2021
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Browsing 2021 by Author "Aggarwal, Amit"
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Item A Clinical Case of Neonatal Alloimmune Neutropenia(2021) Aggarwal, Amit; Bruce, Keelie; McCavit, TimothyBackground: Congenital neutropenia is a common problem in neonates and has myriad etiologies. Neonatal alloimmune neutropenia (NAIN) is among the most common causes of congenital neutropenia with an incidence of 2 per 1000 live births and has an excellent prognosis. NAIN is defined by maternally produced IgG antibodies directed against paternal and fetal neutrophil antigens, most commonly HNA-1a, HNA-1b, and HNA-2a. Case: A 3-week-old female presented with omphalitis and was found to have an absolute neutrophil count (ANC) count of 0. Once admitted and started on IV antibiotics, serial CBC monitoring revealed persistence of neutropenia with ANC counts < 300. On hospital day 3, she was started empirically on subcutaneous filgrastim (GCSF) 5 mcg/kg. Due to a modest response in her ANC, the filgrastim dose was subsequently increased to a max of 30mcg/kg before discharge. Evaluations for etiologies of neutropenia included antineutrophil antibody testing, genetic testing for severe congenital neutropenia (SCN), and a bone marrow exam. SCN was the initial, provisional diagnosis. However, the bone marrow aspiration revealed marked granulocytic hyperplasia with large clusters of promyelocytes, myelocytes, and metamyelocytes and a maturation arrest at the myelocyte – metamyelocyte stage. Antibody neutrophil antibody testing was positive for the HNA-1b antibody, whereas the genetic screen for SCN did not reveal pathogenic mutations. Therefore, she was diagnosed with NAIN and was subsequently weaned off GCSF with a normal ANC. Conclusion: This case illustrates an unusual presentation of NAIN and demonstrates the clinical heterogeneity of this disease state.Item Delayed Recovery of a Prolonged Total IntraVenous Anesthesia Procedure with Risks of Malignant Hyperthermia(2021) Aggarwal, Amit; Faisal, Annum; Hussain, MahammadBackground: Spinal Cord Ependymoma (SCE) is an intramedullary tumor that requires surgical intervention. Total Intravenous Anesthesia (TIVA) is indicated for such neurosurgery cases. The pharmacodynamics and pharmacokinetics of each drug used must be factored to safely extubate and maintain the airway postoperatively. Case: A 57-year-old male with a history of pulmonary hypertension presented to the hospital with complaints of gait difficulty and sensory deficits secondary to SCE. The patient was scheduled for surgery, and the decision was made to do TIVA due to a family history of Malignant Hyperthermia. Three continuous IV drips were placed: propofol, titrated between 125-300 mcg/kg/min, ketamine at 5 mcg/kg/min, and sufentanil at 0.3 mcg/kg/hr. The patient required a phenylephrine infusion at 35 mcg/min to maintain hemodynamics, which had to be titrated up to 75mcg near the 11-hour point due to severe hypotension. Following extubation, the patient was placed on an oral airway with a simple O2 mask in place. He was noted to have snoring respirations with oxygen desaturating to the low 80's. A jaw thrust was done, and he was placed on a non-rebreather mask. Due to a fixed obtunded state, a hasty decision to re-intubate was made without proper revaluation and communication between providers. The patient was then re-extubated 1.5 hours later with minimal post-op complications. Conclusions: This case illustrates the challenges of prolonged TIVA in the assessment of safely extubating patients while maintaining the airway in the postoperative period.