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A 33-year-old woman visited our hospital due to visual loss. Her BP was 280/150 mm Hg and pulse rate was 111 beats per minute. A urinalysis showed protein in urine, suggesting kidney injury. A transthoracic echocardiography showed left ventricular hypertrophy. A Cardiac magnetic resonance imaging suggested left ventricular endocardial edema or inflammation. Ophthalmoscopy showed optic disc edema and hard exudates in both eyes. A brain MRI showed multiple high-intensity areas at the pons and white matter of the cerebrum and cerebellum. Although the patient had malignant hypertension, she was successfully treated by medication.

Introduction Atypical hemolytic uremic syndrome (aHUS) is a rare form of thrombotic microangiopathy (TMA) often caused by alternative complement dysregulation. Patients with aHUS can present with malignant hypertension (MHT), which may also cause TMA. Methods This analysis of the Global aHUS Registry (NCT01522183) assessed demographics and clinical characteristics in eculizumab-treated and not-treated patients with aHUS, with (n = 71) and without (n = 1026) malignant hypertension, to further elucidate the potential relationship between aHUS and malignant hypertension. Results While demographics were similar, patients with aHUS + malignant hypertension had an increased need for renal replacement therapy, including kidney transplantation (47% vs 32%), and more pathogenic variants/anti-complement factor H antibodies (56% vs 37%) than those without malignant hypertension. Not-treated patients with malignant hypertension had the highest incidence of variants/antibodies (65%) and a greater need for kidney transplantation than treated patients with malignant hypertension (65% vs none). In a multivariate analysis, the risk of end-stage kidney disease or death was similar between not-treated patients irrespective of malignant hypertension and was significantly reduced in treated vs not-treated patients with aHUS + malignant hypertension (adjusted HR (95% CI), 0.11 [0.01–0.87], P = 0.036). Conclusions These results confirm the high severity and poor prognosis of untreated aHUS and suggest that eculizumab is effective in patients with aHUS ± malignant hypertension. Furthermore, these data highlight the importance of accurate, timely diagnosis and treatment in these populations and support consideration of aHUS in patients with malignant hypertension and TMA. Trial registration details Atypical Hemolytic-Uremic Syndrome (aHUS) Registry. Registry number: NCT01522183 (first listed 31st January, 2012; start date 30th April, 2012). Graphical abstract

Abstract In malignant hypertension, far more severe kidney injury occurs than in the “benign” form of the disease. The role of high blood pressure and the renin–angiotensin–aldosterone system is well recognized, but the pathogenesis of the renal injury of malignant hypertension (MH) remains incompletely understood. Using the rat model of two-kidney, one-clip renovascular hypertension in which some but not all animals develop MH, we performed a transcriptomic analysis of gene expression by RNA sequencing to identify transcriptional changes in the kidney cortex specific for MH. Differential gene expression was assessed in three groups: MH, non-malignant hypertension (NMH), and normotensive, sham-operated controls. To distinguish MH from NMH, we considered two factors: weight loss and typical renovascular lesions. Mean blood pressure measured intraarterially was elevated in MH (220 ± 6.5 mmHg) as well as in NMH (192 ± 6.4 mmHg), compared to controls (119 ± 1.7 mmHg, p Key messages The most severe hypertension-induced kidney injury occurs in malignant hypertension. In a rat model of malignant hypertension, we assessed transcriptional responses in the kidney exposed to high blood pressure. A broad stimulation of inflammatory mechanisms was observed, but a few specific pathways were activated only in the malignant form of the disease, notably activation of the complement cascades. Complement inhibitors may alleviate the thrombotic microangiopathy of malignant hypertension even in the absence of primary complement abnormalities.

Background Thrombotic microangiopathy (TMA) is a complication of malignant hypertension (mHTN) attributed to high blood pressure (BP). However, no studies have investigated in patients with mHTN of different aetiologies whether the presence of TMA is associated with specific causes of mHTN. Methods We investigated the presence of TMA (microangiopathic haemolytic anaemia and thrombocytopenia) in a large and well-characterized cohort of 199 patients with mHTN of different aetiologies [primary HTN 44%, glomerular diseases 16.6%, primary atypical haemolytic uraemic syndrome (aHUS) 13.1%, renovascular HTN 9.5%, drug-related HTN 7%, systemic diseases 5.5%, endocrine diseases 4.5%]. Outcomes of the study were kidney recovery and kidney failure. Results Patients with TMA [40 cases (20.1%)] were younger, were more likely female and had lower BP levels and worse kidney function at presentation. Their underlying diseases were primary aHUS (60%), drug-related mHTN (15%), glomerular diseases [all of them immunoglobulin A nephropathy (IgAN); 10%], systemic diseases (10%) and primary HTN (5%). The presence of TMA was 92.3% in primary aHUS, 42.9% in drug-related HTN, 36.4% in systemic diseases, 12.1% in glomerular diseases and 2.3% in primary HTN. No patient with renovascular HTN or mHTN caused by endocrine diseases developed TMA, despite BP levels as high as patients with TMA. A higher proportion of TMA patients developed kidney failure as compared with patients without TMA (56.4% versus 38.9%, respectively). Conclusions The presence of TMA in patients with mHTN should guide the diagnosis towards primary aHUS, drug-related mHTN, some systemic diseases and IgAN, while it is exceptional in other causes of mHTN.

Malignant hypertensive retinopathy is associated with characteristic fundus findings that typically do not include proliferative retinal vascular changes. We present the case of a 34-year-old patient who had bilateral decreased vision and was found to have malignant hypertension with hypertensive retinopathy changes along with unforeseen bilateral neovascularization and vitreous hemorrhage. Detailed history and extensive systemic and ophthalmic workup failed to reveal an alternative explanation for her proliferative retinopathy. Blood pressure control and panretinal photocoagulation halted further deterioration. Malignant hypertensive retinopathy can rarely cause profound retinal ischemia leading to retinal neovascularization. This case further supports the presence of “proliferative hypertensive retinopathy” that needs to be identified and addressed urgently through collaboration between internists and ophthalmologists.

