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SUN-LB087 Successful Prevention of Common Complications of Severe Hypercalcemia Secondary to Subcutaneous Fat Necrosis of the Newborn Following Whole Body Therapeutic Hypothermia

Background: Subcutaneous fat necrosis (SCFN) is a rare form of panniculitis mostly seen in neonates with a history of significant perinatal stress and may be associated with life-threatening hypercalcemia. Clinical Case: An infant boy born by emergent C-section at 39 4/7 weeks to a 32 y/o G2P0 mothe...

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Detalles Bibliográficos
Autores principales: Ilaka-Chibuluzo, Sandra, Conway, Thomas, Nagaraja, Soumya, Levine, Robert, Issacson, Teofilita
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Endocrine Society 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6553011/
http://dx.doi.org/10.1210/js.2019-SUN-LB087
Descripción
Sumario:Background: Subcutaneous fat necrosis (SCFN) is a rare form of panniculitis mostly seen in neonates with a history of significant perinatal stress and may be associated with life-threatening hypercalcemia. Clinical Case: An infant boy born by emergent C-section at 39 4/7 weeks to a 32 y/o G2P0 mother due to pre-eclampsia, emerged limp, cyanotic and apneic with heart rate<100 bpm and thick meconium-stained amniotic fluid. Following positive pressure ventilation, his heart rate improved, but, his poor respiratory effort and poor muscle tone persisted. Apgar scores were 2, 5, 6 and 8 at 1, 5, 10 and 15 minutes, respectively. The infant was transferred to the NICU on continuous positive airway pressure. Whole body therapeutic hypothermia (WBTH) was initiated based on the presence of neurologic dysfunction and markers of hypoxic-ischemic injury: metabolic acidosis (cord blood pH 6.89, incalculable bicarbonate), elevated lactate (4.1 mmol/L, n 0.5 - 2 mmol/L), abnormal liver function tests (AST 811 IU/L, n 13 - 39; ALT 1175 IU/L, n 4 - 36 IU/L; LDH >4000 IU/L, n 100 - 250 IU/L), abnormal coagulation panel (PT 75.4 sec, n 9.7 - 12.4 sec; PTT 58.7 sec, n 25 - 37 sec; INR 6.85, n 0.9 - 1.2) and thrombocytopenia (platelet count 90 K/uL, n 150 - 450 K/uL). IV fluids, platelet transfusions and antibiotics were given for presumed sepsis. WBTH was completed on day of life (DOL) 3 and infant was re-warmed overnight. SCFN presented on DOL 4 as erythematous, tender, nodular indurations throughout the skin over the dorsum. Supportive therapy was given: repositioning, morphine for pain during feeding and handling and bacitracin ointment to areas of skin desquamation. Labs were consistent with SCFN. On DOL 18, total serum calcium (TSC) increased to 11.8 mg/dL (n 8.8 - 11.2 mg/dL), intact parathyroid hormone (1 pg/mL, n 14 - 72 pg/mL), PTH related peptide (<2.0 pmol/L, n <2.0 pmol/L), 25 hydroxyvitamin D3 (22.8 ng/mL, n 20 - 50 ng/mL) and 1,25 dihydroxyvitamin D3 (80.7 pg/mL, n 19 - 79.3 pg/mL). Mother discontinued all vitamin supplementation and breastmilk was supplemented with low-calcium infant formula. TSC peaked on DOL 28 to 13.2 mg/dL, and oral furosemide (2 mg/kg/dose, BID) was started. On DOL 30, oral prednisolone (2mg/kg/day) and polycitrate for urine alkalization and prevention of nephrocalcinosis were initiated. Furosemide was discontinued on DOL 32. IV fluids and polycitrate were discontinued on DOL 42. Oral prednisolone was continued following a taper schedule. Urine calcium to urine creatinine ratios initially showed a marked elevation with peak of 1.67 (n <0.86) but eventually began to decline throughout the course of therapy. Head and repeat renal ultrasounds were normal. The infant was discharged home on DOL 50 with down-trending TSC levels and completed a slow oral prednisolone taper on DOL 130. Conclusion: Early recognition and treatment of SCFN and associated hypercalcemia is crucial to prevent morbidity and mortality. Unless otherwise noted, all abstracts presented at ENDO are embargoed until the date and time of presentation. For oral presentations, the abstracts are embargoed until the session begins. Abstracts presented at a news conference are embargoed until the date and time of the news conference. The Endocrine Society reserves the right to lift the embargo on specific abstracts that are selected for promotion prior to or during ENDO.