvovirus B19 infection. ...

  7. Acute myositis associated with concurrent infection of rotavirus and norovirus in a 2-year-old girl

    Kei Yamamoto

    2015-09-01

    Full Text Available Rotavirus and norovirus are common pathogens associated with gastroenteritis in children. Although rotavirus occasionally induces central nervous system disease, only 3 cases with rotavirus-induced acute myositis have been reported in the English literature. We recently treated a female patient with acute myositis associated with gastroenteritis induced by concurrent infection with rotavirus and norovirus. Having suffered from gastroenteritis for 3 days, she suddenly developed myositis affecting her lower extremities with concomitant creatine kinase elevation. Herein, we present our patient and review the previous cases including those reported in the Japanese literature.

  8. Childhood Acute Lymphoblastic Leukemia

    Pui, Ching-Hon; Yang, Jun J; Hunger, Stephen P;

    2015-01-01

    PURPOSE: To review the impact of collaborative studies on advances in the biology and treatment of acute lymphoblastic leukemia (ALL) in children and adolescents. METHODS: A review of English literature on childhood ALL focusing on collaborative studies was performed. The resulting article was...

  9. Acute hemiplegia in childhood

    Okuno, Takehiko; Takao, Tatsuo; Itoh, Masatoshi; Konishi, Yukuo; Nakano, Shozo (Kyoto Univ. (Japan). Faculty of Medicine)

    1983-04-01

    The results of CT in 100 patients with acute hemiplegia in childhood are reported here. The etiology was various: 2 patients had infratentorial brain tumors, 56 had cerebral vascular diseases, 3 had head injuries, 16 had intracranial infectious diseases, one had postinfectious encephalomyelitis, one had multiple sclerosis, 2 had epilepsy, and the diagnosis of 19 were unknown. Eleven patients had a normal CT and a good prognosis. As for the type of onset, there were patients of type 1 with fever and 42 with convulsions and unconsciousness; those of type 2 with convulsions and unconsciousness were 12, and those of type 3 without fever and convulsions were 46. This classification is assumed to be useful, as the type of onset is characteristic of the etiology. Six patients were diagnosed correctly by repeated examinations, although the first CT did not reveal any remarkable findings. Capsular infarction, occlusion of the posterior cerebral artery in acute hemiplegia in childhood, abnormal findings of the internal capsule, thalamus, and midbrain in a patient with postinfectious encephalomyelitis, and a diffuse low density in the CT of the unilateral hemisphere in the patients with acute encephalopathy and acute hemiplegia of an obscure origin have been found after the introduction of computerized tomography.

  10. Clinical and laboratory description of a series of cases of acute viral myositis

    Silvana Paula Cardin

    2015-10-01

    Full Text Available ABSTRACT OBJECTIVE: Describe the clinical and laboratory profile, follow-up, and outcome of a series of cases of acute viral myositis. METHOD: A retrospective analysis of suspected cases under observation in the emergency department was performed, including outpatient follow-up with the recording of respiratory infection and musculoskeletal symptoms, measurement of muscle enzymes, creatine phosphokinase (CPK, lactate dehydrogenase (LDH, transaminases (AST and ALT, blood count, C-reactive protein, and erythrocyte sedimentation rate in the acute phase and during follow-up until normalization. RESULTS: Between 2000 and 2009, 42 suspected cases were identified and 35 (27 boys were included. The median age was 7 years and the diagnosis was reported in 89% in the first emergency visit. The observed respiratory symptoms were cough (31%, rhinorrhea (23%, and fever (63%, with a mean duration of 4.3 days. Musculoskeletal symptoms were localized pain in the calves (80%, limited ambulation (57%, gait abnormality (40%, and muscle weakness in the lower limbs (71%, with a mean duration of 3.6 days. There was significant increase in CPK enzymes (5507 ± 9180 U/L, LDH (827 ± 598 U/L, and AST (199 ± 245 U/L, with a tendency to leukopenia (4590 ± 1420 leukocytes/mm3. The complete recovery of laboratory parameters was observed in 30 days (median, and laboratory and clinical recurrence was documented in one case after 10 months. CONCLUSION: Typical symptoms with increased muscle enzymes after diagnosis of influenza and self-limited course of the disease were the clues to the diagnosis. The increase in muscle enzymes indicate transient myotropic activity related to seasonal influenza, which should be considered, regardless of the viral identification, possibly associated with influenza virus or other respiratory viruses.

  11. Coexistence of myositis, transverse myelitis, and Guillain Barré syndrome following Mycoplasma pneumoniae infection in an adolescent

    Yasemin Topcu; Erhan Bayram; Pakize Karaoglu; Uluc Yis; Handan Guleryuz; Semra Hiz Kurul

    2013-01-01

    Guillain-Barré syndrome (GBS) and transverse myelitis may occur coexistently in the pediatric population. This may be explained by a shared epitope between peripheral and central nervous system myelin. Coexistent transverse myelitis, myositis, and acute motor neuropathy in childhood have not been previously described. We describe a 14-year-old female patient with transverse myelitis, myositis, and GBS following Mycoplasma pneumoniae infection. She presented with weakness and walking disabilit...

  12. Acute leukemia in early childhood

    M. Emerenciano

    2007-06-01

    Full Text Available Acute leukemia in early childhood is biologically and clinically distinct. The particular characteristics of this malignancy diagnosed during the first months of life have provided remarkable insights into the etiology of the disease. The pro-B, CD10 negative immunophenotype is typically found in infant acute leukemia, and the most common genetic alterations are the rearrangements of the MLL gene. In addition, the TEL/AML1 fusion gene is most frequently found in children older than 24 months. A molecular study on a Brazilian cohort (age range 0-23 months has detected TEL/AML1+ve (N = 9, E2A/PBX1+ve (N = 4, PML/RARA+ve (N = 4, and AML1/ETO+ve (N = 2 cases. Undoubtedly, the great majority of genetic events occurring in these patients arise prenatally. The environmental exposure to damaging agents that give rise to genetic changes prenatally may be accurately determined in infants since the window of exposure is limited and known. Several studies have shown maternal exposures that may give rise to leukemogenic changes. The Brazilian Collaborative Study Group of Infant Acute Leukemia has found that mothers exposed to dipyrone, pesticides and hormones had an increased chance to give birth to babies with infant acute leukemia [OR = 1.48 (95%CI = 1.05-2.07, OR = 2.27 (95%CI = 1.56-3.31 and OR = 9.08 (95%CI = 2.95-27.96], respectively. This review aims to summarize recent clues that have facilitated the elucidation of the biology of early childhood leukemias, with emphasis on infant acute leukemia in the Brazilian population.

  13. The acute abdomen in childhood

    The acute abdomen in childhood demands early diagnosis for the sake of appropriate and early therapy. A high number of differential diagnosis in the assessment of acute abdominal pain and problems, as well as different causes of diseases in different age of the children, make a partition with respect to the age of the children considerable. A partition in diseases, which are leading to acute abdominal pain in neonates, in toddlers and small infants and in schoolchildren makes sense. In older schoolchildren and adolescents also diseases of the grown up generation are possible as a cause of acute abdominal pain. The lack of compliance (cooperation and communication) in small children obstructs clinical and radiological assessment and elucidates the necessity of knowledge of causes of acute abdominal pain as much as possible. A lot of patience and experience are mandatory in the evaluation of small children and therefore make a dedicated, trained and experienced stuff necessary. A dedicated paediatric radiologist will find a safe and proper technique, and in cooperation with the referring clinician the necessary decision for therapy will be made. (orig.)

  14. Acute childhood leukemia: Nursing care

    Modern therapy for childhood acute leukemia has provided a dramatically improved prognosis over that of just 30 years ago. In the early 1960's survival rates for acute lymphocytic leukemia (ALL) and acute myelogenous leukemia (AML) were 4% and 3%, respectively. By the 1980's survival rates had risen to 72% for all and 25% to 40% for AML. Today, a diagnosis of all carries an 80% survival rate and as high as a 90% survival rate for some low-risk subtypes. Such high cure rates depend on intense and complex, multimodal therapeutic protocols. Therefore, nursing care of the child with acute leukemia must meet the demands of complicated medical therapies and balance those with the needs of a sick child and their concerned family. An understanding of disease process and principles of medical management guide appropriate and effective nursing interventions. Leukemia is a malignant disorder of the blood and blood- forming organs (bone marrow, lymph nodes and spleen). Most believe that acute leukemia results from a malignant transformation of a single early haematopoietic stem cell that is capable of indefinite self-renewal. These immature cells of blasts do not respond to normal physiologic stimuli for differentiation and gradually become the predominant cell in the bone marrow

  15. Focal myositis

    Focal myositis is a rare, benign focal inflammation of muscle. The lesion often presents as a mass that may be mistaken for a soft tissue sarcoma. This report describes the MRI and histopathological features of a case and illustrates how the diagnosis may be suspected on the basis of the MR findings. Copyright (2001) Blackwell Science Pty Ltd

  16. Inclusion Body Myositis

    ... Awards Enhancing Diversity Find People About NINDS NINDS Inclusion Body Myositis Information Page Table of Contents (click ... and Information Additional resources from MedlinePlus What is Inclusion Body Myositis? Inclusion body myositis (IBM) is one ...

  17. MANAGEMENT OF ACUTE RENAL FAILURE WITH DELAYED HYPERCALCEMIA SECONDARY TO SARCOCYSTIS NEURONA-INDUCED MYOSITIS AND RHABDOMYOLYSIS IN A CALIFORNIA SEA LION (ZALOPHUS CALIFORNIANUS).

    Alexander, Amy B; Hanley, Christopher S; Duncan, Mary C; Ulmer, Kyle; Padilla, Luis R

    2015-09-01

    A 3-yr-old captive-born California sea lion (Zalophus californianus) developed Sarcocystis neurona-induced myositis and rhabdomyolysis that led to acute renal failure. The sea lion was successfully managed with fluid therapy, antiprotozoals, antibiotics, anti-inflammatories, antiemetics, gastroprotectants, and diuretics, but developed severe delayed hypercalcemia, a syndrome identified in humans after traumatic or exertion-induced rhabdomyolysis. Treatment with calcitonin was added to the management, and the individual recovered fully. The case emphasizes that animals with rhabdomyolysis-induced renal failure risk developing delayed hypercalcemia, which may be life threatening, and calcium levels should be closely monitored past the resolution of renal failure. PMID:26352981

  18. [Progressive myositis ossificans. Stone man].

    Fonseca, J E; Evangelista, T; Barroso, L; Reis, J; Gomes, A R

    2001-01-01

    Myositis ossificans progressiva (MOP) is a rare hereditary connective tissue disease, genetically inherited as an autossomal dominant trait with complete penetrance but variable expression. The onset usually takes place during childhood and progressive involvement of the spinal cord and proximal extremities leads to immobilization and articular dysfunction. We present a case of a 29-year-old woman with the typical features of MOP and review the pathogenesis, clinical manifestations and treatment options of this rare disease. PMID:11762186

  19. Pipazethate--acute childhood poisoning.

    da Silva, O A; Lopez, M

    1977-01-01

    A previously healthy child who who had accidentally ingested an unknown quantity of 20-mg tablets of pipazethate developed severe acute poisoning with neurologic, metabolic, and cardiovascular disturbances. She recovered with symptomatic and supportive therapy. PMID:589958

  20. Acute acquired comitant esotropia of childhood

    Hesgaard, Helena; Vinding, Troels

    2015-01-01

    : In all, 48 cases were recorded. The mean age at onset was 4.7 years, being significantly higher among children with intracranial disease. Seven cause-specific types of AACE in childhood were identified: The acute accommodative (n = 15, 31%), decompensated monofixation syndrome or esophoria (n = 13...... AACE and by review of literature, we identified seven cause-specific types of AACE. Intracranial disease was present in 6%, and four risk factors were identified to guide clinicians when to perform brain imaging. Findings suggest AACE of childhood to be differentiated from AACE of adulthood....

  1. Coexistence of myositis, transverse myelitis, and Guillain Barré syndrome following Mycoplasma pneumoniae infection in an adolescent

    Yasemin Topcu

    2013-01-01

    Full Text Available Guillain-Barré syndrome (GBS and transverse myelitis may occur coexistently in the pediatric population. This may be explained by a shared epitope between peripheral and central nervous system myelin. Coexistent transverse myelitis, myositis, and acute motor neuropathy in childhood have not been previously described. We describe a 14-year-old female patient with transverse myelitis, myositis, and GBS following Mycoplasma pneumoniae infection. She presented with weakness and walking disability. Weakness progressed to involve all extremities and ultimately, she was unable to stand and sit. Based on the clinical findings, a presumptive diagnosis of myositis was made at an outside institution because of high serum creatine kinase level. The patient was referred to our institution for further investigation. Magnetic resonance imaging of spine revealed enhancing hyperintense lesions in the anterior cervicothoracic spinal cord. The electromyography revealed acute motor polyneuropathy. Serum M. pneumoniae IgM and IgG were positive indicating an acute infection. Repeated M. pneumoniae serology showed a significant increase in Mycoplasma IgG titer. The patient was given intravenous immunoglobulin for 2 days and clarithromycin for 2 weeks. She was able to walk without support after 2 weeks of hospitalization. This paper emphasizes the rarity of concomitant myositis, transverse myelitis, and GBS in children.

  2. Use of clofarabine for acute childhood leukemia

    Masetti, Riccardo

    2010-01-01

    A Pession, R Masetti, K Kleinschmidt, A MartoniPediatric Oncology and Hematology “Lalla Seràgnoli”, University of Bologna, ItalyAbstract: A second-generation of purine nucleoside analogs, starting with clofarabine, has been developed in the course of the search for new therapeutic agents for acute childhood leukemia, especially for refractory or relapsed disease. Clofarabine is a hybrid of fludarabine and cladribine, and has shown to have antileukemic activity i...

  3. Acute Retinal Necrosis in Childhood

    Yoav Y. Pikkel

    2014-05-01

    Full Text Available Background: Acute retinal necrosis (ARN is a viral syndrome consisting of uveitis/vitritis, occlusive vasculitis and peripheral necrosis. Few incidents are reported in children. The etiology is reactivated herpes simplex virus (HSV or varicella-zoster virus (VZV. Treatment with acyclovir is often used. The administration of oral glucocorticosteroids is of unproven benefit. Prognosis is variable but poor. Methods: Three weeks after contracting mild chickenpox, a healthy 4-year-old girl developed blurred vision in her right eye. Severely reduced visual acuity was noted, together with anterior uveitis, ‘mutton-fat' precipitates and vitral flare. Retinal vasculitis with necrosis was present. Serology for toxoplasma, cytomegalovirus and HIV was negative, while HSV and VZV IgG antibodies were positive. She was treated with 30 mg/kg of intravenous methylprednisolone (3 days, 30 mg of oral prednisone (3 days, and tapering for 8 weeks. Intravenous acyclovir was given for 10 days, followed by oral acyclovir for 4 months. Aspirin (100 mg/day was given for 4 months. Results: At 12 months, the girl felt good. Her right eye acuity was 6/9, with an intraocular pressure of 17 mm Hg. The peripheral retina showed scarring but no detachment. Conclusions: This is the first report of a once-daily high-dose methylprednisolone pulse therapy in one of the youngest known ARN cases. Pulsed steroid therapy was based on its known effectiveness in vasculitis, which is the main pathophysiology in ARN. There was no evidence of steroid-related viral over-replication. Our case achieved an excellent clinical and ophthalmic recovery in spite of the poor prognosis. The positive result of this case report provides a basis for further evaluation of high-dose steroid pulse therapy in ARN.

  4. An Unusual Case of Myelitis with Myositis

    Makhija, Prashant; Puri, Vinod; Khwaja, Geeta Anjum; Duggal, Ashish

    2016-01-01

    The Chikungunya virus belonging to genus alphavirus and family togaviridae causes an arthropod-borne self-limiting febrile illness in humans. Neurological complications are rare with myelitis being even a rarer presentation. We report here a patient with Chikungunya fever who developed acute transverse myelitis with myositis during the convalescent period.

  5. Use of clofarabine for acute childhood leukemia

    A Pession

    2010-06-01

    Full Text Available A Pession, R Masetti, K Kleinschmidt, A MartoniPediatric Oncology and Hematology “Lalla Seràgnoli”, University of Bologna, ItalyAbstract: A second-generation of purine nucleoside analogs, starting with clofarabine, has been developed in the course of the search for new therapeutic agents for acute childhood leukemia, especially for refractory or relapsed disease. Clofarabine is a hybrid of fludarabine and cladribine, and has shown to have antileukemic activity in acute lymphoblastic leukemia as well as in myeloid disorders. As the only new antileukemic chemotherapeutic agent to enter clinical use in the last 10 years, clofarabine was approved as an orphan drug with the primary indication of use in pediatric patients. Toxicity has been tolerable in a heavily pretreated patient population, and clofarabine has been demonstrated to be safe, both as a single agent and in combination therapies. Liver dysfunction has been the most frequently observed adverse event, but this is generally reversible. Numerous Phase I and II trials have recently been conducted, and are still ongoing in an effort to find the optimal role for clofarabine in various treatment strategies. Concomitant use of clofarabine, cytarabine, and etoposide was confirmed to be safe and effective in two independent trials. Based on the promising results when used as a salvage regimen, clofarabine is now being investigated for its potential to become part of frontline protocols.Keywords: clofarabine, pediatric acute lymphoblastic leukemia, pediatric acute myeloid leukemia

  6. Relapsed childhood acute lymphoblastic leukemia in the Nordic countries

    Oskarsson, Trausti; Söderhäll, Stefan; Arvidson, Johan;

    2016-01-01

    Relapse is the main reason for treatment failure in childhood acute lymphoblastic leukemia. Despite improvements in the up-front therapy, survival after relapse is still relatively poor, especially for high-risk relapses. The aims of this study were to assess outcomes following acute lymphoblastic...... leukemia relapse after common initial Nordic Society of Paediatric Haematology and Oncology protocol treatment; to validate currently used risk stratifications, and identify additional prognostic factors for overall survival. Altogether, 516 of 2735 patients (18.9%) relapsed between 1992 and 2011 and were...... development of novel approaches is urgently needed to increase survival in relapsed childhood acute lymphoblastic leukemia....

  7. Acute Necrotizing Encephalopathy of Childhood; A Case Report

    Mohammad Reza SALEHIOMRAN

    2013-06-01

    Full Text Available How to Cite this Article: Salehi Omran MR, Nooreddini H, Baghdadi F. Acute Necrotizing Encephalopathy of Childhood; A Case Report. Iran J Child Neurol. 2013 Spring;7(2:51-54. AbstractAcute Necrotizing Encephalopathy of Childhood (ANEC is an atypical disease followed by respiratory or gastrointestinal infection, high fever, which is accompanied with rapid alteration of consciousness and seizures. This disease is seen nearly exclusively in East Asian infants and children who had previously a good health. Serial MRI examinations demonstrated symmetric lesions involving the thalami, brainstem, cerebellum, and white matter. This disease has a poor prognosis, often culminating in profound morbidity and mortality. A 22-month infant with ANEC hospitalized in Amirkola Children Hospital has been evaluated. References1. Mizuguchi M. Acute necrotizing encephalopathy of childhood: a novel form of acute encephalopathy prevalent in Japan and Taiwan. Brain Dev. 1997 Mar;19(2:81-92. Review.2. Wang HS, Huang SC. Acute necrotizing encephalopathy of childhood. Chang Gung Med J 2001 Jan;24(1:1-10.3. Campistol J, Gassió R, Pineda M, Fernandez-Alvarez E. Acute necrotizing encephalopathy of childhood (infantile bilateral  thalamic necrosis: two non-Japanese cases. Dev Med Child Neurol 1998 Nov;40(11:771-4.4. Ito Y, Ichiyama T, Kimura H, Shibata M, Ishiwada N, Kuroki H, Furukawa S, Morishima T. Detection of influenza virus RNA by reverse transcription-PCR and proinflammatory cytokines in influenza-virus-associated encephalopathy. J Med Virol 1999 Aug;58(4:420-5.5. Sugaya N. Influenza-associated encephalopathy in Japan. Semin Pediatr Infect Dis 2002 Apr;13(2:79-84. Review.6. Skelton BW, Hollingshead MC, Sledd AT, Phillips CD, Castillo M. Acute necrotizing encephalopathy of childhood: typical findings in an atypical disease. Pediatr Radiol 2008 Jul; 38(7:810-3.7. Wong AM, Simon EM, Zimmerman RA, Wang HS, Toh CH, Ng SH. Acute necrotizing encephalopathy of childhood

  8. Variable detection of myeloid antigens in childhood acute lymphoblastic leukaemia.

    Howard, M R; Thomas, L; Reid, M. M.

    1994-01-01

    AIMS--To determine whether the use of different sources of anti-CD13 and anti-CD33 monoclonal antibodies leads to discrepant results in childhood acute lymphoblastic leukaemia (ALL), which might contribute to the wide variation in the reported incidence of myeloid antigen expressing ALL in childhood. METHODS--Stored leukaemic cells from 10 children with previously defined myeloid positive ALL were examined. A range of commercially available anti-CD13 and anti-CD33 monoclonal antibodies, direc...

  9. Etiology of common childhood acute lymphoblastic leukemia: the adrenal hypothesis

    Schmiegelow, K.; Vestergaard, T.; Nielsen, S.M.;

    2008-01-01

    The pattern of infections in the first years of life modulates our immune system, and a low incidence of infections has been linked to an increased risk of common childhood acute lymphoblastic leukemia (ALL). We here present a new interpretation of these observations--the adrenal hypothesis...

  10. Acute necrotizing encephalopathy of childhood: report of a Spanish case.

    San Millan, Beatriz; Teijeira, Susana; Penin, Carmen; Garcia, Jose L; Navarro, Carmen

    2007-12-01

    Acute necrotizing encephalopathy of childhood is a rare disease with a broad clinical, radiologic, and biochemical spectrum. In the few postmortem studies published to date, the neuropathologic findings involved symmetric, necrotic brain lesions as the hallmark. Here we report on the clinical and neuropathologic findings of a Spanish child with the most severe form of the disease. PMID:18021928

  11. Second Malignant Neoplasms After Treatment of Childhood Acute Lymphoblastic Leukemia

    Schmiegelow, K.; Levinsen, Mette Frandsen; Attarbaschi, Andishe;

    2013-01-01

    PURPOSE: Second malignant neoplasms (SMNs) after diagnosis of childhood acute lymphoblastic leukemia (ALL) are rare events. PATIENTS AND METHODS: We analyzed data on risk factors and outcomes of 642 children with SMNs occurring after treatment for ALL from 18 collaborative study groups between 1980...

  12. Management of acute moderate and severe childhood malnutrition

    Acute childhood malnutrition affects about a tenth of the world's children under 5 years of age, particularly those living in circumstances of extreme poverty in the developing world. Malnutrition is typically the result of an inadequate diet and is one of the most common diagnoses in children in he...

  13. Myositis ossificans: the mimicker

    Govindarajan, Arunkumar; Sarawagi, Radha; Prakash, Manikka Lakshmanan

    2013-01-01

    A 14-year-old boy presented with upper backache and a painful swelling in the right paraspinal region for 7 days. He had no history of trauma. MRI showed a non-specific ill-defined heterogeneous lesion, which showed intense postcontrast enhancement. Ultrasonogram showed a peripheral sheet of calcification around the lesion. A CT scan showed a faint rim of calcification, which increased in thickness over weeks, confirming the diagnosis as myositis ossificans. We present our approach to the cas...

  14. [Inclusion-body myositis].

    Benveniste, O

    2014-07-01

    Sporadic inclusion-body myositis (sIBM) presents in average at the sixth decade of life and affects three men for one woman. It is a non-lethal, slowly progressive but disabling disease. Except the striated muscles, no other organs (such as the interstitial lung) are involved. The phenotype of this myopathy is particular since it involves the axial muscles (camptocormia, swallowing dysfunction) and limb girdle (notably the quadriceps) but also the distal muscles (in particular the fingers' and wrists' flexors) in a bilateral but non-symmetrical manner. The clinical presentation is then very suggestive of the diagnosis, which remains to be proven by a muscle biopsy. Histological features defining the diagnosis associate endomysial inflammatory infiltrates with frequent invaded fibres (the myositis) and amyloid deposits generally accompanying rimmed vacuoles (the inclusions). There is still today a debate to know if this disease is at its beginning a degenerative or an auto-immune condition. Nonetheless, usual immunosuppressive drugs (corticosteroids, azathioprine, methotrexate) or polyvalent immunoglobulines remain ineffective and even may worsen the handicap. Some controlled randomized trials will soon be launched for this condition, but for now, the best therapeutic approach to slow down the rapidity of progression of the disease is to maintain muscle exercise with the help of the physiotherapists. PMID:24128435

  15. Minimal Residual Disease Assessment in Childhood Acute Lymphoblastic Leukemia

    Thörn, Ingrid

    2009-01-01

    Traditionally, response to treatment in hematological malignancies is evaluated by light microscopy of bone marrow (BM) smears, but due to more effective therapies more sensitive methods are needed. Today, detection of minimal residual disease (MRD) using immunological and molecular techniques can be 100 times more sensitive than morphology. The main aim of this thesis was to compare and evaluate three currently available MRD methods in childhood acute lymphoblastic leukemia (ALL): (i) real-t...

  16. Childhood acute erythroleukemia diagnosis by flow cytometry

    Anjali Sharma

    2011-01-01

    Full Text Available Acute erythroid leukemia in children is very rare. Here is a case of erythroleukemia in a child of age 1.5 years, which was diagnosed on peripheral smear, bone marrow examination, cytochemistry but was confirmed on immunophenotyping. CD45 versus side scatter demonstrated blast population (29% expressing CD45 of variable intensity (dim to negative. The myeloid nature of blast population showed bright expression of cytoplasmic myeloperoxidase (MPO, heterogenous positivity of CD117 and dim expression of CD13, CD33. These blasts also showed bright positivity for CD71 which showed erythroid nature of blasts. Flow cytometry can be comprehensive enough to completely subtype cases of leukemias/myelodysplastic syndromes, polycythemia rubra vera, non-neoplastic conditions like reactive erythroid hyperplasia following immunosuppressive therapy or viral infections or nutritional deficiencies, unlyzed RBCs or thrombocytosis which may mimic acute erythroid leukemia on flow cytometry.

  17. INCLUSION BODY MYOSITIS

    Luh Yeni Laksmini

    2014-09-01

    Full Text Available Inclusion body myositis (IBM merupakan penyakit inflamasi pada otot yang bersifat progresif dengan penyebab yang tidak diketahui dan tidak menunjukkan respon yang baik terhadap berbagai terapi. Gambaran histopatologi IBM ditandai dengan infiltrat sel-sel limfosit diantara ruangan endomisial, di dalam otot dan di sekitar otot dengan fokus-fokus inklusi di dalam miosit (rimmed vacuole serta beberapa serat otot terlihat atrofi dan nekrosis. Dilaporkan wanita, usia 46 tahun dengan IBM. Keluhan utama pasien berupa kelemahan pada kedua tangan, kaki kanan terasa berat jika diangkat sehingga susah berjalan. Pemeriksaan saraf sensorik ekstremitas dekstra dan sinistra dalam batas normal. Pemeriksaan enzim cretinine kinase meningkat secara dramatik. Pemeriksaan histopatologi dari biospi otot gastrocnemius menunjukkan gambaran yang sesuai untuk IBM dan telah dilakukan penanganan dengan pemberian oral methilprednisolon 3x32 mg dan mecobalmin 1x500ìg intravena, namun tidak menunjukkan respon yang baik terhadap terapi dan akhirnya pasien meninggal. [MEDICINA 2013;44:118-123].

  18. Is ketamine a lifesaving agent in childhood acute severe asthma?

    Hendaus, Mohamed A; Jomha, Fatima A; Alhammadi, Ahmed H

    2016-01-01

    Children with acute severe asthma exacerbation are at risk of developing respiratory failure. Moreover, conventional aggressive management might be futile in acute severe asthma requiring intubation and invasive ventilation. The aim of this review is to detail evidence on the use of ketamine in childhood asthma exacerbations. A search of the MEDLINE, EMBASE, and Cochrane databases was performed, using different combinations of the following terms: ketamine, asthma, use, exacerbation, and childhood. In addition, we searched the references of the identified articles for additional articles. We then reviewed titles and included studies that were relevant to the topic of interest. Finally, the search was limited to studies published in English and Spanish from 1918 to June 2015. Due to the scarcity in the literature, we included all published articles. The literature reports conflicting results of ketamine use for acute severe asthma in children. Taking into consideration the relatively good safety profile of the drug, ketamine might be a reasonable option in the management of acute severe asthma in children who fail to respond to standard therapy. Furthermore, pediatricians and pediatric emergency clinicians administering ketamine should be knowledgeable about the unique actions of this drug and its potential side effects. PMID:26955277

  19. What Role for Angiogenesis in Childhood Acute Lymphoblastic Leukaemia?

    P. Schneider

    2011-01-01

    Full Text Available The role of angiogenesis in acute leukaemia has been discussed since the cloning of the gene of vascular endothelial growth factor (VEGF from the acute myelogenous leukemia cell line (HL60 and, thereafter, when the first studies reported increased bone marrow vascularity and elevation of angiogenic cytokines in acute lymphoblastic leukaemia (ALL. VEGF and basic fibroblast growth factor (bFGF are the major proangiogenic cytokines that have been studied, and evaluation of their prognostic impact in childhood ALL has been reported in several studies, though with controversial results. The antiangiogenic response, contributing to the angiogenic balance, has scarcely been reported. The origin of the factors, their prognostic value, and their relevance as good markers of what really happens in the bone marrow are discussed in this paper. The place of antiangiogenic drugs in ALL has to be defined in the global treatment strategy.

  20. Acute leukemia of childhood: A single institution's experience

    Slavković Bojana

    2005-01-01

    Full Text Available The aim of this study was to investigate distribution of immunophenotypic and cytogenetic features of childhood acute leukemia (AL in the cohort of 239 newly diagnosed patients registered at the leading pediatric oncohematology center in the country during a six-year period (1996-2002. With approximately 60-70% of all childhood AL cases in Serbia and Montenegro being diagnosed and treated in this institution the used data represent a valid research sample to draw conclusions for entire country. On the basis of five phenotypic markers, the distribution of immunological subtypes was as follows: 169 (70.7% expressed B-cell marker CD19 (137 were CD10 positive and 32 CD10 negative, 37 (15.5% belonged to T-lineage acute lymphoblastic leukemia (T-ALL (cyCD3 positive, and 33 (13.8% were acute myeloblastic leukemia (AML (CD13 positive and/or CD33 positive in the absence of lymphoid-associated antigens. The ratio of males and females was 1.5:1. Most of the cases were between the ages of 2 and 4, and were predominantly B-lineage acute lymphoblastic leukemia (B-ALL cases. Another peak of age distribution was observed at the age of 7. The frequency of T-ALL (18% of ALL was similar to that reported for Mediterranean countries: France (19.4%, Greece (28.1%, Southern Italy (28.3%, and Bulgaria (28.0%. Cytogenetic analyses were performed in 193 patients: 164 ALL and 29 AML. Normal karyotype was found in 57% of ALL and in 55% of AML patients, while cytogenetic abnormalities including structural, numerical, and complex chromosomal rearrangements were found in 43% of ALL and in 45% of AML patients. Our results represent a contribution to epidemiological aspects of childhood leukemia studies.

  1. Aetiology of childhood acute leukaemias: Current status of knowledge

    Acute leukaemia is a consequence of malignant transformation of a haematopoietic progenitor cell. Molecular studies have revealed a prenatal origin of many childhood leukaemias. According to current models, a pre-leukaemic stem cell clone is generated by a first mutation in utero which, in a minority of children, progresses to leukaemia after receiving further postnatal genetic hits. The nature of pre- and postnatal events involved in leukemogenesis in children is not well understood. Although genetic predisposition and specific environmental exposures may account for individual cases, the bulk of childhood leukaemia cannot be explained by any of these factors. The higher incidence of the most common leukaemia subtype in affluent societies, as well as the age peak between 2-5 y, suggest a contributory role of socioeconomic factors. An abnormal immune response during delayed exposure to common infections provides a plausible mechanism for malignant progression of pre-leukaemic clones in a subgroup of children. As highlighted in this review, a common cause for all types and subtypes of childhood leukaemia is highly unlikely. Deeper insights into the pathogenesis of childhood leukaemia will rely on large-scale and combined epidemiological and bio-molecular studies. (authors)

  2. Myositis specific autoantibodies : specificity and clinical applications

    Hengstman, G.J.D.

    2005-01-01

    The sera of about half of the patients with myositis contain autoantibodies that are specific for this group of diseases compared to other inflammatory connective tissue disorders. In a recent study we showed that these myositis specific autoantibodies (MSAs) are also specific for myositis as compar

  3. Nanoparticle targeted therapy against childhood acute lymphoblastic leukemia

    Satake, Noriko; Lee, Joyce; Xiao, Kai; Luo, Juntao; Sarangi, Susmita; Chang, Astra; McLaughlin, Bridget; Zhou, Ping; Kenney, Elaina; Kraynov, Liliya; Arnott, Sarah; McGee, Jeannine; Nolta, Jan; Lam, Kit

    2011-06-01

    The goal of our project is to develop a unique ligand-conjugated nanoparticle (NP) therapy against childhood acute lymphoblastic leukemia (ALL). LLP2A, discovered by Dr. Kit Lam, is a high-affinity and high-specificity peptidomimetic ligand against an activated α4β1 integrin. Our study using 11 fresh primary ALL samples (10 precursor B ALL and 1 T ALL) showed that childhood ALL cells expressed activated α4β1 integrin and bound to LLP2A. Normal hematopoietic cells such as activated lymphocytes and monocytes expressed activated α4β1 integrin; however, normal hematopoietic stem cells showed low expression of α4β1 integrin. Therefore, we believe that LLP2A can be used as a targeted therapy for childhood ALL. The Lam lab has developed novel telodendrimer-based nanoparticles (NPs) which can carry drugs efficiently. We have also developed a human leukemia mouse model using immunodeficient NOD/SCID/IL2Rγ null mice engrafted with primary childhood ALL cells from our patients. LLP2A-conjugated NPs will be evaluated both in vitro and in vivo using primary leukemia cells and this mouse model. NPs will be loaded first with DiD near infra-red dye, and then with the chemotherapeutic agents daunorubicin or vincristine. Both drugs are mainstays of current chemotherapy for childhood ALL. Targeting properties of LLP2A-conjugated NPs will be evaluated by fluorescent microscopy, flow cytometry, MTS assay, and mouse survival after treatment. We expect that LLP2A-conjugated NPs will be preferentially delivered and endocytosed to leukemia cells as an effective targeted therapy.

  4. Adductor muscle pyo-myositis simulating appendicitis: CT and MR imaging findings; Pyomyosite des mulcles adducteurs mimant une appendicite aigue: aspects tomodensitometriques et IRM

    Coumbaras, M.; Le Hir, P.; Jomaah, N.; Arrive, L.; Tubiana, J.M. [Hopital Saint Antoine, 75 - Paris (France)

    2001-04-01

    Pyo-myositis is a primary bacterial infection of skeletal muscle. This infection tends to occur in the large muscles of the lower extremity. Pyo-myositis of the proximal muscles of the thigh can simulate acute abdominal disease. Early diagnosis improves the outcome. Delayed diagnosis may lead to septicemia and shock. We report the CT and MRI findings in a patient with pyo-myositis of the proximal muscles of the thigh. (authors)

  5. Is ketamine a lifesaving agent in childhood acute severe asthma?

    Hendaus MA

    2016-02-01

    Full Text Available Mohamed A Hendaus,1,2 Fatima A Jomha,3 Ahmed H Alhammadi1,2 1Department of Pediatrics, Section of Academic General Pediatrics, Hamad Medical Corporation, Doha, 2Department of Clinical Pediatrics, Weill Cornell Medical College in Qatar, Doha, Qatar; 3School of Pharmacy, Lebanese International University, Khiara, Lebanon Abstract: Children with acute severe asthma exacerbation are at risk of developing respiratory failure. Moreover, conventional aggressive management might be futile in acute severe asthma requiring intubation and invasive ventilation. The aim of this review is to detail evidence on the use of ketamine in childhood asthma exacerbations. A search of the MEDLINE, EMBASE, and Cochrane databases was performed, using different combinations of the following terms: ketamine, asthma, use, exacerbation, and childhood. In addition, we searched the references of the identified articles for additional articles. We then reviewed titles and included studies that were relevant to the topic of interest. Finally, the search was limited to studies published in English and Spanish from 1918 to June 2015. Due to the scarcity in the literature, we included all published articles. The literature reports conflicting results of ketamine use for acute severe asthma in children. Taking into consideration the relatively good safety profile of the drug, ketamine might be a reasonable option in the management of acute severe asthma in children who fail to respond to standard therapy. Furthermore, pediatricians and pediatric emergency clinicians administering ketamine should be knowledgeable about the unique actions of this drug and its potential side effects. Keywords: asthma, ketamine, children

  6. Myositis ossificans within the intercondylar notch treated arthroscopically

    Leung, Allen H.; Desai, Panna [Hospital for Joint Diseases/New York University, Department of Pathology, New York, NY (United States); Rybak, Leon D. [Hospital for Joint Diseases/New York University, Department of Radiology, New York, NY (United States); Rose, Donald J. [Hospital for Joint Diseases/New York University, Department of Orthopedic Surgery, New York, NY (United States)

    2010-09-15

    We present a case of intraarticular myositis ossificans in the right knee of a child. Myositis ossificans (MO), though relatively rare in childhood and even more uncommon within a joint, should be included in the differential diagnosis of an intra-articular mass when indicated by the typical clinical, radiographic, and histologic findings. An 11-year-old male presented with a history of trauma to his right knee. Four weeks after the initial injury, an MRI demonstrated evidence of an ACL rupture with a ''cystic mass'' within the intercondylar notch along the anterior surface of the torn ligament. At subsequent arthroscopy, the mass noted on MRI was removed. The histology was consistent with MO. The authors believe this to be the first case of MO in the intercondylar notch detected by MRI, treated by arthroscopy, and confirmed by histology. (orig.)

  7. Sarcocystis nesbitti causes acute, relapsing febrile myositis with a high attack rate: description of a large outbreak of muscular sarcocystosis in Pangkor Island, Malaysia, 2012.

    Claire M Italiano

    2014-05-01

    Full Text Available BACKGROUND: From the 17th to 19th January 2012, a group of 92 college students and teachers attended a retreat in a hotel located on Pangkor Island, off the west coast of Peninsular Malaysia. Following the onset of symptoms in many participants who presented to our institute, an investigation was undertaken which ultimately identified Sarcocystis nesbitti as the cause of this outbreak. METHODOLOGY/PRINCIPAL FINDINGS: All retreat participants were identified, and clinical and epidemiological information was obtained via clinical review and self-reported answers to a structured questionnaire. Laboratory, imaging and muscle biopsy results were evaluated and possible sources of exposure, in particular water supply, were investigated. At an average of 9-11 days upon return from the retreat, 89 (97% of the participants became ill. A vast majority of 94% had fever with 57% of these persons experiencing relapsing fever. Myalgia was present in 91% of patients. Facial swelling from myositis of jaw muscles occurred in 9 (10% patients. The median duration of symptoms was 17 days (IQR 7 to 30 days; range 3 to 112. Out of 4 muscle biopsies, sarcocysts were identified in 3. S. nesbitti was identified by PCR in 3 of the 4 biopsies including one biopsy without observed sarcocyst. Non-Malaysians had a median duration of symptoms longer than that of Malaysians (27.5 days vs. 14 days, p = 0.001 and were more likely to experience moderate or severe myalgia compared to mild myalgia (83.3% vs. 40.0%, p = 0.002. CONCLUSIONS/SIGNIFICANCE: The similarity of the symptoms and clustered time of onset suggests that all affected persons had muscular sarcocystosis. This is the largest human outbreak of sarcocystosis ever reported, with the specific Sarcocystis species identified. The largely non-specific clinical features of this illness suggest that S. nesbitti may be an under diagnosed infection in the tropics.

  8. Childhood acute leukemias are frequent in Mexico City: descriptive epidemiology

    Bekker-Méndez Vilma

    2011-08-01

    Full Text Available Abstract Background Worldwide, acute leukemia is the most common type of childhood cancer. It is particularly common in the Hispanic populations residing in the United States, Costa Rica, and Mexico City. The objective of this study was to determine the incidence of acute leukemia in children who were diagnosed and treated in public hospitals in Mexico City. Methods Included in this study were those children, under 15 years of age and residents of Mexico City, who were diagnosed in 2006 and 2007 with leukemia, as determined by using the International Classification of Childhood Cancer. The average annual incidence rates (AAIR, and the standardized average annual incidence rates (SAAIR per million children were calculated. We calculated crude, age- and sex-specific incidence rates and adjusted for age by the direct method with the world population as standard. We determined if there were a correlation between the incidence of acute leukemias in the various boroughs of Mexico City and either the number of agricultural hectares, the average number of persons per household, or the municipal human development index for Mexico (used as a reference of socio-economic level. Results Although a total of 610 new cases of leukemia were registered during 2006-2007, only 228 fit the criteria for inclusion in this study. The overall SAAIR was 57.6 per million children (95% CI, 46.9-68.3; acute lymphoblastic leukemia (ALL was the most frequent type of leukemia, constituting 85.1% of the cases (SAAIR: 49.5 per million, followed by acute myeloblastic leukemia at 12.3% (SAAIR: 6.9 per million, and chronic myeloid leukemia at 1.7% (SAAIR: 0.9 per million. The 1-4 years age group had the highest SAAIR for ALL (77.7 per million. For cases of ALL, 73.2% had precursor B-cell immunophenotype (SAAIR: 35.8 per million and 12.4% had T-cell immunophenotype (SAAIR 6.3 per million. The peak ages for ALL were 2-6 years and 8-10 years. More than half the children (58.8% were

  9. Side effects of treatment in childhood acute leukemia, 2

    We evaluated delayed neurotoxicities in treatment of childhood acute leukemia. Of 28 patients treated over 2 years who were examined on computed tomography of brain scans, 7 patients had abnormal findings. These abnormalities included two cases of leukoencephalopathy, three cases of intracranial calcifications, and two of ventricular dilatation. These patients were under 6 years old at the onset of disease, especially under 3 years old. Also, delayed neurotoxicities developed after relapse of leukemia, especially CNS relapse. It was considered that these were caused by cranial irradiation, intravenous methotrexate injection, intrathecal methotrexate, and sometimes high-dose Ara-C therapy, etc. Most of the cases of leukoencephalopathy were associated with treatment of intermediate-dose or high-dose methotrexate after relapse. These abnormalities must be carefully considered in the treatment of younger children with leukemia and patients with relapse. (author)

  10. Pharmacogenetics Influence Treatment Efficacy in Childhood Acute Lymphoblastic Leukemia

    Devidsen, M.L.; Dalhoff, K.; Schmiegelow, K.

    2008-01-01

    Pharmacogenetics covers the genetic variation affecting pharmacokinetics and pharmacodynamics. and their influence on drug-response phenotypes. The genetic variation includes an estimated 15 million single nucleotide polymorphisms (SNP) and is a key determinator for the interindividual differences...... treatment response. In the future, high-throughput, low-cost, genetic platforms will allow screening of hundreds or thousands of targeted SNPs to give a combined gene-dosage effect ( = individual SNP risk profile), which may allow pharmacogenetic-based individualization of treatment Udgivelsesdato: 2008/11...... in treatment resistance and toxic side effects. As most childhood acute lymphoblastic leukemia treatment protocols include up to 13 different chemotherapeutic agents, the impact of individual SNPs has been difficult to evaluate. So far Focus has mainly been on the widely used glucocorticosteroids...

  11. Eight different viral agents in childhood acute gastroenteritis.

    Bozkurt, Derya; Selimoğlu, Mukadder Ayşe; Otlu, Barış; Sandıkkaya, Ayşe

    2015-01-01

    Viral gastroenteritis is the most frequent cause of acute gastroenteritis (AGE) of childhood. The aim of this study was to determine the prevalence of viral agents including astrovirus, rotavirus, adenovirus, enterovirus, norovirus, parechovirus, Aichivirus and sapovirus in children with AGE in a pediatric Turkish population. Fecal specimens of 240 children with AGE were investigated by polymerase chain reaction, and viral agents were identified in 131 (54.6%) samples. The distribution of viral agents was as follows: 56 (42.8%) norovirus, 44 (33.6%) rotavirus, 29 (22.1%) enterovirus, 21 (16.0%) adenovirus, 21 (16.0%) parechovirus, 5 (3.8%) sapovirus and 1 (0.8%) Aichivirus. Single and multiple viral agents were detected in 38.8% and 15.8% of patients, respectively. The duration of hospitalization was longer in children with multiple viral agents than in those infected with a single viral agent (p<0.001). While the highest rate of rotavirus infection was detected in winter, the highest rate of norovirus was found in the summer. In conclusion, norovirus and rotavirus are the most frequent causes of childhood AGE in our country. PMID:26613223

  12. Update in inclusion body myositis.

    Machado, P.; Brady, S; Hanna, M. G.

    2013-01-01

    Purpose of review The purpose of this study is to review recent scientific advances relating to the natural history, cause, treatment and serum and imaging biomarkers of inclusion body myositis (IBM). Recent findings Several theories regarding the aetiopathogenesis of IBM are being explored and new therapeutic approaches are being investigated. New diagnostic criteria have been proposed, reflecting the knowledge that the diagnostic pathological findings may be absent in patients with clinical...

  13. Cardiac function in survivors of childhood acute myeloid leukemia treated with chemotherapy only

    Jarfelt, Marianne; Andersen, Niels Holmark; Glosli, Heidi;

    2015-01-01

    OBJECTIVES: We report cardiac function of patients treated for Childhood acute myeloid leukemia with chemotherapy only according to three consecutive Nordic protocols. METHODS: Ninety-eight of 138 eligible patients accepted examination with standardized echocardiography. Results were compared with...

  14. Personalization of dexamethasone therapy in childhood acute lymphoblastic leukaemia.

    Jackson, Rosanna K; Irving, Julie A E; Veal, Gareth J

    2016-04-01

    Dexamethasone is a key component in the treatment of childhood acute lymphoblastic leukaemia (ALL). Despite playing a key role in the improved survival of ALL over several decades, intensification of dexamethasone therapy has also contributed to the increased toxicity associated with treatment, which is now seen to be at unacceptable levels given the favourable disease prognosis. Therefore the focus for treatment is now shifting towards reducing toxicity whilst maintaining current survival rates. As approximately 50% of patients were successfully treated on less intensive protocols of the 1980s, it has been questioned whether therapy intensification is necessary in all patients. Furthermore, there remains a subset of children who are still not cured of their disease. New strategies are therefore needed to identify patients who could benefit from dose reduction or intensification. However, adjusting a potentially life threatening therapy is a challenging task, particularly given the heterogeneous nature of ALL. This review focuses on the potential for patient stratification based on our current knowledge of dexamethasone pharmacokinetics, pharmacogenetics and the action of dexamethasone at the cellular level. A carefully designed, combined approach is needed if we are to achieve the aim of improved personalization of dexamethasone therapy for future patients. PMID:26729065

  15. Survival in France after childhood acute leukaemia and non-Hodgkin's lymphoma (1990-2000).

    Goubin, Aurélie; Auclerc, Marie-Françoise; Auvrignon, Anne; Patte, Catherine; Bergeron, Christophe; Hémon, Denis; Clavel, Jacqueline

    2006-01-01

    This article describes the survival after childhood acute leukaemia (AL) and non-Hodgkin's lymphoma (NHL) of French population aged less than 15 years. The French National Registry of Childhood Leukaemia and Lymphoma recorded 3995 cases of acute lymphoblastic leukaemia (ALL), 812 of acute myeloid leukaemia (AML) and 1137 of NHL over the period from 1990 to 2000. Overall survival rates at 5 years were 82% (95% CI 80-83), 58% (95% CI 54-61) and 87% (95% CI 85-89) for ALL, AML and NHL, respectiv...

  16. Higher risk for acute childhood lymphoblastic leukaemia in Swedish population centres 1973-94

    Hjalmars, U; G. Gustafsson; . .

    1999-01-01

    A population-based sample of acute childhood leukaemia cases in Sweden 1973–94 was analysed by a geographical information system (GIS) for spatial leukaemia distribution in relation to population density. The annual incidence rate for acute lymphoblastic leukaemia (ALL) was 3.6, and for acute non-lymphoblastic leukaemia (ANLL) 0.7, cases per 100 000 children. Incidence rates in population centres, constituting 1.3% of Sweden's land area and approximately 80% of the population, compared with t...

  17. Rationale for an international consortium to study inherited genetic susceptibility to childhood acute lymphoblastic leukemia

    Sherborne, Amy L.; Hemminki, Kari; Kumar, Rajiv; Bartram, Claus R.; Stanulla, Martin; Schrappe, Martin; Petridou, Eleni; Semsei, Ágnes F.; Szalai, Csaba; Sinnett, Daniel; Krajinovic, Maja; Healy, Jasmine; Lanciotti, Marina; Dufour, Carlo; Indaco, Stefania; El-Ghouroury, Eman A; Sawangpanich, Ruchchadol; Hongeng, Suradej; Pakakasama, Samart; Gonzalez-Neira, Anna; Ugarte, Evelia L.; Leal, Valeria P.; Espinoza, Juan P.M.; Kamel, Azza M.; Ebid, Gamal T.A.; Radwan, Eman R.; Yalin, Serap; Yalin, Erdinc; Berkoz, Mehmet; Simpson, Jill; Roman, Eve; Lightfoot, Tracy; Hosking, Fay J.; Vijayakrishnan, Jayaram; Greaves, Mel; Houlston, Richard S.

    2011-01-01

    Acute lymphoblastic leukemia is the major pediatric cancer in developed countries. To date most association studies of acute lymphoblastic leukemia have been based on the candidate gene approach and have evaluated a restricted number of polymorphisms. Such studies have served to highlight difficulties in conducting statistically and methodologically rigorous investigations into acute lymphoblastic leukemia risk. Recent genome-wide association studies of childhood acute lymphoblastic leukemia have provided robust evidence that common variation at four genetic loci confers a modest increase in risk. The accumulated experience to date and relative lack of success of initial efforts to identify novel acute lymphoblastic leukemia predisposition loci emphasize the need for alternative study designs and methods. The International Childhood Acute Lymphoblastic Leukaemia Genetics Consortium includes 12 research groups in Europe, Asia, the Middle East and the Americas engaged in studying the genetics of acute lymphoblastic leukemia. The initial goal of this consortium is to identify and characterize low-penetrance susceptibility variants for acute lymphoblastic leukemia through association-based analyses. Efforts to develop genome-wide association studies of acute lymphoblastic leukemia, in terms of both sample size and single nucleotide polymorphism coverage, and to increase the number of single nucleotide polymorphisms taken forward to large-scale replication should lead to the identification of additional novel risk variants for acute lymphoblastic leukemia. Ethnic differences in the risk of acute lymphoblastic leukemia are well recognized and thus in assessing the interplay between inherited and non-genetic risk factors, analyses using different population cohorts with different incidence rates are likely to be highly informative. Given that the frequency of many acute lymphoblastic leukemia subgroups is small, identifying differential effects will realistically only be

  18. Myositis ossificans: radiologic evaluation of two cases with diagnostic computed tomograms

    Although most physicians associated myositis ossificans with recent, acute trauma, only 40%-60% of patients give such a history. The appearance of a soft tissue mass without a clear history of trauma may suggest a diagnosis of sarcoma, especially because results of a biopsy of the central portion of an area of myositis ossificans may yield immature, undifferentiated tissue resembling a sarcoma. Pain and rapid growth of a mass are more usual in myositis ossificans than in sarcomas, and careful inquiry may reveal stretching injury or chronic trauma associated with normal, vigorous, physical activities. Recognizing the characteristic histologic zoning phenomenon (immature tissue centrally surrounded by more mature tissue and a peripheral shell of benign bone) during the biopsy procedure permits the correct diagnosis of myositis ossificans. Plain radiographs or conventional tomograms may reflect this histologic zoning by demonstrating the typical, mature, outer shell of bone. Although additional radiographic studies are not usually necessary, they may be obtained when the mass is suspected to be a sarcoma. In two patients computed tomographic scans clearly demonstrated well-defined, peripheral shells of mature bone, diagnostic of myositis ossificans

  19. Residential Proximity to Agricultural Pesticide Applications and Childhood Acute Lymphoblastic Leukemia

    Rull, Rudolph P.; Gunier, Robert; Von Behren, Julie; Hertz, Andrew; Crouse, Vonda; Buffler, Patricia A.; Reynolds, Peggy

    2009-01-01

    Ambient exposure from residential proximity to applications of agricultural pesticides may contribute to the risk of childhood acute lymphoblastic leukemia (ALL). Using residential histories collected from the families of 213 ALL cases and 268 matched controls enrolled in the Northern California Childhood Leukemia Study, the authors assessed residential proximity within a half-mile (804.5 meters) of pesticide applications by linking address histories with reports of agricultural pesticide use...

  20. Poor adherence to dietary guidelines among adult survivors of childhood acute lymphoblastic leukemia

    Robien, Kim; Ness, Kirsten K.; Klesges, Lisa M.; Baker, K. Scott; Gurney, James G.

    2008-01-01

    Recent studies indicate that survivors of childhood acute lymphocytic leukemia (ALL) are at increased risk of obesity and cardiovascular disease, conditions that healthy dietary patterns may help ameliorate or prevent. To evaluate the usual dietary intake of adult survivors of childhood ALL, food frequency questionnaire data were collected from 72 participants, and compared with the 2007 WCRF/AICR Cancer Prevention recommendations, the DASH diet, and the 2005 USDA Food Guide. Mean daily energ...

  1. Cervical myositis ossificans traumatica: a rare location

    Baysal, T.; Sarac, K.; Kutlu, R. [Dept. of Radiology, Inonu University, Malatya (Turkey); Baysal, O.; Ersoy, Y. [Dept. of Physical Therapy and Rehabilitation, Inonu Univ., Malatya (Turkey); Elmali, N. [Dept. of Orthopedics and Traumatology, Inonu Univ., Malatya (Turkey)

    1999-05-01

    An unusual case of myositis ossificans traumatica lesion located in the paraspinal region is reported. Despite the contiguity of the lesion with the cervical vertebrae and ominous appearance of the biopsy material, the history of antecedent trauma and computed tomography findings allowed preoperative accurate diagnosis. To our knowledge, myositis ossificans traumatica located in the cervical paraspinal region is very rare. (orig.) With 4 figs., 16 refs.

  2. A Case Report on Parvovirus B19 Associated Myositis

    Oliver, Nathan D.; Auleen Millar; Adrian Pendleton

    2012-01-01

    Introduction: Whilst there are reports of viral myopathies affecting children and the immunocompromised, infective myositis is a relatively rare inflammatory myopathy in adults. The clinical spectrum can range from benign myalgias to more serious complications in certain risk groups. Case Presentation: We present two cases of myositis as a result of parvovirus B19 infection. Conclusion: Viral myositis and parvovirus B19 associated myositis should be considered in adults presenting with signif...

  3. Risk factors for treatment related mortality in childhood acute lymphoblastic leukaemia

    Lund, Bendik; Åsberg, Ann; Heyman, Mats;

    2011-01-01

    BACKGROUND: In spite of major improvements in the cure rate of childhood acute lymphoblastic leukaemia (ALL), 2-4% of patients still die from treatment related complications. PROCEDURE: We investigated the pattern of treatment related deaths (TRDs) and possible risk factors in the NOPHO ALL-92 and...

  4. Risk factors for treatment related mortality in childhood acute lymphoblastic leukaemia

    Lund, Bendik; Åsberg, Ann; Heyman, Mats;

    2011-01-01

    BACKGROUND: In spite of major improvements in the cure rate of childhood acute lymphoblastic leukaemia (ALL), 2-4% of patients still die from treatment related complications. PROCEDURE: We investigated the pattern of treatment related deaths (TRDs) and possible risk factors in the NOPHO ALL-92 and...... towards patients at risk. Pediatr Blood Cancer. © 2010 Wiley-Liss, Inc....

  5. Quantitative assessments of indoor air pollution and the risk of childhood acute leukemia in Shanghai

    We investigated the association between indoor air pollutants and childhood acute leukemia (AL). A total of 105 newly diagnosed cases and 105 1:1 gender-, age-, and hospital-matched controls were included. Measurements of indoor pollutants (including nitrogen dioxide (NO2) and 17 types of volatile organic compounds (VOCs)) were taken with diffusive samplers for 64 pairs of cases and controls. Higher concentrations of NO2 and almost half of VOCs were observed in the cases than in the controls and were associated with the increased risk of childhood AL. The use of synthetic materials for wall decoration and furniture in bedroom was related to the risk of childhood AL. Renovating the house in the last 5 years, changing furniture in the last 5 years, closing the doors and windows overnight in the winter and/or summer, paternal smoking history and outdoor pollutants affected VOC concentrations. Our results support the association between childhood AL and indoor air pollution. - Highlights: • We firstly assessed the effects of indoor air pollution on childhood AL in China. • Indoor air pollutants were assessed by questionnaire and quantitative measurements. • NO2 and 17 types of VOCs were measured in bedrooms of both cases and controls. • Higher concentrations of indoor air pollutants increased the risk of childhood AL. • Indoor behavioral factors and outdoor pollution might affect indoor air pollution. - Higher concentrations of indoor air pollutants were related to an elevated risk of childhood AL

  6. Acute necrotizing encephalopathy of childhood: typical findings in an atypical disease

    Skelton, Brandon W.; Phillips, C.D. [University of Virginia Health System, Department of Neuroradiology, Charlottesville, VA (United States); Hollingshead, Michael C.; Castillo, Mauricio [University of North Carolina School of Medicine, Neuroradiology Section, Chapel Hill, NC (United States); Sledd, Andrew T. [University of Virginia Health System, Department of Pediatrics, Charlottesville, VA (United States)

    2008-07-15

    Acute necrotizing encephalopathy of childhood (ANEC) is a disease entity seen nearly exclusively in East Asian children that is characterized by multifocal, symmetric lesions involving the thalami, brainstem, cerebellum, and white matter. We present a child who developed dramatic neurologic symptoms following a viral prodrome. Serial MRI examinations demonstrated characteristic lesions of ANEC, while laboratory analyses revealed evidence of acute infection with human herpesvirus-6 (HHV-6). We highlight the MRI findings in both the acute and convalescent phases of ANEC, discuss the implications of neuroimaging on the child's clinical course, and emphasize the integral role of the radiologist in correctly diagnosing this rare disease. (orig.)

  7. Necrotising myositis, the deadly impersonator.

    Rahman, A; Abou-Foul, A K; Yusaf, A; Holton, J; Cogswell, L

    2014-01-01

    We report two cases of patients with necrotising myositis who presented initially with limb pain and swelling on a background of respiratory complaints. Patient 1, a previously well 38-year-old female, underwent various investigations in the emergency department for excessive lower limb pain and a skin rash. Patient 2, a 61-year-old female with a background of rheumatoid arthritis and hypertension, presented to accident and emergency feeling generally unwell and was treated for presumed respiratory sepsis. Both deteriorated rapidly and were referred to the plastic surgery team with soft tissue necrosis, impending multiorgan failure and toxaemia. Large areas of necrotic muscle and skin were debrided, which grew group A streptococci, Streptococcus pyogenes. Patient 1 had a high above knee amputation of the left leg with extensive debridement of the right. Despite aggressive surgical intervention and microbiological input with intensive care support, patient 2 died. These two cases highlight the importance of early diagnosis and prompt surgical and pharmacological intervention in managing this life-threatening disease. Pain is the primary symptom with skin changes being a late and subtle sign in a septic patient. The Laboratory Risk Indicator for Necrotising Fasciitis (LRINEC) may be of use if there is concern to aid diagnosis of this life-threatening disease. PMID:25506031

  8. Necrotising Myositis, the Deadly Impersonator

    A. Rahman

    2014-01-01

    Full Text Available We report two cases of patients with necrotising myositis who presented initially with limb pain and swelling on a background of respiratory complaints. Patient 1, a previously well 38-year-old female, underwent various investigations in the emergency department for excessive lower limb pain and a skin rash. Patient 2, a 61-year-old female with a background of rheumatoid arthritis and hypertension, presented to accident and emergency feeling generally unwell and was treated for presumed respiratory sepsis. Both deteriorated rapidly and were referred to the plastic surgery team with soft tissue necrosis, impending multiorgan failure and toxaemia. Large areas of necrotic muscle and skin were debrided, which grew group A streptococci, Streptococcus pyogenes. Patient 1 had a high above knee amputation of the left leg with extensive debridement of the right. Despite aggressive surgical intervention and microbiological input with intensive care support, patient 2 died. These two cases highlight the importance of early diagnosis and prompt surgical and pharmacological intervention in managing this life-threatening disease. Pain is the primary symptom with skin changes being a late and subtle sign in a septic patient. The Laboratory Risk Indicator for Necrotising Fasciitis (LRINEC may be of use if there is concern to aid diagnosis of this life-threatening disease.

  9. Mutations in LPIN1 cause recurrent acute myoglobinuria in childhood.

    Zeharia, Avraham; Shaag, Avraham; Houtkooper, Riekelt H; Hindi, Tareq; de Lonlay, Pascale; Erez, Gilli; Hubert, Laurence; Saada, Ann; de Keyzer, Yves; Eshel, Gideon; Vaz, Frédéric M; Pines, Ophry; Elpeleg, Orly

    2008-10-01

    Recurrent episodes of life-threatening myoglobinuria in childhood are caused by inborn errors of glycogenolysis, mitochondrial fatty acid beta-oxidation, and oxidative phosphorylation. Nonetheless, approximately half of the patients do not suffer from a defect in any of these pathways. Using homozygosity mapping, we identified six deleterious mutations in the LPIN1 gene in patients who presented at 2-7 years of age with recurrent, massive rhabdomyolysis. The LPIN1 gene encodes the muscle-specific phosphatidic acid phosphatase, a key enzyme in triglyceride and membrane phospholipid biosynthesis. Of six individuals who developed statin-induced myopathy, one was a carrier for Glu769Gly, a pathogenic mutation in the LPIN1 gene. Analysis of phospholipid content disclosed accumulation of phosphatidic acid and lysophospholipids in muscle tissue of the more severe genotype. Mutations in the LPIN1 gene cause recurrent rhabdomyolysis in childhood, and a carrier state may predispose for statin-induced myopathy. PMID:18817903

  10. Mutations in LPIN1 Cause Recurrent Acute Myoglobinuria in Childhood

    Zeharia, Avraham; Shaag, Avraham; Houtkooper, Riekelt H.; Hindi, Tareq; De Lonlay, Pascale; Erez, Gilli; Hubert, Laurence; Saada, Ann; de Keyzer, Yves; Eshel, Gideon; Vaz, Frédéric M.; Pines, Ophry; Elpeleg, Orly

    2009-01-01

    Recurrent episodes of life-threatening myoglobinuria in childhood are caused by inborn errors of glycogenolysis, mitochondrial fatty acid beta-oxidation, and oxidative phosphorylation. Nonetheless, approximately half of the patients do not suffer from a defect in any of these pathways. Using homozygosity mapping, we identified six deleterious mutations in the LPIN1 gene in patients who presented at 2–7 years of age with recurrent, massive rhabdomyolysis. The LPIN1 gene encodes the muscle-spec...

  11. Medical and Surgical Treatment in Pediatric Orbital Myositis Associated with Coxsackie Virus

    Pedro Gil

    2015-01-01

    Full Text Available Purpose. To report a case of orbital myositis associated with Coxsackie virus and its medical and surgical approach. Methods. Complete ophthalmological examination and imaging and analytical investigation were performed. Results. A 6-year-old male presented with subacute painless binocular horizontal diplopia. Examination revealed bilateral best-corrected visual acuity (BCVA of 20/20 and right eye 45-prism-dioptre (PD esotropia in near and distance fixations, with no motility restrictions. Serologic screening was positive for Coxsackie virus acute infection and computerized tomography (CT suggested right eye medial rectus orbital myositis. An oral corticosteroid 1.0 mg/kg/day regimen was started. A new CT after two months showed symmetrical lesions in both medial rectus muscles. Corticosteroids were increased to 1.5 mg/kg/day. After imagiological resolution on the 4th month, alternating 45 PD esotropia persisted. Bilateral 7 mm medial rectus recession was performed after 1 year without spontaneous recovery. At 1-year follow-up, the patient is orthophoric with 200′′ stereopsis and bilateral 20/20 BCVA. Conclusions. To our knowledge, this is the first reported case of orbital myositis associated with Coxsackie virus. This is also the first reported case of isolated strabismus surgery after orbital myositis in pediatric age, highlighting the favourable aesthetic and functional outcomes even in cases of late ocular motility disorders.