Malignant hypertension is a term that has been used to describe patients with high blood pressure (BP) and multiple complications (target organ damage) with a poor prognosis. Today, the term hypertensive crisis is used to describe patients who have severe BP elevations as follows: Systolic blood pressure (SBP) greater than 180 mmHg and diastolic blood pressure (DBP) greater than 120 mm Hg. The present study aims to analyze the pathophysiology of malignant hypertension. This is a study with a qualitative approach, where the integrative literature review was adopted, which according to Galvão (2012), is a construction of a broad analysis of the literature with pre-defined steps. Conducted through the Virtual Health Library (BVS) in Pubmed databases, using the crossings of the English descriptors “malignant hypertension”, “diagnosis”. Of the different types of hypertensive emergencies, malignant hypertension (MTH) is characterized by extreme elevations in BP and acute microvascular damage that affects various organs, in particular the retina, brain, and kidneys. HTM is likely to be underdiagnosed, being classified as a hypertensive emergency or unattended for several reasons. Malignant hypertension is a systemic disease that causes severe damage to the brain, heart, kidneys, and eyes. Blockers of the renin-angiotensin system appear to be the mainstay of treatment. Management of the clinical management of hypertension through consensus gained from clinical experience and evidence of inadequate quality is required. La hipertensión maligna es un término que se ha utilizado para describir a los pacientes con presión arterial (PA) alta y múltiples complicaciones (daño de órganos diana) con un mal pronóstico. Hoy en día, el término crisis hipertensiva se utiliza para describir a los pacientes que tienen elevaciones graves de la PA de la siguiente manera: presión arterial sistólica (PAS) superior a 180 mmHg y presión arterial diastólica (PAD) superior a 120 mmHg. El presente estudio tiene como objetivo analizar la fisiopatología de la hipertensión maligna. Se trata de un estudio con abordaje cualitativo, donde se adoptó la revisión integrativa de la literatura, que según Galvão (2012), es una construcción de un amplio análisis de la literatura con pasos predefinidos. Realizado a través de la Biblioteca Virtual en Salud (BVS) en las bases de datos Pubmed, utilizando los cruces de los descriptores en inglés “hipertensión maligna”, “diagnóstico”. De los diferentes tipos de emergencias hipertensivas, la hipertensión maligna (HTM) se caracteriza por elevaciones extremas de la PA y daño microvascular agudo que afecta a varios órganos, en particular la retina, el cerebro y los riñones. Es probable que la HTM esté infradiagnosticada, clasificándose como una emergencia hipertensiva o desatendida por varias razones. La hipertensión maligna es una enfermedad sistémica que provoca graves daños en el cerebro, el corazón, los riñones y los ojos. Los bloqueadores del sistema renina-angiotensina parecen ser la base del tratamiento. Se requiere manejo del manejo clínico de la hipertensión a través del consenso obtenido de la experiencia clínica y evidencia de calidad inadecuada. A hipertensão maligna é um termo que tem sido utilizado para descrever pacientes com pressão arterial (PA) elevada e múltiplas complicações (lesão de órgão-alvo) com prognóstico ruim. Hoje, o termo crise hipertensivo é empregado para descrever pacientes que apresentam elevações graves da PA da seguinte forma: Pressão arterial sistólica (PAS) maior que 180 mmHg e pressão arterial diastólica (PAD) maior que 120 mm Hg. O presente estudo tem como objetivo analisar a fisiopatologia da hipertensão maligna. Trata-se de um estudo de abordagem qualitativa, onde se adotou a revisão integrativa da literatura, que conforme Galvão (2012), é uma construção de uma análise ampla da literatura com passos pré-definidos. Realizado através da Biblioteca Virtual de Saúde (BVS) nas bases de dados do Pubmed, usando os cruzamentos dos descritores em inglês “malignant hypertension”, “diagnosis”. Dos diferentes tipos de emergências hipertensivas, a hipertensão maligna (HTM) é caracterizada por elevações extremas da PA e danos microvasculares agudos que afetam vários órgãos, em particular a retina, o cérebro e os rins. A HTM provavelmente é subdiagnosticada, sendo classificada como emergência hipertensiva ou não atendida por vários motivos. A hipertensão maligna é uma doença sistêmica que causa graves danos ao cérebro, coração, rins e olhos. Os bloqueadores do sistema renina-angiotensina parecem ser a base do tratamento. É necessária uma gestão do manejo clínico da hipertensão por meio do consenso obtido a partir de experiência clínica e evidências de qualidade inadequada.

Background Malignant hypertension is a condition characterized by severe hypertension and multiorganic ischemic complications. The underlying cause of malignant hypertension can be primary or secondary hypertension, and identification of the cause is mandatory to select the correct treatment to control blood pressure and reduce end-organ damage. Hypertensive retinopathy is a disease that has short-term and long-term consequences for the overall health and mortality of patients. Objective The aim of this article is to present a case of malignant hypertension and hypertnive maculopathy detected in a female patient as well as the positive response that occurs after administration of only one ampoule of triamcinolone subtenonially in two doses in monthly intervals. Case report A 50-year-old patient comes to our clinic for an ophthalmological consultation after noticing impaired vision back a few months, especially noticeable on her left eye. Examination of the fundus visualizes PNO of unclear boundaries, especially in the inferior quadrants, mottled hemorrhages localized around the neuroretinal rim, edematous macula with hard deposits in the form of stelatae primarily on the left eye, and cotton wool exudates on the retina on both sides, nevus chorioidee of the right eye. Blood vessels hypertonically altered. On the OCT images of the macula, neurosensory retinal ablations are observed on both sides, intraretinal fluid with intraretinal hard deposits is present, more pronounced on the left. The seriousness of the condition is explained to the patient, as well as that in this state it requires urgent hospitalization, therapy is prescribed in the form of carbonic anhydrase inhibitors to preserve the macula, and a treatment algorithm is arranged in the form of subtenonial application of triamcinolone after dehospitalization. At check-ups, there is a subjective and objective positive shift in the patient's condition. Conclusion The presence of hypertensive retinopathy may help to stratify the patient when assessing the future risk of stroke, coronary artery disease, and heart and kidney failure, even if the hypertension is well controlled.There is also a need to emphasize the obligatory symbiosis of internal medicine and ophthalmological disciplines, as well as to point out the fact that triamcinolone, as one of the very available and more affordable drugs, very effectively helps in the algorithm of treatment of these patients.

Malignant hypertension is a hypertensive emergency, with rapid disease progression and poor prognosis. Although recognized as a separate entity more than a century ago, significant knowledge gaps remain about its pathogenesis and treatment. This narrative review summarizes current viewpoints, research gaps, and challenges with a view to pooling future efforts at improving treatment and prognosis.

Thrombotic thrombocytopenic purpura (TTP) is a type of thrombotic microangiopathy (TMA) related to a deficiency of ADAMTS13 protein, which could lead to fatal outcomes. TTP presents a vast array of symptoms, making its diagnosis a challenge to physicians. In this report, we discuss the case of an 80-year-old male who presented with abdominal pain and hypertension with underlying severe thrombocytopenia and hemolysis. Although his presentation could also be secondary to malignant hypertension, he was promptly diagnosed with TTP based on these findings. TTP is a hematologic emergency, and it should be kept in the differential diagnosis when a patient presents with thrombocytopenia and hemolysis with or without accompanying symptoms.

Background Acute DeBakey type I and type II aortic dissections are indications for emergent surgical repair; however, there are currently no standard protocols in the management of isolated supra-aortic dissections. Prompt diagnosis and management of an isolated innominate artery dissection are necessary to prevent distal malperfusion and thromboembolic sequelae. Case presentation A 50-year-old Caucasian gentleman presented with chest pain radiating to his jaw and right arm. He had no recent history of trauma. On physical exam, he was neurologically intact and malignantly hypertensive. Computed tomographic angiography of the chest and neck confirmed a spontaneous isolated innominate artery dissection without ascending aorta involvement. Given the lack of evidence for rupture, distal emboli, and/or end-organ malperfusion, the decision was made for initial non-operative management—anti-impulse regimen, antiplatelet therapy, and close follow-up. Conclusions Medical management of a spontaneous isolated innominate artery dissection is appropriate for short-term and potentially long-term therapy. This not only spares the patient from a potentially unnecessary surgical operation but also provides the surgeon and the patient the time to plan for a surgical approach if it becomes necessary.

Malignant hypertension is a rare condition characterized by severe hypertension and multi-organ ischemic damage. Marked activation of renin-angiotensin system is observed in many patients, but its persistence over time is not known. We report a case of 42-year-old woman presented with severe hypertension and multi-organ damage. Initial evaluation showed elevated value of direct renin concentration with normal plasma aldosterone concentration, and a nodular lesion in the left adrenal gland. The differential diagnosis between primary and secondary form of hypertension had to be questioned. Consequently the patient was followed up for 20 months. Repeated checks showed a significant increase in renin levels with a normal aldosterone concentration and regression of organ damage. After 20 months renin values returned within normal limits. Hyperreninemia persisting over a long period of time has not been fully explained. Long term follow-up allowed us to attribute malignant hypertension to de novo essential hypertension.

Systemic hypertension is related to a variety of retinal manifestations. Reported herein is a case of malignant hypertension associated with bilateral foveal neurosensory detachment in a middle age woman with stenosis of the left renal artery. The present case outlines the importance of recognizing ocular manifestations as a possible consequence of life-threatening systemic disease.

