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Mardawig Alebouyeh 《Pediatric hematology and oncology》2004,22(1):1-9
Pediatric hematology and oncology (PHO) is a rapidly expanding field. It has been our goal to meet the needs and increasing demands for comprehensive medical care of children suffering from chronic blood diseases and malignancies. In the past decade we have been able expand and optimize the PHO services throughout Iran, in general and in respect to their prevalence and clinical importance, by trained pediatric hematologist-oncologists, pediatric surgeons and improved para clinical facilities. Major beta-thalassemics receive blood transfusion and chelation therapy according to the current standards mostly at regional blood banks centers. To curb major beta-thalassemia a premarital screening program has been enacted and abortion has been legitimized if major thalassemia is diagnosed by CVS. Hemophiliacs are supervised and treated as indicated by Iranian Hemophilia Comprehensive Care Centers (IHCCC). Screening for transfusion related complications and transmitted viral diseases (HBV, HCV and HIV) in both cohorts are carried out in regular intervals and necessary management will be then carried out as indicated at respective specialized units. Childhood malignancies are treated according to protocols adopted from accredited institutions in the USA and Europe, with almost comparable results. BMT is available for selected patients with beta-thalassemia or malignancies. By going to public we have been able to rise general awareness about chronic blood diseases and childhood malignancies and have initiated establishment of parents groups and formation of NGOs to support these children and their families. Foundation of Iranian Society of Pediatric Hamatology and Oncology (ISPHO) in the year 2000 has been another step forward to consolidate and coordinate the available manpower and facilities.
Conclusion: By evaluation of the country's main problems and shortcomings and conduction of collaborative studies and operation planning one will succeed to get the expected feedback and scientifically based approach to provide medical services to far reaching underdeveloped regions. 相似文献
Conclusion: By evaluation of the country's main problems and shortcomings and conduction of collaborative studies and operation planning one will succeed to get the expected feedback and scientifically based approach to provide medical services to far reaching underdeveloped regions. 相似文献
3.
Price VE Zielenska M Chilton-MacNeill S Smith CR Pappo AS 《Pediatric blood & cancer》2005,45(1):20-24
BACKGROUND: To describe the clinical characteristics, molecular features, treatment, and outcome of six pediatric patients with gastrointestinal stromal tumors (GISTs). PATIENTS AND METHODS: Retrospective clinical review of GISTs, seen at The Hospital for Sick Children (HSC) Toronto, over an 11-year period. All specimens were stained for the CD 117 and CD 34 antigens. Three specimens were sequenced for mutations in exons 9, 11, and 13 of the c-kit gene. RESULTS: Five patients were evaluated and treated at HSC and one was referred for histopathological consultation only. The median patient age at diagnosis was 13.6 years, (6.9-14.8 years); four were female. All patients presented with anemia secondary to gastrointestinal (GI) bleeding. The disease was localized in five patients and two had other malignancies consistent with the diagnoses of Carney's triad. Immunohistochemical staining for CD 117 and CD 34 showed heavy cytoplasmic localization in all of the tumor cells. A novel point mutation of KIT in codon 456 of exon 9 was found in one case. Complete surgical resection was achieved in the five patients managed at our center and none received adjuvant therapies. Disease recurred locally in one patient. Four patients are alive and one is lost to follow-up. CONCLUSIONS: In children and adolescents, GISTs should be considered in the differential diagnosis of anemia secondary to GI hemorrhage. The absence of an exon 11 mutation and the identification of a novel mutation in exon 9 suggest that pediatric GISTs may respond differently to currently available targeted therapies and therefore should be studied within the context of collaborative group trials. 相似文献
4.
《Pediatric hematology and oncology》2013,30(8):636-640
In childhood mature cystic teratomas are the most common type of ovarian germ cell tumors. Tumors of neuroepithelial origin are extremely rare in mature teratomas. To the authors’ knowledge, this is the first case report oligodendroglioma arising in a mature cystic ovarian teratoma in children. A 13-year-old girl presented with a history of pelvic pain for 2 months. An ultrasound examination showed a monolateral multicystic, calcific ovarian mass, measuring approximately 6 × 5 cm, arising in the left over and adnexia, suggesting a teratoma. An exploratorative laparotomy and ooferectomy were performed. On histopatological examination, various samples from cystic areas had mature tissues from all 3 germ cell layers, including skin, bone, bronchial structures, and cerebellum. Sections from solid areas showed uniform population of monotonous, rounded cells with indistinct cytoplasm and having perinuclear halo (fried-egg appearance) on a fibrillary, neuropilic background. Microcalcifications as calcospherites were noted in the stroma. Based on the typical morphological features, the cased was diagnosed as mature cystic teratoma with low-grade oligodendroglioma. To the authors’ knowledge, there are a few reports in the literature of an oligodendroglioma arising in an ovarian teratoma in adults and this presented patient is the first case in childhood. 相似文献
5.
