5,015 results match your criteria Journal of Pediatric Hematology/Oncology[Journal]


Angiopoietin-2 as a Marker of Retinopathy in Children and Adolescents With Sickle Cell Disease: Relation to Subclinical Atherosclerosis.

J Pediatr Hematol Oncol 2019 Apr 15. Epub 2019 Apr 15.

Departments of Pediatric.

Objectives: Angiopoietin-2 (Ang-2) is a multifaceted cytokine that functions in both angiogenesis and inflammation. A proangiogenic state has been found in adults with sickle cell disease (SCD), mainly because of elevated Ang-2 levels. We determined Ang-2 level in 40 children and adolescents with SCD compared with 40 healthy controls and assessed its relation to retinopathy as well as carotid intimamedia thickness (CIMT). Read More

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http://dx.doi.org/10.1097/MPH.0000000000001486DOI Listing
April 2019
2 Reads

Presacral Medulloepithelioma: Case Report and Literature Review.

J Pediatr Hematol Oncol 2019 Apr 15. Epub 2019 Apr 15.

Division of Haematology/Oncology.

Medulloepithelioma is a rare early childhood tumor typically presenting in the intraocular region and neuroaxis. We report a rare case of a 2-year-old girl that presented with a peripheral medulloepithelioma in the presacral region. Examination of the tumor revealed that it lacked amplification of the 19q13. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001460DOI Listing

A Case of Childhood Blastic Phase Chronic Myeloid Leukemia With Minor BCR-ABL.

J Pediatr Hematol Oncol 2019 Apr 15. Epub 2019 Apr 15.

Department of Pediatrics, National Center for Global Health and Medicine.

Chronic myeloid leukemia (CML) is commonly associated with major BCR-ABL transcript. We present a child with blastic phase CML associated with minor BCR-ABL transcript without prior CML diagnosis. Diagnosis was achieved by fluorescence in situ hybridization of peripheral blood neutrophils, which identified 90% as BCR-ABL positive. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001488DOI Listing
April 2019
2 Reads

Steroid-induced Bradycardia During Induction Chemotherapy in Children and Young Adults Diagnosed With Acute Lymphoblastic Leukemia and Lymphoblastic Lymphoma.

J Pediatr Hematol Oncol 2019 Apr 15. Epub 2019 Apr 15.

Pediatrics, Division of Pediatric Hematology and Oncology, Monroe Carell Jr Children's Hospital at Vanderbilt.

Systemic corticosteroids are widely used for the treatment of acute lymphoblastic leukemia (ALL) and lymphoblastic lymphoma. Anecdotal case reports demonstrate bradycardia in patients receiving corticosteroids; however, a more in-depth analysis is lacking. This study aimed to describe the incidence, timing, and outcomes of bradycardia in children with ALL receiving corticosteroids during induction chemotherapy at our center from 2010 to 2016. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001483DOI Listing
April 2019
7 Reads

Neuroblastoma in Adolescents and Children Older than 10 Years: Unusual Clinicopathologic and Biological Features.

J Pediatr Hematol Oncol 2019 Apr 9. Epub 2019 Apr 9.

Department of Pathology, Children's Mercy Hospital/University of Missouri Kansas City School of Medicine, Kansas City, MO.

Neuroblastoma (NB) in children older than 10 years is rare. We reviewed our archives for patients with NB aged 10 to 18 years and summarized their clinicopathologic/genetic records. Of 96 patients, 4 patients were identified in this age group. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001485DOI Listing
April 2019
1 Read

What is the Role of Hematopoietic Cell Transplantation (HCT) for Pediatric Acute Lymphoblastic Leukemia (ALL) in the Age of Chimeric Antigen Receptor T-Cell (CART) Therapy?

J Pediatr Hematol Oncol 2019 Apr 9. Epub 2019 Apr 9.

Division of Pediatric Hematology-Oncology, Medical College of Wisconsin, Milwaukee, WI.

CD19 chimeric antigen receptor T-cell (CART) therapy has revolutionized the treatment of patients with relapsed/refractory hematologic malignancies, especially B-cell acute lymphoblastic leukemia. As CART immunotherapy expands from clinical trials to FDA-approved treatments, a consensus among oncologists and hematopoietic cell transplant (HCT) physicians is needed to identify which patients may benefit from consolidative HCT post-CART therapy. Here, we review CD19 CART therapy and the outcomes of published clinical trials, highlighting the use of post-CART HCT and the pattern of relapse after CD19 CART. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001479DOI Listing

Frequency and Determinants of Invasive Fungal Infections in Children With Solid and Hematological Malignancies in a Nonallogeneic Stem Cell Transplantation Setting: A Narrative Review.