  12. Miosite e rabdomiólise na doença mão-pé-boca na infância Myositis and rhabdomyolysis in hand-foot-mouth disease in childhood

    Maria Helena Vaisbich

    2010-03-01

    Full Text Available OBJETIVO: Relatar um caso de doença mão-pé-boca complicada por miosite, rabdomiólise e hepatite, interessante por ser a doença frequente em crianças e poder apresentar complicações graves, apesar de raras. DESCRIÇÃO DO CASO: Paciente de três anos de idade, sexo feminino, com história de febre por três dias, seguida pelo aparecimento de lesões ulceradas em mucosa oral e mialgia intensa. Após três dias, voltou a apresentar febre por mais dois dias (febre bifásica. Nesses dois dias, apresentou lesões eritematosas pelo corpo, principalmente nos pés, mãos e face, e procurou atendimento médico. Evoluiu com aumento de enzimas musculares e hepáticas (CPK com valor máximo de 345.007U/L, TGO 2041U/L, TGP 1589U/L, gama-GT 94U/L e aumento transitório da creatinina sérica, com clearance de creatinina estimado pela estatura de 73mL/minuto/1,73m2 de superfície corporal. Houve melhora progressiva, com hidratação vigorosa e alcalinização da urina, sem necessidade de diálise. COMENTÁRIOS: Trata-se de uma criança com doença mão-pé-boca, com miosite, rabdomiólise e hepatite. São enfatizados os critérios clínicos laboratoriais para o diagnóstico e a importância da monitorização das complicações da doençaOBJECTIVE: To report a hand-foot-mouth disease case, complicated by myositis, rhabdomyolisis and hepatitis, since this is a common disease in children and can result in rare but severe complications. CASE DESCRIPTION: A three-year-old girl with fever for three days, followed by the appearance of ulcerative lesions in the oral mucosa and severe muscular pain; after three days, she presented fever for two more days. At the same time, she had widespread erythematosus rash, especially in her hands, feet and face. She presented high levels of muscular and hepatic enzymes (maximum value of CPK 345.007IU/L, AST 2041IU/L, ALT 1589IU/L, GT 94IU/L, and transitory increase in serum creatinine (maximum value of 0.73mg/dL, creatinine

  13. Ploidy and clinical characteristics of childhood acute myeloid leukemia

    Sandahl, Julie Damgaard; Kjeldsen, Eigil; Abrahamsson, Jonas;

    2014-01-01

    We report the first large series (n = 596) of pediatric acute myeloid leukemia (AML) focusing on modal numbers (MN) from the population-based NOPHO-AML trials. Abnormal karyotypes were present in 452 cases (76%) and numerical aberrations were present in 40% (n = 237) of all pediatric AML. Among...... with early onset (median age 2 years), female sex (57%), and a dominance of acute megakaryoblastic leukemia (AMKL) (29%). Hypodiploidy constituted 8% of all AML and was associated with older age (median age 9 years), male predominance (60%), FAB M2 (56%), and t(8;21)(q22;q22) (56%) with loss of sex...

  14. Cure rates of childhood acute lymphoblastic leukemia in Lithuania and the benefit of joining international treatment protocol

    Vaitkevičienė, Goda; Matuzevičienė, Rėda; Stoškus, Mindaugas;

    2014-01-01

    BACKGROUND: Childhood acute lymphoblastic leukemia (ALL) represents the largest group of pediatric malignancies with long-term survival rates of more than 80% achieved in developed countries. Epidemiological data and survival rates of childhood ALL in Lithuania were lacking. Therefore, the aim of...

  15. Does childhood misfortune raise the risk of acute myocardial infarction in adulthood?

    Morton, Patricia M; Mustillo, Sarah A; Ferraro, Kenneth F

    2014-03-01

    Whereas most research on acute myocardial infarction (AMI) has focused on more proximal influences, such as adult health behaviors, the present study examines the early origins of AMI. Longitudinal data were drawn from the National Survey of Midlife Development in the United States (N = 3032), a nationally representative survey of men and women aged 25-74, which spans from 1995 to 2005. A series of event history analyses modeling age of first AMI investigated the direct effects of accumulated and separate domains of childhood misfortune as well as the mediating effects of adult health lifestyle and psychosocial factors. Findings reveal that accumulated childhood misfortune and child maltreatment increased AMI risk, net of several adult covariates, including family history of AMI. Smoking fully mediated the effects of both accumulated childhood misfortune and child maltreatment. These findings reveal the importance of the early origins of AMI and health behaviors as mediating factors. PMID:24581071

  16. Pharmacokineties of vincristine monotherapy in childhood acute lymphoblastic leukemia

    Groninger, E; Meeuwsen-de Boer, T; Koopmans, P; UGes, D; Sluiter, W; Veerman, A; Kamps, W

    2002-01-01

    We studied vincristine pharmacokinetics in 70 children newly diagnosed with acute lymphoblastic leukemia, after a single dose of vincristine as monotherapy. Vincristine plasma concentrations were measured by HPLC analysis. A two-compartment, first-order pharmacokinetic model was fitted to the data b

  17. Molecular mechanisms of glucocorticoid resistance in childhood acute lymphoblastic leukemia

    W.J.E. Tissing (Wim)

    2006-01-01

    textabstractAcute lymphoblastic leukemia (ALL) is the most common form of cancer in children, with 110 – 120 newly diagnosed children in the Netherlands each year. ALL is a haematological malignancy of lymphoid precursor cells and can be divided into two sub-groups: B-cell precursor ALL and T-cell p

  18. Periostitis and localised myositis in polyarteritis nodosa

    Full text: A 20-year-old man with previously diagnosed polyarteritis nodosa was referred for a bone scan to investigate longstanding knee and lower leg pain. The patient's symptoms worsened with any reduction of steroid dose and his physician was concerned about avascular necrosis of the hips. Plain x-rays of the lower limbs were normal. The blood pool images showed bilaterally increased activity in the anterior muscle compartments of the lower legs, suggestive of localised myositis. Three-hour delayed images showed widespread, increased subperiosteal activity with no evidence of avascular necrosis. Subsequent MRI scanning showed patchy muscle enhancement in both lower legs, also typical of myositis. A muscle biopsy was performed which demonstrated features of both myositis and vasculitis. The patient remains dependent on high-dose steroids for symptom relief. Localised myositis has previously been reported in polyarteritis nodosa and is a recognised, albeit rare, complication of the disorder, the basis of which is not well understood. Diagnostic muscle biopsy should be directed at involved muscle groups, which are best detected with MRI. Lower limb periostitis is well described in polyarteritis nodosa and may result in gross deformity. Several cases have previously been reported in the literature based on radiographic abnormalities, which were not present in our patient. Bone scanning demonstrated the sub-periosteal activity well in our subject and is suggested as a useful investigation in patients with polyarteritis nodosa who complain of lower limb pain. Copyright (2003) The Australian and New Zealand Society of Nuclear Medicine Inc

  19. Consensus definitions of 14 severe acute toxic effects for childhood lymphoblastic leukaemia treatment

    Schmiegelow, Kjeld; Attarbaschi, Andishe; Barzilai, Shlomit;

    2016-01-01

    toxic effects, that no two protocols shared identical definitions of all toxic effects, and that no toxic effect definition was shared by all protocols. Using the Delphi method over three face-to-face plenary meetings, consensus definitions were obtained for all 14 toxic effects. In the overall......Although there are high survival rates for children with acute lymphoblastic leukaemia, their outcome is often counterbalanced by the burden of toxic effects. This is because reported frequencies vary widely across studies, partly because of diverse definitions of toxic effects. Using the Delphi...... method, 15 international childhood acute lymphoblastic leukaemia study groups assessed acute lymphoblastic leukaemia protocols to address toxic effects that were to be considered by the Ponte di Legno working group. 14 acute toxic effects (hypersensitivity to asparaginase, hyperlipidaemia, osteonecrosis...

  20. Executive Function, Coping, and Behavior in Survivors of Childhood Acute Lymphocytic Leukemia*

    Campbell, Laura K.; Scaduto, Mary; Van Slyke, Deborah; Niarhos, Frances; Whitlock, James A.; Compas, Bruce E.

    2008-01-01

    Objective To examine the role of executive function in coping and behavioral outcomes in childhood acute lymphocytic leukemia (ALL) survivors. Methods We examined associations among several domains of executive function (working memory, behavioral inhibition, cognitive flexibility, and self-monitoring), coping, and emotional/behavioral problems in 30 children and adolescents ages 10- to 20-years old who completed treatment for ALL and 30 healthy controls matched on age and sex. Results We fou...

  1. The Role of HDACs Inhibitors in Childhood and Adolescence Acute Leukemias

    Riccardo Masetti; Salvatore Serravalle; Carlotta Biagi; Andrea Pession

    2011-01-01

    Acute leukemia is the most common type of childhood and adolescence cancer, characterized by clonal proliferation of variably differentiated myeloid or lymphoid precursors. Recent insights into the molecular pathogenesis of leukemia have shown that epigenetic modifications, such as deacetylation of histones and DNA methylation, play crucial roles in leukemogenesis, by transcriptional silencing of critical genes. Histone deacetylases (HDACs) are potential targets in the treatment of leukaemia,...

  2. TLR Stimulation of Bone Marrow Lymphoid Precursors from Childhood Acute Leukemia Modifies Their Differentiation Potentials

    Elisa Dorantes-Acosta; Eduardo Vadillo; Adriana Contreras-Quiroz; Juan Carlos Balandrán; Lourdes Arriaga-Pizano; Jessica Purizaca; Sara Huerta-Yepez; Elva Jiménez; Wendy Aguilera; Aurora Medina-Sanson; Héctor Mayani; Rosana Pelayo

    2013-01-01

    Acute leukemias are the most frequent childhood malignancies worldwide and remain a leading cause of morbidity and mortality of relapsed patients. While remarkable progress has been made in characterizing genetic aberrations that may control these hematological disorders, it has also become clear that abnormalities in the bone marrow microenvironment might hit precursor cells and contribute to disease. However, responses of leukemic precursor cells to inflammatory conditions or microbial comp...

  3. Host genome variations and risk of infections during induction treatment for childhood acute lymphoblastic leukaemia

    Lund, Bendik; Wesolowska-Andersen, Agata; Lausen, Birgitte;

    2014-01-01

    Objectives: To investigate association of host genomic variation and risk of infections during treatment for childhood acute lymphoblastic leukaemia (ALL). Methods: We explored association of 34 000 singlenucleotide polymorphisms (SNPs) related primarily to pharmacogenomics and immune function...... to risk of infections among 69 ALL patients on induction therapy. Results: Forty-eight (70%) patients experienced infectious events including 23 with positive blood cultures. Infectious events and positive blood cultures were associated significantly with 24 and 21 SNPs, respectively (P

  4. Acute childhood diarrhoea in northern Ghana: epidemiological, clinical and microbiological characteristics

    Danikuu Francis

    2007-09-01

    Full Text Available Abstract Background Acute diarrhoea is a major cause of childhood morbidity and mortality in sub-Saharan Africa. Its microbiological causes and clinico-epidemiological aspects were examined during the dry season 2005/6 in Tamale, urban northern Ghana. Methods Stool specimens of 243 children with acute diarrhoea and of 124 control children were collected. Patients were clinically examined, and malaria and anaemia were assessed. Rota-, astro-, noro- and adenoviruses were identified by (RT- PCR assays. Intestinal parasites were diagnosed by microscopy, stool antigen assays and PCR, and bacteria by culturing methods. Results Watery stools, fever, weakness, and sunken eyes were the most common symptoms in patients (mean age, 10 months. Malaria occurred in 15% and anaemia in 91%; underweight (22% and wasting (19% were frequent. Intestinal micro-organisms were isolated from 77% of patients and 53% of controls (P P = 0.02. Conclusion Rotavirus-infection is the predominant cause of acute childhood diarrhoea in urban northern Ghana. The abundance of putative enteropathogens among controls may indicate prolonged excretion or limited pathogenicity. In this population with a high burden of diarrhoeal and other diseases, sanitation, health education, and rotavirus-vaccination can be expected to have substantial impact on childhood morbidity.

  5. The diagnosis and management of acute childhood illness: is there a role for health visitors?

    Kendrick, D; Young, A; Futers, D

    2000-12-01

    Much of the published literature on nurse practitioners relates to nurses with backgrounds, training and nursing experience other than health visiting. Health visitors however may be ideally placed to develop their role in the identification and management of acute childhood illness, and such a development may benefit patients, the health service and health visitors themselves. This study examines the current practice and attitudes of heath visitors in identifying and managing acute childhood illness using a postal questionnaire survey in one community National Health Service (NHS) Trust. The majority of responding health visitors reported that they were already identifying and advising on a range of acute childhood illnesses. Their confidence in undertaking these activities varied by condition. They were less confident in identifying and advising on conditions requiring the use of diagnostic equipment, viral illnesses which are becoming increasingly rare, and those with potentially serious outcomes. More knowledge and more training were seen as methods for increasing confidence. Most health visitors could see advantages in taking on this role; almost two thirds felt it would become part of the role of the health visitor in the future and a high level of interest was expressed in developing such a role. PMID:11136418

  6. Growth hormone deficiency predicts cardiovascular risk in young adults treated for acute lymphoblastic leukemia in childhood.

    Link, Katarina; Moëll, Christian; Garwicz, Stanislaw; Cavallin-Ståhl, Eva; Björk, Jonas; Thilén, Ulf; Ahrén, Bo; Erfurth, Eva Marie

    2004-10-01

    Acute lymphoblastic leukemia (ALL) is the most common childhood malignancy, and until recently prophylactic cranial radiotherapy (CRT) was important for achieving long-term survival. Hypothalamic-pituitary hormone insufficiency is a well-recognized consequence of CRT for childhood cancer. Another problem is increased cardiovascular risk, which has been shown in long-term survivors of other childhood cancers. In the only previously reported study on cardiovascular risk after childhood ALL, obesity and dyslipidemia were recorded in a small subgroup treated with CRT, compared with patients treated with chemotherapy. The mechanisms behind the increase in cardiovascular risk in survivors of childhood cancer are not clarified. The aim of the present study was to elucidate mechanisms of increased cardiovascular risk in former childhood ALL patients. A group of 44 ALL survivors (23 males, median age 25 yr, range 19-32 yr at the time of study) treated with CRT (median 24 Gy, 18-30 Gy) at a median age of 5 yr (1-18 yr) and chemotherapy were investigated for prevalence of GH deficiency and cardiovascular risk factors. Comparison was made with controls randomly selected from the general population and individually matched for sex, age, smoking habits, and residence. All patients and controls underwent a GHRH-arginine test, and patients with a peak GH 3.9 microg/liter or greater were further investigated with an additional insulin tolerance test. Significantly higher plasma levels of insulin (P = 0.002), blood glucose (P = 0.01), and serum levels of low-density lipoprotein cholesterol, apolipoprotein (Apo) B, triglycerides, fibrinogen, and leptin (all P childhood ALL patients, a significant increase in cardiovascular risk factors was recorded. We suggest that GH deficiency, induced by CRT, is a primary cause for this because strong correlations between the stimulated GH peak and several of the cardiovascular risk factors were observed. PMID:15472198

  7. Myositis Ossificans of Psoas Muscle: Magnetic Resonance Imaging Findings

    Myositis ossificans is a benign, localized, self-limiting ossifying soft tissue mass with unknown pathogenesis. It may be confused with a malignant tumor of bone or soft tissues, especially in the early stages of the disease. In this report, we present a case of myositis ossificans affecting unilateral psoas muscle, which to our knowledge is a very uncommon location. There was no history of trauma. We describe the imaging findings and clues to early diagnosis of myositis ossificans

  8. Cranial radiation in childhood acute lymphocytic leukemia. Neuropsychologic sequelae

    A battery of neuropsychologic tests was administered ''blindly'' to 18 children with acute lymphocytic leukemia (ALL) who had been randomly assigned to treatment regimens with or without cranial radiation. These children were all in complete continuous remission for more than 3 1/2 years and were no longer receiving therapy. The results indicated no substantial differences between groups as a function of radiation therapy. However, decreased neuropsychologic performance was found when the entire sample was compared with population norms. These data do not support the hypothesis that cranial radiation therapy is responsible for the neuropsychologic sequelae seen in these survivors of ALL. Post hoc multiple regression analysis indicated that parental education levels accounted for more of the neuropsychologic variability seen in these children than other factors such as age at diagnosis, type of therapy, or sex of child

  9. Side effect of cranial radiation in childhood acute leukemia, 1

    We examined the somnolence syndrome, which is one of the side effects of cranial irradiation. Out of 53 patients in acute leukemia who had received cranial irradiation, nine patients (17%) developed the somnolence syndrome. Patients with the somnolence syndrome showed slow waves on EEG. Some patients had ventricular dilatation and widening of sulci before cranial irradiation on CT findings, but these findings improved after cranial irradiation. Out of nine cases with the somnolence syndrome, 6 patients survived and did not experience difficulties in school. But one patient showed calcification on CT brain scan. It is considered that the cause of the somnolence syndrome is a trasient inhibition of myelin synthesis and most patients improved without serious sequelae. It is necessary to follow up many cases of somnolence syndrome. (author)

  10. Oral rehydration salts, zinc supplement and rota virus vaccine in the management of childhood acute diarrhea

    Abdulwahab MA Telmesani

    2010-01-01

    Full Text Available Acute diarrhea remains a major cause of morbidity and mortality in children. Since the introduction of oral rehydration salts (ORS mortality has dropped to less than 50% worldwide. Low osmolarity ORS improved the outcome and reduced the hospitalization further. Zinc difficiency has been found to be associated with severe episodes of acute diarrhea. Zinc supplement in developing countries did reduce the incidence and prevalence of diarrhea. In addition, Zinc supplement significantly reduced the severity of diarrhea and duration of the episode. In the Americas and Europe, Rota virus vaccine was 90% effective in preventing severe episodes of severe rotavirus gastroenteritis. This review concludes that low osmolarilty ORS, zinc supplementation and rotavirus vaccine are major factors in reducing the morbidity, mortality and hospitalization resulting from to acute gastroenteritis in childhood.

  11. Prognosis and complications of acute childhood leukemia after prophylactic treatment of the central nervous

    From 1970 through 1979, 22 children with acute childhood leukemia and in remimmion were treated with preventive central nervous system (CNS) irradiation and simultaneous intrathecal methotrexate. A minimum follow-up duration was five months. Of 22 cases, 20 were acute lymphocytic leukemia (ALL) and 2 were acute myelocytic leukemia (ALL). Five-year cumulative survival rate and five-year relapse free survival rate of ALL case were 48% and 46% respectively. Nor neurological disorders after the prophylactic combined therapy were recognized clinically by the time when this follow-up was finished. Of 15 children with ALL who were followed by computed tomography of the brain, 5(33%) had abnormal findings. Dilatation of the ventricles were seen in 3 cases, and low density areas of the occipital regions in 2 cases. (author)

  12. Outcome following late marrow relapse in childhood acute lymphoblastic leukemia

    Thirty-four children with acute lymphoblastic leukemia, who developed bone marrow relapse after treatment was electively stopped, received reinduction, consolidation, continuing therapy, and intrathecal (IT) methotrexate (MTX). Sixteen children who relapsed within six months of stopping treatment had a median second-remission duration of 26 weeks; all next relapses occurred in the bone marrow. In 18 children who relapsed later, the median duration of second remission was in excess of two years, but after a minimum of four years follow-up, 16 patients have so far relapsed again (six in the CNS). CNS relapse occurred as a next event in four of 17 children who received five IT MTX injections only and in two of 14 children who received additional regular IT MTX. Although children with late marrow relapses may achieve long second remissions, their long-term out-look is poor, and regular IT MTX does not afford adequate CNS prophylaxis. It remains to be seen whether more intensive chemotherapy, including high-dose chemoradiotherapy and bone marrow transplantation, will improve the prognosis in this group of patients

  13. The clinical significance of lung hypoexpansion in acute childhood asthma

    Spottswood, Stephanie E. [Department of Radiology, Medical College of Virginia, Virginia Commonwealth University Health System, Box 980615, 23298-0615, Richmond, VA (United States); Department of Radiology, The Children' s Hospital of the King' s Daughters, 601 Children' s Lane, Norfolk, VA 23507 (United States); Allison, Kelley Z.; Narla, Lakshmana D.; Lowry, Patricia A. [Department of Radiology, Medical College of Virginia, Virginia Commonwealth University Health System, Box 980615, 23298-0615, Richmond, VA (United States); Lopatina, Olga A.; Sethi, Narinder N. [School of Medicine, Medical College of Virginia, Virginia Commonwealth University Health System, Richmond, VA (United States); Nettleman, Mary D. [Department of Internal Medicine, B-427 Clinical Center, Michigan State University, East Lansing, MI 48824 (United States)

    2004-04-01

    Many children experiencing acute asthmatic episodes have chest radiographs, which may show lung hyperinflation, hypoinflation, or normal inflation. Lung hypoinflation may be a sign of respiratory fatigue and poor prognosis. To compare the clinical course in children with asthma according to the degree of lung inflation on chest radiographs. We conducted a retrospective study during a 24-month period (from July 1999 to July 2001) of children aged 0-17 years, who presented to a pediatric emergency department or outpatient clinic with an asthma exacerbation. Chest radiographs obtained at presentation were reviewed independently by three pediatric radiologists who were blinded to the admission status of the patient. The correlation between hypoinflation and hospital admission was assessed in three age groups: 0-2 years, 3-5 years, and 6-17 years. Hypoinflation on chest radiographs was significantly correlated with hospital admission for children aged 6-17 years (odds ratio 16.00, 95% confidence interval 1.89-135.43). The inter-reader agreement for interpretation of these radiographs was strong, with a kappa score of 0.76. Hypoinflation was not correlated with admission in younger children. Lung hypoinflation is associated with a greater likelihood of hospital admission in children aged 6 years or older. Therefore, hypoinflation was a poor prognostic sign and may warrant more aggressive therapy. (orig.)

  14. The clinical significance of lung hypoexpansion in acute childhood asthma

    Many children experiencing acute asthmatic episodes have chest radiographs, which may show lung hyperinflation, hypoinflation, or normal inflation. Lung hypoinflation may be a sign of respiratory fatigue and poor prognosis. To compare the clinical course in children with asthma according to the degree of lung inflation on chest radiographs. We conducted a retrospective study during a 24-month period (from July 1999 to July 2001) of children aged 0-17 years, who presented to a pediatric emergency department or outpatient clinic with an asthma exacerbation. Chest radiographs obtained at presentation were reviewed independently by three pediatric radiologists who were blinded to the admission status of the patient. The correlation between hypoinflation and hospital admission was assessed in three age groups: 0-2 years, 3-5 years, and 6-17 years. Hypoinflation on chest radiographs was significantly correlated with hospital admission for children aged 6-17 years (odds ratio 16.00, 95% confidence interval 1.89-135.43). The inter-reader agreement for interpretation of these radiographs was strong, with a kappa score of 0.76. Hypoinflation was not correlated with admission in younger children. Lung hypoinflation is associated with a greater likelihood of hospital admission in children aged 6 years or older. Therefore, hypoinflation was a poor prognostic sign and may warrant more aggressive therapy. (orig.)

  15. Two cases of intracerebral calcification in childhood acute leukemia

    Two children with acute lynphocytic leukemia (ALL) had intracerebral calcifications. The first case was a girl diagnosed as ALL in her seventh month. She developed two episodes of meningeal leukemia. She was treated by intrathecal methotrexate (MTX) and 19.80 Gy of whole-brain irradiation. Three months after irradiation, CT revealed low-density areas around both the lateral ventricles, especially at the anterior horns, suggesting necrotizing leukoencephalopathy. Seven months after irradiation, CT revealed bilateral gyriform calcifications of both cerebral hemispheres. The second case was a boy diagnosed as ALL at 1.5 years old. He was treated by prop hylactic intrathecal MTX and 24.00 Gy of whole-brain irradiation. Fourteen months after irradiation, CT revealed multiple small punctated calcifications of both cerebral hemispheres. The combination of whole-brain irradiation with MTX therapy was perhaps the cause of the high incidence of these calcifications, because the irradiation doses in these cases were too small to cause these injuries. (author)

  16. Fungal myositis in children: serial ultrasonographic findings

    Kwon, Jung Hwa; Lee, Hee Jung; Choi, Jin Soo [Keimyung University School of Medicine, Daegu (Korea, Republic of)

    2003-08-01

    To evaluate serial ultrasonographic findings of fungal myositis in children. Eleven lesions caused by fungal myositis and occurring in six children were included in this study. Eight lesions in five children were histopathologically proven and the other three were clinically diagnosed. Serial ultrasonographic findings were retrospectively evaluated in terms of size, location, margin, internal echotexture and adjacent cortical change occurring during the follow-up period ranging from five days to two months. Three patients (50%) had multiple lesions. The sites of involvment were the thigh (n=4), calf (n=3), chest wall (n=2), abdominal wall (n=1) and forearm (n=1). Initially, diffuse muscular swelling was revealed, with ill-defined hypoechoic lesions confined to the muscle layer (n=8). Follow-up examination of eight lesions over a period of 5-10 days showed that round central echogenic lesions were surrounded by previous slightly echogenic lesions (n=6, 75%). Long-term follow-up of five lesions over a two-month period revealed periosteal thickening in one case (20%), and the peristence of echogenic solid nodules in four (80%). Pathologic examination showed that the central lesions correlated with a fungus ball and the peripheral slightly echogenic lesions corresponded to hematoma and necrosis. Serial ultrasonographic findings of fungal myositis in children revealed relatively constant features in each case. In particular, the findings of muscular necrosis and a fungus ball over a period of 5-14 days were thought to be characteristic.

  17. New genetics and diagnosis of childhood B-cell precursor acute lymphoblastic leukemia

    Christine Harrison

    2011-06-01

    Full Text Available Over the last 50 years, while significant advances have been made in the successful treatment of childhood leukaemia, similar progress has been made in understanding the genetics of the disease. In childhood B-cell precursor acute lymphoblastic leukaemia (BCP-ALL, the incidences of individual chromosomal abnormalities are well established and cytogenetics provides a reliable tool for risk stratification for treatment. In spite of this role, a number of patients will relapse. Increasing numbers of additional genetic changes, including deletions and mutations, are being discovered. Their associations with established cytogenetic subgroups and with each other remain unclear. Whether they have a link to outcome is the most important factor in terms of refinement of risk factors in relation to clinical trials. For a number of newly identified abnormalities, appropriately modified therapy has significantly improved outcome. Alternatively, some of these aberrations are providing novel molecular markers for targeted therapy.

  18. Deletion analysis of p16(INKa) and p15(INKb) in relapsed childhood acute lymphoblastic leukemia.

    Graf Einsiedel, Hagen; Taube, Tillmann; Hartmann, Reinhard; Wellmann, Sven; Seifert, Georg; Henze, Günter; Seeger, Karl

    2002-06-15

    This study aimed at determining the prevalence of INK4 deletions and their impact on outcome in 125 children with acute lymphoblastic leukemia (ALL) at first relapse using real-time quantitative polymerase chain reaction. Patients were enrolled into relapse trials ALL-REZ BFM (ALL-Relapse Berlin-Frankfurt-Münster) 90 and 96. The prevalence of p16(INK4a) and p15(INK4b) homozygous deletions was 35% (44 of 125) and 30% (38 of 125), respectively. A highly significant association of both gene deletions was found with the 2 major adverse prognostic factors known for relapsed childhood ALL: T-cell immunophenotype and first remission duration. There was no correlation between INK4 deletions and probability of event-free survival. These findings argue against an independent prognostic role of INK4 deletions in relapsed childhood ALL. PMID:12036898

  19. Peritoneal Dialysis in Childhood Acute Kidney Injury: Experience in Southwest Nigeria

    Ademola, Adebowale Dele; Asinobi, Adanze Onyenonachi; Ogunkunle, Oluwatoyin Olufunmilayo; Yusuf, Bamidele Nurudeen; Ojo, Olalekan Ezekiel

    2012-01-01

    ♦ Background: The choices for renal replacement therapy (RRT) in childhood acute kidney injury (AKI) are limited in low-resource settings. Peritoneal dialysis (PD) appears to be the most practical modality for RRT in young children with AKI in such settings. Data from sub-Saharan Africa on the use of PD in childhood AKI are few. ♦ Methods: We performed a retrospective study of children who underwent PD for AKI at a tertiary-care hospital in southwest Nigeria from February 2004 to March 2011 (85 months). ♦ Results: The study included 27 children (55.6% female). Mean age was 3.1 ± 2.6 years, with the youngest being 7 days, and the oldest, 9 years. The causes of AKI were intravascular hemolysis (n = 11), septicemia (n = 8), acute glomerulonephritis (n = 3), gastroenteritis (n = 3), and hemolytic uremic syndrome (n = 2). Peritoneal dialysis was performed manually using percutaneous or adapted catheters. Duration of PD ranged from 6 hours to 12 days (mean: 5.0 ± 3.3 days). The main complications were peritonitis (n = 10), pericatheter leakage (n = 9), and catheter outflow obstruction (n = 5). Of the 27 patients, 19 (70%) survived till discharge. ♦ Conclusions: In low-resource settings, PD can be successfully performed for the management of childhood AKI. In our hospital, the use of adapted catheters may have contributed to the high complication rates. Peritoneal dialysis should be promoted for the management of childhood AKI in low-resource settings, and access to percutaneous or Tenckhoff catheters, dialysis fluid, and automated PD should be increased. PMID:22550119

  20. Childhood central nervous system leukemia: historical perspectives, current therapy, and acute neurological sequelae

    Laningham, Fred H. [St. Jude Children' s Research Hospital, Division of Diagnostic Imaging, Department of Radiological Sciences, Memphis, TN (United States); University of Tennessee Health Sciences Center, Memphis, TN (United States); Kun, Larry E. [St. Jude Children' s Research Hospital, Division of Radiation Oncology, Department of Radiological Sciences, Memphis, TN (United States); University of Tennessee Health Sciences Center, Memphis, TN (United States); Reddick, Wilburn E.; Ogg, Robert J. [St. Jude Children' s Research Hospital, Division of Translational Imaging Research, Department of Radiological Sciences, Memphis, TN (United States); Morris, E.B. [St. Jude Children' s Research Hospital, Department of Oncology, Memphis, TN (United States); Pui, Ching-Hon [St. Jude Children' s Research Hospital, Department of Oncology, Memphis, TN (United States); University of Tennessee Health Sciences Center, Memphis, TN (United States)

    2007-11-15

    During the past three decades, improvements in the treatment of childhood leukemia have resulted in high cure rates, particularly for acute lymphoblastic leukemia (ALL). Unfortunately, successful therapy has come with a price, as significant morbidity can result from neurological affects which harm the brain and spinal cord. The expectation and hope is that chemotherapy, as a primary means of CNS therapy, will result in acceptable disease control with less CNS morbidity than has been observed with combinations of chemotherapy and radiotherapy over the past several decades. In this review we discuss the poignant, historical aspects of CNS leukemia therapy, outline current methods of systemic and CNS leukemia therapy, and present imaging findings we have encountered in childhood leukemia patients with a variety of acute neurological conditions. A major objective of our research is to understand the neuroimaging correlates of acute and chronic effects of cancer and therapy. Specific features related to CNS leukemia and associated short-term toxicities, both disease- and therapy-related, are emphasized in this review with the specific neuroimaging findings. Specific CNS findings are similarly important when treating acute myelogenous leukemia (AML), and details of leukemic involvement and toxicities are also presented in this entity. Despite contemporary treatment approaches which favor the use of chemotherapy (including intrathecal therapy) over radiotherapy in the treatment of CNS leukemia, children still occasionally experience morbid neurotoxicity. Standard neuroimaging is sufficient to identify a variety of neurotoxic sequelae in children, and often suggest specific etiologies. Specific neuroimaging findings frequently indicate a need to alter antileukemia therapy. It is important to appreciate that intrathecal and high doses of systemic chemotherapy are not innocuous and are associated with acute, specific, recognizable, and often serious neurological

  1. Childhood central nervous system leukemia: historical perspectives, current therapy, and acute neurological sequelae

    During the past three decades, improvements in the treatment of childhood leukemia have resulted in high cure rates, particularly for acute lymphoblastic leukemia (ALL). Unfortunately, successful therapy has come with a price, as significant morbidity can result from neurological affects which harm the brain and spinal cord. The expectation and hope is that chemotherapy, as a primary means of CNS therapy, will result in acceptable disease control with less CNS morbidity than has been observed with combinations of chemotherapy and radiotherapy over the past several decades. In this review we discuss the poignant, historical aspects of CNS leukemia therapy, outline current methods of systemic and CNS leukemia therapy, and present imaging findings we have encountered in childhood leukemia patients with a variety of acute neurological conditions. A major objective of our research is to understand the neuroimaging correlates of acute and chronic effects of cancer and therapy. Specific features related to CNS leukemia and associated short-term toxicities, both disease- and therapy-related, are emphasized in this review with the specific neuroimaging findings. Specific CNS findings are similarly important when treating acute myelogenous leukemia (AML), and details of leukemic involvement and toxicities are also presented in this entity. Despite contemporary treatment approaches which favor the use of chemotherapy (including intrathecal therapy) over radiotherapy in the treatment of CNS leukemia, children still occasionally experience morbid neurotoxicity. Standard neuroimaging is sufficient to identify a variety of neurotoxic sequelae in children, and often suggest specific etiologies. Specific neuroimaging findings frequently indicate a need to alter antileukemia therapy. It is important to appreciate that intrathecal and high doses of systemic chemotherapy are not innocuous and are associated with acute, specific, recognizable, and often serious neurological

  2. Infective myositis A one-case report

    Daoyou Zhou; Jianwen Guo; Yan Huang

    2008-01-01

    BACKGROUND: Infective myositis is rare. The retrospective report of clinical data and symptoms from one patient with infective myositis will hopefully provide more information for clinicians in the diagnosis of this disease. METHODS: A male patient, 65 years old, was admitted with "fever and muscle pain since four days ago, accompanied by inertia of all limbs for one day", to the First Department of Neurology, Guangdong Provincial Hospital of Traditional Chinese Medicine on April 25th, 2005. Following admission, a history of diseases was record, and detailed physical and neurological examinations were performed. During the examination, symmetrical myasthenia appeared, tendon reflex disappeared, and creatine kinase levels were increased 500 times higher than normal. The patient was primarily diagnosed with hypokalemic periodic paralysis, myositis, Guillain-Barre syndrome, and upper respiratory infection. Subsequently, the patient was treated with the following: cefuroxime for infection, potassium supplements, breviscapin for promoting blood circulation through the removal of stasis, and ATP/CO-A for myocardial nutrition. Antiviral drugs were not administered. However, laboratory samples were continuously monitored. Creatine kinase levels decreased to normal, and muscle pain was obviously relieved following antibiotics treatment. The results led to a final diagnosis of infective myositis.RESULTS: Four days after treatment (April 29th), muscular tenderness and throat congestion were obviously improved (+/-). The neurological examination showed the patient was conscious, cooperated with treatment, and had normal intellect. No abnormalities of the cranial nerve were observed upon examination. Proximal and distal muscle strength and muscular tensions of the four limbs were all normal. Reflexes of the right biceps brachii muscle and its tendon were decreased, and knee tendon and Achilles tendon reflex were not induced. A Babinski reflex was not detected. The neurological

  3. Gene Dose Effects of GSTM1, GSTT1 and GSTP1 Polymorphisms on Outcome in Childhood Acute Lymphoblastic Leukemia

    Borst, Louise; Buchard, Anders; Rosthoj, Susanne;

    2012-01-01

    Children with acute lymphoblastic leukemia (ALL) react very differently to chemotherapy. One explanation for this is inherited genetic variation. The glutathione S-transferase (GST) enzymes inactivate a number of chemotherapeutic drugs administered in childhood ALL therapy. Two multiplexing metho...... influence outcome in childhood ALL.......Children with acute lymphoblastic leukemia (ALL) react very differently to chemotherapy. One explanation for this is inherited genetic variation. The glutathione S-transferase (GST) enzymes inactivate a number of chemotherapeutic drugs administered in childhood ALL therapy. Two multiplexing methods...... were applied for genotyping the GSTM1 and GSTT1 genes (distinguishing between 0, 1, or 2 gene copies) and the GSTP1 313 A>G polymorphism, simultaneously. A total of 263 childhood ALL patients were genotyped. No gene dose effect on outcome was demonstrated with either GST polymorphisms. Grouping of GSTM...

  4. The role of radiation therapy in childhood acute leukemia. A review from the viewpoint of basic and clinical radiation oncology

    Radiation therapy has been playing important roles in the treatment of childhood acute leukemia since the 1970s. The first is the preventive cranial irradiation for central nervous system therapy in acute lymphoblastic leukemia. The second is the total body irradiation as conditioning before bone marrow transplantation for children with acute myeloid leukemia in first remission and with acute lymphoblastic leukemia in second remission. Although some late effects have been reported, a part of them could be overcome by technical improvement in radiation and salvage therapy. Radiation therapy for children might have a successful outcome on a delicate balance between efficiencies and potential late toxicities. The role of radiation therapy for childhood acute leukemia was reviewed from the standpoint of basic and clinical radiation oncology in this paper. (author)

  5. Acute necrotizing encephalopathy of childhood: a fatal complication of swine flu

    Acute necrotizing encephalopathy of childhood (ANEC) is a rare condition characterized by the presence of multifocal symmetrical brain lesions involving mainly thalami, brainstem, cerebellum and white matter. ANEC is a serious and life threatening complication of simple viral infections. We present a case of a young child who developed this condition with classical clinical and radiological findings consistent with ANEC, secondary to swine flu (H1N1). He needed ventilatory support and had profound motor and intellectual deficit on discharge. We report this case with aim of raising awareness about this fatal complication of swine flu which has become a global health care issue these days. (author)

  6. Ophthalmic evaluation of long-term survivors of childhood acute lymphoblastic leukemia

    Thirty-four long-term survivors of childhood acute lymphoblastic leukemia (ALL) underwent comprehensive ophthalmic examinations to detect retinopathy or other ocular sequelae. Sixteen of the 34 patients received whole brain radiation (greater than or equal to 2400 rad). All 18 patients in the non-radiated group had normal eye examinations, while 4 of 16 in the radiated group had ocular abnormalities. None of the ocular abnormalities could be definitely attributed to radiation and all patients had normal visual acuity. No radiation retinopathy was found in either group

  7. Myasthenia gravis and masticatory muscle myositis in a dog

    Clooten, Jennifer K.; Woods, J. P.; Smith-Maxie, Laura L.

    2003-01-01

    A 21-month-old, castrated male Vizsla was presented for pelvic limb weakness, difficulty opening his mouth, ptyalism, voice change, and urinary incontinence. Myasthenia gravis and masticatory myositis were diagnosed. The unusual clinical findings, diagnosis, treatment, and case outcome are described, followed by a brief discussion of myasthenia gravis and masticatory myositis.

  8. Consensus definitions of 14 severe acute toxic effects for childhood lymphoblastic leukaemia treatment: a Delphi consensus.

    Schmiegelow, Kjeld; Attarbaschi, Andishe; Barzilai, Shlomit; Escherich, Gabriele; Frandsen, Thomas Leth; Halsey, Christina; Hough, Rachael; Jeha, Sima; Kato, Motohiro; Liang, Der-Cherng; Mikkelsen, Torben Stamm; Möricke, Anja; Niinimäki, Riitta; Piette, Caroline; Putti, Maria Caterina; Raetz, Elizabeth; Silverman, Lewis B; Skinner, Roderick; Tuckuviene, Ruta; van der Sluis, Inge; Zapotocka, Ester

    2016-06-01

    Although there are high survival rates for children with acute lymphoblastic leukaemia, their outcome is often counterbalanced by the burden of toxic effects. This is because reported frequencies vary widely across studies, partly because of diverse definitions of toxic effects. Using the Delphi method, 15 international childhood acute lymphoblastic leukaemia study groups assessed acute lymphoblastic leukaemia protocols to address toxic effects that were to be considered by the Ponte di Legno working group. 14 acute toxic effects (hypersensitivity to asparaginase, hyperlipidaemia, osteonecrosis, asparaginase-associated pancreatitis, arterial hypertension, posterior reversible encephalopathy syndrome, seizures, depressed level of consciousness, methotrexate-related stroke-like syndrome, peripheral neuropathy, high-dose methotrexate-related nephrotoxicity, sinusoidal obstructive syndrome, thromboembolism, and Pneumocystis jirovecii pneumonia) that are serious but too rare to be addressed comprehensively within any single group, or are deemed to need consensus definitions for reliable incidence comparisons, were selected for assessment. Our results showed that none of the protocols addressed all 14 toxic effects, that no two protocols shared identical definitions of all toxic effects, and that no toxic effect definition was shared by all protocols. Using the Delphi method over three face-to-face plenary meetings, consensus definitions were obtained for all 14 toxic effects. In the overall assessment of outcome of acute lymphoblastic leukaemia treatment, these expert opinion-based definitions will allow reliable comparisons of frequencies and severities of acute toxic effects across treatment protocols, and facilitate international research on cause, guidelines for treatment adaptation, preventive strategies, and development of consensus algorithms for reporting on acute lymphoblastic leukaemia treatment. PMID:27299279

  9. Comparison of MRI and renal cortical scintigraphy findings in childhood acute pyelonephritis: preliminary experience

    Objective: The diagnosis of acute pyelonephritis in children remains a clinical challenge. It may cause permanent renal scar formation and results in the chronic renal failure if prompt diagnosis and treatment are delayed. The purpose of this study is to compare magnetic resonance imaging (MRI) and renal cortical scintigraphy (RCS) findings in childhood acute pyelonephritis and to determine pyelonephritic foci in the acute phase. Materials and method: Twenty children (15 females and five males) with symptoms dysuria, enuresis, costovertebral pain, fever of 37.5 degree sign C or more and/or positive urine culture were imaged by unenhanced turbo spin echo T2, spin echo T1-weighted, pre- and post-gadolinium inversion recovery MRI and RCS. Both imaging techniques were read independently by two radiologists and nuclear medicine specialists. Sensitivity and specificity of MRI in detecting acute pyelonephritic foci and scar lesions were calculated. Furthermore, in order to calculate the reliability of MRI over RCS in differentiating scar tissue and acute pyelonephritic foci, follow-up MRI studies were done in six patients after treatment of acute pyelonephritis. Results: Sensitivity and specificity of MRI in the detection of pyelonephritic lesions were found to be 90.9 and 88.8%, respectively. There is no statistically significant difference in lesion detection between the two diagnostic modalities (P>0.05). Conclusion: Post-gadolinium MR images show significant correlation with RCS in the determination of renal pathology. Moreover, the ability of discriminating acute pyelonephritic foci and renal scar in early stages of disease is the superiority of MRI

  10. Comparison of MRI and renal cortical scintigraphy findings in childhood acute pyelonephritis: preliminary experience

    Kovanlikaya, Arzu; Okkay, Nese; Cakmakci, Handan E-mail: cakmakh@egenet.com.tr; Oezdogan, Oezhan; Degirmenci, Berna; Kavukcu, Salih

    2004-01-01

    Objective: The diagnosis of acute pyelonephritis in children remains a clinical challenge. It may cause permanent renal scar formation and results in the chronic renal failure if prompt diagnosis and treatment are delayed. The purpose of this study is to compare magnetic resonance imaging (MRI) and renal cortical scintigraphy (RCS) findings in childhood acute pyelonephritis and to determine pyelonephritic foci in the acute phase. Materials and method: Twenty children (15 females and five males) with symptoms dysuria, enuresis, costovertebral pain, fever of 37.5 degree sign C or more and/or positive urine culture were imaged by unenhanced turbo spin echo T2, spin echo T1-weighted, pre- and post-gadolinium inversion recovery MRI and RCS. Both imaging techniques were read independently by two radiologists and nuclear medicine specialists. Sensitivity and specificity of MRI in detecting acute pyelonephritic foci and scar lesions were calculated. Furthermore, in order to calculate the reliability of MRI over RCS in differentiating scar tissue and acute pyelonephritic foci, follow-up MRI studies were done in six patients after treatment of acute pyelonephritis. Results: Sensitivity and specificity of MRI in the detection of pyelonephritic lesions were found to be 90.9 and 88.8%, respectively. There is no statistically significant difference in lesion detection between the two diagnostic modalities (P>0.05). Conclusion: Post-gadolinium MR images show significant correlation with RCS in the determination of renal pathology. Moreover, the ability of discriminating acute pyelonephritic foci and renal scar in early stages of disease is the superiority of MRI.

  11. The frequency of NPM1 mutations in childhood acute myeloid leukemia

    Karamolegou Kalliopi

    2010-10-01

    Full Text Available Abstract Background Mutations in the nucleophosmin (NPM1 gene have been solely associated with childhood acute myeloid leukemia (AML. We evaluated the frequency of NPM1 mutations in childhood AML, their relation to clinical and cytogenetic features and the presence of common FLT3 and RAS mutations. Results NPM1 mutations were found in 8% of cases. They involved the typical type 'A' mutation and one novel mutation characterized by two individual base pair substitutions, which resulted in 2 amino acid changes (W290 and (S293 in the NPM protein. FLT3/ITD mutations were observed in 12% of the cases and in one NPM1-mutated case bearing also t(8;21 (q22;q22. No common RAS mutations were identified. Conclusions A relatively consistent NPM1 mutation rate was observed, but with variations in types of mutations. The role of different types of NPM1 mutations, either individually or in the presence of other common gene mutations may be essential for childhood AML prognosis.

  12. Late effects of treatment in survivors of childhood acute lymphoblastic leukaemia

    The overall aim of this study was a comprehensive assessment of the nature and severity of the late effects of treatment in a group of children surviving acute lymphoblastic leukaemia. In the absence of damage preceding treatment, late effects could be ascribed to treatment. Cranial irradiation, methotrexate, L-asparaginase and cytosine arabinoside are therapeutic modalities most likely to cause injury to the central nervous system. Survivors of childhood leukaemia also showed an increase in weight-for-height during and after therapy which appeared to be the consequence of a loss in statural growth as well as increasing weight-for-age. Assessment of endocrine function in leukaemia survivors indicated abnormalities in the regulation of growth hormone and thyroid stimulating hormone in some patients. Survivors of childhood leukaemia were shown to have an intellectual deficit compared with their siblings and a high incidence of visual-perceptual defects. The intellectual effects of lower doses of cranial irradiation are as yet unknown. A variety of minor neurological abnormalities were detected among leukaemia survivors and thought to be related to preceding central nervous system 'prophylactic' chemotherapy and irradiation. A new instrument, the functional deficit score, was derived to reflect overall outcome in survivors of childhood leukaemia. With few exceptions, leukaemia survivors in this study had received 2400 rads of deep x-ray therapy as cranial irradiation. This dosage has since been reduced world-wide. Current cranial irradiation 'prophylaxis' consists of 1800 rad of megavoltage radiotherapy

  13. Parental Tobacco Smoking and Acute Myeloid Leukemia: The Childhood Leukemia International Consortium.

    Metayer, Catherine; Petridou, Eleni; Aranguré, Juan Manuel Mejía; Roman, Eve; Schüz, Joachim; Magnani, Corrado; Mora, Ana Maria; Mueller, Beth A; de Oliveira, Maria S Pombo; Dockerty, John D; McCauley, Kathryn; Lightfoot, Tracy; Hatzipantelis, Emmanouel; Rudant, Jérémie; Flores-Lujano, Janet; Kaatsch, Peter; Miligi, Lucia; Wesseling, Catharina; Doody, David R; Moschovi, Maria; Orsi, Laurent; Mattioli, Stefano; Selvin, Steve; Kang, Alice Y; Clavel, Jacqueline

    2016-08-15

    The association between tobacco smoke and acute myeloid leukemia (AML) is well established in adults but not in children. Individual-level data on parental cigarette smoking were obtained from 12 case-control studies from the Childhood Leukemia International Consortium (CLIC, 1974-2012), including 1,330 AML cases diagnosed at age controls. We conducted pooled analyses of CLIC studies, as well as meta-analyses of CLIC and non-CLIC studies. Overall, maternal smoking before, during, or after pregnancy was not associated with childhood AML; there was a suggestion, however, that smoking during pregnancy was associated with an increased risk in Hispanics (odds ratio = 2.08, 95% confidence interval (CI): 1.20, 3.61) but not in other ethnic groups. By contrast, the odds ratios for paternal lifetime smoking were 1.34 (95% CI: 1.11, 1.62) and 1.18 (95% CI: 0.92, 1.51) in pooled and meta-analyses, respectively. Overall, increased risks from 1.2- to 1.3-fold were observed for pre- and postnatal smoking (P < 0.05), with higher risks reported for heavy smokers. Associations with paternal smoking varied by histological type. Our analyses suggest an association between paternal smoking and childhood AML. The association with maternal smoking appears limited to Hispanic children, raising questions about ethnic differences in tobacco-related exposures and biological mechanisms, as well as study-specific biases. PMID:27492895

  14. Deregulated WNT signaling in childhood T-cell acute lymphoblastic leukemia

    WNT signaling has been implicated in the regulation of hematopoietic stem cells and plays an important role during T-cell development in thymus. Here we investigated WNT pathway activation in childhood T-cell acute lymphoblastic leukemia (T-ALL) patients. To evaluate the potential role of WNT signaling in T-cell leukomogenesis, we performed expression analysis of key components of WNT pathway. More than 85% of the childhood T-ALL patients showed upregulated β-catenin expression at the protein level compared with normal human thymocytes. The impact of this upregulation was reflected in high expression of known target genes (AXIN2, c-MYC, TCF1 and LEF). Especially AXIN2, the universal target gene of WNT pathway, was upregulated at both mRNA and protein levels in ∼40% of the patients. When β-CATENIN gene was silenced by small interfering RNA, the cancer cells showed higher rates of apoptosis. These results demonstrate that abnormal WNT signaling activation occurs in a significant fraction of human T-ALL cases independent of known T-ALL risk factors. We conclude that deregulated WNT signaling is a novel oncogenic event in childhood T-ALL

  15. Childhood Sjögren syndrome presenting as acute brainstem encephalitis.

    Matsui, Yoriko; Takenouchi, Toshiki; Narabayashi, Atsushi; Ohara, Kentaro; Nakahara, Tadaki; Takahashi, Takao

    2016-01-01

    Sjögren syndrome is an autoimmune disease characterized by dry mouth and eyes, known as sicca symptoms. The exact spectrum of neurological involvement, especially of the central nervous system, in childhood Sjögren syndrome has not been well defined. We report a girl who presented with acute febrile brainstem encephalitis. In retrospect, she had exhibited a preceding history of recurrent conjunctivitis and strong halitosis that could be considered as sicca symptoms. The histopathology results of a minor salivary biopsy, the presence of anti-SSA/Ro antibody, and keratoconjunctivitis confirmed the diagnosis of Sjögren syndrome. Commonly observed features in previously reported patients with childhood Sjögren syndrome and central nervous system complications have included fever at the time of neurologic presentation, cerebrospinal fluid pleocytosis, abnormal neuroimaging, and positivity for several specific antibodies. In children presenting with unknown acute febrile encephalopathy, Sjögren syndrome should be included in the differential diagnosis, especially when sicca symptoms are present. PMID:26006751

  16. TLR Stimulation of Bone Marrow Lymphoid Precursors from Childhood Acute Leukemia Modifies Their Differentiation Potentials

    Elisa Dorantes-Acosta

    2013-01-01

    Full Text Available Acute leukemias are the most frequent childhood malignancies worldwide and remain a leading cause of morbidity and mortality of relapsed patients. While remarkable progress has been made in characterizing genetic aberrations that may control these hematological disorders, it has also become clear that abnormalities in the bone marrow microenvironment might hit precursor cells and contribute to disease. However, responses of leukemic precursor cells to inflammatory conditions or microbial components upon infection are yet unexplored. Our previous work and increasing evidence indicate that Toll-like receptors (TLRs in the earliest stages of lymphoid development in mice and humans provide an important mechanism for producing cells of the innate immune system. Using highly controlled co-culture systems, we now show that lymphoid precursors from leukemic bone marrow express TLRs and respond to their ligation by changing cell differentiation patterns. While no apparent contribution of TLR signals to tumor progression was recorded for any of the investigated diseases, the replenishment of innate cells was consistently promoted upon in vitro TLR exposure, suggesting that early recognition of pathogen-associated molecules might be implicated in the regulation of hematopoietic cell fate decisions in childhood acute leukemia.

  17. Cranial radiotherapy predisposes to abdominal adiposity in survivors of childhood acute lymphocytic leukemia

    Advances in treatment of acute lymphocytic leukemia increased the likelihood of developing late treatment-associated effects, such as abdominal adiposity, increasing the risk of cardiovascular disease in this population. Cranial radiotherapy is one of the factors that might be involved in this process. The aim of this study was to determine the effect of cranial radiotherapy on adiposity indexes in survivors of acute lymphocytic leukemia. A comparative cross-sectional study of 56 acute lymphocytic leukemia survivors, chronological age between 15 and 24 years, assigned into two groups according to the exposure to cranial radiotherapy (25 irradiated and 31 non-irradiated), assessed according to body fat (dual energy X-ray absorptiometry), computed tomography scan-derived abdominal adipose tissue, lipid profile, and insulin resistance. Cranial radiotherapy increased body fat and abdominal adipose tissue and altered lipid panel. Yet, lipids showed no clinical relevance so far. There were significantly more obese patients among those who received cranial radiotherapy (52% irradiated versus 22.6% non-irradiated), based on dual energy X-ray absorptiometry body fat measurements. Nonetheless, no association was observed between cranial radiotherapy and body mass index, waist circumference, waist-to-height ratio or insulin resistance. Adolescent and young adult survivors of childhood acute lymphocytic leukemia showed an increase in body fat and an alteration of fat distribution, which were related to cranial radiotherapy. Fat compartment modifications possibly indicate a disease of adipose tissue, and cranial radiotherapy imports in this process

  18. Studying Biomarkers in Samples From Younger Patients With Acute Myeloid Leukemia

    2016-05-17

    Childhood Acute Monoblastic Leukemia (M5a); Childhood Acute Monocytic Leukemia (M5b); Childhood Acute Myeloblastic Leukemia Without Maturation (M1); Childhood Acute Myeloid Leukemia/Other Myeloid Malignancies; Childhood Acute Myelomonocytic Leukemia (M4)

  19. Acute childhood morbidities in rural Wardha : Some epidemiological correlates and health care seeking

    Deshmukh P

    2009-08-01

    (87.5% sought health care, where majority (66.1% received treatment from private clinics. The final model suggested that anemia and mother′s poor educational status are predictors of childhood morbidity. Conclusions: Nutritional anemia and mother′s poor educational status are the most important risk factors of acute childhood morbidity. There is need to revitalize existing health care delivery and child health programs in rural India with emphasis on immediate correction of nutritional anemia.

  20. Polymorphisms in the ABCB1 gene and effect on outcome and toxicity in childhood acute lymphoblastic leukemia

    Gregers, J; Gréen, H; Christensen, I J;

    2015-01-01

    The membrane transporter P-glycoprotein, encoded by the ABCB1 gene, influences the pharmacokinetics of anti-cancer drugs. We hypothesized that variants of ABCB1 affect outcome and toxicity in childhood acute lymphoblastic leukemia (ALL). We studied 522 Danish children with ALL, 93% of all those e...

  1. Outcome of treatment in childhood acute lymphoblastic leukaemia with rearrangements of the 11q23 chromosomal region

    Pui, CH; Gaynon, PS; Boyett, JM; Chessells, JM; Baruchel, A; Kamps, W; Silverman, LB; Biondi, A; Harms, DO; Vilmer, E; Schrappe, M; Camitta, B

    2002-01-01

    Background The prognosis and optimum treatment of childhood acute lymphoblastic leukaemia (ALL) with abnormalities of chromosomal band 11q23 are controversial. We aimed to identify prognostic factors that might help in planning future therapy, and to assess the effectiveness of haemopoietic stem-cel

  2. Mercaptopurine metabolite levels are predictors of bone marrow toxicity following high-dose methotrexate therapy of childhood acute lymphoblastic leukaemia

    Vang, Sophia Ingeborg; Schmiegelow, Kjeld; Frandsen, Thomas;

    2015-01-01

    High-dose methotrexate (HD-MTX) courses with concurrent oral low-dose MTX/6-mercaptopurine (6MP) for childhood acute lymphoblastic leukaemia (ALL) are often followed by neutro- and thrombocytopenia necessitating treatment interruptions. Plasma MTX during HD-MTX therapy guides folinic acid rescue ...

  3. Guidelines on dermatomyositis - excerpt from the interdisciplinary S2k guidelines on myositis syndromes by the German Society of Neurology.

    Sunderkötter, Cord; Nast, Alexander; Worm, Margitta; Dengler, Reinhard; Dörner, Thomas; Ganter, Horst; Hohlfeld, Reinhard; Melms, Arthur; Melzer, Nico; Rösler, Kai; Schmidt, Jens; Sinnreich, Michael; Walter, Maggi C; Wanschitz, Julia; Wiendl, Heinz

    2016-03-01

    The present guidelines on dermatomyositis (DM) represent an excerpt from the interdisciplinary S2k guidelines on myositis syndromes of the German Society of Neurology (available at www.awmf.org). The cardinal symptom of myositis in DM is symmetrical proximal muscle weakness. Elevated creatine kinase, CRP or ESR as well as electromyography and muscle biopsy also provide important diagnostic clues. Pharyngeal, respiratory, cardiac, and neck muscles may also be affected. Given that approximately 30 % of patients also develop interstitial lung disease, pulmonary function tests should be part of the diagnostic workup. Although the cutaneous manifestations in DM are variable, taken together, they represent a characteristic and crucial diagnostic criterion for DM. Approximately 5-20 % of individuals exhibit typical skin lesions without any clinically manifest muscle involvement (amyopathic DM). About 30 % of adult DM cases are associated with a malignancy. This fact, however, should not delay the treatment of severe myositis. Corticosteroids are the therapy of choice in myositis (1-2 mg/kg). Additional immunosuppressive therapy is frequently required (azathioprine, for children methotrexate). In case of insufficient therapeutic response, the use of intravenous immunoglobulins is justified. The benefit of rituximab has not been conclusively ascertained yet. Acute therapeutic management is usually followed by low-dose maintenance therapy for one to three years. Skin lesions do not always respond sufficiently to myositis therapy. Effective treatment for such cases consists of topical corticosteroids and sometimes also calcineurin inhibitors. Systemic therapies shown to be effective include antimalarial agents (also in combination), methotrexate, and corticosteroids. Intravenous immunoglobulins or rituximab may also be helpful. UV protection is an important prophylactic measure. PMID:26972210

  4. TESTIN Induces Rapid Death and Suppresses Proliferation in Childhood B Acute Lymphoblastic Leukaemia Cells.

    Robert J Weeks

    Full Text Available Childhood acute lymphoblastic leukaemia (ALL is the most common malignancy in children. Despite high cure rates, side effects and late consequences of the intensive treatments are common. Unquestionably, the identification of new therapeutic targets will lead to safer, more effective treatments. We identified TES promoter methylation and transcriptional silencing as a very common molecular abnormality in childhood ALL, irrespective of molecular subtype. The aims of the present study were to demonstrate that TES promoter methylation is aberrant, to determine the effects of TES re-expression in ALL, and to determine if those effects are mediated via TP53 activity.Normal fetal and adult tissue DNA was isolated and TES promoter methylation determined by Sequenom MassARRAY. Quantitative RT-PCR and immunoblot were used to confirm re-expression of TES in ALL cell lines after 5'-aza-2'-deoxycytidine (decitabine exposure or transfection with TES expression plasmids. The effects of TES re-expression on ALL cells were investigated using standard cell proliferation, cell death and cell cycle assays.In this study, we confirm that the TES promoter is unmethylated in normal adult and fetal tissues. We report that decitabine treatment of ALL cell lines results in demethylation of the TES promoter and attendant expression of TES mRNA. Re-expression of TESTIN protein in ALL cells using expression plasmid transfection results in rapid cell death or cell cycle arrest independent of TP53 activity.These results suggest that TES is aberrantly methylated in ALL and that re-expression of TESTIN has anti-leukaemia effects which point to novel therapeutic opportunities for childhood ALL.

  5. High white blood cell count at diagnosis of childhood acute lymphoblastic leukaemia

    Vaitkeviciene, Goda; Forestier, Erik; Hellebostad, Marit;

    2011-01-01

    Prognostic impact of peripheral blood white blood cell count (WBC) at the diagnosis of childhood acute lymphoblastic leukaemia (ALL) was evaluated in a population-based consecutive series of 2666 children aged 1-15 treated for ALL between 1992 and 2008 in the five Nordic countries (Denmark, Finland......, Iceland, Norway and Sweden). Ten-year event-free (pEFS(10 y)) survival and overall (pOS(10 y)) survival were 0.75 ± 0.01 and 0.85 ± 0.01, respectively. Although treatment intensity was determined by WBC, non-remission and relapsed patients still had significantly higher WBC than those in remission for B-cell...

  6. Comparison of three methods of central-nervous-system prophylaxis in childhood acute lymphoblastic leukaemia

    A retrospective comparison was made of three methods of central-nervous-system prophylaxis in childhood acute lymphoblastic leukaemia; (1) intrathecal methotrexate only; (2) intermediate-dose methotrexate infusion and intrathecal methotrexate and; (3) 2400 rads cranial irradiation and intrathecal methotrexate. The incidence of primary meningeal relapse was statistically significantly lower in both standard-risk patients (age > 24 months and 120 months and/or white-cell count > 20 000) whose central-nervous-system prophylaxis included cranial irradiation. The disease-free and overall survival of irradiated increased-risk patients was significantly better than that of unirradiated increased-risk patients. The disease-free survival of standard-risk patients who received intermediate-dose methotrexate was statistically superior to that of the remaining standard-risk patients. There were no significant differences in overall survival between the three groups of standard-risk patients. (author)

  7. Health-related quality of life assessment in Indonesian childhood acute lymphoblastic leukemia

    Sutaryo

    2008-11-01

    Full Text Available Abstract Background Most studies on Health-related Quality of Life (HRQOL in children with cancer were conducted in developed countries. The aims of this study were to assess the HRQOL in childhood acute lymphoblastic leukemia (ALL patients in Indonesia and to assess the influence of demographic and medical characteristics on HRQOL. Methods After cultural linguistic validation, a cross-sectional study of HRQOL was conducted with childhood ALL patients and their guardians in various phases of treatment using the Pediatric Quality of Life Inventory™ (PedsQL™ 4.0 Generic Core Scale and the Pediatric Quality of Life Inventory™ (PedsQL™ 3.0 Cancer Module. Results Ninety-eight guardians and 55 patients participated. The internal consistency of both scales ranged from 0.57 to 0.92. HRQOL of Indonesian patients was comparable with those in developed countries. There were moderate to good correlations between self-reports and proxy-reports, however guardians tended to report worse HRQOL than patients. Children of the 2–5 year-group significantly had more problems in procedural anxiety, treatment anxiety and communication subscales than in older groups (p Conclusion Younger children had more problems in procedural anxiety, treatment anxiety and communication subscales. Therefore, special care during intervention procedures is needed to promote their normal development. Psychosocial support should be provided to children and their parents to facilitate their coping with disease and its treatment.