V. V. Joshi 《Indian journal of pediatrics》1994,61(5):497-512
AIDS in children is a multisystem disease. The various infections, degenerative, proliferative and vascular lesions can be
classified into three categories based on the known, presumed or undetermined pathogenesis. The primary lesions are due to
HIV infection. The associated lesions are related to direct or indirect sequelae of HIV infection or its treatment. The third
category is of lesions of undetermined pathogenesis. The pediatric pathologist plays an important role in the study and management
of AIDS by demonstrating new pathologic lesions, by making the etiologic diagnosis of infection in children with AIDS, and
by providing clinicopathologic correlation which leads to better understanding of the disease process and its natural history.
Diagnosis of neoplastic disorders is also made by the pathologist. There is a dearth of systematic pathologic study of AIDS
in children in developing countries. Although no basic differences between pathologic lesions in pediatric AIDS in Western
countries, and in developing countries is expected, such a study would lead to better understanding and better management
of the disorder as it affects children from the developing countries. 相似文献
6.
Ricardo Drut M. Alba Greco Carmen Gutierrez Beatriz de Leon-Bojorge Dora Menezes Armando Peruga Graciela Quijano Cecilia Ridaura Monica Siminovich Pedro Valencia Mercedes Weissenbacher 《Fetal and pediatric pathology》1997,17(4):569-576
The present report describes opportunistic infections found at 74 autopsies of pediatric HIV AIDS patients performed at several hospitals in Latin American countries. Fungal infections were the most common (53 cases), Candida sp. (39.18 ) and Pneumocystis carinii (20.27 ) being the most frequently recognized. Other fungal diseases included histoplasmosis, aspergillosis, and cryptococcosis. Viral infections were present in 31 cases, 38.7 being due to cytomegalovirus. Other viruses recognized included herpes simplex and adenovirus. Additional opportunistic infections were due to Mycobacterium avium-intracellulare, toxoplasmosis, and tuberculosis. Nonspecific bacterial bronchopneumonia was present in 11 cases. Cytomegalovirus and P. carinii coinfection was the most common association found. In this series patients died at a younger age (72 at or younger than 1 year old) and there was a slightly higher number of cases of histoplasmosis and brain toxoplasmosis than in other previously published series of infants and children. 相似文献
7.
Persistent diarrhea associated with AIDS 总被引:1,自引:0,他引:1
GT Keusch DM Thea M Kamenga K Kakanda M Mbala C Brown F Davachi 《Acta paediatrica (Oslo, Norway : 1992)》1992,81(S383):45-48
Chronic diarrhea and wasting are very common manifestations of AIDS in adults in developing countries. Etiologic studies show that protozoa (including Cryptosporidium parvum, Isospora belli , and Enterocytozoon bieniusi ) and Mycobacterium avium-intracellulara are the most frequently identified pathogens. Limited data in children suggest that common enteric pathogens are equally as likely in HIV+ and HIV- babies. Preliminary analysis of an ongoing longitudinal study of 469 babies born to mothers with known HIV serostatus in Kinshasa, Zaire, reveals progression of acute to persistent diarrhea is six times greater in HIV+ compared to HIV- babies, and 3.5 times greater in HIV- babies born of HIV+ mothers in comparison to HIV- babies with HIV- mothers. HIV+ babies were also at greater risk than HIV- babies to have recurrent episodes of diarrhea (RR = 2.3). Fifty percent of the deaths were due to acute or persistent diarrhea, and were strongly associated with HIV infection. Efforts to improve child survival in AIDS infected populations will need to address HIV infections in both mothers and infants. 相似文献
8.
《Pediatric hematology and oncology》2013,30(1):68-72
The relevancy of the urinary tract as a source of infection during febrile neutropenia is not known. The authors sought to determine the frequency of urinary tract infections (UTIs) in pediatric cancer patients with febrile neutropenia. Urine was collected from a mid-stream void before the administration of antibiotics. Demographic, clinical, and laboratory data were collected. The frequency of UTI and usefulness of urinalysis and localizing signs in predicting UTI in pediatric cancer patients with fever and neutropenia were determined. Forty-five patients had 58 febrile neutropenic episodes eligible for study participation. No patient presented with localizing signs. The urinalysis was negative in 53 episodes and positive in 5 episodes. Four patients had 5 UTIs. The frequency of UTI was 8.6% (5 of 58 febrile neutropenia episodes). Four patients had bacteremia, none of whom had a UTI. The sensitivity, specificity, and negative predictive value of urinalysis was 40%, 94%, and 94%, respectively, and for localizing signs was undefined, 100%, and 91%, respectively. UTI is as common as bacteremia in the current pediatric cancer patients with fever and neutropenia. Urinalysis and urine culture should be obtained routinely as part of the diagnostic evaluation of patients with fever and neutropenia. 相似文献
9.
