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Idiopathic Hypereosinophilic Syndrome Associated With Pulmonary Hypertension in an Infant
Hypereosinophilic syndrome (HES) is a very rare disease during childhood. It involves the different organs like skin, gastrointestinal system, heart and lungs, besides pulmonary hypertension (PHT) is a very rare morbidity of HES that may cause life-threatening complications. PHT improves with the treatment of hypereosinophilia, without the need for pulmonary vasodilator therapy. Here, we present a case of PHT developed after recovery of pulmonary infiltration in an infant with idiopathic HES. We revealed that pulmonary pressure returned to normal range in parallel with the decrease in eosinophil count with steroid treatmen...
Source: Journal of Pediatric Hematology Oncology - June 30, 2022 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Transition for Adolescents and Young Adults With Sickle Cell Disease in a US Midwest Urban Center: A Multilevel Perspective on Barriers, Facilitators, and Future Directions
Conclusion: Our study highlights the importance of multilevel barriers and facilitators for AYA-SCD transition from pediatric to adult care. Future studies could use implementation science frameworks to understand local context and identify strategies and intervention characteristics to improve transition programming. These efforts will ultimately reduce health disparities and ensure health equity. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - June 30, 2022 Category: Hematology Tags: Online Articles: Original Article Source Type: research

Clinical Significance of MicroRNA-29a and MicroRNA-100 Gene Expression in Pediatric Acute Myeloid Leukemia: Erratum
No abstract available (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - June 30, 2022 Category: Hematology Tags: Erratum Source Type: research

Pyrites: An Intraocular Mass
No abstract available (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - June 30, 2022 Category: Hematology Tags: Pyrite Source Type: research

Pediatric Donor Cell Acute Lymphoblastic Leukemia Following Bone Marrow Transplant for GATA2 Mutation
We present a 3-year-old female who developed a donor-derived B-cell acute lymphoblastic leukemia 2 years post unrelated HSCT for GATA2 germline mutation. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - June 30, 2022 Category: Hematology Tags: Clinical and Laboratory Observations Source Type: research

Phyllodes Tumor as a Secondary Neoplasm in Survivors of Childhood Cancer: A Tertiary Care Cancer Centre Experience
We report a series of 6 female childhood cancer survivors who developed PT as SN. The median age at primary diagnosis was 13 years. Their primary tumors were bone sarcoma (4) and acute leukemia (2), and all were treated with chemotherapy, predominantly with alkylating agents and/or anthracyclines. None had received direct radiotherapy to the chest wall. Subsequently, PT were detected after a median interval of 7.5 years, with 2 patients developing bilateral and malignant PT. The series highlights a rare SN in childhood cancer survivors, underscoring the importance of regular long-term follow-up. (Source: Journal of Pediatr...
Source: Journal of Pediatric Hematology Oncology - June 30, 2022 Category: Hematology Tags: Clinical and Laboratory Observations Source Type: research

Hemolysis After Medication Exposure in Pediatric Patients With G6PD Deficiency
Hemolysis in glucose-6-phosphate dehydrogenase (G6PD) deficiency varies by mutation status and the oxidative stressor. Although classified by percent of enzymatic deficiency, variability in normal G6PD values clouds assessment of hemolysis risk by level. This was a retrospective, single institution, cohort study assessing risk of postexposure medication-induced hemolysis in G6PD deficient patients. Exposures occurred in 87 of 1415 deficient patients. Only 2 of 87 medication-exposed patients had hemolytic episodes and both had very low enzymatic activity. No hemolytic events occurred with G6PD levels>7 units/g hemoglobin....
Source: Journal of Pediatric Hematology Oncology - June 30, 2022 Category: Hematology Tags: Clinical and Laboratory Observations Source Type: research

Optic Nerve Germinoma and Transient Spontaneous Regression—More Than Meets the Eye
This report highlights the importance of clinical and radiologic monitoring of intracranial germinoma, even in the event of initial spontaneous improvement. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - June 30, 2022 Category: Hematology Tags: Clinical and Laboratory Observations Source Type: research

Treatment-related Correlates of Growth in Children With Sickle Cell Disease in the DISPLACE Cohort
This study investigated growth and hemolysis in children with SCD in the DISPLACE (Dissemination and Implementation of Stroke Prevention Looking at the Care Environment) cohort. The database includes 5287 children, of which, 3305 had at least 2 growth measurements over a 5-year period. Body mass index was converted to z-scores (zBMI), and 19.8%, 66.1%, 14.2% of children were classified as underweight, normal, and overweight/obese, respectively. Multivariable analysis of growth was conducted and included variables: age, sex, blood pressure, hemoglobin, reticulocyte count, treatment with chronic red cell transfusion therapy ...
Source: Journal of Pediatric Hematology Oncology - June 30, 2022 Category: Hematology Tags: Original Articles Source Type: research

