Diagnosis of thrombotic microangiopathy (TMA) is challenging due to its close association with other forms of microangiopathic hemolytic anemia, such as malignant hypertension and disseminated intravascular coagulation, and because other manifestations including cytopenia and acute kidney injury are manifestations of other medical comorbidities. Further challenges for accurate diagnosis include distinguishing between primary and secondary TMA, as well as between hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP). TTP is typically differentiated from HUS by the presence of more severe thrombocytopenia, along with a higher frequency of altered mental status with relatively preserved renal function. However, the clinical course can vary among patients, requiring polymerase chain reaction testing of patient stools for enterohemorrhagic Escherichia coli and a disintegrin and metalloproteinase with thrombospondin type 1 motif 13 (ADAMTS13) assay. To reduce the mortality rate, prompt initiation of plasmapheresis is important in cases where TPP cannot be excluded. Future advances enabling more rapid testing for ADAMTS13 levels will reduce the need for unnecessary plasmapheresis, so that treatment strategy can be more optimized.
Acute Kidney Injury ; Anemia, Hemolytic ; Comorbidity ; Diagnosis ; Diagnosis, Differential ; Disseminated Intravascular Coagulation ; Enterohemorrhagic Escherichia coli ; Hemolytic-Uremic Syndrome ; Humans ; Hypertension, Malignant ; Mortality ; Plasma Exchange ; Plasmapheresis ; Polymerase Chain Reaction ; Purpura, Thrombotic Thrombocytopenic ; Thrombocytopenia ; Thrombospondins ; Thrombotic Microangiopathies

Paraganglioma is an uncommon neuroendocrine tumor of cells that originate in the autonomic nervous system. Some paragangliomas have the ability to secrete catecholamines, similar to secretions in pheochromocytoma. For this reason, paragangliomas may cause malignant hypertension in patient, upon being administered anesthesia, or during surgery, this may lead to a life-threatening condition, despite the tumor having been diagnosed before conducting the procedure. Therefore, it is important to take adequate actions for reducing the occurrence of morbidity and mortality during surgery. Here, we describe a successful anesthetic management in a patient diagnosed with retroperitoneal paraganglioma invading the iliac bone.
Anesthesia ; Autonomic Nervous System ; Catecholamines ; Humans ; Hypertension, Malignant ; Mortality ; Neuroendocrine Tumors ; Paraganglioma* ; Pheochromocytoma

A 35-year-old man presented with progressive dyspnea and hemoptysis. His blood pressure was 230/140 mmHg and serum creatinine level was 20.13 mg/dL. Chest radiography and computed tomography revealed pulmonary hemorrhage. His renal function was low, thus emergent renal replacement therapy was required. Malignant hypertension and acute kidney injury were diagnosed, and antihypertensive therapy and hemodialysis started immediately. Renal biopsy was performed to examine the underlying disease. Typical pathological changes of malignant hypertension, fibrinoid necrosis of the afferent arterioles, and proliferative endoarteritis at the interlobular arteries were observed. His renal function improved gradually and pulmonary hemorrhage completely disappeared with administration of antihypertensive agents. Here, we report this rare case of malignant hypertension with pulmonary alveolar hemorrhage and speculate that the hemorrhage may be related to vascular injuries at the alveolar capillary level caused by malignant hypertension.
Acute Kidney Injury ; Adult ; Antihypertensive Agents ; Arteries ; Arterioles ; Biopsy ; Blood Pressure ; Capillaries ; Creatinine ; Dialysis* ; Dyspnea ; Hemoptysis ; Hemorrhage* ; Humans ; Hypertension, Malignant* ; Necrosis ; Pulmonary Alveoli ; Radiography ; Renal Dialysis ; Renal Replacement Therapy ; Thorax ; Vascular System Injuries

