Search

Background: Diagnosing posterior circulation strokes is challenging due to non-focal symptoms and their overlap with anterior circulation ischemia. Common presentations include lateral medullary syndrome (Wallenberg) and cerebellar infarctions, with symptoms like nystagmus, truncal ataxia, tongue deviation, internuclear ophthalmoplegia, and contralateral pain and temperature impairment. These strokes are often missed on NCCT head scans. High clinical suspicion and evolving neurological signs should prompt a CT angiogram of the head and neck, though MR angiography remains the gold standard for confirming vertebral artery dissection. Case: A 35-year-old male with a history of hypertension, chronic kidney disease, pre-diabetes, left ventricular hypertrophy, and previous left Bell’s palsy presented with sudden left facial paralysis, dizziness, blurred vision, nausea, vomiting, and a blood pressure of 217/141 mmHg. In the emergency room, his vitals included a pulse of 110 bpm, temperature of 36.6 °C, respiratory rate of 23/min, and a BMI of 47.5 kg/m². Examination revealed bilateral nystagmus, ongoing cranial nerve VII deficits, slurred speech, and left upper limb ataxia. A stroke alert was triggered, and an initial non-contrast head CT was negative. Given an NIH Stroke Scale score of 3 and persistent symptoms, a CT angiogram was performed which showed occlusion of the left vertebral artery in the distal V3 and V4 segments, while the basilar artery remained patent with right-dominant circulation. MRI confirmed a left lateral medullary stroke. Management included placing a nasogastric tube, initiating dual antiplatelet therapy (Aspirin 75 mg and Clopidogrel 75 mg), and administering antihypertensives (Hydralazine, Lisinopril-Hydrochlorothiazide, Metoprolol, and Amlodipine). The patient was discharged three days later with instructions to continue dual antiplatelet therapy for 90 days. Conclusion: Malignant hypertension can trigger vertebral artery dissection, leading to a posterior circulation stroke, which has an ambiguous clinical presentation and is often missed on a non-contrast head CT. If clinical symptoms and exam findings suggest this condition, a CT angiogram of the head and neck should be performed, with MRI and MR angiography used for confirmation. Treatment decisions are complicated by the choice between anticoagulants and antiplatelets with antiplatelets being preferred due to their safety profile.

IntroductionBased on office blood pressure (BP) values, hypertension is categorized into three stages: stage 1 (140–159/90–99 mmHg), stage 2 (160–179/100–109 mmHg), and stage 3 (≥180/≥110 mmHg). Malignant hypertension (MHT) is characterized by extreme BP elevation (systolic blood pressure above 200 mmHg and diastolic blood pressure above 130 mmHg) and acute microvascular damage affecting various organs, particularly the retinas, brain, and kidneys.ObjectivesThe pathogenesis, predisposing variables, therapy, and preventive strategies for MHT were examined in this review.Conclusions and recommendationsMalignant hypertension requires prompt and efficient treatment because it is the most severe kind of hypertension that affects target organs. At the same time, there are a number of alternatives available for treating MHT. The International Society of Hypertension 2020 and European Society of Cardiology/European Society of Hypertension 2018 recommendations suggest using labetalol and nicardipine as the first-line choice, with urapidil and nitroprusside serving as alternative medications. Elevated risk of MHT has been linked to many socio-demographic and genetic factors.

Abstract Kidney injury is one of the detrimental consequences of primary malignant hypertension (pMHTN). There is a paucity of non-invasive biomarkers to enhance diagnosis and elucidate the underlying mechanisms. This study aims to explore urine protein biomarkers for pMHTN associated renal damage. In the discovery phase, urine samples were collected from 8 pMHTN, 19 disease controls (DCs), and 5 healthy controls (HCs). In-gel digestion combined with liquid chromatography–tandem mass spectrometry (LC–MS/MS) approach was used for identification of proteins associated with pMHTN. In the validation phase, the differentially expressed proteins were validated by ELISA assay in cohort with 10 pMHTN patients, 37 DCs, and 30 HCs. Compared to DCs and HCs, a specific band between 15 and 25 kDa was found in 7 out of 8 patients with pMHTN. Further LC–MS/MS analysis revealed 5 differentially expressed proteins. ELISA validation demonstrated that urinary complement factor D (CFD) was significantly up regulated in pMHTN. By receiver operating characteristic curve analysis, urinary CFD/Cr showed moderate potential in discriminating pMHTN from DCs (the area under curve: 0.822, 95% CI 0.618–0.962). Urinary CFD may be a potential biomarker for pMHTN with its elevation indicative of the activation of the alternative complement pathway in pMHTN.