Objective Thrombotic microangiopathy (TMA) is often first detected on a renal biopsy performed for renal manifestations. Apart from hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura, there are various secondary conditions associated with TMA. This study analyzes the clinico-pathological spectrum, etiological factors and renal outcome of TMA diagnosed on renal biopsy. Material and method A retrospective evaluation of renal biopsies for TMA over 5.5 years was performed. Clinical and laboratory data was collected from patient records. Results A total of 40 biopsies from 39 patients showed TMA comprising 33 native and 7 transplant biopsies. Malignant hypertension (n=13) was the most common etiology in native biopsies followed by postpartum TMA (n=7), atypical HUS (aHUS) (n=7), and lupus nephritis (n=6). TMA in transplant biopsies was due to acute rejection (n=4) and CNI toxicity (n=3). Serum creatinine was high in most patients (mean 5.6 + 2.5 mg/ dl). aHUS showed the highest mean LDH levels and the lowest average platelet counts. Renal biopsies in malignant hypertension and postpartum TMA showed isolated arterial changes while aHUS and lupus nephritis showed both glomerular and arterial involvement. Postpartum TMA and aHUS had poor renal outcome requiring renal replacement therapy. Conclusion Most postpartum TMA and aHUS had systemic features of TMA while malignant hypertension and lupus nephritis showed 'isolated renal TMA'. This emphasizes the importance of careful evaluation of renal biopsies even in the absence of systemic features of TMA.

BACKGROUND Malignant hypertension is macrovascular and microvascular endothelial injury responsible for multiple organ damage. Considering the anatomical and functional homologies between the posterior pole of the eye and the kidney, ophthalmological explorations may inform clinicians on the mechanisms underpinning concurrent kidney injury in this condition. More specifically, we investigated whether the wall-to-lumen ratio (WLR) of retinal arterioles measured by adaptive optics ophthalmoscopy could be correlated to WLR of kidney arterioles as determined by pathology. We sought to estimate the incidence of retinal arteriole occlusion a supposedly uncommon complication of malignant hypertension. METHODS All patients hospitalized in our renal Intensive Care Unit for malignant hypertension between 2016 and 2019 were referred to ophthalmological examinations. RESULTS Twenty-seven patients were included. Median retinal WLR was 0.39 [0.31–0.47] and was correlated with initial systolic (r = 0.56, P = 0.003) and mean blood pressure (r = 0.46, P = 0.02) upon admission. The retinal WLR was not correlated to renal pathological findings, as assessed by juxtaglomerular WLR (r = 0.38, P = 0.2), ratio of glomerulosclerosis (r = −0.39, P = 0.2), or tubulointerstitial fibrosis (r = −0.45, P = 0.08). Retinal WLR was not associated with neurological or cardiovascular end-organ damage. Branch retinal artery occlusion was detected in 18.5% of patients and exudative retinal detachment (ERD) in 29.6% of patients, without any significant correlation with canonical signs of retinal hypertension including optic disc swelling. CONCLUSIONS In the setting of malignant hypertension, we failed to demonstrate a significant relationship between WLR and other meaningful end-organ injuries. However, branch retinal artery occlusion and ERD may have been hitherto underestimated.

Background Axillary artery access is rarely used for demanding percutaneous transcatheter interventions. However, there are many clear advantages. Case summary We describe this attractive approach in a 3-week-old premature neonate (bodyweight of 1.2 kg) with severe aortic coarctation. Percutaneous transcatheter intervention was performed with analgo-sedation and local anaesthesia; and a coronary stent was placed with a low fluoroscopy time of 2 min. Malignant systemic hypertension (160/54 mmHg) was effectively treated without any residual blood pressure gradient, with the aim for definitive surgery with stent resection and end-to-end anastomosis at the age of 6–12 months. Discussion Axillary artery access is an attractive, alternative approach to treat newborns and premature infants with low body weight with complex heart diseases.

Malignant hypertension (hypertensive emergency), is an extreme elevation of blood pressure under certain conditions that can lead to organ damage and other serious consequences. It is a common condition that affects about one in three Americans, according to the Centers for Disease Control and Prevention. An elevation of systolic blood pressure above 180 mmHg and diastolic blood pressure above 120 mmHg is considered a hypertensive emergency. This article addresses the case of a 61-year-old female patient who presented to the ER with a semicomatose and gasping condition and response to painful stimuli with an unclear voice. She also had unstable vital signs, with a blood pressure of 370/200 mmHg, a pulse rate of 115, a respiratory rate of 22, and a pulse oximetry of 96%, but no fever. Her son provided a brief history and reported that a stressful condition had occurred at home one hour before; she had begun to scream and been brought to the ER by ambulance in the condition described above. Cardiac monitoring and an electrocardiograph were performed and indicated a normal condition besides the unstable vital signs. Oxygen was administered via a nasal cannula with 20 mg of intravenous hydralazine, and the patient’s blood pressure improved progressively. Moreover, she regained consciousness with no end-organ damage. A hypertensive emergency is usually associated with end-organ damage, such as heart, kidney, eye, or brain damage. However, in this case, despite the extreme elevation of her blood pressure, the patient suffered no organ damage. It is essential to manage the extreme elevation of blood pressure as soon as possible and monitor the patient for consequences.

Introduction: Thrombotic microangiopathy (TMA) is characterized by endothelial injury followed by formation of multiple microthrombi in the target organs due to various causes, including malignant hypertension (MHT). Here, we reported a rare case of MHT with splenic TMA changes. Case concerns: A 27-year-old Chinese Han male with a history of hypertension and proteinuria, admitted to our hospital because of renal failure with MHT and thrombocytopenia. Diagnosis: This case diagnosed with TMA based on the patient's MHT and thrombocytopenia. The patient final diagnosis was confirmed by the spleen pathological findings, other differential diagnoses were ruled out. Interventions: The patient was treated with hemodialysis and intravenous antihypertensive agents, and his condition gradually improved. However, he suddenly complained of abdominal pain and went into hemorrhagic shock, which was due to spontaneous spleen rupture on the third day of hospitalization. The pathological evidence after splenectomy showed splenic TMA. Outcomes: Hemodialysis was continued and the blood pressure was under control until his discharge from our hospital. Conclusion: Spontaneous splenic rupture could be a rare and critical complication associated with MHT-induced TMA, and it requires careful clinical attention.

Hypertensive emergencies are those situations where very high blood pressure (BP) values are associated with acute organ damage, and therefore, require immediate, but careful, BP reduction. The type of acute organ damage is the principal determinant of: (i) the drug of choice, (ii) the target BP, and (iii) the timeframe in which BP should be lowered. Key target organs are the heart, retina, brain, kidneys, and large arteries. Patients who lack acute hypertension-mediated end organ damage do not have a hypertensive emergency and can usually be treated with oral BP-lowering agents and usually discharged after a brief period of observation.

