Hamad Mohammed Alghanim, Mohamed Eltawel, Abdulmajeed Ibrahim Alhaidari, Muhannad Mohammed Alobaid, Areej Mofareh Moghairi, Fahad Sufiani, Naveed Ahmad
Hereditary cancer predisposition accounts for more than 10% of all cancers in pediatric age group and this is increasingly recognized as an important entity because of modern sequencing techniques. We report a rare association of two concurrent cancer predisposition syndromes, BRCA2 and PMS2, in a young child who presented with concurrent malignancies including Wilms tumor, myelodysplastic syndrome and an indeterminate brain lesion who succumbed to his disease. Multiple synchronous malignancies present difficult clinical and psycho-social challenges which need to be carefully addressed in the setting of a multi-disciplinary team approach...
February 2, 2023: Pediatric Hematology and Oncology
Laura L Donahoe, Serina Patel, Soumitra Tole, Alexandra P Zorzi, Lennox Huang, Osami Honjo, Marc de Perrot
A 13-year-old girl presented with hypoxemia during adjuvant chemotherapy for an osteosarcoma of the left distal femur. She underwent an amputation complicated by a post-operative pulmonary embolism (PE). Three months post-operatively, she was admitted to hospital with severe hypoxemia and diagnosed with pulmonary hypertension on echocardiogram in the context of extensive bilateral PE on computed tomography. She was planned for elective pulmonary thromboendarterectomy, but rapidly deteriorated requiring emergent surgery...
February 2023: Pediatric Hematology and Oncology
Alexandra Bohm, Caleigh Campbell, Cheryl Peters, Natasha Datoo
The presence of an anterior mediastinal mass should prompt rapid triage, workup and treatment to effectively manage and prevent emergent complications. Implementation of an AMM protocol can ensure the response is standardized and coordinated. Importantly, such a protocol can encourage prompt multi-disciplinary communication to mitigate risks associated with procedures required for timely diagnosis. The aim of this review is to evaluate the BC Children's Hospital's Pediatric New/Suspected Anterior Mediastinal Mass (AMM) Protocol...
February 2023: Pediatric Hematology and Oncology
Ilona J Frieden, Anthony J Mancini, Francine Blei, David H Darrow, Daniel P Krowchuk
No abstract text is available yet for this article.
January 23, 2023: Pediatric Hematology and Oncology
Jennifer Geel, Marc Hendricks, Yasmin Goga, Beverley Neethling, Vutshilo Netshituni, Rema Mathew, Johani Vermeulen, Anel van Zyl, Fareed Omar, Jan du Plessis, Liezl du Plessis, Elelwani Madzhia, Thandeka Ngcana, Thanushree Naidoo, Lizette Louw, Daynia E Ballot, Monika L Metzger
Introduction: Collaborative studies have contributed to improved survival of pediatric Hodgkin lymphoma in well-resourced settings, but few are documented in resource-constrained countries. The South Africa Children's Cancer Study Group initiated harmonization of management protocols in 2015. This article analyzes barriers and enablers of the process. Methods: Clinician-researchers at 11 state-funded pediatric oncology units completed preparatory questionnaires in June 2018. Parameters included infrastructure, access to therapeutic modalities and clinician numbers...
January 20, 2023: Pediatric Hematology and Oncology
Natasha Bala, Joseph Stanek, Vilmarie Rodriguez, Anthony Villella
Patients with sickle cell disease (SCD) have a high risk for venous thromboembolism which is associated with increased risk of mortality. Studies examining risk of pulmonary embolism (PE) in children with SCD are lacking. This study was conducted in children with SCD between 0-21 years of age using a nationwide administrative database in the United States- Pediatric Health Information System (PHIS) from January 2010 to June 2021. Diagnostic codes and imaging, procedure, and pharmaceutical billing codes were used to identify PE and potential clinical, demographic, and utilization risk factors...
January 16, 2023: Pediatric Hematology and Oncology
Alison M R Castle, Brianna Empringham, Lisa M Pinto, Anita Villani, Nisha Kanwar, Lesleigh S Abbott, Sarah L Sawyer
Neurofibromatosis Type 1 (NF1) is a neurocutaneous syndrome characterized by multiple café-au-lait macules, neurofibromas, and predisposition to malignancies, including rhabdomyosarcomas (RMS). Somatic NF1 mutations occur in RMS and other cancers, and ∼1% of patients with RMS have NF1. We describe three patients who presented prior to one year of age with RMS and were subsequently diagnosed with NF1. Compared to sporadic RMS, patients with this cancer predisposition syndrome are diagnosed younger, genitourinary sites are more common, and tumors are almost exclusively the embryonal subtype...