Kidney injury is one of the detrimental consequences of primary malignant hypertension (pMHTN). There is a paucity of non-invasive biomarkers to enhance diagnosis and elucidate the underlying mechanisms. This study aims to explore urine protein biomarkers for pMHTN associated renal damage. In the discovery phase, urine samples were collected from 8 pMHTN, 19 disease controls (DCs), and 5 healthy controls (HCs). In-gel digestion combined with liquid chromatography–tandem mass spectrometry (LC–MS/MS) approach was used for identification of proteins associated with pMHTN. In the validation phase, the differentially expressed proteins were validated by ELISA assay in cohort with 10 pMHTN patients, 37 DCs, and 30 HCs. Compared to DCs and HCs, a specific band between 15 and 25 kDa was found in 7 out of 8 patients with pMHTN. Further LC–MS/MS analysis revealed 5 differentially expressed proteins. ELISA validation demonstrated that urinary complement factor D (CFD) was significantly up regulated in pMHTN. By receiver operating characteristic curve analysis, urinary CFD/Cr showed moderate potential in discriminating pMHTN from DCs (the area under curve: 0.822, 95% CI 0.618–0.962). Urinary CFD may be a potential biomarker for pMHTN with its elevation indicative of the activation of the alternative complement pathway in pMHTN. [ABSTRACT FROM AUTHOR]

This article presents a case study of a 29-year-old man who arrived at the emergency department with extremely high blood pressure and kidney dysfunction. The patient experienced symptoms such as headache and weakness, and further examination revealed retinopathy and left ventricular hypertrophy. These findings indicated a hypertensive emergency, specifically malignant hypertension associated with thrombotic microangiopathy. The article emphasizes the need for timely diagnosis and treatment in cases of this condition, and discusses the challenges of accurately diagnosing it. [Extracted from the article]

BACKGROUND The rehospitalization rate in a hypertensive emergency is high, indicating the necessity for optimizing its long-term management. The role of the renin–angiotensin system (RAS) blockade in this disorder remains uncertain. METHODS We conducted a retrospective analysis involving 20 admitted patients who received aliskiren, a direct renin inhibitor (DRI), for the management of hypertensive emergency associated with elevated plasma renin activity (PRA). We analyzed the changes in blood pressure (BP), kidney function, and RAS activity in the subacute and chronic phases. RESULTS The use of DRI was associated with a marked reduction in PRA (median, from 25.0 to 1.2 ng/ml/h) and serum aldosterone levels (from 404 to 130 pg/ml) during the index admission. BP also decreased from 226/143 to 142/80 mm Hg. A comparison of clinical characteristics according to the renal function indicated that dialysis-dependent patients had higher aldosterone levels than non-dialysis-dependent patients at admission, despite comparable BP levels. After a median follow-up of 567 days in non-dialysis-dependent patients with DRI, eGFR levels were significantly increased from 14.3 to 23.1 ml/min/1.73 m2. PRA levels were consistently suppressed at 0.8 ng/ml/h. We found a significant correlation between the degree of PRA suppression and changes in eGFR (r  = –0.58), indicating that the effective blockade of RAS is associated with the preservation of eGFR in the study subjects. CONCLUSIONS DRI can successfully suppress PRA in patients with high-renin hypertensive emergency in both subacute and chronic phases. An efficient RAS blockade is associated with preserved renal function in these patients. [ABSTRACT FROM AUTHOR]

Abstract Background Elabela, a recently discovered hormonal peptide containing 32 amino acids, is a ligand for the apelin receptor. It can lower blood pressure and attenuate renal fibrosis. However, the clinicopathological relationship between Elabela level and renal damage caused by benign hypertension (BHT) and malignant hypertension (MHT) has not been elucidated. Therefore, we investigated the clinicopathological correlation between serum Elabela level and renal damage caused by BHT and MHT. Methods The participants comprised 50 patients and 25 age-matched healthy adults. The 50 patients were separated into two groups: MHT (n = 25) and BHT groups (n = 25). We analyzed their medical histories, demographics, and clinical examinations, including physical and laboratory tests. Results The results showed that serum Elabela level decreased gradually with a continuous increase in blood pressure from the healthy control group, BHT, to MHT. Moreover, Elabela levels negatively correlated with BMI (R = − 0.27, P = 0.02), SBP (r = − 0.64, P

Thrombotic microangiopathy (TMA) has been observed in some patients receiving interferon beta (IFNβ) therapy for relapsing-remitting multiple sclerosis, but little is known about its clinical features and outcomes. We searched the literature to identify cases with IFNβ-related TMA and assessed their pattern of organ involvement, the presence of prodromal manifestations, the treatments used, and the outcomes. Thirty-five articles met the inclusion criteria, and data of 67 patients were collected. The median duration of IFNβ therapy before the diagnosis of TMA was 8 years, and 56/67 (84%) presented with acute kidney injury (AKI), of which 33 required acute dialysis. All but three patients had manifestations during the four weeks before TMA onset, including flu-like symptoms, headache, and worsening blood pressure control. In only two patients, ADAMTS13 activity was reduced, while 27% had low C3 levels. However, none showed causative genetic mutations associated with development of atypical hemolytic uremic syndrome. All patients discontinued IFNβ, 34 (55%) also received plasma exchange, and 12 (18%) received eculizumab. Complete renal recovery was achieved by 20 patients (30%), while 13 (20%) developed end-stage renal disease. Among those with AKI requiring dialysis, eculizumab therapy was associated with a significantly reduced risk of ESRD compared with plasma exchange. Therefore, TMA with features of aHUS mainly occurs after prolonged treatment with IFNβ and is preceded by prodromes, which may lead to an early diagnosis before life-threatening complications occur. Eculizumab appears beneficial in cases with severe kidney involvement, which supports a role of the complement system in the pathogenesis of these forms. [ABSTRACT FROM AUTHOR]