J Pediatr Hematol Oncol 2019 Apr 9. Epub 2019 Apr 9.

Princess Máxima Center for Pediatric Oncology.

Invasive fungal infections (IFIs) are an important cause of morbidity and mortality in children with cancer. An overview of studies on the frequency and determinants of IFI in pediatric oncology patients in nonallogeneic stem cell transplantation settings is lacking. We performed a literature review in Pubmed and Embase, and included 13 prospective and 23 retrospective studies. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001468DOI Listing
April 2019
3 Reads

Pediatric Patients With SHH Medulloblastoma Fail Differently as Compared With Adults: Possible Implications for Treatment Modifications.

J Pediatr Hematol Oncol 2019 Apr 9. Epub 2019 Apr 9.

Departments of Radiation Oncology.

Purpose: The purpose of this work was to study the diversity of sonic hedgehog (SHH) medulloblastoma across different age groups with an emphasis on patterns of relapse.

Methods: All data for the study were obtained through review of medical records, imaging, radiation charts, treatment planning, and chemotherapy details.

Results: Sixty-three patients with SHH medulloblastoma were identified from a prospectively maintained database and classified into 3 groups-infantile: ≤3 years (i-SHH, n=11); pediatric: >3 to <18 years (p-SHH, n=21); and adult: ≥18 years (a-SHH; n=31). Read More

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http://dx.doi.org/10.1097/MPH.0000000000001484DOI Listing
April 2019
1 Read

Brincidofovir as a Salvage Therapy in Controlling Adenoviremia in Pediatric Recipients of Hematopoietic Stem Cell Transplant.

J Pediatr Hematol Oncol 2019 Apr 8. Epub 2019 Apr 8.

Faculty of Medicine and Health, University of Leeds, Leeds, UK.

Adenovirus infection is a well-known complication in patients receiving hematopoietic stem cell transplantation (HSCT). Brincidofovir (BCV) is an orally bioavailable lipid conjugate of cidofovir, which has activity against adenoviruses. We present a review of adenovirus infections treated with BCV which were unresponsive to cidofovir initially in 4 patients and it was used upfront in one patient. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001480DOI Listing
April 2019
1 Read

A Pediatric Case of Transformed Mycosis Fungoides in a BRCA2 Positive Patient.

J Pediatr Hematol Oncol 2019 Apr 8. Epub 2019 Apr 8.

Children's National Medical Center, Washington, DC.

Cutaneous T-cell lymphomas are very rare in children. Although mycosis fungoides is the most common of these rare cutaneous T-cell lymphomas in children, transformation to an aggressive malignancy remains extremely uncommon, and there are no clear guidelines for clinical management in the pediatric population. In addition, the increased usage of next-generation sequencing for pediatric patients with unusual malignancies may result in the discovery of pathogenic germline mutations, though the association between these mutations and the patient's cancer is not always clear. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001481DOI Listing
April 2019
1 Read

Non-transfusion-dependent β-Thalassemia Because of a Single β-Thalassemia Mutation and Coinherited α-Globin Gene Triplication: Need for Increased Awareness to Prevent Incorrect and Delayed Diagnosis.

J Pediatr Hematol Oncol 2019 Apr 8. Epub 2019 Apr 8.

Division of Hematology.

The thalassemias are genetically complex and usually autosomal recessive. We describe 5 unrelated individuals with non-transfusion-dependent β-thalassemia (NTDT), some with apparently dominant transmission, because of a single β-thalassemia mutation coinherited with a triplicated α-globin locus. Each had an initial, incorrect diagnosis of β-thalassemia trait. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001470DOI Listing
April 2019
1 Read

A New Homozygous Mutation (c.393-394del TA/c.393-394del TA) in the NT5C3 Gene Associated With Pyrimidine-5'-Nucleotidase Deficiency: A Case Report.

J Pediatr Hematol Oncol 2019 Apr 3. Epub 2019 Apr 3.

Division of Pediatric Hematology, Dr. Behçet Uz Children's Hospital, Izmir, Turkey.