  8. Pre-hospital care seeking behaviour for childhood acute respiratory infections in south-western Nigeria.

    Ukwaja, Kingsley N; Talabi, Ademola A; Aina, Olufemi B

    2012-12-01

    WHO/UNICEF currently recommend that childhood malaria and pneumonia be managed together in the community; most African countries are in the process of developing this policy. We conducted a cross-sectional study to determine maternal awareness of general danger signs of childhood illnesses and the prevalence, determinants and sources of pre-hospital treatment by mothers during their child's acute respiratory illness in a poor urban community in south-western Nigeria. A total of 226 mothers were interviewed. Only 4.9% of the mothers were aware of the two pneumonia symptoms: difficult breathing and fast breathing. About 75% of the children were given pre-hospital medication at home and only 16.5% of them received the drugs within 24 hour of symptom recognition. Drug shops/patent medicine vendors (PMVs; 70.6%) were the most common source of care. Wishing to try home management first (46.6%); waiting for the child to improve (14.4%) and lack of money (31.6%) delayed care-seeking. Older maternal age (aOR 2.3; 95% CI 1.2-4.4) and having a child with cough and difficult and/or fast breathing (aOR 2.3; 95% CI 1.1-5.2) were positive predictors of pre-hospital treatment. Maternal education and adequately equipping PMVs could improve prompt access to integrated community-based child health services in Nigeria. PMID:24029675

  9. Acute myocardial ischemia in adults secondary to missed Kawasaki disease in childhood.

    Rizk, Sherif R Y; El Said, Galal; Daniels, Lori B; Burns, Jane C; El Said, Howaida; Sorour, Khaled A; Gharib, Soliman; Gordon, John B

    2015-02-15

    Coronary artery aneurysms that occur in 25% of untreated Kawasaki disease (KD) patients may remain clinically silent for decades and then thrombose resulting in myocardial infarction. Although KD is now the most common cause of acquired heart disease in children in Asia, the United States, and Western Europe, the incidence of KD in Egypt is unknown. We tested the hypothesis that young adults in Egypt presenting with acute myocardial ischemia may have coronary artery lesions because of KD in childhood. We reviewed a total of 580 angiograms of patients ≤40 years presenting with symptoms of myocardial ischemia. Coronary artery aneurysms were noted in 46 patients (7.9%), of whom 9 presented with myocardial infarction. The likelihood of antecedent KD as the cause of the aneurysms was classified as definite (n = 10), probable (n = 29), or equivocal (n = 7). Compared with the definite and probable groups, the equivocal group had more traditional cardiovascular risk factors, smaller sized aneurysms, and fewer coronary arteries affected. In conclusion, in a major metropolitan center in Egypt, 6.7% of adults aged ≤40 years who underwent angiography for evaluation of possible myocardial ischemia had lesions consistent with antecedent KD. Because of the unique therapeutic challenges associated with these lesions, adult cardiologists should be aware that coronary artery aneurysms in young adults may be because of missed KD in childhood. PMID:25555655

  10. Intracellular Signaling Pathways Involved in Childhood Acute Lymphoblastic Leukemia; Molecular Targets.

    Layton Tovar, Cristian Fabián; Mendieta Zerón, Hugo

    2016-06-01

    Acute lymphoblastic leukemia (ALL) is a malignant disease characterized by an uncontrolled proliferation of immature lymphoid cells. ALL is the most common hematologic malignancy in early childhood, and it reaches peak incidence between the ages of 2 and 3 years. The prognosis of ALL is associated with aberrant gene expression, in addition to the presence of numerical or structural chromosomal alterations, age, race, and immunophenotype. The Relapse rate with regard to pharmacological treatment rises in childhood; thus, the expression of biomarkers associated with the activation of cell signaling pathways is crucial to establish the disease prognosis. Intracellular pathways involved in ALL are diverse, including Janus kinase/Signal transducers and transcription activators (JAK-STAT), Phosphoinositide-3-kinase-protein kinase B (PI3K-AKT), Ras mitogen-activated protein kinase (Ras-MAPK), Glycogen synthase kinase-3β (GSK-3β), Nuclear factor-kappa beta (NF-κB), and Hypoxia-inducible transcription factor 1α (HIF-1α), among others. In this review, we present several therapeutic targets, intracellular pathways, and molecular markers that are being studied extensively at present. PMID:27065575

  11. [Markers of metabolic syndrome and peptides regulating metabolism in survivors of childhood acute lymphoblastic leukemia].

    Skoczeń, Szymon; Tomasik, Przemysław; Balwierz, Walentyna; Surmiak, Marcin; Sztefko, Krystyna; Galicka-Latała, Danuta

    2011-01-01

    Along with the growing epidemic of overweight the risk of atherosclerosis, cardiovascular disease morbidity and mortality are increasing markedly. Metabolic syndrome (MS) is a condition clustering together several risk factors of those complications such as visceral obesity, glucose intolerance, arterial hypertension and dislipidemia. The risk of obesity in acute lymphoblastic leukemia (ALL) survivors is higher than in general population. We aimed to assess (1) the relationships between chosen adipokines and neuropeptides, chemotherapy, CRT, and body fatness and (2) evaluate adipokines and neuropeptides concentrations as a new markers of MS in children. We conducted cross-sectional evaluation of 82 ALL survivors (median age: 13.2 years; range: 4,8-26,2; median time from treatment: 3.2 years), including fasting laboratory testing: peptides (leptin, GLP-1, orexin, PYY, apelin), total cholesterol and its fractions, triglycerides; anthropometric measurements (weight, height), systolic and diastolic blood pressure. We estimated percentiles of body mass index and percentiles of blood pressure. Between 82 survivors overweight and diastolic hypertension was diagnosed in 31% of patients (35% in CRT group) and 15% respectively. At least one abnormality in lipids concentrations was found in 43%. Girls were more affected than boys. Statistically significant increased in leptin and apelin concentrations and decreased in soluble leptin receptor concentrations in the overweight group were observed compared to the non overweight subjects. Significant increase in orexin levels in females who had received CRT compared to those who had not received CRT was found. CRT is the main risk factor of elevated of body mass among survivors of childhood leukemia. Dyslipidemia and hypertension, along with increased adiposity indicate higher risk of MS development. Girls are more affected than boys. Leptin, orexin and apelin seem to be good markers of increased adiposity especially after CRT

  12. Late thyroid complications in survivors of childhood acute leukemia. An L.E.A. study

    Oudin, Claire; Auquier, Pascal; Bertrand, Yves; Chastagner, Philippe; Kanold, Justyna; Poirée, Maryline; Thouvenin, Sandrine; Ducassou, Stephane; Plantaz, Dominique; Tabone, Marie-Dominique; Dalle, Jean-Hugues; Gandemer, Virginie; Lutz, Patrick; Sirvent, Anne; Villes, Virginie; Barlogis, Vincent; Baruchel, André; Leverger, Guy; Berbis, Julie; Michel, Gérard

    2016-01-01

    Thyroid complications are known side effects of irradiation. However, the risk of such complications in childhood acute leukemia survivors who received either central nervous system irradiation or hematopoietic stem cell transplantation is less described. We prospectively evaluated the incidence and risk factors for thyroid dysfunction and tumors in survivors of childhood acute myeloid or lymphoid leukemia. A total of 588 patients were evaluated for thyroid function, and 502 individuals were assessed for thyroid tumors (median follow-up duration: 12.6 and 12.5 years, respectively). The cumulative incidence of hypothyroidism was 17.3% (95% CI: 14.1–21.1) and 24.6% (95% CI: 20.4–29.6) at 10 and 20 years from leukemia diagnosis, respectively. Patients who received total body irradiation (with or without prior central nervous system irradiation) were at higher risk of hypothyroidism (adjusted HR: 2.87; P=0.04 and 2.79, P=0.01, respectively) as compared with transplanted patients who never received any irradiation. Patients transplanted without total body irradiation who received central nervous system irradiation were also at higher risk (adjusted HR: 3.39; P=0.02). Patients irradiated or transplanted at older than 10 years of age had a lower risk (adjusted HR: 0.61; P=0.02). Thyroid malignancy was found in 26 patients (5.2%). Among them, two patients had never received any type of irradiation: alkylating agents could also promote thyroid cancer. The cumulative incidence of thyroid malignancy was 9.6% (95% CI: 6.0–15.0) at 20 years. Women were at higher risk than men (adjusted HR: 4.74; P=0.002). In conclusion, thyroid complications are frequent among patients who undergo transplantation after total body irradiation and those who received prior central nervous system irradiation. Close monitoring is thus warranted for these patients. Clinicaltrials.gov identifier: NCT 01756599. PMID:26969082

  13. Late thyroid complications in survivors of childhood acute leukemia. An L.E.A. study.

    Oudin, Claire; Auquier, Pascal; Bertrand, Yves; Chastagner, Philippe; Kanold, Justyna; Poirée, Maryline; Thouvenin, Sandrine; Ducassou, Stephane; Plantaz, Dominique; Tabone, Marie-Dominique; Dalle, Jean-Hugues; Gandemer, Virginie; Lutz, Patrick; Sirvent, Anne; Villes, Virginie; Barlogis, Vincent; Baruchel, André; Leverger, Guy; Berbis, Julie; Michel, Gérard

    2016-06-01

    Thyroid complications are known side effects of irradiation. However, the risk of such complications in childhood acute leukemia survivors who received either central nervous system irradiation or hematopoietic stem cell transplantation is less described. We prospectively evaluated the incidence and risk factors for thyroid dysfunction and tumors in survivors of childhood acute myeloid or lymphoid leukemia. A total of 588 patients were evaluated for thyroid function, and 502 individuals were assessed for thyroid tumors (median follow-up duration: 12.6 and 12.5 years, respectively). The cumulative incidence of hypothyroidism was 17.3% (95% CI: 14.1-21.1) and 24.6% (95% CI: 20.4-29.6) at 10 and 20 years from leukemia diagnosis, respectively. Patients who received total body irradiation (with or without prior central nervous system irradiation) were at higher risk of hypothyroidism (adjusted HR: 2.87; P=0.04 and 2.79, P=0.01, respectively) as compared with transplanted patients who never received any irradiation. Patients transplanted without total body irradiation who received central nervous system irradiation were also at higher risk (adjusted HR: 3.39; P=0.02). Patients irradiated or transplanted at older than 10 years of age had a lower risk (adjusted HR: 0.61; P=0.02). Thyroid malignancy was found in 26 patients (5.2%). Among them, two patients had never received any type of irradiation: alkylating agents could also promote thyroid cancer. The cumulative incidence of thyroid malignancy was 9.6% (95% CI: 6.0-15.0) at 20 years. Women were at higher risk than men (adjusted HR: 4.74; P=0.002). In conclusion, thyroid complications are frequent among patients who undergo transplantation after total body irradiation and those who received prior central nervous system irradiation. Close monitoring is thus warranted for these patients. Clinicaltrials.gov identifier: NCT 01756599. PMID:26969082

  14. Extraocular myositis in a female puppy

    O. Adegboye

    2015-04-01

    Full Text Available Extraocular myositis (EOM is not commonly encountered in dogs. It is generally diagnosed based on clinical features of exophthalmos without third eyelid protrusion, pain or vision loss. The traditional treatment of choice is prednisolone. This report describes a case of a mixed-breed puppy with clinical signs consistent with EOM, the use of ascorbic acid as an adjuvant to traditional corticosteroid therapy and rapid resolution of the condition without recurrence. It also shows that prolapse of the third eyelid and ptosis of the lower eyelids are possible signs of EOM during recovery. This is the first report of this sort from Africa and therefore the report is of epidemiological significance.

  15. The Eleventh International Childhood Acute Lymphoblastic Leukemia Workshop Report: Ponte di Legno, Italy, 6-7 May 2009

    Biondi, A; Baruchel, A; Hunger, S;

    2009-01-01

    An international childhood acute lymphoblastic leukemia (ALL)working group was formed during the 27th annual meeting of the International Society of Pediatric Oncology in 1995. Since then, 10 workshops have been held to address many issues that help advance treatment outcome of childhood ALL but...... require international collaboration (Table 1). The group was fondly named after 'Ponte di Legno,' a place in Lombardy, Italy, because the first major workshop was held there. In celebration of the 10th anniversary of the first major meeting, the group returned to Ponte di Legno on 6 and 7 May 2009 for its...

  16. The impact of CYP3A5*3 on risk and prognosis in childhood acute lymphoblastic leukemia

    Borst, Louise; Wallerek, Sandra; Dalhoff, Kim Peder; Rasmussen, Kirsten K; Wesenberg, Finn; Skov Wehner, Peder; Schmiegelow, Kjeld

    2011-01-01

    Objectives:  Acute lymphoblastic leukemia (ALL) is the most common cancer in childhood; however, little is known of the molecular etiology and environmental exposures causing the disease. Cytochrome P450 3A5 (CYP3A5) plays a crucial role in the catalytic oxidation of endogenous metabolites and...... of experiencing an event was almost eight times higher compared to those having at least one A allele (P = 0.045, hazard ratio = 7.749; 95% CI, 1.044-57.52). Conclusions:  This study shows that genetics may play a role in the risk of developing childhood ALL and indicates that improved treatment...

  17. The impact of CYP3A5*3 on risk and prognosis in childhood acute lymphoblastic leukemia

    Borst, Louise; Wallerek, Sandra; Dalhoff, Kim; Rasmussen, Kirsten; Wesenberg, Finn; Wehner, Peder Skov; Schmiegelow, Kjeld

    2011-01-01

    Objectives: Acute lymphoblastic leukemia (ALL) is the most common cancer in childhood; however, little is known of the molecular etiology and environmental exposures causing the disease. Cytochrome P450 3A5 (CYP3A5) plays a crucial role in the catalytic oxidation of endogenous metabolites and toxic...... experiencing an event was almost eight times higher compared to those having at least one A allele (P = 0.045, hazard ratio = 7.749; 95% CI, 1.044-57.52). Conclusions: This study shows that genetics may play a role in the risk of developing childhood ALL and indicates that improved treatment stratification of...

  18. The Impact of Childhood Acute Otitis Media on Parental Quality of Life in a Prospective Observational Cohort Study

    Holl, Katsiaryna; Rosenlund, Mats; Giaquinto, Carlo; Silfverdal, Sven-Arne; Carmona, Alfonso; Larcombe, James; Garcia-Sicilia, Jose; Fuat, Ahmet; Eulalia Munoz, Maria; Luisa Arroba, Maria; Sloesen, Brigitte; Vollmar, Jens; Pircon, Jean-Yves; Liese, Johannes G

    2015-01-01

    Background and Objectives Acute otitis media (AOM) not only affects childhood quality of life (QoL), but can also affect parental QoL. We adapted a previously published questionnaire on the effect of childhood recurrent ear, nose and throat infections on parental QoL for use with AOM and used it in an observational, multicentre, prospective study of children with AOM. Methods The AOM-specific parental QoL questionnaire grouped 15 items into emotional, daily disturbance, total and overall pare...

  19. Osteomyelitis of Myositis Ossificans in Arm – First Case Report

    Mishra, Pankaj Kumar; Singhal, Prakhar; Shukla, Jiten; Maravi, Deepak Singh

    2014-01-01

    Introduction: Myositis ossificans [MO] is a benign heterotropic bone forming (often- self resolving) pathology of bone and soft tissue. Here we are reporting the first time in literature for osteomyelitis of myositis ossificans in arm of a male due to trauma as a perusal of rare entity. Case Report: It is a case report of a 25 years old male presented to us in out-patient department with chief complaint of discharging wounds over mid part of left arm since six months. Clinically provisional diagnosis of chronic osteomylitis of left humerus made and his x-ray sought. X- Ray showed geographic appearance of myositis ossificans around the upper two third of left arm. Sinuses curetted and infected bone (part of myositis ossificans) removed and sent for biopsy. Now the patient is discharge and sinus free, and has resumed his work. Conclusion: Osteomyleitis of myositis ossificans should be recognized as a possible differential diagnosis chronic discharging sinus. This type of presentation of myositis ossificans is rarest. PMID:27299005

  20. Allergy and risk of childhood acute lymphoblastic leukemia: a population-based and record-based study.

    Chang, Jeffrey S; Tsai, Yi-Wen; Tsai, Chia-Rung; Wiemels, Joseph L

    2012-12-01

    A deficit of normal immune stimulation in early childhood is a suspected risk factor for both childhood acute lymphoblastic leukemia (ALL) and allergies. The present study utilized a population-based case-control design using medical claims data from the National Health Insurance Research Database of Taiwan to evaluate the association between allergy and childhood leukemia. Eight hundred forty-six childhood ALL patients who were newly diagnosed during 2000 to 2008 and were older than 1 but less than 10 years of age were individually matched with 3,374 controls based on sex, birth date, and time of diagnosis (reference date for the controls). Conditional logistic regression was performed to assess the association between childhood ALL and allergies. An increased risk of ALL was observed with having an allergy less than 1 year before the case's ALL diagnosis (odds ratio (OR) = 1.7, 95% confidence interval (CI): 1.5, 2.0), more than 1 year before the case's diagnosis (OR = 1.3, 95% CI: 1.1, 1.5), and before the age of 1 year (OR = 1.4, 95% CI: 1.1, 1.7). These results suggest that the pathogenesis of childhood ALL and allergy share a common biologic mechanism. PMID:23171876

  1. Initial Management of Childhood Acute Immune Thrombocytopenia: Single-Center Experience of 32 Years.

    Yildiz, Inci; Ozdemir, Nihal; Celkan, Tiraje; Soylu, Selen; Karaman, Serap; Canbolat, Aylin; Dogru, Omer; Erginoz, Ethem; Apak, Hilmi

    2015-01-01

    Immune thrombocytopenia (ITP) is an acute self-limited disease of childhood, mostly resolving within 6 months irrespective of whether therapy is given or not. Treatment options when indicated include corticosteroids, intravenous immune globulin (IVIG), and anti-RhD immunoglobulin. We reviewed our 32 years' experience for first-line therapy of acute ITP. Five hundred forty-one children (mean age: 5.3 years) diagnosed and treated for ITP were evaluated retrospectively. Among 491 acute ITP patients, IVIG was used in 27%, high-dose steroids in 27%, low-dose steroids in 20%, anti-D immunoglobulin G (IgG) in 2%, and no therapy in 22%. When the initial response (platelets >50 × 10(9)/L) to first-line treatment modalities were compared, 89%, 84%, and 78% patients treated by low-dose steroids, high-dose steroids, and IVIG responded to treatment, respectively (P > .05). Mean time to recovery of platelets was 16.8, 3.8, and 3.0 days in patients treated with low-dose steroids, high-dose steroids, and IVIG, respectively (P < .0001). Thrombocytopenia recurred in 23% of low-dose steroid, 39% of high-dose steroid, and in 36% of IVIG (P < .0001) treatment groups. Of 108 patients who were observed alone, 4 (3%) had a recurrence on follow-up and only 2 of these required treatment subsequently. Recurrence was significantly less in no therapy group compared with children treated with 1 of the 3 options of pharmacotherapy (P < .0001). Response rates were similar between patients treated by IVIG and low- and high-dose steroids; however, time to response was slower in patients treated with low-dose steroids compared with IVIG and high-dose steroids. PMID:26154620

  2. FLT3 and NPM1 gene mutations in childhood acute myeloblastic leukemia.

    Mukda, Ekchol; Pintaraks, Katsarin; Sawangpanich, Rachchadol; Wiangnon, Surapon; Pakakasama, Samart

    2011-01-01

    Mutations of receptor tyrosine kinases are implicated in the constitutive activation and development of human hematologic malignancies. Mutations in fms-like tyrosine kinase 3 (FLT3) gene including internal tandem duplication (ITD) and point mutation in the tyrosine kinase domain (TKD) as well as in nucleoplasmin (NPM1) gene are associated with pathogenesis of acute myeloblastic leukemia (AML). Several reports have demonstrated high incidences of the FLT3 and NPM1 mutations in adult AML patients. Since the pathogenesis of pediatric AML is different from that of adult and the FLT3 and NPM1 mutations have not been well characterized in childhood AML. Therefore, the objective of this study was to determine the frequencies of FLT3 and NPM1 mutations in 64 newly diagnosed childhood AML patients. All blood and bone marrow samples were previously diagnosed with AML by using flow cytometry and/or cytochemistry. FLT3-ITD and FLT3-TKD were detected by PCR and PCR-RFLP methods, respectively. The NPM1 mutation was analyzed by PCR and direct DNA sequencing. The FLT3 mutations were detected in 7 of 64 (11.1%), including FLT3-ITD in 4 of 64 (6.3%) and FLT-TKD in 3 of 62 (4.8%). The NPM1 mutation was not detected in this cohort. By multivariate analysis, white blood cell counts, peripheral blood and bone marrow blast cell counts at diagnosis were significantly higher in children with FLT3-ITD (P<0.05). In addition, the median percentage of CD117 was significantly higher in leukemic blast cells with FLT3-ITD than those with wild type (P=0.01). We did not find any FLT3 mutations in children aged less than 5 years. The AML M3 cell type was most frequently associated with FLT3 gene mutations (50%). In conclusion, the FLT3 mutations was found in 11.1% but none of NPM1 mutation was detected in Thai children with AML. These data support the hypothesis of different biology and pathogenesis between adult and childhood AML. PMID:22126574

  3. DNA incorporation of 6-thioguanine nucleotides during maintenance therapy of childhood acute lymphoblastic leukaemia and non-Hodgkin lymphoma

    Hedeland, Rikke L; Hvidt, Kristian; Nersting, Jacob;

    2010-01-01

    To explore the DNA incorporation of 6-thioguanine nucleotide levels (DNA-6TGN) during 6-mercaptopurine (6MP) therapy of childhood acute lymphoblastic leukaemia (ALL) and non-Hodgkin lymphoma (NHL) and its relation to erythrocyte levels of their metabolites: 6-thioguanine-nucleotides (E-6TGN), met......), methylated metabolites (E-MeMP), Methotrexate polyglutamates (E-MTX), and to thiopurine methyltransferase activity (TPMT)....

  4. DNA incorporation of 6-thioguanine nucleotides during maintenance therapy of childhood acute lymphoblastic leukaemia and non-Hodgkin lymphoma

    Hedeland, Rikke L.; Hvidt, Kristian; Nersting, Jacob; Rosthøj, Susanne; Dalhoff, Kim; Lausen, Birgitte; Schmiegelow, Kjeld

    2009-01-01

    Abstract Purpose To explore the DNA incorporation of 6-thioguanine nucleotide levels (DNA-6TGN) during 6-mercaptopurine (6MP) therapy of childhood acute lymphoblastic leukaemia (ALL) and non-Hodgkin lymphoma (NHL) and its relation to erythrocyte levels of their metabolites: 6-thioguanine-nucleotides (E-6TGN), methylated metabolites (E-MeMP), Methotrexate polyglutamates (E-MTX), and to thiopurine methyltransferase activity (TPMT). ...

  5. BLOOD FLOW RESTRICTED RESISTANCE TRAINING ATTENUATES MYOSTATIN GENE EXPRESSION IN A PATIENT WITH INCLUSION BODY MYOSITIS

    2014-01-01

    Inclusion body myositis is a rare idiopathic inflammatory myopathy that produces extreme muscle weakness. Blood flow restricted resistance training has been shown to improve muscle strength and muscle hypertrophy in inclusion body myositis. Objective: The aim of this study was to evaluate the effects of a resistance training programme on the expression of genes related to myostatin (MSTN) signalling in one inclusion body myositis patient. Methods: A 65-year-old man with inclusion body myositi...

  6. Prelabor cesarean delivery and early-onset acute childhood leukemia risk.

    Thomopoulos, Thomas P; Skalkidou, Alkistis; Dessypris, Nick; Chrousos, George; Karalexi, Maria A; Karavasilis, Theodoros G; Baka, Margarita; Hatzipantelis, Emmanuel; Kourti, Maria; Polychronopoulou, Sophia; Sidi, Vasiliki; Stiakaki, Eftichia; Moschovi, Maria; Loutradis, Dimitrios; Petridou, Eleni Th

    2016-03-01

    The long-term impact of cesarean delivery (CD) on the health of the offspring is being explored methodically. We sought to investigate the effect of birth by (a) prelabor and (b) during-labor CD on the risk of early-onset (≤3 years) acute lymphoblastic leukemia (ALL), specifically of its prevailing precursor B (B-ALL) subtype. A total of 1099 incident cases of ALL (957 B-ALL), 131 of acute myeloid leukemia (AML), and their 1 : 1 age-matched and sex-matched controls, derived from the Nationwide Registry for Childhood Hematological Malignancies (1996-2013), were analyzed using multivariate regression models. A null association was found between prelabor and/or during labor CD and either ALL (B-ALL) or AML in the 0-14 age range. By contrast, birth by CD increased significantly the risk of early-onset ALL [odds ratioCD (ORCD)=1.57, 95% confidence interval (CI): 1.10-2.24] mainly on account of prelabor CD (ORprelaborCD=1.66, 95% CI: 1.13-2.43). The respective figures were even higher for the early-onset precursor B-ALL (ORCD=1.66, 95% CI: 1.15-2.40 and ORprelaborCD=1.79, 95% CI: 1.21-2.66), whereas no association emerged for early-onset AML. Prelabor CD, which deprives exposure of the fetus/infant to the presumably beneficial effect of stress hormones released in both vaginal labor and during labor CD, was associated exclusively with an increased risk of early-onset ALL, particularly the precursor B-ALL subtype. If confirmed, these adverse long-term outcomes of CD may point to re-evaluation of prelabor CD practices and prompt scientific discussion on the best ways to simulate the effects of vaginal delivery, such as a precesarean induction of labor. PMID:25793919

  7. Computed Tomography in the Diagnosis of Myositis Ossificans – Case Report

    The term myositis ossificans refers to the formation of ossifications in the muscles, ligaments and fascias, usually as a result of trauma. Fibrodysplasia ossificans progressiva is a rare genetic disease in which heterotopic ossifications appear in early childhood and are accompanied by feet and spine defects. We present a case of a 31-year-old woman with massive heterotopic ossifications who suffered multiple injuries. We would like to emphasize the role of computed tomography in the exact localization of ossifications. Thanks to the volume rendering techniques and 3D image reconstructions, it is possible to precisely determine the position of ossifications in relation to the internal organs and blood vessels, allowing to schedule the surgery to remove the lesions

  8. Factors associated with IQ scores in long-term survivors of childhood acute lymphoblastic leukemia

    To identify factors which might be associated with intellectual function following treatment for childhood acute lymphoblastic leukemia, 50 long-term survivors were studied using the Wechsler Intelligence Scale for Children-Revised. All patients were diagnosed between 1972 and 1974 and were treated on a single clinical trial protocol with identical induction and maintenance chemotherapy plus central nervous system prophylaxis that included cranial radiation. The mean full scale IQ score for the group was 95 (SEM 2.0), with mean verbal IQ of 94.4 and mean performance IQ of 96.9. Factors which were found to be closely associated with a lower IQ score included female sex (in both verbal IQ and full-scale IQ), longer duration of chemotherapy (in performance IQ), and younger age at the time of radiation (in both verbal IQ and full-scale IQ). The age at the time of radiation was found to be significantly correlated with discrepancy between verbal and performance IQ, with younger age being associated with verbal IQ scores higher than performance IQ scores. When analyses were performed within specific subgroups of patients defined by sex and age at the time of radiation, dose of cranial radiation, concomitant intrathecal methotrexate therapy, and duration of therapy were all found to be correlated with a lower level of intellectual function. These preliminary findings provide direction for future studies to help identify high-risk patients

  9. Childhood acute disseminated encephalomyelitis: the role of brain and spinal cord MRI

    Background. It is recognised that the clinical and radiological spectrum of childhood acute disseminated encephalomyelitis (ADEM) is wide. Objective. To determine whether initial MRI features are predictive of clinical outcome and to determine the role of MRI in the management of ADEM. Materials and methods. The MRI scans of ten consecutive children (eight boys, two girls), clinically and radiologically diagnosed to have ADEM, were retrospectively reviewed. Follow-up MRI was available for eight patients. Results. Lesions ranged from small and punctate (<1 cm) to moderate sized and confluent (4-5 cm) to diffuse and extensive. Spinal cord lesions, seen in five of seven children, were contiguous or segmental. Seven children (70%) made good clinical recovery while three children (30%) remained severely handicapped. There was no correlation between the site, extent and pattern of involvement and clinical outcome. However, the evolution of MRI findings on follow-up correlated well with the subsequent clinical course and outcome. Conclusions. Although the extent and site of lesions on initial MRI scans are not predictive of clinical outcome, early MRI of the brain and spine is useful in aiding clinical diagnosis, and subsequent follow-up MRI is helpful in monitoring disease progression. (orig.)

  10. Factors associated with IQ scores in long-term survivors of childhood acute lymphoblastic leukemia

    Robison, L.L.; Nesbit, M.E. Jr.; Sather, H.N.; Meadows, A.T.; Ortega, J.A.; Hammond, G.D.

    To identify factors which might be associated with intellectual function following treatment for childhood acute lymphoblastic leukemia, 50 long-term survivors were studied using the Wechsler Intelligence Scale for Children-Revised. All patients were diagnosed between 1972 and 1974 and were treated on a single clinical trial protocol with identical induction and maintenance chemotherapy plus central nervous system prophylaxis that included cranial radiation. The mean full scale IQ score for the group was 95 (SEM 2.0), with mean verbal IQ of 94.4 and mean performance IQ of 96.9. Factors which were found to be closely associated with a lower IQ score included female sex (in both verbal IQ and full-scale IQ), longer duration of chemotherapy (in performance IQ), and younger age at the time of radiation (in both verbal IQ and full-scale IQ). The age at the time of radiation was found to be significantly correlated with discrepancy between verbal and performance IQ, with younger age being associated with verbal IQ scores higher than performance IQ scores. When analyses were performed within specific subgroups of patients defined by sex and age at the time of radiation, dose of cranial radiation, concomitant intrathecal methotrexate therapy, and duration of therapy were all found to be correlated with a lower level of intellectual function. These preliminary findings provide direction for future studies to help identify high-risk patients.

  11. Neuropsychological sequelae of central nervous system prophylaxis in survivors of childhood acute lymphoblastic leukemia

    We assessed neuropsychologically 106 children with acute lymphoblastic leukemia (ALL) who had all received cranial irradiation for the prevention of central nervous system (CNS) leukemia 1-13 years previously. Children were assessed for adverse late effects of their therapy, using age-appropriate Wechsler measures of overall intellectual ability and supplementary tests. Forty-five siblings near in age to the patients were tested as controls. The patients who had had the most intensive central nervous system (CNS) prophylaxis were found to have a WISC-R Full Scale IQ 17 points lower than the sibling control group. Performance IQ was more affected than verbal IQ. The patients were more easily distracted and less able to concentrate. The severity of the aftereffects was related to younger age at the time of CNS prophylaxis and to a higher dose of cranial irradiation but not to time since CNS prophylaxis. CNS prophylaxis using a combination of cranial irradiation and intrathecal methotrexate has lowered the incidence of CNS relapse in childhood ALL but is associated with considerable long-term morbidity in survivors

  12. Current Strategies for the Detection of Minimal Residual Disease in Childhood Acute Lymphoblastic Leukemia

    Rocha, Juliana Maria Camargos; Xavier, Sandra Guerra; de Lima Souza, Marcelo Eduardo; Assumpção, Juliana Godoy; Murao, Mitiko; de Oliveira, Benigna Maria

    2016-01-01

    Acute lymphoblastic leukemia (ALL) is the most common cancer in children. Current treatment strategies for childhood ALL result in long-term remission for approximately 90% of patients. However, the therapeutic response is worse among those who relapse. Several risk stratification approaches based on clinical and biological aspects have been proposed to intensify treatment in patients with high risk of relapse and reduce toxicity on those with a greater probability of cure. The detection of residual leukemic cells (minimal residual disease, MRD) is the most important prognostic factor to identify high-risk patients, allowing redefinition of chemotherapy. In the last decades, several standardized research protocols evaluated MRD using immunophenotyping by flow cytometry and/or real-time quantitative polymerase chain reaction at different time points during treatment. Both methods are highly sensitive (10−3 a 10−5), but expensive, complex, and, because of that, require qualified staff and frequently are restricted to reference centers. The aim of this article was to review technical aspects of immunophenotyping by flow cytometry and real-time quantitative polymerase chain reaction to evaluate MRD in ALL. PMID:27158437

  13. Two cases of radiation myositis probably induced by recall phenomenon

    Two cases of radiation myositis following gemcitabine-based chemotherapy for advanced non-small cell lung cancer are presented. In the two cases, myositis appeared 3 and 5.5 months after the completion of radiation therapy and 2 and 2.5 months after the beginning of chemotherapy, respectively. The affected areas, the upper back and right thigh, appeared as areas of increased signal intensity on T2-weighted MR imaging and were enhanced by the administration of Gd-DTPA in the skeletal muscles. These coincided with the previously irradiated area. Although radiation myositis is a rare complication, it is important to be careful of radiation recall induced by chemotherapeutic agents. (author)

  14. Combination Chemotherapy in Treating Young Patients With Down Syndrome and Acute Myeloid Leukemia or Myelodysplastic Syndromes

    2016-03-16

    Childhood Acute Basophilic Leukemia; Childhood Acute Eosinophilic Leukemia; Childhood Acute Erythroleukemia (M6); Childhood Acute Megakaryocytic Leukemia (M7); Childhood Acute Minimally Differentiated Myeloid Leukemia (M0); Childhood Acute Monoblastic Leukemia (M5a); Childhood Acute Monocytic Leukemia (M5b); Childhood Acute Myeloblastic Leukemia With Maturation (M2); Childhood Acute Myeloblastic Leukemia Without Maturation (M1); Childhood Acute Myelomonocytic Leukemia (M4); Childhood Myelodysplastic Syndromes; de Novo Myelodysplastic Syndromes; Secondary Acute Myeloid Leukemia; Secondary Myelodysplastic Syndromes; Untreated Childhood Acute Myeloid Leukemia and Other Myeloid Malignancies

  15. The metabolic syndrome in survivors of childhood acute lymphoblastic leukemia in Isfahan, Iran

    Nahid Reisi

    2009-04-01

    Full Text Available

    • BACKGROUND: To determine the prevalence of metabolic syndrome in survivors of childhood leukemia in Isfahan, Iran.
    • METHODS: During a 4-year period (2003 to 2007, 55 children (33 male and 22 female diagnosed with ALL at Unit of Hematology/ Oncology, Department of Pediatrics, Isfahan University of Medical Science, were enrolled in this crosssectional study. Metabolic syndrome was defined using the modified version of Adult Treatment Panel (ATP III criteria. Insulin resistance was defined based on the homeostasis model assessment index (HOMA-IR.
    • RESULTS: The mean age of participates was 10.4 years (range 6-19 years and the mean interval since completion of chemotherapy was 35 months. Twenty percent (11/55 of survivors (10 male, 1 female met criteria for diagnosis of metabolic syndrome. Obesity was observed in one forth of patients and nearly 3/4 of obese patients had metabolic syndrome. High serum insulin levels were found in 16% of participants and in 63% of obese survivors. The mean insulin levels in survivors with metabolic syndrome was three-times more than those without (28.3 mu/l vs. 9.57 mu/l, p = 0.004. Insulin resistance was detected in 72.7% of survivors with metabolic syndrome and it was  ositively correlated with serum triglycerides (0.543, p < 0.001, systolic and diastolic BP (0.348, p = 0.01 and 0.368, p = 006 respectively, insulin levels (0.914, p < 0.001 and blood sugar (0.398, p = 003.
    • CONCLUSIONS: The prevalence of metabolic syndrome in survivors of childhood leukemia in Iran is higher than developed countries. Nearly all of the obese patients had metabolic syndrome. Weight control and regular physical exercise are recommended to the survivors.
    • KEYWORDS: Acute lymphoblastic leukemia, metabolic syndrome, obesity, children.

  16. Delayed Sciatic Nerve Injury Resulting From Myositis Ossificans Traumatica.

    Guan, Zhe; Wilson, Thomas J; Jacobson, Jon A; Hollon, Todd C; Yang, Lynda J-S

    2016-05-01

    A motorcyclist sustained multiple-system trauma, including a left buttock hematoma requiring decompression and evacuation. Presentation for severe hip pain and lower extremity weakness was delayed. Imaging revealed myositis ossificans traumatica compressing the sciatic nerve in the buttock. The patient underwent sciatic nerve decompression with resection of heterotopic calcification, resulting in improvement in pain and left lower extremity function. This case illustrates the contrast in differential diagnosis of peripheral nerve injury immediately posttrauma and that occurring in a slow, delayed fashion posttrauma. Myositis ossificans may be an underrecognized complication of trauma but should be considered in cases of delayed peripheral nerve injury after trauma. PMID:26548968

  17. Preferentially Expressed Antigen of Melanoma (PRAME and Wilms’ Tumor 1 (WT 1 Genes Expression in Childhood Acute Lymphoblastic Leukemia, Prognostic Role and Correlation with Survival

    Engy El Khateeb

    2015-03-01

    CONCLUSION: It is concluded that the expression of PRAME and WT1 genes are indicators of favorable prognosis and can be useful tools for monitoring minimal residual disease (MRD in acute leukemia especially in patients without known genetic markers. Differential expression between acute leukemia patients and healthy volunteers suggests that the immunogenic antigens (PRAME and WT1 are potential candidates for immunotherapy in childhood acute leukemia.

  18. Pattern of acute poisonings in childhood in Ankara: what has changed in twenty years?

    Andiran, Nesibe; Sarikayalar, Fikriye

    2004-01-01

    Poisoning represents one of the most common medical emergencies in childhood, and epidemiological properties differ from country to country. Thus, special epidemiological surveillance for each country is necessary to determine the problem according to which preventive measures can be taken. The purpose of this study was to clarify the characteristics of acute poisoning cases admitted to a pediatric referral hospital. All poisoned patients under 17 years of age, except for cases food poisoning, presenting to the Emergency Department (ED) from January 1995 to December 2000 were determined. The information about each case was recorded on standardized forms and a retrospective chart review survey was done. Complete epidemiological and clinical data were obtained for 489 patients. The mean age of all poisoned patients (mean +/- standard deviation) was 5.96 +/- 4.87 years, and the age range was 0.01 to 17 years. Three hundred and thirty-one children, forming 63.6% of all patients, were under five years of age. Slightly more boys (52.3%) than girls were intoxicated at ages less than 10 years, after which more girls (79%) than boys were involved. The majority of all cases were due to accidental poisoning (78.1% of all poisonings) which occurred mostly in children under five years of age (73.3%). While accidental poisonings (97.1%) were the most common mode of poisoning between 1-5 years, self-poisonings (67.3%) had the highest ratio in cases over 10 years of age. In patients younger than one year of age, 74.2% of all poisonings were due to therapeutical error. Drugs were the most frequent offending agent (57.7%), followed by ingestion of a caustic/corrosive substance (16.8%) and carbon monoxide (CO) intoxication (9.4%). Analgesics were the most common agents, forming 23.7% of all poisonings due to drugs, followed by ingestion of multiple drugs and tricyclic antidepressants at ratios of 21.6% and 9.6%, respectively. The most common route of poisoning was ingestion of the

  19. Different molecular mechanisms causing 9p21 deletions in acute lymphoblastic leukemia of childhood.

    Novara, Francesca; Beri, Silvana; Bernardo, Maria Ester; Bellazzi, Riccardo; Malovini, Alberto; Ciccone, Roberto; Cometa, Angela Maria; Locatelli, Franco; Giorda, Roberto; Zuffardi, Orsetta

    2009-10-01

    Deletion of chromosome 9p21 is a crucial event for the development of several cancers including acute lymphoblastic leukemia (ALL). Double strand breaks (DSBs) triggering 9p21 deletions in ALL have been reported to occur at a few defined sites by illegitimate action of the V(D)J recombination activating protein complex. We have cloned 23 breakpoint junctions for a total of 46 breakpoints in 17 childhood ALL (9 B- and 8 T-lineages) showing different size deletions at one or both homologous chromosomes 9 to investigate which particular sequences make the region susceptible to interstitial deletion. We found that half of 9p21 deletion breakpoints were mediated by ectopic V(D)J recombination mechanisms whereas the remaining half were associated to repeated sequences, including some with potential for non-B DNA structure formation. Other mechanisms, such as microhomology-mediated repair, that are common in other cancers, play only a very minor role in ALL. Nucleotide insertions at breakpoint junctions and microinversions flanking the breakpoints have been detected at 20/23 and 2/23 breakpoint junctions, respectively, both in the presence of recombination signal sequence (RSS)-like sequences and of other unspecific sequences. The majority of breakpoints were unique except for two cases, both T-ALL, showing identical deletions. Four of the 46 breakpoints coincide with those reported in other cases, thus confirming the presence of recurrent deletion hotspots. Among the six cases with heterozygous 9p deletions, we found that the remaining CDKN2A and CDKN2B alleles were hypermethylated at CpG islands. PMID:19484265

  20. The combination effects of bendamustine with antimetabolites against childhood acute lymphoblastic leukemia cells.

    Goto, Shoko; Goto, Hiroaki; Yokosuka, Tomoko

    2016-05-01

    Bendamustine combined with other drugs is clinically efficacious for some adult lymphoid malignancies, but to date there are no reports of the use of such combinatorial approaches in pediatric patients. We investigated the in vitro activity of bendamustine combined with other antimetabolite drugs on B cell precursor acute lymphoblastic leukemia (BCP-ALL) cell lines established from pediatric patients with refractory or relapsed ALL. We also developed a mathematically drown improved isobologram method to assess the data objectively. Three BCP-ALL cell lines; YCUB-2, YCUB-5, and YCUB-6, were simultaneously exposed to various concentrations of bendamustine and cladribine, cytarabine, fludarabine, or clofarabine. Cell growth inhibition was determined using the WST-8 assay. Combinatorial effects were estimated using our improved isobologram method with IC80 (drug concentration corresponding to 80 % of maximum inhibition). Bendamustine alone inhibited ALL cell growth with mean IC80 values of 11.30-18.90 μg/ml. Combinations of bendamustine with other drugs produced the following effects: (1) cladribine; synergistic-to-additive on all cell lines; (2) cytarabine; synergistic-to-additive on YCUB-5 and YCUB-6, and synergistic-to-antagonistic on YCUB-2; (3) fludarabine; additive-to-antagonistic on YCUB-5, and synergistic-to-antagonistic on YCUB-2 and YCUB-6; (4) clofarabine; additive-to-antagonistic on all cell lines. Flow cytometric analysis also showed the combination effects of bendamustine and cladribine. Bendamustine/cladribine or bendamustine/cytarabine may thus represent a promising combination for salvage treatment in childhood ALL. PMID:26886449

  1. Salivirus in Children and Its Association with Childhood Acute Gastroenteritis: A Paired Case-Control Study.

    Jie-Mei Yu

    Full Text Available Salivirus was recently discovered in children with gastroenteritis and in sewage. Though a causative role for salivirus in childhood gastroenteritis was suggested in the previous study, the relationship between salivirus and acute gastroenteritis has not yet been clearly clarified. The sewage strain reported by Ng, although represented by incomplete genome sequencing data, was distinct from previously reported saliviruses, and had not previously been detected in humans. A case-control study examining 461 paired stool samples from children with diarrhea and healthy controls (1:1 was conducted in this study. Also, common diarrheal viruses were detected and complete genome of a salivirus was determined. Results showed that salivirus was detected in 16 (3.5% and 13 (2.8% of the case and control samples, respectively; no differences in detection rates (p=0.571 or mean values of viral loads (p=0.400 were observed between the groups. Multivariate Cox regression revealed no association between salivirus and gastroenteritis (p=0.774. The data also demonstrated that salivirus infection did not exacerbate clinical symptoms of gastroenteritis in children. Furthermore, complete genome sequence of a salivirus recovered from the feces of a child with diarrhea (i.e., SaliV-FHB shared a 99% nucleotide identity with the sewage strain. In conclusion, a paired case-control study did not support a causative role for salivirus strains detected in this study with pediatric gastroenteritis. This study also demonstrated that all known saliviruses can be detected in the feces of children with or without gastroenteritis.

  2. Long-term sequelae after chemotherapy and radiotherapy for childhood acute lymphoblastic leukemia

    Background: Effective forms of treatment for acute lymphoblastic leukemia (ALL) in childhood now result in survival rates of more than 70%. With improving cure rates increasing interest has been focused on adverse late effects caused by chemotherapy and cranial irradiation. Methods: We investigated 40 survivors, 22 males and 18 females with an average age of 15.8 years (6.6 to 28.1), all treated at the Children's Hospital of Innsbruck, Austria, after a follow-up of 9.2 years (4.6 - 20) on average in continuous complete remission. Our evaluation included cardiac status, growth, endocrinological function and a wide variety of other clinical and laboratory investigations. To identify cardiac dysfunction due to anthracyclines we performed Dobutamine-Stress-Echocardiography (DSE) using a graded dosage regimen (1.0, 2.5 and 5.0 μg/kg/min). We compared the DSE data with those obtained from 17 age-matched control subjects. Results: Conventional echocardiography revealed only 4 patients (11.4%) with abnormalities of left ventricular contractility (measured as left ventricular shortening fraction). In contrast DSE detected a 3-fold higher percentage of patients with cardiac dysfunction. There was no correlation between total cumulative anthracycline dose, age at time of diagnosis and length of follow-up with incidence of abnormalities. Primary hypothyroidism in 4 patients (10%) and a permanent linear growth retardation in 5 patients (12%, all of which had been irradiated) were the only endocrinological problems detected. No survivor showed hemato-immunological disturbances. Remarkably, transfusion- associated sequelae, liver or kidney dysfunction were not found. Conclusion: The high incidence of late cardiac effects requires continued monitoring of patients after ALL treatment. In this respect, DSE revealed to be a sensitive method. (author)

  3. High Throughput Drug Sensitivity Assay and Genomics- Guided Treatment of Patients With Relapsed or Refractory Acute Leukemia

    2016-05-19

    Acute Leukemia of Ambiguous Lineage; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Acute Myeloid Leukemia; Recurrent Childhood Acute Lymphoblastic Leukemia; Recurrent Childhood Acute Myeloid Leukemia; Refractory Adult Acute Lymphoblastic Leukemia; Refractory Childhood Acute Lymphoblastic Leukemia

  4. Brucellosis presenting as piriformis myositis: a case report

    Romanos Odysseas

    2011-03-01

    Full Text Available Abstract Introduction Myositis is a rare bacterial muscle infection. Involvement of the piriformis muscle has been rarely reported in the literature. In this report we describe a case of piriformis myositis due to Brucella melitensis, which to the best of our knowledge is the first such case presented in the literature. Case presentation We report the case of a 19-year-old Caucasian man who presented to our institution with fever and right hip pain. Brucellosis was suspected, but the clinical suspicion was for spondylodiscitis. A pelvic magnetic resonance imaging scan allowed prompt diagnosis of inflammatory involvement of the right piriformis muscle. Blood culture results were positive for B. melitensis. Our patient was treated with antibiotics, and follow-up magnetic resonance imaging scans showed resolution of the inflammation. Conclusion Brucellosis can present as piriformis myositis. The clinical diagnosis of piriformis myositis is difficult, as it can mimic other common entities such as referred back pain from spondylodiscitis. Magnetic resonance imaging is the method of choice for establishing the diagnosis in the early stages of the disease, as late diagnosis can lead to abscess formation and the need for drainage.

  5. Management of a femoral fracture complicated by clostridial myositis

    A clinical case of clostridial myositis secondary to a comminuted femoral fracture is described. This case is unusual because, despite the severe degree of obvious muscle necrosis and gas production, the dog had minimal signs of systemic toxicity. Union of the fracture was achieved but six months postoperatively muscular contracture had resulted in permanent stifle extension

  6. DNA methylation for subtype classification and prediction of treatment outcome in patients with childhood acute lymphoblastic leukemia

    Milani, Lili; Lundmark, Anders; Kiialainen, Anna; Nordlund, Jessica; Flaegstad, Trond; Forestier, Erik; Heyman, Mats; Jonmundsson, Gudmundur; Kanerva, Jukka; Schmiegelow, Kjeld; Söderhäll, Stefan; Gustafsson, Mats; Lönnerholm, Gudmar; Syvänen, Ann-Christine

    2010-01-01

    Despite improvements in the prognosis of childhood acute lymphoblastic leukemia (ALL), subgroups of patients would benefit from alternative treatment approaches. Our aim was to identify genes with DNA methylation profiles that could identify such groups. We determined the methylation levels of 1320...... ALL and gene sets that discriminated between subtypes of ALL and between ALL and controls in pairwise classification analyses. We also identified 20 individual genes with DNA methylation levels that predicted relapse of leukemia. Thus, methylation analysis should be explored as a method to improve...

  7. Risk-Based Classification System of Patients With Newly Diagnosed Acute Lymphoblastic Leukemia

    2016-04-07

    Adult B Acute Lymphoblastic Leukemia; Adult T Acute Lymphoblastic Leukemia; Childhood B Acute Lymphoblastic Leukemia; Childhood T Acute Lymphoblastic Leukemia; Untreated Adult Acute Lymphoblastic Leukemia; Untreated Childhood Acute Lymphoblastic Leukemia

  8. Disruption of Learning Processes by Chemotherapeutic Agents in Childhood Survivors of Acute Lymphoblastic Leukemia and Preclinical Models

    Emily B. Bisen-Hersh, Philip N. Hineline, Ellen A. Walker

    2011-01-01

    Full Text Available Objective: With the survival rate of acute lymphoblastic leukemia (ALL surpassing 90 percent within this decade, new research is emerging in the field of late effects. A review of the research investigating the relationship of treatment regimens for ALL to specific late effect deficits, underlying mechanisms, and possible remediation is warranted to support continued studies.Methods: The clinical literature was briefly surveyed to describe the occurrence and topography of late effects, specifically neurocognitive deficits. Additionally, the preclinical literature was reviewed to uncover potential underlying mechanisms of these deficits. The advantages of using rodent models to answer these questions are outlined, as is an assessment of the limited number of rodent models of childhood cancer treatment.Results: The literature supports that childhood survivors of ALL exhibit academic difficulties and are more likely to be placed in a special education program. Behavioral evidence has highlighted impairments in the areas of attention, working memory, and processing speed, leading to a decrease in full scale IQ. Neurophysiological and preclinical evidence for these deficits has implicated white matter abnormalities and acquired brain damage resulting from specific chemotherapeutic agents commonly used during treatment.Conclusions: The exact role of chemotherapeutic agents in learning deficits remains mostly unknown. Recommendations for an improved rodent model of learning deficits in childhood cancer survivors are proposed, along with suggestions for future directions in this area of research, in hopes that forthcoming treatment regimens will reduce or eliminate these types of impairments.

  9. Confirmation of childhood acute lymphoblastic leukemia variants, ARID5B and IKZF1, and interaction with parental environmental exposures.

    Tiffany-Jane Evans

    Full Text Available Genome wide association studies (GWAS have established association of ARID5B and IKZF1 variants with childhood acute lymphoblastic leukemia (ALL. Epidemiological studies suggest that environmental factors alone appear to make a relatively minor contribution to disease risk. The polygenic nature of childhood ALL predisposition together with the timing of environmental triggers may hold vital clues for disease etiology. This study presents results from an Australian GWAS of childhood ALL cases (n = 358 and population controls (n = 1192. Furthermore, we utilised family trio (n = 204 genotypes to extend our investigation to gene-environment interaction of significant loci with parental exposures before conception, and child's sex and age. Thirteen SNPs achieved genome wide significance in the population based case/control analysis; ten annotated to ARID5B and three to IKZF1. The most significant SNPs in these regions were ARID5B rs4245595 (OR 1.63, CI 1.38-1.93, P = 2.13×10(-9, and IKZF1 rs1110701 (OR 1.69, CI 1.42-2.02, p = 7.26×10(-9. There was evidence of gene-environment interaction for risk genotype at IKZF1, whereby an apparently stronger genetic effect was observed if the mother took folic acid or if the father did not smoke prior to pregnancy (respective interaction P-values: 0.04, 0.05. There were no interactions of risk genotypes with age or sex (P-values >0.2. Our results evidence that interaction of genetic variants and environmental exposures may further alter risk of childhood ALL however, investigation in a larger population is required. If interaction of folic acid supplementation and IKZF1 variants holds, it may be useful to quantify folate levels prior to initiating use of folic acid supplements.

  10. The Circadian Schedule for Childhood Acute Lymphoblastic Leukemia Maintenance Therapy does not Influence Event-Free Survival in the NOPHO ALL92 Protocol

    Clemmensen, Kim K. B.; Christensen, Regitse H.; Shabaneh, Diana N.;

    2014-01-01

    BACKGROUND: The event-free survival of childhood acute lymphoblastic leukemia (ALL) has been reported to be superior when oral methotrexate (MTX) and 6-mercaptopurine (6MP) maintenance therapy (MT) is administered in the evening compared to the morning. PROCEDURE: In the ALL92 MT study we prospec...

  11. Relapsed childhood high hyperdiploid acute lymphoblastic leukemia: presence of preleukemic ancestral clones and the secondary nature of microdeletions and RTK-RAS mutations

    Davidsson, J; Paulsson, K; Lindgren, D;

    2010-01-01

    Although childhood high hyperdiploid acute lymphoblastic leukemia is associated with a favorable outcome, 20% of patients still relapse. It is important to identify these patients already at diagnosis to ensure proper risk stratification. We have investigated 11 paired diagnostic and relapse samp...

  12. Current status of total body irradiation in conditioning regimen for childhood acute lymphoblastic leukemia. Survey in the Japan Association of Childhood Leukemia Study (JACLS) Group

    We surveyed methods of total body irradiation (TB I) in conditioning regimens of stem cell transplantation (SCT) for children with acute lymphoblastic leukemia (ALL) at participating institutions of the Japan Association of Childhood Leukemia Study (JACLS) ALL-97 protocol. We obtained information about TBI from 25 institutions. Total dose of 12 Gy fractionated by four to six in two to three days for TBI was conducted in 22 of 25 institutions. High-risk patients, such as patients with Philadelphia positive ALL, received over 12 Gy in five institutions. Beam direction and patient's positioning were horizontal and lateral respectively in 15 institutions. Shielding of lung and/or eyes and boost irradiation to central nervous system and/or testis were done in 24 and 11 institutions respectively, but in various ways. We have to keep in mind that a great variety of TBI have been undergone in each institution when we intend to interpret multi-institutional trials of treatment including SCT for patients with ALL. (author)

  13. Cost-effective multiplexing before capture allows screening of 25 000 clinically relevant SNPs in childhood acute lymphoblastic leukemia

    Wesolowska, Agata; Dalgaard, M. D.; Borst, L.;

    2011-01-01

    Genetic variants, including single-nucleotide polymorphisms (SNPs), are key determiners of interindividual differences in treatment efficacy and toxicity in childhood acute lymphoblastic leukemia (ALL). Although up to 13 chemotherapeutic agents are used in the treatment of this cancer, it remains a...... model disease for exploring the impact of genetic variation due to well-characterized cytogenetics, drug response pathways and precise monitoring of minimal residual disease. Here, we have selected clinically relevant genes and SNPs through literature screening, and on the basis of associations with key...... designed a cost-effective, high-throughput capture assay of â¼25â000 clinically relevant SNPs, and demonstrated that multiple samples can be tagged and pooled before genome capture in targeted enrichment with a sufficient sequencing depth for genotyping. This multiplexed, targeted sequencing method allows...

  14. Myositis complicating benzathine penicillin-G injection in a case of rheumatic heart disease

    Francis, Joshua R.; Wyber, Rosemary; Remenyi, Bo; Croser, David; Carapetis, Jonathan

    2016-01-01

    A 7-year old boy developed myositis secondary to intramuscular injection of benzathine penicillin-G in the context of secondary prophylaxis for rheumatic heart disease. Side effects of intramuscular delivery of benzathine penicillin-G are well described and include injection site pain and inflammation, but myositis, as depicted on magnetic resonance imaging in this case, has not previously been described. PMID:27051573

  15. Scintigraphic evaluation of Lyme disease: Gallium-67 imaging of Lyme myositis

    Kengen, R.A.; v.d. Linde, M.; Sprenger, H.G.; Piers, D.A. (Univ. Hospital, Groningen (Netherlands))

    1989-10-01

    A patient suffering from Lyme disease had cardiac conduction abnormalities, symptoms of arthritis, and myalgia. A Ga-67 image showed evidence of endomyocarditis, but intense skeletal muscle uptake pointed to Lyme myositis. Reference is made to two other case reports of Lyme myositis.

  16. Scintigraphic evaluation of Lyme disease: Gallium-67 imaging of Lyme myositis

    A patient suffering from Lyme disease had cardiac conduction abnormalities, symptoms of arthritis, and myalgia. A Ga-67 image showed evidence of endomyocarditis, but intense skeletal muscle uptake pointed to Lyme myositis. Reference is made to two other case reports of Lyme myositis

  17. Myositis complicating benzathine penicillin-G injection in a case of rheumatic heart disease

    Joshua R. Francis; Rosemary Wyber; Bo Remenyi; David Croser; Jonathan Carapetis

    2016-01-01

    A 7-year old boy developed myositis secondary to intramuscular injection of benzathine penicillin-G in the context of secondary prophylaxis for rheumatic heart disease. Side effects of intramuscular delivery of benzathine penicillin-G are well described and include injection site pain and inflammation, but myositis, as depicted on magnetic resonance imaging in this case, has not previously been described.

  18. Effect of azole antifungal therapy on vincristine toxicity in childhood acute lymphoblastic leukaemia

    Schie, R.M. van; Bruggemann, R.J.M.; Hoogerbrugge, P.M.; Loo, D.M. te

    2011-01-01

    BACKGROUND: Vincristine is one of the cornerstones of the treatment of children with acute lymphoblastic leukaemia (ALL). Constipation, and peripheral and central neurotoxicities are the most common side effects. A comparative study exploring vincristine toxicity in individual patients receiving vin

  19. Methotrexate resistance in relation to treatment outcome in childhood acute lymphoblastic leukemia

    Wojtuszkiewicz, Anna; Peters, Godefridus J; van Woerden, Nicole L;

    2015-01-01

    BACKGROUND: Methotrexate (MTX) eradicates leukemic cells by disrupting de novo nucleotide biosynthesis and DNA replication, resulting in cell death. Since its introduction in 1947, MTX-containing chemotherapeutic regimens have proven instrumental in achieving curative effects in acute lymphoblastic...

  20. Prenatal origin of chromosomal translocations in acute childhood leukemia: Implications and future directions

    McHale, C M; Smith, M. T.

    2004-01-01

    We, and others, have demonstrated an in utero origin for translocations associated with childhood leukemia, with latency periods in some cases exceeding 10 years. The mechanism of generation of most of the translocations is thought to be aberrant repair following abortive apoptosis, rather than V(D)J recombination or exposure to topoisomerase II inhibitors. Folate supplementation may prevent some of the chromosome breakage leading to translocation formation. Translocations t(8;21) and t(12;21...

  1. Acute lymphoblastic leukemia of childhood presenting as aplastic anemia: report of two cases

    Laura Villarreal-Martínez; José Carlos Jaime-Pérez; Marisol Rodríguez-Martínez; Oscar González-Llano; David Gómez-Almaguer

    2012-01-01

    Acute lymphoblastic leukemia is the most common malignancy in pediatric patients; its diagnosis is usually easy to establish as malignant lymphoblasts invade the bone marrow and peripheral blood. Some acute lymphoblastic leukemia patients may initially present with pancytopenia and a hypoplastic bone marrow leading to the initial diagnosis of aplastic anemia. In most of these patients clinical improvement occurs, with normalization of the complete blood count within six months, although recov...

  2. ARID5B and IKZF1 variants, selected demographic factors, and childhood acute lymphoblastic leukemia: A report from the Children’s Oncology Group

    Linabery, Amy M.; Blommer, Crystal N.; Spector, Logan G; Davies, Stella M.; Robison, Leslie L.; Ross, Julie A.

    2013-01-01

    Interactions between common germline variants in ARID5B and IKZF1 and other known childhood acute lymphoblastic leukemia (ALL) risk factors were queried using biospecimens and data from 770 ALL cases and 384 controls. Case-control comparisons revealed dosedependent associations between ARID5B rs10821936, ARID5B rs10994982, and IKZF1 rs11978267 and childhood ALL overall, and B lineage and B lineage hyperdiploid ALL examined separately (all allelic odds ratios≥1.33, Ptrend≤0.001). No heterogene...

  3. Decrease in cerebral metabolic rate of glucose after high-dose methotrexate in childhood acute lymphocytic leukemia

    We measured changes in the regional cerebral metabolic rate of glucose (rCMRGlu) using 18F-fluorodeoxyglucose and positron emission tomography for the assessment of neurotoxicity in childhood acute lymphocytic leukemia treated with high-dose methotrexate (HD-MTX) therapy. We studied 8 children with acute lymphocytic leukemia (mean age: 9.6 years) treated with HD-MTX (200 mg/kg or 2,000 mg/M2) therapy. CMRGlu after HD-MTX therapy was most reduced (40%) in the patient who had central nervous system leukemia and was treated with the largest total doses of both intrathecal MTX (IT-MTX) and HD-MTX. CMRGlu in the whole brain after HD-MTX therapy was reduced by an average of 21% (P less than 0.05). The reductions of CMRGlu in 8 patients were correlated with total doses of both IT-MTX (r = 0.717; P less than 0.05) and systemic HD-MTX (r = 0.784; P less than 0.05). CMRGlu of the cerebral cortex, especially the frontal and occipital cortex, was reduced more noticeably than that of the basal ganglia and white matter. We suggest that the measurement of changes in rCMRGlu after HD-MTX therapy is useful for detecting accumulated MTX neurotoxicity

  4. "Orbiting around" the orbital myositis: clinical features, differential diagnosis and therapy.

    Montagnese, F; Wenninger, S; Schoser, B

    2016-04-01

    Orbital myositis (OM) is a rare disease whose clinical heterogeneity and different treatment options represent a diagnostic and therapeutic challenge. We aim to review the state of knowledge on OM, also describing a cohort of patients diagnosed in our centre, to highlight some remarkable clinical features. A literature review was conducted in PubMed and Medline databases. The herein described cohort is composed of seven OM patients, diagnosed according to clinical, laboratory and neuroradiological features, whose clinical data were retrospectively analysed. OM is a non-infectious, inflammatory process primarily involving extraocular eye-muscles. It typically presents as an acute to sub-acute, painful ophthalmoplegia with signs of ocular inflammation, but atypical cases without pain or with a chronic progression have been described. The wide range of OM mimicking diseases make a prompt diagnosis challenging but orbit MRI provides valuable clues for differential diagnosis. Timely treatment is greatly important as OM promptly responds to steroids; nevertheless, partial recovery or relapses often occur. In refractory, recurrent or steroid-intolerant cases other therapeutic options (radiotherapy, immunosuppressants, immunoglobulins) can be adopted, but the most effective therapeutic management is yet to be established. In this review, we provide a detailed clinical description of OM, considering the main differential diagnoses and suggesting the most useful investigations. In light of the currently available data on therapy efficacy, we propose a therapeutic algorithm that may guide neurologists in OM patients' management. PMID:26477021

  5. Outcome of allogeneic hematopoietic stem cell transplantation for childhood acute lymphoblastic leukemia in second complete remission: a single institution study

    Eun-Jung Lee

    2012-03-01

    Full Text Available Purpose : The survival rate for childhood acute lymphoblastic leukemia (ALL has improved significantly. However, overall prognosis for the 20 to 25% of patients who relapse is poor, and allogeneic hematopoietic stem cell transplantation (HSCT offers the best chance for cure. In this study, we identified significant prognostic variables by analyzing the outcomes of allogeneic HSCT in ALL patients in second complete remission (CR. Methods : Fifty-three ALL patients (42 men, 79% who received HSCT in second CR from August 1991 to February 2009 were included (26 sibling donor HSCTs, 49%; 42 bone marrow transplantations, 79%. Study endpoints included cumulative incidence of acute and chronic graft-versus-host disease (GVHD, relapse, 1-year transplant-related mortality (TRM, disease-free survival (DFS, and overall survival (OS. Results : Cumulative incidences of acute GVHD (grade 2 or above and chronic GVHD were 45.3% and 28.5%, respectively. The estimated 5-year DFS and OS for the cohort was 45.2¡?#?.8%; and 48.3¡?#?%,; respectively. Only donor type, i.e., sibling versus unrelated, showed significant correlation with DFS in multivariate analysis (P=0.010. The rates of relapse and 1 year TRM were 28.9¡?#?.4%; and 26.4¡?#?.1%;, respectively, and unrelated donor HSCT (P=0.002 and HLA mismatch (P =0.022 were significantly correlated with increased TRM in univariate analysis. Conclusion : In this single institution study spanning more than 17 years, sibling donor HSCT was the only factor predicting a favorable result in multivariate analysis, possibly due to increased TRM resulting from unrelated donor HSCT.