Background: Elabela, a recently discovered hormonal peptide containing 32 amino acids, is a ligand for the apelin receptor. It can lower blood pressure and attenuate renal fibrosis. However, the clinicopathological relationship between Elabela level and renal damage caused by benign hypertension (BHT) and malignant hypertension (MHT) has not been elucidated. Therefore, we investigated the clinicopathological correlation between serum Elabela level and renal damage caused by BHT and MHT. Methods: The participants comprised 50 patients and 25 age-matched healthy adults. The 50 patients were separated into two groups: MHT (n = 25) and BHT groups (n = 25). We analyzed their medical histories, demographics, and clinical examinations, including physical and laboratory tests. Results: The results showed that serum Elabela level decreased gradually with a continuous increase in blood pressure from the healthy control group, BHT, to MHT. Moreover, Elabela levels negatively correlated with BMI (R = − 0.27, P = 0.02), SBP (r = − 0.64, P < 0.01), DBP (r = − 0.58, P < 0.01), uric acid (r = − 0.39, P < 0.01), bun (r = − 0.53, P < 0.01), and Scr (r = − 0.53 P < 0.01) but positively correlated with eGFR (r = 0.54, P < 0.01). Stepwise multivariate linear regression analysis showed that SBP was the variable most related to Elabela (t = − 5.592, P < 0.01). Conclusions: Serum Elabela levels decreased in patients with hypertension, especially malignant hypertension, and has the potential to be a marker of hypertension-related kidney damage. [ABSTRACT FROM AUTHOR]

Malignant hypertension (MH) can precipitate and worsen renal thrombotic microangiopathy. Also, renal thrombotic microangiopathies (TMA) can cause malignant hypertension. Case reports regarding this clinical presentation are limited. A 36-year-old man presented with low urine output and bilateral leg swelling for five days with two episodes of haemoptysis. He was identified as a patient with hypertensive emergency with hypertension mediated organ damage (HMOD) in cardiac and renal functions with thrombotic microangiopathy. Renal biopsy revealed focal acute tubular injury with moderate tubulointerstitial nephritis and hypertensive vascular changes. Multiple anti-hypertensive medications were used for adequate blood pressure control. Despite that, the patient had worsening renal function, and eventually became dependent on haemodialysis. Malignant hypertension has to be considered as one of the aetiologies of TMA as it can lead to end-stage renal disease (ESRD) and poor outcomes. Diagnosis is difficult when both entities are presenting together.

Introduction: Malignant hypertension (mHTN) is a hypertensive emergency. Thrombotic microangiopathy (TMA) is a widespread complication of mHTN. Few studies have evaluated whether glomerular hematuria provides prognostic information for renal dysfunction in patients with mHTN-associated TMA. Methods: This observational cohort study included 292 patients with mHTN-associated TMA based on renal biopsy. Propensity-score matching (PSM) analysis was conducted to adjust for clinical characteristics in a comparison between with and without glomerular hematuria. Cox regression was employed to identify risk factors for renal prognosis. Results: A total of 70 patients with glomerular hematuria were compared to 222 patients with non-glomerular hematuria. After PSM, 67 pairs of patients with mHTN-associated TMA were matched. Patients with glomerular hematuria exhibited lower serum albumin levels, higher 24-h proteinuria, and a higher prevalence of glomerular sclerosis than those with non-glomerular hematuria. Glomerular hematuria was independently associated with deteriorated renal function compared with non-glomerular hematuria (HR: 0.51; 95% CI: 0.29–0.89, p = 0.019). This association remained significant after PSM (HR: 0.51; 95% CI: 0.28–0.91, p = 0.022). Additionally, glomerular hematuria was independently associated with renal replacement therapy (RRT) (HR: 3.14; 95% CI: 2.06–5.66, p < 0.001). This difference remained significant after PSM comparison (HR: 2.41; 95% CI: 1.34–4.33, p = 0.003). Furthermore, despite intensive blood pressure control, patients with glomerular hematuria experienced a higher incidence of RRT and a poorer recovery in renal function, specifically a 25% reduction of creatinine levels, compared to patients with non-glomerular hematuria. Conclusion: Glomerular hematuria is significantly associated with an increased risk of adverse renal outcomes in patients with mHTN-associated TMA.

Abstract Background Thrombotic microangiopathies (TMAs) are rare but can be severe in kidney transplant. recipients (KTR). Methods We analysed the epidemiology of adjudicated TMA in consecutive KTR during the. 2009–2021 period. Results TMA was found in 77/1644 (4.7%) KTR. Early TMA (n = 24/77 (31.2%); 1.5% of all KTR) occurred during the first two weeks ((median, IQR) 3 [1–8] days). Triggers included acute antibody-mediated rejection (ABMR, n = 4) and bacterial infections (n = 6). Graft survival (GS) was 100% and recurrence rate (RR) was 8%. Unexpected TMA (n = 31/77 (40.2%); 1.5/1000 patient-years) occurred anytime during follow-up (3.0 (0.5–6.2) years). Triggers included infections (EBV/CMV: n = 10; bacterial: n = 6) and chronic active ABMR (n = 5). GS was 81% and RR was 16%. Graft-failure associated TMA (n = 22/77 (28.6%); 2.2% of graft losses) occurred after 8.8 (4.9–15.5) years). Triggers included acute (n = 4) or chronic active (n = 14) ABMR, infections (viral: n = 6; bacterial: n = 5) and cancer (n = 6). 15 patients underwent transplantectomy. RR was 27%. Atypical (n = 6) and typical (n = 2) haemolytic and uremic syndrome, and isolated CNI toxicity (n = 4) were rare. Two-third of biopsies presented TMA features. Conclusions TMA are mostly due to ABMR and infections; causes of TMA are frequently combined. Management often is heterogenous. Our nosology based on TMA timing identifies situations with distinct incidence, causes and prognosis.