Purpose.To assess the state of blood flow in retrobulbar vessels using the method of color Doppler imaging in thrombotic microangiopathy (TMA) associated with atypical hemolytic uremic syndrome (aHUS), malignant hypertension (MH) and catastrophic antiphospholipid syndrome (CAPS).Methods. The study involved 16 patients aged from 18 to 43 years with TMA associated with aHUS (13 patients), MH (2 patients) and CAPS (1 patient). All patients underwent a study of the state of blood flow in the vessels of the retrobulbar space by color Doppler imaging using a multifunctional ultrasonic diagnostic device Voluson 730 Pro and Voluson E8. The spectrum of blood flow in the ophthalmic artery (OA), central retinal artery (CRA), central retinal vein (CRV), in the medial and lateral short posterior ciliary arteries (SPCA) was recorded and the following parameters were determined: maximum systolic velocity (Vsyst), final diastolic velocity (Vdiast), peripheral resistance index (RI), pulsation index (PI).Results. The analysis of the Doppler blood flow spectrum in all patients with TMA syndrome revealed a decrease in Vsyst in the vessels of the retrobulbar space, more pronounced in the CRA, with an increase in the venous component of the Doppler spectrum. In patients with TMA associated with aHUS and MH Vdiast in the studied vessels were in normal limits or increased, RI and PI were reduced. In aHUS a statistically significant correlation was found between Vdiast in OA and the level of haptoglobin in blood serum, which is a classic marker of intravascular hemolysis.Conclusion. Color Doppler imaging of retrobulbar vessels is an important method for the diagnosis of ocular hemodynamics disorders in the syndrome of TMA. All patients with TMA syndrome are characterized with a decrease in the systolic component of the Doppler spectrum of blood flow in the vessels of the retrobulbar space, especially in the CRA, with an increase in the venous component. With aHUS and MH diastolic blood flow in the retrobulbar vessels corresponds to the norm or increases, RI and PI decrease. In TMA associated with aHUS, the final diastolic velocity of blood flow in OA depends on the level of microvascular hemolysis.

Background Severe hypertension can induce thrombotic microangiopathy (TMA) in the renal vasculature, the occurrence of which has been linked to mechanical stress to the endothelium. Complement defects may be the culprit of disease in patients who present with severe renal disease and often progress to ESRD, despite BP control. Methods We studied a well defined cohort of 17 patients with hypertension-associated TMA to define the prevalence of complement defects by a specific ex vivo serum-based microvascular endothelial cell assay. Results Compared with normal human serum and samples from patients with hypertensive arterionephrosclerosis, 14 of 16 (87.5%) serum samples collected at presentation from 16 patients with hypertension-associated TMA induced abnormal C5b9 formation on microvascular endothelial cells. We detected rare variants in complement genes in eight of 17 (47%) patients. ESRD occurred in 14 of 17 (82%) patients, and recurrent TMA after transplant occurred in seven of 11 (64%) donor kidneys. Eculizumab improved the renal function in three patients and prevented TMA recurrence in an allograft recipient. Conclusions These observations point to complement defects as the key causative factor of ESRD and recurrent TMA after transplant in patients presenting with severe hypertension. Complement defects can be identified by measurements of complement activation on microvascular endothelial cells, which should substantially influence treatment and prognosis.

Hypertensive heart disease with biventricular cardiac failure is not common in clinical practice. This diagnosis requires an extensive diagnostic search. We present the clinical case of the male patient of 38 aged. He was admitted to the clinic with heart failure 3-4 NYHA class. EchoCG revealed symmetric hypertrophy of the left ventricle up to 18 mm without its dilatation, a decrease in ejection fraction up to 42%, restrictive hemodynamics, overload of the right chambers, severe pulmonary hypertension (60 mm Hg). The clinical status included persistent arterial hypertension (180-220 and 120-150 mm Hg), effusion in both pleural cavities and pericardium, ascites, renal failure. During examination (multispiral computed tomography, magnetic resonance imaging, scintigraphy with 131I-MIBG), bladder paraganglioma was diagnosed (normatenafrin 1468 μg/day). The resection of the tumor was performed, according to immunohistochemical research – neuroendocrine carcinoma, G1. After 3 months a partial regression of hypertension and cardiac failure was observed with the preservation of a high level of creatinine. The criteria and differential diagnosis of the hypertensive heart disease and the syndrome of primary myocardial hypertrophy, diagnostics of the urinary bladder paraganglioma, complex mechanisms of myocardial damage within the pheochromocytoma and its prognosis are discussed.

Thrombotic microangiopathy (TMA) is a pattern of endothelial damage that can be found in association with diverse clinical conditions such as malignant hypertension. Although the pathophysiological mechanisms differ, accumulating evidence links complement dysregulation to various TMA syndromes and in particular the atypical hemolytic uremic syndrome. Here, we evaluated the role of complement in nine consecutive patients with biopsy-proven renal TMA attributed to severe hypertension. Profound hematologic symptoms of TMA were uncommon. In six Out of nine patients, we found mutations C3 in three, CFI in one, CD46 in one, and/or CFH in two patients either with or without the risk CFH-H3 haplotype in four patients. Elevated levels of the soluble C5b-9 and renal deposits of C3c and C5b-9 along the vasculature and/or glomerular capillary wall, confirmed complement activation in vivo. In contrast to patients without genetic defects, patients with complement defects invariably progressed to end-stage renal disease, and disease recurrence after kidney transplantation seems common. Thus, a subset of patients with hypertension-associated TMA falls within the spectrum of complement-mediated TMA, the prognosis of which is poor. Hence, testing for genetic complement abnormalities is warranted in patients with severe hypertension and TMA on renal biopsy to adopt suitable treatment options and prophylactic measures.

Introduction: Two major product groups originate from the arachidonic acid metabolic pathway of cytochromes P450: epoxyeicosatrienoic acid (EETs) and 19 and 20-hydroxyeicosatetraenoic acid (19- and 20-HETE). These metabolites play an important role in the regulation of blood pressure, inflammatory responses, regulation of sodium excretion and other crucial physiological processes. Hypothesis: Our studies were based on the hypothesis that abnormalities in the production and function of these cytochrome P450 metabolites significantly contribute to the pathophysiology of hypertension development, in particular in the angiotensin II-dependent models. Objective: To investigate if the increased bioavailability of the above-mentioned metabolites in the kidney tissue will result in blood pressure reduction in the ANG II - dependent rat model of hypertension. Methods: The two methods to increase the concentration of EETs was chosen. In the first part of the study, we administered a soluble epoxide hydrolase inhibitor cAUCB [cis-4- [4- (3-adamantan-1-yl- ureido) cyclohexyloxy] benzoic acid, at a dose of 26 mg.l-1 administered in drinking water], an enzyme responsible for inactivation of biologically active forms of EETs. In the second series of the experiments we applied a synthetic EET analogue, called...