  6. Controversies in the diagnosis and management of childhood acute immune thrombocytopenic purpura.

    Segel, George B; Feig, Stephen A

    2009-09-01

    Acute immune thrombocytopenic purpura (ITP) occurs most commonly in young children who present with severe isolated thrombocytopenia and purpura. A marrow examination is not required unless glucocorticoids are used, lest treatment mask incipient acute lymphoblastic leukemia, but controversy exists here. The recommendations for evaluation and management remain controversial, since prospective controlled trials have not been done. There is some consensus based on experience and empiric data. Almost all children with acute ITP will recover completely without therapy. Although the various treatments may increase the platelet count, they do not influence the outcome of the illness, may increase cost, and cause significant side effects. Therefore, careful observation may be the best management option for the patient with ITP, in the absence of severe bleeding. The data available relevant to these issues are discussed. PMID:19165890

  7. Epidemiological, clinical and prognostic profile of childhood acute bacterial meningitis in a resource poor setting

    Bankole Peter Kuti

    2015-01-01

    Full Text Available Background: Childhood bacterial meningitis is a neurologic emergency that continues to kill and maims children particularly in developing countries with poor immunization coverage. Objective: This study set out to assess the hospital incidence, pattern of presentation, etiologic agents, outcome and determinants of mortality among the children admitted with bacterial meningitis at the Wesley Guild Hospital (WGH, Ilesa. Patients and Methods: We carried out a retrospective review of admitted cases of bacterial meningitis in children aged one month to 15 years at the WGH, Ilesa over a three year period by looking at the hospital records. Factors in the history and examinations were compared among survivors and those that died to determine factors significantly associated with mortality in these children. Results: Eighty-one (5.5% of the 1470 childhood admissions during the study period had bacterial meningitis. Male preponderance was observed and two-thirds of the children were infants. More cases were admitted during the wet rainy season than during the dry harmattan season. Haemophilus influenzae type B and Streptococcus pneumoniae were the leading etiologic agents and ciprofloxacin and ceftriaxone adequately cover for these organisms. Twenty-two (27.2% of the 81 children died, while 34 (42.0% survived with neurologic deficits. Children with multiple seizures, coma, neck retraction, hyponatremia, hypoglycorrhachia, turbid CSF as well as Gram positive meningitis at presentation were found to more likely to die (P < 0.05. None of these factors however independently predict mortality. Conclusion: Childhood bacterial meningitis often results in death and neurologic deficit among infants and young children admitted at the WGH, Ilesa. Children diagnosed with meningitis who in addition had multiple seizures, neck retraction and coma at presentation are at increased risk of dying.

  8. Pubertal development and fertility in survivors of childhood acute myeloid leukemia treated with chemotherapy only

    Molgaard-Hansen, Lene; Skou, Anne-Sofie; Juul, Anders;

    2013-01-01

    More than 60% of children with acute myeloid leukemia (AML) become long-term survivors. Most are cured using chemotherapy without hematopoietic stem cell transplantation (HSCT). We report on pubertal development and compare self-reported parenthood among AML survivors and their siblings.......More than 60% of children with acute myeloid leukemia (AML) become long-term survivors. Most are cured using chemotherapy without hematopoietic stem cell transplantation (HSCT). We report on pubertal development and compare self-reported parenthood among AML survivors and their siblings....

  9. Duration of adrenal insufficiency during treatment for childhood acute lymphoblastic leukemia

    Vestergaard, Therese Risom; Juul, Anders; Lausten-Thomsen, Ulrik; Lausen, Birgitte Frederiksen; Hjalgrim, Henrik; Kvist, Tine Kajsa; Andersen, Elisabeth Anne Wreford; Schmiegelow, Kjeld

    2011-01-01

    Children with acute lymphoblastic leukemia (ALL) recive high doses of glucocorticosteroid as part of their treatment. This may lead to suppression of the hypothalamic-pituitary-adrenal axis, acute adrenal insufficiency, and ultimately to life-threatening conditions. This study explores the adrenal...... function in 96 children with ALL treated according to common protocols. After cessation of induction glucocorticosteroid therapy, they received hydrocortisone substitution therapy (10 mg/m/24 h) until an adrenocorticotropic hormone test (250 µg tetracosatide) showed a sufficient adrenal response [plasma (p...

  10. Late cardiac effects of anthracycline containing therapy for childhood acute lymphoblastic leukemia

    Rathe, Mathias; Carlsen, Niels L T; Oxhøj, Henrik

    2007-01-01

    At present about 80% of children with acute lymphoblastic leukemia (ALL) will be cured following treatment with multi-drug chemotherapy. A major concern for this growing number of survivors is the risk of late effects of treatment. The aim of this study was to determine whether signs of cardiomyo......At present about 80% of children with acute lymphoblastic leukemia (ALL) will be cured following treatment with multi-drug chemotherapy. A major concern for this growing number of survivors is the risk of late effects of treatment. The aim of this study was to determine whether signs of...

  11. Levofloxacin in Preventing Infection in Young Patients With Acute Leukemia Receiving Chemotherapy or Undergoing Stem Cell Transplantation

    2016-04-08

    Acute Leukemias of Ambiguous Lineage; Bacterial Infection; Diarrhea; Fungal Infection; Musculoskeletal Complications; Neutropenia; Recurrent Childhood Acute Lymphoblastic Leukemia; Recurrent Childhood Acute Myeloid Leukemia; Secondary Acute Myeloid Leukemia; Untreated Childhood Acute Myeloid Leukemia and Other Myeloid Malignancies

  12. Inclusion-Body Myositis Associated with Alzheimer’s Disease

    Danijela Levacic

    2013-01-01

    Full Text Available Sporadic inclusion-body myositis (s-IBM is a myopathy that is characterized by progressive weakness and muscle pathology demonstrating inflammation and rimmed vacuoles. In addition, similar to the pathology observed in the brains of patients with Alzheimer’s disease, the deposition of beta-amyloid and phosphorylated tau proteins in muscle fibers has been reported. These shared pathologic features have prompted hypotheses suggesting a shared etiology of these two conditions. We report a case of a 73-year-old woman initially diagnosed with s-IBM who later developed Alzheimer’s disease.

  13. Therapy-Related Myelodysplastic Syndrome Following Treatment for Childhood Acute Lymphoblastic Leukemia: Outcome of Patients Registered in the EWOG-MDS 98/06 Studies

    Strahm, Birgitte; Amann, Roland; De Moerloose, Barbara;

    Objective: Therapy-related myelodysplastic syndrome (tMDS) following treatment of childhood acute lymphoblastic leukemia (ALL) is one of the most frequently observed secondary malignancies in survivors of childhood cancer. Allogeneic stem cell transplantation (SCT) is the only curative treatment....... This analysis was performed to asses the outcome of patients with tMDS following treatment for childhood ALL reported to the EWOG-MDS study group. Patients and Transplant Procedure: Forty-three patients (19 male/24 female) were diagnosed with tMDS between August 1989 and August 2009. The median age at diagnosis......, cyclophosphamide and melphalan (Bu/Cy/Mel) (23), an alternative busulfan based regimen (6), a radiation based regimen (5) or others (3). Results: After a median follow up of 4.1 (0.5 – 9.4) years, 14 patients are alive in first complete remission (CR). Seventeen patients developed relapse after a median time...

  14. Quality of health in survivors of childhood acute myeloid leukemia treated with chemotherapy only

    Molgaard-Hansen, Lene; Glosli, Heidi; Jahnukainen, Kirsi;

    2011-01-01

    More than 60% of children with acute myeloid leukemia (AML) become long-term survivors, and approximately 50% are cured with chemotherapy only. Limited data exist about their long-term morbidity and social outcomes. The aim of the study was to compare the self-reported use of health care services...

  15. Response of acute cerebral lupus in childhood to pulse methylprednisolone in reduced dosage.

    Hanmer, O.; Saltissi, D.

    1986-01-01

    Intravenous methylprednisolone pulse therapy in a reduced dose of 10 mg/kg on three consecutive days was successfully used in the treatment of a 9 year old girl with acute cerebral lupus in coma. It is recommended for further evaluation in children with severe primary central nervous system (CNS) disease.

  16. Granulocytic Sarcoma as the First Sign of Acute Leukemia in Childhood

    Aalia R Sufi

    2012-01-01

    Full Text Available Acute myeloid leukemia (AML may rarely involve the orbit as a solid tumor termed granulocytic sarcoma. This report describes the case of a child who presented with rapidly progressive unilateral proptosis and was diagnosed as rhabdomyosarcoma. However subsequent examination of the peripheral blood film revealed AML. Thus proptosis may present as the initial manifestation of AML.

  17. Prognosis in childhood and adult acute lymphoblastic leukaemia : a question of maturation?

    Plasschaert, SLA; Kamps, WA; Vellenga, E; de Vries, EGE; de Bont, ESJM

    2004-01-01

    Acute lymphoblastic leukaemia (ALL) is a disease diagnosed in children as well as adults. Progress in the treatment of ALL has led to better survival rates, however, children have benefited more from improved treatment modalities than adults. Recent evidence has underscored that the difference in ch

  18. Prediction of immunophenotype, treatment response, and relapse in childhood acute lymphoblastic leukemia using DNA microarrays

    Willenbrock, Hanni; Juncker, Agnieszka; Schmiegelow, K.; Knudsen, Steen; Ryder, L.P.

    2004-01-01

    Gene expression profiling is a promising tool for classification of pediatric acute lymphoblastic leukemia ( ALL). We analyzed the gene expression at the time of diagnosis for 45 Danish children with ALL. The prediction of 5-year event-free survival or relapse after treatment by NOPHO-ALL92 or 2000...

  19. Symptom Differences in Acute and Chronic Presentation of Childhood Post-Traumatic Stress Disorder.

    Famularo, Richard; And Others

    1990-01-01

    Twenty-four child abuse victims, age 5-13, were diagnosed with posttraumatic stress disorder (PTSD). Children with the acute form of PTSD exhibited such symptoms as difficulty falling asleep, hypervigilance, nightmares, and generalized anxiety. Children exhibiting chronic PTSD exhibited increased detachment, restricted range of affect,…

  20. Heterogeneous cytogenetic subgroups and outcomes in childhood acute megakaryoblastic leukemia: A retrospective international study

    H. Inaba (Hiroto); Y. Zhou (Yinmei); O. Abla (Oussama); S. Adachi (Susumu); A. Auvrignon (Anne); H.B. Beverloo (Berna); E.S.J.M. de Bont (Eveline); T.-T. Chang (Tai-Tsung); U. Creutzig; M.N. Dworzak (Michael); S. Elitzur (Sarah); A. Fynn (Alcira); E. Forestier (Erik); H. Hasle (Henrik); D.-C. Liang (Der-Cherng); V. Lee (Vincent); F. Locatelli (Franco); R. Masetti (Riccardo); B. de Moerloose (Barbara); D. Reinhardt (Dirk); L. Rodriguez (Laura); N. van Roy (Nadine); S. Shen (Shuhong); T. Taga (Takashi); D. Tomizawa (Daisuke); A.E.J. Yeoh (Allen E. J.); M. Zimmermann (Martin); S.C. Raimondi (Susana)

    2015-01-01

    textabstractComprehensive clinical studies of patients with acute megakaryoblastic leukemia (AMKL) are lacking. We performed an international retrospective study on 490 patients (age ≤18 years) with non-Down syndrome de novo AMKL diagnosed from 1989 to 2009. Patients with AMKL (median age 1.53 years

  1. Fine motor and handwriting problems after treatment for childhood acute lymphoblastic leukemia

    ReindersMesselink, HA; Schoemaker, MM; Hofte, M; Goeken, LNH; Kingma, A; vandenBriel, MM; Kamps, WA

    1996-01-01

    Motor skills were investigated in 18 children 2 years after treatment for acute lymphoblastic leukemia (ALL). Cross and fine motor functioning were examined with the Movement Assessment Battery for Children. Handwriting as a specific fine motor skill was studied with a computerized writing task. We

  2. Acute Childhood Cardiorenal Syndrome and Impact of Cardiovascular Morbidity on Survival

    Wasiu A. Olowu

    2011-01-01

    Full Text Available Cardiorenal syndrome (CRS clinical types, prevalence, aetiology, and acute cardiovascular morbidity impact on the outcome of acute kidney function perturbation were determined. Forty-seven of 101 (46.53% patients with perturbed kidney function had CRS. Types 3 and 5 CRS were found in 10 and 37 patients, respectively. Type 3 CRS was due to acute glomerulonephritis (AGN; =7, captopril (=1, frusemide (=1, and hypovolaemia (=1. Malaria-associated haemoglobinuria (=20, septicaemia (=11, lupus nephritis (=3, tumour lysis syndrome (=2, and acute lymphoblastic leukaemia (=1 caused Type 5 CRS. The cumulative mortality in hypertensive CRS was similar to nonhypertensive CRS (51.4% versus 40.9%; =.119. Mortality in CRS and non-CRS was similar (45.7% versus 24.5%; =.053. Type 5 survived better than type 3 CRS (66.7% versus 12.5%; =.001. Risk factors for mortality were Type 3 CRS (=.001, AGN-associated CRS (=.023, dialysis requiring CRS (=.008, and heart failure due to causes other than anaemia (=.003. All-cause-mortality was 34.2%. Preventive measures aimed at the preventable CRS aetiologies might be critical to reducing its prevalence.

  3. Computed tomographic-arteriographic correlates in acute basal ganglionic infraction of childhood

    Fifteen children who presented with acute hemiparesis and basal ganglionic infarction were studied by computed tomography and arteriography (11 patients). Arteriography demonstrated focal vascular disease in 10 patients. Current management of these pediatric patients requires computed tomography, arteriography and complete laboratory investigation. (orig.)

  4. The role of ABC-transporters in childhood and adult acute lymphoblastic leukemia

    Plasschaert, Sabine Louise Anne

    2005-01-01

    Acute lymphoblastic leukemia is a disease characterized by an uncontrolled proliferation and maturation arest of lymphoid progenitor cells in the bone marrow, resulting in an excesso f malignant cells. The disease has a peak incidence between the age of 2-5 years, and a low and steady rise from the

  5. The role of ABC-transporters in childhood and adult acute lymphoblastic leukemia

    Plasschaert, Sabine Louise Anne

    2005-01-01

    Acute lymphoblastic leukemia is a disease characterized by an uncontrolled proliferation and maturation arest of lymphoid progenitor cells in the bone marrow, resulting in an excesso f malignant cells. The disease has a peak incidence between the age of 2-5 years, and a low and steady rise from the age of 40 ... Zie: Summary

  6. Is there an increased risk of metabolic syndrome among childhood acute lymphoblastic leukemia survivors? A developing country experience.

    Mohapatra, Sonali; Bansal, Deepak; Bhalla, A K; Verma Attri, Savita; Sachdeva, Naresh; Trehan, Amita; Marwaha, R K

    2016-03-01

    Data on metabolic syndrome (MS) in survivors of childhood acute lymphoblastic leukemia (ALL) from developing countries are lacking. The purpose of this single-center, uncontrolled, observational study was to assess the frequency of MS in our survivors. The survivors of ALL ≤15 years at diagnosis, who had completed therapy ≥2 years earlier, were enrolled. Anthropometric measurements (weight, height, waist circumference), biochemistry (glucose, insulin, triglycerides, high-density lipoprotein [HDL], thyroid function tests, C-reactive protein [CRP], magnesium), measurement of blood pressure, and Tanner staging were performed. MS was defined by International Diabetes Federation (IDF) and the National Cholesterol Education Program Third Adult Treatment Panel guidelines (NCEP ATP III) criteria, modified by Cook et al. (Arch Pediatr Adolesc Med. 2003;157:821-827) and Ford et al. (Diabetes Care. 2005;28:878-881). The median age of 76 survivors was 11.9 years (interquartile range [IQR]: 9.6-13.5). Twenty-four (32%) survivors were obese or overweight. The prevalence of insulin resistance (17%), hypertension (7%), hypertriglyceridemia (20%), and low HDL (37%) was comparable to the prevalence in children/adolescents in historical population-based studies from India. The prevalence of MS ranged from 1.3% to 5.2%, as per different defining criteria. Cranial radiotherapy, age at diagnosis, sex, or socioeconomic status were not risk factors for MS. The prevalence of MS in survivors of childhood ALL, at a median duration of 3 years from completion of chemotherapy, was comparable to the reference population. The prevalence of being obese or overweight was, however, greater than historical controls. PMID:26984439

  7. Identification of de Novo Fanconi Anemia in Younger Patients With Newly Diagnosed Acute Myeloid Leukemia

    2016-05-13

    Childhood Acute Erythroleukemia (M6); Childhood Acute Megakaryocytic Leukemia (M7); Childhood Acute Minimally Differentiated Myeloid Leukemia (M0); Childhood Acute Monoblastic Leukemia (M5a); Childhood Acute Monocytic Leukemia (M5b); Childhood Acute Myeloblastic Leukemia With Maturation (M2); Childhood Acute Myeloblastic Leukemia Without Maturation (M1); Childhood Acute Myelomonocytic Leukemia (M4); Childhood Myelodysplastic Syndromes; Chronic Myelomonocytic Leukemia; de Novo Myelodysplastic Syndromes; Fanconi Anemia; Refractory Anemia; Refractory Anemia With Excess Blasts; Refractory Anemia With Excess Blasts in Transformation; Refractory Anemia With Ringed Sideroblasts; Secondary Myelodysplastic Syndromes; Untreated Childhood Acute Myeloid Leukemia and Other Myeloid Malignancies

  8. Detection of prognostically relevant genetic abnormalities in childhood B-cell precursor acute lymphoblastic leukaemia: recommendations from the Biology and Diagnosis Committee of iBFM-SG

    Harrison, Christine J; Haas, Oskar A.; Harbott, W; Biondi, Andrea; Stanulla, Martin; Trka, Jan; Izraeli, Shai

    2010-01-01

    Abstract Treatment of childhood acute lymphoblastic leukaemia (ALL) has improved considerably in recent years. A contributing factor has been the improved stratification for treatment according to a number of factors including genetic determinants of outcome. Here we review the current diagnostic criteria of genetic abnormalities in precursor B-ALL (BCP-ALL), including the relevant technical approaches and the application of the most appropriate methods for the detection of each ab...

  9. Role of glutathione S-transferase M1, T1 and P1 gene polymorphisms in childhood acute lymphoblastic leukemia susceptibility in a Turkish population

    Mehmet Guven; Selin Unal; Duygu Erhan; Nihal Ozdemir; Safa Baris; Tiraje Celkan; Merve Bostancı; Bahadir Batar

    2015-01-01

    The variations between different individuals in the xenobiotic metabolizing enzymes' activity were shown to modify susceptibility to childhood acute lymphoblastic leukemia (ALL). Polymorphisms associated with genes coding for the glutathione S-transferase (GST) enzyme were known to affect the metabolism of different carcinogens. The aim of this study was to evaluate the influence of the GSTM1 and GSTT1 deletion polymorphisms, and the GSTP1 Ile105Val single nucleotide polymorphism (SNP) on the...

  10. After the chemotherapy: potential mechanisms for chemotherapy-induced delayed skeletal muscle dysfunction in survivors of acute lymphoblastic leukaemia in childhood

    Celena eScheede-Bergdahl; R Thomas Jagoe

    2013-01-01

    There is evidence that survivors of childhood cancers, such as acute lymphoblastic leukaemia (ALL), have increased rates of longterm skeletal muscle dysfunction. This places them at higher risk of physical restriction and functional impairment as well as potentially contributing to observed increases in cardiovascular disease and insulin resistance in later life. The mechanisms underlying these changes in skeletal muscle are unknown but chemotherapy drugs used in treatment for ALL are strong...

  11. Lymphoid Progenitor Cells from Childhood Acute Lymphoblastic Leukemia Are Functionally Deficient and Express High Levels of the Transcriptional Repressor Gfi-1

    Jessica Purizaca; Adriana Contreras-Quiroz; Elisa Dorantes-Acosta; Eduardo Vadillo; Lourdes Arriaga-Pizano; Silvestre Fuentes-Figueroa; Horacio Villagomez-Barragán; Patricia Flores-Guzmán; Antonio Alvarado-Moreno; Hector Mayani; Isaura Meza; Rosaura Hernandez; Sara Huerta-Yepez; Rosana Pelayo

    2013-01-01

    Acute lymphoblastic leukemia (ALL) is the most frequent malignancy of childhood. Substantial progress on understanding the cell hierarchy within ALL bone marrow (BM) has been recorded in the last few years, suggesting that both primitive cell fractions and committed lymphoid blasts with immature stem cell-like properties contain leukemia-initiating cells. Nevertheless, the biology of the early progenitors that initiate the lymphoid program remains elusive. The aim of the present study was to ...

  12. Acute Gastroenteritis During Childhood in Bolu, Turkey: 3 years of experience

    Mervan Bekdaş

    2013-01-01

    Full Text Available We evaluated 6563 children with the complaint of diarrhea. 29.1% were below 2 years of age, 37.4% were 2-5 years of age and 33.3% were over 5 years of age. 22.3% were admitted during spring, 33.5% were admitted during summer, 23.6% were admitted during autumn and 20.4% were admitted during winter. Rotavirus antigen was found in 16.1% cases. 7.6% of the patients were hospitalized. The direct medical cost for out-patient's clinic was $18.7 and for hospitalized patients was $74.3. The most common agent in the childhood gastroenteritis was rotavirus. Viral agents were identified frequently during winter and bacterial and parasitic agents were identified frequently during summer.

  13. High concordance of subtypes of childhood acute lymphoblastic leukemia within families

    Schmiegelow, K.; Thomsen, U Lautsen; Baruchel, A; Pacheco, CE; Pieters, R; Pombo-de-Oliveira, MS; Andersen, Elisabeth Anne Wreford; Rostgaard, K; Hjalgrim, Helle; Pui, CH

    2012-01-01

    of developing ALL. This international collaboration identified 54 sibships with two (N=51) or more (N=3) cases of childhood ALL (ages <18 years). The 5-year event-free survival for 61 patients diagnosed after 1 January 1990 was 0.83±0.05. Ages at diagnosis (Spearman correlation coefficient, rS=0.......41, P=0.002) were significantly correlated, but not WBCs (rS=0.23, P=0.11). In 18 sibships with successful karyotyping in both cases, six were concordant for high-hyperdiploidy (N=3), t(12;21) [ETV6/RUNX1] (N=1), MLL rearrangement (N=1) or t(1;19)(q23/p13) (N=1). Eleven sibships were ALL...

  14. Childhood Acute Respiratory Infections and Household Environment in an Eastern Indonesian Urban Setting

    Tomoyuki Shibata

    2014-11-01

    Full Text Available This pilot study evaluated the potential effect of household environmental factors such as income, maternal characteristics, and indoor air pollution on children’s respiratory status in an Eastern Indonesian community. Household data were collected from cross-sectional (n = 461 participants and preliminary childhood case-control surveys (pneumonia cases = 31 diagnosed within three months at a local health clinic; controls = 30. Particulate matter (PM2.5 and PM10 was measured in living rooms, kitchens, children’s bedrooms, and outside areas in close proximity once during the case-control household interviews (55 homes and once per hour from 6 a.m. to midnight in 11 homes. The household survey showed that children were 1.98 times (p = 0.02 more likely to have coughing symptoms indicating respiratory infection, if mothers were not the primary caregivers. More children exhibited coughing if they were not exclusively breastfed (OR = 2.18; p = 0.06 or there was a possibility that their mothers were exposed to environmental tobacco smoke during pregnancy (OR = 2.05; p = 0.08. This study suggests that household incomes and mother’s education have an indirect effect on childhood pneumonia and respiratory illness. The concentrations of PM2.5 and PM10 ranged from 0.5 to 35.7 µg/m3 and 7.7 to 575.7 µg/m3, respectively, based on grab samples. PM was significantly different between the case and control groups (p < 0.01. The study also suggests that ambient air may dilute indoor pollution, but also introduces pollution into the home from the community environment. Effective intervention programs need to be developed that consider multiple direct and indirect risk factors to protect children.

  15. Childhood acute respiratory infections and household environment in an Eastern Indonesian urban setting.

    Shibata, Tomoyuki; Wilson, James L; Watson, Lindsey M; LeDuc, Alyse; Meng, Can; Ansariadi; La Ane, Ruslan; Manyullei, Syamsuar; Maidin, Alimin

    2014-12-01

    This pilot study evaluated the potential effect of household environmental factors such as income, maternal characteristics, and indoor air pollution on children's respiratory status in an Eastern Indonesian community. Household data were collected from cross-sectional (n = 461 participants) and preliminary childhood case-control surveys (pneumonia cases = 31 diagnosed within three months at a local health clinic; controls = 30). Particulate matter (PM2.5 and PM10) was measured in living rooms, kitchens, children's bedrooms, and outside areas in close proximity once during the case-control household interviews (55 homes) and once per hour from 6 a.m. to midnight in 11 homes. The household survey showed that children were 1.98 times (p = 0.02) more likely to have coughing symptoms indicating respiratory infection, if mothers were not the primary caregivers. More children exhibited coughing if they were not exclusively breastfed (OR = 2.18; p = 0.06) or there was a possibility that their mothers were exposed to environmental tobacco smoke during pregnancy (OR = 2.05; p = 0.08). This study suggests that household incomes and mother's education have an indirect effect on childhood pneumonia and respiratory illness. The concentrations of PM2.5 and PM10 ranged from 0.5 to 35.7 µg/m3 and 7.7 to 575.7 µg/m3, respectively, based on grab samples. PM was significantly different between the case and control groups (p pollution, but also introduces pollution into the home from the community environment. Effective intervention programs need to be developed that consider multiple direct and indirect risk factors to protect children. PMID:25429685

  16. Does childhood misfortune raise the risk of acute myocardial infarction in adulthood?

    Morton, Patricia M.; Mustillo, Sarah A.; Ferraro, Kenneth F.

    2013-01-01

    Whereas most research on acute myocardial infarction (AMI) has focused on more proximal influences, such as adult health behaviors, the present study examines the early origins of AMI. Longitudinal data were drawn from the National Survey of Midlife Development in the United States (N=3,032), a nationally representative survey of men and women aged 25–74, which spans from 1995 to 2005. A series of event history analyses modeling age of first AMI investigated the direct effects of accumulated ...

  17. Hereditary and acquired p53 gene mutations in childhood acute lymphoblastic leukemia.

    Felix, C A; Nau, M M; Takahashi, T.; Mitsudomi, T.; Chiba, I.; Poplack, D G; Reaman, G H; Cole, D E; Letterio, J J; Whang-Peng, J

    1992-01-01

    The p53 gene was examined in primary lymphoblasts of 25 pediatric patients with acute lymphoblastic leukemia by the RNase protection assay and by single strand conformation polymorphism analysis in 23 of 25 cases. p53 mutations were found to occur, but at a low frequency (4 of 25). While all four mutations were identified by single strand conformation polymorphism, the comparative sensitivity of RNase protection was 50% (2 of 4). Heterozygosity was retained at mutated codons in 3 of 4 cases. ...

  18. Neurotoxicity during induction treatment of childhood acute lymphoblastic leukaemia: Two case reports

    Kostić Gordana

    2009-01-01

    Full Text Available Introduction. During chemotherapy of acute lymphoblastic leukaemia (ALL, children sometimes exhibit neurological disturbances. Chemiotherapeutic regimens include methotrexate, administered either intravenously or via intrathecal route. Although multiple drugs are used in addition to methotrexate, the acute neurotoxicity reported in patients is usually attributed to methotrexate. The acute neurotoxicity usually results in stroke-like symptoms such as aphasia, weakness, sensory deficits, ataxia and seizures. Outline of Cases. From 2002 until January 2008, 32 children with ALL were diagnosed and treated at the Children's Hospital in Niš. The patients' age ranged from 1.5 to 16 years. They were treated in accordance with the protocol ALL IC-BFM 2002 (ALL Intercontinental Berlin Frankfurt M'nster 2002. Two of the patients (6.25% exhibited neurotoxicity. After the occurrence of neurological symptoms, the patients were ophthalmologically and neurologically examined. In addition, the magnetic resonance (MR imaging, computerized tomography and electroencephalography were applied. The paper presents two patients, aged 9 and 15 years respectively, who exhibited acute neurotoxicity - methotrexate encephalopathy during ALL treatment. Both patients had tonic-clonic seizures and neurological symptoms in the course of the induction therapy. Neurotoxicity occurred 7 days after the third, and 3 days after the fourth intrathecal methotrexate therapy. MR images confirmed multi-focal morphological changes of brain density in one of the patients, while the other patient had normal CT reading. Even though the development significantly differed, the changes were reversible in both patients. Conclusion. The neurotoxicity in patients with ALL can be combined with significant structural changes of the brain, but also morphological changes can be absent. Several questions concerning aetiology and treatment of neurological events are raised.

  19. Japanese encephalitis--an important cause of acute childhood encephalopathy in Lucknow, India.

    Kumar, R.; Mathur, A.; Kumar, A.(State University of New York at Buffalo, Buffalo, USA); Sharma, S.; Saksena, P. N.; Chaturvedi, U. C.

    1988-01-01

    Eighty-six randomly selected children between 6 months and 12 years of age admitted with acute unexplained encephalopathy over a one year period were examined for evidence of Japanese encephalitis. One or more indicators of the infection were present in 36 (41.8%). Viral isolation from brain tissue was possible in 2 of 12 patients and from cerebrospinal fluid in 19 out of 62 patients. Serological evidence of probable Japanese encephalitis was found in 21 out of 36 patients. Japanese encephali...

  20. Acute myocardial ischemia in adults secondary to missed Kawasaki disease in childhood

    Rizk, SRY; El Said, G; Daniels, LB; Burns, JC; El Said, H; Sorour, KA; Gharib, S; Gordon, JB

    2015-01-01

    © 2015 Elsevier Inc. All rights reserved. Coronary artery aneurysms that occur in 25% of untreated Kawasaki disease (KD) patients may remain clinically silent for decades and then thrombose resulting in myocardial infarction. Although KD is now the most common cause of acquired heart disease in children in Asia, the United States, and Western Europe, the incidence of KD in Egypt is unknown. We tested the hypothesis that young adults in Egypt presenting with acute myocardial ischemia may have ...

  1. Post-induction residual leukemia in childhood acute lymphoblastic leukemia quantified by PCR correlates with in vitro prednisolone resistance

    Schmiegelow, K; Nyvold, C; Seyfarth, J;

    2001-01-01

    Most prognostic factors in childhood acute lymphoblastic leukemia (ALL) are informative for groups of patients, whereas new approaches are needed to predict the efficacy of chemotherapy for the individual patient. The residual leukemia following 4 weeks of induction therapy with prednisolone......, vincristine, doxorubicin and i.t. methotrexate and the in vitro resistance to prednisolone, vincristine, and doxorubicin were measured in 30 boys and 12 girls with B (n = 34) or T lineage (n = 8) ALL. The residual leukemia was quantified after 2 (MRD-D15, n = 29) and 4 weeks (MRD-PI, n = 42) of induction...... more pronounced when B cell precursor and T cell leukemia were analyzed separately (B cell precursor ALL: MRD-PI vs prednisolone LC50: n = 33, rs = 0.47, P = 0.006; T cell ALL: MRD-PI vs prednisolone resistance: n = 8, rs = 0.84, P = 0.009). After a median follow-up of 5.0 years (75% range 3...

  2. Absolute lymphocyte count at the end of induction therapy is a prognostic factor in childhood acute lymphoblastic leukemia.

    Hirase, Satoshi; Hasegawa, Daiichiro; Takahashi, Hironobu; Moriwaki, Kensuke; Saito, Atsuro; Kozaki, Aiko; Ishida, Toshiaki; Yanai, Tomoko; Kawasaki, Keiichiro; Yamamoto, Nobuyuki; Kubokawa, Ikuko; Mori, Takeshi; Hayakawa, Akira; Nishimura, Noriyuki; Nishio, Hisahide; Iijima, Kazumoto; Kosaka, Yoshiyuki

    2015-11-01

    Recent studies have reported that the absolute lymphocyte count (ALC) during induction therapy is predictive of treatment outcome in de novo acute lymphoblastic leukemia (ALL); however, the significance of ALC on outcomes remains controversial. In the present study, we assessed the significance of ALC at day 29 (ALC-29), the end of induction therapy, on outcomes in our Japanese cohort. The outcomes of 141 patients aged ≤18 years with newly diagnosed ALL who were enrolled on the JACLS ALL-02 at our hospitals were analyzed in terms of ALC-29. Patients with ALC-29 ≥750/μL (n = 81) had a superior 5-year EFS (95.2 ± 2.7 vs 84.3 ± 4.8 %, P = 0.016) and OS (100 vs 87.0 ± 4.7 %, P = 0.0062). A multivariate analysis identified ALC-29 ≥750/μL as a significant predictor of improved EFS and OS after controlling for confounding factors. A multiple linear regression model revealed a significant inverse relationship between the percentage of blasts in bone marrow on day 15 and ALC-29 (P = 0.005). These results indicate that ALC is a simple prognostic factor in childhood ALL, and, thus, has the potential to refine current risk algorithms. PMID:26440971

  3. An adult patient who developed malignant fibrous histiocytoma 9 years after radiation therapy for childhood acute lymphoblastic leukemia

    Kato, Yasuhiro [National Hiroshima Hospital, Higashi-Hiroshima (Japan); Ohno, Norioki; Horikawa, Yoko; Nishimura, Shin-ichiro; Ueda, Kazuhiro; Shimose, Shoji [Hiroshima Univ. (Japan). School of Medicine

    2002-12-01

    A 24-year-old Japanese man with a history of acute lymphoblastic leukemia, which occurred during childhood, developed malignant fibrous histiocytoma of his left knee. His past history revealed that he had undergone leukemic blast cell invasion of the left knee and subsequent radiation therapy 9 years ago. The total radiation doses for the upper part of the left tibia and the lower part of the left femur were 60 Gy and 40 Gy, respectively. Neither distant metastasis nor a relapse of leukemia occurred. A curative resection of the left femur with a noninvasive margin was performed. Adjuvant chemotherapy including high-dose methotrexate was given successfully before and after surgery; this was followed by relapse-free survival for 3 years. The nature of postirradiation malignant fibrous histiocytoma is highly aggressive. When a patient complains of persistent symptoms in a previously irradiated field, the possibility of this tumor must be taken into account. The importance of early diagnosis cannot be over-emphasized. (author)

  4. Clofarabine-based combination chemotherapy for relapse and refractory childhood acute lymphoblastic leukemia.

    Arakawa, Yuki; Koh, Katsuyoshi; Aoki, Takahiro; Kubota, Yasuo; Oyama, Ryo; Mori, Makiko; Hayashi, Mayumi; Hanada, Ryoji

    2014-11-01

    Clofarabine, one of the key treatment agents for refractory and relapsed acute lymphoblastic leukemia (ALL), achieves a remission rate of approximately 30% with single-agent clofarabine induction chemotherapy. However, a remission rate of approximately 50% was reported with a combination chemotherapy regimen consisting of clofarabine, etoposide, and cyclophosphamide. We treated two cases with refractory and relapsed ALL with combination chemotherapy including clofarabine; one was an induction failure but the other achieved remission. Both cases developed an infectious complication (NCI-CTCAE grade 3) and body pain with infusion. Prophylactic antibiotic and opioid infusions facilitated avoiding septic shock and pain. Further investigation of such cases is required. PMID:25501414

  5. Risk-adapted stratification and treatment of childhood acute lymphoblastic leukaemia

    Systematic enrolment of children and adolescents with acute lymphoblastic leukaemia (ALL) into clinical trials has allowed the establishment of prognostic parameters derived from initial diagnostic findings. More important, these trials have significantly contributed to the reduction of disease recurrence as much as to the reduction of acute and late side effects. Some problems that are related to the specificity of the parameters used for risk assessment were not overcome: high tumour load by white blood cell count (WBC), age and (rare) cytogenetic subtypes (e.g. t9;22) may characterise a significant proportion of children and adolescents with high-risk ALL. Most patients who will eventually relapse do not present with characteristic features at initial diagnosis. It appears feasible through careful response assessment to identify these patients at risk of relapse, who present initially without specific features. Earlier trials of the ALL-BFM (Berlin/Frankfurt/Muenster) study group and others have demonstrated that inadequate leukaemic blast reduction in the peripheral blood or bone marrow after the first few days of therapy is highly predictive of treatment failure. Using clone-specific polymerase chain reaction-based detection of minimal residual disease (MRD) as done in trial AIEOP-BFM ALL 2000 allowed a close surveillance of specific treatment elements when applied in MRD positive patients. This may facilitate innovative chemotherapy approaches and a more rational use of allogeneic haematopoietic stem cell transplantation. In addition, genetic signatures of treatment response or failure have been identified. (authors)

  6. Acute Motor Axonal Neuropathy (Aman) With Motor Conduction Blocks In Childhood; Case Report.

    Yildirim, Serhan; Adviye, Rahşan; Gül, Hakan Levent; Türk Börü, Ülkü

    2016-01-01

    Objective Acute motor axonal neuropathy (AMAN), characterized with decreased compound muscle action potentials (CMAP) and absence of demyelinating findings in electrophysiological studies, is a subtype of Guillain-Barre Syndrome (GBS). A 4 yr-old male patient presented with ascending weakness, dysarthria and dysphagia to İstanbul Dr. Lütfi Kırdar Kartal Training and Research Hospital Neurology outpatient for three days to in 2012. Dysphonia, restricted eye movements, flaccid tetraplegia and areflexia were found in neurological examination. There were motor conduction blocks in all peripheral nerves in electrophysiological studies.According to these findings the patient was diagnosed as Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP). Reduction of CMAP amplitudes in posterior tibial nerve, absence of CMAPs in median, ulnar and peroneal nerves and loss of motor conduction blocks were found in following electrophysiological studies. According to these findings, patient was diagnosed as AMAN. Motor conduction blocks may appear in early stage of AMAN and they disappear in later examinations. That's why electrophysiological studies must be repeated in patients with GBS. PMID:27057191

  7. Childhood acute lymphoblastic leukaemia: experience from a single tertiary care facility of Pakistan

    Objective: To evaluate the demographic features, outcome and prognostic factors seen in children with acute lymphoplastic leukaemia at a tertiary care hospital. Methods: The retrospective descriptive study was conducted at Aga Khan University Hospital, Karachi, comprising data related to children below 15 years of age and treated between January 1997 and December 2006. Kaplan Meir survival curves were used to describe overall and event-free survival rates. Cox Proportional Hazards model was used to describe factors associated with death and relapse. SPSS 16 was the main statistical tool. Results: Of the total 121 children diagnosed with the condition, 79 (65.3%) were males; 86 (71.1%) patients were between 1-9 years of age; Immunophenotyping was done in 99 (81.81%) patients: 86 (87%) cases had precursor B and 13 (13.13%) had precursor T. Of the total, 106(87.6%) patients opted for treatment, while 15 (11.6%) were lost to follow-up. Besides, 26(21.7%) patients had at least one relapse; the most common site being bone marrow in 13 (50%) followed by central nervous system in 9 (36.6%). There were 20(16.5%) deaths in the sample. Infection was the most frequent cause of death. The event-free survival and overall survival was 63% (n=76) and 65% (n=79) respectively. Conclusion: Through the clinical characteristics of children with acute lymphoblastic leukamia were similar to those reported in literature, the outcomes were inferior. The high rate of infections and relapse warrant better supportive care and risk-based approach. (author)

  8. Human Parvovirus B19 in childhood acute lymphoblastic leukaemia in basrah

    Objective: To investigate the association of human parvovirus B19 infection with the onset of acute lymphoblastic leukaemia and its effect on TEL-AML-1 fusion gene and the presence of mutant P53. Methods: The case-control study was conducted at Basrah Hospital for Paediatrics and Gynaecology, Basrah, Iraq, from May 2009 to April 2010. A total of 100 blood samples were collected from 40 newly diagnosed cases and 60 healthy children to serve as control matched by age and gender. Human parvovirus B19-IgG and anti-P53 antibody were detected by enzyme-linked immunosorbent assay and TEL-AML-1 fusion gene was detected by reverse transcriptase-polymerase chain reaction on extracted ribonucleic acid from fresh blood samples using specified primers. SPSS 15 was used for statistical analysis. Results: A higher proportion of human parvovirus B19-positive cases was found in leukaemic patients (n=19; 47.5%) compared to 12 (20%) in the control group (p<0.05). There was significant association between Tel-Amyl-1 translocation and human parvovirus-B19 infection as 10 (71.4%) of TEL-AML-1 translocation-positive cases had human parvovirus-B19 IgG. On the other hand, there was no association between such infections and P53 gene mutation in the patients. Conclusion: Human parvovirus-B19 infection is common in the population, with higher prevalence among leukaemic patients with significant association between human parvovirus-B19 and TEL-AML-1 fusion gene in patients of acute lymphoblastic leukaemia. (author)

  9. Signal recognition particle (SRP) positive myositis in a patient with cryptogenic organizing pneumonia (COP)

    Wantke, Felix; Kneussl, Meinhard; Hubner, Marianne; Derfler, Kurt; Brücke, Thomas; Schmaldienst, Sabine

    2009-01-01

    Abstract We report of a 46-year-old female patient with cryptogen organizing pneumonia preceeding the rare SRP positive necrotising myositis without cardiac involvement and no sign of dysphagia. Myositis showed full regression without oral immune suppression but with extracorporeal treatment, performed as a combined therapy of plasmaexchange and immunoadsorption. After 33-month of treatment, anti-SRP antibodies were not detectable any more.

  10. Myositis complicating benzathine penicillin-G injection in a case of rheumatic heart disease

    Joshua R. Francis

    2016-01-01

    Full Text Available A 7-year old boy developed myositis secondary to intramuscular injection of benzathine penicillin-G in the context of secondary prophylaxis for rheumatic heart disease. Side effects of intramuscular delivery of benzathine penicillin-G are well described and include injection site pain and inflammation, but myositis, as depicted on magnetic resonance imaging in this case, has not previously been described.

  11. Core biopsy as a simple and effective diagnostic tool in head and neck focal myositis.

    Tan, Chun Yee; Chong, Sheldon; Shaw, Chi-Kee Leslie

    2015-12-01

    Most unilateral head and neck masses are benign, although malignancy is a possibility in some cases. However, there are other rare causes of unilateral neck masses, such as focal myositis, which is a rare, benign condition belonging to the family of inflammatory pseudotumors of the skeletal muscles, with rare presentations in the head and neck region. Focal myositis presents as a rapidly enlarging neck mass that can be misdiagnosed by fine-needle aspiration biopsy and/or radiologic imaging as either an infective or a neoplastic process. To date, there are only 5 reported cases of adult focal myositis of the sternocleidomastoid muscle in the medical literature. In this article, the authors present 2 cases involving patients with focal myositis of the sternocleidomastoid muscle that were successfully diagnosed with core-needle biopsy and managed conservatively. The pros and cons of fine-needle aspiration biopsy and core-needle biopsy are discussed. Based on the authors' results, fine-needle aspiration biopsy universally fails to provide the diagnosis of focal myositis. In contrast, core-needle biopsy successfully diagnosed focal myositis in both of our patients. Both of them had complete resolution with conservative management. PMID:26670758

  12. Increased μ-Calpain Activity in Blasts of Common B-Precursor Childhood Acute Lymphoblastic Leukemia Correlates with Their Lower Susceptibility to Apoptosis.

    Anna Mikosik

    Full Text Available Childhood acute lymphoblastic leukemia (ALL blasts are characterized by inhibited apoptosis promoting fast disease progress. It is known that in chronic lymphocytic and acute myeloid leukemias the reduced apoptosis is strongly related with the activity of calpain-calpastatin system (CCS composed of cytoplasmic proteases--calpains--performing the modulatory proteolysis of key proteins involved in cell proliferation and apoptosis, and of their endogenous inhibitor--calpastatin. Here, the CCS protein abundance and activity was for the first time studied in childhood ALL blasts and in control bone marrow CD19+ B cells by semi-quantitative flow cytometry and western blotting of calpastatin fragments resulting from endogenous calpain activity. Significantly higher μ-calpain (CAPN1 gene transcription, protein amounts and activity (but not those of m-calpain, with calpastatin amount and transcription of its gene (CAST greatly varying were observed in CD19(+ ALL blasts compared to control cells. Significant inverse relation between the amount/activity of calpain and spontaneous apoptosis was noted. Patients older than 10 years (considered at higher risk displayed increased amounts and activities of blast calpain. Finally, treatment of blasts with the tripeptide calpain inhibitors II and IV significantly and in dose-dependent fashion increased the percentage of blasts entering apoptosis. Together, these findings make the CCS a potential new predictive tool and therapeutic target in childhood ALL.

  13. Tubercular myositis of infraspinatus: a rare clinical entity

    Vikas Verma

    2016-08-01

    Full Text Available Tuberculosis of the musculoskeletal system is generally confined to bones and joints. The surrounding soft tissue is secondarily infected. Tuberculous bursitis, tenosynovitis and primary pyomyositis are rarer manifestations of the disease. Of these, primary tuberculouspyomyositis is probably the rarest entity. We report a case of tubercular myositis of infraspinatus in an 8 year-old female who presented with pain, low grade fever, weight loss, anorexia, progressively increasing pain in the scapular region and restriction of movements. There was no history of trauma, diabetes, immunosuppression, corticosteroid usage, or renal failure. History of contact was present. Tenderness was present along the medial border of scapula and movements of upper extremity requiring movement of the scapula were painful and grossly restricted. MRI of the scapulothoracic region and shoulder revealed small amount of fluid along medial border of scapula with T2 hyperintensity of infraspinatus. Histopathology showed caseous necrosis, inflammatory cells and granulomatous cells suggestive of tuberculosis. Polymerase Chain Reaction for Mycobacterium tuberculosis was found to be positive. Patient was started on four-drug antitubercular treatment and regular dressings. The patient's general condition improved and at 4 weeks post starting ATT, there was no pain and the patient was able to perform complete range of movement. This is probably the first reported case of tubercular myositis of infraspinatus in an immunocompetent patient without any identifiable focus elsewhere in the body. Rarity of the condition, presence of characteristic findings on MRI and histopathology make the case illustrative for young Orthopaedics surgeons. [Int J Res Med Sci 2016; 4(8.000: 3619-3621

  14. Post chemotherapy blood and bone marrow regenerative changes in childhood acute lymphoblastic leukemia a prospective study

    Rashmi Kushwaha

    2014-01-01

    Full Text Available Context: This study was done to assess the Serial peripheral blood and bone marrow changes in patients of Acute Lymphoblastic Leukemia on chemotherapy. Aims: To assess the therapy related serial bone marrow changes in patients of Acute Lymphoblastic Leukemia. Settings and Design: Prospective study, carried out in Lymphoma- Leukemia Lab, Department of Pathology, K.G.M.U from March 2011 to March 2012. A total of 60 cases were studied Materials and Methods: History, complete hemogram, bone marrow examination at pretherapy (Day-0, intratherapy (Day-14, and end of induction chemotherapy (Day-28 were done. Peripheral blood smears were evaluated at regular interval to assess clearance of blast cells. Statistical analysis used: The statistical analysis was done using SPSS (Statistical Package for Social Sciences Version 15.0 statistical Analysis Software. The values were represented in Number (% and Mean ± SD. The following Statistical formulas were used: Mean, standard deviation, Chi square test, Paired "t" test, Student ′t′ test, Level of significance P Results: Incidence of ALL-L1 (46.7% and ALL-L2 (53.3% was equal. ALL-L2 patients had poor survival.Day 0 (D-0 bone marrow was hypercellular with flooding of marrow by leukemic cells. High levels of tumor load at D′0′ were associated with poor survival. 14 th day of Induction phase showed significant decrease in hemoglobin and TLC as compared to D ′0′ parameters. D28 showed marrow regeneration. Cellularity, Blast%, and Leukemic Index showed significant drop from day ′0′ to day 14 due to myelosupression, whereas regeneration reflected by increased cellularity as per day 28 marrow. Lymphocytosis (>20% at end of induction chemotherapy had better survival and longer remission.Risk of mortality was directly proportional to blast clearance and was a major independent prognostic factor for achievement of complete remission. Conclusions: A bone marrow examination at the end of induction

  15. Cytometric evaluation of transferrin receptor 1 (CD71 in childhood acute lymphoblastic leukemia

    Anna Płoszyńska

    2012-07-01

    Full Text Available Transferrin receptor 1 (CD71 is a transmembrane glycoprotein responsible for cellular iron uptake. Higher expression of CD71 has been identified as a negative prognostic marker for numerous solid tumor types and for some lymphomas. The aim of this study was to evaluate CD71 expression on acute lymphoblastic leukemia (ALL cells and to follow its possible clinical correlations. Sixty one patients, aged 1–17 years and diagnosed with ALL, were enrolled in the study. CD71 expression was analyzed on the bone marrow blastic cells by flow cytometry. CD71 expression on the  eukemic blasts was diversified; in most patients, all blastic cells showed expression of CD71, but levels of expression varied. CD71 expression was statistically higher on T-lineage leukemias. Within the B lineage ALL, a significant difference in CD71 expression existed between precursor B ALL and mature B-ALL, which showed higher CD71 expression. CD71 expression positively correlated with Hgb concentration at diagnosis. Initial risk group assessment and therapy response were not correlated with CD71 expression, although disease free and overall survival times tended to be shorter in patients with B-lineage leukemias with initial high CD71 expression.

  16. Effect of methotrexate and doxorubicin cumulative doses on superoxide dismutase levels in childhood acute lymphoblastic leukemia

    Khalida Fetriyani Ningsih

    2015-09-01

    Full Text Available Background Acute lymphoblastic leukemia (ALL is the most common malignancy in children. Chemotherapeutic drugs for ALL such as methotrexate (Mtx and doxorubicin produce reactive oxygen species (ROS, a type of free radical. The ROS can reduce levels of antioxidants in the body, including superoxide dismutase (SOD. Decreased SOD levels can cause DNA, lipid, and protein damage, which in turn may lead to adverse effects and treatment failure. Objective To determine the effect of Mtx and doxorubicin cumulative doses on SOD levels in children with ALL. Methods We conducted a retrospective cohort study in children with ALL who underwent chemotherapy in Dr. Sardjito Hospital in October 2011 who had completed the induction phase. Risk factors for decreased SOD levels were analyzed by Cox regression and hazard ratio, with a significant level of P <0.05. Results Of 40 patients enrolled, Mtx ≥ 3000 mg/m2 significantly decreased SOD levels (HR 9.959; 95%CI 2.819 to 35.183; P=0.001. However, doxorubicin ≥90 mg/m2 did not significantly decrease SOD levels (HR 0.59 95%CI 0.194 to 1.765; P=0.34. Conclusion Methotrexate is associated with decreased SOD levels in children with ALL. However, doxorubicin is not associated with decreased SOD levels in the same patient population.

  17. [Endocrinologic function following cranial irradiation in acute lymphoblastic leukemia in childhood].

    Lackner, H; Schwingshandl, J; Pakisch, B; Knoblauch, S; Mutz, I; Urban, C

    1991-01-01

    Endocrinological function was evaluated in 31 children after successful treatment of acute lymphoblastic leukaemia. All patients had received combination chemotherapy and 12-24Gy of cranial irradiation according to the German therapy protocols BFM-81, BFM-83 and BFM-86. Height, weight, bone age and pubertal development, as well as hypothalamic-pituitary function were measured. Long-term linear growth was unaffected in all patients. However, 9 patients showed subnormal serum growth hormone levels in response to pharmacological stimulation of the pituitary. All patients had normal levels of T3 and T4, but one patient showed an increased response of thyrotropin to thyrotropin releasing hormone. All prepubertal and postpubertal children demonstrated appropriate secretion of follicle-stimulation hormone (FSH) and luteinizing hormone (LH) after stimulation with LH-releasing hormone (LH-RH). 3 pubertal girls showed adequate oestradiol levels, but abnormally high levels of gonadotropins in response to LH-RH. Sexual development was normal in two of them, but the third had a late menarche and irregular menses. The significance of these findings is discussed in the context of recommendations possibly to further reduce or completely delete prophylactic cranial irradiation. PMID:1759459

  18. Meningosis prophylaxis with intrathecal /sup 198/Au-colloid and methotrexate in childhood acute lymphocytic leukemia

    Since 1972, telecobalt irradiation plus intrathecal methotrexate (ITMTX) has been successfully replaced in Jena by intrathecal colloidal radioactive gold (/sup 198/Au) plus ITMTX for meningosis prophylaxis in leukemia. Seventy-three children with acute lymphocytic leukemia (ALL) were given 1.24-4.89 mCi (45.8-181 MBq) of colloidal 198Au IT after successful initiation of remission. During cytostatic therapy, the following relapses occurred: meningosis leucaemica, five patients (6.8%); bone-marrow relapse and the meningosis leucaemica, one patient; and bone-marrow relapse, 20 patients (27.4%). In 18 children, combination chemotherapy was terminated after two and a half or three years of treatment. After that time, one meningeal relapse and six bone-marrow relapses occurred. Within the first 24 hours after application of radioactive gold, headaches, vomiting, and fever occurred in less than 10% of the children. An apathy syndrome, leukecephalopathy, or severe infections, were not observed in a single case. Radioactive gold spreads in the subarachnoid space and is phagocytized by the arachnoidea. The tumoricide effect extends selectively over the space of distribution of the latent meningosis leucaemia. The cerebral parenchyma remains unaffected by radiation. Thus, radioactive gold may be preferable to telecobalt irradiation in preventing central nervous system leukemia

  19. The development of cerebral CT changes during treatment of acute lymphocytic leukemia in childhood

    Twenty-three children with acute lymphocytic leukemia (ALL) were examined with cranial CT at least twice with a minimal interval of 10 months. The first CT was performed at the time of diagnosis in 11 children and during therapy in 12; all but two were normal on the first CT examination. These two had slight enlargement of the ventricular system and subarachnoid space at the time of diagnosis. These findings were unchanged on the second CT examinations. Seven patients, all in remission from leukemia of the central nervous system manifested abnormal findings on later CTs. Low density areas in the periventricular white matter were seen in the brains of three, with increasing subcortical calcification in one of these cases. Five children had slight enlargement of the ventricular system and subarachnoid space, especially of the basal and Sylvian cisterns. Later CT examinations in five, plus brain autopsy in two cases, revealed unchanged or progressive conditions. The CT findings have been related to the treatment and some characteristics of the disease. The frequency of CT abnormalities was higher in patients who had received therapeutic irradiation and intraventricular methotrexate treatment. The possible reasons for the CT abnormalities are discussed. (orig.)

  20. Psychological Impact of Chemotherapy for Childhood Acute Lymphoblastic Leukemia on Patients and Their Parents.

    Sherief, Laila M; Kamal, Naglaa M; Abdalrahman, Hadel M; Youssef, Doaa M; Abd Alhady, Mohamed A; Ali, Adel S A; Abd Elbasset, Maha Aly; Hashim, Hiatham M

    2015-12-01

    To assess the self-esteem of pediatric patients on chemotherapy for acute lymphoblastic leukemia (ALL) and psychological status of their parents.The psychological status of 178 children receiving chemotherapy for ALL and their parents was assessed using parenting stress index (PSI) to determine the degree of stress the parents are exposed to using parent's and child's domains. Self-esteem Scale was used to determine the psychological status of patients.The study revealed significant low level of self-esteem in 84.83% of patients. Their parents had significant psychological stress. PSI was significantly associated with parents' low sense of competence, negative attachment to their children, feeling of high restriction, high depression, poor relation to spouse, high social isolation variables of parent's domains. It was significantly associated with low distraction, negative parents' reinforcement, low acceptability, and high demanding variables of child's domains. Long duration of disease was the most detrimental factor among demographic data of the patients.Chemotherapy for ALL has a significant impact on the psychological status of both patients and their parents with high prevalence of low self-esteem in children and high degree of stress in their parents. PMID:26705211

  1. EVALUATION OF SURFACE MARKERS IN CHILDHOOD ACUTE LYMPHOCYTIC LEUKEMIA BEFORE AND AFTER THERAPY

    A.Massoud

    1980-10-01

    Full Text Available In our study of surface markers of Iranian children affected by acute lymphocytic leukemia, the majority(80 %had non-T, non-B, or null cell leukemia. The null- cell l e ukemia had a better p r ogno s l s as was conf irmed by o t h ers. Twe l v e of our patie nts who had null cell l e ukem i a are a live and well 18 mont hs to 20 mont hs a f t e r initiat i on of the r a py. Three of t he patients who had l e s s than 27' null c e l l s in t he i nitial study died i n a pe r i od o f less than o ne year after d i agnosis. Af t e r therapy while on remission , there was a decrease i n the percentage o f nu l l c e l ls ( i n nul l c e l l l e ukemi a a nd an i ncre ase i n t h e p e r centage of T a nd B lymphocyt e s i n the pe r i phe r a l b lood which approachta o n the a ge and sex-matched norma l control s ubjects .

  2. Childhood acute pyelonephritis: comparison of power Doppler sonography and Tc-DMSA scintigraphy

    Tc 99m DMSA scintigraphy is regarded as the gold standard for the detection and localization of acute pyelonephritis (APN) in children. Power Doppler sonography (PD US) is a radiation-free and cost-effective technique that could be useful in the diagnosis of APN in children. To compare the predictive value of PD US with DMSA scintigraphy in the diagnosis of APN in children. A total of 74 neonates and children with clinical findings consistent with possible upper urinary tract infection were evaluated with PD US and DMSA scintigraphy. Children with anatomic (grey-scale) abnormalities were excluded. A total of 147 kidneys were examined within the first 48 h after the onset of symptoms. Each kidney was divided into three zones (upper, middle, and lower third). APN was diagnosed by PD US in 46 kidneys. Sensitivity and specificity for detecting APN using DMSA scintigraphy as the reference standard were 73.8% and 85.7%, respectively. There was good agreement between PD US and DMSA scintigraphy in the localization of lesions. In clinically suspected APN, PD US has acceptable specificity and sensitivity, if performed within the first 48 h and could be helpful in neonates and children under 3 months of age in whom the use of scintigraphy is generally discouraged. (orig.)

  3. Renal, gastrointestinal, and hepatic late effects in survivors of childhood acute myeloid leukemia treated with chemotherapy only--a NOPHO-AML study

    Skou, Anne-Sofie; Glosli, Heidi; Jahnukainen, Kirsi; Jarfelt, Marianne; Jónmundsson, Guðmundur K; Malmros-Svennilson, Johan; Nysom, Karsten; Hasle, Henrik

    2014-01-01

    performed. Eighty-five of 94 (90%) eligible sibling controls completed a similar questionnaire. Siblings had no clinical examination or blood sampling performed. RESULTS: At a median of 11 years (range 4-25) after diagnosis, renal, gastrointestinal, and hepatic disorders were rare both in survivors of......BACKGROUND: We investigated the spectrum, frequency, and risk factors for renal, gastrointestinal, and hepatic late adverse effects in survivors of childhood acute myeloid leukemia (AML) without relapse treated with chemotherapy alone according to three consecutive AML trials by the Nordic Society...... childhood AML and in sibling controls, with no significant differences. Ferritin was elevated in 21 (21%) AML survivors but none had biochemical signs of liver damage. Viral hepatitis was present in three and cholelithiasis in two AML survivors. One adult survivor had hypertension, two had slightly elevated...

  4. Recent advances in the diagnosis and management of childhood acute promyelocytic leukemia

    Eun Sun Yoo

    2011-03-01

    Full Text Available Since the successful introduction of all-trans-retinoic acid (ATRA and its combination with anthracycline-containing chemotherapy, the prognosis for acute promyelocytic leukemia (APL has markedly improved. With ATRA and anthracycline-based-chemotherapy, the complete remission rate is greater than 90%, and the long-term survival rate is 70&#8210;89%. Moreover, arsenic trioxide (ATO, which was introduced for APL treatment in 1994, resulted in excellent remission rates in relapsed patients with APL, and more recently, several clinical studies have been designed to explore its role in initial therapy either alone or in combination with ATRA. APL is a rare disease in children and is frequently associated with hyperleukocytosis, which is a marker for higher risk of relapse and an increased incidence of microgranular morphology. The frequency of occurrence of the promyelocytic leukemia/ retinoic acid receptor-alpha (PML/RAR?#6752;isoforms bcr 2 and bcr 3 is higher in children than in adults. Although recent clinical studies have reported comparable long-term survival rates in patients with APL, therapy for APL in children is challenging because of the risk of early death and the potential long-term cardiac toxicity resulting from the need to use high doses of anthracyclines. Additional prospective, randomized, large clinical trials are needed to address several issues in pediatric APL and to possibly minimize or eliminate the need for chemotherapy by combining ATRA and ATO. In this review article, we discuss the molecular pathogenesis, diagnostic progress, and most recent therapeutic advances in the treatment of children with APL.

  5. Back pain and vertebral compression: an unusual presentation of childhood acute lymphoblastic leukemia.

    Hafiz, M G; Islam, A; Siddique, R

    2010-01-01

    Junayet, a nine years and six months old boy was admitted to the hospital because of back pain and vertebral compression fractures. The boy had been well until two months earlier, when he began to have back pain after falling on his back along with occasional fever. The pain was intermittent initially but gradually it became constant. One month before admission, he fell again and the back pain became deteriorated. He was mildly pale, liver was palpable, skin survey revealed normal, BCG scar mark was present. He had bone pain, cervical lymphadenopathy and a tender swelling on the lumbusacral region. Two weeks before admission, the hematological findings were suggestive of leukemia of lymphoblastic type. Biochemical values were normal except high level of serum lactate dehydrogenase (LDH). Cerebrospinal fluid (CSF) examination was free of malignant cell. Skeletal survey showed diffuse osteopenia of the thoracic and lumber spine with multiple compression fracture of the vertebral bodies of D7, D8, D12 and L1, L3 and L5 with increased disc space. Radiograph of the chest also showed diffuse osteopenia of ribs. Magnetic resonance (MRI) showed uniform signal intensity in the marrow throughout the spine with compressed fracture of the same vertebrae. Bone marrow morphology study and the cytochemistry of the aspirated marrow were consistent with acute lymphoblastic leukemia (ALL-L2). Then, he was started protocol based chemotherapy for induction of remission, consolidation, high dose methotrexate and maintenance therapy. Now, he is on regular follow up with repeated hematological and radiological examinations. Following six month of chemotherapy the boy was found with significant improvement of his physical, hematological and radiological abnormalities. PMID:20046187

  6. Spectrum of centrosome autoantibodies in childhood varicella and post-varicella acute cerebellar ataxia

    Stinton Laura M

    2003-09-01

    Full Text Available Abstract Background Sera from children with post-varicella infections have autoantibodies that react with centrosomes in brain and tissue culture cells. We investigated the sera of children with infections and post-varicella ataxia and related conditions for reactivity to five recombinant centrosome proteins: γγ-enolase, pericentrin, ninein, PCM-1, and Mob1. Methods Sera from 12 patients with acute post-varicella ataxia, 1 with post-Epstein Barr virus (EBV ataxia, 5 with uncomplicated varicella infections, and other conditions were tested for reactivity to cryopreserved cerebellum tissue and recombinant centrosome proteins. The distribution of pericentrin in the cerebellum was studied by indirect immunofluorescence (IIF using rabbit antibodies to the recombinant protein. Antibodies to phospholipids (APL were detected by ELISA. Results Eleven of 12 children with post-varicella ataxia, 4/5 children with uncomplicated varicella infections, 1/1 with post-EBV ataxia, 2/2 with ADEM, 1/2 with neuroblastoma and ataxia, and 2/2 with cerebellitis had antibodies directed against 1 or more recombinant centrosome antigens. Antibodies to pericentrin were seen in 5/12 children with post-varicella ataxia but not in any of the other sera tested. IIF demonstrated that pericentrin is located in axons and centrosomes of cerebellar cells. APL were detected in 75% of the sera from children with post-varicella ataxia and 50% of children with varicella without ataxia and in none of the controls. Conclusion This is the first study to show the antigen specificity of anti-centrosome antibodies in children with varicella. Our data suggest that children with post-varicella ataxia have unique autoantibody reactivity to pericentrin.

