Management of perinatal HPP during critical illness/ECMO
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Neetu Krishnan
and
Nancy Dunbar
Abstract
Objectives
With the advent of asfotase alfa, the enzyme replacement therapy (ERT) approved for hypophosphatasia (HPP), health care providers need to navigate management of ERT during critical illness.
Case presentation
We present the case of a young girl, treated with ERT for severe perinatal HPP, who had cardiorespiratory arrest in the setting of influenza A. Her life-saving treatment involving extra corporeal membrane oxygenation (ECMO) required a two-week interruption of ERT leading to persistent hypercalcemia and hyperphosphatemia. A three year old female presented with respiratory distress and blood tinged secretions. She was influenza A positive with bilateral opacities on chest X-ray (CXR). Worsening respiratory distress and bradycardic arrest required intubation, CPR and venoarterial ECMO cannulation. She remained on ECMO for 10 days with anticoagulation restrictions requiring her thrice-weekly subcutaneous ERT to be held. Hypercalcemia (12.3 mg/dL) and hyperphosphatemia (7.6 mg/dL) developed two weeks after restarting ERT and resolved six weeks later.
Conclusions
We highlight that the obligatory cessation of ERT while on ECMO led to the loss of functional TNSALP with a profound decrease in bone mineralization leading to excess circulating calcium and phosphorus. In cases where it is necessary to interrupt ERT, we advise close monitoring of calcium and phosphorous levels.
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Research funding: None declared.
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Author contributions: All authors have accepted responsibility for the entire content of this manuscript and approved its submission.
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Competing interests: ND is on the speaker bureau for Alexion and has been a consultant for them in the past.
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Informed consent: Informed consent was obtained from all individuals included in this study.
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Ethical approval: The local Institutional Review Board deemed the study exempt from review.
References
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Articles in the same Issue
- Frontmatter
- Review Article
- Medullary thyroid carcinoma in children: current state of the art and future perspectives
- Original Articles
- Newly defined peroxisomal disease with novel ACBD5 mutation
- Pediatric adrenocortical tumors cohort characteristics and long-term follow-up at a single Argentinian tertiary center
- Sleep disorder and behavior problems in children with type 1 diabetes mellitus
- Increased hepcidin levels and non-alcoholic fatty liver disease in obese prepubertal children: a further piece to the complex puzzle of metabolic derangements
- Screening for testicular adrenal rest tumors among children with congenital adrenal hyperplasia at King Fahad Medical City, Saudi Arabia
- The association between vitamin D levels and metabolic syndrome components among metropolitan adolescent population
- Utility of wrist circumference in recognition of metabolic syndrome in overweight and obese South Indian children and adolescents
- Ketoacidosis in new-onset type 1 diabetes: did the severity increase during the COVID-19 pandemic?
- Predictors of decreased bone mineral density in childhood systemic lupus erythematosus: possible role of osteoprotegerin gene polymorphisms
- Psychiatric behavioral effect and characteristics of type 2 diabetes mellitus on Japanese patients with Prader-Willi syndrome: a preliminary retrospective study
- Biochemical predictors of metabolically unhealthy obesity in children and adolescents
- Adiposity rebound in very-low-birth-weight infants
- The association between a metabolically healthy overweight/obesity phenotype and markers of inflammation among Chinese children and adolescents aged 10–18 years
- Case Reports
- Management of perinatal HPP during critical illness/ECMO
- Wolfram-like syndrome – another face of a rare disease in children
- Two Japanese siblings with arginase-1 deficiency identified using a novel frameshift mutation of ARG1 (p.Lys41Thrfs∗2)
- An atypical presentation of a pathogenic STK11 gene variant in siblings not fulfilling the clinical diagnostic criteria for Peutz-Jeghers Syndrome