Context: cardiac damage is now recognized as part of the definition of malignant hypertension. It can manifest itself as troponin elevation, systolic dysfunction or disproportionate left ventricular hypertrophy. However, its pathogenesis remains uncertain. Cardiac MRI seems to be an ideal tool to progress in the understanding of its pathophysiology. Method: we retrospectively included patients from the Bordeaux cohort of malignant hypertension who received cardiac MRI between January 2009 and January 2020. MRIs were considered acute if the post-diagnosis delay was less than or equal to 40 days. We collected telediastolic, telestolic, LVEF, left ventricular mass, segmental kinetics from SSFP sequences, and the presence or absence of pathological late enhancement 10 minutes after injection of gadolinium chelate by a PSIR sequence (± HR). The most recent cardiac MRIs included T2-weighted and T1 mapping sequences. Results: we included 39 patients out of the 202 present in the Bordeaux cohort. The mean time to complete cardiac MRI was 20.9 days (±17.8). 33 patients received an acute MRI, performed with a mean time of 14.8 days (±10.3). MRI data in this subgroup showed a mean indexed LVM of 127.1 g/m2 (±41.8). There were no significant anomalies on the T2-weighted sequences. The mean VEC was 24.4 (± 2.6%). Late enhancement abnormalities were found in 34.4% of patients, mostly of mesomyocardial location. Only two patients had subendocardial elevation without a context suggestive of acute coronary syndrome. Follow-up MRI was performed in 18 of these patients with a significant decrease in indexed LVM (92.8 ± 20.1gr/m2 (p < 0.05)) and a significant increase in ECV (26.7 ± 2.6% (p = 0.049)). Conclusion: these data suggest that the disproportionate LVH sometimes observed in the acute phase of malignant hypertension appears to be secondary to a mixed mechanism. The latter would associate myocyte hyperplasia and excessive fibrotic damage, probably due to over activation of the ARAS. The absence of ischemic sequelae on the late enhancement sequences suggests a diffuse microvascular cause explaining myocardial ischemia, a hypothesis consistent with the pathophysiology of the disease., Contexte : l'atteinte cardiaque est aujourd'hui reconnue dans le cadre de la définition de l'HTA maligne. Elle peut se manifester par une élévation de troponine, une dysfonction systolique ou encore une hypertrophie ventriculaire gauche disproportionnée. Sa pathogénèse reste néanmoins incertaine. L'IRM cardiaque semble un outil idéal pour progresser dans la compréhension de sa physiopathologie. Méthode : nous avons inclus rétrospectivement les patients de la cohorte bordelaise d'HTA maligne ayant bénéficié d’une IRM cardiaque entre janvier 2009 et janvier 2020. Les IRM étaient considérées en phase aiguë si le délai de réalisation post diagnostic était inférieur ou égal à 40 jours. Nous avons recueilli les volumes télédiastolique, télésystolique, la FEVG, la masse ventriculaire gauche, la cinétique segmentaire à partir de séquences SSFP, et la présence ou non de rehaussement tardif pathologique 10 minutes après injection de chélate de gadolinium par une séquence PSIR (± HR). Les IRM cardiaques les plus récentes incluaient des séquences pondérées T2 et de T1 mapping. Résultats : nous avons inclus 39 patients sur les 202 présents dans la cohorte bordelaise. Le délai moyen de réalisation de l’IRM cardiaque était de 20,9 jours (±17,8). 33 patients ont bénéficié d’une IRM en phase aiguë, réalisée avec un délai moyen de 14,8 jours (±10,3). Les données de l’IRM dans ce sous-groupe retrouvaient une MVG indexée moyenne de 127,1 gr/m2 (± 41,8). Il n’y avait pas d’anomalies significatives sur les séquences pondérées T2. L’ECV moyen était de 24,4 (± 2,6 %). Des anomalies du rehaussement tardif ont été retrouvées chez 34,4 % des patients, majoritairement de localisation mésomyocardique. Seuls deux patients présentaient du rehaussement sous endocardique sans contexte évocateur de syndrome coronarien aigu. Une IRM de suivi a été réalisée chez 18 de ces patients retrouvant une diminution significative de la MVG indexée (92,8 ± 20,1gr/m2 (p < 0,05)) et une majoration significative de l’ECV (26,7 ± 2,6 % (p = 0,049)). Conclusion : ces données suggèrent que l’HVG disproportionnée parfois observée à la phase aiguë d’HTA maligne semble secondaire à un mécanisme mixte. Ce dernier associerait une hyperplasie myocytaire et une atteinte fibrotique excessive, probablement sous l’effet de l’hyper activation du SRAA. L’absence de séquelle ischémique sur les séquences de rehaussement tardif chez la majorité des patients suggère une cause micro vasculaire diffuse expliquant l’ischémie myocardique, hypothèse cohérente avec la physiopathologie de la maladie.

Primary renal sarcomas are rare comprising from 0.8 to 2.7% of adult renal tumours. They cannot be distinguished clinically or radiologically from the more common renal cell carcinomas. Leiomyosarcoma is the most common histological subtype accounting for around 50-60% of renal sarcomas. Leiomyosarcomas arise from the renal capsule, renal vein or the renal pelvis. They present with nonspecific clinical symptoms and signs and usually have a dismal outcome. Rarely atypical presentations such as acute tumour rupture and hematuria are encountered. We report a 27-year-old woman who presented with malignant hypertension. The hypertension was controlled emergently with antihypertensive agents. In view of the young age, a thorough workup was conducted to identify the cause of hypertension. Imaging studies revealed a solid enhancing renal tumour compressing the renal vasculature. As the staging workup did not reveal any evidence of metastases, the patient underwent right-sided open radical nephrectomy after adequate control of hypertension. The histological examination uncovered the renal tumour to be a leiomyosarcoma. Postoperative period was uneventful and she was doing well on one year follow up. Surprisingly the blood pressure normalised postoperatively and the patient was weaned off of her antihypertensive medications. This case is presented to highlight the atypical acute presentation of primary renal leiomyosarcoma with relatively good prognosis. Timely diagnosis and meticulous surgical resection improved the prognosis of this aggressive renal malignancy.

Objectives: Malignant hypertension (MHT) is defined as severe hypertension accompanied by ischemic failure of one or more organs. The aims of this study were to evaluate the current clinical and etiologic profiles of MHT. Methods: As a retrospective study, we selected all patients admitted to our center from January 2013 to December 2016. Seventy patients with MHT were included. Results: The average age of the patients was 40 years, and more than half of the patients were male (78.57%). There were 24 patients with essential hypertension, accounting for 34.29% of the patients, and 46 with secondary hypertension, accounting for 65.71% of the patients. For secondary MHT, systemic vasculitis (25.57%) was the most common cause, followed by severe obstructive sleep apnea syndrome (15.71%), primary renal parenchymal hypertension (11.43%), primary aldosteronism (7.14%), and Cushing syndrome (1.43%) and nutcracker phenomenon (1.43%). Twenty patients with systemic vasculitis were characterized by severe hypertension accompanied by damage to two or more target organs of differing severity. The levels of white blood cells, hypersensitive C-reactive protein, serum creatinine, and 24-hour urinary protein were above their normal range. Conclusion: Systemic vasculitis may be one of the main causes of MHT, and has been underestimated in the past. In future clinical work, clinicians need to pay more attention to patients with systemic vasculitis.

Malignant hypertension is listed among the causes of secondary thrombotic microangiopathy (TMA), but pathogenic mutations in complement genes have been reported in patients with hypertension-induced TMA. Here we investigated the frequency and severity of hypertension in 55 patients with primary atypical hemolytic uremic syndrome (aHUS). A genetic analysis was performed in all patients, and funduscopic examination was performed in all the patients with Grades 2 and 3 hypertension. A cohort of 110 patients with malignant hypertension caused by diseases other than aHUS served as control. Thirty-six patients with aHUS presented Grade 2 or Grade 3 hypertension and funduscopic examination showed malignant hypertension in 19. Genetic abnormalities in complement were found in 19 patients (37% among patients with malignant hypertension). Plasmapheresis was performed in 46 patients and 26 received eculizumab. Renal and hematological responses were significantly lower after plasmapheresis (24%) than after eculizumab (81%). Renal survival was significantly higher in patients treated with eculizumab (85% at one, three and five years) compared to patients who did not receive this treatment (54%, 46% and 41%), respectively. Response to eculizumab was independent of hypertension severity and the presence of complement genetic abnormalities. Among patients with malignant hypertension caused by other diseases the prevalence of TMA was very low (5%). Thus, severe and malignant hypertension are common among patients with aHUS and eculizumab treatment leads to a higher renal survival when compared to plasmapheresis. However, TMA is uncommon among patients presenting with malignant hypertension caused by diseases other than aHUS., Work in this study was supported by the Instituto de Salud Carlos III /Fondo Europeo de Desarrollo Regional (ISCIII/FEDER) grants PI13/02502, PICI14/00350, and PI16/01685 (to MP), and grants PI13/00802, PI14/00883 and PI17/00130 (to JAM.); the ISCIII/FEDER Programa Miguel Servet, grants CP10/00479 and CPII16/00017 (to JAM); the Spanish “Ministerio de Economía y Competitividad/FEDER” [SAF2015-66287-R] (to SRdeC); the Autonomous Region of Madrid (S2017/BMD-3673) (to SRdeC, EA and MP); Red de Investigación Renal (RedInRen) (RD12/0021/0029) (to MP), the “Fundación Inocente” (Madrid, Spain).] (to SRdC), and the Spanish Society of Nephrology and Fundación Renal Ínigo Álvarez de Toledo (FRIAT) (to JAM).

