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.
ACE inhibitors are widely used and well-tolerated drugs. Angioedema is a well-known adverse effect, which involves the viscera rarely. This is a case of a 44-year-old African-American man with newly diagnosed hypertension, who presented with lower abdominal pain and diarrhoea. Based on the clinical picture and radiographic findings, lisinopril-induced intestinal angioedema was diagnosed. He recovered with supportive treatment, and the lisinopril was permanently discontinued. The mechanism of angioedema is thought to be the inhibition of ACE-mediated degradation of bradykinin, which is a peptide responsible for vasodilation and increased vascular permeability. While the external angioedema is unmistakable, intestinal angioedema has a relatively non-specific presentation and chronology, often leading to missed diagnosis and unnecessary interventions. Most common symptoms are abdominal pain and diarrhoea. Characteristic radiographic findings include ‘doughnut sign’ and ‘stacked coin’ appearance. Treatment is supportive. ACE inhibitors should be discontinued to prevent a recurrence.
With more women getting pregnant at later ages than in the past, the incidence of malignancies in pregnancy is on the rise. Common malignancies of pregnancy are melanoma, breast cancer, cervical cancer, lymphomas, and leukemias. Colorectal carcinoma is rare in pregnancy, with an estimated incidence of 1 in 13',000 cases. We describe such a case of colorectal carcinoma in pregnancy (CRC-p), in a 31-year-old patient. She presented in the 21 st week of her second pregnancy with constipation of two weeks duration despite appropriate medical management. This prompted further evaluation with abdominal imaging revealing partial small bowel obstruction of unclear etiology. She was treated surgically with subtotal colectomy with ileostomy. Pathologic evaluation revealed Stage III B: pT3N2a adenocarcinoma with mucinous features of the sigmoid colon with lymph node metastases. Adjuvant FOLFOX chemotherapy was started in the third trimester and was continued postpartum for a total of 12 cycles. She is doing well, and ileostomy reversal is being planned at the time of writing this. Advancing maternal age is a significant risk factor for CRC-p. Common presenting symptoms in CRC-p include bleeding per rectum, abdominal pain, vomiting, and constipation. The frequent occurrence of many of these symptoms, as well as risks and restrictions associated with diagnostic modalities such as computed tomography scan and colonoscopy during pregnancy, makes the diagnosis challenging. Colonoscopy, followed by pathology evaluation, remains the standard diagnostic method in CRC-p. Management of CRC-p is determined by multiple variables such as the stage of the disease, gestational age, and most importantly, patient wishes. Surgical resection is performed following the diagnosis if the gestational age is less than 20 weeks and delayed until after delivery if gestational age is above 20 weeks. 5fluorouracil based chemotherapy regimens are used in second and third trimesters, in patients with stage III CRC-p. Prognosis has been reported variably. Despite advanced stages at presentation, most of the studies indicate a similar prognosis compared to CRC in the nonpregnant population. Two-year survival was found to be 64.4% in one case series.
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