Journal of Pediatric Hematology Oncology This is an RSS file. You can use it to subscribe to this data in your favourite RSS reader or to display this data on your own website or blog.

Successful Haploidentical Bone Marrow Transplantation of an Infant With a Novel Mutation in SAMD9L Gene (Ataxia-Pancytopenia Syndrome)
Data regarding the outcomes of hematopoietic stem cell transplant (HSCT) for the management of SAMD9L-associated ataxia-pancytopenia syndrome remains limited. We depict the case of a 2-month-old male with a novel mutation in the SAMD9L gene, presenting with respiratory failure, pancytopenia and severe developmental delay. He experienced graft failure 2 months after a 4/6 HLA-matched cord HSCT. At 9 months old, an unsuccessful unrelated donor search prompted a haploidentical HSCT with successful engraftment. He sustains excellent donor chimerism and has improved developmentally over 2 years posttransplant. This case demonst...
Source: Journal of Pediatric Hematology Oncology - October 1, 2022 Category: Hematology Tags: Clinical and Laboratory Observations Source Type: research

Medulloblastoma and Down Syndrome: An Extremely Rare Association
Medulloblastoma has a reduced incidence in Down syndrome (DS). This protective characteristic has not been clarified yet. Here, we report the second case of SHH medulloblastoma and DS documented in the literature. A complete surgery was performed followed by reduced craniospinal irradiation dose and adjuvant chemotherapy. No evidence of tumor recurrence was observed. The overall survival was 9.1 years. No family history or physical stigma of other hereditary predisposition syndrome was found. In the elucidation of this extremely rare association, future case reports play an important role in defining the spectrum of brain ...
Source: Journal of Pediatric Hematology Oncology - October 1, 2022 Category: Hematology Tags: Clinical and Laboratory Observations Source Type: research

Rare Presentation of Atypical Hemolytic Uremic Syndrome in a Child With Acute Lymphocytic Leukemia and Pancreatitis
We report a pediatric case of B-cell acute lymphoblastic leukemia complicated by pancreatitis with concomitant aHUS following induction chemotherapy. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - October 1, 2022 Category: Hematology Tags: Clinical and Laboratory Observations Source Type: research

An Infant With Hereditary Fructose Intolerance and a Novel Presentation of Disseminated Intravascular Coagulopathy Following Pyloromyotomy
We present a 2-month-old male infant diagnosed with pyloric stenosis who developed disseminated intravascular coagulopathy following pyloromyotomy. Unexplained persistent coagulopathy, acute liver failure, and metabolic dysfunction led to whole-exome sequencing, which revealed compound heterozygous variants in ALDOB (p.Arg60Ter and p.Ala150Pro), diagnostic of hereditary fructose intolerance. Shortly after initiating a fructose-free diet, our patient had resolution of his coagulopathy, hepatic, and metabolic dysfunction. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - October 1, 2022 Category: Hematology Tags: Clinical and Laboratory Observations Source Type: research

Case Report of a Massive Life-threatening Neonatal Thrombosis Treated With a Targeted, Goal-oriented Scheme of Urokinase
Conclusions: Laboratory and ultrasound results may help in adjusting the duration of the thrombolytic treatment, allowing for longer therapeutic schemes that could optimize treatment success. In addition, our case may suggest a possible combined role of C-reactive protein and procalcitonin as an early diagnostic aid in neonatal thrombosis. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - October 1, 2022 Category: Hematology Tags: Clinical and Laboratory Observations Source Type: research

Ifosfamide-induced Encephalopathy With Rapid Response to Thiamine: A Pediatric Case
We describe an episode of encephalopathy developed at the third cycle of ifosfamide treatment in a child with Ewing sarcoma. With the administration of thiamin, the encephalopathy resolved and no episode was noted during subsequent courses of ifosfamide. Previous use of cisplatin, concomitant use of opioids, low levels of serum albumin and hemoglobin, and elevated levels of serum creatinine are potential risk factors for IIE. The current case illustrates the possibility of IIE even in the absence of such additional risk factors, treated successfully with thiamin and draws attention to the need for close neurological monito...
Source: Journal of Pediatric Hematology Oncology - October 1, 2022 Category: Hematology Tags: Clinical and Laboratory Observations Source Type: research

Acral Skin Rash Caused by Altered Mercaptopurine Metabolism in Maintenance Therapy for B-Cell Acute Lymphoblastic Leukemia
We report a child who developed a painful acral rash and mucositis while undergoing maintenance therapy for B-cell acute lymphoblastic leukemia without infectious or known drug etiology. Thiopurine metabolites were skewed toward 6-methylmercaptopurine. Two weeks after allopurinol was added and 6-mercaptopurine (6-MP) dose adjusted, the cutaneous manifestations and other constitutional symptoms resolved. We posit that the rash was because of 6-MP toxicity related to skewed metabolism, adding to the growing list of toxicity related to altered 6-MP metabolism. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - October 1, 2022 Category: Hematology Tags: Clinical and Laboratory Observations Source Type: research