Hypertension is common in hemolytic uremic syndrome (HUS) and often difficult to control. Local renin-angiotensin activation is believed to be an important part of thrombotic microangiopathy, leading to a vicious cycle of progressive renal injury and intractable hypertension. This has been demonstrated in vitro via enhanced tissue factor expression on glomerular endothelial cells which is enhanced by angiotensin II. We report two pediatric cases of atypical HUS with severe refractory malignant hypertension, in which we targeted the renin-angiotensin system by using intravenous (IV) enalaprilat, oral aliskiren, and oral enalapril with quick and dramatic response of blood pressure. Both drugs, aliskiren and IV enalaprilat, were effective in controlling hypertension refractory to multiple antihypertensive medications. These appear to be promising alternatives in the treatment of severe atypical HUS-induced hypertension and hypertensive emergency.

A case is reported of a 26-year-old primiparous woman in the 32nd week of gestation who presented to the emergency department with the symptoms of a severe headache, nausea and vomiting. The patient was diagnosed with preeclampsia that later progressed to eclampsia. This state was characterized by a sudden onset of a headache and diplopia that advanced to cortical blindness and precipitated significant alterations in mental status, most notable being global amnesia that resolved within 48 h. A post-partum magnetic resonance imaging of the brain in FLAIR mode revealed multiple cortico-subcortical areas of hyperintense signals suggestive of edematous lesions that chiefly involved occipital and parietal lobes with additional atypical manifestations. Such radiologic findings suggested a posterior reversible encephalopathy syndrome variant with the global amnesia as an extraordinary constituent. This unique feature should be acknowledged when treating a preeclamptic or hypertensive patient that exhibits neurological symptomatology and vision disturbances.

Background: Although malignant hypertension begets multiple target organ damage, there is limited data on patients with severe renal injury and evident malignant hypertension in renal histopathology. Methods: We assessed the baseline demographic, histopathological findings and clinical outcomes in this retrospective analysis of patients with biopsy-proven malignant hypertension. Results: Thirty cases were analysed, the mean age of patients was 40 ± 11.5 years, 28 (93.3%) were males and the average systolic and diastolic blood pressures at hospitalisation were 197.04 ± 24.14 and 117.41 ± 18.31 mmHg, respectively. Severe retinopathy was seen in 10 (33.3%). The median eGFR at admission was 6.3 (IQR 4.4–9.15) mL/min and 21 (72.4%) needed dialysis. Nine (30%) cases with glomerular crescents were having the primary glomerular disease (7 IgAN, 1 C3 glomerulonephritis, 1 membranoproliferative glomerulonephritis) and 17 (56.6%) had thrombotic microangiopathy. Three-month ESRD free survival was 34.5% (n = 10) and the ESRD cohort had more incidence of dialysis requiring kidney injury at presentation (94.4% vs. 40% in the non-ESRD cohort). Patient survival at 1 year was 50%. Isolated malignant hypertension, differed from others with regard to lesser incidence of severe retinopathy, less glomerular sclerosis (29.61 ± 15.86 vs. 48.45% ± 30.78; P = 0.03), absence of crescents (P = 0.02), more incidence of tuft wrinkling (100% vs. 35%, P = 0.00) and total vessel occlusion (P = 0.02). Conclusion: Clinicopathologically, accelerated essential hypertension differs from hypertension of glomerular disease. Degree of kidney injury at presentation is risk predictor for long-term morbidity in malignant hypertension.

This photo essay describes the fundoscopic and optical coherence tomography (OCT) grading and characteristics of hypertensive retinopathy (HTR). A 34-year-old male presented with decreased vision of 20/40 N10 in both eyes following diagnosis of malignant hypertension. Fundus examination showed papilledema, intraretinal transudates, cotton wool spots, arteriovenous crossing changes, and superficial and deep hemorrhages with a partial macular star consistent with Grade 4 HTR. OCT macula revealed changes in the nerve fiber layer, outer nuclear layer, outer plexiform layer, and outer photoreceptor layer comprising inner segment/outer segment (OS) layer, external limiting membrane, and cone OS tips under the fovea with varied central subfoveal thickness. The fundoscopy grading of HTR was Keith–Wagener–Barker Grade 4 and that of OCT grading was Ahn Grade 3.

Presentamos un caso clínico, hasta ahora no descrito, de paciente trasplantado renal que desarrolla hipertensión arterial maligna tras el cambio de sirolimus por tacrolimus. Además presentó trombopenia y anemia que como primera orientación podrían hacer pensar en un síndrome hemolítico urémico, pero la evolución del caso confirmó que todo fue debido a la hipertensión maligna., We report a case, undescribed until now, of a renal transplant patient who developed a malignant hypertension after change sirolimus by tacrolimus. Also presented thrombocytopenia and anemia as a first orientation could suggest a hemolytic uremic síndrome, but subsequent events confirmed that everythins was due to malignant hypertension.

A 33-year-old male presented to our clinic with low vision in both eyes that started during the previous week. Visual acuity was 20/63 in the right eye and 20/50 in the left eye. Fundus examination revealed signs of hypertensive retinopathy; thus, a multidisciplinary approach was adopted for the diagnosis and treatment of this patient. We consulted the nephrology and cardiology departments on this case. Upon diagnosing malignant hypertension and renal failure, the patient was put on hemodialysis. His visual acuity was 20/20 at 6 months, whereas foveal assessment on optical coherence tomography angiography revealed neither marked superficial and deep capillary density loss and foveal avascular zone enlargement nor a decrease in disc flow and radial peripapillary capillary density. Early diagnosis and treatment of malignant hypertension are critical in preventing progression of end-organ damage including the eyes. Optical coherence tomography angiography may be useful in cases when fundus fluorescein angiography is relatively contraindicated (e.g., renal failure).

We hypothesized that vascular actions of 20-hydroxyeicosatetraenoic acid (20-HETE), the product of cytochrome P450 (CYP450)-dependent ω-hydroxylase, potentiate prohypertensive actions of angiotensin II (ANG II) in Cyp1a1-Ren-2 transgenic rats, a model of ANG II-dependent malignant hypertension. Therefore, we evaluated the antihypertensive effectiveness of 20-HETE receptor antagonist (AAA) in this model. Malignant hypertension was induced in Cyp1a1-Ren-2 transgenic rats by activation of the renin gene using indole-3-carbinol (I3C), a natural xenobiotic. Treatment with AAA was started either simultaneously with induction of hypertension or 10 days later, during established hypertension. Systolic blood pressure (SBP) was monitored by radiotelemetry, indices of renal and cardiac injury, and kidney ANG II levels were determined. In I3C-induced hypertensive rats, early AAA treatment reduced SBP elevation (to 161 ± 3 compared with 199 ± 3 mmHg in untreated I3C-induced rats), reduced albuminuria, glomerulosclerosis index, and cardiac hypertrophy (P

Introduction: Capillary leak syndrome (CLS) is characterized by hypoproteinemia, diffused pitting edema, noncardiogenic pulmonary edema, and hypotension. By far, there are no related reports of CLS secondary to malignant hypertension (MHT). A 33-year-old male was admitted to our hospital with the diagnosis of CLS on the background of MHT. Patient concerns: A 33-year-old male was admitted with a 6-day history of worsening dyspnea, chest distress, and diffused pitting edema accompanied by very high blood pressure (200/145 mm Hg). Diagnoses: The tests and examinations showed hypoalbuminemia (26.7 g/L), pulmonary edema, and normal heart function. However, the expected massive proteinuria was absent (1.5 g/24 h). After diuretic and other antihypertensive therapy, the blood pressure reduced gradually; meanwhile, the symptoms of dyspnea and chest distress were improved quickly, and edema in his legs was also reduced. It is surprising that there was no change of pulmonary edema signs on imaging scan, and hypoalbuminemia remained with only mild proteinuria. Thus, our provisional diagnosis of this patient was CLS secondary to MHT. Interventions and outcomes: We administered intravenous immunoglobulin, sulodexide, and renin-angiotensin system inhibitor to the patient for repairing vascular endothelium and improving the function of vascular endothelium. Before discharge, the patient's edema disappeared and the chest X-ray turned to normal. The level of serum albumin also increased to 35.1 g/L along with the overall improvement. Finally, the renal biopsy revealed malignant hypertensive glomerulosclerosis. All these clinical manifestations were consistent with CLS caused by MHT. Lessons: Up to now, there has been no case report of CLS caused by MHT. We should pay more attention to CLS induced by MHT, try to diagnose it as soon as possible, and give prompt treatment to CLS and primary disease.