Midaortic syndrome (MAS) is a rare vascular disease that commonly causes renovascular hypertension. The lumen of the abdominal aorta narrows and the ostia of the branches show stenosis. MAS is associated with diminished pulses in the lower extremities compared with the upper extremities, severe hypertension with higher blood pressure in the upper rather than lower extremities, and an abdominal bruit. The clinical symptoms are variable, and recognition in children with hypertension can aid early diagnosis and optimal treatment. Hypertension with MAS is malignant and often refractory to several antihypertensive drugs. Recently, radiologic modalities have been developed and have led to numerous interventional procedures. We describe the case of a 3-year-old boy presenting with left ventricular hypertrophy whose severely elevated blood pressure led to the diagnosis of idiopathic MAS. This case highlights the importance of measuring blood pressure and conducting a detailed physical examination to diagnose MAS. This is the first reported case of idiopathic MAS diagnosed in childhood in Korea.
Antihypertensive Agents ; Aorta, Abdominal ; Aortic Coarctation ; Blood Pressure ; Child ; Child, Preschool* ; Constriction, Pathologic ; Diagnosis ; Early Diagnosis ; Humans ; Hypertension ; Hypertension, Malignant* ; Hypertension, Renovascular ; Hypertrophy, Left Ventricular ; Korea ; Lower Extremity ; Male* ; Physical Examination ; Renal Artery Obstruction ; Upper Extremity ; Vascular Diseases

Noonan syndrome is an autosomal dominant disorder characterized by dysmorphic facial features, congenital heart defects and short stature. To date, renal artery stenosis has not been associated with Noonan syndrome. We report the case of a 27-year old male who presented with malignant hypertension associated with renal artery stenosis, dysmorphic facial features, pectus excavatum, pulmonary stenosis and hypertrophic cardiomyopathy who was diagnosed with Noonan syndrome.
Adult* ; Balloon Valvuloplasty ; Cardiomyopathy, Hypertrophic ; Funnel Chest ; Heart Defects, Congenital ; Humans ; Hypertension, Malignant* ; Male ; Noonan Syndrome* ; Pulmonary Valve Stenosis ; Renal Artery Obstruction

Idiopathic membranous glomerulonephritis (IMGN) is commonly diagnosed in adults with proteinuria. Rapid deterioration of renal function is a rare complication of IMGN, except when accompanied by renal vein thrombosis, malignant hypertension, or other underlying disease, including lupus nephritis. Here, we present a case of rapid deterioration of renal function in a patient with MGN superimposed with anti-neutrophil cytoplasmic antibody (ANCA)-associated rapidly progressive crescentic glomerulonephritis (RPGN). Overall, about 20 cases of MGN with ANCA-associated RPGN have been reported. This case of biopsy-proven MGN with ANCA-associated RPGN is the first to be reported in Korea.
Adult ; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis ; Antibodies ; Antibodies, Antineutrophil Cytoplasmic ; Cytoplasm ; Glomerulonephritis ; Glomerulonephritis, Membranous ; Humans ; Hypertension, Malignant ; Korea ; Lupus Nephritis ; Membranes ; Proteinuria ; Renal Veins ; Thrombosis

PURPOSE: To evaluate changes in subfoveal choroidal thickness in patients with malignant hypertension. METHODS: A total of 12 eyes of six malignant hypertension patients were included in the present study. Intraocular pressure, blood pressure (systolic and diastolic), and choroidal thickness were measured before and after blood pressure control. Choroidal thickness was measured using enhanced depth imaging optical coherence tomography (EDI-OCT). The changes in choroidal extravascular density of the EDI-OCT image after blood pressure control were evaluated by comparing brightness values obtained with Adobe Photoshop software. RESULTS: The subfoveal choroidal thickness (SFCT) of malignant hypertension patients was 412.63 +/- 66.55 microm (mean +/- SD), which was thicker than in normal patients. After blood pressure control, SFCT decreased significantly to 356.96 +/- 59.08 microm (mean +/- SD) (p = 0.002). The choroidal extravascular density of the EDI-OCT image decreased after blood pressure control (p = 0.002), and the mean change was 17.21 +/- 7.56. CONCLUSIONS: The choroid is thickened in patients with malignant hypertension, and its thickness decreases after blood pressure control. This suggests that changes in blood pressure may affect choroidal thickness.
Blood Pressure ; Choroid* ; Humans ; Hypertension, Malignant* ; Hypertensive Retinopathy ; Intraocular Pressure ; Tomography, Optical Coherence