Purpose: To report the first case of malignant hypertension who presented with severe outer nuclear layer hemorrhage affecting the fovea and had rapid recovery in findings after intravitreal injection of bevacizumab. Case Report: A 72-year-old woman presented with complaints of blurred vision in her left eye. An extensive systemic and ophthalmic examination confirmed the diagnosis of outer nuclear layer hemorrhage caused by malignant hypertension. An intravitreal injection of bevacizumab (1.25 mg/0.05 mL) was administered to the left eye. The outer nuclear layer hemorrhage completely resolved three weeks after intravitreal bevacizumab injection treatment, and the visual acuity improved significantly. Conclusion: Malignant hypertensive retinopathy may present with outer nuclear layer hemorrhage affecting the fovea and intravitreal anti-VEGF therapy may be beneficial in these cases. [ABSTRACT FROM AUTHOR]

Background: Thrombotic Microangiopathy (TMA) is a syndrome characterized by the presence of anemia, thrombocytopenia and organ damage and has multiple etiologies. The primary aim is to develop an algorithm to classify TMA (TMA-INSIGHT score). Methods: This was a single-center retrospective cohort study including hospitalized patients with TMA at a single center. We included all consecutive patients diagnosed with TMA between 2012 and 2021. TMA was defined based on the presence of anemia (hemoglobin level < 10 g/dL) and thrombocytopenia (platelet count < 150,000/µL), signs of hemolysis, and organ damage. We classified patients in eight categories: infections; Malignant Hypertension; Transplant; Malignancy; Pregnancy; Thrombotic Thrombocytopenic Purpura (TTP); Shiga toxin-mediated hemolytic uremic syndrome (STEC-SHU) and Complement Mediated TMA (aHUS). We fitted a model to classify patients using clinical characteristics, biochemical exams, and mean arterial pressure at presentation. Results: We retrospectively retrieved TMA phenotypes using automatic strategies in electronic health records in almost 10 years (n = 2407). Secondary TMA was found in 97.5% of the patients. Primary TMA was found in 2.47% of the patients (TTP and aHUS). The best model was LightGBM with accuracy of 0.979, and multiclass ROC-AUC of 0.966. The predictions had higher accuracy in most TMA classes, although the confidence was lower in aHUS and STEC-HUS cases. Conclusion: Secondary conditions were the most common etiologies of TMA. We retrieved comorbidities, associated conditions, and mean arterial pressure to fit a model to predict TMA and define TMA phenotypic characteristics. This is the first multiclass model to predict TMA including primary and secondary conditions. [ABSTRACT FROM AUTHOR]

Color Doppler imaging allows a detailed non-invasive assessment of the state of blood flow in the retrobulbar vessels in patients with various vascular pathologies. Malignant hypertension (MH) is a clinical syndrome characterized by severe diastolic arterial hypertension (140 mm Hg or more), leading to ischemic damage to various organs — kidneys, heart, brain, eyes. Patients with MH often present with thrombotic microangiopathy (TMA), a rare life-threatening condition characterized by multiple systemic thromboses of the microvasculature.Purpose: to assess the state of blood flow in retrobulbar vessels by color Doppler imaging in TMA associated with MH.Methods: 10 patients (20 eyes) (age 43.5 ± 6.2 years) with TMA associated with MH underwent a study of the state of blood flow in the vessels of the retrobulbar space by color Doppler imaging. Qualitative and quantitative analyzes of retinal and choroidal circulation were assessed.Results. Analysis of the Doppler spectrum of blood flow in TMA associated with MH revealed a statistically significant increase in maximum systolic (Vsyst) and finale diastolic blood flow velocity (Vdiast) in the ophthalmic artery (OA), as well as Vsyst in the medial short posterior ciliary arteries (SPCA), compared with the control group (p < 0.05). In 2 patients (20 %) of the study group, a significant bilateral decrease in the diastolic component of the spectrum was found up to the isoline (Vdiast = 0) and a maximum increase in the peripheral resistance index (RI = 1.0). Statistically significant correlations were found between some laboratory parameters of blood serum (hemoglobin, lactate dehydrogenase, creatinine), as well as the estimated glomerular filtration rate and hemodynamic parameters in the central retinal artery (CRA), lateral (SPCA) and OA (p < 0.05).Conclusion. The results of the study of ocular hemodynamics using color Doppler imaging in TMA associated with MH are presented for the first time. TMA associated with MH is characterized by a statistically significant increase in the maximum systolic blood flow velocity in the OA and SPCA with the development of an ischemic state in the retinal and choroidal microcirculation in some patients. An increase in TMA activity in MH leads to a change in the microcirculation of the retina and choroid of an ischemic nature. An increase in blood creatinine in patients with TMA with MH is statistically significantly associated with a decrease in systolic blood flow velocity in the CRA.