Malignant hypertension may cause many ocular symptoms such as focal arteriolar contraction, cotton-wool spots, macular edema, retinal hemorrhages, hyperpigmented spots, optic disc edema, macular stars. Malignant hypertension should not be forgotten in a patient with optic disc edema and macular star even if the patient does not use anti-hypertensive drugs. Here a case of vision loss due to malignant hypertension induced optic disc edema and macular star was presented.Malignant hypertension may cause many ocular symptoms such as focal arteriolar contraction, cotton-wool spots, macular edema, retinal hemorrhages, hyperpigmented spots, optic disc edema, macular stars. Malignant hypertension should not be forgotten in a patient with optic disc edema and macular star even if the patient does not use anti-hypertensive drugs. Here a case of vision loss due to malignant hypertension induced optic disc edema and macular star was presented, Malign hipertansiyon fokal arteriol daralması, cotton-wool spotlar, maküla ödemi, retinal kanamalar, hiperpigmente spotlar, optik disk ödemi, makülar yıldız görünümü gibi birçok oküler bulguya neden olabilir. Optik disk ödemi ve makülar yıldız görünümü ile gelen bir hastada antihipertansif ilaç kullanmıyor olsa bile malign hipertansiyon unutulmamalıdır. Bu yazıda malign hipertansiyona bağlı optik disk ödemi ve makülar yıldız gelişmesi nedeniyle görme kaybına yol açan bir olgu sunuldu.Hipertansiyon dünyada önlenebilir ölüm nedenleri arasında birinci sırada olup günümüzde yetişkin nüfusun yaklaşık %31,8’ini etkilemektedir (1). Malign hipertansiyon ilk kez 1928 de Keith ve ark. tarafından retinit, belirgin tansiyon yüksekliği ve renal fonksiyon bozukluğunu kapsayan bir tablo olarak tariflenmiştir (2).

Introduction: Epoxyeicosatrienoic acids (EETs) are converted by the enzyme soluble epoxid hydrolase (sEH) to the biologically inactive dihydroxyeicosatrienoic acids (DHETs). EETs are significantly involved in the control of blood pressure, they influence vascular tone and renal transport mechanism. sEH inhibitor reduce blood pressure by increasing the bioavailability of EETs in many models of hypertension. Aim of the study: To determine that sEH inhibitor decreases blood pressure and improves the renal function during the development of malignant hypertension in transgenic rats after the induction of the mouse renin gene. Methods: Hypertension in Cyp1a1-Ren-2 transgenic rats was induced through a dietary administration of the natural xenobiotic indole-3-carbinol (I3C, 0.3 %) for 3 and 11 days. I3C activates the renin gene. At the same time, during a three-day induction of hypertension, the inhibitor of nitric oxide synthase L-NAME (600 mg/l) was administered in drinking water. The sEH inhibitor c-AUCB was given in drinking water at a dose of 13 or 26 mg/l, starting 48 hours before the initiation of I3C and L-NAME administration. Radiotelemetric measurement of blood pressure was performed and renal excretory parameters were monitored in the conscious animals. The effects on renal hemodynamics and...

Malignant hypertension is a severe form of hypertension which pathogenesis is not fully elucidated. The aim of our study was to investigate the role of Angiotensin II on hemodynamic and oxidative stress parameters in model of malignant hypertension by using losartan. Adult males were divided into three groups: normotensive Wister rats, control SHR and losartan treated SHR. Control and SHR group received a single bolus dosage of saline, while the SHR + LOS group received a losartan. Blood pressure, cardiac output, both total peripheral and carotid vascular resistance and carotid blood flow were measured. CAT, SOD and TBARS were determined in the blood samples. The treatment significantly decreased the blood pressure. There was no significant change in cardiac output or carotid blood flow, but treatment resulted in diminishment of vascular resistances, both total peripheral and carotid. There was a tendency to restore the values of SOD activity towards those of the normotensive group, but there were no change in CAT and TBARS levels. Taken together, our results show that a single application of losartan has a strong hypotensive effect. Angiotensin II seems to have a significant role in the malignant hypertension syndrome and it partially affects oxidative stress parameters. [Projekat Ministarstva nauke Republike Srbije, br. OI 175096]

Infection-related glomerulonephritis presents commonly as acute nephritic illness, hypertension, hypocomplementinemia following an episode of pharyngitis or pyoderma. Atypical features like thrombotic microangiopathy (TMA), produced by neuraminidase antigen targeting endothelium have been described rarely. We report a case of TMA secondary to malignant hypertension, coexisting with post infectious glomerulonephritis.

A nulliparous pregnant woman in her mid-20s and in the 32nd week of gestation presented to the emergency department with severe headache and vomiting. She had an uneventful medical history ; however, the physical examination upon hospital admission revealed a hypertensive emergency, papilledema, and 2+ dipstick proteinuria. Upon establishing the diagnosis of preeclampsia, aggressive therapy with corticosteroids, antihypertensive medication, and seizure prophylaxis was initiated. Hemodynamic stability was achieved within 24 hours and the patient remained in the observation unit located within the gynecology clinic. On the ninth day postadmission, however, her condition abruptly deteriorated and advanced to imminent eclampsia, accompanied by transient vision loss, altered mental status, and acute hypertensive crisis. After the patient underwent successful emergent delivery via caesarean section, a laboratory workup revealed hemolysis, elevated liver enzymes, and low platelet count, suggesting HELLP syndrome, a serious complication of eclampsia. This patient concurrently developed posterior reversible encephalopathy syndrome, which was confirmed by magnetic resonance imaging and acute respiratory distress syndrome (the latter presented with diffuse bilateral infiltrates on x-ray and developing pulmonary edema in the absence of cardiac etiology). Because of these life-threatening dynamics, the patient was transferred to the intensive care unit for further treatment. This case is a rare cascade of life-threatening complications that developed in a patient and required skillful multidisciplinary decision making and experienced management within an acute critical care setting. The final outcome of the treatment and intensive care was successful because both the patient and child survived and had no chronic or debilitating sequelae.

Background Interferon-beta (IFN-beta) is one of the most widely prescribed medications for relapsing-remitting multiple sclerosis (RRMS). IFN-related thrombotic microangiopathy (TMA) is a rare but severe complication, with a fulminant clinical onset and a possibly life-threatening outcome that may occur years after a well-tolerated treatment with IFN. Most patients evolve rapidly to advanced chronic kidney disease and eventually to renal failure. Methods We performed a retrospective analysis of TMA cases diagnosed and managed in our Nephrology Department from 2010 to 2015, and performed a literature review of IFN-beta-induced TMA. Results Three cases of TMA among patients treated with IFN-beta were identified who did not show any renal improvement following conventional therapy: IFN withdrawal and plasma exchange (PE, range 8–18) sessions. All of them responded favourably to eculizumab, with progressive clinical and renal improvement, allowing dialysis discontinuation, without recurrence of TMA during a long-term follow-up (range 1–5 years). Conclusions TMA is a recognized severe complication in RRMS patients treated with IFN-beta. Withdrawal of IFN and treatment with PE, steroids or rituximab did not improve the poor renal prognosis in our three patients and in all the previously described cases in the literature. In our experience, eculizumab had a strikingly favourable effect on renal recovery, suggesting a role of IFN-beta as a trigger in complement-mediated TMA. Neurologists and nephrologists should be vigilant to this complication to prevent possibly irreversible renal damage.