Pyrimidine-5-nucleotidase (P5'N-1) deficiency is a rare nonspherocytic hemolytic anemia due to pyrimidine nucleotide deposition within erythrocytes. This rare erythrocyte disorder shows autosomal recessive inheritance with mutation of the pyrimidine-5'-nucleotidase gene, which is localized on 7p15-p14. Consanguinity of parents increases the probability of disease with novel mutations. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001482DOI Listing
April 2019
3 Reads

Dyskeratosis Congenita and Oral Cavity Squamous Cell Carcinoma: Report of a Case and Literature Review.

J Pediatr Hematol Oncol 2019 Apr 3. Epub 2019 Apr 3.

Departments of Otolaryngology.

Dyskeratosis congenita is a rare genetic condition of telomerase dysfunction in which patients are at an increased risk of squamous cell carcinoma (SCCa) of the oral cavity. We present here the youngest patient in the literature with a diagnosis of SCCa. We discuss the literature and management of this advanced presentation of SCCa in a child, stressing the importance of palliative care involvement in facilitating medical decision making. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001478DOI Listing
April 2019
3 Reads

Primary Mediastinal Large B-Cell Lymphoma in a Child Presenting With Superior Mediastinal Syndrome and Chylous Pleural and Pericardial Effusion.

J Pediatr Hematol Oncol 2019 Apr 3. Epub 2019 Apr 3.

Departments of Pediatric Oncology.

Primary mediastinal (thymic) large B-cell lymphoma is an aggressive B-cell lymphoma. It comprises <3% of all pediatric non-Hodgkin lymphomas (NHLs). Primary mediastinal (thymic) large B-cell lymphoma usually presents with serous pleural effusion, but presentation with chylous pleural and pericardial effusions is rare. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001472DOI Listing
April 2019
4 Reads

Effect of Sickle Cell Anemia Therapies on the Natural History of Growth and Puberty Patterns.

J Pediatr Hematol Oncol 2019 Apr 3. Epub 2019 Apr 3.

Department of Pediatrics, University of Alabama at Birmingham, Alabama.

As pediatric patients with sickle cell anemia (SCA) have impaired growth and puberty patterns, we studied the effect of disease-modifying therapies on growth and puberty patterns for patients with SCA receiving hydroxyurea (HU), transfusions, or no therapy. We performed a retrospective study of children with SCA in whom anthropometric measurements and therapy type were recorded. Penalized smoothing splines were fitted to estimate growth curves and growth velocity, and linear mixed models were used to examine differences across treatment groups. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001477DOI Listing
April 2019
3 Reads

Late Recurrence of Wilms Tumor After 17 Years: A Case Report.

J Pediatr Hematol Oncol 2019 Apr 3. Epub 2019 Apr 3.

Departments of Pediatrics.

Wilms tumor is the most common renal malignancy in children. Most of Wilms tumor recurrences occur within 2 years of the first diagnosis. Relapse after 5 years after the first diagnosis is called "late recurrence" and is rare in Wilms tumor. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001473DOI Listing
April 2019
9 Reads

Nucleated Red Blood Cells in Children With Sickle Cell Disease Hospitalized for Pain.

J Pediatr Hematol Oncol 2019 Apr 3. Epub 2019 Apr 3.

Division of Hematology, Oncology & Stem Cell Transplant, Ann & Robert H. Lurie Children's Hospital of Chicago.

Acute chest syndrome (ACS) and transfusion requirements are common and difficult to predict during hospitalizations for acute vaso-occlusive episodes (VOE) among individuals with sickle cell disease (SCD). This study examined the relationship between nucleated red blood cell (NRBC) counts during hospitalization for VOE and development of ACS or transfusion requirement among children with SCD. Retrospective chart review was performed for 264 encounters of patients with SCD hospitalized for uncomplicated VOE who had NRBC count data at admission during a 5-year period. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001467DOI Listing
April 2019
2 Reads

Roseamonas gilardii Bacteremia in a Patient With HbSβ0-thalassemia: Clinical Implications and Literature Review.

J Pediatr Hematol Oncol 2019 Apr 3. Epub 2019 Apr 3.

Department of Pediatrics, Division of Pediatric Hematology and Oncology.

Roseamonas gilardii is a gram-negative coccobacillus identified in immunocompromised pediatric patients. A 5-year-old male with a history of HbSβ thalassemia status postsurgical splenectomy presented to the emergency department with fever. Blood cultures grew R. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001476DOI Listing
April 2019
1 Read

Therapeutic Benefit of Blood Transfusion in a Patient With Novel PGK1 Mutation (c.461T>C [p.L154P]).