  7. Garlic compounds selectively kill childhood pre-B acute lymphoblastic leukemia cells in vitro without reducing T-cell function: Potential therapeutic use in the treatment of ALL

    Greg Hodge

    2008-03-01

    Full Text Available Greg Hodge1, Stephen Davis2, Michael Rice1, Heather Tapp1, Ben Saxon1, Tamas Revesz11Haematology/Oncology Department, Women’s and Children’s Hospital, North Adelaide, Australia; 2Department of Mycology, Women’s and Children’s Hospital, North Adelaide, AustraliaAbstract: Drugs used for remission induction therapy for childhood precursor-B acute lymphoblastic leukemia (ALL are nonselective for malignant cells. Several garlic compounds have been shown to induce apoptosis of cancer cells and to alter lymphocyte function. To investigate the effect of garlic on the apoptosis of ALL cells and lymphocyte immune function, cells from newly diagnosed childhood ALL patients were cultured with several commonly used chemotherapeutic agents and several garlic compounds. Apoptosis, lymphocyte proliferation and T-cell cytokine production were determined using multiparameter flow cytometry. At concentrations of garlic compounds that did not result in significant increases in Annexin V and 7-AAD staining of normal lymphocytes, there was a significant increase in apoptosis of ALL cells with no alteration of T-cell proliferation as determined by CD25/CD69 upregulation or interferonγ, interleukin-2 or tumor necrosis factor-α intracellular cytokine production. In contrast, the presence of chemotherapeutic agents resulted in nonselective increases in both lymphocyte and ALL apoptosis and a decrease in T-cell proliferation and cytokine production. In conclusion, we show selective apoptosis of malignant cells by garlic compounds that do not alter T-cell immune function and indicate the potential therapeutic benefit of garlic compounds in the treatment of childhood ALL.Keywords: childhood precursor-B acute lymphoblastic leukemia, garlic, apoptosis, immune function, intracellular cytokines

  8. Temsirolimus, Dexamethasone, Mitoxantrone Hydrochloride, Vincristine Sulfate, and Pegaspargase in Treating Young Patients With Relapsed Acute Lymphoblastic Leukemia or Non-Hodgkin Lymphoma

    2015-07-09

    Childhood B Acute Lymphoblastic Leukemia; Childhood T Acute Lymphoblastic Leukemia; Mature T-Cell and NK-Cell Non-Hodgkin Lymphoma; Recurrent Childhood Acute Lymphoblastic Leukemia; Recurrent Childhood Lymphoblastic Lymphoma

  9. Heterogeneous cytogenetic subgroups and outcomes in childhood acute megakaryoblastic leukemia: a retrospective international study.

    Inaba, Hiroto; Zhou, Yinmei; Abla, Oussama; Adachi, Souichi; Auvrignon, Anne; Beverloo, H Berna; de Bont, Eveline; Chang, Tai-Tsung; Creutzig, Ursula; Dworzak, Michael; Elitzur, Sarah; Fynn, Alcira; Forestier, Erik; Hasle, Henrik; Liang, Der-Cherng; Lee, Vincent; Locatelli, Franco; Masetti, Riccardo; De Moerloose, Barbara; Reinhardt, Dirk; Rodriguez, Laura; Van Roy, Nadine; Shen, Shuhong; Taga, Takashi; Tomizawa, Daisuke; Yeoh, Allen E J; Zimmermann, Martin; Raimondi, Susana C

    2015-09-24

    Comprehensive clinical studies of patients with acute megakaryoblastic leukemia (AMKL) are lacking. We performed an international retrospective study on 490 patients (age ≤18 years) with non-Down syndrome de novo AMKL diagnosed from 1989 to 2009. Patients with AMKL (median age 1.53 years) comprised 7.8% of pediatric AML. Five-year event-free (EFS) and overall survival (OS) were 43.7% ± 2.7% and 49.0% ± 2.7%, respectively. Patients diagnosed in 2000 to 2009 were treated with higher cytarabine doses and had better EFS (P = .037) and OS (P = .003) than those diagnosed in 1989 to 1999. Transplantation in first remission did not improve survival. Cytogenetic data were available for 372 (75.9%) patients: hypodiploid (n = 18, 4.8%), normal karyotype (n = 49, 13.2%), pseudodiploid (n = 119, 32.0%), 47 to 50 chromosomes (n = 142, 38.2%), and >50 chromosomes (n = 44, 11.8%). Chromosome gain occurred in 195 of 372 (52.4%) patients: +21 (n = 106, 28.5%), +19 (n = 93, 25.0%), +8 (n = 77, 20.7%). Losses occurred in 65 patients (17.5%): -7 (n = 13, 3.5%). Common structural chromosomal aberrations were t(1;22)(p13;q13) (n = 51, 13.7%) and 11q23 rearrangements (n = 38, 10.2%); t(9;11)(p22;q23) occurred in 21 patients. On the basis of frequency and prognosis, AMKL can be classified to 3 risk groups: good risk-7p abnormalities; poor risk-normal karyotypes, -7, 9p abnormalities including t(9;11)(p22;q23)/MLL-MLLT3, -13/13q-, and -15; and intermediate risk-others including t(1;22)(p13;q13)/OTT-MAL (RBM15-MKL1) and 11q23/MLL except t(9;11). Risk-based innovative therapy is needed to improve patient outcomes. PMID:26215111

  10. The association of reduced folate carrier 80G>A polymorphism to outcome in childhood acute lymphoblastic leukemia interacts with chromosome 21 copy number

    Gregers, Jannie; Christensen, Ib Jarle; Dalhoff, Kim; Lausen, Birgitte Frederiksen; Schroeder, Henrik; Rosthoej, Steen; Carlsen, Niels; Schmiegelow, Kjeld; Peterson, Curt

    2010-01-01

    with chromosome 21 copy number in the leukemic clone. A total of 500 children with acute lymphoblastic leukemia treated according to the common Nordic treatment protocols were included, and we found that the RFC AA variant was associated with a 50% better chance of staying in remission compared with GG......The reduced folate carrier (RFC) is involved in the transport of methotrexate (MTX) across the cell membrane. The RFC gene (SLC19A1) is located on chromosome 21, and we hypothesized that the RFC80 G>A polymorphism would affect outcome and toxicity in childhood leukemia and that this could interact...

  11. ZAP-70 as A Possible Prognostic Factor in Childhood Acute Lymphoblastic Leukemia

    Background: Zeta-chain-associated protein (ZAP- 70) is a 70kD adaptor protein that acts quickly after T cell activation to propagate signal. The role of ZAP-70 in Tcell function is well established, and in the previous years, this molecule was considered to be T-cell specific. More recent data have documented a role of ZAP-70 in B cells. Interest in ZAP-70 has grown since it has been shown, through gene expression profiling, that it is expressed in a subset of cases of chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL). Purpose: The aim of this study was to investigate the expression of ZAP-70 in leukemic blasts of 50 newly diagnosed patients of B-lineage acute lymphoblastic leukemia (ALL), and to assess the correlation between ZAP-70 expression and various prognostic factors and outcome. Patients and Methods: This study included 50 pediatric patients with newly diagnosed B-lineage ALL. They were 28 males (56%) and 22 females (44%) presented to the Pediatric Oncology Department, National Cancer Institute, Cairo University, during the period from 2005 to 2007. The age range was 2 to 17 years with a mean of 8.58±5.8 years and median 8 years. All patients were subjected at presentation to a full clinical history and physical examination. Patients diagnosed with ALL were enrolled on St. Jude Total XV protocol: standard risk and low risk according to results of primary investigation. Immuno phenotyping was done using monoclonal antibodies which were analyzed on Coulter XL (Panel included CD1, CD2, CD3, CD4, CD5, CD7, CD8, CD10, CD19, CD22, Cytoplasmic m, anti k, anti l, CD13, CD33, anti classII MHC and TdT). Cases were considered ZAP-70 positive when exhibiting a ZAP/GAPDH (Glyceraldehyde- 3-phosphate dehydrogenase) ratio ³0.13. Results: The study revealed expression of ZAP-70 in 5/50 cases (10%). There was no statistically significant relation between ZAP-70 expression and the following: age, Total Leukocytic Count, hepatomegaly and splenomegaly. There

  12. Prevalence of Gene Rearrangements in Mexican Children with Acute Lymphoblastic Leukemia: A Population Study—Report from the Mexican Interinstitutional Group for the Identification of the Causes of Childhood Leukemia

    Vilma Carolina Bekker-Méndez; Enrique Miranda-Peralta; Juan Carlos Núñez-Enríquez; Irma Olarte-Carrillo; Francisco Xavier Guerra-Castillo; Ericka Nelly Pompa-Mera; Alicia Ocaña-Mondragón; Angélica Rangel-López; Roberto Bernáldez-Ríos; Aurora Medina-Sanson; Elva Jiménez-Hernández; Raquel Amador-Sánchez; José Gabriel Peñaloza-González; José de Diego Flores-Chapa; Arturo Fajardo-Gutiérrez

    2014-01-01

    Mexico has one of the highest incidences of childhood leukemia worldwide and significantly higher mortality rates for this disease compared with other countries. One possible cause is the high prevalence of gene rearrangements associated with the etiology or with a poor prognosis of childhood acute lymphoblastic leukemia (ALL). The aims of this multicenter study were to determine the prevalence of the four most common gene rearrangements [ETV6-RUNX1, TCF3-PBX1, BCR-ABL1, and MLL rearrangement...

  13. Elevated IgG antibody to Sarcocystis cruzi associated with eosinophilic myositis in cattle.

    Ely, R W; Fox, J C

    1989-01-01

    Blood sera, skeletal muscle, and cardiac muscle from 24 bovine carcasses condemned for eosinophilic myositis by US Department of Agriculture meat inspection veterinarians were compared with similar specimens from 35 random carcasses passed for human consumption. Fluorescence values determined by using a fluorometric immunoassay system were used to measure the specific antibodies to Sarcocystis cruzi. The values were significantly elevated in carcasses condemned for eosinophilic myositis as compared to carcasses passed for human consumption. The elevated fluorescence values appeared to be more than coincidental, suggesting that S. cruzi may be a causative agent of eosinophilic myositis. Microscopic examination of affected muscle revealed lesions typical of eosinophilic myositis. Lesions were characterized by extensive multifocal areas of myofiber hyaline degeneration, necrosis with sarcoplasmic fragmentation, mineralization of occasional myofibers, and atrophy of fibers with varied stages of fibrosis. Inflammatory cell exudates were predominantly eosinophils, with some macrophages and lymphocytes, and extravasated erythrocytes within, and adjacent to, affected myofibers. Affected muscles contained more S. cruzi than unaffected muscle from passed carcasses. However, a distinct cause and effect relationship could not be determined between the parasite and the presence of eosinophilic myositis. PMID:2518705

  14. Support for social rehabilitation of childhood acute lymphoblastic leukemia patients. Psychological and educational assessment by the K-ABC

    Intellectual impairment in pediatric acute lymphoblastic leukemia (ALL) is thought to be caused by the effect of treatment on the central nervous system. We therefore assessed the characteristics and tendencies of patients' cognitive ability by using the K-ABC (Kaufman Assessment Battery for Children), an intelligence test. The subjects were 28 patients treated for ALL (males 18, females 10, age 4.7-12.0 years). The patients who took the K-ABC test were divided into irradiation group (15 patients who received brain irradiation as prophylactic treatment) and a non-irradiation group (13 patients whose brain was not irradiated), and evaluated the results. The K-ABC consists of a cognition processing scale and an acquisition level, and the cognition processing scale consists of a sequential processing scale and simultaneous processing scale. Patients were assessed in regard to various factors: 1. sex, 2. age of onset, 3. length of hospital stay, 4. age at the time of irradiation, 5. radiation dose, 6. score on the cognition processing scale, and multiple comparisons were made based on analysis of variance, least significant differences (1, 2, 3, 6), and the t-test (4, 5). Sequential processing ability was impaired in the patients with impaired cognitive processing in both groups. Part of simultaneous processing ability (ability to understand spatial relationships) tended to be reduced in the irradiation group in addition to the impairment in sequential processing ability, and factors 1 and 4 influenced cognitive ability in the irradiation group. The ability of girls decreased more than in boys. When children were irradiated below 4 years of age, their ability decreased even more. Regardless of whether they had received radiation therapy, all of the patients had received chemotherapy, including methotrexate, etc., and the anticancer drugs may have reduced their cognitive ability. The reduction of simultaneous processing ability may have been caused by the addition of

  15. Survival outcome following isolated central nervous system relapse treated with additional chemotherapy and craniospinal irradiation in childhood acute lymphoblastic leukemia

    Purpose: An analysis of survival outcome following isolated central nervous system (CNS) relapse treated with craniospinal irradiation (CSI) and additional chemotherapy in children with acute lymphoblastic leukemia (ALL) was conducted. Methods and Materials: Eighteen of 344 pediatric patients with ALL who attained initial complete remission on the St. Jude Children's Research Hospital 'Study XI' prospective protocol (1984-1988) developed a CNS relapse as first adverse event. Median interval to isolated CNS relapse was 7.5 months (range = 2-40 months) after achieving initial complete remission. At diagnosis, 14 of the 18 children were categorized as 'high risk' for subsequent leukemic relapse. Preventive cranial irradiation [PCI (18 Gy)] was delivered as planned to one of the 14 'high-risk' children. The other 13 'high-risk' patients experienced a CNS relapse during the first year of continuation therapy prior to week 52 of planned PCI. All four 'low-risk' patients experienced a CNS relapse beyond the first year of continuation therapy; none were scheduled to receive PCI. Following isolated CNS relapse, all 18 patients were treated on a prospective contingency of 'Study XI' trial consisting of intensified reinduction chemotherapy, weekly intrathecal methotrexate/hydrocortisone/Ara-C x 4-6 injections, craniospinal irradiation (cranium to 24.0 Gy and spine to 15.0 Gy at 1.5 Gy/fraction) and maintenance systemic therapy for a minimum of 1 year. Results: Ten of 18 patients remain in continuous complete secondary remission at 17 to 50 months post-CNS relapse. Second sites of relapse in the remaining eight children were as follows: CNS in four, bone marrow in three, and bilateral testicular in one patient. Each of these eight patients died of progressive leukemia. At a median follow-up of 40 months post-initial CNS relapse, the 3-year secondary Kaplan-Meier survival and event-free survival are 72% and 56%, respectively. Minimal long-term neurotoxicity was associated with

  16. Support for social rehabilitation of childhood acute lymphoblastic leukemia patients. Psychological and educational assessment by the K-ABC

    Izumi, Mayuko [Ochanomizu Univ., Tokyo (Japan); Hosoya, Ryouta; Oohira, Mutsuro; Kaneko, Takashi; Matsushita, Taketsugu

    2000-10-01

    Intellectual impairment in pediatric acute lymphoblastic leukemia (ALL) is thought to be caused by the effect of treatment on the central nervous system. We therefore assessed the characteristics and tendencies of patients' cognitive ability by using the K-ABC (Kaufman Assessment Battery for Children), an intelligence test. The subjects were 28 patients treated for ALL (males 18, females 10, age 4.7-12.0 years). The patients who took the K-ABC test were divided into irradiation group (15 patients who received brain irradiation as prophylactic treatment) and a non-irradiation group (13 patients whose brain was not irradiated), and evaluated the results. The K-ABC consists of a cognition processing scale and an acquisition level, and the cognition processing scale consists of a sequential processing scale and simultaneous processing scale. Patients were assessed in regard to various factors: 1. sex, 2. age of onset, 3. length of hospital stay, 4. age at the time of irradiation, 5. radiation dose, 6. score on the cognition processing scale, and multiple comparisons were made based on analysis of variance, least significant differences (1, 2, 3, 6), and the t-test (4, 5). Sequential processing ability was impaired in the patients with impaired cognitive processing in both groups. Part of simultaneous processing ability (ability to understand spatial relationships) tended to be reduced in the irradiation group in addition to the impairment in sequential processing ability, and factors 1 and 4 influenced cognitive ability in the irradiation group. The ability of girls decreased more than in boys. When children were irradiated below 4 years of age, their ability decreased even more. Regardless of whether they had received radiation therapy, all of the patients had received chemotherapy, including methotrexate, etc., and the anticancer drugs may have reduced their cognitive ability. The reduction of simultaneous processing ability may have been caused by the addition

  17. After the chemotherapy: potential mechanisms for chemotherapy-induced delayed skeletal muscle dysfunction in survivors of acute lymphoblastic leukaemia in childhood

    Celena eScheede-Bergdahl

    2013-04-01

    Full Text Available There is evidence that survivors of childhood cancers, such as acute lymphoblastic leukaemia (ALL, have increased rates of longterm skeletal muscle dysfunction. This places them at higher risk of physical restriction and functional impairment as well as potentially contributing to observed increases in cardiovascular disease and insulin resistance in later life. The mechanisms underlying these changes in skeletal muscle are unknown but chemotherapy drugs used in treatment for ALL are strongly implicated. Normal skeletal muscle growth, development and function are dependent on correctly functioning muscle satellite cells, muscle motor neurons and muscle mitochondria. Each of these key components is potentially susceptible to damage by chemotherapy in childhood, particularly prolonged courses including repeated administration of combination chemotherapy. If this chemotherapy-induced damage is not fully reversible, impairment of satellite cells, muscle motor innervation and mitochondria could, either singly or together, lead to the emergence of delayed or persistent skeletal muscle dysfunction many years later. The known effects of individual drugs used in the treatment of ALL are outlined and the need for specific targeted studies to investigate the mechanisms underlying persistent muscle dysfunction in survivors of childhood cancers is highlighted.

  18. Nontraumatic Myositis Ossificans of Hip: A Case Presentation

    Yunus Oc

    2016-01-01

    Full Text Available In most of the cases trauma is the leading etiology and the nontraumatic myositis ossificans (MO is a very rare condition. We present an MO case without any trauma occurring. A 36-year-old female patient with a history of pain and restriction of range of motion of the left hip was admitted. Hip motions were restricted with 10–60° of flexion, 10° of internal rotation, 20° of external rotation, 10° of abduction, and 10° of adduction. There was no history of trauma and familial involvement. The biopsy of the lesion revealed mature bone tissue confirming our diagnosis of MO. The mass was removed surgically and postoperatively the patient was treated with a single dose radiotherapy with 800 gyc. MO is a benign and well differentiated bone formation or in other words heterotopic ossification of the muscle tissue. It has a prevalence of less than 1/1 million. Trauma is the most frequent etiological factor seen in almost 60–75% of the cases. Nontraumatic MO is very rare in the literature. Our patient had no history of trauma or familial involvement. Combination of the surgical excision with radiotherapy in the treatment of the MO of the hip may give satisfactory results.

  19. Nontraumatic Myositis Ossificans of Hip: A Case Presentation.

    Oc, Yunus; Ozcan, Muhammed Sefa; Sezer, Hasan Basri; Kilinc, Bekir Eray; Eren, Osman Tugrul

    2016-01-01

    In most of the cases trauma is the leading etiology and the nontraumatic myositis ossificans (MO) is a very rare condition. We present an MO case without any trauma occurring. A 36-year-old female patient with a history of pain and restriction of range of motion of the left hip was admitted. Hip motions were restricted with 10-60° of flexion, 10° of internal rotation, 20° of external rotation, 10° of abduction, and 10° of adduction. There was no history of trauma and familial involvement. The biopsy of the lesion revealed mature bone tissue confirming our diagnosis of MO. The mass was removed surgically and postoperatively the patient was treated with a single dose radiotherapy with 800 gyc. MO is a benign and well differentiated bone formation or in other words heterotopic ossification of the muscle tissue. It has a prevalence of less than 1/1 million. Trauma is the most frequent etiological factor seen in almost 60-75% of the cases. Nontraumatic MO is very rare in the literature. Our patient had no history of trauma or familial involvement. Combination of the surgical excision with radiotherapy in the treatment of the MO of the hip may give satisfactory results. PMID:27437157

  20. Myositis ossificans around shoulder following military training programme

    Mustafa C Kir

    2011-01-01

    Full Text Available The myositis ossificans around shoulder in military recruits are not reported yet. Three young male soldiers presented with complaints of palpable mass at the anterior aspect of shoulder; tenderness around the superior part of deltopectoral groove close to acromioclavicular joint; and restriction of shoulder motion. They also noticed ecchymosis and pain around the coracoid process and anterior shoulder region during regular firing exercises. Plain X-rays and computerized tomography showed extra-capsular, dense, irregular structure in the space between pectoralis and deltoid muscles which correlated with heterotopic bone. One patient refused surgical intervention because of the completion of his military serving period. Surgical excision was performed for the other two patients. During surgical exploration, both ossified masses were found in deltopectoral region and mostly in fibers of clavicular and acromial parts of deltoid muscle. Pathological reports confirmed the structure of masses as mature trabecular bone. Postoperatively indomethacin treatment and active shoulder exercises were started until the full range of motion was regained. Mini soft bag was used on the rifle contact area of the shoulder. No complications or recurrences were observed during the 24 months of followup period.

  1. [Necrotising fasciitis and myositis as serious complications after liposuction].

    Nagelvoort, R W Klein; Hulstaert, P F; Kon, M; Schuurman, A H

    2002-12-14

    Three days after liposuction of the lower abdomen, a 41-year-old woman was admitted for toxic shock-like syndrome with necrotising fasciitis and myositis, caused by Lancefield-group-A beta-haemolytic streptococci. The patient was treated by radical debridement of the skin, subcutis, fasciae and part of the pectoral muscle, plus antibiotics. Postoperatively she required artificial respiration for respiratory insufficiency. One week after the operation the wound was covered by transplantation of autologous skin. The patient survived, but was seriously disfigured. Necrotising fasciitis is a progressive soft-tissue infection, characterised by widespread necrosis of the superficial and deep fascia, often associated with severe systemic toxic reactions. Unless quickly recognised and aggressively treated, the course is often fatal. Due to the absence of cutaneous findings in the early stages, diagnosis is difficult. Important diagnostic aids are routine laboratory tests, contrast-MRI and a combination of the finger test and frozen-section biopsy. Treatment consists of early radical debridement, broad-spectrum antibiotics and supportive care. In a later stage, soft-tissue reconstruction with autografts or artificial skin grafts and skin transposition can be performed. PMID:12518522

  2. Randomized double blind trial of ciprofloxacin prophylaxis during induction treatment in childhood acute lymphoblastic leukemia in the WK-ALL protocol in Indonesia

    Widjajanto PH

    2013-02-01

    Full Text Available Pudjo H Widjajanto,1 Sumadiono Sumadiono,1 Jacqueline Cloos,2,3 Ignatius Purwanto,1 Sutaryo Sutaryo,1 Anjo JP Veerman1,21Pediatric Hematology and Oncology Division, Department of Pediatrics, Dr Sardjito Hospital, Medical Faculty, Universitas Gadjah Mada, Yogyakarta, Indonesia; 2Pediatric Oncology/Hematology Division, Department of Pediatrics, 3Department of Hematology, VU University Medical Center, Amsterdam, The NetherlandsObjectives: Toxic death is a big problem in the treatment of childhood acute lymphoblastic leukemia (ALL, especially in low-income countries. Studies of ciprofloxacin as single agent prophylaxis vary widely in success rate. We conducted a double-blind, randomized study to test the effects of ciprofloxacin monotherapy as prophylaxis for sepsis and death in induction treatment of the Indonesian childhood ALL protocol.Methods: Patients were randomized to the ciprofloxacin arm (n = 58 and to the placebo arm (n = 52. Oral ciprofloxacin monotherapy or oral placebo was administered twice a day. All events during induction were recorded: toxic death, abandonment, resistant disease, and complete remission rate.Results: Of 110 patients enrolled in this study, 79 (71.8% achieved CR. In comparison to the placebo arm, the ciprofloxacin arm had lower nadir of absolute neutrophil count during induction with median of 62 (range: 5–884 versus 270 (range: 14–25,480 × 109 cells/L (P > 0.01, greater risks for experiencing fever (50.0% versus 32.7%, P = 0.07, clinical sepsis (50.0% versus 38.5%, P = 0.22, and death (18.9% versus 5.8%, P = 0.05.Conclusion: In our setting, a reduced intensity protocol in a low-income situation, the data warn against using ciprofloxacin prophylaxis during induction treatment. A lower nadir of neutrophil count and higher mortality were found in the ciprofloxacin group.Keywords: ciprofloxacin, prophylaxis, childhood acute lymphoblastic leukemia, randomized trial, low-income country

  3. Differences in meiotic recombination rates in childhood acute lymphoblastic leukemia at an MHC class II hotspot close to disease associated haplotypes.

    Pamela Thompson

    Full Text Available Childhood Acute Lymphoblastic Leukemia (ALL is a malignant lymphoid disease of which B-cell precursor- (BCP and T-cell- (T ALL are subtypes. The role of alleles encoded by major histocompatibility loci (MHC have been examined in a number of previous studies and results indicating weak, multi-allele associations between the HLA-DPB1 locus and BCP-ALL suggested a role for immunosusceptibility and possibly infection. Two independent SNP association studies of ALL identified loci approximately 37 kb from one another and flanking a strong meiotic recombination hotspot (DNA3, adjacent to HLA-DOA and centromeric of HLA-DPB1. To determine the relationship between this observation and HLA-DPB1 associations, we constructed high density SNP haplotypes of the 316 kb region from HLA-DMB to COL11A2 in childhood ALL and controls using a UK GWAS data subset and the software PHASE. Of four haplotype blocks identified, predicted haplotypes in Block 1 (centromeric of DNA3 differed significantly between BCP-ALL and controls (P = 0.002 and in Block 4 (including HLA-DPB1 between T-ALL and controls (P = 0.049. Of specific common (>5% haplotypes in Block 1, two were less frequent in BCP-ALL, and in Block 4 a single haplotype was more frequent in T-ALL, compared to controls. Unexpectedly, we also observed apparent differences in ancestral meiotic recombination rates at DNA3, with BCP-ALL showing increased and T-ALL decreased levels compared to controls. In silico analysis using LDsplit sotware indicated that recombination rates at DNA3 are influenced by flanking loci, including SNPs identified in childhood ALL association studies. The observed differences in rates of meiotic recombination at this hotspot, and potentially others, may be a characteristic of childhood leukemia and contribute to disease susceptibility, alternatively they may reflect interactions between ALL-associated haplotypes in this region.

  4. Myositis ossificans circumscripta of the triceps due to overuse in a female swimmer

    Saartje Defoort

    2012-01-01

    Full Text Available Myositis ossificans is a rare condition characterized by non-neoplastic heterotopic bone formation in soft tissue and skeletal muscle. It is a benign and often self-limiting disease with no need for surgery. Here, we describe a young female swimmer with myositis ossificans circumscripta of the triceps due to overuse. Because of the benign character of the lesion, conservative treatment was initiated with rest and anti-inflammatory drugs. She obtained complete resolution after 6 months and was able to return to normal sporting activities. Myositis ossificans circumscripta is a rare benign lesion with an excellent prognosis. Most lesions in athletes occur due to contusions or strains; however, overuse is now described as well. Spontaneous resolution is seen in almost all cases. Cases in which, despite conservative treatment, a painful mass persists, surgical excision can be considered.

  5. Identification of residual leukemic cells by flow cytometry in childhood B-cell precursor acute lymphoblastic leukemia: verification of leukemic state by flow-sorting and molecular/cytogenetic methods

    Obro, Nina F; Ryder, Lars P; Madsen, Hans O;

    2012-01-01

    Reduction in minimal residual disease, measured by real-time quantitative PCR or flow cytometry, predicts prognosis in childhood B-cell precursor acute lymphoblastic leukemia. We explored whether cells reported as minimal residual disease by flow cytometry represent the malignant clone harboring...... immunophenotype and antigen modulation) that highlight important methodological pitfalls. These findings demonstrate that with sufficient experience, flow cytometry is reliable for minimal residual disease monitoring in B-cell precursor acute lymphoblastic leukemia, although rare cases require supplementary PCR...

  6. Preferentially Expressed Antigen of Melanoma (PRAME) and Wilms’ Tumor 1 (WT 1) Genes Expression in Childhood Acute Lymphoblastic Leukemia, Prognostic Role and Correlation with Survival

    Khateeb, Engy El; Morgan, Dalia

    2014-01-01

    BACKGROUND: Acute lymphocytic leukemia (ALL) is the most common hematologic malignancy in children. In young children it is also largely curable, with more than 90% of afflicted children achieving long-term remission. PRAME (Preferentially expressed antigen of melanoma) gene belongs to Group 3 class I HLA-restricted widely expressed antigens in which genes encoding widely expressed tumor antigens have been detected in many normal tissues as well as in histologically different types of tumors with no preferential expression on a certain type of cancer. It has been found to be expressed in a variety of cancer cells as leukemia & lymphoma. PRAME monitoring can be useful for detection of minimal residual disease and subsequent relapses particularly those leukemias in which specific tumor markers are unavailable. Wilms’ tumor1 (WT1) gene was identified as a gene that plays an important role in normal kidney development and inactivation of its function was shown to result in the development of Wilms’ tumors in paediatric patients. Disruption of WT1 function has been implicated in the formation of many different tumor types. AIM: to study how PRAME & WT 1 genes expression patterns influence cancer susceptibility & prognosis. PATIENTS & METHODS: 50 patients with denovo childhood acute lymphoblastic leukemia, as well as 50 age and sex matched apparently healthy volunteers were genotyped for PRAME and WT1 genes expression by reverse transcription polymerase chain reaction (RT-PCR). RESULTS: PRAME gene was expressed in 34 of the patients (68%) and WT1 gene was expressed in 26 of the patients (52%). Expression of both genes was significantly higher compared to controls (P antigens (PRAME and WT1) are potential candidates for immunotherapy in childhood acute leukemia.

  7. Combination Chemotherapy With or Without Bone Marrow Transplantation in Treating Children With Acute Myelogenous Leukemia or Myelodysplastic Syndrome

    2013-01-15

    Childhood Acute Erythroleukemia (M6); Childhood Acute Megakaryocytic Leukemia (M7); Childhood Acute Monoblastic Leukemia (M5a); Childhood Acute Monocytic Leukemia (M5b); Childhood Acute Myeloblastic Leukemia With Maturation (M2); Childhood Acute Myeloblastic Leukemia Without Maturation (M1); Childhood Acute Myelomonocytic Leukemia (M4); Childhood Myelodysplastic Syndromes; Chronic Myelomonocytic Leukemia; de Novo Myelodysplastic Syndromes; Refractory Anemia; Refractory Anemia With Excess Blasts; Refractory Anemia With Excess Blasts in Transformation; Refractory Anemia With Ringed Sideroblasts; Secondary Myelodysplastic Syndromes; Untreated Childhood Acute Myeloid Leukemia and Other Myeloid Malignancies

  8. Focal Inflammatory Myositis on 18F-FDG PET/CT.

    Bennett, Olivia; Ravi Kumar, Aravind S; Agnew, Julie

    2016-06-01

    Focal myositis is a rare benign inflammatory pseudotumor that can mimic malignancy, clinically and on imaging. A 34-year-old man presented with a 3-week history of sudden-onset, nontender, left upper neck mass that was nonresolving with antibiotics. Anatomical imaging was concerning for a sarcoma of the sternocleidomastoid muscle with possible regional nodal metastases and surrounding inflammatory change. F-FDG PET/CT showed marked FDG uptake extending around the anterior border of the sternocleidomastoid muscle with no FDG-avid local nodal disease. Core biopsy of the sternocleidomastoid muscle and adjacent node revealed inflammatory changes. A diagnosis of focal inflammatory myositis was made. PMID:27055132

  9. Inclusion body myositis, muscle blood vessel and cardiac amyloidosis, and transthyretin Val122Ile allele.

    Askanas, V; Engel, W K; Alvarez, R B; Frangione, B; Ghiso, J; Vidal, R

    2000-04-01

    Typical of sporadic inclusion body myositis muscle biopsies are vacuolated muscle fibers containing intracellular amyloid deposits and accumulations of "Alzheimer-characteristic" proteins. There is no muscle blood vessel or cardiac amyloidosis. We report on a 70-year-old African-American man homozygous for the transthyretin Val122Ile allele who has both sporadic inclusion body myositis and cardiac amyloidosis. His unique pathological features included transthyretin immunoreactivity in prominent muscle blood vessel amyloid and congophilic amyloid deposits within vacuolated muscle fibers. PMID:10762172

  10. Pediatric influenza‐associated myositis – Nebraska, 2001–2007

    Buss, Bryan F.; Shinde, Vivek M.; Safranek, Thomas J.; Uyeki, Timothy M.

    2009-01-01

    Objective  Influenza‐associated myositis (IAM), characterized by severe lower‐extremity myalgia and reluctance to walk, is a complication of influenza among children. We investigated IAM in Nebraska during six influenza seasons, 2001–2007. Methods  During 2006–2007, we requested reports of severe influenza illness among persons aged <18 years and investigated medical records to identify and confirm IAM cases defined as severe myalgia with elevated serum creatinine kinase level in a patient aged <18 years, occurring within 7 days of laboratory confirmed influenza illness onset. Statewide hospital discharge data (HDD) were reviewed to identify retrospectively confirmed IAM cases during 2006–2007 and five previous seasons, by using surveillance data to define periods of influenza activity. Statewide IAM incidence was estimated for 2001–2002 through 2006–2007. Results  During 2006–2007, a total of 13 IAM cases were confirmed by enhanced surveillance. Median age was 6 years (range, 4–11 years). Influenza diagnosis was established by viral isolation from six patients (one influenza A and five influenza B) and rapid diagnostic tests for seven. Twelve (92%) patients, including one who died, were hospitalized for a median of 3 days (range, 1–4 days). Review of HDD identified 12 retrospectively confirmed IAM cases during 2006–2007, including four not reported through enhanced surveillance, and only one during five previous seasons (2003–2004). The HDD‐derived, retrospectively confirmed statewide IAM incidence estimates/1 00 000 population aged <18 years were 2·693 and 0·225 during 2006–2007 and 2003–2004, respectively. Conclusion  An IAM epidemic occurred in Nebraska during the 2006–2007 influenza season. PMID:19903210

  11. Cured meat, vegetables, and bean-curd foods in relation to childhood acute leukemia risk: A population based case-control study

    Su Li

    2009-01-01

    Full Text Available Abstract Background Consumption of cured/smoked meat and fish leads to the formation of carcinogenic N-nitroso compounds in the acidic stomach. This study investigated whether consumed cured/smoked meat and fish, the major dietary resource for exposure to nitrites and nitrosamines, is associated with childhood acute leukemia. Methods A population-based case-control study of Han Chinese between 2 and 20 years old was conducted in southern Taiwan. 145 acute leukemia cases and 370 age- and sex-matched controls were recruited between 1997 and 2005. Dietary data were obtained from a questionnaire. Multiple logistic regression models were used in data analyses. Results Consumption of cured/smoked meat and fish more than once a week was associated with an increased risk of acute leukemia (OR = 1.74; 95% CI: 1.15–2.64. Conversely, higher intake of vegetables (OR = 0.55; 95% CI: 0.37–0.83 and bean-curd (OR = 0.55; 95% CI: 0.34–0.89 was associated with a reduced risk. No statistically significant association was observed between leukemia risk and the consumption of pickled vegetables, fruits, and tea. Conclusion Dietary exposure to cured/smoked meat and fish may be associated with leukemia risk through their contents of nitrites and nitrosamines among children and adolescents, and intake of vegetables and soy-bean curd may be protective.

  12. Vitamin D and bone minerals status in the long-term survivors of childhood acute lymphoblastic leukemia

    Nahid Reisi

    2015-01-01

    Conclusions: ALL treatment is associated with the increase in prevalence of vitamin D insufficiency in the childhood ALL survivors and since the low vitamin D level potentially increases the risk of low bone density, subsequent malignancies, and cardiovascular disease in the survivors, close follow-up of such patients are highly recommended to prevent the stated complications.

  13. Genomic profiling of thousands of candidate polymorphisms predicts risk of relapse in 778 Danish and German childhood acute lymphoblastic leukemia patients

    Wesolowska, Agata; Borst, L.; Dalgaard, Marlene Danner;

    2015-01-01

    Childhood acute lymphoblastic leukemia survival approaches 90%. New strategies are needed to identify the 10–15% who evade cure. We applied targeted, sequencing-based genotyping of 25 000 to 34 000 preselected potentially clinically relevant singlenucleotide polymorphisms (SNPs) to identify host...... genome profiles associated with relapse risk in 352 patients from the Nordic ALL92/2000 protocols and 426 patients from the German Berlin–Frankfurt–Munster (BFM) ALL2000 protocol. Patients were enrolled between 1992 and 2008 (median follow-up: 7.6 years). Eleven cross-validated SNPs were significantly...... associated with risk of relapse across protocols. SNP and biologic pathway level analyses associated relapse risk with leukemia aggressiveness, glucocorticosteroid pharmacology/response and drug transport/metabolism pathways. Classification and regression tree analysis identified three distinct risk groups...

  14. High white blood cell count at diagnosis of childhood acute lymphoblastic leukaemia: biological background and prognostic impact. Results from the NOPHO ALL-92 and ALL-2000 studies

    Vaitkeviciene, G; Forestier, E; Hellebostad, M;

    2011-01-01

    Prognostic impact of peripheral blood white blood cell count (WBC) at the diagnosis of childhood acute lymphoblastic leukaemia (ALL) was evaluated in a population-based consecutive series of 2666 children aged 1–15 treated for ALL between 1992 and 2008 in the five Nordic countries (Denmark, Finland......, Iceland, Norway and Sweden). Ten-year event-free (pEFS10y) survival and overall (pOS10y) survival were 0.75 ± 0.01 and 0.85 ± 0.01, respectively. Although treatment intensity was determined by WBC, nonremission and relapsed patients still had significantly higher WBC than those in remission for B-cell...

  15. Radiation-recall myositis presenting as low-back pain (2010: 4b)

    We report on a patient with a history of Ewing sarcoma who underwent surgery and subsequent adjuvant chemotherapy and radiotherapy. He developed low-back pain 6 months after the end of the radiotherapy and during consolidation chemotherapy. Magnetic resonance imaging showed evidence of myositis corresponding to the 'radiation-recall phenomenon', an inflammatory reaction of irradiated tissue. (orig.)

  16. Statin-induced focal myositis of the upper extremity. A report of two cases

    Wagner, M., E-mail: wagner.radiologie@herzchirurgie.de [Department of Radiology, Herz- und Gefaessklinik GmbH, Salzburger Leite 1, D-97616 Bad Neustadt an der Saale (Germany); Muehldorfer-Fodor, M.; Prommersberger, K.J. [Department of Handsurgery, Herz- und Gefaessklinik GmbH, Bad Neustadt an der Saale (Germany); Schmitt, R. [Department of Radiology, Herz- und Gefaessklinik GmbH, Salzburger Leite 1, D-97616 Bad Neustadt an der Saale (Germany)

    2011-02-15

    Statins are widely used to lower increased cholesterol levels with the aim to prevent major cardiovascular events. However, they bare the risk of myotoxic side effects. We report on two patients with focal weakness and pain in the upper extremities. In both patients, abnormal MRI signal heights in the muscle groups involved were indicative of the final diagnosis of focal myositis during statin therapy.

  17. Loci on 7p12.2, 10q21.2 and 14q11.2 are associated with risk of childhood acute lymphoblastic leukemia

    Papaemmanuil, Elli; Hosking, Fay J; Vijayakrishnan, Jayaram; Price, Amy; Olver, Bianca; Sheridan, Eammon; Kinsey, Sally E; Lightfoot, Tracy; Roman, Eve; Irving, Julie A E; Allan, James M.; Tomlinson, Ian P; Taylor, Malcolm; Greaves, Mel; Houlston, Richard S

    2016-01-01

    To identify risk variants for childhood acute lymphoblastic leukemia (ALL) we conducted a genome-wide association study of 2 case-control series, analyzing the genotypes of 291,423 tagging SNP genotypes in a total of 907 ALL cases and 2,398 controls. We identified risk loci for ALL at 7p12.2 (IKZF1, rs4132601; OR = 1.69, P = 1.20 x 10-19), 10q21.2 (ARIDB5, rs7089424; OR = 1.65, P = 6.69 x 10-19) and 14q11.2 (CEBPE, rs2239633; OR = 1.34, P = 2.88 x 10-7). The 10q21.2 (ARIDB5) risk association appears to be selective for the subset of B-cell precursor ALL with hyperdiploidy. These data show that common low-penetrance susceptibility alleles contribute to the risk of developing childhood ALL and provide novel insight into disease causation of this hematological cancer; notably all 3 risk variants map to genes involved in transcriptional regulation and differentiation of B-cell progenitors. PMID:19684604

  18. Utility of Global Longitudinal Strain by Echocardiography to Detect Left Ventricular Dysfunction in Long-Term Adult Survivors of Childhood Lymphoma and Acute Lymphoblastic Leukemia.

    Christiansen, Jon R; Massey, Richard; Dalen, Håvard; Kanellopoulos, Adriani; Hamre, Hanne; Fosså, Sophie D; Ruud, Ellen; Kiserud, Cecilie E; Aakhus, Svend

    2016-08-01

    Measuring left ventricular (LV) global longitudinal strain (GLS) is recommended in screening of long-term cancer survivors for cardiotoxicity. However, there are limited data on GLS in this setting, in particular in survivors with apparently normal LV function without risk factors of impaired GLS. In the present study, we measured GLS in 191 adult survivors of childhood lymphoma or acute lymphoblastic leukemia, with normal LV ejection fraction and fractional shortening (FS) and without known hypertension, diabetes mellitus, myocardial infarction, or stroke. We compared GLS in the survivors with 180 controls. Mean GLS was -19.0 ± 2.2% in the survivor group and -21.4 ± 2.0% in the controls (p cancer treatment. Survivors treated with mediastinal radiotherapy had an odds ratio of impaired GLS of 5.2 (95% confidence interval 2.2 to 12) compared with other survivors. Survivors treated with cumulative anthracycline doses >300 mg/m(2) had an odds ratio of 4.8 (95% confidence interval 1.7 to 14) of impaired GLS. In conclusion, this study demonstrates a high proportion of LV dysfunction assessed by GLS in apparently healthy adult survivors of childhood cancer. Impaired GLS was associated with previous exposure to mediastinal radiotherapy and high doses of anthracyclines. The prognostic role of measuring GLS in this specific patient population should be examined in prospective studies. PMID:27296561

  19. Efficacy of ultrasound elastography in detecting active myositis in children: can it replace MRI?

    Berko, Netanel S.; Levin, Terry L. [Montefiore Medical Center, Department of Radiology, Bronx, NY (United States); Hay, Arielle [Montefiore Medical Center, Department of Pediatrics, Division of Pediatric Rheumatology, Bronx, NY (United States); Miami Children' s Hospital, Department of Pediatrics, Miami, FL (United States); Sterba, Yonit; Wahezi, Dawn [Montefiore Medical Center, Department of Pediatrics, Division of Pediatric Rheumatology, Bronx, NY (United States)

    2015-09-15

    Juvenile idiopathic inflammatory myopathy is a rare yet potentially debilitating condition. MRI is used both for diagnosis and to assess response to treatment. No study has evaluated the performance of US elastography in the diagnosis of this condition in children. To assess the performance of compression-strain US elastography in detecting active myositis in children with clinically confirmed juvenile idiopathic inflammatory myopathy and to compare its efficacy to MRI. Children with juvenile idiopathic inflammatory myopathy underwent non-contrast MR imaging as well as compression-strain US elastography of the quadriceps muscles. Imaging findings from both modalities were compared to each other as well as to the clinical determination of active disease based on physical examination and laboratory data. Active myositis on MR was defined as increased muscle signal on T2-weighted images. Elastography images were defined as normal or abnormal based on a previously published numerical scale of muscle elastography in normal children. Muscle echogenicity was graded as normal or abnormal based on gray-scale sonographic images. Twenty-one studies were conducted in 18 pediatric patients (15 female, 3 male; age range 3-19 years). Active myositis was present on MRI in ten cases. There was a significant association between abnormal MRI and clinically active disease (P = 0.012). US elastography was abnormal in 4 of 10 cases with abnormal MRI and in 4 of 11 cases with normal MRI. There was no association between abnormal elastography and either MRI (P > 0.999) or clinically active disease (P > 0.999). Muscle echogenicity was normal in 11 patients; all 11 had normal elastography. Of the ten patients with increased muscle echogenicity, eight had abnormal elastography. There was a significant association between muscle echogenicity and US elastography (P < 0.001). The positive and negative predictive values for elastography in the determination of active myositis were 75% and 31

  20. Efficacy of ultrasound elastography in detecting active myositis in children: can it replace MRI?

    Juvenile idiopathic inflammatory myopathy is a rare yet potentially debilitating condition. MRI is used both for diagnosis and to assess response to treatment. No study has evaluated the performance of US elastography in the diagnosis of this condition in children. To assess the performance of compression-strain US elastography in detecting active myositis in children with clinically confirmed juvenile idiopathic inflammatory myopathy and to compare its efficacy to MRI. Children with juvenile idiopathic inflammatory myopathy underwent non-contrast MR imaging as well as compression-strain US elastography of the quadriceps muscles. Imaging findings from both modalities were compared to each other as well as to the clinical determination of active disease based on physical examination and laboratory data. Active myositis on MR was defined as increased muscle signal on T2-weighted images. Elastography images were defined as normal or abnormal based on a previously published numerical scale of muscle elastography in normal children. Muscle echogenicity was graded as normal or abnormal based on gray-scale sonographic images. Twenty-one studies were conducted in 18 pediatric patients (15 female, 3 male; age range 3-19 years). Active myositis was present on MRI in ten cases. There was a significant association between abnormal MRI and clinically active disease (P = 0.012). US elastography was abnormal in 4 of 10 cases with abnormal MRI and in 4 of 11 cases with normal MRI. There was no association between abnormal elastography and either MRI (P > 0.999) or clinically active disease (P > 0.999). Muscle echogenicity was normal in 11 patients; all 11 had normal elastography. Of the ten patients with increased muscle echogenicity, eight had abnormal elastography. There was a significant association between muscle echogenicity and US elastography (P < 0.001). The positive and negative predictive values for elastography in the determination of active myositis were 75% and 31

  1. PARC/CCL18 Is a Plasma CC Chemokine with Increased Levels in Childhood Acute Lymphoblastic Leukemia

    Struyf, Sofie; Schutyser, Evemie; Gouwy, Mieke; Gijsbers, Klara; Proost, Paul; Benoit, Yves; Opdenakker, Ghislain; Van Damme, Jo; Laureys, Geneviève

    2003-01-01

    Chemokines play an important role in leukocyte mobilization, hematopoiesis, and angiogenesis. Tissue-specific expression of particular chemokines also influences tumor growth and metastasis. Here, the CC chemokine pulmonary and activation-regulated chemokine (PARC)/CCL18 was measured in pediatric patients with acute lymphoblastic leukemia (ALL) or acute myeloid leukemia (AML). Surprisingly, PARC immunoreactivity was consistently detected in plasma from healthy donors. After purification to ho...

  2. Long Term Follow%u2013Up, Treatment and Prognosis of Acute Transverse Myelitis Patients In Childhood

    Mehmet Canpolat

    2013-01-01

    Aim: To overview the medical history, clinical signs, imaging studies, laboratory data and treatment effectiveness in children with acute idiopathic transverse myelitis. Material and Method: Eight patients under the age of 15 years who presented acute transverse myelitis were included in the study by using the criteria of the Transverse Myelitis Consortium Working Group (2002). Chart analysis, clinical evaluation, imaging studies, laboratory data and treatment effectiveness were evaluated ret...

  3. Total Marrow and Lymphoid Irradiation and Chemotherapy Before Donor Transplant in Treating Patients With Myelodysplastic Syndrome or Acute Leukemia

    2016-08-10

    Adult Acute Lymphoblastic Leukemia in Complete Remission; Acute Myeloid Leukemia in Remission; Previously Treated Myelodysplastic Syndrome; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Acute Myeloid Leukemia; Recurrent Childhood Acute Lymphoblastic Leukemia; Recurrent Childhood Acute Myeloid Leukemia; Childhood Acute Lymphoblastic Leukemia in Complete Remission

  4. Analysis of factors influencing the overall effect of racecadotril on childhood acute diarrhea. Results from a real-world and post-authorization surveillance study in Venezuela

    Jose Chacón

    2010-06-01

    Full Text Available Jose ChacónOn behalf of the Racecadotril Post-authorization Record Group; Centro Clinico Profesional Caracas, Caracas, VenezuelaAbstract: Drug efficacy might differ from clinical trial results when performed in clinical daily conditions. Therefore, it is mandatory to conduct trials about effectiveness to improve external validity. This post-authorization, open-label, noncontrolled, prospective, multicenter, observational, and naturalistic trial was designed to search for factors influencing the racecadotril overall effect on childhood acute watery diarrhea in a real-world setting of Venezuela. There were 3,873 children with acute watery diarrhea treated with racecadotril, an enkephalin breakdown blocker plus oral rehydration therapy by 97 pediatricians. Evaluations were carried out daily until emission of two consecutive formed stools or absence of watery bowel movements for 24 hours. The primary end-point was time-to-relief, defined as the time from first racecadotril dose to the last watery bowel movement time. Age, gender, nursing type, nursing status during diarrhea, diarrhea severity, and co-medication were considered as factors in the statistical analysis. The primary end-point was evaluated by factors using UNIANOVA, and post-hoc tests were done. A multiple regression analysis was carried out to identify factors affecting drug performance, racecadotril effectiveness and tolerability overall assessment was searched by physicians and patients, and inter-observer agreement was evaluated by kappa statistics. The mean time-to-relief was 18.5 ± 12.5 hours [95% confidence interval 17.9–19.0] and the diarrhea severity was the only variable with significant and independent weight on racecadotril effectiveness explaining 23% of time-to-relief variance, but even in severe diarrhea cases this time was less than 24 hours. High agreement about satisfactory perception on effectiveness and tolerability was reached among physicians and patients. In

  5. Severe myositis on commencement of efavirenz, abacavir and lamivudine, in the absence of lactic acidosis or classical abacavir hypersensitivity

    Parsonage, Mirella Jane; Barlow, Gavin; Lillie, Patrick; Moss, Peter; Adams, Katherine; Thaker, Hiten

    2009-01-01

    Myositis in HIV may be due to HIV itself, or to opportunistic infection, malignancy or drug treatment. Severe myositis or rhabdomyolysis have never been reported with the commonly used nucleoside reverse transcriptase inhibitor abacavir, although creatine phosphokinase may rise modestly, particularly if abacavir hypersensitivity occurs. We report an unusual case of abacavir use associated with a thousand-fold rise in creatine phosphokinase in the absence of features of hypersensitivity. The c...

  6. Statin Intolerance Because of Myalgia, Myositis, Myopathy, or Myonecrosis Can in Most Cases be Safely Resolved by Vitamin D Supplementation

    Maksim Khayznikov; Kallish Hemachrandra; Ramesh Pandit; Ashwin Kumar; Ping Wang; Glueck, Charles J.

    2015-01-01

    Background: Low serum vitamin D can cause myalgia, myositis, myopathy, and myonecrosis. Statin-induced myalgia is a major and common cause of statin intolerance. Low serum vitamin D and statins, additively or synergistically, cause myalgia, myositis, myopathy, and/or myonecrosis. Statin-induced myalgia in vitamin D deficient patients can often be resolved by vitamin D supplementation, normalizing serum vitamin D levels. Aims: In 74 men and 72 women (age 59 ± 14 years) intolerant to ≥2 statins...

  7. Acute Childhood Illnesses and Health Seeking Behaviour among under five children in a village of Hooghly district, West Bengal

    Indira Dey (Pal

    2012-04-01

    Full Text Available Background: – Acute respiratory infections and diarrhoeal diseases are important causes of morbidity in children worldwide. IMNCI component is addressing these two illnesses in a major way and is concentrating on health care practices of community. Objective: – to find out their health seeking behaviour. Methodology: – A community based , cross-sectional study was conducted in the Mollasimla village of Hooghly district of West Bengal using 2 weeks recall for acute illnesses. Results – It was found that 56.8%, 23.8% and 18.9% children suffered from ARI, fever and diarrhea respectively. Overall treatment rate was above 93% and most of the children were treated in hospitals and health centre. Conclusion: – Acute illnesses are still largely prevalent in the rural community. As mothers are the first care givers, they should be made aware of the preventive measures and the need for seeking treatment.

  8. White versus gray matter function as seen on neuropsychological testing following bone marrow transplant for acute leukemia in childhood

    Fiona S Anderson

    2008-03-01

    Full Text Available Fiona S Anderson1, Alicia S Kunin-Batson1, Joanna L Perkins2, K Scott Baker31Divisions of Pediatric Clinical Neuroscience; 2Department of Pediatric Hematology/Oncology, Children’s Hospitals and Clinics, Minneapolis, MN, USA and 3Hematology/Oncology/BMT, Department of Pediatrics, University of Minnesota, Minneapolis, MN, USAAbstract: Current theory suggests that neurocognitive late effects of treatments for childhood cancer such as difficulties with attention, processing speed and visual-motor ability are the result of white matter damage. Neuroimaging studies have produced a variety of white matter findings. However, although white matter is thought to be differentially affected, previous studies have not demonstrated a discrepancy between white and gray matter function. The present study included 36 children treated for childhood leukemia with hematopoietic stem cell transplant (HCT. Their performance on neurocognitive measures traditionally thought to measure white matter was compared to performance on measures thought to measure gray matter function. Composite white and gray matter standard scores were created based on neuropsychological measures that individuals with known white or gray matter damage perform poorly. As predicted, composite white matter scores (mean = 98.1 were significantly lower (t = 2.26, p = 0.03 than composite gray matter scores (mean = 102.5. Additionally, as gray matter performance increased, the difference between gray and white matter scores increased (R = 0.353, p = 0.035. Overall, the results of this study support the current theory that white matter damage is responsible for the more subtle neurocognitive late effects resulting from treatment for childhood leukemia.Keywords: late effects of cancer treatment, leukemia, neuropsychology, white matter, brain function

  9. Childhood osteomyelitis-incidence and differentiation from other acute onset musculoskeletal features in a population-based study

    Reiseter Tor

    2008-10-01

    Full Text Available Abstract Background Osteomyelitis can be difficult to diagnose and there has previously not been a prospective approach to identify all children in a defined geographic area. The aim of this study was to assess the annual incidence of osteomyelitis in children, describe the patient and disease characteristics in those with acute ( Methods In a population-based Norwegian study physicians were asked to refer all children with suspected osteomyelitis. Children with osteomyelitis received follow-up at six weeks, six months and thereafter as long as clinically needed. Results The total annual incidence rate of osteomyelitis was 13 per 100 000 (acute osteomyelitis 8 and subacute osteomyelitis 5 per 100 000. The incidence was higher in patients under the age of 3 than in older children (OR 2.9, 95%: CI 2.3–3.7. The incidence of non-vertebral osteomyelitis was higher than the incidence of vertebral osteomyelitis (10 vs. 3 per 100 000; p = .002. Vertebral osteomyelitis was more frequent in girls than in boys (OR 7.0, 95%: CI 3.3–14.7. ESR ≥ 40 mm/hr had the highest positive predictive laboratory value to identify osteomyelitis patients at 26% and MRI had a positive predictive value of 85%. Long-bone infection was found in 16 (43% patients. ESR, CRP, white blood cell count, neutrophils and platelet count were higher for patients with acute osteomyelitis than for patients with subacute osteomyelitis. Subacute findings on MRI and doctor's delay were more common in subacute osteomyelitis than in acute osteomyelitis patients. Blood culture was positive in 26% of the acute osteomyelitis patients and was negative in all the subacute osteomyelitis patients. Conclusion The annual incidence of osteomyelitis in Norway remains high. ESR values and MRI scan may help to identify osteomyelitis patients and differentiate acute and subacute osteomyelitis.

  10. Long-term results of NOPHO ALL-92 and ALL-2000 studies of childhood acute lymphoblastic leukemia

    Schmiegelow, K; Forestier, E; Hellebostad, M; Donovan, Martin Heyman; Kristinsson, J; Söderhäll, S; Taskinen, M

    2010-01-01

    Analysis of 2668 children with acute lymphoblastic leukemia (ALL) treated in two successive Nordic clinical trials (Nordic Society of Paediatric Haematology and Oncology (NOPHO) ALL-92 and ALL-2000) showed that 75% of all patients are cured by first-line therapy, and 83% are long-term survivors. ...

  11. Minimal residual disease-based risk stratification in Chinese childhood acute lymphoblastic leukemia by flow cytometry and plasma DNA quantitative polymerase chain reaction.

    Suk Hang Cheng

    Full Text Available Minimal residual disease, or MRD, is an important prognostic indicator in childhood acute lymphoblastic leukemia. In ALL-IC-BFM 2002 study, we employed a standardized method of flow cytometry MRD monitoring for multiple centers internationally using uniformed gating, and determined the relevant MRD-based risk stratification strategies in our local patient cohort. We also evaluated a novel method of PCR MRD quantitation using peripheral blood plasma. For the bone marrow flow MRD study, patients could be stratified into 3 risk groups according to MRD level using a single time-point at day-15 (Model I (I-A: 10%, or using two time-points at day-15 and day-33 (Model II (II-A: day-15<10% and day-33<0.01%, II-B: day-15 ≥ 10% or day-33 ≥ 0.01% but not both, II-C: day-15 ≥ 10% and day-33 ≥ 0.01%, which showed significantly superior prediction of relapse (p = .00047 and <0.0001 respectively. Importantly, patients with good outcome (frequency: 56.0%, event-free survival: 90.1% could be more accurately predicted by Model II. In peripheral blood plasma PCR MRD investigation, patients with day-15-MRD ≥ 10(-4 were at a significantly higher risk of relapse (p = 0.0117. By multivariate analysis, MRD results from both methods could independently predict patients' prognosis, with 20-35-fold increase in risk of relapse for flow MRD I-C and II-C respectively, and 5.8-fold for patients having plasma MRD of ≥ 10(-4. We confirmed that MRD detection by flow cytometry is useful for prognostic evaluation in our Chinese cohort of childhood ALL after treatment. Moreover, peripheral blood plasma DNA MRD can be an alternative where bone marrow specimen is unavailable and as a less invasive method, which allows close monitoring.

  12. Sarcocystis fayeri-Induced Granulomatous and Eosinophilic Myositis in 2 Related Horses.

    Herd, H R; Sula, M M; Starkey, L A; Panciera, R J; Johnson, E M; Snider, T A; Holbrook, T C

    2015-11-01

    This report describes 2 genetically related paint mares, case Nos. 1 and 2, presented to the Oklahoma State University Boren Veterinary Medical Teaching Hospital for chronic weight loss and abnormal gait, respectively. Notable findings in both cases included marked persistent eosinophilia and multiple intramuscular lateral thoracic masses. Histologic examination of masses revealed eosinophilic, centrally necrotic granulomas and marked eosinophilic myositis. Granulomas in case No. 1 also contained intralesional Sarcocystis sp material, and adjacent muscle fibers contained intact protozoal cysts. Case No. 1 developed severe refractory muscle pain and recurrent esophageal dysphagia. At necropsy, disseminated, grossly visible granulomas were present throughout all examined striated muscles. Nested polymerase chain reaction of the 18S rRNA gene revealed >99% homology with Sarcocystis fayeri. Sarcocystis spp are apicomplexan protozoa that infect striated muscle of many omnivorous species, typically without inciting clinical disease. Sarcocystosis should be considered a rare cause of granulomatous eosinophilic myositis and choke in horses. PMID:25957356

  13. Miosite ossificante progressiva: relato de caso Myositis ossificans progressiva: case report

    Frederico Barra de Moraes

    2012-01-01

    Full Text Available A miosite ossificante progressiva é uma doença rara, com menos de 1.000 casos descritos, autossômica dominante. O paciente apresenta edemas, devidos a processos inflamatórios, que vão se calcificando, com perda da mobilidade da região afetada. O objetivo deste trabalho é descrever um caso de miosite ossificante progressiva, apresentando as manifestações clínicas e discutindo os tratamentos disponíveis (ácido ascórbico oral e bifosfonato endovenoso.Myositis Ossificans Progressiva is a rare autosomal dominant disease with less than 1,000 case reports. Such patients presents edema, caused by inflammatory processes that progressively calcify, and with loss of mobility in the region affected. The objective of this study was to describe a case of myositis ossificans progressiva, present its clinical manifestations and discuss the treatments available (oral ascorbic acid and intravenous bisphosphonate.

  14. A case of parosteal osteosarcoma with a rare complication of myositis ossificans

    Spinelli Maria Silvia

    2012-11-01

    Full Text Available Abstract We report the case of a parosteal osteosarcoma of the distal ulna, treated with wide resection without reconstruction. The patient developed lung metastasis and a mass in the interosseus membrane of the forearm proximally to the osteotomy. The lung mass was found to be a metastasis from parosteal osteosarcoma and the biopsy of the forearm mass revealed a myositis ossificans. The suspicion of a recurrence of parosteal osteosarcoma, already metastatic, led to a second wide resection with no reconstruction. A slice of the radial cortex was taken during this second procedure. From a histological point of view, good margins were achieved and diagnosis of myositis ossificans was confirmed. Two months later, a radius fracture occurred and a synthesis, with plate and screws, as added with poly(methyl methacrylate (PMMA to reconstruct the bone loss, was performed. Indication of the reconstructive technique and the complication after distal ulna resection in oncologic surgery are discussed in this paper.

  15. Vaccine-induced myositis with intramuscular sterile abscess formation: MRI and ultrasound findings

    Polat, Ahmet Veysel; Bekci, Tumay; Selcuk, Mustafa Bekir [Ondokuz Mayis University, Department of Radiology, Faculty of Medicine, Samsun (Turkey); Dabak, Nevzat [Ondokuz Mayis University, Department of Orthopaedics and Traumatology, Faculty of Medicine, Samsun (Turkey); Ulu, Esra Meltem Kayahan [Samsun Medical Park Hospital, Department of Radiology, Samsun (Turkey)

    2015-12-15

    Although limb swelling is a well-known complication of vaccination, its rarity and wide band of differential diagnosis of limb swelling make it a diagnostic challenge. In this case report, we describe three cases of vaccine-induced myositis with intramuscular sterile abscess formation in patients with limb swelling and their magnetic resonance imaging and ultrasonography findings. Both radiologists and clinicians should be familiar with this rare entity, its clinical and imaging spectrum, and follow-up strategies. (orig.)

  16. Transthyretin Val122Ile, accumulated Abeta, and inclusion-body myositis aspects in cultured muscle.

    Askanas, Valerie; Engel, W King; McFerrin, Janis; Vattemi, Gaetano

    2003-07-22

    Cultured muscle fibers (CMF) from a patient with inclusion-body myositis (IBM) and cardiac amyloidosis associated with the transthyretin (TTR) Val122Ile mutation contained aspects of the IBM phenotype: vacuolation, congophilic inclusions, and clusters of immunocolocalizing amyloid beta-peptide (Abeta) and TTR accumulations. These abnormalities are never present in normal human CMF. These perturbations were greatly increased after Abeta precursor protein gene transfer. The TTR mutation may be a genetic predisposition factor for the patient's IBM. PMID:12874414

  17. Significance of Myositis Autoantibody in Patients with Idiopathic Interstitial Lung Disease

    Song, Ju Sun; Hwang, Jiwon; Cha, Hoon-Suk; Jeong, Byeong-Ho; Suh, Gee Young; Chung, Man Pyo; Kang, Eun-Suk

    2015-01-01

    Purpose Some patients with interstitial lung disease (ILD) related to connective tissue disease (CTD) have a delayed diagnosis of the underlying CTD when the ILD is categorized as idiopathic. In this study, we evaluated the frequency of myositis autoantibodies in patients diagnosed with idiopathic ILD and investigated the clinical significance stemming from the presence of the antibodies. Materials and Methods A total 32 patients diagnosed with idiopathic ILD were enrolled in this study. We a...