Clinical Characteristics, Molecular Background, and Survival of Egyptian Patients With Gaucher Disease Over a 20-Year Follow-up
This study analyzes the general disease characteristics, impact of enzyme replacement therapy (ERT), and overall survival (OS) of 156 Egyptian patients with Gaucher disease (GD) enrolled on hormone replacement from 1998 to 2017. The mean age at diagnosis was 32.46±12.68 months. Anemia was noted at diagnosis in 50%, thrombocytopenia in 30.7%, severe splenomegaly in 58.7%, severe hepatomegaly in 11.9%, and skeletal findings were detected in 24.3% of the patients. The most prevalent GD type was type 3 (54.5%). Twenty-two of type 3 patients had no neurological manifestations at diagnosis, and 12 developed variable central ner...
Source: Journal of Pediatric Hematology Oncology - June 30, 2022 Category: Hematology Tags: Original Articles Source Type: research

Treatment Individualization Using Pharmacokinetic Studies and Joint Ultrasound Imaging in Pediatric Patients With Hemophilia
Hemophilia is characterized by bleeding diathesis, primarily affecting the joints. Prophylactic use of missing factor aims at limiting the number of bleeds and, in the long term, the risk of permanent joint damage. However, standard prophylactic regimens are usually applied empirically, not adjusting for variations in bleeding phenotype or drug metabolism. Aim of the present study was to evaluate the need for individualizing prophylaxis, with guidance of pharmacokinetic (PK) studies and joint ultrasound in a setting of everyday clinical practice. To evaluate adequacy of applied regimens, joint status was assessed using the...
Source: Journal of Pediatric Hematology Oncology - June 30, 2022 Category: Hematology Tags: Original Articles Source Type: research

Attitudes and Practices of Pediatric Oncologists Regarding Palliative Care Consultation for Pediatric Oncology Patients
Conclusions: Compared with the current practice for various pediatric oncology departments, palliative care was not consulted as often as oncologists desired. While barriers to palliative care consultation are outlined in the literature, exploration of how to address them as well as identification of barriers specific to oncologists who are in favor of palliative care consultation are not well-described. Further research exploring these specific barriers is necessary to understand the disconnect between oncologists’ attitudes and palliative care consultation. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - June 30, 2022 Category: Hematology Tags: Original Articles Source Type: research

Abdominal Complications During Treatment for Pediatric Acute Myeloid Leukemia
In conclusion, abdominal complications were frequent with infection considered the predominate cause. Most patients with hyperbilirubinemia fulfilled the criteria for sinusoidal obstruction syndrome. AML treatment might be associated with appendicitis. Patients suffering from concurrent AP and sepsis had a high risk of TRM indicating that high awareness of abdominal complications is essential to reduce mortality, especially during sepsis. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - June 30, 2022 Category: Hematology Tags: Original Articles Source Type: research

Mutational Analysis of the VPREB1 Gene of Pre-BCR Complex in a Cohort of Sporadic Pediatric Patients With B-Cell Acute Lymphoblastic Leukemia
In this study, using polymerase chain reaction and direct sequencing of 88 pediatric patients with B-ALL, we investigated the genomic region of the VPREB1 gene to find sequence variations of this gene. Our study presented ten homozygous and heterozygous point mutations and heterozygous nucleotide deletions, in the VPREB1 gene in 36 boys and 32 girls’ patients. Our Bioinformatics assay results presented that these variations may alter the RNA folding, protein structure, and therefore probable effect on the protein function. These results propose that nucleotide changes probably contribute to B-ALL pathogenesis. (Source: J...
Source: Journal of Pediatric Hematology Oncology - June 30, 2022 Category: Hematology Tags: Original Articles Source Type: research

Unresectable Clival Giant Cell Tumor, Tumor Control With Denosumab After Relapse: A Case Report and Systematic Review of the Literature
Giant cell tumors (GCTs) of the skull base are rare entities. Although considered histologically benign, GCTs are locally aggressive with a high rate of local recurrence. The present case describes a 14-year-old girl with a clival GCT who underwent long-term therapy with denosumab after local relapse. To our knowledge, it is the second case described with a follow-up term>2 years from the start of denosumab and who did not receive any other adjuvant treatment besides denosumab. The patient achieved a local control of the disease. According to the few available data, radical excision with adjuvant therapy helps in long-term...
Source: Journal of Pediatric Hematology Oncology - June 30, 2022 Category: Hematology Tags: Review Articles Source Type: research