Semha Berbero
lu Mü nevver Bü yü kpamukcu Faik Sarialioglu Canan Akyü z nci lhan 《Pediatric hematology and oncology》1995,12(2):171-178
Between September 1991 and April 1993 the hepatitis B vaccination with recombinant hepatitis B vaccine was administered in 41 cancer patients following first diagnosis. All patients were under 16 year of age, with negative hepatitis B virus (HBV) serology and normal hepatic function. They received 40 μg of vaccine by injection into the deltoid muscle at 0, 1, and 2, months, with a fourth dose planned at the 4th month for nonresponders. At 1 year a booster dose was given. All the patients began vaccination within 1 month following diagnosis, and periodic serologic follow-up was performed immediately after each vaccination and also in the 6th, 9th, and 12th months after vaccination. Patients with production of anti-HBs at a titer equal to or greater than 10 mIU/L were considered seropositive. The seroconversion rates were 12.4%, 21.9%, 41.0%, and 48.7% after the first, second, third, and fourth monthly doses, respectively. Seroconversion rates were 56.0% at 6 months, 67.5% at 9 months, and 70.5% at 12 months. Geometric mean antibody titers were 212 and 373 mIU/L at 9 and 12 months, respectively. No serious side effects were observed. HBV vaccination is recommended for pediatric cancer patients. 相似文献
10.
Beverly Barton Rogers Colleen Conlin PA Charles F. Timmons M. D. PhD D. Brian Dawson Karen Krisher Walter S. Andrews 《Fetal and pediatric pathology》1997,17(3):391-400
Epstein-Barr virus (EBV)-associated illnesses in posttransplant patients are difficult to diagnose. Attempts to aid in the diagnosis of such illnesses using the polymerase chain reaction (PCR) analysis for EBV have met with variable success due to the potential exquisite sensitivity of the assay. We have designed a relatively insensitive EBV PCR assay and compared the results with objective evidence of EBV activity including serologic response and in situ hybridization for the EBV genome. Eighty-five specimens from 65 patients were analyzed by the EBV PCR using DNA from whole blood. EBV serologic evaluation was done on 53 of the samples and in situ hybridization for EBV (EBER-1 mRNA) on 46 paired liver biopsies. Of 85 samples, 25 (29 ) were positive for EBV using the PCR assay. Intensity of amplification was graded 0.5-1 (weak) to 3 (strong). Using these criteria, 19 EBV PCR-positive samples were graded 0.5-1 , 5 were graded 2 , and 1 was graded 3 . Of the moderate to strongly positive samples (2 or 3 ), five of six had two or more EBER-1-positive cells in the liver biopsies. Of the remaining 40 liver biopsies with either negative or weak positive PCR results, 3 had only single cells positive for EBER-1; the remainder were negative. In addition, PCR-positive results correlated with increasing EBV anti-early antigen antibody (P.005) and viral capsid antigen IgG immunoglobulin G VCA (P.05) EBV-positive results using the PCR assay correlated with objective evidence for increased EBV burden in children after liver transplantation. These preliminary data suggest that this PCR test may be useful to help guide immunosuppressive therapy in the posttransplant patient. Further evaluation using larger numbers of patients will be necessary to confirm this. 相似文献
11.
In order to assess the significance of gastrointestinal protein loss as a cause of plasma protein hypercatabolism in patients with nephrotic syndrome, seven nephrotic children were studied simultaneously with radioiodine labelled albumin and a test substance, 51,Fe-labelled iron dextran, for evaluation of gastrointestinal protein loss. All the patients displayed a pronounced hypercatabolism of albumin, which could not be accounted for by urinary protein loss. A slightly increased gastrointestinal protein loss was demonstrated in 4 patients, whereas, in 3 patients, the faecal loss of macromolecules was within normal limits. The relation between protein catabolism and faecal loss of macromolecules was compared in the nephrotic children and in 20 patients with protein-losing enteropathies. It is concluded that gastrointestinal protein loss is insignificant in the nephrotic syndrome, and unable to account for the marked endogenous hypercatabolism of albumin. Albumin synthesis was normal or somewhat increased. An abnormally high fraction of the total albumin mass was present in the intravascular space in 4 of the 7 children. 相似文献
12.