Abstract Background Malignant hypertension is a model of the rapid changes that a high afterload and renin-angiotensin levels can induce on target organs such as the heart. We present a case of a young man affected by malignant hypertension with multi-organ involvement who showed quick remission after adequate antihypertensive treatment. Case summary A 41-year-old jazz pianist with a family history of coronary artery disease presented to the emergency department for asthenia and epigastric pain, associated with right eye visual impairment. No neurological symptoms. An echocardiogram showed left ventricular hypertrophy with severe impairment of ejection fraction (22%) due to global hypokinesia. There was renal insufficiency (creatinine 2.51 mg/dl, eGFR 34 ml/min, HS Troponin T 127 pg/dl, NT pro-BNP 22,672 pg/ml, CRP 32 mg/L, sodium 129 mEq/L with normal kaliemia. The following day, anterior T wave inversion was observed in the electrocardiogram. At a cardiac magnetic resonance. Concentric hypertrophy of the left ventricle was observed with normal myocardial T1 mapping values (1100 ± 38 ms), ruling out Fabry’s disease. There was no obvious myocardial edema at T2 weighted. The viral panel for acute myocarditis resulted in negative. After the exclusion of all possible secondary causes, malignant essential hypertension was the final diagnosis, and additional tests confirmed multi-organ damage. An ophthalmological examination demonstrated hypertensive retinopathy with hemorrhages, cottony exudates, and macular lipid exudation, especially in the right eye. A brain MRI showed small areolas of T2 hyperintensity in the white matter of both cerebral hemispheres, suggestive of chronic microangiopathy, and left nuclear micro lacunar ischemia. The patient was treated with full-dose calcium channel blockers (amlodipine 10 mg), beta-blockers (bisoprolol 10 mg), alpha2 agonists (slow-release clonidine patch), selective a1 blocker (doxazosine 16 mg), and furosemide 50 mg. After the exclusion of renal stenosis and improvement of renal function, Ramipril was added up to a final dose of 10 mg daily. In parallel with the achievement of a satisfactory blood pressure control, visual impairment disappeared with a reduction of retinal ischemic exudates and hemorrhages at the ophthalmological follow-up assessment. At the time of discharge, an echocardiographic re-evaluation confirmed concentric hypertrophy of the left ventricle with markedly reduced wall thicknesses, with a partial recovery in left ventricular ejection function (EF 44%). Six months after discharge, the patient is in good general condition under optimal medical therapy (without furosemide) with normalized blood pressure values (130–140/80 mmHg). At 6 months from discharge, creatinine was only mildly increased (1.5 mg/dl, eGFR 59.5 ml/min) and NT-pro BNP nearly normalized (452 pg/ml). Discussion Malignant hypertension is a cardiovascular emergency and requires immediate and careful intervention to lower blood pressure and reduce organ injury. It is an exclusion diagnosis that can be advanced once the causes of secondary hypertension have been excluded. Remission of organ injury is possible and might be rapid under adequate antihypertensive treatment, but patients require a close follow-up.

Abstract Background We present a case of hypertensive choroidopathy due to malignant hypertension with exudative retinal detachment as a sole finding. We use OCT- angiography for initial diagnosis and report findings from extensive follow up. Case presentation A 51-year-old female with no past medical history, presented to our clinic with painless loss of vision in her left eye. Fundus examination revealed only exudative retinal detachment in her left eye that was confirmed with Optical Coherence Tomography. Fluorescein angiography showed hyperfluorescent spots with leakage in late phases. OCTA manifested a focal dark area in the choriocapillaris slab corresponding to flow signal voids, signifying regions of non-perfusion. Her blood pressure was 220/120 mmHG. Complete blood work -up failed to reveal any other possible etiology. During follow-up period of 9 months blood pressure normalized, patient regained visual function and choriocapillaris perfusion was completely restored. Discussions and conclusions Hypertensive choroidopathy with exudative retinal detachment can be the only sign of malignant hypertension and no pre-existing history of a systemic disease is required in order to become apparent. OCTA reveals areas of non-perfusion at choriocapillaris level, proving that it is an essential tool in the diagnosis and follow up of patients with hypertensive choroidopathy. Finally, we propose that early diagnosis prevents permanent damage of the RPE and leads to complete choroidal remodeling and better visual outcomes.

Background: Thrombotic microangiopathies (TMAs) are rare but can be severe in kidney transplant. recipients (KTR). Methods: We analysed the epidemiology of adjudicated TMA in consecutive KTR during the. 2009–2021 period. Results: TMA was found in 77/1644 (4.7%) KTR. Early TMA (n = 24/77 (31.2%); 1.5% of all KTR) occurred during the first two weeks ((median, IQR) 3 [1–8] days). Triggers included acute antibody-mediated rejection (ABMR, n = 4) and bacterial infections (n = 6). Graft survival (GS) was 100% and recurrence rate (RR) was 8%. Unexpected TMA (n = 31/77 (40.2%); 1.5/1000 patient-years) occurred anytime during follow-up (3.0 (0.5–6.2) years). Triggers included infections (EBV/CMV: n = 10; bacterial: n = 6) and chronic active ABMR (n = 5). GS was 81% and RR was 16%. Graft-failure associated TMA (n = 22/77 (28.6%); 2.2% of graft losses) occurred after 8.8 (4.9–15.5) years). Triggers included acute (n = 4) or chronic active (n = 14) ABMR, infections (viral: n = 6; bacterial: n = 5) and cancer (n = 6). 15 patients underwent transplantectomy. RR was 27%. Atypical (n = 6) and typical (n = 2) haemolytic and uremic syndrome, and isolated CNI toxicity (n = 4) were rare. Two-third of biopsies presented TMA features. Conclusions: TMA are mostly due to ABMR and infections; causes of TMA are frequently combined. Management often is heterogenous. Our nosology based on TMA timing identifies situations with distinct incidence, causes and prognosis. [ABSTRACT FROM AUTHOR]