  18. Investigating novel therapeutic approaches for sporadic inclusion body myositis (sIBM)

    Ahmed, M.

    2012-01-01

    Sporadic inclusion body myositis (sIBM) is the most common acquired muscle disease affecting adults over the age of 50. Although the aetiology of this disease remains unclear, there is evidence for both inflammatory and myodegenerative processes in sIBM muscle pathology. In particular, abnormal protein aggregation is characteristic of affected muscle, with inclusion bodies incorporating amyloid-beta precursor protein (β-APP) among many others. Therapeutic interventions tested to date for s...

  19. Diagnostic value of markers of muscle degeneration in sporadic inclusion body myositis

    Dubourg, O.; Wanschitz, J; Maisonobe, T.; Béhin, A.; Allenbach, Y.; Herson, S; Benveniste, O

    2011-01-01

    Sporadic inclusion body myositis (s-IBM) is characterized histologically by the association of concomitant inflammatory and degenerative processes. We evaluated the sensitivity and specificity of different markers of the degenerative process in order to refine the histological diagnosis. We performed an immunohistochemical study with antibodies directed against ubiquitin, amyloid-β precursor protein (AβPP), amyloid-β (Aβ), SMI-31, SMI-310, Tar-DNA binding protein-43 (TDP-43) and p62 on s-IBM ...

  20. Management of necrotizing myositis in a field hospital: a case report

    Bharathi Ramanathan; Sharma Vinay; Sood Rohit; Chakladar Arunava; Singh Pragnya; Raman Deep

    2009-01-01

    Abstract Necrotizing myositis is a rare and fatal disease of skeletal muscles caused by group A beta hemolytic streptococci (GABHS). Its early detection by advanced imaging forms the basis of current management strategy. Paucity of advanced imaging in field/rural hospitals necessitates adoption of management strategy excluding imaging as its basis. Such a protocol, based on our experience and literature, constitutes: i. Prompt recognition of the clinical triad: disproportionate pain; precipit...

  1. Heterotopic ossification (myositis ossificans) in acquired immune deficiency syndrome. Detection by gallium scintigraphy

    A case of heterotopic ossification (myositis ossificans) secondary to the central nervous system complications of acquired immune deficiency syndrome (AIDS) is reported. Because of the overwhelming suspicion of infection in this patient, this diagnosis was not considered until a gallium scan revealed the typical findings of heterotopic ossification. Because of the increasing utilization of gallium imaging in the AIDS population, every imaging specialist should be aware of this potential disorder

  2. Caspofungin Acetate or Fluconazole in Preventing Invasive Fungal Infections in Patients With Acute Myeloid Leukemia Who Are Undergoing Chemotherapy

    2016-02-22

    Adult Acute Megakaryoblastic Leukemia (M7); Adult Acute Minimally Differentiated Myeloid Leukemia (M0); Adult Acute Monoblastic Leukemia (M5a); Adult Acute Monocytic Leukemia (M5b); Adult Acute Myeloblastic Leukemia With Maturation (M2); Adult Acute Myeloblastic Leukemia Without Maturation (M1); Adult Acute Myeloid Leukemia in Remission; Adult Acute Myeloid Leukemia With 11q23 (MLL) Abnormalities; Adult Acute Myeloid Leukemia With Del(5q); Adult Acute Myeloid Leukemia With Inv(16)(p13;q22); Adult Acute Myeloid Leukemia With t(16;16)(p13;q22); Adult Acute Myeloid Leukemia With t(8;21)(q22;q22); Adult Acute Myelomonocytic Leukemia (M4); Adult Erythroleukemia (M6a); Adult Pure Erythroid Leukemia (M6b); Childhood Acute Erythroleukemia (M6); Childhood Acute Megakaryocytic Leukemia (M7); Childhood Acute Minimally Differentiated Myeloid Leukemia (M0); Childhood Acute Monoblastic Leukemia (M5a); Childhood Acute Monocytic Leukemia (M5b); Childhood Acute Myeloblastic Leukemia With Maturation (M2); Childhood Acute Myeloblastic Leukemia Without Maturation (M1); Childhood Acute Myeloid Leukemia in Remission; Childhood Acute Myelomonocytic Leukemia (M4); Fungal Infection; Neutropenia; Recurrent Adult Acute Myeloid Leukemia; Recurrent Childhood Acute Myeloid Leukemia; Secondary Acute Myeloid Leukemia; Untreated Adult Acute Myeloid Leukemia; Untreated Childhood Acute Myeloid Leukemia and Other Myeloid Malignancies

  3. The Role of Type I Insulin Like Growth Factor Receptor (IGF-IR) in Adult and Childhood Acute Lymphoblastic Leukemia

    Background: Type 1 insulin like growth factor receptor (IGF-IR) is over expressed in many tumors including hematological cancers. It is a critical signaling molecule for tumor cell proliferation and survival. Data suggest that IGF-IR antibodies can effectively and specifically inhibit cancer cell growth in vitro and in vivo. Blockage of IGF-IR expression could be a promising therapeutic approach for the management of cancer patients. Aim of Work: To characterize the expression pattern of IGF-IR gene in malignant lymphoblasts of children and adults suffering from ALL in relation to clinical features at diagnosis. Patients and Methods: The expression of IGF-IR was analyzed in 60 patients with ALL, 30 childhood ALL (16 newly diagnosed and 14 in complete remission) and 30 adulthood ALL (15 newly diagnosed and 15 in complete remission) together with 20 normal age and sex matched healthy controls using a Real-Time Quantitative Reverse- Transcriptase Polymerase Chain Reaction (RTQ-PCR) to assess the possible relation, association or correlation between IGF-IR expression and ALL clinical and laboratory features at diagnosis. Results: IGF-IR was expressed in all 60 patients with ALL; the expression levels of IGF-IR were significantly higher in newly diagnosed patients than in patients in complete remission (CR) and controls (p<0.001). There were no statistically significant differences in the expression of IGF-IR between patients with different clinical and laboratory features. Conclusion: IGF-1R seems to play a crucial role in patients with ALL since it is expressed in all ALL cases (adulthood and childhood). Therefore, new therapeutic agents targeting IGF-1R may provide a better chance for those patients

  4. Severe, Steroid-responsive, Myositis Mimicking Necrotizing Fasciitis following Orthopedic Surgery: A Pyoderma Variant with Myonecrosis

    Alistair B. Reid, FRACP

    2014-06-01

    Full Text Available Summary: Postoperative pyoderma gangrenosum is a rare neutrophilic dermatosis that may be confused for necrotizing fasciitis. The inflammatory response is triggered by the trauma of surgery and thus must be managed nonsurgically. Clinical and pathological findings in the 2 diseases can be identical, leading to misdiagnosis and massive surgical defects from the ensuing surgery. This report documents a severe case of postsurgical pyoderma following an elective rotator cuff repair presenting with myositis and myonecrosis. The patient was initially treated as having an infection, which resulted in multiple aggressive surgical debridements. Despite this, the patient continued to deteriorate and was in a critical and hemodynamically unstable condition. Following administration of high-dose intravenous corticosteroids, the patient made a dramatic recovery and went on to have internal fixation of the shoulder and closure of the wound with a combination of a free flap and a rotational flap. Extensive myositis, as seen in this case, has not been previously reported in postoperative pyoderma gangrenosum variants. Clinicians should be aware that the presence of myositis and myonecrosis should not preclude this diagnosis.

  5. An animal model to study toxicity of central nervous system therapy for childhood acute lymphoblastic leukemia: Effects on growth and craniofacial proportion

    Many long term survivors of childhood acute lymphoblastic leukemia have short stature, as well as craniofacial and dental abnormalities, as side effects of central nervous system prophylactic therapy. An animal model is presented to assess these adverse effects on growth. Cranial irradiation (1000 cGy) with and without prednisolone (18 mg/kg i.p.) and methotrexate (2 mg/kg i.p.) was administered to 17- and 18-day-old Sprague-Dawley male and female rats. Animals were weighed 3 times/week. Final body weight and body length were measured at 150 days of age. Femur length and craniofacial dimensions were measured directly from the bones, using calipers. For all exposed groups there was a permanent suppression of weight gain with no catch-up growth or normal adolescent growth spurt. Body length was reduced for all treated groups, as were the ratios of body weight to body length and cranial length to body length. Animals subjected to cranial irradiation exhibited microcephaly, whereas those who received a combination of radiation and chemotherapy demonstrated altered craniofacial proportions in addition to microcephaly. Changes in growth patterns and skeletal proportions exhibited sexually dimorphic characteristics. The results indicate that cranial irradiation is a major factor in the growth failure in exposed rats, but chemotherapeutic agents contribute significantly to the outcome of growth and craniofacial dimensions

  6. The impact of therapy for childhood acute lymphoblastic leukaemia on intelligence quotients; results of the risk-stratified randomized central nervous system treatment trial MRC UKALL XI

    Vargha-Khadem Faraneh

    2011-10-01

    Full Text Available Abstract Background The MRC UKALLXI trial tested the efficacy of different central nervous system (CNS directed therapies in childhood acute lymphoblastic leukaemia (ALL. To evaluate morbidity 555/1826 randomised children underwent prospective psychological evaluations. Full Scale, verbal and performance IQs were measured at 5 months, 3 years and 5 years. Scores were compared in; (1 all patients (n = 555 versus related controls (n = 311, (2 low-risk children (presenting white cell count (WCC 9/l randomised to intrathecal methotrexate (n = 197 versus intrathecal and high-dose intravenous methotrexate (HDM (n = 202, and (3 high-risk children (WCC ≥ 50 × 109/l, age ≥ 2 years randomised to HDM (n = 79 versus cranial irradiation (n = 77. Results There were no significant differences in IQ scores between the treatment arms in either low- or high-risk groups. Despite similar scores at baseline, results at 3 and 5 years showed a significant reduction of between 3.6 and 7.3 points in all three IQ scores in all patient groups compared to controls (P Conclusion Children with ALL are at risk of CNS morbidity, regardless of the mode of CNS-directed therapy. Further work needs to identify individuals at high-risk of adverse CNS outcomes. Trial registration ISRCTN: ISRCTN16757172

  7. Effects of radiation on testicular function in long-term survivors of childhood acute lymphoblastic leukemia: A report from the Children Cancer Study Group

    Testicular function was evaluated in 60 long-term survivors of childhood acute lymphoblastic leukemia (ALL). All the patients were treated on two consecutive Children Cancer Study Group protocols and received identical chemotherapy and either 18 or 24 Gy radiation therapy (RT) to one of the following fields: craniospinal plus 12 Gy abdominal RT including the gonads (group 1); craniospinal (group 2); or cranial (group 3). The median age at the time of their last evaluation was 14.5 years (range, 10.5 to 25.7), which took place a median of 5.0 years (range, 1 to 10.3) after discontinuing therapy. The incidence of primary germ cell dysfunction as judged by raised levels of follicle-stimulating hormone (FSH) and/or reduced testicular volume was significantly associated with field of RT; 55% of group 1, 17% of group 2, and 0% of group 3 were abnormal (P = .002). Leydig cell function, as assessed by plasma concentrations of luteinizing hormone (LH) and testosterone, and pubertal development, was unaffected in the majority of subjects regardless of RT field. These data indicate that in boys undergoing therapy for ALL, germ cell dysfunction is common following testicular irradiation and can occur following exposure to scattered irradiation from craniospinal RT. In contrast, Leydig cell function appears resistant to direct irradiation with doses as high as 12 Gy

  8. Baicalein Triggers Mitochondria-Mediated Apoptosis and Enhances the Antileukemic Effect of Vincristine in Childhood Acute Lymphoblastic Leukemia CCRF-CEM Cells

    Yun-Ju Chen

    2013-01-01

    Full Text Available Acute lymphoblastic leukemia (ALL accounts for approximately 75% of childhood leukemia, and chemotherapy remains the mainstay therapy. Baicalein is an active flavonoid used in traditional Chinese medicine and has recently been found to have anticancer, anti-inflammatory, and antiallergic properties. This study aims to investigate the molecular apoptotic mechanisms of baicalein in CCRF-CEM leukemic cells and to evaluate the combined therapeutic efficacy of baicalein with several commonly used chemotherapeutic drugs in CCRF-CEM cells. Our results demonstrate that baicalein induces mitochondria-dependent cleavage of caspases-9 and -3 and PARP with concomitant decreases in IAP family proteins, survivin, and XIAP. Furthermore, our results present for the first time that baicalein triggers a convergence of the intrinsic and extrinsic apoptotic pathways via the death receptor-caspase 8-tBid signaling cascade in CCRF-CEM cells. In addition, we also present for the first time that the combination of baicalein and vincristine results in a synergistic therapeutic efficacy. Overall, this combination strategy is recommended for future clinical trials in the treatment of pediatric leukemia owing to baicalein’s beneficial effects in alleviating the vomiting, nausea, and skin rashes caused by chemotherapy.

  9. Slower early response to treatment and distinct expression profile of childhood high hyperdiploid acute lymphoblastic leukaemia with DNA index < 1.16.

    Zaliova, Marketa; Hovorkova, Lenka; Vaskova, Martina; Hrusak, Ondrej; Stary, Jan; Zuna, Jan

    2016-09-01

    Acute lymphoblastic leukaemias (ALL) with 51-67 chromosomes are defined as high hyperdiploid (HHD) and are generally associated with good prognosis. However, several studies show heterogeneity in HHD ALL and suggest that the favourable prognosis is associated rather with higher ploidy defined by DNA index (DNAi) ≥ 1.16 or with a presence of specific single or combined trisomies. HHD ALL with DNAi < 1.16 are only rarely studied separately. Using single nucleotide polymorphism array, we analysed 89 childhood HHD ALL patients divided into groups with lower (<1.16; n = 34) and higher (≥1.16; n = 55) DNAi. We assessed treatment response, presence of secondary aberrations, mutations in RAS pathway genes and CREBBP and also gene expression profile (GEP) to reveal differences between the two subgroups. Cases with 51-54 chromosomes had DNAi 1.1-1.16 and cases with 55-67 chromosomes had DNAi ≥ 1.16. The groups with lower and higher DNAi had distinct response to early treatment and distinct GEP. The better response of the group with higher DNAi was associated with specific trisomies (trisomy of chromosome 10 or combined with trisomies 4 and/or 17). Our results suggest that cytogenetically defined HHD ALL can in fact be divided into two biologically distinguishable subgroups and that DNAi 1.16 is a relevant value to separate between the two. © 2016 Wiley Periodicals, Inc. PMID:27163296

  10. Lymphoid Progenitor Cells from Childhood Acute Lymphoblastic Leukemia Are Functionally Deficient and Express High Levels of the Transcriptional Repressor Gfi-1

    Jessica Purizaca

    2013-01-01

    Full Text Available Acute lymphoblastic leukemia (ALL is the most frequent malignancy of childhood. Substantial progress on understanding the cell hierarchy within ALL bone marrow (BM has been recorded in the last few years, suggesting that both primitive cell fractions and committed lymphoid blasts with immature stem cell-like properties contain leukemia-initiating cells. Nevertheless, the biology of the early progenitors that initiate the lymphoid program remains elusive. The aim of the present study was to investigate the ability of lymphoid progenitors from B-cell precursor ALL BM to proliferate and undergo multilineage differentiation. By phenotype analyses, in vitro proliferation assays, and controlled culture systems, the lymphoid differentiation potentials were evaluated in BM primitive populations from B-cell precursor ALL pediatric patients. When compared to their normal counterparts, functional stem and progenitor cell contents were substantially reduced in ALL BM. Moreover, neither B nor NK or dendritic lymphoid-cell populations developed recurrently from highly purified ALL-lymphoid progenitors, and their proliferation and cell cycle status revealed limited proliferative capacity. Interestingly, a number of quiescence-associated transcription factors were elevated, including the transcriptional repressor Gfi-1, which was highly expressed in primitive CD34+ cells. Together, our findings reveal major functional defects in the primitive hematopoietic component of ALL BM. A possible contribution of high levels of Gfi-1 expression in the regulation of the stem/progenitor cell biology is suggested.

  11. Complications in the central nervous system during chemotherapy for childhood acute lymphoblastic leukemia. JACLS ALL-02 study

    We evaluated central nervous system (CNS) complications treated under the ALL-02 protocol of the Japan Association of Childhood Leukemia Study (JACLS) from April 2002 to March 2005. According to National Cancer Institute (NCI) Toxicity Criteria, 17 events of grade 3 and 4 CNS complications were reported in 15 out of 541 patients. Out of these CNS complications, leukoencephalopathy was seen in 5 patients; seizure in 5; cerebrovascular disease in 3; conscious disturbance in 2; and hypertensive encephalopathy and reversible posterior leukoencephalopathy syndrome in one patient each. The complications were intensively observed during induction therapy and the last of the early phase chemotherapy. The protocol treatment was stopped or modified in most patients after CNS complications. MRI imaging demonstrated no improvement in one patient with leukoencephalopathy who developed an isolated CNS relapse, while other patients were alive and remain in their first complete remission without any neurological sequelae. Further studies will be required to analyze risk factors for CNS complications during chemotherapy not accompanied by irradiation and to establish alternative treatments after the appearance of such CNS complications. (author)

  12. An animal model to study toxicity of central nervous system therapy for childhood acute lymphoblastic leukemia: Effects on behavior

    Central nervous system prophylactic therapy used in the treatment of acute lymphoblastic leukemia can reduce intelligence quotient scores and impair memory and attention in children. Cranial irradiation, intrathecal methotrexate, and steroids are commonly utilized in acute lymphoblastic leukemia therapy. How they induce neurotoxicity is unknown. This study employs an animal model to explore the induction of neurotoxicity. Male and female Sprague-Dawley rats at 17 and 18 days of age were administered 18 mg/kg prednisolone, 2 mg/kg methotrexate, and 1000 cGy cranial irradiation. Another 18-day-old group was administered 1000 cGy cranial irradiation but no drugs. Matching controls received saline and/or a sham exposure to radiation. All animals at 6 weeks and 4 months of age were tested for alterations in spontaneous behavior. A computer pattern recognition system automatically recorded and classified individual behavioral acts displayed during exploration of a novel environment. Measures of behavioral initiations, total time, and time structure were used to compare treated and control animals. A permanent sex-specific change in the time structure of behavior was induced by the prednisolone, methotrexate, and radiation treatment but not by radiation alone. Unlike hyperactivity, the effect consisted of abnormal clustering and dispersion of acts in a pattern indicative of disrupted development of sexually dimorphic behavior. This study demonstrates the feasibility of an animal model delineating the agent/agents responsible for the neurotoxicity of central nervous system prophylactic therapy

  13. Next-generation-sequencing of recurrent childhood high hyperdiploid acute lymphoblastic leukemia reveals mutations typically associated with high risk patients.

    Chen, Cai; Bartenhagen, Christoph; Gombert, Michael; Okpanyi, Vera; Binder, Vera; Röttgers, Silja; Bradtke, Jutta; Teigler-Schlegel, Andrea; Harbott, Jochen; Ginzel, Sebastian; Thiele, Ralf; Husemann, Peter; Krell, Pina F I; Borkhardt, Arndt; Dugas, Martin; Hu, Jianda; Fischer, Ute

    2015-09-01

    20% of children suffering from high hyperdiploid acute lymphoblastic leukemia develop recurrent disease. The molecular mechanisms are largely unknown. Here, we analyzed the genetic landscape of five patients at relapse, who developed recurrent disease without prior high-risk indication using whole-exome- and whole-genome-sequencing. Oncogenic mutations of RAS pathway genes (NRAS, KRAS, FLT3, n=4) and deactivating mutations of major epigenetic regulators (CREBBP, EP300, each n=2 and ARID4B, EZH2, MACROD2, MLL2, each n=1) were prominent in these cases and virtually absent in non-recurrent cases (n=6) or other pediatric acute lymphoblastic leukemia cases (n=18). In relapse nucleotide variations were detected in cell fate determining transcription factors (GLIS1, AKNA). Structural genomic alterations affected genes regulating B-cell development (IKZF1, PBX1, RUNX1). Eleven novel translocations involved the genes ART4, C12orf60, MACROD2, TBL1XR1, LRRN4, KIAA1467, and ELMO1/MIR1200. Typically, patients harbored only single structural variations, except for one patient who displayed massive rearrangements in the context of a germline tumor suppressor TP53 mutation and a Li-Fraumeni syndrome-like family history. Another patient harbored a germline mutation in the DNA repair factor ATM. In summary, the relapse patients of our cohort were characterized by somatic mutations affecting the RAS pathway, epigenetic and developmental programs and germline mutations in DNA repair pathways. PMID:26189108

  14. An animal model to study toxicity of central nervous system therapy for childhood acute lymphoblastic leukemia: Effects on behavior

    Mullenix, P.J.; Kernan, W.J.; Tassinari, M.S.; Schunior, A.; Waber, D.P.; Howes, A.; Tarbell, N.J. (Forsyth Research Institute, Boston, MA (USA))

    1990-10-15

    Central nervous system prophylactic therapy used in the treatment of acute lymphoblastic leukemia can reduce intelligence quotient scores and impair memory and attention in children. Cranial irradiation, intrathecal methotrexate, and steroids are commonly utilized in acute lymphoblastic leukemia therapy. How they induce neurotoxicity is unknown. This study employs an animal model to explore the induction of neurotoxicity. Male and female Sprague-Dawley rats at 17 and 18 days of age were administered 18 mg/kg prednisolone, 2 mg/kg methotrexate, and 1000 cGy cranial irradiation. Another 18-day-old group was administered 1000 cGy cranial irradiation but no drugs. Matching controls received saline and/or a sham exposure to radiation. All animals at 6 weeks and 4 months of age were tested for alterations in spontaneous behavior. A computer pattern recognition system automatically recorded and classified individual behavioral acts displayed during exploration of a novel environment. Measures of behavioral initiations, total time, and time structure were used to compare treated and control animals. A permanent sex-specific change in the time structure of behavior was induced by the prednisolone, methotrexate, and radiation treatment but not by radiation alone. Unlike hyperactivity, the effect consisted of abnormal clustering and dispersion of acts in a pattern indicative of disrupted development of sexually dimorphic behavior. This study demonstrates the feasibility of an animal model delineating the agent/agents responsible for the neurotoxicity of central nervous system prophylactic therapy.

  15. PARC/CCL18 Is a Plasma CC Chemokine with Increased Levels in Childhood Acute Lymphoblastic Leukemia

    Struyf, Sofie; Schutyser, Evemie; Gouwy, Mieke; Gijsbers, Klara; Proost, Paul; Benoit, Yves; Opdenakker, Ghislain; Van Damme, Jo; Laureys, Geneviève

    2003-01-01

    Chemokines play an important role in leukocyte mobilization, hematopoiesis, and angiogenesis. Tissue-specific expression of particular chemokines also influences tumor growth and metastasis. Here, the CC chemokine pulmonary and activation-regulated chemokine (PARC)/CCL18 was measured in pediatric patients with acute lymphoblastic leukemia (ALL) or acute myeloid leukemia (AML). Surprisingly, PARC immunoreactivity was consistently detected in plasma from healthy donors. After purification to homogeneity, the presence of intact PARC (1–69) and processed PARC (1–68) in normal human plasma was confirmed by sequence and mass spectrometry analysis. Furthermore, PARC serum levels were significantly increased in children with T-ALL and prepreB-ALL compared to control serum samples, whereas serum levels in AML and preB-ALL patients were not significantly different from controls. In contrast, the hemofiltrate CC chemokine-1 (HCC-1)/CCL14 was not found to be a biomarker in any of these patients’ strata, whereas the cytokine interleukin-6 (IL-6) was significantly decreased in AML and prepreB-ALL. Stimulated leukocytic cell lines or lymphoblasts from patients produced IL-8/CXCL8 or macrophage inflammatory protein-1α (MIP-1α/CCL3) but not PARC, not even after IL-4 or IL-10 treatment. However, PARC was produced by superantigen or IL-4 stimulated monocytes co-cultured with lymphocytes or lymphoblastic cells. Serum PARC levels thus constitute a novel leukemia marker, possibly reflecting tumor/host cell interactions in the circulation. PMID:14578205

  16. [Acute leukemia in childhood: present status of 100 cases after 7 years of complete remission (author's transl)].

    Schaison, G; Jacquillat, C; Lemercier, N; Weil, M; Alby, N; Auclerc, M F; Boiron, M; Bernard, J

    1980-01-01

    Amongst 1,200 leukemie children treated between 1958 and 1971, 60 are in complete remission for more than 10 years and 100 for more than 7 years. There were 96 acute lymphoid and 4 acute myeloid leukemias. Ten patients who have relapsed in the past have not done so lately. The F/M sex ratio is 1.5. Poor prognostic features were initially absent in 2/3 of cases. In 1/3 there was associated hyperleucocytosis and/or tumours. 93 children are in remission, their treatment having been stopped for 1 to 12 years. Five children relapsed and 4 are in a second remission for more than 2 years. Two children died in remission: one from a hepatocarcinoma and one from cardiac failure. These patients have been shown to have the following: 1) normal growth; 2) normal puberty: 8 patients have been able to reproduce, giving 10 children, one with multiple malformations; 3) school achievement and later socioprofessional behaviour has been normal. The patients have often sought a medical or paramedical career. Sequelae are minimal, psychological problems being minimal in the child. With the protocols used, mean remission curve shows a plateau after 9 years and complete definitive care is achieved in 92 per cent of patients surviving at 7 years. The very distant future outlook is not known. No other malignant haematological disease has occurred but one child died from a carcinoma. PMID:6931627

  17. Intracranial hemorrhage in acute and chronic childhood immune thrombocytopenic purpura over a ten-year period: an Egyptian multicenter study.

    Elalfy, Mohsen; Elbarbary, Nancy; Khaddah, Normine; Abdelwahab, Magy; El Rashidy, Farida; Hassab, Hoda; Al-Tonbary, Youssef

    2010-01-01

    Intracranial hemorrhage (ICH) is a rare but major cause of death in immune thrombocytopenic purpura (ITP). The authors reviewed data of 1,840 patient with ITP, from 5 pediatric hematology centers in Egypt from 1997 to 2007, to study the incidence and risk factors of ICH. Ten cases of ICH were identified with a median age at presentation of 7.5 years; 4 patients had acute ITP, 2 persistent and 4 chronic. The platelet count was late referral to a specialized center. Our results suggest that treatment does not prevent ICH and that it can occur at any time during the course of the disease. Delayed referral can be considered a risk factor for unfavorable outcome of ICH, highlighting the importance of teaching sessions for patients and their parents to minimize subsequent morbidity and mortality of ICH in children with ITP. PMID:19955713

  18. CT studies before and after CNS treatment for acute lymphoblastic leukemia and malignant non-Hodgkin's lymphoma in childhood

    CT was performed on 72 children with acute lymphoblasitc leukemia or non-Hodgkin's lymphoma. Thirty-two of these patients were investigated prior to CNS radiation and intrathecal methotrexate therapy. Ten of these patients (31%) were known to have hydrocephalic dilatation of the CSF spaces. Clinical data and subsequent observations with analysis of the CT findings show that no difference in the attenuation values of brain tissue occurs in the absence of a CNS relapse. The percentage of abnormal findings before and after therapy remains constant. The adverse late effects described in the CT literature seem principally to be damage diagnosed too late. It is questionable if the CT demonstration of dilated CSF spaces before treatment has a prognostic significance. (orig.)

  19. The clinical importance of myeloid antigen coexpression and TEL-AML1 mutation in patients with childhood acute lymphoblastic leukemia

    Ayşen Türedi Yıldırım

    2013-03-01

    Full Text Available Objective: In this study, we aim to investigate the relationship,if any, between clinical features, prognosis, and thecoexpressions and TEL-AML1 mutation in patients withacute lymphoblastic leukemia (ALL.Methods: Eigthy-three patients with acute lymphoblasticleukemia were retrospectively examined. Age, gender,White blood cell count, hemoglobin level, platelet count,ALL subtypei (B or T ALL, risk groups, surface antigensdeteceted by flow cytometry, existence of TEL-AML1 mutations,response, remission and relapse status at 8., 15.ve 33. Days of treatment were recorded and analyzed.Results: 15 (18% out of 83 were identified with aberrantantigen expression. Of these patients, twelve (14.4%had myeloid antigen coexpression (CD13 and/or CD33,two with B cell ALL had CD2 and CD7 coexpressions respectively,one with T cell ALL had CD19 coexpression.No significant differences were found between patientswith and without myeloid antigen coexpression in terms ofhemoglobin levels, white blood cells and platelet counts,responses given on the 8th, 15th, and 30th days on the treatment,risk groups, and relapse (p>0.05. Myeloid antigencoexpression was found in 4 of 13 patients who were identifiedwith TEL-AML1 mutation. No significant relationshipwas found between this mutation and coexpressions. Norelapse and exitus were observed in four patients with coexpressionand TEL-AML1.Conclusion: The prognosis and clinical features showsno statistically significant relationship with the presence ofneither Myeloid antigen expression nor TEL-AML1 mutation.We believe, however, the future studies involving biggersample sizes will prove to be useful in terms of moreconvincing results. J Clin Exp Invest 2013; 4(1: 90-94Key words: Acute lenfoblastic leukemia, coexpression,TEL-AML1 mutation, prognosis

  20. Biological Therapy in Treating Patients With Advanced Myelodysplastic Syndrome, Acute or Chronic Myeloid Leukemia, or Acute Lymphoblastic Leukemia Who Are Undergoing Stem Cell Transplantation

    2013-07-03

    Adult Acute Myeloid Leukemia With 11q23 (MLL) Abnormalities; Adult Acute Myeloid Leukemia With Inv(16)(p13;q22); Adult Acute Myeloid Leukemia With t(15;17)(q22;q12); Adult Acute Myeloid Leukemia With t(16;16)(p13;q22); Adult Acute Myeloid Leukemia With t(8;21)(q22;q22); B-cell Adult Acute Lymphoblastic Leukemia; B-cell Childhood Acute Lymphoblastic Leukemia; Childhood Chronic Myelogenous Leukemia; Childhood Myelodysplastic Syndromes; Chronic Myelomonocytic Leukemia; Essential Thrombocythemia; Polycythemia Vera; Previously Treated Myelodysplastic Syndromes; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Acute Myeloid Leukemia; Recurrent Childhood Acute Lymphoblastic Leukemia; Recurrent Childhood Acute Myeloid Leukemia; Refractory Anemia With Excess Blasts; Refractory Anemia With Excess Blasts in Transformation; Relapsing Chronic Myelogenous Leukemia; Secondary Acute Myeloid Leukemia; T-cell Adult Acute Lymphoblastic Leukemia; T-cell Childhood Acute Lymphoblastic Leukemia

  1. A comparative study of central nervous system irradiation and intensive chemotherapy early in remission of childhood acute lymphocytic leukemia

    A study was designed to determine whether a one-week course of intensive chemotherapy and 2400 rads craniospinal irradiation prolonged complete remission of acute lymphocytic leukemia (ALL) in children. Of 110 patients entered into the study, 104 (94%) attained complete remission, 94 of whom were available for the 2 randomizations. They were randomly assigned to receive or not receive one week of high-dosage intravenous chemotherapy and, 4 weeks later, were again randomized to receive or not receive 2400 rads cobalt-60 craniospinal irradiation. Patients randomized for no irradiation were to receive identical radiotherapy only if and when central nervous system (CNS) leukemia developed. The one week of intensive chemotherapy had no effect on the duration of remission or on the frequency or site of relapse, but irradiation had marked effect. Complete remission was terminated by CNS leukemia in only 2 of 45 children who received ''prophylactic'' craniospinal irradiation compared to 27 to 49 not irradiated. FIve of the 25 children who were given ''therapeutic'' irradiation for demonstrated CNS leukemia have already had recurrences despite continuous hematologic remission. Under the conditions of this study, the authors conclude that one week of intensive chemotherapy does not prolong remission, that 2400 rads craniospinal irradiation early in remission prevents or delays CNS leukemia and prolongs complete remission, and that once CNS leukemia develops, 2400 rads craniospinal irradiation is not sufficient to eradicate it

  2. Presence of FLT3-ITD and high BAALC expression are independent prognostic markers in childhood acute myeloid leukemia.

    Staffas, Anna; Kanduri, Meena; Hovland, Randi; Rosenquist, Richard; Ommen, Hans Beier; Abrahamsson, Jonas; Forestier, Erik; Jahnukainen, Kirsi; Jónsson, Ólafur G; Zeller, Bernward; Palle, Josefine; Lönnerholm, Gudmar; Hasle, Henrik; Palmqvist, Lars; Ehrencrona, Hans

    2011-11-24

    Mutation status of FLT3, NPM1, CEBPA, and WT1 genes and gene expression levels of ERG, MN1, BAALC, FLT3, and WT1 have been identified as possible prognostic markers in acute myeloid leukemia (AML). We have performed a thorough prognostic evaluation of these genetic markers in patients with pediatric AML enrolled in the Nordic Society of Pediatric Hematology and Oncology (NOPHO) 1993 or NOPHO 2004 protocols. Mutation status and expression levels were analyzed in 185 and 149 patients, respectively. Presence of FLT3-internal tandem duplication (ITD) was associated with significantly inferior event-free survival (EFS), whereas presence of an NPM1 mutation in the absence of FLT3-ITD correlated with significantly improved EFS. Furthermore, high levels of ERG and BAALC transcripts were associated with inferior EFS. No significant correlation with survival was seen for mutations in CEBPA and WT1 or with gene expression levels of MN1, FLT3, and WT1. In multivariate analysis, the presence of FLT3-ITD and high BAALC expression were identified as independent prognostic markers of inferior EFS. We conclude that analysis of the mutational status of FLT3 and NPM1 at diagnosis is important for prognostic stratification of patients with pediatric AML and that determination of the BAALC gene expression level can add valuable information. PMID:21967978

  3. Contributing Factors for Acute Illness/Injury from Childhood Pesticide Exposure in North Carolina, USA, 2007–2013

    Nirmalla Barros

    2016-02-01

    Full Text Available Between 2007 and 2013, there were 685 events with evidence of a relationship between pesticide exposure and acute illness/injury among persons less than 18 years old in North Carolina (United States. Median age of children affected was 4.3 years (range: 0.2–17.9. Distribution by gender was similar across all age groups. One fatality and four high severity events were observed. The greatest proportion (42% of events had ocular exposures, followed by dermal (25% and inhalation (18% exposures. When more than one route of exposure occurred, dermal and ocular routes were the most common (46%. Almost all events took place indoors and 32 events involved contact with pets. Insecticides (53% and insect repellants (31% were the most frequent agents contributing to these events. Manual application of pesticides contributed to the greatest number of events (25%, while application through a pressurized can and use of a trigger pump were involved in 21% and 15% of events, respectively. Additional contributors were due to inappropriate storage of pesticides and improper use of the pesticide. These contributing factors can be removed or minimized if pesticides are stored outside the residence or out of the reach of children and pets, and adequate ventilation is ensured whenever pesticides are applied.

  4. Intrinsic and chemo-sensitizing activity of SMAC-mimetics on high-risk childhood acute lymphoblastic leukemia.

    Schirmer, M; Trentin, L; Queudeville, M; Seyfried, F; Demir, S; Tausch, E; Stilgenbauer, S; Eckhoff, S M; Meyer, L H; Debatin, K-M

    2016-01-01

    SMAC-mimetics represent a targeted therapy approach to overcome apoptosis resistance in many tumors. Here, we investigated the efficacy of the SMAC-mimetic BV6 in B-cell precursor acute lymphoblastic leukemia (BCP-ALL). In ALL cell lines, intrinsic apoptosis sensitivity was associated with rapid cIAP degradation, NF-κB activation, TNF-α secretion and induction of an autocrine TNF-α-dependent cell death loop. This pattern of responsiveness was also observed upon ex vivo analysis of 40 primograft BCP-ALL samples. Treatment with BV6 induced cell death in the majority of ALL primografts including leukemias with high-risk and poor-prognosis features. Inhibition of cell death by the TNF receptor fusion protein etanercept demonstrated that BV6 activity is dependent on TNF-α. In a preclinical NOD/SCID/huALL model of high-risk ALL, marked anti-leukemia effectivity and significantly prolonged survival were observed upon BV6 treatment. Interestingly, also in vivo, intrinsic SMAC-mimetic activity was mediated by TNF-α. Importantly, BV6 increased the effectivity of conventional induction therapy including vincristine, dexamethasone and asparaginase leading to prolonged remission induction. These data suggest SMAC-mimetics as an important addendum to efficient therapy of pediatric BCP-ALL. PMID:26775704

  5. Comparative genomic hybridization in childhood acute lymphoblastic leukemia: correlation with interphase cytogenetics and loss of heterozygosity analysis.

    Scholz, I; Popp, S; Granzow, M; Schoell, B; Holtgreve-Grez, H; Takeuchi, S; Schrappe, M; Harbott, J; Teigler-Schlegel, A; Zimmermann, M; Fischer, C; Koeffler, H P; Bartram, C R; Jauch, A

    2001-01-15

    We used comparative genomic hybridization (CGH) to study DNA copy number changes in 71 children with acute lymphoblastic leukemia (ALL) including 50 B-lineage and 21 T-ALLs. Forty-two patients (59%) showed genomic imbalances whereby gains were more frequently observed than losses (127 vs. 29). Gains most commonly affected the entire chromosomes 21 and 10 (19.7% each), 6, 14, 18, X (15.5% each), 17 (14.1%) and 4 (11.3%). Highly hyperdiploid karyotypes (chromosome number >50) occurred more frequently in B-lineage than in T-lineage ALL (24% vs. 4.8%). In both cell lineages deletions were mainly detected on 9p (14.1%) and 12p (8.4%), and on 6q in T-lineage ALL (4.2%). These findings were compared with loss of heterozygosity (LOH) of 6q, 9p, 11q, and 12p previously performed in 56 of the 71 patients. Among 54 sites of LOH, CGH revealed losses of the respective chromosome arms in 17 LOH-positive regions (31.5%). G-banding analysis and interphase cytogenetics with subregional probes for 14 loci confirmed the presence of genomic imbalances as detected by CGH. We, therefore, conclude that, in the absence of cytogenetic data, CGH represents a suitable method for identifying hyperdiploid karyotypes as well as prognostically relevant deletions in ALL patients. PMID:11172898

  6. Minimal effects of E. coli and Erwinia asparaginase on the coagulation system in childhood acute lymphoblastic leukemia: a randomized study.

    Risseeuw-Appel, I M; Dekker, I; Hop, W C; Hählen, K

    1994-01-01

    A randomized study was done in twenty newly diagnosed children with acute lymphoblastic leukemia. Ten children were treated with Escherichia coli L-asparaginase, and ten with Erwinia chrysanthemi L-asparaginase. L-asparaginase (ASP) treatment started halfway during ALL-induction treatment with vincristine, prednisone, daunorubicin and intrathecal methotrexate. The mean activated partial thromboplastin time (APTT) level in all children demonstrated a significant fall (P II, V, VII and X stayed within the normal range, while F VIII and F IX were elevated. During the entire period of induction therapy, the ATIII activity remained within the normal range in both treatment groups. The protein C values, however, demonstrated a steady decline from 140% at start of ASP treatment to a mean of 81% and 93%, respectively, at the end of the ASP therapy in the E. coli and Erwinia group. Five of the ten children treated with E. coli ASP demonstrated protein C levels below 70% at the end of ASP therapy, opposed to none of the Erwinia treated patients (P = 0.03). We suggest that the effect of ASP resulting in decreased coagulation factor synthesis is in part counterbalanced by the effect of prednisone on the coagulation system, when ASP is administered at the end of ALL induction treatment. The overall effect of ASP either of E. coli or of Erwinia on the hemorrhagic system reveals a slight imbalance towards thrombosis, mainly because of a gradual decrease in protein C activity. This imbalance is less pronounced in the Erwinia group. PMID:8058004

  7. 小儿急性偏瘫综合征%Study on acute hemiplegia syndrome in childhood

    王纪文

    2004-01-01

    小儿急性偏瘫综合征(acute hemiplegia syndrome in childhood.AHS)早在1897年即由Sigmund Freud详细描述,直到1960年以后才有较多报道。小儿急性偏瘫是由多种原因引起的以急性一侧肢体瘫痪为主要表现的临床综合征,目前该诊断名词倾向于只用于未找到特异原闪的急性偏瘫,其他则可用更为明确的诊断如:脑动脉血栓形成等代替,本文仍延用以往文献称谓,泛指上述情况。美国有学者调查15岁以下儿童中,脑卒中引起的急性偏瘫为2.48/万。国内尚缺乏流行病学方面的调查。其发病机制主要是脑血流灌注不足,导致局部脑组织缺血、坏死累及锥体束的功能。

  8. Inherent illnesses and attacks: an ethnographic study of interpretations of childhood Acute Respiratory Infections (ARIs in Manhiça, southern Mozambique

    Pell Christopher

    2011-07-01

    Full Text Available Abstract Background Pneumonia is a leading cause of childhood hospitalisation and child mortality in Africa. This study explores local interpretations of Acute Respiratory Infections (ARIs, focusing on caretakers of children under five in the context of hospital care seeking. Methods The study took place in Manhiça, southern Mozambique and used Focused Ethnographic Study tools (FES including field exercises and interviews. Results Understandings of terms used to describe ARIs differed between caretakers and hospital staff. Children's sicknesses that hospital staff diagnosed as ARIs were interpreted by caretakers as intermittent "attacks" of xifuva, a permanent, inherent and incurable chest illness. Caretakers thought that it was possible to manage and treat the attacks, which were caused by immediate natural factors such as food or the weather, but not the underlying illness, which was seen as having more indirect and social causes. Explanations of illness could not be neatly separated into pluralistic categories, but were characterised by syncretism, with "lay" and "biomedical" terms and concepts intermingling in practical care-seeking interactions between caretakers and health staff. Conclusions Health promotion should take into account the syncretism involved in explanations of ARIs in the context of practical care seeking for children. In doing so, it should draw upon lay interpretations and terminologies in order to stress the importance of seeking hospital care for all xifuva-type illnesses as well as seeking care for any subsequent attacks of an already diagnosed xifuva. However, this should be undertaken with awareness that the meanings of the terms used in practical care-seeking interactions may change over time. Health communication about ARIs should therefore be ongoing and evidence-based, even if ARIs appear to be well understood.

  9. Immunophenotype of adult and childhood acute lymphoblastic leukemia: changes at first relapse and clinico-prognostic implications.

    Guglielmi, C; Cordone, I; Boecklin, F; Masi, S; Valentini, T; Vegna, M L; Ferrari, A; Testi, A M; Foa, R

    1997-09-01

    The immunologic features of leukemic cells at the time of 1st hematologic relapse were compared to those obtained at initial diagnosis in 128 patients (69 children and 59 adults) with acute lymphoblastic leukemia (ALL) treated at a single institution. An immunophenotypic change was observed in 59 cases (46%), more frequently in T (20/25) than in B (39/103) lineage ALL (80 vs 38%, P=0.0008), but with a similar incidence in adults and children. Of these cases, 34 (24 B- and 10 T-ALL) changed at relapse their intralineage subgroup affiliation, although no complete shift from B to T lineage ALL, or vice versa, was observed. The myeloid antigens CD13 and/or CD33 were frequently lost (2/5 cases) or acquired (12/123 cases) at relapse. In 21 cases, the immunophenotype at relapse was more undifferentiated than at diagnosis, while it was more differentiated in 13 cases. Initial treatment intensity or preceding treatment with teniposide did not affect the phenotypic profile at relapse. Complete response (CR) rate to salvage therapy and event-free survival were not influenced by the immunophenotypic shifts, nor by the presence, at relapse, of leukemic cells expressing the myeloid antigens CD13 and/or CD33. Univariate analysis suggested that prognosis after relapse was dependent on the duration of 1st CR, patients' age and immunophenotype at the time of diagnosis, with a worse outcome for patients with T lineage ALL and for patients with the less differentiated subgroup of B lineage ALL (CD19+ and CD10-). Multivariate analysis showed that only two factors, duration of 1st CR and grade of immunologic differentiation at diagnosis, have independent prognostic value in relapsed ALL. PMID:9305605

  10. Early mechanical dysfunction of the diaphragm in the muscular dystrophy with myositis (Ttnmdm) model

    Lopez, Michael A.; Pardo, Patricia S.; Cox, Gregory A.; Boriek, Aladin M.

    2008-01-01

    A complex rearrangement mutation in the mouse titin gene leads to an in-frame 83-amino acid deletion in the N2A region of titin. Autosomal recessive inheritance of the titin muscular dystrophy with myositis (Ttnmdm/mdm) mutation leads to a severe early-onset muscular dystrophy and premature death. We hypothesized that the N2A deletion would negatively impact the force-generating capacity and passive mechanical properties of the mdm diaphragm. We measured in vitro active isometric contractile ...

  11. Myositis Ossificans Mimicking Sarcoma, the Importance of Diagnostic Imaging – Case Report

    Myositis ossificans is localized inflammatory process affecting skeletal muscles. Very rarely it can affect one of the neck muscles and present as a neck tumor, it can be misdiagnosed as the clinical, radiological and histological examinations can mimic a sarcoma. We report a 29 year old female patient with neck tumor suspected to be a sarcoma who underwent full diagnostics imaging and open bipsy with histopatological examination, afterwards surgical excision was performed. The aim of this study was to present the differential diagnosis based on diagnostics imaging between MO and malignant tumors, such as parosteal sarcoma, synovial sarcoma and malignant fibrous histiocytoma

  12. Inflammatory myositis complicating hypocomplementemic urticarial vasculitis despite on-going immunosuppression.

    Chew, Gary Y J; Gatenby, Paul A

    2007-08-01

    We present a patient with previously diagnosed hypocomplementemic urticarial vasculitis syndrome, with skin, lung, and renal involvement, who presented with congestive cardiac failure. During the course of her hospitalization, she was also found to have profound proximal muscle weakness in both upper and lower limbs associated with raised creatinine kinase levels. A muscle biopsy was performed, which demonstrated evidence of an inflammatory myositis with vasculitis, which had returned despite on-going immunosuppression. This occurrence of a new autoimmune disease may well be an example of the "waste disposal" hypothesis. PMID:16944070

  13. NEUTROPHILIC MYOSITIS ASSOCIATED WITH PYODERMA GANGRENOSUM IN A BREAK-DANCER

    Hisashi Tamiya

    2013-10-01

    Full Text Available Neutrophilic myositis is an extremely rare condition, cases of which have been reported in association with neutrophilic dermatosis, inflammatory bowel disease and malignant hematological disease. The disorder is histologically characterized by a sterile infiltration of neutrophils throughout muscle, with necrosis of muscle fibres. We here report the case of a young male who also had associated pyoderma gangrenosum, and who presented with necrotizing fasciitis-like manifestations. In this case, although there were no other underlying disorders, compulsive exertional stress due to break-dancing was thought to be a precipitant. Debridement of the necrotic tissues combined with oral corticosteroid treatment was effective.

  14. In Inclusion-Body Myositis Muscle Fibers Parkinson-Associated DJ-1 is Increased and Oxidized

    Terracciano, Chiara; Nogalska, Anna; Engel, W. King; Wojcik, Slawomir; Askanas, Valerie

    2008-01-01

    Sporadic inclusion-body myositis (s-IBM) is the most common muscle disease of older persons. The muscle-fiber molecular phenotype exhibits similarities to both Alzheimer-disease (AD) and Parkinson-disease (PD) brains, including accumulations of amyloid-β, phosphorylated tau, α-synuclein and parkin, as well as evidence of oxidative stress and mitochondrial abnormalities. Early-onset autosomal-recessive PD can be caused by mutations in the DJ-1 gene, leading to its inactivation. DJ-1 has anti-o...

  15. Sporadic inclusion body myositis: variability in prevalence and phenotype and influence of the MHC

    Mastaglia, Fl

    2009-01-01

    Sporadic inclusion body myositis (sIBM) is the most common myopathy presenting over the age of 40 years but its prevalence varies considerably in different populations. Genetic factors play a part in the pathogenesis of sIBM and in Caucasians susceptibility has been linked to the HLA-DR3 allele and the 8.1 MHC ancestral haplotype (AH) which is also associated with other autoimmune diseases. The variable prevalence of sIBM in different populations may be related to differences in the populatio...

  16. Evaluation of ETV6/RUNX1 Fusion and Additional Abnormalities Involving ETV6 and/or RUNX1 Genes Using FISH Technique in Patients with Childhood Acute Lymphoblastic Leukemia.

    Aydin, Cigdem; Cetin, Zafer; Manguoglu, Ayse Esra; Tayfun, Funda; Clark, Ozden Altiok; Kupesiz, Alphan; Akkaya, Bahar; Karauzum, Sibel Berker

    2016-06-01

    Childhood acute lymphoblastic leukemia (ALL) is the most common type of childhood leukemia. Specifically, ALL is a malignant disorder of the lymphoid progenitor cells, with a peak incidence among children aged 2-5 years. The t(12;21)(p13;q22) translocation occurs in 25 % of childhood B cell precursor ALL. In this study, bone marrow samples were obtained from 165 patients with childhood ALL. We analyzed the t(12;21) translocation and other related abnormalities using the fluorescent in situ hybridization (FISH) technique with the ETV6(TEL)/RUNX1(AML1) ES dual color translocation probe. Conventional cytogenetic analyses were also performed. ETV6 and RUNX1 related chromosomal abnormalities were found in 42 (25.5 %) of the 165 patients with childhood ALL. Among these 42 patients, structural changes were detected in 33 (78.6 %) and numerical abnormalities in 9 (21.4 %). The frequency of FISH abnormalities in pediatric ALL cases were as follows: 8.5 % for t(12;21)(p13;q22) ETV6/RUNX1 fusion, 6.0 % for RUNX1 amplification, 3.0 % for tetrasomy/trisomy 21, 1.8 % for ETV6 deletion, 1.21 % for ETV6 deletion with RUNX1 amplification, 1.21 % for ETV6 amplification with RUNX1 amplification, 0.6 % for polyploidy, 0.6 % for RUNX1 deletion, and 0.6 % for diminished ETV6 signal. The most common structural abnormality was the t(12;21) translocation, followed by RUNX1 amplification and ETV6 deletion, while the most commonly observed numerical abnormality was trisomy 21. PMID:27065576

  17. Sporadic inclusion body myositis in Japanese is associated with the MHC ancestral haplotype 52.1.

    Scott, Adrian Phillip; Allcock, Richard James Nigel; Mastaglia, Frank; Nishino, Ichizo; Nonaka, Ikuya; Laing, Nigel

    2006-05-01

    In Caucasians, sporadic inclusion body myositis has been associated with the MHC ancestral haplotypes; HLA-A1, B8, DR3 (8.1AH) and HLA-B35, DR1 (35.2AH). It is not known whether these haplotypes carry susceptibility for the disease in other ethnic groups. We report here the results of HLA-B and -DRB1 typing using a high-resolution sequence-based technique in a cohort of 31 Japanese patients with definite sIBM. Patient allele frequencies were 40.3% for HLA-B*5201 (10.7% in controls: pHLA-DRB1*1502 (10% in controls: p<0.001). Both alleles were found together as part of a conserved haplotype (52.1AH) at a frequency of 37.1% in patients (8.4% in controls: p<0.001). This is the first description of a haplotypic MHC association with sporadic inclusion body myositis in Japanese patients. These findings indicate that different MHC ancestral haplotypes are associated with sIBM in different ethnic groups and further emphasize the importance of genetic factors in this condition. PMID:16564169

  18. MR imaging findings of focal myositis: a pseudotumour that may mimic muscle neoplasm

    Gaeta, Michele; Mazziotti, Silvio; Minutoli, Fabio; Genitori, Antonino; Blandino, Alfredo [University of Messina, A.O.U. ' ' Policlinico G. Martino' ' , Department of Radiological Sciences, Messina (Italy); Toscano, Antonio; Rodolico, Carmelo [University of Messina, A.O.U. ' ' Policlinico G. Martino' ' , Department of Neurosciences, Psychiatry and Anaesthesiology, Messina (Italy)

    2009-06-15

    The authors describe magnetic resonance (MR) findings in eight patients with histologically confirmed focal myositis. In each patient, axial TSE T1-weighted and fast short-tau inversion recovery (STIR) images were obtained using a 1.5-T MR scanner. Three patients also underwent dynamic contrast-enhanced MR examination using a GE T1-weighted sequence. The following features were evaluated: anatomical distribution, extent of the involvement, signal intensity characteristics, dynamic enhancement pattern and outcome at follow-up examinations. Seven of eight lesions were located in the lower extremities, one of eight in the arm; four of eight involved part of a muscle, two of eight diffusely involved a muscle and two of eight showed multifocal involvement of two or more muscles. All lesions were hyperintense on fast-STIR images: the hyperintensity was homogeneous in six of eight and inhomogeneous in two of eight. On T1-weighted unenhanced images, all lesions but two appeared isointense or slightly hypointense in comparison to normal muscles; two lesions showed a slight hyperintensity. Dynamic enhancement pattern corresponded to the type usually seen in benign soft tissue lesions. All lesions disappeared. Focal myositis is an uncommon pseudotumour which should be considered in the differential diagnosis of muscular masses and myopathies. (orig.)

  19. A novel Sarcocystis-associated encephalitis and myositis in racing pigeons.

    Olias, P; Gruber, A D; Heydorn, A O; Kohls, A; Mehlhorn, H; Hafez, H M; Lierz, M

    2009-04-01

    Sarcosporidian cysts in the skeletal muscle of domestic pigeons (Columba livia f. domestica) have previously been attributed to infection with Sarcocystis falcatula, which is shed in the faeces of the opossum (Didelphis virginiana). Here, we describe fatal spontaneous encephalitis and myositis associated with Sarcocystis infections in three flocks of racing pigeons with 47 of 244 animals affected. The clinical course was characterized by depression, mild diarrhoea, torticollis, opisthotonus, paralysis and trembling. Histopathological examination of 13 pigeons revealed generalized severe granulomatous and necrotizing meningoencephalitis and myositis with sarcosporidian cysts. Light and transmission electron microscopy identified cysts in heart and skeletal muscle of 1 to 2 mm in length and 20 to 50 microm in width. These were subdivided into small chambers by fine septae and filled with lancet-shaped cystozoites (7.5 x 1.5 microm) and dividing metrocytes, which is characteristic for Sarcocystis. The cysts had smooth walls and were devoid of protrusions typical of S. falcatula. Polymerase chain reaction amplification and sequencing of the internal transcribed spacer region (ITS-1) and the complete 28S rRNA identified a novel Sarcocystis species with only 51% ITS-1 nucleotide sequence similarity with S. falcatula. A phylogenetic comparison of the 28S rRNA revealed close sequence homologies with Frenkelia microti, Frenkelia glareoli and Sarcocystis neurona. The clinical, histopathological, electron microscopic and genetic data are unlike any previously described protozoan infections in pigeons, suggesting a novel, severe disease due to an as yet undescribed Sarcocystis species. PMID:19322710

  20. [Case of systemic myositis and subacute sensory neuropathy concomitant with signet-ring cell carcinoma].

    Yasuda, Chiharu; Yakushiji, Yusuke; Tokunaga, Osamu; Hara, Hideo; Nishino, Ichizo

    2010-04-01

    A 72-year-old woman referred to our hospital because of slowly progressive (over 2 years) muscle weakness and paresthesias of the lower limbs. On neurological examination, weakness and muscle atrophies were noted in the distal upper limbs as well as the proximal lower limbs. She had also paresthesias of the legs. The level of creatinine phosphokinase (CK) was 126 IU/l. The magnetic resonance imaging demonstrated gadolinium enhancement of the nerve roots at the L4-S2 vertebrate levels. Nerve conduction study showed decreased compound muscle action potential and motor conduction velocity of tibial and peroneal nerves. Biopsy of the left biceps brachii muscle showed variations in fiber size, endomysial mononuclear cell infiltration and the findings like a rimmed vacuole. Although almost of her findings were in accord with clinical features of inclusion body myositis, strong inflammatory cellular influences allowed us to administer corticosteroid therapy. Because her weakness was well responded to steroid therapy, polymyositis was considered as differential diagnosis. Then, further examinations were investigated to search any occult neoplasm, and detected the early gastric cancer. Total gastrectomy was performed later, and the pathological diagnosis was made as a signet-ring cell carcinoma. To our knowledge, this is the first report of systemic myositis and subacute sensory neuropathy concomitant with signet-ring cell carcinoma. These symptoms might be occurred as a result of paraneoplastic syndrome associated with satellite effects of the signet-ring cell carcinoma. PMID:20411807

  1. Clinical characteristics of patients with myositis and autoantibodies to different fragments of the Mi-2 beta antigen.

    Hengstman, G.J.D.; Vree Egberts, W.T.M.; Seelig, H.P.; Lundberg, I.E.; Moutsopoulos, H.M.; Doria, A.; Mosca, M.; Vencovsky, J.; Venrooij, W.J.W. van; Engelen, B.G.M. van

    2006-01-01

    OBJECTIVES: To assess the clinical implications of autoantibodies directed against different parts of the Mi-2 beta autoantigen in patients with myositis. METHODS: A systematic assessment of the clinical, laboratory, and histological characteristics of 48 anti-Mi-2 positive patients from six Europea

  2. Professor Wang Xiafang's experience in treating acute attack of childhood asthma by "Xuanfei Tongluo Pingchuan Decoction"%王霞芳运用宣肺通络平喘汤治疗发作期小儿哮喘经验

    李华

    2011-01-01

    介绍王霞芳教授运用宣肺通络平喘汤治疗发作期小儿哮喘的经验.认为小儿哮喘发作期以风邪外袭、痰饮内伏为主要病因病机,治疗宜宣肺化痰、止咳平喘、兼祛风通络,以自拟宣肺通络平喘汤治疗本病,疗效显著.%This paper introduces Professor Wang Xiafang' s clinical experience in the treatment of acute attack of childhood asthma by “Xuanfei Tongluo Pingchuan Decoction”.It is held that acute attack of childhood asthma results from invasion of exogenous wind and accumulation of endogenous phlegm-fluid; its treatment aims to release lung and resolve phlegm, ease cough and suppress asthma, and dispel wind and unblock collateral; “Xuanfei Tongluo Pingchuan Decoction” was composed in clinical practice to treat it, and excellent results have been achieved.

  3. Childhood Leukemia

    ... cells. It is the most common type of childhood cancer. Your blood cells form in your bone ... in the bones or joints Risk factors for childhood leukemia include having a brother or sister with ...

  4. Childhood Stress

    ... 5 Things to Know About Zika & Pregnancy Childhood Stress KidsHealth > For Parents > Childhood Stress Print A A ... and feel stress to some degree. Sources of Stress Stress is a function of the demands placed ...

  5. Childhood Obesity

    Wilkinson, Justine; Howard, Simon

    2014-01-01

    Childhood obesity has important consequences for health and wellbeing both during childhood and also in later adult life. The rising prevalence of childhood obesity poses a major public health challenge in both developed and developing countries by increasing the burden of chronic non-communicable diseases. Despite the urgent need for effective preventative strategies, there remains disagreement over its definition due to a lack of evidence on the optimal cut-offs linking childhood BMI to dis...

  6. Birthweight and Childhood Cancer

    Paltiel, Ora; Tikellis, Gabriella; Linet, Martha; Golding, Jean; Lemeshow, Stanley; Phillips, Gary; Lamb, Karen; Stoltenberg, Camilla; Håberg, Siri E; Strøm, Marin; Granstrøm, Charlotta; Northstone, Kate; Klebanoff, Mark; Ponsonby, Anne-Louise; Milne, Elizabeth; Pedersen, Marie; Kogevinas, Manolis; Ha, Eunhee; Dwyer, Terence

    2015-01-01

    BACKGROUND: Evidence relating childhood cancer to high birthweight is derived primarily from registry and case-control studies. We aimed to investigate this association, exploring the potential modifying roles of age at diagnosis and maternal anthropometrics, using prospectively collected data from...... the International Childhood Cancer Cohort Consortium. METHODS: We pooled data on infant and parental characteristics and cancer incidence from six geographically and temporally diverse member cohorts [the Avon Longitudinal Study of Parents and Children (UK), the Collaborative Perinatal Project (USA......). Childhood cancer (377 cases diagnosed prior to age 15 years) risk was analysed by type (all sites, leukaemia, acute lymphoblastic leukaemia, and non-leukaemia) and age at diagnosis. We estimated hazard ratios (HR) and 95% confidence intervals (CI) from Cox proportional hazards models stratified by cohort...

  7. Childhood Cancer

    ... Story" 5 Things to Know About Zika & Pregnancy Childhood Cancer KidsHealth > For Parents > Childhood Cancer Print A A A Text Size What's ... in children, but can happen. The most common childhood cancers are leukemia , lymphoma , and brain cancer . As ...

  8. Fludarabine Phosphate and Total-Body Irradiation Followed by Donor Peripheral Blood Stem Cell Transplant in Treating Patients With Acute Lymphoblastic Leukemia or Chronic Myelogenous Leukemia That Has Responded to Treatment With Imatinib Mesylate, Dasatinib, or Nilotinib

    2015-07-20

    Adult Acute Lymphoblastic Leukemia in Remission; Blastic Phase Chronic Myelogenous Leukemia; Childhood Acute Lymphoblastic Leukemia in Remission; Chronic Myelogenous Leukemia, BCR-ABL1 Positive; Chronic Phase Chronic Myelogenous Leukemia; Philadelphia Chromosome Positive Adult Precursor Acute Lymphoblastic Leukemia; Philadelphia Chromosome Positive Childhood Precursor Acute Lymphoblastic Leukemia; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Childhood Acute Lymphoblastic Leukemia; Relapsing Chronic Myelogenous Leukemia

  9. Epidemiology of childhood cancer

    Terracini Benedetto

    2011-01-01

    Abstract At least in economically developed countries, in the last decades, the incidence of childhood cancer has increased and the increase is unlikely to be an artefact. Causes of the increase have not been identified: a role of preventable environmental exposures is possible. Changes have also occurred in the age distribution of acute lymphoblastic leukaemia. Currently, children with cancer can be successfully treated and cured. However, access to the best therapy differs widely among coun...

  10. A Rare Case of Mixed Connective Tissue Disease (MCTD) with Intricate Features of Lupus, Polymyositis and Rheumatoid Arthritis Presenting with Severe Myositis

    S, Lokesh; Tony, Kadavanu; Raghupathy,; V, Suresh; Malepati, Balakrishna

    2015-01-01

    Mixed connective tissue disease (MCTD) includes clinical and laboratorial manifestations of systemic lupus erythematosus, scleroderma and polymyositis along with high titres of anti-U1RNP antibodies. In the initial phases of the disease, muscle enzyme levels increase but the disease remains generally subclinical. Presentation with myositis is uncommon. Our objective is to report a rare case of a patient who presented with a severe onset of myositis characterized by dysphagia, an increase in m...

  11. Genome-wide Association Study Identifies HLA 8.1 Ancestral Haplotype Alleles as Major Genetic Risk Factors for Myositis Phenotypes

    Miller, Frederick W.; Chen, Wei; O’Hanlon, Terrance P.; Cooper, Robert G.; Vencovsky, Jiri; Rider, Lisa G; Danko, Katalin; Wedderburn, Lucy R; Lundberg, Ingrid E; Pachman, Lauren M.; Reed, Ann M.; Ytterberg, Steven R.; Padyukov, Leonid; Selva-O’Callaghan, Albert; Radstake, Timothy R.

    2015-01-01

    Autoimmune muscle diseases (myositis) comprise a group of complex phenotypes influenced by genetic and environmental factors. To identify genetic risk factors in patients of European ancestry, we conducted a genome-wide association study (GWAS) of the major myositis phenotypes in a total of 1710 cases, which included 705 adult dermatomyositis; 473 juvenile dermatomyositis; 532 polymyositis; and 202 adult dermatomyositis, juvenile dermatomyositis or polymyositis patients with anti-histidyl tRN...

  12. Acute Otitis Media

    A Joshan

    2000-07-01

    Full Text Available Acute Otitis Media is a common problem of childhood. It is characterized by otalgia, fever, irritability, anorexia and vomiting or diarrhea. Diagnosis is most often made by pneumatic otoscopy. Acute Otitis Media is mostly caused by S.pneumonia or H.influenza there are sensitive to penicillins.

