Pheochromocytoma is a rare adrenal tumor that is classically associated with the triad of paroxysmal tachycardia, diaphoresis, and headaches. However, it can have myriad manifestations. We present a case of a 31-year-old male who presented with abdominal pain, hypertensive emergency, and renal failure. Abdominal imaging demonstrated a left adrenal mass. Plasma metanephrines (153 pg/ml, n<57) and normetanephrines (1197 pg/ml, n<148) were noted to be elevated, leading to the diagnosis of pheochromocytoma. Intravenous antihypertensives were utilized to control his blood pressure. Hemodialysis was initiated given the degree of renal dysfunction. The patient subsequently developed hemolytic anemia, requiring the transfusion of multiple units of packed red cells. He developed acute respiratory failure leading to intubation, but was thereafter liberated from the ventilator following clinical stabilization. Uncontrolled hypertension precipitated by pheochromocytoma can cause microangiopathic hemolytic anemia and renal insufficiency. This case is notable not only for the occurrence of this rare presentation, but also for the severity of manifestations in a young male with no known significant comorbidities.
Acquired thrombotic thrombocytopenic purpura (TTP) is a rare thrombotic microangiopathy with varied etiology and manifestations. It is uncommon for acute pancreatitis to trigger TTP. A 59-year-old man hospitalized with acute pancreatitis developed fever, acute kidney injury, thrombocytopenia, and microangiopathic hemolytic anemia (MAHA) on his second day in the hospital. Based on clinical suspicion and a high PLASMIC score indicating a severe deficiency in ADAMTS13 (a disintegrin-like and metalloprotease with thrombospondin type 1 motif no. 13) activity, a presumptive diagnosis of TTP was made. He was treated with plasmapheresis with improvement in his hemoglobin and platelet count. Severely deficient ADAMTS13 activity causing accumulation of large von Willebrand factor (VWF) multimers and subsequent formation of platelet rich microthrombi are thought to be the mechanisms of development of TTP. Proinflammatory mediators released during the systemic inflammatory response seen in acute pancreatitis can promote VWF activity and inhibit ADAMTS13 activity. Diffuse endothelial injury as a result of the inhibition of vascular endothelial growth factor (VEGF)mediated endothelial protection as well as production of excessive reactive oxygen species during an episode of acute pancreatitis also contributes to the pathogenesis of TTP. Thrombocytopenia and MAHA in a systemic inflammatory state should raise the suspicion for TTP. The PLASMIC score can further aid in the diagnosis and early initiation of plasmapheresis, which is key to the outcome.
Tetanus is a life-threatening infectious neurological disorder that is now a rare disease due to the institution of wide-spread vaccination strategies. We present an uncommon case of generalized severe tetanus with consequent respiratory failure requiring mechanical ventilation, which was associated with dysautonomia. A 20-year-old unvaccinated female presented with neck stiffness and diffuse muscle spasms following a laceration sustained 3 weeks prior. She was admitted to the intensive care unit for mechanical ventilation and was treated with immunoglobulin, tetanus toxoid, metronidazole, and high doses of sedatives. She also developed dysautonomia, with alternating bradycardia and tachycardia, as well as fluctuating blood pressure. She was successfully extubated and discharged. We also review the epidemiology, pathophysiology, and management of tetanus and discuss dysautonomia in the setting of tetanus.
N-terminal pro-brain-type natriuretic peptide (NT-proBNP) is an established biomarker for pulmonary hypertension (PH), as an indicator of disease severity, and a predictor of mortality and morbidity. Red Cell Distribution Width (RDW), the measure of anisocytosis amongst red cells, is increasingly being realized to be a biomarker for various conditions. The purpose of this study was to assess the correlation between RDW and NT-proBNP in the setting of the new definition of PH as per the 6th World Symposium, which includes all patients with a mean pulmonary artery pressure (mPAP) of over 20 mm Hg. METHODS: Patients who underwent right heart catheterization (RHC) at our hospital system between November 2014 and November 2019 were included in this retrospective study. Exclusion criteria included patients under the age of 18, active malignancy, known hemoglobinopathy, blood transfusion within one month of laboratory testing, and a lack of RDW or NT-proBNP measured within 3 months of RHC. Hemodynamic parameters obtained on RHC were documented. The closest RDW and NT-proBNP values obtained within 3 months of the RHC were noted. Student's t-test was used to compare NT-proBNP between patients with elevated RDW (> 14.5%) and normal RDW (# 14.5%). Secondly, mean RDW was compared between elevated BNP ($ 300 pg/mL) and normal BNP (< 300 pg/mL). RESULTS: 231 patients were included in this study. 176 of them had RHC-proven PH. Amongst PH patients, there was a statistically significant significant difference in the mean NT-proBNP between the elevated RDW (> 14.5%, n ¼ 90) and the nonelevated RDW (# 14.5%, n ¼ 86) groups (7401.57 vs 3663.83 pg/mL, p ¼ 0.0076). Patients with elevated NT-proBNP ($ 300 pg/ mL, n ¼ 142) tended to have a higher RDW than those with normal BNP (< 300 pg/mL, n ¼ 34) (15.03 vs 14.36%, p ¼ 0.0264). These relationships held true when generalized to include patients without PH. The elevated RDW group (n ¼ 100) had a significantly higher NT-proBNP than the normal RDW group (n ¼ 131) (6866.19 vs 3136.6 pg/mL, p ¼ 0.002). The elevated NT-proBNP group (n ¼ 172) also had a higher RDW than the normal NT-proBNP group (n ¼ 69) (14.84 vs 13.96%, p ¼ 0.0003). CONCLUSIONS: NT-proBNP has previously been shown to be a valid biomarker for assessing disease severity, and to predict mortality and morbidity in patients with PH. The correlation between RDW and NT-proBNP in the setting of PH implies that RDW may also be a useful surrogate marker for disease severity in PH. CLINICAL IMPLICATIONS: RDW is a relatively cheap marker that could be used alongside other biomarkers such as NT-proBNP to measure the severity of disease in PH. Further studies are required to directly validate this relationship.
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