J Pediatr Hematol Oncol 2019 Apr 3. Epub 2019 Apr 3.

University of Tennessee College of Medicine Chattanooga, Chattanooga, TN.

Phosphoglycerate kinase (PGK) is glycolytic enzyme critical in the creation of adenosine triphosphate. Mutations in the gene for this enzyme, PGK1, are associated with PGK deficiency, which is characterized by neurological symptoms, nonhereditary spherocytic hemolytic anemia, and myopathy. We present a 20-year-old male with a novel c. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001463DOI Listing
April 2019
2 Reads

Osteopoikilosis With Germline LEMD3 Mutation Mimicking Bone Metastases in a Girl With a Concurrent Secreting Mixed Germ Cell Tumor.

J Pediatr Hematol Oncol 2019 Apr 3. Epub 2019 Apr 3.

Division of Pediatric Oncology.

Osteopoikilosis (OPK) is a rare, benign, asymptomatic bone disease causing dense bone lesions, which could be interpreted as bone metastasis. The symmetric distribution, lack of bone destruction, and location differentiate OPK from metastatic disease. It is essential to be aware of this benign condition to prevent diagnostic errors. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001457DOI Listing
April 2019
3 Reads

Recurrent Skin Langerhan Cell Histiocytosis Successfully Treated With Indomethacin Monotherapy.

J Pediatr Hematol Oncol 2019 Apr 3. Epub 2019 Apr 3.

Department of Pediatrics, Division of Hematology/Oncology, University of Alabama at Birmingham.

Langerhans cell histiocytosis (LCH) often has a recurrent and refractory course despite multiagent treatment modalities. Common relapse treatments include intense or prolonged cytotoxic chemotherapy regimens. There are a few prior reports that the nonsteroidal anti-inflammatory drug indomethacin demonstrated activity against bone LCH. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001474DOI Listing
April 2019
1 Read

Trilineage Dysplasia in an Adolescent With Germline GATA2 Mutation.

J Pediatr Hematol Oncol 2019 Mar 29. Epub 2019 Mar 29.

Department of Pathology and Laboratory Medicine, University of Vermont Medical Center.

The GATA family of DNA binding proteins consists of six different transcription factors (GATA1-6), each with diverse biologic function. The GATA2 protein has been shown to be vital for proliferation and maintenance of hematopoietic stem cells; mutations result in variable phenotypes including myelodysplastic syndrome. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001469DOI Listing
March 2019
4 Reads

Allogenic Hematopoietic Cell Transplantation in Thalassemia Major: A Single-center Retrospective Analysis From India.

J Pediatr Hematol Oncol 2019 Mar 29. Epub 2019 Mar 29.

Center for Bone Marrow Transplant, BLK Super Speciality Hospital, New Delhi, India.

Thalassemia is a major public health problem in developing countries. Sibling matched hematopoietic stem cell transplantation (HCT) is the recommended treatment for thalassemia major (TM). We retrospectively analyzed our data of thalassemia major patients who underwent HCT at a tertiary care center in Northern India from January 2008 to September 2017. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001475DOI Listing
March 2019
1 Read

Successful Treatment of Relapsed Isolated Extraocular Retinoblastoma in the Right Fibula With High-dose Chemotherapy and Autologous Stem Cell Transplantation.

J Pediatr Hematol Oncol 2019 Mar 29. Epub 2019 Mar 29.

Department of Paediatric Haematology, Oncology, Blood and Marrow Transplantation, Apollo Cancer Institutes, Chennai, Tamil Nadu, India.

Literature on high-dose chemotherapy followed by autologous stem cell rescue in relapsed retinoblastoma is limited to <150 cases reported so far. We present our experience and the challenges faced in the management of a 7-year-old boy with relapsed isolated extraocular retinoblastoma in the right fibula who received salvage chemotherapy followed by high-dose chemotherapy and autologous stem cell rescue. Electrolyte disturbances, renal tubulopathy, and seizures were the most significant transplant-related morbidity. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001462DOI Listing
March 2019
2 Reads

Chemotherapy Wait Times in a Network of Pediatric Oncology Clinics.

J Pediatr Hematol Oncol 2019 Mar 29. Epub 2019 Mar 29.

Affiliate Program Office, St. Jude Children's Research Hospital, Memphis, TN.