  13. Intercostal myositis ossificans misdiagnosed as osteosarcoma in a 10-year-old child

    Koob, Meriam; Durckel, Jean; Dosch, Jean-Claude; Dietemann, Jean-Louis [Hopital de Hautepierre, Service de Radiologie II, Hopitaux Universitaires, Strasbourg Cedex (France); Entz-Werle, Natacha [Hopitaux Universitaires, Hopital de Hautepierre, Service d' Onco-hematologie pediatrique, Strasbourg Cedex (France)

    2010-12-15

    Myositis ossificans (MO) is a rare benign cause of heterotopic bone formation within soft tissue. It most commonly affects adolescents and young adults, typically in the limbs and following trauma. Very few cases have been reported in children. We report here a case of nontraumatic MO occurring in a 10-year-old girl with an uncommon location in the 5th right intercostal space; it was initially misdiagnosed and treated as osteosarcoma. Imaging findings including plain radiographs, CT, MRI, bone scintigraphy and PET-CT are described. This case highlights the central role played by imaging in diagnosis, thus avoiding biopsy that can erroneously suggest osteosarcoma as the diagnosis, as occurred in this case. (orig.)

  14. Myositis ossificans traumatica in young children: report of three cases and review of the literature

    Myositis ossificans traumatica (MOT) is a rare musculoskeletal disorder in young children. Clinical and imaging presentation in the early stage of disease makes it difficult to differentiate between infection and musculoskeletal neoplasms, particularly in the absence of a history of trauma. Three cases of MOT in children under the age of 10 years, two with inferential trauma, are presented and the findings on different imaging modalities are discussed with reference to the existing literature. While findings based on a single imaging technique, including MRI, may be rather non-specific and even misleading, the combination of different modalities can assist in the consideration of MOT as a possible diagnosis. For example, the demonstration of soft-tissue haematoma on US would suggest the traumatic origin. A rational imaging approach is proposed. (orig.)

  15. Asymptomatic hyper-creatine-kinase-emia as sole manifestation of inclusion body myositis

    Josef Finsterer

    2013-06-01

    Full Text Available Sporadic inclusion body myositis (sIBM usually manifests with painless weakness of the hand, finger and hip flexors. Absence of symptoms or signs, but mild hyper-CK-emia as the sole manifestation of IBM, has not been reported. We report the case of a 73-year-old male who presented with asymptomatic recurrent hyper-CK-emia ranging from 200 to 1324 U/L (n<171 U/L, since 10 years. Clinical neurologic investigation, nerve conduction studies and EMG were non-informative. Muscle biopsy surprisingly revealed sIBM. sIBM may be asymptomatic and may manifest with hyper-CK-emia exclusively. So, it has to be included in the differential diagnoses of asymptomatic hyper-CK-emia.

  16. Management of necrotizing myositis in a field hospital: a case report

    Bharathi Ramanathan

    2009-04-01

    Full Text Available Abstract Necrotizing myositis is a rare and fatal disease of skeletal muscles caused by group A beta hemolytic streptococci (GABHS. Its early detection by advanced imaging forms the basis of current management strategy. Paucity of advanced imaging in field/rural hospitals necessitates adoption of management strategy excluding imaging as its basis. Such a protocol, based on our experience and literature, constitutes: i. Prompt recognition of the clinical triad: disproportionate pain; precipitous course; and early loss of power- in a swollen limb with/without preceding trauma. ii. Support of clinical suspicion by 2 ubiquitous laboratory tests: gram staining- of exudates from bullae/muscles to indicate GABHS infection; and CPK estimation- to indicate myonecrosis. iii. Replacement of empirical antibiotics with high intravenous doses of sodium penicillin and clindamycin iv. Exploratory fasciotomy: to confirm myonecrosis without suppuration- its hallmark v. Emergent radical debridement vi. Primary closure with viable flaps – unconventional, if need be.

  17. Intercostal myositis ossificans misdiagnosed as osteosarcoma in a 10-year-old child

    Myositis ossificans (MO) is a rare benign cause of heterotopic bone formation within soft tissue. It most commonly affects adolescents and young adults, typically in the limbs and following trauma. Very few cases have been reported in children. We report here a case of nontraumatic MO occurring in a 10-year-old girl with an uncommon location in the 5th right intercostal space; it was initially misdiagnosed and treated as osteosarcoma. Imaging findings including plain radiographs, CT, MRI, bone scintigraphy and PET-CT are described. This case highlights the central role played by imaging in diagnosis, thus avoiding biopsy that can erroneously suggest osteosarcoma as the diagnosis, as occurred in this case. (orig.)

  18. General Information about Adult Acute Myeloid Leukemia

    ... Treatment Childhood AML Treatment Research Adult Acute Myeloid Leukemia Treatment (PDQ®)–Patient Version General Information About Adult Acute Myeloid Leukemia Go to Health Professional Version Key Points Adult ...

  19. Treatment Option Overview (Adult Acute Lymphoblastic Leukemia)

    ... Treatment Childhood AML Treatment Research Adult Acute Lymphoblastic Leukemia Treatment (PDQ®)–Patient Version General Information About Adult Acute Lymphoblastic Leukemia Go to Health Professional Version Key Points Adult ...

  20. Treatment Option Overview (Adult Acute Myeloid Leukemia)

    ... Treatment Childhood AML Treatment Research Adult Acute Myeloid Leukemia Treatment (PDQ®)–Patient Version General Information About Adult Acute Myeloid Leukemia Go to Health Professional Version Key Points Adult ...

  1. General Information about Adult Acute Lymphoblastic Leukemia

    ... Treatment Childhood AML Treatment Research Adult Acute Lymphoblastic Leukemia Treatment (PDQ®)–Patient Version General Information About Adult Acute Lymphoblastic Leukemia Go to Health Professional Version Key Points Adult ...

  2. Stages of Adult Acute Myeloid Leukemia

    ... Treatment Childhood AML Treatment Research Adult Acute Myeloid Leukemia Treatment (PDQ®)–Patient Version General Information About Adult Acute Myeloid Leukemia Go to Health Professional Version Key Points Adult ...

  3. Stages of Adult Acute Lymphoblastic Leukemia

    ... Treatment Childhood AML Treatment Research Adult Acute Lymphoblastic Leukemia Treatment (PDQ®)–Patient Version General Information About Adult Acute Lymphoblastic Leukemia Go to Health Professional Version Key Points Adult ...

  4. Treatment Options for Adult Acute Myeloid Leukemia

    ... Treatment Childhood AML Treatment Research Adult Acute Myeloid Leukemia Treatment (PDQ®)–Patient Version General Information About Adult Acute Myeloid Leukemia Go to Health Professional Version Key Points Adult ...

  5. Treatment Options for Adult Acute Lymphoblastic Leukemia

    ... Treatment Childhood AML Treatment Research Adult Acute Lymphoblastic Leukemia Treatment (PDQ®)–Patient Version General Information About Adult Acute Lymphoblastic Leukemia Go to Health Professional Version Key Points Adult ...

  6. 儿童高危急性淋巴细胞白血病治疗策略%Therapeutic strategies for childhood high-risk acute lymphoblastic leukemia

    卢新夭

    2013-01-01

    Contemporary treatments have resulted in 5-year event-free survival rates (EFS) of approximately 75% to 80% for childhood acute lymphoblastic leukemia (ALL). Relapses of ALL in children were more often in HR-ALL but also in very few non-HR-ALL. Thus current clinical study of ALL has focused on improving the outcome of a few subtypes of HR-ALL. Infants with ALL have a particularly high risk of treatment failure. Infant ALL Interfant-99 study found that MLL rearrangement, age younger than 6 months, poor response to a prednisone prophase and high WBC count were strong independent predictive factors for poor prognosis in infants with ALL. Treatments with hybrid protocol, including both lymphoid- and myeloid-directed treatment elements, also contain HD-MTX and high dose Ara-C ( HD-Ara-C) , will further improve the outcome for infant ALL. Children Philadelphia chromosome positive ALL ( Ph + ALL) was associated with a high relapse rate when treated with chemotherapy alone. The Children' s Oncology Group (COG) AALL0031 trial showed that the addition of tyrosine kinase inhibitors (TKIs) imatinib to intensive chemotherapy resulted in 3-year EFS more than historical control treated with chemotherapy alone. These findings create a new paradigm for integrating molecularly targeted agents with intensified chemotherapy. Children with T-ALL have had a worse outcome than with the precursor B-cell ALL previously. With more intensified chemotherapy , outcomes for children T-ALL were improved, approaching those for the precursor B-cell ALL. Recently, COG decided to treat children with T-cell ALL with separate protocols different from those for the precursor B-cell ALL, and the protocols of BFM for children with T-ALL have been the same as those of the precursor B-cell ALL. Early precursor T-cell ALL, a novel subtype of T-cell ALL, was identified by gene expression profiling, flow cytometry, and single nucleotide polymorphism array analyses. ETP-ALL, identified in 13% of T-cell ALL

  7. Acute neuromuscular weakness associated with dengue infection

    Harmanjit Singh Hira

    2012-01-01

    Full Text Available Background: Dengue infections may present with neurological complications. Whether these are due to neuromuscular disease or electrolyte imbalance is unclear. Materials and Methods: Eighty-eight patients of dengue fever required hospitalization during epidemic in year 2010. Twelve of them presented with acute neuromuscular weakness. We enrolled them for study. Diagnosis of dengue infection based on clinical profile of patients, positive serum IgM ELISA, NS1 antigen, and sero-typing. Complete hemogram, kidney and liver functions, serum electrolytes, and creatine phosphokinase (CPK were tested. In addition, two patients underwent nerve conduction velocity (NCV test and electromyography. Results: Twelve patients were included in the present study. Their age was between 18 and 34 years. Fever, myalgia, and motor weakness of limbs were most common presenting symptoms. Motor weakness developed on 2 nd to 4 th day of illness in 11 of 12 patients. In one patient, it developed on 10 th day of illness. Ten of 12 showed hypokalemia. One was of Guillain-Barré syndrome and other suffered from myositis; they underwent NCV and electromyography. Serum CPK and SGOT raised in 8 out of 12 patients. CPK of patient of myositis was 5098 IU. All of 12 patients had thrombocytopenia. WBC was in normal range. Dengue virus was isolated in three patients, and it was of serotype 1. CSF was normal in all. Within 24 hours, those with hypokalemia recovered by potassium correction. Conclusions: It was concluded that the dengue virus infection led to acute neuromuscular weakness because of hypokalemia, myositis, and Guillain-Barré syndrome. It was suggested to look for presence of hypokalemia in such patients.

  8. Individualized toxicity-titrated 6-mercaptopurine increments during high-dose methotrexate consolidation treatment of lower risk childhood acute lymphoblastic leukemia

    Frandsen, Thomas Leth; Abrahamsson, Jonas; Lausen, Birgitte Frederiksen;

    2011-01-01

    This study explored the feasibility and toxicity of individualized toxicity-titrated 6-mercaptopurine (6MP) dose increments during post-remission treatment with High-dose methotrexate (HDM) (5000 mg/m2, ×3) in 38 patients with Childhood (ALL). Patients were increased in steps of 25 mg 6MP/m2 per ...

  9. Prevalence of Gene Rearrangements in Mexican Children with Acute Lymphoblastic Leukemia: A Population Study—Report from the Mexican Interinstitutional Group for the Identification of the Causes of Childhood Leukemia

    Vilma Carolina Bekker-Méndez

    2014-01-01

    Full Text Available Mexico has one of the highest incidences of childhood leukemia worldwide and significantly higher mortality rates for this disease compared with other countries. One possible cause is the high prevalence of gene rearrangements associated with the etiology or with a poor prognosis of childhood acute lymphoblastic leukemia (ALL. The aims of this multicenter study were to determine the prevalence of the four most common gene rearrangements [ETV6-RUNX1, TCF3-PBX1, BCR-ABL1, and MLL rearrangements] and to explore their relationship with mortality rates during the first year of treatment in ALL children from Mexico City. Patients were recruited from eight public hospitals during 2010–2012. A total of 282 bone marrow samples were obtained at each child’s diagnosis for screening by conventional and multiplex reverse transcription polymerase chain reaction to determine the gene rearrangements. Gene rearrangements were detected in 50 (17.7% patients. ETV6-RUNX1 was detected in 21 (7.4% patients, TCF3-PBX1 in 20 (7.1% patients, BCR-ABL1 in 5 (1.8% patients, and MLL rearrangements in 4 (1.4% patients. The earliest deaths occurred at months 1, 2, and 3 after diagnosis in patients with MLL, ETV6-RUNX1, and BCR-ABL1 gene rearrangements, respectively. Gene rearrangements could be related to the aggressiveness of leukemia observed in Mexican children.

  10. 门冬酰胺酶致急淋白血病患儿两次脑血栓形成1例%Asparaginase Induced Cerebral Thrombosis For Twice In One Childhood Acute Lymphoblastic Leukemia Case

    王成军; 汪俭; 李艳; 许喆; 陈天平

    2015-01-01

    Asparaginase depletion can specific affect the synthesis of asparagine protein in tumor cell, it is one of the core drugs for treating childhood acute lymphoblastic leukemia, it can improve the cure rate. Effect of asparaginase on coagulation is great influence, and a two-way risk of both thrombosis and bleeding exist. We report that asparaginase induced cerebral thrombosis for twice in one childhood ALL patient and our clinical treatment course, which should provide reference for clinical treatment in these patients treated with asparaginase for future.%门冬酰胺酶能特异性消耗门冬酰胺影响肿瘤细胞蛋白质的合成,是儿童急性淋巴细胞白血病治疗的核心药物之一,对提高儿童急淋治愈率的贡献很大.门冬酰胺酶对机体凝血功能的影响也很大,同时有血栓形成及出血的双向风险.该文报道了1例门冬酰胺酶致急性淋巴细胞白血病患儿两次脑血栓形成及临床干预经过,为以后此类患儿的临床治疗提供参考.

  11. Prevalence of Gene Rearrangements in Mexican Children with Acute Lymphoblastic Leukemia: A Population Study—Report from the Mexican Interinstitutional Group for the Identification of the Causes of Childhood Leukemia

    Bekker-Méndez, Vilma Carolina; Miranda-Peralta, Enrique; Núñez-Enríquez, Juan Carlos; Olarte-Carrillo, Irma; Guerra-Castillo, Francisco Xavier; Pompa-Mera, Ericka Nelly; Ocaña-Mondragón, Alicia; Bernáldez-Ríos, Roberto; Medina-Sanson, Aurora; Jiménez-Hernández, Elva; Amador-Sánchez, Raquel; Peñaloza-González, José Gabriel; de Diego Flores-Chapa, José; Fajardo-Gutiérrez, Arturo; Flores-Lujano, Janet; Rodríguez-Zepeda, María del Carmen; Dorantes-Acosta, Elisa María; Bolea-Murga, Victoria; Núñez-Villegas, Nancy; Velázquez-Aviña, Martha Margarita; Torres-Nava, José Refugio; Reyes-Zepeda, Nancy Carolina; González-Bonilla, Cesar; Mejía-Aranguré, Juan Manuel

    2014-01-01

    Mexico has one of the highest incidences of childhood leukemia worldwide and significantly higher mortality rates for this disease compared with other countries. One possible cause is the high prevalence of gene rearrangements associated with the etiology or with a poor prognosis of childhood acute lymphoblastic leukemia (ALL). The aims of this multicenter study were to determine the prevalence of the four most common gene rearrangements [ETV6-RUNX1, TCF3-PBX1, BCR-ABL1, and MLL rearrangements] and to explore their relationship with mortality rates during the first year of treatment in ALL children from Mexico City. Patients were recruited from eight public hospitals during 2010–2012. A total of 282 bone marrow samples were obtained at each child's diagnosis for screening by conventional and multiplex reverse transcription polymerase chain reaction to determine the gene rearrangements. Gene rearrangements were detected in 50 (17.7%) patients. ETV6-RUNX1 was detected in 21 (7.4%) patients, TCF3-PBX1 in 20 (7.1%) patients, BCR-ABL1 in 5 (1.8%) patients, and MLL rearrangements in 4 (1.4%) patients. The earliest deaths occurred at months 1, 2, and 3 after diagnosis in patients with MLL, ETV6-RUNX1, and BCR-ABL1 gene rearrangements, respectively. Gene rearrangements could be related to the aggressiveness of leukemia observed in Mexican children. PMID:25692130

  12. Acute myelogenous leukemia (AML) -- children

    ... Leung WH, Pounds S, Cao X, e t al. Definition of cure in childhood acute myeloid leukemia. Cancer . ... M. is also a founding member of Hi-Ethics and subscribes to the principles of the Health ...

  13. Childhood Obesity

    Aydın, Ahmet; Koca, Fahrettin; Fıçıcıoğlu, Can; Çam, Halit; Mıkla, Şerare

    1995-01-01

    Management of childhood obesity and its early and late complications are among the most difficult problems confronted by pediatricians and practitioners The purpose of this review is to provide information for the evaluation and treatment of childhood obesity Key nbsp;words: nbsp;Child Obesity Etiology Management Complications

  14. Intrachromosomal amplification of chromosome 21 (iAMP21) detected by ETV6/RUNX1 FISH screening in childhood acute lymphoblastic leukemia: a case report

    Daniela Ribeiro Ney Garcia; Alejandro Mauricio Arancibia; Ribeiro, Raul C.; Marcelo Gerardin Poirot Land; Maria Luiza Macedo Silva

    2013-01-01

    Chromosome abnormalities that usually define high-risk acute lymphoblastic leukemia are the t(9;22)/ breakpoint cluster region protein-Abelson murine leukemia viral oncogene homolog 1, hypodiploid with < 44 chromosomes and 11q23/ myeloid/lymphoid leukemia gene rearrangements. The spectrum of acute lymphoblastic leukemia genetic abnormalities is nevertheless rapidly expanding. Therefore, newly described chromosomal aberrations are likely to have an impact on clinical care in the near future. R...

  15. A Rare Case of Mixed Connective Tissue Disease (MCTD) with Intricate Features of Lupus, Polymyositis and Rheumatoid Arthritis Presenting with Severe Myositis.

    S, Lokesh; Tony, Kadavanu; Raghupathy; V, Suresh; Malepati, Balakrishna

    2015-03-01

    Mixed connective tissue disease (MCTD) includes clinical and laboratorial manifestations of systemic lupus erythematosus, scleroderma and polymyositis along with high titres of anti-U1RNP antibodies. In the initial phases of the disease, muscle enzyme levels increase but the disease remains generally subclinical. Presentation with myositis is uncommon. Our objective is to report a rare case of a patient who presented with a severe onset of myositis characterized by dysphagia, an increase in myopathy and joint involvement suggestive of RA. The patient was initiated on pulse corticosteroid therapy along with methotrexate in view of her elevated Creatine Kinase levels and biopsy findings that were suggestive of severe myositis. The patient showed clinical and laboratory improvement with this regimen. Though severe myositis and arthritis can occur in overlap syndrome, MCTD evolved as a separate disease entity due to presence of high titres of Anti U1-RNP antibodies. The authors emphasize that this is an extremely rare presentation of MCTD with only two previous cases seen in literature, one of a 13 year old child and the other being an adult female both of whom had evidence of myositis on presentation. PMID:25954655

  16. Childhood pneumonia and vitamin A

    Farhad Heidarian

    2014-04-01

    Full Text Available One of the major causes of mortality in children younger than 5 years old is acute lower respiratory tract infections (ALRI. ALRI clinical features are cough, tachypnea, fever, coryza, chest retraction, crackles and wheeze. Increased white blood cell count with left shift might happen in pneumonia. C-reactive protein (CRP and erythrocyte sedimentation rate (ESR might rise in children with respiratory tract infections. Vitamin A deficiency is associated with severe childhood infections. The effect of vitamin A supplementation in childhood pneumonia depends on the prevalence and the level of vitamin A deficiency in the population. Some studies confirmed that retinol levels were significantly higher after recovery from acute pneumonia compared to acute phase. But there were no significant association between serum retinol level and the clinical manifestation.

  17. Intramuscular inoculation of cattle with Sarcocystis antigen results in focal eosinophilic myositis.

    Vangeel, L; Houf, K; Geldhof, P; Nollet, H; Vercruysse, J; Ducatelle, R; Chiers, K

    2012-02-10

    Bovine eosinophilic myositis (BEM) is a subclinical myopathy characterized by multifocal white to grey-green discolorations in skeletal muscles, heart, tongue and oesophagus. These lesions are found at slaughter or during meat cutting and result in considerable economic losses. The etiology and pathogenesis are unclear, although it has been suggested, that Sarcocystis species are involved. To elucidate their role, two calves were repeatedly injected intramuscularly with adjuvanted Sarcocystis antigen. The morphological changes at the injection sites in these calves were histologically and immunohistochemically compared to spontaneous lesions from 44 BEM condemned carcasses sampled in slaughterhouses. Experimental intramuscular injection of Sarcocystis antigen resulted in lesions at the injection sites that were similar to the lesions of natural cases of BEM. They were characterized by massive infiltration of eosinophilic granulocytes, reactive macrophages (MAC387(+) cells), T-cells (CD3(+)) and B-cells (CD20(+)). Both in the experimental and in the natural cases, COX-2 expression was present in endothelial cells adjacent to lesional areas. MHC class II(+) staining was found amongst others in muscle cells surrounding the lesion. These results show that Sarcocystis antigens can induce an inflammatory response in bovine muscle having the characteristics of natural BEM. PMID:21852041

  18. Complex mitochondrial DNA rearrangements in individual cells from patients with sporadic inclusion body myositis

    Rygiel, Karolina A.; Tuppen, Helen A.; Grady, John P.; Vincent, Amy; Blakely, Emma L.; Reeve, Amy K.; Taylor, Robert W.; Picard, Martin; Miller, James; Turnbull, Doug M.

    2016-01-01

    Mitochondrial DNA (mtDNA) rearrangements are an important cause of mitochondrial disease and age related mitochondrial dysfunction in tissues including brain and skeletal muscle. It is known that different mtDNA deletions accumulate in single cells, but the detailed nature of these rearrangements is still unknown. To evaluate this we used a complementary set of sensitive assays to explore the mtDNA rearrangements in individual cells from patients with sporadic inclusion body myositis, a late-onset inflammatory myopathy with prominent mitochondrial changes. We identified large-scale mtDNA deletions in individual muscle fibres with 20% of cytochrome c oxidase-deficient myofibres accumulating two or more mtDNA deletions. The majority of deletions removed only the major arc but ∼10% of all deletions extended into the minor arc removing the origin of light strand replication (OL) and a variable number of genes. Some mtDNA molecules contained two deletion sites. Additionally, we found evidence of mitochondrial genome duplications allowing replication and clonal expansion of these complex rearranged molecules. The extended spectrum of mtDNA rearrangements in single cells provides insight into the process of clonal expansion which is fundamental to our understanding of the role of mtDNA mutations in ageing and disease. PMID:27131788

  19. Demographic and clinical features of inclusion body myositis in North America

    Paltiel, A. David; Ingvarsson, Einar; Lee, Donald K. K.; Leff, Richard L.; Nowak, Richard J.; Petschke, Kurt D.; Richards-Shubik, Seth; Zhou, Ange; Shubik, Martin; O’Connor, Kevin C.

    2016-01-01

    Objective Define the demographics, natural history, and clinical management of patients with inclusion body myositis (IBM). Background Few studies of the demographics, natural history, and clinical management of IBM have been performed in a large patient population. Methods A cross-sectional, self-reporting survey was conducted. Results The mean age of the 916 participants was 70.4 years, the male-to-female ratio was 2:1, and the majority reported difficulty with ambulation and activities of daily living. The earliest symptoms included impaired use and weakness of arms and legs. The mean time from first symptoms to diagnosis was 4.7 years. Half reported that IBM was their initial diagnosis. A composite functional index negatively associated with age, disease duration, and positively associated with participation in exercise. Conclusion These data are valuable for informing patients how IBM manifestations are expected to impair daily living and indicate that self-reporting could be used to establish outcome measures in clinical trials. PMID:25557419

  20. IgD Multiple Myeloma Paraproteinemia as a Cause of Myositis

    I. Colombo

    2010-01-01

    Full Text Available A 48-years old man was diagnosed an IgD-k multiple myeloma (MM at age 38 years for which he successfully underwent chemotherapy and bone marrow transplant. He then developed a graft-versus-host disease (GVHD whose manifestations included, three years later, a polymyositis, diagnosed at muscle biopsy and successfully treated with steroids. Few months after polymyositis remission, myeloma relapsed and the patient was treated with thalidomide for six years with good remission. Soon after thalidomide suspension, MM relapsed again and the patient came to our observation for a new onset of neuromuscular symptoms. He underwent both muscle and peripheral nerve biopsy to discriminate between myositis (paraproteinemia versus GVHD, amyloidosis, and thalidomide toxicity. The first muscle biopsy showed an inflammatory pattern with necrotic fibres, macrophagical invasion (CD68 positive, rare interstitial cellular infiltrates (CD8 positive and CD4 negative, widespread anti-HLA positivity and negative antiMAC. The second muscle biopsy showed the same inflammatory pattern plus an involvement of blood vessels. Direct immunofluorescence for IgD showed diffuse positivity along the sarcolemmal in both muscle biopsies. Sural nerve biopsy demonstrated both demyelinating and axonal aspects with no inflammatory infiltrates, but positivity for HLA and MAC. Congo Red was negative in both skeletal muscle and peripheral nerve.

  1. Childhood proptosis

    Proptosis in children is a hallmark of orbital diseases which can present a diagnostic challenge requiring thoughtful investigation. The aim of this review is to provide the reader an overview of the subject of childhood proptosis with an emphasis on the systematic and practical approach for the work-up of proptosis in children. Use of proper imaging studies is essential for the correct diagnosis. Computed tomography is a good screening test for any space occupying lesion of the orbit. Proptosis describes eye prominence due to space occupying orbital lesions. Congenital lesions usually present in the first decade of life. Acquired orbital lesions such as lymphangiomas, orbital varix, rhabdomyosarcoma and neural tumors may present at the end of the first decade of life. Metastatic tumors to the orbit, adenocarcinoma of lacrimal gland and rapidly growing masses may present with proptosis associated with pain. Visual loss can be the presenting symptoms in the patients with optic nerve (ON) gliomas, orbital meningiomas and posteriorly located tumors. Cystic lesions of the orbit may be congenital or acquired, dermoid cysts being the most common congenital orbital lesions. Some of the vascular lesions of the orbit include capillary hemangiomas, lymphangiomas, orbital varix, and arteriovenous malformations. Inflammatory process of the orbit in children include cellulitis and pseudotumor. Neural tumors such as neurofibromas, ON gilomas and meningiomas are less common causes of proptosis in children. Rhabdomyosarcoma is the most common primary orbital malignancy in children which can present with acute proptosis and is one of the few life-threatening diseases seen initially by an ophthalmologist. Secondary orbital tumors invade the orbit from adjacent sinuses, cranium or extended from the eye itself. The most common distant metastases in children include neuroblastoma and Ewing's sarcoma. Although many orbital processes can be diagnosed based on history, clinical

  2. Flow Cytometric DNA index, G-band Karyotyping, and Comparative Genomic Hybridization in Detection of High Hyperdiploidy in Childhood Acute Lymphoblastic Leukemia

    Nygaard, Ulrikka; Larsen, Jacob; Kristensen, Tim D; Wesenberg, Finn; Jonsson, Olafur G; Carlsen, Niels T; Forestier, Erik; Kirchhoff, Maria; Larsen, Jørgen K; Schmiegelow, Kjeld; Christensen, Ib Jarle

    2006-01-01

    High hyperdiploid acute lymphoblastic leukemia in children is related to a good outcome. Because these patients may be stratified to a low-intensity treatment, we have investigated the sensitivity of flow cytometry (FCM), G-band karyotyping (GBK), and high-resolution comparative genomic...... hybridization (HR-CGH) in detecting high hyperdiploid leukemic clones. Twenty-six girls and 34 boys with acute lymphoblastic leukemia diagnosed in 1998 to 1999 were analyzed by FCM, GBK, and HR-CGH. The correlations between DNA indices obtained by FCM, GBK, and HR-CGH were significant (rs=0.61 to 0.77; P<0...

  3. Flow Cytometric DNA index, G-band Karyotyping, and Comparative Genomic Hybridization in Detection of High Hyperdiploidy in Childhood Acute Lymphoblastic Leukemia

    Nygaard, Ulrikka; Larsen, Jacob; Kristensen, Tim D;

    2006-01-01

    High hyperdiploid acute lymphoblastic leukemia in children is related to a good outcome. Because these patients may be stratified to a low-intensity treatment, we have investigated the sensitivity of flow cytometry (FCM), G-band karyotyping (GBK), and high-resolution comparative genomic hybridiza......High hyperdiploid acute lymphoblastic leukemia in children is related to a good outcome. Because these patients may be stratified to a low-intensity treatment, we have investigated the sensitivity of flow cytometry (FCM), G-band karyotyping (GBK), and high-resolution comparative genomic...

  4. Childhood Leukemia

    Leukemia is cancer of the white blood cells. It is the most common type of childhood cancer. ... blood cells help your body fight infection. In leukemia, the bone marrow produces abnormal white blood cells. ...

  5. Childhood leukaemia

    The debate on whether there is any link between leukaemia clusters and nuclear installations has been raging since the early eighties. A Government Inquiry found no link between childhood leukaemia and residence near Seascale, an area near British Nuclear Fuels Sellafield plant. Research in the 1980s linked childhood leukaemia to fathers' occupations prior to conception in the Seascale plant but also to workers in the iron, steel, farming and chemical industries. This article reviews research findings to date. (UK)

  6. Outcome After First Relapse in Children With Acute Lymphoblastic Leukemia : A Report Based on the Dutch Childhood Oncology Group (DCOG) Relapse ALL 98 Protocol

    van den Berg, H.; de Groot-Kruseman, H. A.; Damen-Korbijn, C. M.; de Bont, E. S. J. M.; Schouten-van Meeteren, A. Y. N.; Hoogerbrugge, P. M.

    2011-01-01

    Background. We report on the treatment of children and adolescents with acute lymphoblastic leukemia (ALL) in first relapse. The protocol focused on: (1) Intensive chemotherapy preceding allogeneic stem cell transplantation (SCT) in early bone marrow relapse; (2) Rotational chemotherapy in late rela

  7. Outcome after first relapse in children with acute lymphoblastic leukemia: a report based on the Dutch Childhood Oncology Group (DCOG) relapse all 98 protocol

    Berg, H. van den; Groot-Kruseman, H.A. de; Damen-Korbijn, C.M.; Bont, E.S. de; Schouten-van Meeteren, A.Y.; Hoogerbrugge, P.M.

    2011-01-01

    BACKGROUND: We report on the treatment of children and adolescents with acute lymphoblastic leukemia (ALL) in first relapse. The protocol focused on: (1) Intensive chemotherapy preceding allogeneic stem cell transplantation (SCT) in early bone marrow relapse; (2) Rotational chemotherapy in late rela

  8. Outcome of ETV6/RUNX1-positive childhood acute lymphoblastic leukaemia in the NOPHO-ALL-1992 protocol: frequent late relapses but good overall survival

    Forestier, Erik; Heyman, Mats; Andersen, Mette K; Autio, Kirsi; Blennow, Elisabeth; Borgström, Georg; Golovleva, Irina; Heim, Sverre; Heinonen, Kristina; Hovland, Randi; Johannsson, Johann H; Kerndrup, Gitte; Nordgren, Ann; Rosenquist, Richard; Swolin, Birgitta; Johansson, Bertil

    2008-01-01

    The prognostic impact of t(12;21)(p13;q22) [ETV6/RUNX1 fusion] in paediatric acute lymphoblastic leukaemia (ALL) has been extensively debated, particularly with regard to the frequency of late relapses and appropriate treatment regimens. We have retrospectively collected 679 ALLs with known ETV6/...

  9. In vitro drug resistance and prognostic impact of p16(INK4A)/p15(INK4B) deletions in childhood T-cell acute lymphoblastic leukaemia

    Ramakers-van Woerden, NL; Pieters, R; Slater, RM; Loonen, A.H.; Beverloo, HB; van Drunen, E; Heyman, M; Moreno, TC; Rots, MG; van Wering, ER; Kamps, WA; Janka-Schaub, GE; Veerman, AJP

    2001-01-01

    p16 gene deletions are present in about 70% of primary paediatric T-cell acute lymphoblastic leukaemia (T-ALL) and 20% of common/precursor B-cell ALL cases. It is not clear what the impact of the frequent p16 deletions is within the subgroup of T-lineage ALL. We studied the relationship between p16/

  10. Pneumocystis jiroveci pneumonia prophylaxis during maintenance therapy influences methotrexate/6-mercaptopurine dosing but not event-free survival for childhood acute lymphoblastic leukemia

    Levinsen, Mette; Shabaneh, Diana; Bohnstedt, Cathrine;

    2012-01-01

    Trimethoprim-sulfamethoxazole (TMP/SMX) is used in children with acute lymphoblastic leukemia (ALL) to prevent Pneumocystis pneumonia (PCP). We explored to which extent TMP/SMX influenced methotrexate (MTX)/6-mercaptopurine (6MP) dosage, myelosuppression, and event-free survival (EFS) during...

  11. Intrachromosomal amplification of chromosome 21 (iAMP21 detected by ETV6/RUNX1 FISH screening in childhood acute lymphoblastic leukemia: a case report

    Daniela Ribeiro Ney Garcia

    2013-01-01

    Full Text Available Chromosome abnormalities that usually define high-risk acute lymphoblastic leukemia are the t(9;22/ breakpoint cluster region protein-Abelson murine leukemia viral oncogene homolog 1, hypodiploid with < 44 chromosomes and 11q23/ myeloid/lymphoid leukemia gene rearrangements. The spectrum of acute lymphoblastic leukemia genetic abnormalities is nevertheless rapidly expanding. Therefore, newly described chromosomal aberrations are likely to have an impact on clinical care in the near future. Recently, the rare intrachromosomal amplification of chromosome 21 started to be considered a high-risk chromosomal abnormality. It occurs in approximately 2-5% of pediatric patients with B-cell precursor acute lymphoblastic leukemia. This abnormality is associated with a poor outcome. Hence, an accurate detection of this abnormality is expected to become very important in the choice of appropriate therapy. In this work the clinical and molecular cytogenetic evaluation by fluorescence in situ hybridization of a child with B-cell precursor acute lymphoblastic leukemia presenting the rare intrachromosomal amplification of chromosome 21 is described.

  12. [A case of myasthenia gravis with invasive thymoma associated with diffuse panbronchiolitis, alopecia, dysgeusia, cholangitis and myositis].

    Maekawa, Risa; Shibuya, Hideki; Hideyama, Takuto; Shiio, Yasushi

    2014-01-01

    A 43-year-old man was admitted to our hospital because of diplopia, ptosis, and dysphagia that had begun three years previously. He was diagnosed with myasthenia gravis (MG) and invasive thymoma and treated with corticosteroid, thymectomy, and radiation therapy. Ten years after the thymectomy, computed tomography (CT) showed metastasis of the thymoma in the left lower lobe of the lung. Two years after this recurrence, when the patient was 55, respiratory symptoms such as wheezing, persistent cough, and dyspnea appeared. Chronic sinusitis, diffuse centrilobular opacities on CT, and positivity for HLA-B54 led to a diagnosis of diffuse panbronchiolitis (DPB). Despite treatment with clarithromycin, the respiratory symptoms worsened. The patient developed alopecia and body hair loss at the age of 56 followed by dysgeusia, cholangitis, and myositis with positivity for anti-Kv1.4 antibodies. Although treatment with an increased dose of corticosteroid improved hair loss, dysgeusia, cholangitis, and myositis, he died of progression of DPB and serious respiratory infection at the age of 58. In this case, various autoimmune disorders occurred together with MG as complications of thymoma. Although alopecia, dysgeusia, and myositis are already known as complications of MG associated with thymoma, cholangitis is not well-recognized since there have been few reports suggesting a causal relationship between cholangitis and thymoma. Furthermore, DPB caused by immunodeficiency and respiratory tract hypersensitivity associated with thymoma and HLA-B54, respectively, is the distinctive feature of our case. Neurologists should be aware that various organs can be damaged directly and indirectly by abnormal T cells from thymoma in patients with MG. PMID:25283823

  13. Acute management of stones

    Jung, Helene; Osther, Palle J S

    2015-01-01

    INTRODUCTION: Stone management is often conservative due to a high spontaneous stone passage rate or non-symptomatic calyceal stones that do not necessarily require active treatment. However, stone disease may cause symptoms and complications requiring urgent intervention. MATERIAL AND METHODS......: In this review, we update latest research and current recommendations regarding acute management of stones, with particular focus on imaging, pain management, active stone interventions, medical expulsive therapy, and urolithiasis in pregnancy and childhood. RESULTS: Acute stone management should be planned...

  14. Miositis iatrogénica en cerditos A case of iatrogenic myositis in piglest

    M. ALONSO-SPILSBURY

    2003-01-01

    Full Text Available Se observó un caso de miositis infecciosa debido a iatrogenia en lechones destetados. Seis lechones de cuatro camadas mostraron cojera uni y bilateral de los miembros traseros, inhabilidad para moverse, posición de perro sentado e hiperflexión de los miembros posteriores. Las lesiones encontradas a la necropsia fueron: osteocondrosis, osteomielitis, nódulos linfáticos inguinales aumentados 30 veces su tamaño normal y abscesos entre las falanges. Otros hallazgos fueron presencia de manchas de hierro en músculos semitendinoso y semimembranoso, sangre acuosa, palidez de la canal y esplenomegalia. Se concluyó que los desórdenes esqueléticos y la cojera junto con la bubonalgia observados en estos lechones se debieron a inyecciones de hierro realizadas en forma séptica e inadecuada por operarios de la granjaA case of infectious myositis due to yatrogenia was observed in weaning piglets. Six piglets from four litters were affected showing unilateral and bilateral hind limb, knuckling of the metatarsalphalangeal joint, inability to move, dog sitting position, and hyperflexion of the posterior digits.Necropsy findings showed: osteochondrosis, osteomyelitis, bubos increased more than 30 times the normal size and, abscesses between phalanges. Other conditions seen were: iron spots in semitendinous and semimembranous muscles, aqueous blood, paleness and splenomegaly. It was concluded that the skeletal disorders and the lameness with buboalgia seen in these pigs were caused by inadequate iron injections performed by unskilled workers at the farm

  15. The impact of childhood acute rotavirus gastroenteritis on the parents’ quality of life: prospective observational study in European primary care medical practices

    Domingo Javier

    2012-05-01

    Full Text Available Abstract Background Rotavirus (RV is the commonest cause of acute gastroenteritis in infants and young children worldwide. A Quality of Life study was conducted in primary care in three European countries as part of a larger epidemiological study (SPRIK to investigate the impact of paediatric rotavirus gastroenteritis (RVGE on affected children and their parents. Methods A self-administered questionnaire was linguistically validated in Spanish, Italian and Polish. The questionnaire was included in an observational multicentre prospective study of 302 children aged Results Questionnaire responses showed that acute RVGE in a child adversely affects the parents’ daily life as well as the child. Parents of children with RVGE experience worry, distress and impact on their daily activities. RVGE of greater clinical severity (assessed by the Vesikari scale was associated with higher parental worries due to symptoms and greater changes in the child’s behaviour, and a trend to higher impact on parents’ daily activities and higher parental distress, together with a higher score on the symptom severity scale of the questionnaire. Conclusions Parents of a child with acute RVGE presenting to primary care experience worry, distress and disruptions to daily life as a result of the child’s illness. Prevention of this disease through prophylactic vaccination will improve the daily lives of parents and children.

  16. C-reactive protein in childhood dermatomyositis

    Haas, R. H.; Dyck, R.F.; Dubowitz, V; Pepys, M. B.

    1982-01-01

    Serum levels of C-reactive protein (CRP) were determined in 9 patients with childhood dermatomyositis. Four children were seen during clinical relapse and all had serum CRP levels less than 1 mg/l. In addition direct immunofluorescent staining of muscle biopsies from 4 patients showed no evidence of CRP deposition in muscle tissue. Such patients appear to be able to produce CRP in response to acute infections, and it is suggested therefore that the pathological process in childhood dermatomyo...

  17. Progress in the management of childhood asthma

    Vichyanond, Pakit; Pensrichon, Rattana; Kurasirikul, Suruthai

    2012-01-01

    Asthma has become the most common chronic disease in childhood. Significant advances in epidemiological research as well as in therapy of pediatric asthma have been made over the past 2 decades. In this review, we look at certain aspects therapy of childhood asthma, both in the past and present. Literature review on allergen avoidance (including mites, cockroach and cat), intensive therapy with β2-agonists in acute asthma (administering via continuous nebulization and intravenous routes), a r...

  18. Childhood obesity

    Heitmann, Berit L; Koplan, Jeffrey; Lissner, Lauren

    2009-01-01

    Despite progress toward assuring the health of today's young population, the 21(st) century began with an epidemic of childhood obesity. There is general agreement that the situation must be addressed by means of primary prevention, but relatively little is known about how to intervene effectively....... The evidence behind the assumption that childhood obesity can be prevented was discussed critically in this roundtable symposium. Overall, there was general agreement that action is needed and that the worldwide epidemic itself is sufficient evidence for action. As the poet, writer, and scholar...

  19. Loperamide in acute diarrhoea in childhood: results of a double blind, placebo controlled multicentre clinical trial. Diarrhoeal Diseases Study Group (UK).

    1984-01-01

    A total of 315 young children with acute diarrhoea were included in a double blind, hospital based multicentre trial of loperamide at two dose levels (0.8 mg and 0.4 mg/kg/24 h), given with standard oral rehydration therapy versus placebo plus oral rehydration therapy. The overall recovery rate was slowest in the placebo group and fastest in the group given loperamide 0.8 mg. Comparisons between weights on admission and weights by day 3 showed that a larger proportion of children in the loper...

  20. Methotrexate/6-mercaptopurine maintenance therapy influences the risk of a second malignant neoplasm after childhood acute lymphoblastic leukemia: results from the NOPHO ALL-92 study

    Schmiegelow, Kjeld; Al-Modhwahi, Ibrahim; Andersen, Mette Klarskov;

    2009-01-01

    Among 1614 children with acute lymphoblastic leukemia (ALL) treated with the Nordic Society for Paediatric Haematology and Oncology (NOPHO) ALL-92 protocol, 20 patients developed a second malignant neoplasm (SMN) with a cumulative risk of 1.6% at 12 years from the diagnosis of ALL. Nine of the 16...... = .07) were related to increased risk of SMN. Thiopurine methyltransferase (TPMT) methylates 6MP and its metabolites, and thus reduces cellular levels of cytotoxic 6-thioguanine nucleotides. Of 524 patients who had erythrocyte TPMT activity measured, the median TPMT activity in 9 patients developing an...

  1. Genome-wide association study identifies HLA 8.1 ancestral haplotype alleles as major genetic risk factors for myositis phenotypes.

    Miller, F W; Chen, W; O'Hanlon, T P; Cooper, R G; Vencovsky, J; Rider, L G; Danko, K; Wedderburn, L R; Lundberg, I E; Pachman, L M; Reed, A M; Ytterberg, S R; Padyukov, L; Selva-O'Callaghan, A; Radstake, T R; Isenberg, D A; Chinoy, H; Ollier, W E R; Scheet, P; Peng, B; Lee, A; Byun, J; Lamb, J A; Gregersen, P K; Amos, C I

    2015-10-01

    Autoimmune muscle diseases (myositis) comprise a group of complex phenotypes influenced by genetic and environmental factors. To identify genetic risk factors in patients of European ancestry, we conducted a genome-wide association study (GWAS) of the major myositis phenotypes in a total of 1710 cases, which included 705 adult dermatomyositis, 473 juvenile dermatomyositis, 532 polymyositis and 202 adult dermatomyositis, juvenile dermatomyositis or polymyositis patients with anti-histidyl-tRNA synthetase (anti-Jo-1) autoantibodies, and compared them with 4724 controls. Single-nucleotide polymorphisms showing strong associations (PAH8.1) defined essentially all the genetic risk in the phenotypes studied. Although the HLA DRB1*03:01 allele showed slightly stronger associations with adult and juvenile dermatomyositis, and HLA B*08:01 with polymyositis and anti-Jo-1 autoantibody-positive myositis, multiple alleles of AH8.1 were required for the full risk effects. Our findings establish that alleles of the AH8.1 comprise the primary genetic risk factors associated with the major myositis phenotypes in geographically diverse Caucasian populations. PMID:26291516

  2. Busulfan and Etoposide Followed by Peripheral Blood Stem Cell Transplant and Low-Dose Aldesleukin in Treating Patients With Acute Myeloid Leukemia

    2015-08-04

    Adult Acute Myeloid Leukemia in Remission; Adult Acute Myeloid Leukemia With 11q23 (MLL) Abnormalities; Adult Acute Myeloid Leukemia With Del(5q); Adult Acute Myeloid Leukemia With t(15;17)(q22;q12); Adult Acute Myeloid Leukemia With t(16;16)(p13;q22); Childhood Acute Myeloid Leukemia in Remission; Recurrent Adult Acute Myeloid Leukemia; Recurrent Childhood Acute Myeloid Leukemia

  3. Childhood pneumonia and vitamin A

    Farhad Heidarian; Tahereh Ansarinezhad

    2014-01-01

    One of the major causes of mortality in children younger than 5 years old is acute lower respiratory tract infections (ALRI). ALRI clinical features are cough, tachypnea, fever, coryza, chest retraction, crackles and wheeze. Increased white blood cell count with left shift might happen in pneumonia. C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) might rise in children with respiratory tract infections. Vitamin A deficiency is associated with severe childhood infections. The...

  4. High proportions of CD4⁺ T cells among residual bone marrow T cells in childhood acute lymphoblastic leukemia are associated with favorable early responses.

    Lustfeld, Imke; Altvater, Bianca; Ahlmann, Martina; Ligges, Sandra; Brinkrolf, Peter; Rosemann, Annegret; Moericke, Anja; Rossig, Claudia

    2014-01-01

    Residual nonmalignant T cells in the bone marrow of patients with acute leukemias may be involved in active immune responses to leukemic cells. Here, we investigated the phenotypic signature of T cells present at diagnosis in 39 pediatric patients with acute lymphoblastic leukemia (ALL) treated within standardized ALL-BFM study protocols. Previously described age associations of lymphocyte subpopulations in the peripheral blood of healthy children were reproduced in leukemic bone marrow. Analysis of individual lymphocyte parameters and risk-associated variables using univariate linear regression models revealed a correlation of higher CD4/CD8 ratios at diagnosis with a favorable bone marrow response on day 15. Separate analysis of CD4⁺ cells with the CD4⁺CD25(hi)FoxP3⁺ T(reg) cell phenotype showed that the association was caused by non-T(reg) CD4⁺ cells. The association of higher CD4/CD8 ratios with a favorable bone marrow response on day 15 of treatment persisted in a cohort extended to 69 patients. We conclude that CD4⁺ non-T(reg) cells in leukemic bone marrow at diagnosis may have a role in early response to treatment. Prospective analysis of the CD4/CD8 ratio in a large cohort of pediatric patients is now needed. Moreover, future experiments will establish the functional role of the individual T cell subsets in immune control in pediatric ALL. PMID:24021585

  5. Cyclophosphamide and Busulfan Followed by Donor Stem Cell Transplant in Treating Patients With Myelofibrosis, Acute Myeloid Leukemia, or Myelodysplastic Syndrome

    2014-04-03

    Adult Acute Myeloid Leukemia in Remission; Adult Acute Myeloid Leukemia With 11q23 (MLL) Abnormalities; Adult Acute Myeloid Leukemia With Del(5q); Adult Acute Myeloid Leukemia With Inv(16)(p13;q22); Adult Acute Myeloid Leukemia With t(15;17)(q22;q12); Adult Acute Myeloid Leukemia With t(16;16)(p13;q22); Adult Acute Myeloid Leukemia With t(8;21)(q22;q22); Childhood Acute Myeloid Leukemia in Remission; Childhood Myelodysplastic Syndromes; de Novo Myelodysplastic Syndromes; Essential Thrombocythemia; Myelodysplastic Syndrome With Isolated Del(5q); Polycythemia Vera; Previously Treated Myelodysplastic Syndromes; Primary Myelofibrosis; Recurrent Adult Acute Myeloid Leukemia; Recurrent Childhood Acute Myeloid Leukemia; Secondary Acute Myeloid Leukemia; Secondary Myelodysplastic Syndromes; Secondary Myelofibrosis; Untreated Adult Acute Myeloid Leukemia; Untreated Childhood Acute Myeloid Leukemia and Other Myeloid Malignancies

  6. Childhood Obesity

    Yuca, Sevil Ari, Ed.

    2012-01-01

    This book aims to provide readers with a general as well as an advanced overview of the key trends in childhood obesity. Obesity is an illness that occurs due to a combination of genetic, environmental, psychosocial, metabolic and hormonal factors. The prevalence of obesity has shown a great rise both in adults and children in the last 30 years.…

  7. Childhood Obesity

    2013-08-06

    In this podcast, Dr. Tom Frieden, CDC Director, discusses the decrease in childhood obesity rates and what strategies have been proven to work to help our children grow up and thrive.  Created: 8/6/2013 by National Center for Injury Prevention and Control.   Date Released: 3/6/2014.

  8. Characterization of DLK1+ cells emerging during skeletal muscle remodeling in response to myositis, myopathies, and acute injury

    Andersen, Ditte C; Petersson, Stine J; Jørgensen, Louise H;

    2009-01-01

    Delta like 1 (DLK1) has been proposed to act as a regulator of cell fate determination and is linked to the development of various tissues including skeletal muscle. Herein we further investigated DLK1 expression during skeletal muscle remodeling. Although practically absent in normal adult muscle......, DLK1 was upregulated in all human myopathies analyzed, including Duchenne- and Becker muscular dystrophies. Substantial numbers of DLK1(+) satellite cells were observed in normal neonatal and Duchenne muscle, and furthermore, myogenic DLK1(+) cells were identified during muscle regeneration in animal...... models in which the peak expression of Dlk1 mRNA and protein coincided with that of myoblast differentiation and fusion. In addition to perivascular DLK1(+) cells, interstitial DLK1(+) cells were numerous in regenerating muscle, and in agreement with colocalization studies of DLK1 and CD90/DDR2, qPCR of...

  9. Daily supplementation of D-ribose shows no therapeutic benefits in the MHC-I transgenic mouse model of inflammatory myositis.

    William Coley

    Full Text Available BACKGROUND: Current treatments for idiopathic inflammatory myopathies (collectively called myositis focus on the suppression of an autoimmune inflammatory response within the skeletal muscle. However, it has been observed that there is a poor correlation between the successful suppression of muscle inflammation and an improvement in muscle function. Some evidence in the literature suggests that metabolic abnormalities in the skeletal muscle underlie the weakness that continues despite successful immunosuppression. We have previously shown that decreased expression of a purine nucleotide cycle enzyme, adenosine monophosphate deaminase (AMPD1, leads to muscle weakness in a mouse model of myositis and may provide a mechanistic basis for muscle weakness. One of the downstream metabolites of this pathway, D-ribose, has been reported to alleviate symptoms of myalgia in patients with a congenital loss of AMPD1. Therefore, we hypothesized that supplementing exogenous D-ribose would improve muscle function in the mouse model of myositis. We treated normal and myositis mice with daily doses of D-ribose (4 mg/kg over a 6-week time period and assessed its effects using a battery of behavioral, functional, histological and molecular measures. RESULTS: Treatment with D-ribose was found to have no statistically significant effects on body weight, grip strength, open field behavioral activity, maximal and specific forces of EDL, soleus muscles, or histological features. Histological and gene expression analysis indicated that muscle tissues remained inflamed despite treatment. Gene expression analysis also suggested that low levels of the ribokinase enzyme in the skeletal muscle might prevent skeletal muscle tissue from effectively utilizing D-ribose. CONCLUSIONS: Treatment with daily oral doses of D-ribose showed no significant effect on either disease progression or muscle function in the mouse model of myositis.

  10. Interphase fluorescent in situ hybridization deletion analysis of the 9p21 region and prognosis in childhood acute lymphoblastic leukaemia (ALL)

    Kuchinskaya, Ekaterina; Heyman, Mats; Nordgren, Ann;

    2011-01-01

    Interphase fluorescent in situ hybridization (FISH) was applied on diagnostic BM smears from 519 children with acute lymphoblastic leukaemia (ALL) in order to establish the frequency and prognostic importance of 9p21 deletion in children enrolled in the Nordic Society of Paediatric Haematology and...... Oncology (NOPHO) - 2000 treatment protocol. Among the patients, 452 were diagnosed with B-cell precursor (BCP)-ALL and 66 with T-ALL. A higher incidence of 9p21 deletions was found in T-ALL (38%) compared to BCP-ALL (15·7%). Homozygous deletions were found in 19·7% of T-ALL and 4·0% of BCP-ALL; hemizygous...

  11. Outcome of ETV6/RUNX1-positive childhood acute lymphoblastic leukaemia in the NOPHO-ALL-1992 protocol: frequent late relapses but good overall survival

    Forestier, E.; Heyman, M.; Andersen, Mette Klarskov;

    2008-01-01

    The prognostic impact of t(12;21)(p13;q22) [ETV6/RUNX1 fusion] in paediatric acute lymphoblastic leukaemia (ALL) has been extensively debated, particularly with regard to the frequency of late relapses and appropriate treatment regimens. We have retrospectively collected 679 ALLs with known ETV6....../RUNX1 status, as ascertained by fluorescence in situ hybridization or reverse-transcription polymerase chain reaction, treated according to the Nordic Society of Paediatric Haematology and Oncology -ALL-1992 protocol. The assigned risk groups/treatment modalities for the 171 (25%) patients with t(12...... almost 50% of all relapses occurring > or = 5 years after diagnosis. Of all relapses after 6 years, 80% occurred in the t(12;21)-positive group. The overall survival was 94% at 5 years and 88% at 10 years; thus, the treatment of patients in second or later remission is usually successful. As yet, there...

  12. Thiopurine methyltransferase activity is related to the risk of relapse of childhood acute lymphoblastic leukemia: results from the NOPHO ALL-92 study

    Schmiegelow, K; Forestier, E; Kristinsson, J; Söderhäll, S; Vettenranta, K; Weinshilboum, R; Wesenberg, F

    2008-01-01

    Myelotoxicity during thiopurine therapy is enhanced in patients, who because of single nucleotide polymorphisms have decreased activity of the enzyme thiopurine methyltransferase (TPMT) and thus more thiopurine converted into 6-thioguanine nucleotides. Of 601 children with acute lymphoblastic leu...... wild type might have their cure rate improved, if the pharmacokinetics/-dynamics of TPMT low-activity patients could be mimicked without a concurrent excessive risk of second cancers....... worse; P=0.02) were related to risk of relapse. Despite a lower probability of relapse, patients in the low TPMT activity group did not have superior survival (P=0.82), possibly because of an excess of secondary cancers among these 75 patients (P=0.07). These data suggest that children with ALL and TPMT...

  13. Medical Research Council leukaemia trial--UKALL V: an attempt to reduce the immunosuppressive effects of therapy in childhood acute lymphoblastic leukemia. Report to the Council by the Working Party on Leukaemia in Childhood.

    Chessells, J M; Durrant, J; Hardy, R M; Richards, S

    1986-12-01

    The Medical Research Council UKALL V trial for children with standard-risk acute lymphoblastic leukemia (ALL) (aged 1 to 14 years, leucocyte count less than 20 X 10(9)/L) was designed to determine whether the immunosuppressive effects of treatment could be reduced without sacrifice of antileukemic effect by alterations in the type of continuing therapy or in fractionation of cranial irradiation. Remission was achieved in 496 children on standard induction therapy, and 309 children received 24 Gy of cranial irradiation in ten to 16 fractions over 21 days, and 174 received 21 Gy in five to nine fractions over 21 days. The type of radiotherapy administered had no influence on relapse at any site or rate of death in remission. All 496 children were randomized to receive chemotherapy for 2 or 3 years with 6-mercaptopurine and methotrexate either as a continuous (group C) or a semicontinuous (group G) regimen or as a five-day pulse every 3 weeks (group I). All groups also received vincristine and prednisolone every 6 weeks. With a minimum follow-up of almost 7 years, patients in group I had significantly fewer remission deaths (P = .025) but a much higher rate of bone marrow relapse than those in group C or G (P = .002). There was an overall benefit for 3 years of chemotherapy compared with 2 years, which in contrast to previous studies, was more apparent in girls and in patients in groups C and G. Testicular relapse occurred in 37 boys, including 19 patients off therapy, with a previously negative biopsy. The overall results confirmed the prognostic significance of initial leucocyte count, even among these standard-risk patients, while girls had a superior rate of disease-free survival, but not of hematologic remission. It is concluded that, even among standard-risk patients, the prognosis is influenced by the height of the initial leukocyte count. While alterations in the fractionation of cranial irradiation do not appear to have influenced disease-free survival

  14. Maternal immunoglobulin E and childhood leukemia.

    Chang, Jeffrey S; Buffler, Patricia A; Metayer, Catherine; Chokkalingam, Anand P; Patoka, Joe; Kronish, Daniel; Wiemels, Joseph L

    2009-08-01

    Childhood leukemia, particularly acute lymphoblastic leukemia (ALL), has long been hypothesized to be affected by abnormal immune responses to microbial challenges stemming from a lack of immune modulation in early childhood. Studies of allergies suggest that a child's immune development may be modulated by maternal immune status. We conducted a study to explore the relationship between maternal immunoglobulin E (IgE) and childhood leukemia and to investigate whether maternal immune status can influence childhood leukemia risk. Serum total and specific IgE (respiratory and food) were measured in biological mothers of 352 children (193 healthy controls and 159 leukemia cases, including 139 ALL cases) ages <8 years who were enrolled in the Northern California Childhood Leukemia Study. Odds ratios associated with maternal IgE were calculated using unconditional logistic regression adjusted for child's age, sex, race/ethnicity, and annual household income. A positive association between childhood leukemia or ALL and elevated levels of maternal serum total IgE was observed, especially among Hispanics. In addition, a positive association was observed between childhood leukemia or ALL and maternal respiratory or food IgE status. These results suggest that maternal immune function may play a crucial role in the etiology of childhood leukemia, although additional studies need to be conducted to confirm the results of this study and provide a perspective on mechanisms. PMID:19622720

  15. Childhood vitiligo

    Aparna Palit

    2012-01-01

    Full Text Available Childhood vitiligo is often encountered in dermatological practice. When present in infancy or early childhood, various nevoid and hereditary disorders are to be differentiated. In many cases, familial aggregation of the disease is seen and other autoimmune disorders may be associated. Segmental presentation is more common, and limited body surface area involvement is usual in this age group. Children with vitiligo often suffer from anxiety and depression because of their unusual appearance. Management of vitiligo in children is difficult as therapeutic options are restricted when compared to that in adult patients. Selection of treatment should be careful in these patients with the aim to achieve best results with minimal side effects as well as relieving patients′ and parents′ anxiety.

  16. Infections During Induction Therapy of Protocol CCLG-2008 in Childhood Acute Lymphoblastic Leukemia: A Single-center Experience with 256 Cases in China

    Si-Dan Li

    2015-01-01

    Full Text Available Background: Infections remain a major cause of therapy-associated morbidity and mortality in children with acute lymphoblastic leukemia (ALL. Methods: We retrospectively analyzed the medical charts of 256 children treated for ALL under the CCLG-2008 protocol in Beijing Children′s Hospital. Results: There were 65 infectious complications in 50 patients during vincristine, daunorubicin, L-asparaginase and dexamethasone induction therapy, including microbiologically documented infections (n = 12; 18.5%, clinically documented infections (n = 23; 35.3% and fever of unknown origin (n = 30; 46.2%. Neutropenia was present in 83.1% of the infectious episodes. In all, most infections occurred around the 15 th day of induction treatment (n = 28, and no patients died of infection-associated complications. Conclusions: The infections in this study was independent of treatment response, minimal residual diseases at the end of induction therapy, gender, immunophenotype, infection at first visit, risk stratification at diagnosis, unfavorable karyotypes at diagnosis and morphologic type. The infection rate of CCLG-2008 induction therapy is low, and the outcome of patients is favorable.

  17. Outcome of central nervous system relapses in childhood acute lymphoblastic leukaemia--prospective open cohort analyses of the ALLR3 trial.

    Ashish Narayan Masurekar

    Full Text Available The outcomes of Central Nervous System (CNS relapses in children with acute lymphoblastic leukaemia (ALL treated in the ALL R3 trial, between January 2003 and March 2011 were analysed. Patients were risk stratified, to receive a matched donor allogeneic transplant or fractionated cranial irradiation with continued treatment for two years. A randomisation of Idarubicin with Mitoxantrone closed in December 2007 in favour of Mitoxantrone. The estimated 3-year progression free survival for combined and isolated CNS disease were 40.6% (25·1, 55·6 and 38.0% (26.2, 49.7 respectively. Univariate analysis showed a significantly better survival for age <10 years, progenitor-B cell disease, good-risk cytogenetics and those receiving Mitoxantrone. Adjusting for these variables (age, time to relapse, cytogenetics, treatment drug and gender a multivariate analysis, showed a poorer outcome for those with combined CNS relapse (HR 2·64, 95% CI 1·32, 5·31, p = 0·006 for OS. ALL R3 showed an improvement in outcome for CNS relapses treated with Mitoxantrone compared to Idarubicin; a potential benefit for matched donor transplant for those with very early and early isolated-CNS relapses.Controlled-Trials.com ISRCTN45724312.

  18. Infections During Induction Therapy of Protocol CCLG-2008 in Childhood Acute Lymphoblastic Leukemia: A Single-center Experience with 256 Cases in China

    Si-Dan Li; Yong-Bing Chen; Zhi-Gang Li; Run-Hui Wu; Mao-Quan Qin; Xuan Zhou; Jin Jiang

    2015-01-01

    Background:Infections remain a major cause of therapy-associated morbidity and mortality in children with acute lymphoblastic leukemia (ALL).Methods:We retrospectively analyzed the medical charts of 256 children treated for ALL under the CCLG-2008 protocol in Beijing Children's Hospital.Results:There were 65 infectious complications in 50 patients during vincristine,daunorubicin,L-asparaginase and dexamethasone induction therapy,including microbiologically documented infections (n =12; 18.5%),clinically documented infections (n =23; 35.3%) and fever of unknown origin (n =30; 46.2%).Neutropenia was present in 83.1% of the infectious episodes.In all,most infections occurred around the 15t1h day of induction treatment (n =28),and no patients died of infection-associated complications.Conclusions:The infections in this study was independent of treatment response,minimal residual diseases at the end of induction therapy,gender,immunophenotype,infection at first visit,risk stratification at diagnosis,unfavorable karyotypes at diagnosis and morphologic type.The infection rate of CCLG-2008 induction therapy is low,and the outcome of patients is favorable.

  19. Single nucleotide polymorphisms in non-coding region of the glucocorticoid receptor gene and prednisone response in childhood acute lymphoblastic leukemia.