The most common cause of malignant hypertension in children is renal or renovascular pathology. The combination with lethargy suggests a diagnosis of hypertensive crisis with hypertensive encephalopathy. Here, we present a case of severe hypertension with lethargy as the sole presenting symptoms of ileocecal intussusception. Both normalized following surgical repositioning. We conclude that malignant hypertension and lethargy can be presenting symptoms of intussusception.

Malignant hypertension develops in some cases of hypertension but not in others. We hypothesized that an impaired neovascularization and a reduced capillary supply characterizes the malignant course of experimental hypertension. Two-kidney, one-clip renovascular hypertension was induced in rats; controls (sham) were sham operated. To distinguish malignant hypertension from non-malignant hypertension, we considered two factors: weight loss, and the number of typical vascular lesions (onion skin lesions and fibrinoid necroses) per kidney section of the nonclipped kidney. Animals in the upper half for both criteria were defined as malignant hypertensives. After 5 weeks, mean arterial blood pressure was elevated to the same degree in malignant hypertension and non-malignant hypertension whereas plasma renin and aldosterone were significantly higher in malignant hypertensives. The expression of plasminogen activator inhibitor-1 was elevated (up to 14-fold) in non-malignant but significantly more increased (up to 36-fold) in malignant hypertensive rats, compared to sham. As a bioassay for neovascularization, the area of granulation tissue ingrowth in polyvinyl discs (implanted subcutaneously) was reduced in malignant hypertension compared to non-malignant hypertension and sham, while there was no difference between non-malignant hypertension and sham. The number of renal and left ventricular capillaries was significantly lower in malignant hypertension compared to non-malignant hypertension, as was the number of proliferating endothelial cells. We conclude that an impaired neovascularization and capillarization occurs in malignant renovascular hypertension but not in the non-malignant course of the disease despite comparable blood pressure levels. This might contribute to the unique vascular lesions and progressive target organ damage observed in malignant hypertension.

Malignant hypertension develops in some cases of hypertension but not in others. We hypothesized that an impaired neovascularization and a reduced capillary supply characterizes the malignant course of experimental hypertension. Two-kidney, one-clip renovascular hypertension was induced in rats; controls (sham) were sham operated. To distinguish malignant hypertension from non-malignant hypertension, we considered two factors: weight loss, and the number of typical vascular lesions (onion skin lesions and fibrinoid necroses) per kidney section of the nonclipped kidney. Animals in the upper half for both criteria were defined as malignant hypertensives. After 5 weeks, mean arterial blood pressure was elevated to the same degree in malignant hypertension and non-malignant hypertension whereas plasma renin and aldosterone were significantly higher in malignant hypertensives. The expression of plasminogen activator inhibitor-1 was elevated (up to 14-fold) in non-malignant but significantly more increased (up to 36-fold) in malignant hypertensive rats, compared to sham. As a bioassay for neovascularization, the area of granulation tissue ingrowth in polyvinyl discs (implanted subcutaneously) was reduced in malignant hypertension compared to non-malignant hypertension and sham, while there was no difference between non-malignant hypertension and sham. The number of renal and left ventricular capillaries was significantly lower in malignant hypertension compared to non-malignant hypertension, as was the number of proliferating endothelial cells. We conclude that an impaired neovascularization and capillarization occurs in malignant renovascular hypertension but not in the non-malignant course of the disease despite comparable blood pressure levels. This might contribute to the unique vascular lesions and progressive target organ damage observed in malignant hypertension.

Diffuse alveolar hemorrhage is a clinicopathological syndrome that often leads to respiratory failure, with associated hemoptysis and anemia. Chest radiograph presents non-specific findings of perihilar infiltrates, while computed tomography shows ground-glass attenuation or areas of consolidation with interlobular septal thickening. Bronchoalveolar lavage is used to confirm the clinical and radiological suspicion. While vasculitis and other causes of pulmonary renal syndrome are the most common causes of diffuse alveolar hemorrhage, malignant hypertension should be considered in the proper clinical setting. We present a case report of a 51-year-old previously healthy patient that was diagnosed with diffuse alveolar hemorrhage and acute renal failure due to malignant hypertension, through clinical and radiological findings.

We present the case of a 64-year-old male patient in whom malignant phase of hypertension developed during dialysis therapy. Hypertension was resistant to pharmacological therapy with seven antihypertensive drugs and dialysis therapy with ultrafiltration. In this patient bilateral nephrectomy was performed as a rescue therapy. It led to better control of blood pressure and allowed to reduce the number and dosage of antihypertensive medications.

Acute aortic syndrome is a pathological process with low incidence compared with acute coronary syndrome, although with a worse prognosis in the short term, which is why its early diagnosis and urgent treatment are essential to the favorable evolution of thepatient. Electrocardiographic changes suggestive of myocardial ischemia, with acute evolution, are rare in acute aortic syndrome. Even in the presence of a suggestive thoracic pain and high levels of blood pressure, an adequate differential diagnosis is of utmost importance, since the administration of anticoagulation and antiplatelet therapy in high doses can have a dramatic impact on patient outcome. Two cases are presented in which electrocardiographic changes determined the action to take in patients with acute aortic syndrome.

Key Clinical Message A 24-year-old man with diarrhea found to have acute renal failure with microangiopathic hemolytic anemia (MAHA). A diagnosis of hemolytic uraemic syndrome (HUS) was made. He was initiated on plasma exchange and hemodialysis. On day 6, he was started on eculizumab. His renal functions progressively improved. His main complication during eculizumab therapy was hypertension-related posterior reversible encephalopathy syndrome.

Introduction This study was performed to determine the effects of high-salt diet on the magnitude of the increases in systolic blood pressure (SBP) and kidney tissue angiotensin (ANG) II levels that occur after induction of ANG II-dependent malignant hypertension in Cyp1a1-Ren2 transgenic rats with inducible expression of the mouse Ren2 renin gene [strain name: TGR(Cyp1a1Ren2)]. Methods Cyp1a1- Ren2 rats (n=6) were fed a normal diet containing 0.3% indole-3- carbinol (I3C) for 10days to induce ANG II-dependent malignant hypertension. Results Rats induced with I3C exhibited increases in SBP and elevations of ANG II levels in kidney cortex and medulla. In a second group of rats (n=6), high-salt intake alone did not alter basal SBP; however, subsequent dietary administration of 0.3% I3C during continued high-salt intake elicited a substantially greater increase in SBP than observed in rats fed a normal salt diet. ANG II levels in kidney cortex and medulla of rats induced with I3C and fed a high-salt diet were elevated similarly to those in rats induced with I3C alone. Chronic administration of the AT 1 receptor antagonist, losartan (100mg/L in drinking water, n=6), markedly attenuated the I3C-induced increase in SBP and prevented the augmentation of ANG II levels in kidney cortex and medulla in rats induced with I3C and maintained on a high-salt diet. Conclusions Activation of AT 1 receptors contributes to the augmented blood pressure and elevated kidney tissue ANG II levels that occur in Cyp1a1-Ren2 transgenic rats with malignant hypertension maintained on a high-salt diet.