Patient satisfaction with medical care delivery is an important aspect of value-based health care. Providers strive to provide optimal patient satisfaction. Among a network of ambulatory pediatric oncology affiliate clinics, we conducted patient satisfaction surveys and found that the lowest scores were related to delays in the administration of chemotherapy. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001466DOI Listing
March 2019
1 Read
0.956 Impact Factor

A Unique Case of a Pediatric Patient With Blastic Plasmacytoid Dendritic Cell Neoplasm, Guillain Barre Syndrome, and Hemophagocytic Lymphohistiocytosis.

J Pediatr Hematol Oncol 2019 Mar 29. Epub 2019 Mar 29.

Division of Pediatric Hematology/Oncology, Children's Hospital of Eastern Ontario (CHEO), Ottawa, ON, Canada.

Blastic plasmacytoid dendritic cell neoplasm is a rare hematopoietic malignancy with a poor prognosis that is seen primarily in the elderly population. We describe a pediatric patient with blastic plasmacytoid dendritic cell neoplasm who subsequently developed Guillain Barre syndrome followed by hemophagocytic lymphohistiocytosis. All 3 conditions are uncommon, particularly in the pediatric population. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001464DOI Listing
March 2019
2 Reads

Role of Initiating Supportive Care Preceding Veno-occlusive Disease Diagnosis Following Allogeneic Hematopoietic Stem Cell Transplantation in Children.

J Pediatr Hematol Oncol 2019 Mar 29. Epub 2019 Mar 29.

Pediatric Blood and Marrow Transplantation Program, Division of Hematology/Oncology, Children's Hospital of Michigan.

Severe veno-occlusive disease (VOD) following hematopoietic stem cell transplantation has a high mortality rate. The clinical course of VOD, role of preemptive and aggressive supportive care, and outcomes were investigated in a retrospective study from 2007 to 2014. Defibrotide was not available in all but one case with VOD at our center during the study. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001455DOI Listing
March 2019
3 Reads

Typical or Atypical Hemolytic Uremic Syndrome and the Use of Eculizumab: 4 Illustrative Cases.

J Pediatr Hematol Oncol 2019 Mar 29. Epub 2019 Mar 29.

Pediatric Nephrology, Cliniques Universitaires St. Luc (UCL), Brussels, Belgium.

Typical hemolytic uremic syndrome (HUS) in children is caused mostly by Escherichia coli 0157:H7 in our country. Atypical HUS (aHUS) causes include Streptococcus pneumoniae, methyl malonic aciduria, deficiency of ADAMST 13, and genetic or acquired disorder of the complement. Treatment of HUS relies on supportive measures while treatment of aHUS includes plasmapheresis and specific treatments. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001449DOI Listing
March 2019
2 Reads

A Novel Deletion in the RPL5 Gene in a Lebanese Child With Diamond Blackfan Anemia Unresponsive to Steroid Treatment.

J Pediatr Hematol Oncol 2019 Mar 29. Epub 2019 Mar 29.

Department of Pediatrics, University of Freiburg, Freiburg, Germany.

Diamond-Blackfan Anemia (DBA) is a rare inherited form of pure red cell aplasia that usually manifests in infancy or early childhood, and is characterized by normochromic macrocytic anemia and bone marrow erythroblastopenia. The majority of DBA cases are associated with mutations in ribosomal protein genes. Here, we describe a Lebanese girl with RPL5-mutated DBA unresponsive to steroid treatment who died from complications following late hematopoietic stem cell transplantation performed at the age of 15 years. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001435DOI Listing
March 2019
2 Reads

Mycobacterium Avium Complex Presenting as Pulmonary Nodules in a Child With Sickle Cell Disease.

J Pediatr Hematol Oncol 2019 Mar 29. Epub 2019 Mar 29.

Departments of Pediatrics, Texas A&M Health Science Center, Scott & White Medical Center-Temple, Temple, TX.

The case of a 10-year-old child with sickle cell disease with pulmonary nodules and prolonged fevers is reported here. The child was first diagnosed with sarcoidosis based on lung biopsy, but unresponsiveness to therapy led to a second lung biopsy, which revealed the true diagnosis of mycobacterium avium complex disease. Multiple possible explanations for why the patient became infected exist. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001458DOI Listing
March 2019
1 Read

Infantile Pyknocytosis: An Uncommon Cause of Newborn Hemolytic Anemia.

J Pediatr Hematol Oncol 2019 Mar 29. Epub 2019 Mar 29.

Baylor College of Medicine.