January 10, 2023: Pediatric Hematology and Oncology
Olive S Eckstein, M Brooke Bernhardt, Chelsey G Hood, Vivekanudeep Karri, Stephen F Kralik, Kenneth L McClain
Patients with Langerhans cell histiocytosis (LCH) have been effectively treated with intravenous cytarabine. Intravenous or subcutaneous cytarabine infusions have been effective for leukemia patients, and pharmacokinetic studies have shown very similar blood levels of the drug with either route. We present three LCH patients treated with subcutaneous cytarabine either because intravenous access could not be maintained or due to patient refusal. One patient with pulmonary and skin LCH had a complete response...
January 10, 2023: Pediatric Hematology and Oncology
Irene Nanyanga, Gideon Kurigamba Kwikiriza, Barnabas Atwiine, Ruth Namazzi, Victor Musiime, Joyce Balagadde Kambugu, Jaques van Heerden
Neuroblastoma (NB) is the most common extracranial solid tumor of childhood. The complete burden and outcomes in Uganda are unknown. The study was a multicenter retrospective chart review of children aged between 0 to 15 years diagnosed with NB from 2010 to 2020. Demographic, clinical and tumor-related characteristics were extracted for analysis. Kaplan-Meier survival curves and Cox regression models were used to determine the one-year overall survival (OS) and identify prognostic factors. Seventy-five patients were evaluated, with a median age at diagnosis of 48 months (IQR 26-108 months)...
December 23, 2022: Pediatric Hematology and Oncology
Sinta Prastiana Dewi, Soehartati A Gondhowiardjo, Irawan Mangunatmadja, Renindra A Aman, Henry Kodrat, T B Mayang Permata, Handoko
Indonesia is a rapidly growing lower-middle-income country (LMIC) located in Southeast Asia. It has 267.3 million inhabitants, with 31.6% (84.4 million) children. According to GLOBOCAN 2020, Indonesia had the highest prevalence of pediatric cancer cases in Southeast Asia (43.5%), and brain tumors had the third-highest incidence in Indonesia. Treating children with brain tumors with radiotherapy is challenging, especially the late treatment effects that can affect their quality of life (QoL). This study aimed to show the QoL in children with brain tumors after radiotherapy in Dr...
November 29, 2022: Pediatric Hematology and Oncology
Marie-Claude Pelland-Marcotte, Soumitra Tole, Eve Bouhelier, Susan Lee, Jessica Halparin, Cherry Mammen, Karen Lyons, Ali Amid
No abstract text is available yet for this article.
November 25, 2022: Pediatric Hematology and Oncology
Maria Aline Ferreira de Cerqueira, Luciana Maria Fortes Magalhães Castelo Branco Couto, Mírian Perpétua Palha Dias Parente, Juan Clinton Llerena
Strokes affect up to 10% of children with sickle-cell disease (SCD). The most commonly used strategy to prevent a first-time stroke or its recurrence is to perform periodic red blood cell transfusions. This article aims to evaluate the quality of life (QoL) of children and adolescents with SCD undergoing a chronic transfusion regimen (CTR) for stroke prophylaxis, according to their caregivers' perception. A cross-sectional study was conducted using a sociodemographic interview with an application of a validated instrument (Pediatric Quality of Life Inventory) involving 16 caregivers of patients with SCD aged <18 years undergoing CTR in a reference center...
November 24, 2022: Pediatric Hematology and Oncology
Richelle C Waldner, Gregory M T Guilcher, Dania A Monagel, Josephine Ho, Sunil Desai, Elizabeth T Rosolowsky
No abstract text is available yet for this article.
November 16, 2022: Pediatric Hematology and Oncology
Chalinee Monsereenusorn, Wanwipha Malaithong, Nawachai Lertvivatpong, Apichat Photia, Piya Rujkijyanont, Chanchai Traivaree
Children with cancer often require sedation before undergoing invasive procedures. Fentanyl, ketamine, and midazolam are effective drugs widely used for procedural sedation. This study aimed to investigate the efficacy and safety of midazolam-fentanyl (M-F) compared with midazolam-ketamine (M-K) for bedside procedural sedation among pediatric oncology patients. A randomized, double-blinded, crossover trial was conducted among children with cancer requiring procedural sedation for invasive procedures. Patients were randomly assigned either intravenous M-F or M-K and subsequently received the alternate regimens following the crossover design of the study...