Hypertensive nephrosclerosis is usually associated with chronic hypertension, which increases the risk of progressive renal disease. Among the causes of malignant hypertension, thrombotic microangiopathy is complicated and is associated with renal dysfunction at the time of diagnosis. In this case, a young man with hypertension presented with renal failure and thrombocytopenia in the emergency department. This case emphasizes the importance of early recognition of renal failure and thrombocytopenia among patients with uncontrolled hypertension.
Blood Pressure* ; Diagnosis ; Emergency Service, Hospital ; Humans ; Hypertension ; Hypertension, Malignant ; Nephrosclerosis ; Renal Insufficiency ; Thrombocytopenia ; Thrombotic Microangiopathies*

Thrombotic microangiopathy (TMA) is a microvascular thrombotic lesion caused by endothelial injury and subsequent formation of platelet rich thrombus. TMA is first described as a classical pathologic feature of HUS/TTP. Renal biopsy finding of TMA represents kidney involvement of HUS/TTP as well as other diseases such as malignant hypertension, drug toxicity, eclampsia, pre-eclampsia, and several systemic infections. Autoimmune diseases and transplant kidney sometime also have TMA. It is needed to consider a complete autoimmune work-up of patients presenting with TMA including tests for ANA, ANCA, and ADAMTS13 inhibitory antibodies, because there are several reports of association with TMA in patients of SLE, anti-phospholipid syndrome, and ANCA-associated vasculitis.
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis ; Antibodies ; Antibodies, Antineutrophil Cytoplasmic ; Antiphospholipid Syndrome ; Autoimmune Diseases ; Biopsy ; Blood Platelets ; Drug Toxicity ; Eclampsia ; Female ; Humans ; Hypertension, Malignant ; Kidney ; Pre-Eclampsia ; Pregnancy ; Thrombosis ; Thrombotic Microangiopathies ; Transplants

Pheochromocytoma is a rare neuroendocrine tumor that is usually derived from adrenal medulla or chromaffin cells along with sympathetic ganglia. In Western countries, the prevalence of pheochromocytoma is estimated to be between 1:6,500 and 1:2,500, compared with an incidence in the United States of 500 to 1,100 cases per year. Despite this low incidence, pheochromocytoma should always be considered for differential diagnoses because previous studies have shown that this condition can be cured in approximately 90% of cases. However, an untreated tumor is likely to be fatal due to catecholamine-induced malignant hypertension, heart failure, myocardial infarction, stroke, ventricular arrhythmias or metastatic disease. Symptoms that result primarily from excess circulating catecholamines and hypertension include severe headaches, generalized inappropriate sweating and palpitations (with tachycardia or occasionally bradycardia). Pheochromocytoma, however, has highly variable and heterogeneous clinical manifestations, including fever, general weakness and dyspepsia, and can be observed in patients who are suffering from infectious diseases. Several of such case reports have been presented, but most of these included infectious patients with high blood pressure and severe fluctuations. In this study, we presented the case of a 53-year-old male who showed normal blood pressure, but had a sustained fever. He was diagnosed with diabetic ketoacidosis, infective endocarditis and asymptomatic adrenal incidentaloma. Despite treatment with antibiotics and valve replacement, the fever persisted. After the patient underwent evaluation for the fever, adrenal incidentaloma was identified as pheochromocytoma. After removal of the abdominal mass, his fever improved.
Adrenal Gland Neoplasms ; Adrenal Medulla ; Anti-Bacterial Agents ; Arrhythmias, Cardiac ; Blood Pressure ; Catecholamines ; Chromaffin Cells ; Communicable Diseases ; Diabetic Ketoacidosis ; Diagnosis, Differential ; Dyspepsia ; Endocarditis ; Fever ; Ganglia, Sympathetic ; Headache ; Heart Failure ; Humans ; Hypertension ; Hypertension, Malignant ; Incidence ; Male ; Middle Aged ; Myocardial Infarction ; Neuroendocrine Tumors ; Pheochromocytoma ; Prevalence ; Stress, Psychological ; Stroke ; Sweat ; Sweating ; Tachycardia ; United States