Background Nephrosclerosis is one of the histopathological consequences of severe or malignant hypertension (MH), some of the pathophysiology of which has been extrapolated from essential polygenetic arterial hypertension. Despite our recent description of unsuspected ciliopathies with MH, causes of MH in young patients with severe renal impairment are poorly understood. Methods To refine and better describe the MH phenotype, we studied clinical and prognostic factors in young patients receiving a kidney biopsy following their first episode of MH. Patients were identified retrospectively and prospectively from eight centres over a 35-year period (1985–2020). Keywords were used to retrospectively enrol patients irrespective of lesions found on renal biopsy. Results A total of 114 patients were included, 77 (67%) of whom were men, average age 34 years, 35% Caucasian and 34% African origin. An isolated clinical diagnosis of severe nephrosclerosis was suggested in only 52% of cases, with 24% primary glomerulopathies. Only 7% of patients had normal renal function at diagnosis, 25% required emergency dialysis and 21% were eventually transplanted. Mortality was 1% at the last follow-up. Independent prognostic factors significantly associated with renal prognosis (6-month dialysis) and predictive of end-stage renal disease were serum creatinine on admission {odds ratio [OR] 1.56 [95% confidence interval (CI) 1.34–1.96], P  < .001} and renal fibrosis >30% [OR 10.70 (95% CI 1.53–112.03), P  = .03]. Astonishingly, the presence of any thrombotic microangiopathy lesion on renal biopsy was an independent, protective factor [OR 0.14 (95% CI 0.02–0.60), P  = .01]. The histopathological hallmark of nephrosclerosis was found alone in only 52% of study patients, regardless of ethnicity. Conclusions This suggests that kidney biopsy might be beneficial in young patients with MH. [ABSTRACT FROM AUTHOR]

Diagnostic radiology is essential for providing targeted management of different diseases. Thus, there has been a dramatic increase in the demand for medical imaging. However, acute compartment syndrome (ACS) is one of the clinical scenarios in which radiology has limited value. The authors report a nontraumatic spontaneous ACS in the forearm of a 56-year-old female. The roles of Ultrasound and MRI, if available, are also illustrated. Limited reports of spontaneous ACS are published in the literature; we hope this case adds to the limited data. Our goal in reporting this case is to improve clinical practice with favorable outcomes for the patients involved globally by alert to the onset of ACS to promote early detection and timely fasciotomy. Also, we aim to increase awareness among physicians and radiologists of the limitations of radiology in specific clinical scenarios. Finally, it may aid in illuminating a possible link between malignant hypertension, spontaneous bleeding/hematoma, and ACS.

Introduction: We previously reported that malignant hypertension is associated with impaired capillary density of target organs. Here, we tested the hypothesis that stabilization of hypoxia-inducible factor (HIF) in a modified “preconditioning” approach prevents the development of malignant hypertension. To stabilize HIF, we employed pharmacological inhibition of HIF prolyl hydroxylases (PHD), that profoundly affect HIF metabolism. Methods: Two-kidney, one-clip renovascular hypertension (2K1C) was induced in rats; controls were sham operated. 2K1C rats received either intermittent injections of the PHD inhibitor ICA (2-(1-chloro-4-hydroxyisoquinoline-3- carboxamido) acetate) or placebo. Thirty-five days after clipping, the frequency of malignant hypertension was assessed (based on weight loss and the occurrence of characteristic vascular lesions). In addition, kidney injury was compared between all ICA treated versus all placebo treated 2K1C, regardless of the occurrence of malignant hypertension. HIF stabilization was evaluated by immunohistochemistry, and HIF target gene expression by RT-PCR. Results: Blood pressure was elevated to the same degree in ICA- and placebotreated 2K1C compared to control rats. ICA treatment did not affect the frequency of malignant hypertension or the extent of kidney tissue fibrosis, inflammation, or capillary density. There was a trend towards higher mortality and worse kidney function in ICA-treated 2K1C rats. ICA increased the number of HIF-1α-positive renal tubular cell nuclei and induced several HIF-1 target genes. In contrast, expression of HIF-2α protein as well as HIF-2 target genes were markedly enhanced by 2K1C hypertension, irrespective of ICA treatment. Discussion: We conclude that intermittent PHD inhibition did not ameliorate severe renovascular hypertension in rats. We speculate that the unexpected strong renal accumulation of HIF-2α in renovascular hypertension, which could not be further augmented by ICA, may contribute to the lack of a benefit from PHD inhibition. [ABSTRACT FROM AUTHOR]