Infantile pyknocytosis is a rare cause of neonatal hemolytic anemia, which presents in the first few weeks of life. We report a classic case of infantile pyknocytosis that presented to our institution with rebound hyperbilirubinemia after receiving phototherapy. The infant was found to have a hemoglobin of 5. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001461DOI Listing
March 2019
2 Reads

Comparison of FDG PET/MRI and FDG PET/CT in Pediatric Oncology in Terms of Anatomic Correlation of FDG-positive Lesions.

J Pediatr Hematol Oncol 2019 Mar 29. Epub 2019 Mar 29.

Departments of Nuclear Medicine.

The aims of our study were to compare F-18 fluorodeoxyglucose (FDG) positron-emission tomography/magnetic resonance imaging (PET/MRI) and PET/computed tomography (CT) in pediatric oncology patients in terms of anatomic correlation of FDG-positive lesions, and also to compare diffusion-weighted imaging (DWI) with PET to assess the correlation between apparent diffusion coefficient (ADC) values and standardized uptake value (SUV). Sequential PET/CT and PET/MRI images and/or whole-body DWI and ADC mapping in 34 pediatric patients were retrospectively analyzed. FDG-positive lesions were visually scored for CT, T1-weighted, T2-weighted, and DWI images separately in terms of anatomic correlation of FDG-avid lesions. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001465DOI Listing

Bladder Artery Embolization for Massive Hematuria Treatment in a Patient With Ataxia-Telangiectasia Acute Lymphoblastic Leukemia.

J Pediatr Hematol Oncol 2019 Mar 29. Epub 2019 Mar 29.

Division of Pediatric Intensive Care, Faculty of Medicine, Karadeniz Technical University, Trabzon, Turkey.

Ataxia-telangiectasia (AT) is a hereditary recessive autosomal disorder following a course of progressive cerebellar ataxia, and oculocutaneous telangiectasia. Disease-specific telangiectasias are generally localized in the oculocutaneous region, while telangiectasias located within the bladder are rarely seen in patients with AT. The patient who had been followed-up with a diagnosis of AT since the age of 3 years was later diagnosed with acute lymphoblastic leukemia at the age of 8 years. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001471DOI Listing
March 2019
4 Reads

Nutritional Status, Nutrient Intake, and Food Diversity Among Children With Sickle Cell Anemia.

J Pediatr Hematol Oncol 2019 Apr;41(3):e141-e145

Reference Center for Nutrition of Persons with Sickle Cell Disease (NUTRIFAL), Rio de Janeiro, Brazil.

Children with sickle cell anemia (SCA) often exhibit nutritional deficiencies and are at high risk of dying before the age of 5 years. Ensuring adequate nutrition is a critical part of health care for such children. This study aimed to investigate the association between nutritional status, nutrient intake, and food diversity in children with SCA. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001377DOI Listing
April 2019
1 Read

Advances in Anti-GD2 Immunotherapy for Treatment of High-risk Neuroblastoma.

J Pediatr Hematol Oncol 2019 Apr;41(3):163-169

Department of Pediatrics, Division of Hematology, Oncology and Bone Marrow Transplant.

Neuroblastoma (NBL) is the most common extracranial solid tumor in pediatrics, yet overall survival is poor for high-risk cases. Immunotherapy regimens using a tumor-selective antidisialoganglioside (anti-GD2) monoclonal antibody (mAb) have been studied for several decades now, but have only recently been incorporated into standard of care treatment for patients with high-risk NBL with clear benefit. Here we review a brief history of anti-GD2-based immunotherapy, current areas of neuroblastoma research targeting GD2, and potential diagnostic and therapeutic uses targeting GD2. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001369DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6430125PMC
April 2019
2 Reads

Platelet Indices Alterations in Children With Type 1 Diabetes Mellitus.

J Pediatr Hematol Oncol 2019 Apr 8. Epub 2019 Apr 8.

Pediatric Diseases Research Center, Guilan University of Medical Sciences, Rasht, Iran.

Platelet (PLT) hyperactivity is a key factor which contributes to cardiovascular complications in patients with type 2 diabetes mellitus even in preclinical stages of disease. To the best of our knowledge, there is limited researches in this regard among patients with type 1 diabetes. The aim of this study was to evaluate hematologic indices indicating PLT activity in children with type 1 diabetes. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001454DOI Listing
April 2019
2 Reads

A Study of Congenital Protein C Deficiency With Infancy Onset of CADASIL in a Chinese Baby.