November 2022: Pediatric Hematology and Oncology
Harri Sievänen, Päivi Lähteenmäki, Juho Kari, Sanna Halonen, Hanna Soukka, Vesa Eskola, Sauli Palmu
Successful first diagnostic lumbar puncture (LP) is crucial because intrathecal chemotherapy has not yet protected the central nervous system against cancer cells. If blood contaminates the cerebrospinal fluid (CSF) with blasts, they may enter the central neural system and compromise the patient's health. We retrospectively determined the incidence of traumatic lumbar punctures (TLP) in 2,507 LPs of 250 pediatric hemato-oncology patients aged from one to 18 years, including both diagnostic and intrathecal treatment procedures, and 2,617 LPs of 1,525 other age-matched pediatric patients...
November 2022: Pediatric Hematology and Oncology
Richard Nyeko, Joyce Balagadde Kambugu, Racheal Angom, Hussein Senyonjo, Solomon Kibudde, Fadhil Geriga, Jaques van Heerden
Brain tumors are the most common solid tumors in children and a leading cause of cancer-related mortality in children worldwide. Data on the epidemiology and management of pediatric brain tumors in Uganda are limited. We aimed to assess the clinicopathological profile and management of pediatric brain tumors at the national oncology center in Uganda since the inception of weekly multidisciplinary meetings. Records of children younger than19 years diagnosed with primary brain tumors at Uganda Cancer Institute between 2017 and 2021 were retrospectively reviewed...
October 31, 2022: Pediatric Hematology and Oncology
Veysel Gok, Alper Ozcan, Sinem Ozer, Filiz Karaman, Esra Aykutlu, Ebru Yilmaz, Musa Karakukcu, Atil Bisgin, Ekrem Unal
Aceruloplasminemia inherited autosomal recessively in the ceruloplasmin gene is a progressive disease with iron accumulation in various organs such as the brain, liver, pancreas, and retina. Ceruloplasmin gene encodes ceruloplasmin protein, which has ferroxidase activity and is involved in copper and iron metabolism. Progressive neurotoxicity, retinopathy, and diabetes may develop in about 40-60 decades. In addition, microcytic anemia accompanied by high ferritin and low ceruloplasmin level that develop at earlier ages can be first manifestation...
October 29, 2022: Pediatric Hematology and Oncology
Atalay Mulu Fentie, Zelalem Tilahun Mekonen, Zelalem Gizachew, Mahlet Hailemariam, Stephen M Clark, Jaime Richardson, Benyam Muluneh
Optimal chemotherapy management is substandard in low and middle-income countries. We aimed to identify major gaps to design interventional strategies for improved chemotherapy management at Tikur Anbessa Specialized Hospital (TASH), Ethiopia. This study was conducted using an observational checklist, open-ended questions, record review, and key informant interviews of department heads and focal persons at TASH. Findings were categorized into specific themes that developed. Chemotherapy represented 60.2% of the hospital medication budget...
October 29, 2022: Pediatric Hematology and Oncology
Jaques van Heerden, Mariana Kruger, Tonya Marianne Esterhuizen, Anel van Zyl, Marc Hendricks, Sharon Cox, Hansraj Mangray, Janet Poole, Gita Naidu, Ané Büchner, Mariza de Villiers, Jan du Plessis, Barry van Emmenes, Elmarie Matthews, Yashoda Manickchund, Derek Stanley Harrison
Surgical control has prognostic value in neuroblastoma (NB). Advanced NB is common at diagnosis in South Africa. We investigated the pre-surgery factors that influenced decisions to perform surgical resections. We included 204 patients with high-risk NB from a national retrospective study, who completed induction chemotherapy between 2000 and 2016.The median age was 32.4 months (IQR 15.1 - 53.5 months). Primary tumor resection was achieved in 76.9% of patients between 0-18 months of age, 51...
October 22, 2022: Pediatric Hematology and Oncology
Harsha Lad, Shoma Naskar, S K D B Punyasri Pasupuleti, Rakesh Nahrel, Pradeep Sihare, Giriraj R Chandak, Pradeep K Patra
Sickle cell disease (SCD) is a disease of abnormal hemoglobin associated with severe clinical phenotype and recurrent complications. Hydroxyurea (HU) is one of the US-FDA approved and commonly used drug for the treatment of adult SCD patients with clinical -severity. However, its use in the pediatric groups remains atypical. Despite a high prevalence of the disease in the state Chhattisgarh, there is a lack of evidence supporting its use in pediatric patients. This study aimed to evaluate the pharmacological and clinical efficacy and safety of HU in a large pediatric cohort with SCD from Central India...
October 13, 2022: Pediatric Hematology and Oncology
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