Background: Malignant hypertension is a model of the rapid changes that a high afterload and renin-angiotensin levels can induce on target organs such as the heart. We present a case of a young man affected by malignant hypertension with multi-organ involvement who showed quick remission after adequate antihypertensive treatment. Case summary: A 41-year-old jazz pianist with a family history of coronary artery disease presented to the emergency department for asthenia and epigastric pain, associated with right eye visual impairment. No neurological symptoms. An echocardiogram showed left ventricular hypertrophy with severe impairment of ejection fraction (22%) due to global hypokinesia. There was renal insufficiency (creatinine 2.51 mg/dl, eGFR 34 ml/min, HS Troponin T 127 pg/dl, NT pro-BNP 22,672 pg/ml, CRP 32 mg/L, sodium 129 mEq/L with normal kaliemia. The following day, anterior T wave inversion was observed in the electrocardiogram. At a cardiac magnetic resonance. Concentric hypertrophy of the left ventricle was observed with normal myocardial T1 mapping values (1100 ± 38 ms), ruling out Fabry's disease. There was no obvious myocardial edema at T2 weighted. The viral panel for acute myocarditis resulted in negative. After the exclusion of all possible secondary causes, malignant essential hypertension was the final diagnosis, and additional tests confirmed multi-organ damage. An ophthalmological examination demonstrated hypertensive retinopathy with hemorrhages, cottony exudates, and macular lipid exudation, especially in the right eye. A brain MRI showed small areolas of T2 hyperintensity in the white matter of both cerebral hemispheres, suggestive of chronic microangiopathy, and left nuclear micro lacunar ischemia. The patient was treated with full-dose calcium channel blockers (amlodipine 10 mg), beta-blockers (bisoprolol 10 mg), alpha2 agonists (slow-release clonidine patch), selective a1 blocker (doxazosine 16 mg), and furosemide 50 mg. After the exclusion of renal stenosis and improvement of renal function, Ramipril was added up to a final dose of 10 mg daily. In parallel with the achievement of a satisfactory blood pressure control, visual impairment disappeared with a reduction of retinal ischemic exudates and hemorrhages at the ophthalmological follow-up assessment. At the time of discharge, an echocardiographic re-evaluation confirmed concentric hypertrophy of the left ventricle with markedly reduced wall thicknesses, with a partial recovery in left ventricular ejection function (EF 44%). Six months after discharge, the patient is in good general condition under optimal medical therapy (without furosemide) with normalized blood pressure values (130–140/80 mmHg). At 6 months from discharge, creatinine was only mildly increased (1.5 mg/dl, eGFR 59.5 ml/min) and NT-pro BNP nearly normalized (452 pg/ml). Discussion: Malignant hypertension is a cardiovascular emergency and requires immediate and careful intervention to lower blood pressure and reduce organ injury. It is an exclusion diagnosis that can be advanced once the causes of secondary hypertension have been excluded. Remission of organ injury is possible and might be rapid under adequate antihypertensive treatment, but patients require a close follow-up. [ABSTRACT FROM AUTHOR]

Objectives Scleroderma renal crisis (SRC) is a rare vascular complication of systemic sclerosis with substantial risks for end-stage renal disease and premature death. Activating autoantibodies (Abs) targeting the angiotensin II type 1 (AT1R) and the endothelin-1 type A receptor (ETAR) have been identified as predictors for SRC. Here, we sought to determine their pathogenic significance for acute renal vascular injury potentially triggering kidney failure and malignant hypertension. Methods IgG from patients with SRC was studied for AT1R and ETAR dependent biologic effects on isolated rat renal interlobar arteries and vascular cells including contraction, signalling and mechanisms of receptor activation. Results In myography experiments, patient IgG exerted vasoconstriction sensitive to inhibition of AT1R and ETAR. This relied on MEK-ERK signalling indicating functional relevance of anti-AT1R and anti-ETAR Abs. The contractile response to angiotensin II and endothelin-1 was amplified by patient IgG containing anti-AT1R and anti-ETAR Abs with substantial crosstalk between both receptors implicating autoimmune receptor hypersensitization. Co-immunoprecipitation experiments indicated heterodimerization between both receptor types which may enable the observed functional interrelation by direct structural interactions. Conclusion We provide experimental evidence that agonistic Abs may contribute to SRC. This effect is presumably related to direct receptor stimulation and additional allosteric effects, at least in heterodimeric receptor constellations. Novel therapies targeted at autoimmune hyperactivation of AT1R and ETAR might improve outcomes in severe cases of SRC. [ABSTRACT FROM AUTHOR]

Background: We present a case of hypertensive choroidopathy due to malignant hypertension with exudative retinal detachment as a sole finding. We use OCT- angiography for initial diagnosis and report findings from extensive follow up. Case presentation: A 51-year-old female with no past medical history, presented to our clinic with painless loss of vision in her left eye. Fundus examination revealed only exudative retinal detachment in her left eye that was confirmed with Optical Coherence Tomography. Fluorescein angiography showed hyperfluorescent spots with leakage in late phases. OCTA manifested a focal dark area in the choriocapillaris slab corresponding to flow signal voids, signifying regions of non-perfusion. Her blood pressure was 220/120 mmHG. Complete blood work -up failed to reveal any other possible etiology. During follow-up period of 9 months blood pressure normalized, patient regained visual function and choriocapillaris perfusion was completely restored. Discussions and conclusions: Hypertensive choroidopathy with exudative retinal detachment can be the only sign of malignant hypertension and no pre-existing history of a systemic disease is required in order to become apparent. OCTA reveals areas of non-perfusion at choriocapillaris level, proving that it is an essential tool in the diagnosis and follow up of patients with hypertensive choroidopathy. Finally, we propose that early diagnosis prevents permanent damage of the RPE and leads to complete choroidal remodeling and better visual outcomes. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Isolated spontaneous renal artery dissection (ISRAD) is a rare clinical phenomenon that typically presents with refractory hypertension and abdominal and flank pains but may also induce hematuria and severe headache. The protean manifestations of ISRAD may be misinterpreted as renal colic or intrabdominal pathology and requires a high index of clinical suspicion. Computerized tomography (CT), coupled with angiography (CTA) clarifies the diagnosis. No evidence-based management exists as intervention is reserved for persistent symptoms despite optimal medical treatment. The sporadic incidence remains limited to but a few cases illustrating endovascular management. Herein between 2014 and 2019 are presented three, healthy Caucasian males, ages from 47 to 54 years-old who presented with symptomatic ISRAD, coupled with limited renal infarctions, and treated with mid-renal and segmental arterial stenting.