Surgical Treatment for Pneumothorax and Tumor-bronchial Fistula Secondary to Pulmonary Metastasis of Osteosarcoma in Pediatric and Adolescent Patients
Conclusions: Although it is not a curative surgery, surgery for pneumothorax and tumor-bronchial fistula is acceptable. The operative procedure should be considered on the basis of the predicted prognosis of the patient. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - October 1, 2022 Category: Hematology Tags: Clinical and Laboratory Observations Source Type: research

Knowledge of Required Medical Surveillance Among Adolescent and Young Adult Survivors of Childhood Cancer
Conclusion: Despite attendance at a survivorship clinic, minority of participants (9.5%) demonstrated complete knowledge of surveillance testing needs. Most survivors are aware of some of their surveillance needs. PROMIS scores were not associated with surveillance knowledge. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - October 1, 2022 Category: Hematology Tags: Clinical and Laboratory Observations Source Type: research

Abatacept-based Graft-Versus-Host Disease Prophylaxis in Haplo-identical Hematopoietic Cell Transplant in a High-risk Cohort
Conclusion: An Aba-based regimen can result in reliable engraftment and acceptable GVHD when concerns of organ dysfunction prevents the use of posttransplant cyclophosphamide in haplo-hematopoietic cell transplantation. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - October 1, 2022 Category: Hematology Tags: Clinical and Laboratory Observations Source Type: research

Outcome and Determinants of Neutropenic Enterocolitis in Pediatric Cancer Patients
Conclusions: NEC caused significant morbidity and mortality in pediatric cancer patients. Gut dysbiosis was significantly higher in NEC group suggesting a role in pathogenesis and influencing outcome. This highlights the role of targeted interventions towards gut dysbiosis like prebiotics and probiotics. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - October 1, 2022 Category: Hematology Tags: Original Articles Source Type: research

Factors Associated With Fertility Preservation in a Pediatric, Adolescent and Young Adult Population
Objective: To determine the patient characteristics associated with pursuing fertility preservation (FP) before gonadotoxic therapy in a pediatric, adolescent and young adult patient population. Methods: This is a retrospective cohort study of patient data at Cincinnati Children’s Hospital Medical Center. Demographics, clinical diagnoses, and treatment characteristics were compared between participants that selected FP versus those that declined. Variables were analyzed separately for males and females by logistic regression. Results: Patients with a hematologic cancer were less likely to be eligible fo...
Source: Journal of Pediatric Hematology Oncology - October 1, 2022 Category: Hematology Tags: Original Articles Source Type: research

Acute Hemolytic Anemia Caused by G6PD Deficiency in Children in Mayotte: A Frequent and Severe Complication
Severe hemolytic anemia is a rare complication of glucose-6-phosphate dehydrogenase (G6PD) deficiency. It occurs with the Mediterranean (Med) variant corresponding to a class 2 deficiency according to the World Health Organization (WHO) classification, and it correlates with a severe deficiency in G6PD activity. In Mayotte, the majority of patients have the African (A-) variant as a WHO class 3 deficiency. Yet we have observed numerous cases of severe hemolytic anemia defined by a hemoglobin level of (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - October 1, 2022 Category: Hematology Tags: Original Articles Source Type: research

Enuresis and Hyperfiltration in Children With Sickle Cell Disease
Nocturnal enuresis is a common symptom in children with sickle cell disease (SCD). Risk factors for development of enuresis are currently unknown. An early manifestation of SCD-associated kidney damage is glomerular hyperfiltration. We test the hypothesis that in a pediatric SCD cohort, individuals with hyperfiltration are more likely to have nocturnal enuresis when compared to children without hyperfiltration. To assess the relationship between nocturnal enuresis and hyperfiltration, we retrospectively evaluated children with SCD enrolled in the Evaluation of Nocturnal Enuresis and Barriers to Treatment among Pediatric Pa...
Source: Journal of Pediatric Hematology Oncology - October 1, 2022 Category: Hematology Tags: Original Articles Source Type: research

Sleep Apnea Screening in Children With Sickle Cell Anemia
Conclusion: PSQ is a poor screening tool for detection of OSA in those children with SCA who snore. Physician assessment for snoring could however be an initial approach before polysomnography. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - October 1, 2022 Category: Hematology Tags: Original Articles Source Type: research