C. F. Classen M. Warmuth-Metz K. Papke A. Trotter J. E. A. Wolff S. Wagner 《Pediatric hematology and oncology》2013,30(8):631-637
High-grade gliomas in children are rare and the best treatment is undetermined. The German language group study HIT-GBM compares various induction protocols for subsequent patient cohorts. Currently, cisplatinum, etoposide, ifosfamide, and vincristine are given simultaneously with extended-field radiotherapy. Imaging is done 3 weeks after to define treatment response, followed by 6-weekly controls during consolidation with lomustine, vincristine, and prednisone. The authors report on 2 patients with incompletely resected glioblastoma multiforme in which response was lacking 3 weeks after radiochemotherapy but became evident 12 weeks later. This suggests that later time points are required to assess induction protocol response. 相似文献
13.
Murray M Hatcher H Jessop F Williams D Carroll N Bulusu R Judson I 《Pediatric blood & cancer》2008,50(2):386-388
We report a rare case of advanced, metastatic gastrointestinal stromal tumor (GIST) in a young female. Molecular analysis of the tumor revealed wild-type (WT) KIT and platelet derived growth factor receptor alpha (PDGFRA) gene status with no mutations characteristic of adult GIST. Despite this she had clinical benefit and evidence of radiological response to sequential treatment with the tyrosine kinase inhibitors imatinib and sunitinib. 相似文献
14.
The present study looked for any associations between in vitro drug sensitivity and clinical outcome in pediatric acute lymphoblastic leukemia (ALL) with the standard drugs used for leukemia therapy. A total of 72 samples were analyzed. In vitro sensitivity to drugs was tested by a methyl-thiazol-tetrazolium assay in 6 serial fold dilutions. Apoptosis was determined by TUNEL assay and apoptotic index was calculated for each sample. Patients sensitive to prednisone, asparginase, vincristine, and 6-mercapto purine had higher overall survival compared to patients whose tumor cells were resistant to these drugs (p <. 01). For the other drugs tested, overall survival did not vary from that of the resistant patients. For doxorubicin, asparginase, vincristine, prednisone combination sensitivity, there was a significant worsening of prognosis from the extremely sensitive patients through an intermediate sensitive group to a most resistant group. The present study thus shows that in vitro drug-sensitivity testing provides significant prognostic information in childhood ALL. 相似文献
15.
Joanne Embree 《Paediatrics & child health》2005,10(5):261-263
HIV infection and AIDS among children continues to be a significant problem in developing countries despite the progress that has been made in HIV prevention and AIDS treatment elsewhere during the past two decades. The reasons for this difference are complex and multifactorial. They include the higher background prevalence of infection among adults in some communities in developing countries, the slow implementation in many countries of prenatal HIV screening programs and prophylaxis which can reduce the transmission to infants during labor and delivery, the social and health consequences of not breastfeeding, and the economic realities associated with expensive diagnostic testing and antiretroviral treatment. While the world waits for an effective HIV/AIDS vaccine, to reduce the prevalence of HIV in the community, public health programs need to continue to emphasize proven methods of HIV transmission prevention among groups with a high-risk of HIV acquisition, as well as provide counselling for the general population about personal protection and the provision of compassionate care for those affected. 相似文献
16.
Abstract. The gastrointestinal absorption of penicillin V (pc-V) was investigated in 6 children, 6–12 months old, with suspected coeliac disease. The diagnosis was set after small bowel biopsy and absorption tests of vitamin A and d -xylose. As control groups served 7 children with diarrhoea but with normal small bowel biopsy and/or absorption tests and a group of 9 children with upper respiratory tract infection of the same ages as the children in the test group. The absorption of calcium pc-V in oil suspension (Penicals®) was impaired in the patients with suspected coeliac disease compared to that of the control groups. There was no significantly different absorption of pc-V between the control children with diarrhoea and those with upper respiratory tract infection. After 6–8 months of gluten free diet in the children with suspected coeliac disease their absorptive ability of oral calcium pc-V in suspension form was equal with that of a control group. 相似文献
17.
Fredrika Gauffin Eva Diffner Bertil Gustafsson Ann Nordgren Anette Gjörloff Wingren Birgitta Sander 《Pediatric hematology and oncology》2013,30(1):48-56
PTEN and SHP1 are tumor suppressor genes involved in the regulation of cell cycle control and apoptosis. The authors investigated the protein expression of PTEN and SHP1, by immunohistochemistry in tissue microarrays from bone marrow samples in children, diagnosed with acute lymphoblastic leukaemia and nonmalignant controls. PTEN was overexpressed in diagnostic ALL samples, while SHP1 showed a low expression. Both proteins showed a significant difference in expression compared to nonmalignant controls. The roles of PTEN and SHP1 are not well investigated in pediatric leukemia and could in the future play a role as prognostic factors. 相似文献
18.