J Pediatr Hematol Oncol 2019 Mar 15. Epub 2019 Mar 15.

Departments of Hematology/Oncology.

Objective: The main objectives of this article were to study a severe congenital protein C deficiency (PCD) in a patient with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) and analyze the cause of this case.

Materials And Methods: We had recorded clinical manifestations of the patient, laboratory tests, imaging studies, and gene sequencing of the PROC gene and NOTCH3 gene to study the disease in this family. We checked the change of NOTCH3 protein by immunohistochemistry. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001436DOI Listing
March 2019
1 Read

Homozygous pArg610del Mutation Unusually Associated With Severe Delay of Growth in 2 Acid Sphingomyelinase Deficiency-affected Sibs.

J Pediatr Hematol Oncol 2019 Mar 11. Epub 2019 Mar 11.

Biochemistry Laboratory, UR12ES17 Sfax Medicine School.

Background: Typically, patients with Acid Sphingomyelinase Deficiency (ASMD) because of p.Arg610del mutation, have mild phenotype with normal linear growth.

Observation: We reported the case of 2 Tunisian brothers who have been referred for splenomegaly, polyadenopathies, pubertal, and growth delay. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001447DOI Listing
March 2019
2 Reads
0.956 Impact Factor

Evaluation of Baseline Cardiac Function by Echocardiography and its Association With Nutritional Status in Pediatric Cancer Patients at The Indus Hospital in Karachi, Pakistan.

J Pediatr Hematol Oncol 2019 Mar 12. Epub 2019 Mar 12.

Indus Hospital Research Center, Indus Health Network, Karachi, Pakistan.

Introduction: Evidence on conducting baseline echocardiogram before starting chemotherapy in pediatric cancer patients is limited from developing countries where malnutrition and infections are common and which may result in cardiac dysfunction.

Materials And Methods: A prospective, observational study was conducted from October 2016 to May 2017 at The Indus Hospital, Karachi, Pakistan, among children aged 1 to 16 years suffering from cancer. Echocardiography was performed before starting chemotherapy. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001437DOI Listing
March 2019
4 Reads
0.956 Impact Factor

Systemic Epstein-Barr Virus-positive T-Cell Lymphoma of Childhood Presentation With Hemophagocytosis.

J Pediatr Hematol Oncol 2019 Mar 12. Epub 2019 Mar 12.

Department of Anatomy and Cell Biology, School of Medicine, College of Medicine, Taipei Medical University, Taipei, Taiwan.

A 2-year-old Asian girl presented to our facility for the evaluation of thrombocytopenia. She was treated with intravenous immunoglobulin under the impression of immune thrombocytopenia. However, her body temperature spiked and progressive pancytopenia, hepatosplenomegaly, abnormal liver function, coagulopathy, and pulmonary infiltration developed. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001452DOI Listing
March 2019
3 Reads

Severe Factor X Deficiency Presenting as Febrile Seizure in an Infant.

J Pediatr Hematol Oncol 2019 Mar 12. Epub 2019 Mar 12.

Department of Corneal Surgery and Ophthalmology, SK Soni Hospital, Jaipur, Rajasthan, India.

Factor X deficiency is a severe inherited coagulation disorder, which is characterized by severe systemic bleeding manifestations in affected individuals. It is a rare disorder with a frequency of around 1:1,000,000 in the general population. We present the case of an infant with factor X deficiency who presented with complex febrile seizure. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001453DOI Listing
March 2019
2 Reads

Clinical Features and Treatment Outcomes of Children With Anaplastic Large Cell Lymphoma in Pakistan: A Multicenter Study.

J Pediatr Hematol Oncol 2019 Mar 6. Epub 2019 Mar 6.

Oncology, Aga Khan University Hospital.

Background: Different approaches have been adopted in the treatment of anaplastic large cell lymphoma (ALCL); there is a lack of consensus with regard to standard treatment. Because of paucity of data from low and middle-income countries, we reviewed the clinical features and treatment outcomes of children with ALCL.

Methods: All ALCL patients under 16 years of age diagnosed from 2005 to 2015 at Aga Khan University Hospital and The Indus Hospital were identified. Read More

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http://Insights.ovid.com/crossref?an=00043426-900000000-9762
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http://dx.doi.org/10.1097/MPH.0000000000001451DOI Listing
March 2019
3 Reads

Langerhans Cell Histiocytosis With Vertebral Involvement Diagnosed and Treated Over the Last 15 Years in a Single Canadian Pediatric Academic Institution.

