Hypoglycemia Associated With PEG-asparaginase and 6-MP Therapy During Treatment of Acute Lymphoblastic Leukemia in Pediatric Patients: A Case Series.

BACKGROUND: Asparaginases are a mainstay treatment for pediatric acute lymphoblastic leukemia (ALL). Recent reports identified hypoglycemia associated with asparaginases. Other reports describe hypoglycemia associated with 6-mercaptopurine (6-MP), another fundamental ALL therapy. Little is known about the risk of hypoglycemia associated with ALL therapy, an adverse event that puts children at risk of decreased level of consciousness, seizures, and possibly negative neurocognitive sequelae. METHODS: We performed a retrospective chart review of 6 children with hypoglycemia during ALL treatment in our institution from May 2016 to August 2019. Timing and duration of hypoglycemia relative to polyethylene glycol (PEG)-asparaginase, 6-MP, and corticosteroids were determined. Laboratory values of the critical sample were collected. RESULTS: The median age was 2.75 (interquartile range: 1.88 to 3.63) years. Three patients had trisomy 21. The onset of hypoglycemia was 5 to 19 days after the most recent PEG-asparaginase administration or 6 to 7 months after initiating daily 6-MP. Sixteen hypoglycemic events were documented, and 9/16 had a critical sample drawn. Six events were hypoketotic, associated with PEG-asparaginase. Three were ketotic, associated with 6-MP. Two patients required treatment with diazoxide and cornstarch. CONCLUSIONS: Hypoglycemia associated with PEG-asparaginase occurred later and lasted longer than previous reports with l-asparaginase, with the likely mechanism being hyperinsulinism. 6-MP was associated with ketotic hypoglycemia.

Successful Transjugular Portosystemic Shunt Treatment of Pediatric Sinusoidal Obstruction: Case Report and Review of Literature.

Background: In adults with medically refractory sinusoidal obstruction syndrome (SOS), a transjugular intrahepatic portosystemic shunt (TIPS) has been used successfully to improve portal hypertension and symptoms such as ascites. There is limited data on the use of TIPS for SOS in pediatric patients. Methods: The index case was reviewed retrospectively. PubMed and Medline databases were searched to identify other cases. Results: A 4-year-old male with high-risk neuroblastoma, developed SOS after tandem autologous stem cell transplant. He was medically managed with defibrotide, diuretics, and peritoneal drainage, but, due to refractoriness, he underwent TIPS day +54 following bone marrow transplant. Hepatic venous pressure gradient improved from 17 to 8 mm Hg following TIPS placement with significant improvement in the patient's clinical status and ascites. However, 15 months later, his shunt remained patent, and he remains clinically well with stable liver enzymes. A literature review identified 13 pediatric cases of TIPS for SOS due to varied causes. TIPS caused a median hepatic venous pressure gradient of 9 mmHg (range, 2-38 mm Hg). The mortality following the procedure was 15%, with 2 cases who died at 2- and 11-days post-TIPS. At the time of the last follow-up (range 8-25 months), 5 patients were alive, and 8 were lost to follow-up. Conclusion: We present here a pediatric case of SOS due to stem cell transplant treated successfully with TIPS with a review of the literature. A timely, individualized application of TIPS can be effective in treating children with medication-refractory SOS.

Severe iron deficiency anemia in the paediatric emergency department: A retrospective study.

Background: Transfusion is discouraged in hemodynamically stable children with severe iron deficiency anemia (IDA). Intravenous (IV) iron sucrose (IS) could be an alternative for some patients; however, there is a paucity of data on its use in the paediatric emergency department (ED). Methods: We analyzed patients presenting with severe IDA at the Children's Hospital of Eastern Ontario (CHEO) ED between September 1, 2017, and June 1, 2021. We defined severe IDA as microcytic anemia <70 g/L and either a ferritin <12 ng/mL or a documented clinical diagnosis. Results: Of 57 patients, 34 (59%) presented with nutritional IDA and 16 (28%) presented with IDA secondary to menstrual bleeding. Fifty-five (95%) patients received oral iron. Thirteen (23%) patients additionally received IS and after 2 weeks, the average Hgb was similar to transfused patients. The median time for patients receiving IS without PRBC transfusion to increase their Hgb by at least 20 g/L was 7 days (95%CI 0.7 to 10.5 days). Of 16 (28%) children who were transfused with PRBC, there were three mild reactions, and one patient who developed transfusion associated circulatory overload (TACO). There were two mild and no severe reactions to IV iron. There were no return visits to the ED due to anemia in the following 30 days. Conclusions: Management of severe IDA with IS was associated with a rapid rise in Hgb without severe reactions or returns to ED. This study highlights a strategy for management of severe IDA in hemodynamically stable children that spares them the risks associated with PRBC transfusion. Paediatric specific guidelines and prospective studies are needed to guide the use of IV iron in this population.

Acute myeloid leukaemia presenting as proptosis in an infant.

Paediatric patients with acute myeloid leukaemia (AML) often present with symptoms associated with the disruption of normal haematopoiesis and subsequent cellular deficiencies. Periosteal reactions are common in paediatric leukaemia, but typically manifest as a thin, laminated pattern along long bones. Aggressive periosteal reactions are much less frequently seen. Here, we report a case of paediatric AML initially presenting with proptosis and periorbital swelling caused by aggressive, sunburst periosteal reactions surrounding the sphenoid and zygomatic bones. This unique presentation emphasises the importance of considering leukaemic infiltration in the differential for sunburst periosteal reaction in paediatric patients.