Margarita Rodríguez-Mahou Javier López-Longo Normand Lapointe Luis Carreño Raquel Grau Dolores Gurbindo Eduardo Fernández-Cruz 《Acta paediatrica (Oslo, Norway : 1992)》1994,83(S400):31-34
We have studied sera from 44 children with Human Immunodeficiency Virus infection and Acquired Immunodeficiency Syndrome using immunoblotting, radioimmunoassay, enzymoimmunoassay and indirect immunofluorescence. We have detected a low incidence of antinuclear (2.9%), anti-reticulin (2.9%) and anti-smooth muscle (14.7%) antibodies by indirect immunofluorescence. By enzymoimmunoassay we have detected anti-dsDNA (20.5%) and anti-ENA [anti-nRNP (61.3%), anti-Sm (29.5%), anti-Ro (47.7%) and anti-La (18.1%)] antibodies. Tests for anti-dsDNA by radioimmunoassay were negative, suggesting the presence of low-avidity anti-DNA antibodies. By immunoblotting we have detected anti-C (nRNP) (33.3%), anti-BB' (Sm) (33.3%), anti-Ro (60 KD) (4.5%) and anti-La (11.3%) antibodies. The presence of anti-Ro antibodies was associated with progressive neurological disease. Long-term follow-up studies with larger numbers of patients are necessary to evaluate the clinical significance of the presence of anti-dsDNA and anti-ENA antibodies in children infected with Human Immunodeficiency Virus. 相似文献
19.
Emre Arpali Cihan Karatas Basak Akyollu Serkan Akinci Bilal Gunaydin Oguzhan Sal Ahmet Nayir Burak Kocak 《Pediatric transplantation》2020,24(1)
Urinary tract infection is the most common infectious complication following kidney transplant. Anatomic abnormalities, bladder dysfunction, a positive history of febrile urinary tract infection, and recipient age are reported risk factors. The aim of this study was to determine the risk factors for fUTI, which necessitated hospitalization in the first year after renal transplantation in our pediatric transplant population. A retrospective review of 195 pediatric patients who underwent kidney transplant between 2008 and 2017 from a single institution was performed. All patients admitted to the hospital with fUTI were marked for further analyses. The risk factors including age, gender, dialysis type, history of urologic disorders, and preoperative proteinuria for fUTI in the first year after kidney transplantation and graft survivals were investigated. Independent‐sample t test and chi‐square tests were used for univariate analysis. Exhaustive CHAID algorithm was used for multivariate analysis. The data of 115 male and 80 female patients were retracted. The mean ages of our cohort for males and females were 9.5 ± 5.1 and 10 ± 4.8 years, respectively. The age of the patients at transplant and their gender were found to be a statistically significant risk factors for developing fUTIs. Multivariate analysis showed that fUTI was common in female patients and a subgroup of male patients who had preoperative proteinuria, but no neurogenic bladder had higher risk compared with male patients without proteinuria. Patient surveillance and antibiotic prophylaxis algorithms can be developed to prevent febrile urinary tract infections seen after pediatric kidney transplantation in risky population. 相似文献
20.
Katsuhiro Kure Yong D. Park Tai-Seung Kim William D. Lyman George Lantos Sunhee Lee Sangho Cho Anita L. Belman Karen M. Weidenheim Dennis W. Dickson 《Fetal and pediatric pathology》1989,9(6):655-667
A 6-year-old boy with acquired immunodeficiency syndrome (AIDS) developed aphasia and quadriplegia 3 months before his death. Cerebral vascular ectasia and multiple cerebral infarcts were noted on premortem radiological studies. Postmortem evaluation revealed diffuse aneurysmal dilatation of the circle of Willis associated with fresh and organizing thrombi, destruction of the elastic lamina, and marked intimal fibroplasia. Multiple cerebral infarcts and subacute AIDS encephalitis with basal ganglia calcification were also present. Immunohistochemistry with a monoclonal antibody (anti-gp41) to human immunodeficiency virus (HIV) demonstrated positively stained cells in the arterial wall of the circle of Willis and in the cerebral parenchyma. Double immunostaining demonstrated that gp41-positive cells in the circle of Willis were also positive for a macrophage marker or leukocyte-common antigen, but not with an endothelial marker. Some macrophages or microglia in the cerebrum were also colabeled with anti-gp41. These results suggest that HIV may be directly involved in vascular pathology associated with pediatric AIDS. 相似文献