Notes

Pansharpening regarding WorldView-2 Information through Graph and or chart Regularized Thinning Coding and also Adaptable Combined Glossary.
Intradural extramedullary peripheral primitive neuroectodermal tumor (pPNET) with t(11;22) is a rare clinical finding in the pediatric population with few published cases in the literature. The authors report 3 cases of intradural primary pPNET and discuss the clinical presentation, treatment, and survival of the patients. Clinicians should be vigilant in considering pPNET in the differential diagnosis of extradural masses. The authors also compare the clinical course and outcome of therapy with primary PNET of the central nervous system and Ewing sarcoma family of tumors. In addition, this report highlights the risk for leptomeningeal dissemination at recurrence and discusses the importance of central nervous system-targeted therapy for durable disease control.Children with neurofibromatosis type I (NF1) have a higher predisposition for low-grade astrocytomas of the optic pathway, commonly referred to as optic pathway gliomas (OPGs). OPGs can result in visual deterioration. Treatment outcomes in OPG-NF1 management are often reported around tumor stabilization. We sought to compare vision outcomes associated with different OPG treatment strategies to inform about this important functional metric. A meta-analysis exploring the different modalities to treat children with OPG-NF1 was conducted following Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines using multiple databases. Of the 113 articles identified in the search, 23 full text articles, representing 564 patients, were included for review. These articles included retrospective, prospective, and randomized controlled studies on observation (n=9), chemotherapy (n=19), radiation therapy (n=6), and surgery (n=7). Of the patients undergoing observation, 87% (60/69) demonstrated stable acuity. In the chemotherapy studies, 27.3% (72/264) demonstrated improved acuity/visual field and/or visual-evoked potential amplitudes, 39.4% (104/264) stable acuity, and 33.3% (88/264) deterioration. SP600125 Both the radiation and surgical treatments reported worsening acuity at 90.9% (10/11) and 73.3% (11/15), respectively. Causal associations are not known. Indications for and timing of treatment choice warrant larger scale study to provide further understanding.Coronavirus disease-2019 in children has been linked to various clinical presentation, from paucisymptomatic cutaneous eruptions, to multisystemic inflammatory syndrome. We report the case of an 8-year-old boy who presented with persistent fever and pancytopenia, associated to a skin rash. An extensive etiological workup showed a positive serology for severe acute respiratory syndrome coronavirus 2 and Epstein-Barr virus. A few weeks later, type B acute lymphocytic leukemia was diagnosed. This case underlines the polymorphic appearance of coronavirus disease-2019 and the need for critical appraisal.
Recent advances in immunology, genomics, and cellular therapy have opened numerous therapeutic possibilities in pediatric hematology-oncology, generating new hope in poor prognosis situations. How decisions are made when it comes to treatments and aims needs to be explored in this new technological context. In particular, their impact on the gold standard of early referral to palliative care must be assessed.

Stemming from an ethnographic study combining semistructured interviews and observations carried out in a hematopoietic stem cell transplant unit in a Montréal Pediatric Hospital, we discuss the decision-making process when a patient faces poor prognosis.

Although health care providers individually envisioned that palliative care may be the best course of action for patients receiving emergent therapy, they remained collectively in the curative mode. The intricate relationship between science, hope, caregiver, and care receiver sustains this perspective even when (near) death is the probable outcome. When proven treatment fails, emerging therapeutic possibilities offer new hope that can delay the referral to the palliative care team.
Although health care providers individually envisioned that palliative care may be the best course of action for patients receiving emergent therapy, they remained collectively in the curative mode. The intricate relationship between science, hope, caregiver, and care receiver sustains this perspective even when (near) death is the probable outcome. When proven treatment fails, emerging therapeutic possibilities offer new hope that can delay the referral to the palliative care team.Factor VII (FVII) deficiency is a rare bleeding disorder normally caused by homozygous and compound heterozygous mutations in the F7 gene. Whole-exome sequencing was performed to identify F7 mutations in 3 individuals from 2 unrelated families who were diagnosed with FVII deficiency. Four compound heterozygous mutations were identified and validated in these 3 probands with FVII deficiency. Among the 4 identified mutations, NM_000131.4c.572-1_581del, NM_000131.4c.1250A>G (p.Tyr417Cys), and NM_000131.4c.647G>T (p.Gly216Val) were novel. All 3 novel mutations were predicted to be likely pathogenic by the American College of Medical Genetics and Genomics/Association for Molecular Pathology guidelines.
The common etiology of central nervous system (CNS) complications after allogeneic hematopoietic stem cell transplantation (allo-HSCT) includes CNS infection, metabolic abnormalities, drug toxicity, cerebrovascular events, Epstein-Barr virus-associated posttransplant lymphoproliferative diseases, and hematologic CNS relapse of leukemia. Although graft-versus-host disease (GVHD) is a major complication of allo-HSCT, its CNS involvement is exceedingly rare.

In this report, we describe a patient who exhibited acute myeloid leukemia with t(8;21) (q22;q22) and who suddenly lost visual acuity ~1 year after receipt of allo-HSCT. Given the observation of negative cerebrospinal fluid findings, cyclosporine-related encephalopathy, intracranial hemorrhage, CNS infection, leukemia recurrence, and tumors were excluded. He was diagnosed with both CNS and pulmonary GVHD. After steroid treatment, the lesions gradually reduced in images acquired via cranial and pulmonary computed tomography.

CNS-GVHD is a rare, serious complication of allo-HSCT that is difficult to diagnose.
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