J Pediatr Hematol Oncol 2019 Mar 6. Epub 2019 Mar 6.

Hematology-Oncology Division.

We report 11 children with vertebral lesion of Langerhans cell histiocytosis (LCH) diagnosed and treated between 2000 and 2015. Vertebral lesions were usually present at LCH diagnosis. No child developed neurological symptoms. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001439DOI Listing

QT Interval Prolongation in the Pediatric Oncologic Population on Methadone.

J Pediatr Hematol Oncol 2019 Feb 28. Epub 2019 Feb 28.

Departments of Pediatrics.

Studies have been conducted on adults prescribed with methadone to determine the necessary frequency of QTc monitoring but no consensus has been reached and no similar research has been conducted in the pediatric population. The objective of this retrospective study was to determine the occurrence rate of QTc interval prolongation associated with methadone use in a pediatric oncologic population. In total, 18% of patients developed QTc interval prolongation. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001444DOI Listing
February 2019
2 Reads
0.956 Impact Factor

A Case of Congenital Anaplastic Large Cell Lymphoma in a Very Preterm Low-Birth Weight Neonate.

J Pediatr Hematol Oncol 2019 Feb 28. Epub 2019 Feb 28.

Cleveland Clinic, Cleveland, Ohio.

A premature infant male was born at 30 weeks' gestation with severe coagulopathy and thrombocytopenia. Over the first days of his life, the patient developed evidence of immune hyperactivation with adenopathy, hepatosplenomegaly, and elevated ferritin. Although the patient met diagnostic criteria for hemophagocytic lymphohistiocytosis (HLH), flow cytometric based assays were not consistent with primary HLH. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001450DOI Listing
February 2019
1 Read

Two Occurrences of Leukemia Relapse Due to Mismatched HLA Loss After Haploidentical Stem Cell Transplantation From Different Family Donors With KIR Ligand Mismatch.

J Pediatr Hematol Oncol 2019 Feb 21. Epub 2019 Feb 21.

Department of Pediatric Oncology, Fukushima Medical University Hospital.

Mismatched HLA loss is a cause of leukemia relapse after HLA-haploidentical stem cell transplantation (haplo-SCT). We report a patient with a history of 2 occurrences of leukemia relapse due to mismatched HLA loss after haplo-SCT. He received haplo-SCT from his father but showed leukemia relapse with loss of the maternal HLA haplotype. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001443DOI Listing
February 2019
1 Read

Myeloid Neoplasm With Eosinophilia and FIP1L1-PDGFRA Rearrangement Treated With Imatinib Mesylate: A Pediatric Case With Long-term Follow-up.

J Pediatr Hematol Oncol 2019 Feb 21. Epub 2019 Feb 21.

Jimmy Everest Section of Pediatric Hematology/Oncology, Department of Pediatrics, University of Oklahoma Health Sciences Center Children's Hospital.

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http://dx.doi.org/10.1097/MPH.0000000000001446DOI Listing
February 2019
1 Read

Langerhans Cell Histiocytosis Masquerading as Hypereosinophilia in a Child.

J Pediatr Hematol Oncol 2019 Feb 21. Epub 2019 Feb 21.

Department of Pediatric Hematology Oncology & BMT, Medanta The Medicity Hospital, Gurgaon, India.

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http://dx.doi.org/10.1097/MPH.0000000000001445DOI Listing
February 2019
1 Read

Treatment of Recurrent Refractory Pediatric Pre-B Acute Lymphoblastic Leukemia Using Inotuzumab Ozogamicin Monotherapy Resulting in CD22 Antigen Expression Loss as a Mechanism of Therapy Resistance.

J Pediatr Hematol Oncol 2019 Feb 22. Epub 2019 Feb 22.

Department of Pediatrics, Division of Pediatric Hematology-Oncology, University of California San Diego, Peckham Center for Cancer and Blood Disorders, Rady Children's Hospital San Diego, San Diego.

Background: Inotuzumab ozogamicin is a novel antibody-drug conjugate that targets CD22, a common antigen on pre-B acute lymphoblastic leukemia cells.

Observations: A 7-year-old boy with pre-B acute lymphoblastic leukemia in his second relapse was given 2 cycles of inotuzumab ozogamicin. He responded morphologically with a negative bone marrow evaluation. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001440DOI Listing
February 2019
2 Reads