    Xue, Lu; Li, Chunhuai; Wang, Yue; Sun, Wei; Ma, Cui; He, Yongyan; Yu, Yongli; Cai, Lu; Wang, Liying

    2015-06-01

    Poor prednisone response predicts an inferior outcome in pediatric acute lymphoblastic leukemia (ALL) in Berlin-Frankfurt-Münster (BFM) treatment protocols. Here, we investigated five single nucleotide polymorphisms (SNPs) in both the coding and non-coding regions of the glucocorticoid receptor (GR) gene, and analyzed their association with prednisone responsiveness in vivo in 63 pediatric patients with ALL in China. Of the five SNPs, the rs41423247 and rs7701443 polymorphisms were significantly associated with prednisone response at the allelic level (rs41423247 odds ratio [OR] = 9.58; 95% confidence interval [CI]: 1.23-74.21; p = 0.01; rs7701443 OR = 3.12; 95% CI: 1.08-9; p = 0.02). Two polymorphisms (rs6189/6190 and rs6198) were not observed in the study cohort. Haplotypes composed of CCC alleles and TCG alleles at three loci (rs7701443, Tth111I and BclI) were both associated with prednisone response (p = 0.013; p = 0.028). Our results suggested that polymorphisms in the non-coding region of the GR gene were associated with prednisone response in vivo in pediatric ALL in Han Chinese. PMID:25644744

  20. Ikaros基因与儿童急性淋巴细胞白血病预后的关系%Ikaros and childhood acute lymphoblastic leukemia

    周芬

    2010-01-01

    Ikaros is a transcriptional factor playing an essential role in lymphoid lineage development and differentiation. Ikaros gene deletions occur in some acute lymphoblastic leukemia (ALL) patients, and the most common type of abnormality is overexpression of dominant negative isoforms 6 (Ik6). Deletion of Ikaros gene has an independent association with a very poor outcome in B-cell-progenitor ALL. A new subtype of ALL characterized by the deletion of Ikaros and poor outcome has been identified by researchers, and named BCR/ABL1 like ALL.We can conclude that Ikaros may play an important role in the diagnosis and treatment of pediatric ALL.%Ikaros是淋巴细胞发育和增殖所必需的转录因子,在部分儿童急性淋巴细胞白血病(ALL)中表现为不同的缺失状态,其中以Ik6显性负相亚型过表达多见.Ikaros缺失是B祖细胞型ALL患者预后不良的一个独立危险因素.国外学者最近还确立了ALL的一种新亚型"BCR/ABL1-like ALL",同样以Ikaros缺失、预后不良为主要特征.由此推测,Ikaros对儿童ALL的诊断和治疗可能起着关键的作用.

  1. Childhood psoriasis

    Dogra Sunil; Kaur Inderjeet

    2010-01-01

    Psoriasis is a common dermatosis in children with about one third of all patients having onset of disease in the first or second decade of life. A chronic disfiguring skin disease, such as psoriasis, in childhood is likely to have profound emotional and psychological effects, and hence requires special attention. Psoriasis in children has been reported to differ from that among adults being more frequently pruritic; plaque lesions are relatively thinner, softer, and less scaly; face and flexu...

  2. Childhood Traumatic Grief

    ... Educators Resources for Kids and Teens Childhood Traumatic Grief What is Childhood Traumatic Grief? Children grieve in their own way following the ... child may have a condition called Childhood Traumatic Grief (CTG). Thinking about the person who died—even ...

  3. Childhood Cancer Statistics

    ... Shop With CureSearch Blog Donate Now Select Page Childhood Cancer Statistics Home > Understanding Children’s Cancer > Childhood Cancer Statistics Childhood Cancer Statistics – Graphs and Infographics Number of Diagnoses ...

  4. Tretinoin and Arsenic Trioxide in Treating Patients With Untreated Acute Promyelocytic Leukemia

    2015-12-30

    Adult Acute Promyelocytic Leukemia With t(15;17)(q22;q12); PML-RARA; Childhood Acute Promyelocytic Leukemia With t(15;17)(q22;q12); PML-RARA; Untreated Adult Acute Myeloid Leukemia; Untreated Childhood Myeloid Neoplasm

  5. Bone marrow ectopic expression of a non-coding RNA in childhood T-cell acute lymphoblastic leukemia with a novel t(2;11(q11.2;p15.1 translocation

    Leszl Anna

    2008-10-01

    Full Text Available Abstract Chromosomal translocations play a crucial role in tumorigenesis, often resulting in the formation of chimeric genes or in gene deregulation through position effects. T-cell acute lymphoblastic leukemia (T-ALL is associated with a large number of such rearrangements. We report the ectopic expression of the 3' portion of EST DA926692 in the bone marrow of a childhood T-ALL case showing a t(2;11(q11.2;p15.1 translocation as the sole chromosome abnormality. The breakpoints, defined at the sequence level, mapped within HPS5 (Hermansky Pudlak syndrome 5 intron 1 at 11p15.1, and DA926692 exon 2 at 2q11.2. The translocation was accompanied by a submicroscopic inversion that brought the two genes into the same transcriptional orientation. No chimeric trancript was detected. Interestingly, Real-Time Quantitative (RQ-PCR detected, in the patient's bone marrow, expression of a 173 bp product corresponding to the 3' portion of DA926692. Samples from four T-ALL cases with a normal karyotype and normal bone marrow used as controls were negative. It might be speculated that the juxtaposition of this genomic segment to the CpG island located upstream HPS5 activated DA92669 expression. RQ-PCR analysis showed expression positivity in 6 of 23 human tissues examined. Bioinformatic analysis excluded that this small non-coding RNA is a precursor of micro-RNA, although it is conceivable that it has a different, yet unknown, functional role. To the best of our knowledge, this is the first report, in cancer, of the activation of a small non-coding RNA as a result of a chromosomal translocation.

  6. Immunophenotyping with CD135 and CD117 predicts the FLT3, IL-7R and TLX3 gene mutations in childhood T-cell acute leukemia.

    Noronha, Elda Pereira; Andrade, Francianne Gomes; Zampier, Carolina; de Andrade, Camilla F C G; Terra-Granado, Eugênia; Pombo-de-Oliveira, Maria S

    2016-03-01

    With the combination of immunophenotyping and molecular tests, it is still a challenge to identify the characteristics of T cell acute lymphoblastic leukemia (T-ALL) associated with distinct outcomes. This study tests the possible correlation of cellular expression of CD135 and CD117 with somatic gene mutations in T-ALL. One hundred sixty-two samples were tested, including 143 at diagnosis, 15 from T-lymphoblastic lymphoma at relapse, and four relapse samples from sequential follow-up of T-ALL. CD135 and CD117 monoclonal antibodies were included in the T-ALL panel of flow cytometry. The percentage of cells positivity and the median fluorescence intensity were correlated with gene mutational status. STIL-TAL1, TLX3, FLT3 and IL7R mutations were tested using standard techniques. STIL-TAL1 was found in 24.8%, TLX3 in 12%, IL7R in 10% and FLT3-ITD in 5% of cases. FLT3 and IL7R mutations were mutually exclusive, as were FLT3-ITD and STIL-TAL1. Associations of CD135(high) (p<0.01), CD117(intermediate/high) (p=0.02) and FLT3-ITD, CD117(low) with IL7R(mutated) (p<0.01) and CD135(high) with TLX3(pos) were observed. We conclude that the addition of CD135 and CD117 to the diagnosis can predict molecular aberrations in T-ALL settings, mainly segregating patients with FLT3-ITD, who would benefit from treatment with inhibitors of tyrosine. PMID:26852660

  7. HIT`91 (prospective, co-operative study for the treatment of malignant brain tumors in childhood): accuracy and acute toxicity of the irradiation of the craniospinal axis

    Kortmann, R.D.; Timmermann, B.; Bamberg, M. [Tuebingen Univ. (Germany). Dept. of Radiotherapy; Kuehl, J. [Wuerzburg Univ. (Germany). Children`s Hospital; Willich, N. [Muenster Univ. (Germany). Dept. of Radiotherapy; Flentje, M. [Wuerzburg Univ. (Germany). Dept. of Radiotherapy; Meisner, C. [Tuebingen Univ. (Germany). Inst. for Medical Information Processing

    1999-04-01

    Background: It was the aim of the quality control program of the randomized trial HIT `91 (intensive chemotherapy before irradiation versus maintenance chemotherapy after irradiation) to assess prospectively the quality of neuroaxis irradiation with respect to the protocol guidelines and to evaluate acute toxicity with respect to treatment arm. Patients, Materials and Methods: Data of 134 patients undergoing irradiation of the craniospinal axis were available. Positioning aids, shielding techniques, treatment machines, choice of energy, total dose and fractionation were evaluated. A total of 651 simulation and verification films were analyzed to assess the coverage of the clinical target volume (whole brain, posterior fossa, sacral nerve roots) and deviations of field alignment between simulation and verification of first treatment. Field matching between whole brain and adjacent cranial spinal fields was analyzed with respect to site and width of junction. Acute maximal side effects were evaluated according to a modified WHO score for neurotoxicity, infections, skin, mucosa and myelotoxicity. Results: In 91.3% of patients contemporary positioning aids and individualized shielding techniques were used to assure a reproducible treatment. In 98 patients (73.1%) linear accelerators and in 36 patients (26.8%) {sup 60}Cobalt machines were used. Single and total dose were administered according to the protocol guidelines in more than 90% of patients. In 20.2% of patients the cribriform plate, in 1.4% the middle cranial fossa and in 21.1% the posterior fossa and in 4.5% the 2nd sacral segment were incompletely encompassed by the treatment portals. Ninety-five percent of deviations of field alignment were less than 13.0 mm (whole brain) and 12 mm (cranial spinal field) with a random error between 4.9 and 7.6 mm (whole brain) and 6.9 mm and 9.9 mm (spinal canal), respectively. In 77.5% of patients the junctions between whole brain and cranial spinal fields were placed

  8. Clinical trials of CCLSG L874 and I874 protocols without cranial irradiation for standard-risk acute lymphoblastic leukemia in childhood

    In the CCLSG-874 protocol for children with low-risk (LR) and intermediate-risk (IR) acute lymphoblastic leukemia (ALL), two regimens with or without cranial irradiation (CI) were compared with respect to their ability to prevent central nervous system (CNS) leukemia and to improve overall outcome of ALL. From 1987 to 1990, 82 and 109 evaluable patients were registered into L874 and I874 protocols for LR and IR patients, respectively. All responders to induction therapy were randomized to treatment with 18 Gy of CI plus intrathecal methotrexate (MTX it) or to treatment with high-dose MTX plus MTX it. Patients were then treated with standard maintenance regimens of L874 and I874. At a median follow-up of 39 months (range 14-58 months) there was no difference in the rate of hematologic relapse between the CI group and MTX group. The rate of CNS relapse in the MTX group seemed to be higher (3 of 39 in L874 and 2 of 54 in I874) than that in the CI group (1 of 43 in L874 and 0 of 55 in I874), but these data were not statistically significant. The rates of 4-year event-free survival (EFS) in L874 were 81.1±7.6% (mean±SE) and 75.2±7.9% (ns) for the CI and MTX group, respectively, and the rates of EFS in I874 were 70.0±13.6% and 70.0±9.0% (ns) for the CI and MTX group, respectively. These data suggest that MTX alone may be as effective as CI to prolong disease-free survival in LR and IR ALL although further continuous studies are needed. Analysis of serial CCLSG protocols for ALL from 1981 revealed that the rate of EFS of ALL allover including all risk groups has gradually been increasing from 44.2±3.6% for 811 protocol and 53.1±3.5% for 841 to 65.5±3.6% for the present 874 protocol. (author)

  9. Indoor radon and childhood leukaemia

    This paper summarises the epidemiological literature on domestic exposure to radon and risk for childhood leukaemia. The results of 12 ecological studies show a consistent pattern of higher incidence and mortality rates for childhood leukaemia in areas with higher average indoor radon concentrations. Although the results of such studies are useful to generate hypotheses, they must be interpreted with caution, as the data were aggregated and analysed for geographical areas and not for individuals. The seven available case - control studies of childhood leukaemia with measurement of radon concentrations in the residences of cases and controls gave mixed results, however, with some indication of a weak (relative risk < 2) association with acute lymphoblastic leukaemia. The epidemiological evidence to date suggests that an association between indoor exposure to radon and childhood leukaemia might exist, but is weak. More case - control studies are needed, with sufficient statistical power to detect weak associations and based on designs and methods that minimise misclassification of exposure and provide a high participation rate and low potential selection bias. (authors)

  10. Childhood psoriasis

    Dogra Sunil

    2010-01-01

    Full Text Available Psoriasis is a common dermatosis in children with about one third of all patients having onset of disease in the first or second decade of life. A chronic disfiguring skin disease, such as psoriasis, in childhood is likely to have profound emotional and psychological effects, and hence requires special attention. Psoriasis in children has been reported to differ from that among adults being more frequently pruritic; plaque lesions are relatively thinner, softer, and less scaly; face and flexural involvement is common and guttate type is the characteristic presentation. Whether onset in childhood predicts a more severe form of psoriasis is a matter of controversy, it may cause significant morbidity particularly if it keeps relapsing. Most children have mild form of psoriasis which can be generally treated effectively with topical agents such as emollients, coal tar, corticosteroids, dithranol, calcipotriol etc. according to age and the sites affected. Narrow band UVB is the preferred form of phototherapy in children for moderate to severe disease or in patients not responding to topical therapy alone. Systemic therapies are reserved for more severe and extensive cases that cannot be controlled with topical treatment and/or phototherapy such as severe plaque type, unstable forms like erythrodermic and generalized pustular psoriasis and psoriatic arthritis. There are no controlled trials of systemic therapies in this age group, most experience being with retinoids and methotrexate with favorable results. Cyclosporine can be used as a short-term intermittent crisis management drug. There is an early promising experience with the use of biologics (etanercept and infliximab in childhood psoriasis. Systemic treatments as well as phototherapy have limited use in children due to cumulative dose effects of drugs, low acceptance, and risk of gonadal toxicity. More evidence-based data is needed about the effectiveness and long-term safety of topical

  11. CD10 is a marker for cycling cells with propensity to apoptosis in childhood ALL

    G. Cutrona; Tasso, P; Dono, M; Roncella, S; M. ULIVI; Carpaneto, E M; Fontana, V; Comis, M; F. Morabito; Spinelli, M.; Frascella, E.; Boffa, L C; G. Basso; Pistoia, V.; Ferrarini, M.

    2002-01-01

    CD10 constitutes a favourable prognostic marker for childhood acute lymphoblastic leukaemia. Since correlations between CD10, cell cycle and apoptotic abilities were demonstrated in various cell types, we investigated whether differences existed in the cycling/apoptotic abilities of CD10-positive and CD10-negative B acute lymphoblastic leukaemia cells. Twenty-eight cases of childhood acute lymphoblastic leukaemia (mean age of 6.8 years) were subdivided into two groups according to high (17 ca...

  12. Heterogeneous clinical spectrum of anti-SRP myositis and importance of the methods of detection of anti-SRP autoantibodies: a multicentric study.

    Picard, Cécile; Vincent, Thierry; Lega, Jean-Christophe; Hue, Sophie; Fortenfant, Françoise; Lakomy, Daniela; Humbel, René-Louis; Goetz, Joelle; Molinari, Nicolas; Bardin, Nathalie; Bertin, Daniel; Johanet, Catherine; Chretien, Pascale; Dubucquoi, Sylvain; Streichenberger, Nathalie; Desplat-Jégo, Sophie; Bossuyt, Xavier; Sibilia, Jean; Abreu, Isabelle; Chevailler, Alain; Fabien, Nicole

    2016-06-01

    Anti-signal recognition particle (SRP) antibodies are important serological markers for the diagnosis and the prognosis of idiopathic inflammatory myopathy (IIM), especially to distinguish immune-mediated necrotizing myopathy (IMNM). This study was set up to investigate the phenotype associated with anti-SRP antibodies and to evaluate the methods for detecting these antibodies. Clinical and biological data were retrospectively obtained from 60 adult patients with anti-SRP antibodies detected by a dot immunoassay from 12 centers. Thirty-six (60 %) out of these 60 patients suffered from an IIM, and among them, 21 patients were diagnosed as IMNM. Among patients with a definite IIM, proximal weakness and myalgia were prominent symptoms at the time of diagnosis. Only few patients displayed severe extra-muscular symptoms such as cardiac involvement or severe myositis. Mean creatine kinase levels were high for all patients except for two of them. When testing by indirect immunofluorescence (IIF) on HEp2 cells, the fraction of patients displaying the typical anti-SRP fine speckled staining of the cytoplasm was higher in patients with IIM (30/36) (83 %) than in patients with non-IIM (3/24) (12.5 %) (p < 0.0001). Thirty (91 %) out of 33 patients with a positive immunodot and a characteristic IIF cytoplasmic staining suffered from a clinical definite myositis, whereas only 6 (22 %) out of 27 patients with a positive immunodot but a negative cytoplasmic pattern suffered from a myositis (p < 0.00001). This series highlights the strong heterogeneity of anti-SRP positivity that encompassed IMNM and non-IMNM and supports the necessity of considering both IIF and dot immunoassay to confirm the diagnosis of anti-SRP-associated myositis. PMID:26744256

  13. Ocular myositis as first presenting symptom of human immunodeficiency virus (HIV-1) infection and its response to high-dose cortisone treatment.

    Fabricius, E. M.; Hoegl, I.; Pfaeffl, W.

    1991-01-01

    A 30-year-old male presented with signs of ocular inflammation and motility disturbances in an early stage of HIV infection. The provisional diagnosis of an ocular myositis was confirmed by orbital echograms. A general check up revealed positive anti-smooth-muscle antibodies and antinuclear antibodies as well as a raised erythrocyte sedimentation rate. Oral steroid treatment in addition to steroid eyedrops achieved complete resolution of clinical and sonographic symptoms within 15 weeks. Auto...

  14. Daily Supplementation of D-ribose Shows No Therapeutic Benefits in the MHC-I Transgenic Mouse Model of Inflammatory Myositis

    Coley, William; Rayavarapu, Sree; Jack H. Van der Meulen; Duba, Ayyappa S.; Nagaraju, Kanneboyina

    2013-01-01

    Background Current treatments for idiopathic inflammatory myopathies (collectively called myositis) focus on the suppression of an autoimmune inflammatory response within the skeletal muscle. However, it has been observed that there is a poor correlation between the successful suppression of muscle inflammation and an improvement in muscle function. Some evidence in the literature suggests that metabolic abnormalities in the skeletal muscle underlie the weakness that continues despite success...

  15. Abnormal accumulation of prion protein mRNA in muscle fibers of patients with sporadic inclusion-body myositis and hereditary inclusion-body myopathy.

    Sarkozi, E; Askanas, V.; Engel, W K

    1994-01-01

    Sporadic inclusion-body myositis is the most common progressive muscle disease of older patients. The muscle biopsy demonstrates mononuclear cell inflammation and vacuolated muscle fibers containing paired helical filaments and 6 to 10-nm fibrils, both resembling those of Alzheimer brain, and Congo-red positivity. Hereditary inclusion-body myopathy designates patients cytopathologically similar but without inflammation. In both muscle diseases, prion, and several proteins characteristic of Al...

  16. Childhood obesity.

    Han, Joan C; Lawlor, Debbie A; Kimm, Sue Y S

    2010-05-15

    Worldwide prevalence of childhood obesity has increased greatly during the past three decades. The increasing occurrence in children of disorders such as type 2 diabetes is believed to be a consequence of this obesity epidemic. Much progress has been made in understanding of the genetics and physiology of appetite control and from these advances, elucidation of the causes of some rare obesity syndromes. However, these rare disorders have so far taught us few lessons about prevention or reversal of obesity in most children. Calorie intake and activity recommendations need reassessment and improved quantification at a population level because of sedentary lifestyles of children nowadays. For individual treatment, currently recommended calorie prescriptions might be too conservative in view of evolving insight into the so-called energy gap. Although quality of research into both prevention and treatment has improved, high-quality multicentre trials with long-term follow-up are needed. Meanwhile, prevention and treatment approaches to increase energy expenditure and decrease intake should continue. Recent data suggest that the spiralling increase in childhood obesity prevalence might be abating; increased efforts should be made on all fronts to continue this potentially exciting trend. PMID:20451244

  17. Acute myelogenous leukemia switch lineage upon relapse to acute lymphoblastic leukemia: a case report

    Dorantes-Acosta, Elisa; Arreguin-Gonzalez, Farina; Rodriguez-Osorio, Carlos A; Sadowinski, Stanislaw; Pelayo, Rosana; Medina-Sanson, Aurora

    2009-01-01

    Acute leukemia, the most common form of cancer in children, accounts for approximately 30% of all childhood malignancies, with acute lymphoblastic leukemia being five times more frequent than acute myeloid leukemia. Lineage switch is the term that has been used to describe the phenomenon of acute leukemias that meet the standard French-American-British system criteria for a particular lineage (either lymphoid or myeloid) upon initial diagnosis, but meet the criteria for the opposite lineage a...

  18. Infection and childhood leukemia: review of evidence

    Raquel da Rocha Paiva Maia

    2013-12-01

    Full Text Available OBJECTIVE : To analyze studies that evaluated the role of infections as well as indirect measures of exposure to infection in the risk of childhood leukemia, particularly acute lymphoblastic leukemia. METHODS : A search in Medline, Lilacs, and SciELO scientific publication databases initially using the descriptors “childhood leukemia” and “infection” and later searching for the words “childhood leukemia” and “maternal infection or disease” or “breastfeeding” or “daycare attendance” or “vaccination” resulted in 62 publications that met the following inclusion criteria: subject aged ≤ 15 years; specific analysis of cases diagnosed with acute lymphoblastic leukemia or total leukemia; exposure assessment of mothers’ or infants’ to infections (or proxy of infection, and risk of leukemia. RESULTS : Overall, 23 studies that assessed infections in children support the hypothesis that occurrence of infection during early childhood reduces the risk of leukemia, but there are disagreements within and between studies. The evaluation of exposure to infection by indirect measures showed evidence of reduced risk of leukemia associated mainly with daycare attendance. More than 50.0% of the 16 studies that assessed maternal exposure to infection observed increased risk of leukemia associated with episodes of influenza, pneumonia, chickenpox, herpes zoster, lower genital tract infection, skin disease, sexually transmitted diseases, Epstein-Barr virus, and Helicobacter pylori . CONCLUSIONS : Although no specific infectious agent has been identified, scientific evidence suggests that exposure to infections has some effect on childhood leukemia etiology.

  19. How Are Childhood Cancers Found?

    ... Topic How are childhood cancers treated? How are childhood cancers found? Screening for childhood cancers Screening is testing for a disease such ... in people who don’t have any symptoms. Childhood cancers are rare, and there are no widely ...

  20. Risk Groups for Childhood Acute Lymphoblastic Leukemia

    ... leukemia may come back in the blood and bone marrow , brain, spinal cord , testicles , or other parts of the body. ... lymphoblastic leukemia (ALL) that comes back outside the bone marrow may include the ... to the brain and/or spinal cord for cancer that comes back in the ...

  1. Treatment Option Overview (Childhood Acute Lymphoblastic Leukemia)

    ... leukemia may come back in the blood and bone marrow , brain, spinal cord , testicles , or other parts of the body. ... lymphoblastic leukemia (ALL) that comes back outside the bone marrow may include the ... to the brain and/or spinal cord for cancer that comes back in the ...

  2. General Information about Childhood Acute Lymphoblastic Leukemia

    ... leukemia may come back in the blood and bone marrow , brain, spinal cord , testicles , or other parts of the body. ... lymphoblastic leukemia (ALL) that comes back outside the bone marrow may include the ... to the brain and/or spinal cord for cancer that comes back in the ...

  3. Treatment Options for Childhood Acute Lymphoblastic Leukemia

    ... leukemia may come back in the blood and bone marrow , brain, spinal cord , testicles , or other parts of the body. ... lymphoblastic leukemia (ALL) that comes back outside the bone marrow may include the ... to the brain and/or spinal cord for cancer that comes back in the ...

  4. Childhood medulloblastoma.

    Massimino, Maura; Biassoni, Veronica; Gandola, Lorenza; Garrè, Maria Luisa; Gatta, Gemma; Giangaspero, Felice; Poggi, Geraldina; Rutkowski, Stefan

    2016-09-01

    Medulloblastoma accounts for 15-20% of childhood nervous system tumours. The risk of dying was reduced by 30% in the last twenty years. Patients are divided in risk strata according to post-surgical disease, dissemination, histology and some molecular features such as WNT subgroup and MYC status. Sixty to 70% of patients older than 3 years are assigned to the average-risk group. High-risk patients include those with disseminated and/or residual disease, large cell and/or anaplastic histotypes, MYC genes amplification. Current and currently planned clinical trials will: (1) evaluate the feasibility of reducing both the dose of craniospinal irradiation and the volume of the posterior fossa radiotherapy (RT) for those patients at low biologic risk, commonly identified as those having a medulloblastoma of the WNT subgroup; (2) determine whether intensification of chemotherapy (CT) or irradiation can improve outcome in patients with high-risk disease; (3) find target therapies allowing tailored therapies especially for relapsing patients and those with higher biological risk. PMID:27375228

  5. Childhood Brain Tumors

    ... They are among the most common types of childhood cancers. Some are benign tumors, which aren't ... can still be serious. Malignant tumors are cancerous. Childhood brain and spinal cord tumors can cause headaches ...

  6. Childhood Brain Tumors

    Brain tumors are abnormal growths inside the skull. They are among the most common types of childhood ... still be serious. Malignant tumors are cancerous. Childhood brain and spinal cord tumors can cause headaches and ...

  7. Childhood Overweight and Obesity

    ... Childhood Obesity Facts The prevalence of obesity among low-income children aged 2 through 4 years, by state ... Obesity now affects 1 in 6 children and adolescents in the United States. Childhood Obesity Facts How ...

  8. Reducing Childhood Obesity

    ... Navigation Bar Home Current Issue Past Issues Reducing Childhood Obesity Past Issues / Summer 2007 Table of Contents For ... page please turn Javascript on. The We Can! childhood obesity-prevention program involves parents, caregivers, and community leaders ...

  9. Application of nuclear medicine to heart diseases in childhood

    Various procedures to obtain radioisotopic images of the cardiovascular system were described, and representative cases in childhood were presented. Apparatuses, radiopharmaceuticals such as sup(99m)Tc pertechnatate, 201Thallium, and 133Xenon gas, and their dosage in childhood were briefly reviewed. Several cases of persistent fetal circulation, common A-V valve, tetralogy of Fallot, pulmonary atresia, etc. were presented with figures with special emphasis on acute febrile mucocutaneous lymph node syndrome and 201Thallium myocardial imaging. (Kondo, M.)

  10. Application of nuclear medicine to heart diseases in childhood

    Tsuji, A. (Yamanashi Medical Coll., Tamaho, Kofu (Japan)); Osano, M.

    1981-06-01

    Various procedures to obtain radioisotopic images of the cardiovascular system were described, and representative cases in childhood were presented. Apparatuses, radiopharmaceuticals such as sup(99m)Tc pertechnatate, /sup 201/Thallium, and /sup 133/Xenon gas, and their dosage in childhood were briefly reviewed. Several cases of persistent fetal circulation, common A-V valve, tetralogy of Fallot, pulmonary atresia, etc. were presented with figures with special emphasis on acute febrile mucocutaneous lymph node syndrome and /sup 201/Thallium myocardial imaging.

  11. Domestic Radon and Childhood Cancer in Denmark

    Raaschou-Nielsen, Ole; Andersen, Claus Erik; Andersen, Helle P.;

    2008-01-01

    Background: Higher incidence rates of childhood cancer and particularly leukemia have been observed in regions with higher radon levels, but case-control studies have given inconsistent results. We tested the hypothesis that domestic radon exposure increases the risk for childhood cancer. Methods......: We identified 2400 incident cases of leukemia, central nervous system tumor, and malignant lymphoma diagnosed in children between 1968 and 1994 in the Danish Cancer Registry. Control children (n = 6697) were selected from the Danish Central Population Registry. Radon levels in residences of children...... and the cumulated exposure of each child were calculated as the product of exposure level and time, for each address occupied during childhood. Results: Cumulative radon exposure was associated with risk for acute lymphoblastic leukemia (ALL), with rate ratios of 1.21 (95% confidence interval = 0...

  12. AR-42 and Decitabine in Treating Patients With Acute Myeloid Leukemia

    2016-04-21

    Adult Acute Myeloid Leukemia With 11q23 (MLL) Abnormalities; Adult Acute Myeloid Leukemia With Del(5q); Adult Acute Myeloid Leukemia With Inv(16)(p13;q22); Adult Acute Myeloid Leukemia With t(15;17)(q22;q12); Adult Acute Myeloid Leukemia With t(16;16)(p13;q22); Adult Acute Myeloid Leukemia With t(8;21)(q22;q22); Recurrent Adult Acute Myeloid Leukemia; Recurrent Childhood Acute Myeloid Leukemia; Secondary Acute Myeloid Leukemia; Untreated Adult Acute Myeloid Leukemia

  13. An evaluation of the relationship between the quality of prophylactic cranial radiotherapy in childhood acute leukemia and institutional experience: a Quality Assurance Review Center-Pediatric Oncology Group study

    Purpose: The Pediatric Oncology Group Protocol 9404 was a prospective clinical trial of two forms of chemotherapy in childhood T-cell acute lymphoblastic leukemia and advanced stage T-cell lymphoblastic non-Hodgkin's lymphoma. The protocol called for prophylactic C1 whole brain external beam irradiation, 18 Gy in 2 Gy/fraction for 9 fractions. We hypothesized that a correlation would be found between the number of children irradiated on protocol by an institution and the compliance rate of that institution with radiotherapy quality assurance (QA) guidelines. We also hypothesized that QA compliance would improve as the study progressed. Methods and Materials: We scored the radiation dose as a minor deviation from protocol guidelines if the dose to the prescription point differed from the protocol by 6-10%, and a major deviation if it differed from protocol by >10%. Treatment volumes were scored as a minor deviation if the margins were less than specified or the fields were excessively large. A major deviation was defined as the transection of a potential leukemia-bearing volume such as would be caused by blocking the cribriform plate, optic nerve, or temporal lobe. When the treating physician submitted a treatment plan and simulator film at the initiation of therapy to the Quality Assurance Review Center (QARC), a rapid turn-around review of the plan and suggestions for improvement was provided. At the end of therapy, all simulator and port films were reviewed at the QARC. Results: We reviewed the data from 353 patients treated at 73 institutions in the United States, Canada, and Europe. Of these patients, 2% (n=7) were not assessable for QA because of incomplete information. Minor quality deviations were found in 27.7% of patients (n=98) and major deviations in 7.9% (n=28). The frequency of major deviations for institutions placing 1-4 patients on study was 11% vs. 5.5% for institutions placing ≥5 patients (p5 patients (p not significant). The frequency of major

  14. Causes of childhood leukaemia and lymphoma

    Childhood cancer is rare comprising less than 1% of all malignancies diagnosed each year in developed countries. Leukaemia is the commonest form of cancer in children accounting for around a third of all childhood cancer, with acute lymphoblastic leukaemia (ALL) being the most prevalent. Biologically specific subtypes of ALL and acute myeloblastic leukaemia (AML), the other major morphological type of childhood leukaemia, are characterised by chromosomal changes. Whilst over 200 genes have been associated with chromosomal translocations, to date, only MLL, TEL, and AML1 have been linked with childhood leukaemia. Interestingly, there is increasing evidence to support the theory that gene rearrangements such as these may originate in utero. As with many other human diseases, both genetic and environmental factors have been implicated in the aetiology of the disease. Although much has been documented with regard to diet, smoking, alcohol consumption and recreational and prescription drug use during pregnancy, there is no consistent evidence to support a link with any of these factors and childhood leukaemia. However, findings from studies investigating prenatal and early life exposures are often based on small numbers of cases as both the type of cancer and exposure are rare. Furthermore, accurate information relating to past exposures can be difficult to obtain and is often reliant on self-reporting. To further our understanding of the aetiology of childhood leukaemia and lymphoma, there are areas which clearly warrant investigation. These include collection of parental dietary folate data combined with genetic analysis of the folate related genes, in utero exposure to DNA topoisomerase II inhibitors, and the possible effects of assisted reproduction technology on disease susceptibility

  15. Analyses of karyotypic characteristics and prognosis in pediatric acute myeloblastic leukemia

    阮敏

    2012-01-01

    Objective Acute myeloblastic leukemia(AML) accounts for 15 to 25 percent of childhood acute leukemias. Cytogenetic information is important for diagnosis,classification and prognosis of AML. Our aim was to analyze the relationship between karyotypic characteristics and prognosis of childhood

  16. Immunity and infectious morbidity in childhood ALL treatment : the benefits of intensity reduction

    van Tilburg, C M

    2011-01-01

    With current childhood acute lymphoblastic leukemia (ALL) treatment protocols the cure rate approaches 90%. In the 10 percent of case fatalities, 2 major challenges stand out: incurable relapses of ALL and (infectious) deaths-in-remission. Thus, reducing toxicity is becoming an important goal to further improve childhood ALL survival. The Dutch Childhood Oncology Group (DCOG) ALL 10 protocol was designed to investigate whether a reduction of chemotherapeutic treatment intensity after a standa...

  17. Pharmacokinetics of 6-Thioguanine and 6-Mercaptopurine Combination Maintenance Therapy of Childhood ALL

    Nielsen, Stine N; Frandsen, Thomas L; Nersting, Jacob; Hjalgrim, Lisa L; Schmiegelow, Kjeld

    2015-01-01

    Methotrexate/6-mercaptopurine maintenance therapy of childhood acute lymphoblastic leukemia is challenged by treatment-related hepatotoxicity, failure to achieve the myelosuppressive target, and lack of direct parameters for monitoring treatment efficacy or even intensity. Patients with low...

  18. 婴儿出生史相关因素与儿童急性白血病发病风险的Meta分析%Effects of birth order, maternal abortion and mode of delivery on childhood acute leukemia risk: a meta-analysis

    邹国斌; 沙霞

    2014-01-01

    Objective To evaluate the associations between birth order,maternal abortion and mode of delivery and childhood acute leukemia risk.Method Multiple electronic databases were searched to identify relevant studies up to March 2013 using the search terms " childhood leukemia"," acute lymphoblastic leukemia"," acute myeloid leukemia"," birth order"," abortion "," miscarriage ","cesarean","birth characteristics" and "prenatal risk factor".Data from cohort and case-control studies were analyzed using the Stata software.Result Twenty-three studies were included in this meta-analysis according to the selection criteria.No significant associations were identified for birth order and mode of delivery (birth order =2:OR =0.97,95% CI:0.89-1.05 ; birth order =3:OR =1.00,95% CI:0.91-1.11 ; birth order ≥4:OR =1.02,95% CI:0.87-1.20; mode of delivery:OR =1.05,95% CI:0.96-1.15).However,there was a significant association between maternal abortion and childhood acute leukemia risk (spontaneous abortion:OR =1.21,95% CI:1.05-1.41 ; induced abortion:OR =1.23,95% CI:1.07-1.43).Furthermore,the stratified analysis by disease subtypes showed that spontaneous and induced abortions were significantly associated with the risks of childhood acute myeloid leukemia (OR =1.71,95% CI:1.09-2.70) and acute lymphoblastic leukemia (OR =1.23,95% CI:1.05-1.42),respectively.Conclusion This meta-analysis revealed that maternal abortion might contribute to the childhood acute leukemia risk.%目的 综合评价患儿出生顺序、孕妇流产史及分娩方式与儿童急性白血病发病风险之间的关系.方法 在PubMed、Google学术搜索、中国知网(CNKI)、万方数据库中以“childhoodleukemia"、“acute lymphoblastic leukemia"、“acute myeloid leukemia"、“birth order"、“abortion”、“miscarriage”、“caesarean”、“birth characteristics”、“prenatal risk factor”或其对应的中文词目为检索词,收集2013年3月前有关患儿出生顺序

  19. Molecular events linking cholesterol to Alzheimer’s disease and inclusion body myositis in a rabbit model

    Liu, Qing Yan; Koukiekolo, Roger; Zhang, Dong Ling; Smith, Brandon; Ly, Dao; Lei, Joy X; Ghribi, Othman

    2016-01-01

    Alzheimer’s disease (AD) is the most common neurodegenerative disorder, characterized by cognitive impairment and dementia, resulting from progressive synaptic dysfunction, loss and neuronal cell death. Inclusion body myositis (IBM) is a skeletal muscle degenerative disease, displaying progressive proximal and distal muscle weakness, in association with muscle fiber atrophy, degeneration and death. Studies have shown that the late onset version of AD (LOAD) and sporadic IBM (sIBM) in muscle share many pathological features, including the presence of extracellular plaques of β-amyloid peptides and intracellular tangles of hyperphosphorylated tau proteins. High blood cholesterol is suggested to be a risk factor for LOAD. Many neuropathological changes of LOAD can be reproduced by feeding rabbits a 2% enriched cholesterol diet for 12 weeks. The cholesterol fed rabbit model also simultaneously develops sIBM like pathology, which makes it an ideal model to study the molecular mechanisms common to the development of both diseases. In the present study, we determined the changes of gene expression in rabbit brain and muscle during the progression of LOAD and sIBM pathology using a custom rabbit nucleotide microarray, followed by qRT-PCR analyses. Out of 869 unique transcripts screened, 47 genes showed differential expression between the control and the cholesterol-treated group during the 12 week period and 19 changed transcripts appeared to be common to LOAD and sIBM. The most notable changes are the upregulation of the hemoglobin gene family and the downregulation of the genes required for mitochondrial oxidative phosphorylation in both brain and muscle tissues throughout the time course. The significant overlap on the changes of gene expression in the brain and muscle of rabbits fed with cholesterol-enriched diet supports the notion that LOAD and sIBM may share a common etiology. PMID:27073745

  20. Emergency presentation and management of acute severe asthma in children

    Øymar Knut; Halvorsen Thomas

    2009-01-01

    Abstract Acute severe asthma is one of the most common medical emergency situations in childhood, and physicians caring for acutely ill children are regularly faced with this condition. In this article we present a summary of the pathophysiology as well as guidelines for the treatment of acute severe asthma in children. The cornerstones of the management of acute asthma in children are rapid administration of oxygen, inhalations with bronchodilators and systemic corticosteroids. Inhaled bronc...

  1. Childhood trauma and childhood urbanicity in relation to psychotic disorder

    Frissen, Aleida; Lieverse, Ritsaert; Drukker, Marjan; van Winkel, Ruud; Delespaul, Philippe; Cahn, W

    2015-01-01

    BACKGROUND: Urban upbringing and childhood trauma are both associated with psychotic disorders. However, the association between childhood urbanicity and childhood trauma in psychosis is poorly understood. The urban environment could occasion a background of social adversity against which any effect

  2. Acute Bronchitis

    ... of bronchitis: acute and chronic. Most cases of acute bronchitis get better within several days. But your cough ... that cause colds and the flu often cause acute bronchitis. These viruses spread through the air when people ...

  3. Childhood as a value

    EWELINA PIECUCH

    2011-01-01

    The article encompasses the problems of childhood and its influence on the rest of one's life. I have concentrated on this crucial and specific time in life. It is demonstrated by biology, medicine, psychology, and psychoanalysis that human habits are formed in childhood. Health, hygiene and aesthetic behaviour determine one's further fate and influence life in its entirety. It is that phase of human life that determines the rest of it. In childhood children manifest their cogn...

  4. Biological features of dendritic cells derived from childhood B lineage acute lymphoblastic leukemia in vitro%儿童B系急性淋巴细胞白血病树突状细胞的生物学特性

    庄泳; 李栋; 付金秋; 时庆; 鞠秀丽

    2014-01-01

    目的:体外诱导培养正常儿童与B系急性淋巴细胞白血病(B-ALL)儿童的树突状细胞(DC),比较二者的生物学特性。方法分离10例B-ALL初诊患儿(ALL组)和10例正常儿童(对照组)的外周血单个核细胞,以rhGM-CSF(20 ng/mL)、rhIL-4(10 ng/mL)及TNF-α(10 ng/mL)联合培养8 d,显微镜下观察细胞形态,流式细胞仪检测细胞免疫表型(CD11c、CD80、CD83、CD86),ELISA检测培养上清中 IL-12的浓度,混合淋巴细胞反应(MLR)检测抗原递呈功能,电化学法检测上清中葡萄糖浓度。结果 ALL组细胞未表现出 DC 的典型形态, CD11c、CD80、CD83、CD86表达均较对照组细胞低(P<0.05),分泌IL-12的能力弱于对照组细胞(P<0.05),刺激T淋巴细胞增殖的能力弱于对照组细胞,ALL组细胞培养上清中的葡萄糖浓度高于对照组(P<0.05)。结论B-ALL 来源的DC 成熟度异常,功能减弱,葡萄糖代谢异常可能是其成熟异常的原因之一。%Objective To induce and culture dendritic cells (DC)derived from normal children and childhood B line-age acute lymphoblastic leukemia (B-ALL)patients,and compare their biological features in vitro.Methods Periph-eral blood mononuclear cells (PBMCs)were isolated from 10 B-ALL patients (ALL group)and 10 healthy donors (control group).The isolated PBMCs were co-cultured with rhGM-CSF(20 ng/mL),rh IL-4(10 ng/mL)and TNF-α(10 ng/mL)for 8 days.The morphological features were observed by inverted microscope.CD11c,CD80,CD83, CDD86 expressions were assayed by flow cytometry.The concentration of IL-12 was measured by ELISA.The antigen presenting function of the cells were tested by mixed lymphocyte reaction(MLR).Electrochemical measurement was used for the detection of glucose metabolism.Results The typical DC morphological features were not observed from the cells in ALL group.The cells in ALL group expressed lower levels of CD11c,CD83,CD

  5. A single center, prospective study on the epidemiology of acute childhood poisoning%儿童急性中毒流行病学单中心前瞻性研究

    曹雪笛; 高恒妙; 廖琨; 王新伟; 王龙; 赵光远; 霍枫; 钱素云

    2015-01-01

    目的 分析儿童急性中毒的流行病学特征,探讨预防儿童急性中毒的方法.方法 应用儿童中毒病例信息收集表前瞻性收集2013年1月1日至12月31日就诊于北京儿童医院中毒患儿的流行病学资料,分析儿童急性中毒的年龄、原因、途径、种类和后果.结果 共收集中毒病例353例.<1岁28例(8.0%),~4岁255例(72.2%),~9岁35例(9.9%),≥9岁35例(9.9%).中毒发生在家庭305例(86.4%),家庭以外48例(13.6%).经消化道中毒348例(98.5%),吸入5例(1.5%).患儿自己误服281例(79.4%),家长喂错药或超量喂药48例(13.6%),医源性7例(2.1%),试图自杀12例(3.4%).药物中毒206例(58.4%),化学制剂55例(15.6%),鼠药26例(7.3%),农药45例(12.7%),有毒植物16例(4.5%).353例中,急诊留观治疗297例(84.1%),因病情危重住PICU 56例(15.9%),死亡4例(1.1%).结论 中毒好发年龄为1~4岁,主要原因为误服,毒物种类第一位是药物,导致死亡或严重中毒的种类包括精神类药物、农药、鼠药.预防工作重点为防止幼儿家中误服,主要措施有加强儿童监管、妥善保管家中药物及有毒物品、鼓励使用药品安全包装.%Objective To analyze the epidemiological characteristics of acute childhood poisoning and to explore the methods of prevention.Methods The epidemiological information of each poisoning child presented to Emergency Department, Beijing Children's Hospital, Capital Medical University from Jan 1 st to Dec 31st 2013 were collected with an information collection table.The children's age, causes of poisoning, poison types, ways of poisoning and the consequences of poisoning were analyzed.Results A total of 353 poisoning children presented to our emergency department in 2013.Twenty-eight patients (8.0%) were < 1 year-old.Two hundred and fifty-five patients(72.2%) were between 1 to < 4 year-old.Thirty-five patients (9.9%) were between 4 to <9 year

  6. Childhood Cancer Survivor Study: An Overview

    ... Cancers of Childhood Treatment Childhood Cancer Genomics Research Childhood Cancer Survivor Study: An Overview In 2016, it ... Late Effects of Treatment for Childhood Cancer .) The Childhood Cancer Survivor Study ( CCSS ), funded by the National ...

  7. Cerebral venous thrombosis in childhood

    Huisman, T.A.G.M.; Martin, E.; Willi, U.V. [Dept. of Diagnostic Imaging and Radiology, University Children' s Hospital Zurich (Switzerland); Holzmann, D. [Dept. of Otorhinolaryngology, University Children' s Hospital Zurich, Zurich (Switzerland)

    2001-09-01

    This was a retrospective study to determine different etiologies of cerebral venous thrombosis (CVT) in childhood and to correlate extent and location of thrombosis with the etiology and the age of the child as well as the final outcome. In addition, the radiologic approach is discussed. This was a retrospective analysis of 19 children with CVT. The children were examined by contrast-enhanced dynamic CT. Radiologic findings were correlated with the etiology of CVT. Cerebral venous thrombosis is not as infrequent in children as has been thought. Cerebral venous thrombosis in children can occur due to trauma (n=9), infections (n=7), or coagulation disorders (n=3). Extent and location of thrombosis, as well as complications, final outcome, and therapy, depend on the etiology. Computed tomography remains a valuable primary imaging modality in the diagnosis of CVT in the acutely injured or diseased child. (orig.)

  8. Cerebral venous thrombosis in childhood

    This was a retrospective study to determine different etiologies of cerebral venous thrombosis (CVT) in childhood and to correlate extent and location of thrombosis with the etiology and the age of the child as well as the final outcome. In addition, the radiologic approach is discussed. This was a retrospective analysis of 19 children with CVT. The children were examined by contrast-enhanced dynamic CT. Radiologic findings were correlated with the etiology of CVT. Cerebral venous thrombosis is not as infrequent in children as has been thought. Cerebral venous thrombosis in children can occur due to trauma (n=9), infections (n=7), or coagulation disorders (n=3). Extent and location of thrombosis, as well as complications, final outcome, and therapy, depend on the etiology. Computed tomography remains a valuable primary imaging modality in the diagnosis of CVT in the acutely injured or diseased child. (orig.)

  9. Oral zinc and common childhood infections--An update.

    Basnet, Sudha; Mathisen, Maria; Strand, Tor A

    2015-01-01

    Zinc is an essential micronutrient important for growth and for normal function of the immune system. Many children in developing countries have inadequate zinc nutrition. Routine zinc supplementation reduces the risk of respiratory infections and diarrhea, the two leading causes of morbidity and mortality in young children worldwide. In childhood diarrhea oral zinc also reduces illness duration and risk of persistent episodes. Oral zinc is therefore recommended for the treatment of acute diarrhea in young children. The results from the studies that have measured the therapeutic effect of zinc on acute respiratory infections, however, are conflicting. Moreover, the results of therapeutic zinc for childhood malaria also are so far not promising.This paper gives a brief outline of the current evidence from clinical trials on therapeutic effect of oral zinc on childhood respiratory infections, pneumonia and malaria and also of new evidence of the effect on serious bacterial illness in young infants. PMID:24906347

  10. Prematurity and prescription asthma medication from childhood to young adulthood

    Damgaard, Anne Louise; Hansen, Bo Mølholm; Mathiasen, René; Buchvald, Frederik; Lange, Theis; Greisen, Gorm

    2015-01-01

    INTRODUCTION: Preterm birth is associated with increased risk of asthma-like symptoms and purchase of prescription asthma medication in childhood. We investigated whether this association persists into adulthood and whether it is affected by accounting for neonatal respiratory morbidity (acute re...

  11. Childhood Obesity. ERIC Digest.

    Summerfield, Liane M.

    In this discussion of childhood obesity, the medical and psychological problems associated with the condition are noted. Childhood obesity most likely results from an interaction of nutritional, psychological, familial, and physiological factors. Three factors--the family, low-energy expenditure, and heredity--are briefly examined. Early…

  12. Reframing Early Childhood Leadership

    Stamopoulos, Elizabeth

    2012-01-01

    Rapid changes in Australian education have intensified the role of early childhood leaders and led to unprecedented challenges. The Australian Curriculum (ACARA, 2011), mandated Australian "National Quality Framework" (NQF) for Early Childhood Education & Care (DEEWR, 2010b) and the "National Early Years Learning Framework" (EYLF) (DEEWR, 2009)…

  13. Late endocrine effects of childhood cancer.

    Rose, Susan R; Horne, Vincent E; Howell, Jonathan; Lawson, Sarah A; Rutter, Meilan M; Trotman, Gylynthia E; Corathers, Sarah D

    2016-06-01

    The cure rate for paediatric malignancies is increasing, and most patients who have cancer during childhood survive and enter adulthood. Surveillance for late endocrine effects after childhood cancer is required to ensure early diagnosis and treatment and to optimize physical, cognitive and psychosocial health. The degree of risk of endocrine deficiency is related to the child's sex and their age at the time the tumour is diagnosed, as well as to tumour location and characteristics and the therapies used (surgery, chemotherapy or radiation therapy). Potential endocrine problems can include growth hormone deficiency, hypothyroidism (primary or central), adrenocorticotropin deficiency, hyperprolactinaemia, precocious puberty, hypogonadism (primary or central), altered fertility and/or sexual function, low BMD, the metabolic syndrome and hypothalamic obesity. Optimal endocrine care for survivors of childhood cancer should be delivered in a multidisciplinary setting, providing continuity from acute cancer treatment to long-term follow-up of late endocrine effects throughout the lifespan. Endocrine therapies are important to improve long-term quality of life for survivors of childhood cancer. PMID:27032982

  14. Averting the legacy of kidney disease - focus on childhood

    J.R. Ingelfinger

    2016-01-01

    Full Text Available World Kidney Day 2016 focuses on kidney disease in childhood and the antecedents of adult kidney disease that can begin in earliest childhood. Chronic kidney disease (CKD in childhood differs from that in adults, in that the largest diagnostic group among children includes congenital anomalies and inherited disorders, with glomerulopathies and kidney disease as a consequence of diabetes being relatively uncommon. In addition, many children with acute kidney injury will ultimately develop sequelae that may lead to hypertension and CKD in later childhood or in adult life. Children born early or who are small-for-date newborns have relatively increased risk for the development of CKD later in life. Persons with a high-risk birth and early childhood history should be watched closely in order to help detect early signs of kidney disease in time to provide effective prevention or treatment. Successful therapy is feasible for advanced CKD in childhood; there is evidence that children fare better than adults, if they receive kidney replacement therapy including dialysis and transplantation, although only a minority of children may require this ultimate intervention. Because there are disparities in access to care, effort is needed so that children with kidney disease, wherever they live, may be treated effectively, irrespective of their geographic or economic circumstances. Our hope is that the World Kidney Day will inform the general public, policy makers and caregivers about the needs and possibilities surrounding kidney disease in childhood.

  15. Averting the legacy of kidney disease – focus on childhood

    Julie R. Ingelfinger

    2016-03-01

    Full Text Available World Kidney Day 2016 focuses on kidney disease in childhood and the antecedents of adult kidney disease that can begin in earliest childhood. Chronic kidney disease (CKD in childhood differs from that in adults, as the largest diagnostic group amongst children includes congenital anomalies and inherited disorders, with glomerulopathies and kidney disease in the setting of diabetes being relatively uncommon. In addition, many children with acute kidney injury will ultimately develop sequelae that may lead to hypertensionand CKD in later childhood or in adult life. Children born early or who are small-for-date newborns have relatively increased risk for the development of CKD later in life. Persons with a high-risk birth and early childhood history should be watched closely to help to detect early signs of kidney disease in time to provide effective prevention or treatment. Successful therapy is feasible for advanced CKD in childhood; there is evidence that children fare better than adults, if they receive kidney replacement therapy including dialysis and transplantation, whilst only a minority of children may require this ultimate intervention. Because there are disparities in access to care, effort is needed so that children with kidney disease, wherever they live, may be treated effectively, irrespective of their geographic oreconomic circumstances. Our hope is that World Kidney Day will inform the general public, policymakers and caregivers about the needs and possibilities surrounding kidney disease in childhood.

  16. Averting the Legacy of Kidney Disease - Focus on Childhood.

    Ingelfinger, Julie R; Kalantar-Zadeh, Kamyar; Schaefer, Franz

    2016-04-01

    World Kidney Day 2016 focuses on kidney disease in childhood and the antecedents of adult kidney disease that can begin in earliest childhood. Chronic kidney disease (CKD) in childhood differs from that in adults, as the largest diagnostic group among children includes congenital anomalies and inherited disorders, with glomerulopathies and kidney disease in the setting of diabetes being relatively uncommon. In addition, many children with acute kidney injury will ultimately develop sequelae that may lead to hypertension and CKD in later childhood or in adult life. Children born early or who are small-for date newborns have relatively increased risk for the development of CKD later in life. Persons with a high-risk birth and early childhood history should be watched closely in order to help detect early signs of kidney disease in time to provide effective prevention or treatment. Successful therapy is feasible for advanced CKD in childhood; there is evidence that children fare better than adults, if they receive kidney replacement therapy including dialysis and transplantation, while only a minority of children may require this ultimate intervention. Because there are disparities in access to care, effort is needed so that those children with kidney disease, wherever they live, may be treated effectively, irrespective of their geographic or economic circumstances. Our hope is that World Kidney Day will inform the general public, policy makers and caregivers about the needs and possibilities surrounding kidney disease in childhood. PMID:27536691

  17. A childhood acute lymphoblastic leukemia (ALL case with t(3;17(q23;p13,t(5;12(q31;p13,inv(11(p15q12

    Ayse Cirakoglu

    2008-09-01

    Full Text Available It is known that clonal chromosomal changes in childhood ALL are nonrandom and important markers for diagnosis, prognosis and relaps. In this report we present 4 year-old boy with ALL-L1 who has complex chromosomal rearrangements. Chromosome analysis was performed on bone marrow aspiration sample in relaps after one year from diagnosis and induction chemotherapy. The karyotype was; 46,XY,t(3;17(q23;p13,t(5;12(q31;p13,inv(11(p15q12 [11]/46,XY[8

  18. Individualized toxicity-titrated 6-mercaptopurine increments during high-dose methotrexate consolidation treatment of lower risk childhood acute lymphoblastic leukaemia. A Nordic Society of Paediatric Haematology and Oncology (NOPHO) pilot study

    Frandsen, Thomas L; Abrahamsson, Jonas; Lausen, Birgitte;

    2011-01-01

    This study explored the feasibility and toxicity of individualized toxicity-titrated 6-mercaptopurine (6MP) dose increments during post-remission treatment with High-dose methotrexate (HDM) (5000 mg/m(2), ×3) in 38 patients with Childhood (ALL). Patients were increased in steps of 25 mg 6MP/m(2......) per day if they did not develop myelotoxicity within 2 weeks after HDM. 6MP could be increased in 31 patients (81%). Toxicity was acceptable and did not differ significantly between groups. Patients receiving 75 mg/m(2) per day had significantly shorter duration of treatment interruptions of 6MP than...

  19. Childhood trauma and childhood urbanicity in relation to psychotic disorder

    Frissen, Aleida; Lieverse, Ritsaert; Drukker, Marjan; van Winkel, Ruud; Delespaul, Philippe; [...

    2015-01-01

    Background Urban upbringing and childhood trauma are both associated with psychotic disorders. However, the association between childhood urbanicity and childhood trauma in psychosis is poorly understood. The urban environment could occasion a background of social adversity against which any effect of childhood trauma increases. Also, any impact of the urban environment on likelihood of exposure to childhood trauma could be stronger in children who later develop psychotic disorder. The aim of...

  20. Childhood trauma and childhood urbanicity in relation to psychotic disorder

    Frissen, Aleida; Lieverse, Ritsaert; Drukker, Marjan; van Winkel, Ruud; Delespaul, Philippe; Cahn, W.

    2015-01-01

    BACKGROUND: Urban upbringing and childhood trauma are both associated with psychotic disorders. However, the association between childhood urbanicity and childhood trauma in psychosis is poorly understood. The urban environment could occasion a background of social adversity against which any effect of childhood trauma increases. Also, any impact of the urban environment on likelihood of exposure to childhood trauma could be stronger in children who later develop psychotic disorder. The aim o...

  1. Acute Lymphoblastic Leukemia (ALL) (For Parents)

    ... of WBC) are produced, a child will develop acute lymphoblastic, or lymphoid, leukemia (ALL). This is the most common type of childhood leukemia, affecting about 75% of kids with this cancer of the blood cells. Kids ... (AML) Chronic Myelogenous Leukemia (CML) Cancer ...

  2. Antibiotics for acute otitis media in children

    Venekamp, Roderick P.; Sanders, Sharon L.; Glasziou, Paul P.; Del Mar, Chris B.; Rovers, Maroeska M.

    2015-01-01

    BACKGROUND: Acute otitis media (AOM) is one of the most common diseases in early infancy and childhood. Antibiotic use for AOM varies from 56% in the Netherlands to 95% in the USA, Canada and Australia. This is an update of a Cochrane review first published in The Cochrane Library in Issue 1, 1997 a

  3. Acute Pancreatitis and Pregnancy

    ... Acute Pancreatitis > Acute Pancreatitis and Pregnancy test Acute Pancreatitis and Pregnancy Timothy Gardner, MD Acute pancreatitis is ... of acute pancreatitis in pregnancy. Reasons for Acute Pancreatitis and Pregnancy While acute pancreatitis is responsible for ...

  4. Childhood cancer in Africa.

    Kruger, Mariana; Hendricks, Marc; Davidson, Alan; Stefan, Cristina D; van Eyssen, Ann L; Uys, Ronelle; van Zyl, Anel; Hesseling, Peter

    2014-04-01

    The majority of children with cancer live in low- and middle-income countries (LMICs) with little or no access to cancer treatment. The purpose of the paper is to describe the current status of childhood cancer treatment in Africa, as documented in publications, dedicated websites and information collected through surveys. Successful twinning programmes, like those in Malawi and Cameroon, as well as the collaborative clinical trial approach of the Franco-African Childhood Cancer Group (GFAOP), provide good models for childhood cancer treatment. The overview will hopefully influence health-care policies to facilitate access to cancer care for all children in Africa. PMID:24214130

  5. Childhood Ataxia with Cerebral Hypomyelination Syndrome: a Variant of Patient with Early Childhood Onset Related to EIF2B3 Mutation. A Case Report.

    Perfetto, F; Stoppino, L P; Calì, A; Milillo, P; Grilli, G; Vinci, R; Macarini, L

    2012-03-01

    Childhood ataxia with central nervous system hypomyelination (CACH) syndrome is an autosomal recessive transmitted leukodystrophy characterised by early childhood onset and acute deterioration following febrile illnesses or head trauma. We describe the case of a child with early onset of CACH syndrome. He presented with cerebellar ataxia beginning around two years of age with mild mental retardation. MRI showed diffuse white matter signal changes with thinning of the corpus callosum. PMID:24028880

  6. Inflammatory Myopathies (Myositis)

    ... cell counts must be monitored; increases risk of cancer. methotrexate (Rheumatrex, Interferes with proliferation of B-cells and ... and B-cells (“upstream” of azathio- prine and methotrexate ... in higher doses to treat cancer. Doesn’t affect production of cells other than ...

  7. Establishment and validation of a standard protocol for the detection of minimal residual disease in B lineage childhood acute lymphoblastic leukemia by flow cytometry in a multi-center setting;

    Irving, Julie; Jesson, Jenny; Virgo, Paul; Case, Marian; Minto, Lynne; Eyre, Lisa; Noel, Nigel; Johansson, Ulrika; Macey, Marion; Knotts, Linda; Helliwell, Margaret; Davies, Paul; Whitby, Liam; Barnett, David; Hancock, Jeremy

    2009-01-01

    Minimal residual disease detection, used for clinical management of children with acute lymphoblastic leukemia, can be performed by molecular analysis of antigen-receptor gene rearrangements or by flow cytometric analysis of aberrant immunophenotypes. For flow minimal residual disease to be incorporated into larger national and international trials, a quality assured, standardized method is needed which can be performed in a multi-center setting. We report a four color, flow cytometric protoc...

  8. Improving the treatment of severe acute malnutrition in childhood: a randomized controlled trial of synbiotic-enhanced therapeutic food with long term follow-up of post-treatment mortality and morbidity

    Kerac, M

    2011-01-01

    BACKGROUND: Tackling severe acute malnutrition (SAM) is a global public health priority. This thesis explores two major influences on treatment outcomes: -Treatment efficacy -Patient-related risk factors OBJECTIVES: 1. To explore whether a pre/probiotic mixture (Synbiotic2000 Forte™) improves treatment outcomes (nutritional and clinical) in children affected by SAM. 2. To describe long term outcomes from SAM and identify key mortality risk factors. METHODS: All 1024 malno...

  9. Molecular targeting in childhood malignancies using nanoparticles

    Satake, Noriko; Barisone, Gustavo; Diaz, Elva; Nitin, Nitin; Nolta, Jan; Lam, Kit

    2012-06-01

    The goal of our project is to develop a new therapy for childhood malignancies using nanoformulated siRNA targeting Mxd3, a molecule in the Sonic Hedgehog signaling pathway, which we believe is important for cell survival. We plan to use cancer-specific ligands and superparamagnetic iron oxide nanoparticles (SPIO NPs) to carry siRNA. This delivery system will be tested in mouse xenograft models that we developed with primary cancer tissues. Our current focus is acute lymphoblastic leukemia (ALL), the most common cancer in children. We report our progress to date.

  10. Bronchitis - acute

    ... sharing features on this page, please enable JavaScript. Acute bronchitis is swelling and inflammation in the main passages ... present only for a short time. Causes When acute bronchitis occurs, it almost always comes after having a ...

  11. Bronchitis - acute

    Acute bronchitis is swelling and inflammation in the main passages that carry air to the lungs. The swelling narrows ... makes it harder to breathe. Another symptom of bronchitis is a cough. Acute means the symptoms have ...

  12. Acute Bronchitis

    Bronchitis is an inflammation of the bronchial tubes, the airways that carry air to your lungs. It ... chest tightness. There are two main types of bronchitis: acute and chronic. Most cases of acute bronchitis ...

  13. Cardiovascular Conditions of Childhood

    ... This childhood illness can result in long-term heart complications. Learn the symptoms, diagnosis and treatment for Kawasaki disease. Rheumatic Fever This inflammatory infection can occur after strep ...

  14. Reducing Childhood Obesity

    ... Bar Home Current Issue Past Issues Reducing Childhood Obesity Past Issues / Summer 2007 Table of Contents For ... Ga. were the first three We Can! cities. Obesity Research: A New Approach The percentage of children ...

  15. Childhood Immunization Schedule

    ... Why Immunize? Vaccines: The Basics Instant Childhood Immunization Schedule Recommend on Facebook Tweet Share Compartir Get the ... See Disclaimer for additional details. Based on Immunization Schedule for Children 0 through 6 Years of age ...

  16. Childhood vitiligo: Treatment paradigms

    Amrinder Jit Kanwar

    2012-01-01

    Full Text Available Childhood vitiligo differs from the adults by showing a higher incidence in females, segmental vitiligo being more common and less frequent association with other systemic autoimmune and endocrine disorders.Childhood vitiligo is often associated with a marked psychosocial and long lasting effect on the self-esteem of the affected children and their parents, hence an adequate treatment is very essential. Treatment of vitiligo is indeed a tough challenge for the dermatologists′ more so in the background of childhood vitiligo. Although multiple therapeutic modalities are available in the therapeutic armamentarium, not all can be used in children. This brief report updates regarding various therapies available in the treatment of childhood vitiligo.

  17. Childhood Vaccine Schedule

    ... Navigation Bar Home Current Issue Past Issues Childhood Vaccine Schedule Past Issues / Spring 2008 Table of Contents ... please turn Javascript on. When to Vaccinate What Vaccine Why Birth (or any age if not previously ...

  18. Tooth decay - early childhood

    ... Ribeiro NM, Ribeiro MA. Breastfeeding and early childhood caries: a critical review. J Pediatr (Rio J) . 2004;80:S199-S210. Sexton S, Natale R. Risks and benefits of pacifiers. Am Fam Physician . 2009; ...

  19. Acute pancreatitis

    Bo-Guang Fan; Åke Andrén-Sandberg

    2010-01-01

    Background : Acute pancreatitis continues to be a serious illness, and the patients with acute pancreatitis are at risk to develop different complications from ongoing pancreatic inflammation. Aims : The present review is to highlight the classification, treatment and prognosis of acute pancreatitis. Material & Methods : We reviewed the English-language literature (Medline) addressing pancreatitis. Results : Acute pancreatitis is frequently caused by gallstone disease or excess alcohol ingest...

  20. Acute pancreatitis

    Bo-Guang Fan; Åke Andrén-Sandberg

    2010-01-01

    Background: Acute pancreatitis continues to be a serious illness, and the patients with acute pancreatitis are at risk to develop different complications from ongoing pancreatic inflammation. Aims: The present review is to highlight the classification, treatment and prognosis of acute pancreatitis. Material & Methods: We reviewed the English-language literature (Medline) addressing pancreatitis. Results: Acute pancreatitis is frequently caused by gallstone disease or excess alcohol ingestion....