Introduction: Data has emerged about patients with T2DM may experience DKA and HHS if infected with SARS-CoV-2. There is limited data about new-onset diabetes in patients with COVID-19. We describe a case series of three Peruvian patients with new onset diabetes presenting with DKA who remained insulin dependent several months after infection resolution. Case 1: A 59-year-old man with no significant past medical history and normal glucose presented with fever and dyspnea for five days. He was hospitalized with hypoxemic respiratory failure and tested positive forCOVID19. Hypoxemia improved with supportive care, but on day three, he became lethargic, tachycardic, and tachypneic with 95% oxygen saturation on room air. Biochemistry revealed an anion gap metabolic acidosis with pH 7.3, bicarbonate 10 mmol/L (22–28), β-hydroxybutyrate 5.4 mmol/L (<0.5), and glucose 679 mg/dL. He was treated with continuous insulin infusion. After DKA resolved, he was transitioned to basal-bolus insulin and remained insulin-dependent during outpatient follow-up. Case 2: A 49-year-old man in good health prior to admission, was transferred to our hospital for acute respiratory failure and positive testing for SARS-CoV-2. Two days later he became confused, tachycardic, and tachypneic with 90% oxygen saturation. DKA was diagnosed based on a pH 7.1, bicarbonate 8 mmol/L, β-hydroxybutyrate 5 mmol/L and glucose 625 mg/dL. He was transferred to the ICU for continuous insulin infusion. After resolution of his DKA, subcutaneous insulin was started. Preadmission hemoglobin A1c was 4.5%. He remained on insulin post hospital discharge. Case 3: A 33-year-old man with normal glucose prior to admission was transferred to our hospital from an outpatient office with a two-day history of dyspnea and altered sensorium. He was tachycardic and tachypneic with 96% oxygen saturation on 3L nasal cannula. He tested positive for SARS-CoV-2. DKA was diagnosed with glucose 690 mg/dL, bicarbonate 4 mmol/L, serum β-hydroxybutyrate 5.8 mmol/L and pH 6.6. He was resuscitated with intravenous fluids and an insulin infusion was started. DKA resolved after 5 days and he was discharged home on subcutaneous insulin. He remained insulin-dependent on follow-up. Conclusion: New-onset diabetes with DKA due to SARS-CoV-2 is increasingly recognized, and beta-cell dysfunction can be permanent, resulting in insulin-dependent diabetes. Accordingly, our patients remained insulin-dependent several months post discharge.
Background Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors with an annual incidence in the U.S. of 500-1600 cases per year. Approximately 30-35% of PPGLs harbor germline mutations, while 35-40% are due to somatic mutations. Recent studies have identified the association between somatic mutations of the hypoxia-inducible factor 2 alpha (HIF2A) gene and a new syndrome of PPGLs associated with congenital polycythemia. Patients present with recurrent PPGLs, congenital malformation of intracranial veins, malformation of macula and retina, and type I Chiari malformations. Case A 44-year-old man with history of in-utero stroke, congenital blindness, polycythemia and spinal stenosis had an incidental para-aortic lesion found during workup of back pain. He experienced episodic palpitations associated with diaphoresis, headache and uncontrolled hypertension for the past eight year. There was no family history of neuroendocrine tumors. Dedicated abdominal computer tomography with contrast showed a right adrenal enhancing lesion measuring 1.4×1.2 cm and a conglomerate of heterogenous enhancing periaortic lesions measuring up to 5 cm in the mid-abdomen. Biochemical work up revealed plasma free normetanephrine of 27.5 nmol/L (0.00-0.89) and plasma free metanephrines of 0.49 nmol/L (0.00-0.49). Gallium 68 DOTATATE scan showed multiple avid retroperitoneal lesions measuring up to 4.4 cm and an avid right adrenal nodule. He underwent right adrenalectomy and para-aortic lesion resection with perio-operative alpha blockade. Pathology confirmed synchronous pheochromocytoma (1.5 cm, Ki-67< 1%, with capsular and periadrenal fat invasion) and paraganglioma (9.3 cm, Ki-67 < 1%, with lymphovascular invasion and one out of nine lymph nodes positive for metastatic disease). Post-operatively, his metanephrines normalized and a six-months positron emission tomography scan showed no evidence of disease. Genetic testing has been delayed. Discussion This case illustrates a rare syndrome of PPGLs, congenital blindness, and polycythemia, likely due to somatic mutations of HIF2a. HIF is an alpha/beta heterodimer. HIF2a becomes a transcription factor after dimerization with HIF2b. Any mutations that stabilize HIF protein leads to transcription of hypoxia inducible genes resulting in angiogenesis, polycythemia, and tumorigenesis. In our patient, we suspect the stroke in-utero may have been related to abnormal intracranial angiogenesis and/or polycythemia. Recognition of this syndrome and lifelong surveillance is imperative due to risk of recurrence of PPGL. Targeted therapies such as a HIF2a inhibitor have been proposed and trials are ongoing. Our patient remains disease free on radiographic and biochemical surveillance. Presentation: Sunday, June 12, 2022 12:30 p.m. - 2:30 p.m.
Introduction Cardiac paraganglioma (PGL) are extremely rare tumors. They are derived from chromaffin cells of the neural crest. Up to 40% of PGL foster germline mutations, while some bear somatic mutations. Here, we describe a sympathetic paraganglioma presenting as a cardiac mass, found to have a novel mutation of SDHB. Clinical Case A 64-year-old man with uncontrolled type 2 diabetes mellitus presented with chest pain, progressive fatigue and unintentional weight loss. An echocardiogram demonstrated a right atrial mass. Cardiac magnetic resonance imaging showed a 6.9×5.8×4.9 cm mass in the lateral right atrial wall and right atrioventricular groove, encasing the right coronary artery. Surgical removal was attempted and aborted due to involvement of the tricuspid valve. Incisional biopsy was consistent with paraganglioma. Plasma free normetanephrine was elevated at 1.87 nmol/L (ULN 0.89 nmol/L) and plasma free metanephrine was normal at 0.14 nmol/L (ULN 0.49 nmol/L). Chromogranin A was elevated at 1,038 ng/mL (normal <93 ng/mL). Iodine-123 meta-iodobenzylguanidine scan demonstrated increased and persistent MIBG activity of the cardiac mass. Ga-68 DOTATATE PET/CT scan revealed DOTATATE avidity of the mass without evidence of distant metastatic disease. Next-generation sequencing of the specimen revealed a variant of unknown significance of SDHB H244D at a variant allele frequency of 62.2%. This missense mutation replaces the histidine to aspartic acid, resulting in changes in charge of the side chain from positive to negative. Systemic treatment with tyrosine kinase inhibitor (TKI) cabozantinib was initiated due to unresectable tumor. Three month surveillance with Ga-68 PET/CT DOTATATE scan revealed partial response to treatment. Chromogranin A and normetanephrine are trending down, suggesting biochemical response. Conclusion Cardiac PGL are rare and SDHB mutations are associated with worse prognosis. This is the first case of a missense mutation of SDHB at H244D, which may be clinically relevant in characterizing cardiac paraganglioma and potential response to TKI therapy. Presentation: No date and time listed
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