Abstract Background: To report the findings of fluorescein angiography (FA) and optical coherence tomography angiography (OCTA) in a patient with malignant hypertensive retinopathy. Case Report: A 41year-old male was referred to our clinic with sudden visual loss in both of his eyes after an acute rise of blood pressure (200/150 mmHg). Optic disc swelling, flame shape hemorrhages especially around the optic disc, arterial narrowing, vessel tortuosity, cotton wool spots, hard exudate deposition, and multiple deep orange spots (Elschnig spots) were visible in both eyes. In the OCTA, disruption in the normal tapering patterns of the superficial and deep capillary plexuses was observed. Elschnig spots were observed as hypointense spots in the choriocapillaris slab. Leakage of the optic nerve head was seen in the FA. Conclusion: When compared with the FA, the OCTA can illustrate the ischemic areas and the Elschnig spots with greater detail.

Background To study the prevalence and types of hypertension‐mediated organ damage and the prognosis of patients presenting to the emergency department (ED) with hypertensive emergencies. Methods and Results PubMed was queried from inception through November 30, 2021. Studies were included if they reported the prevalence or prognosis of hypertensive emergencies in patients presenting to the ED. Studies reporting data on hypertensive emergencies in other departments were excluded. The extracted data were arcsine transformed and pooled using a random‐effects model. Fifteen studies (n=4370 patients) were included. Pooled analysis demonstrates that the prevalence of hypertensive emergencies was 0.5% (95% CI, 0.40%–0.70%) in all patients presenting to ED and 35.9% (95% CI, 26.7%–45.5%) among patients presenting in ED with hypertensive crisis. Ischemic stroke (28.1% [95% CI, 18.7%–38.6%]) was the most prevalent hypertension‐mediated organ damage, followed by pulmonary edema/acute heart failure (24.1% [95% CI, 19.0%–29.7%]), hemorrhagic stroke (14.6% [95% CI, 9.9%–20.0%]), acute coronary syndrome (10.8% [95% CI, 7.3%–14.8%]), renal failure (8.0% [95% CI, 2.9%–15.5%]), subarachnoid hemorrhage (6.9% [95% CI, 3.9%–10.7%]), encephalopathy (6.1% [95% CI, 1.9%–12.4%]), and the least prevalent was aortic dissection (1.8% [95% CI, 1.1%–2.8%]). Prevalence of in‐hospital mortality among patients with hypertensive emergency was 9.9% (95% CI, 1.4%–24.6%). Conclusions Our findings demonstrate a pattern of hypertension‐mediated organ damage primarily affecting the brain and heart, substantial cardiovascular renal morbidity and mortality, as well as subsequent hospitalization in patients with hypertensive emergencies presenting to the ED.

Introduction: We previously reported that malignant hypertension is associated with impaired capillary density of target organs. Here, we tested the hypothesis that stabilization of hypoxia-inducible factor (HIF) in a modified “preconditioning” approach prevents the development of malignant hypertension. To stabilize HIF, we employed pharmacological inhibition of HIF prolyl hydroxylases (PHD), that profoundly affect HIF metabolism.Methods: Two-kidney, one-clip renovascular hypertension (2K1C) was induced in rats; controls were sham operated. 2K1C rats received either intermittent injections of the PHD inhibitor ICA (2-(1-chloro-4-hydroxyisoquinoline-3-carboxamido) acetate) or placebo. Thirty-five days after clipping, the frequency of malignant hypertension was assessed (based on weight loss and the occurrence of characteristic vascular lesions). In addition, kidney injury was compared between all ICA treated versus all placebo treated 2K1C, regardless of the occurrence of malignant hypertension. HIF stabilization was evaluated by immunohistochemistry, and HIF target gene expression by RT-PCR.Results: Blood pressure was elevated to the same degree in ICA- and placebo-treated 2K1C compared to control rats. ICA treatment did not affect the frequency of malignant hypertension or the extent of kidney tissue fibrosis, inflammation, or capillary density. There was a trend towards higher mortality and worse kidney function in ICA-treated 2K1C rats. ICA increased the number of HIF-1α-positive renal tubular cell nuclei and induced several HIF-1 target genes. In contrast, expression of HIF-2α protein as well as HIF-2 target genes were markedly enhanced by 2K1C hypertension, irrespective of ICA treatment.Discussion: We conclude that intermittent PHD inhibition did not ameliorate severe renovascular hypertension in rats. We speculate that the unexpected strong renal accumulation of HIF-2α in renovascular hypertension, which could not be further augmented by ICA, may contribute to the lack of a benefit from PHD inhibition.

Introduction: Arterial hypertension (AHT) can debut with a hypertensive crisis (HC), whose prevalence is 1-2%. They are classified as: urgency (HU), emergency (HE) and pseudocrisis (HP). Objective: To determine the clinical characterization and identification of factors associated with HC in the emergency service. Materials and method: Analytical cross-sectional study based on records of patients ≥18 years who attended the emergency room between 2020 and 2021 with blood pressure (BP) ≥180/120 mmHg and a confirmed diagnosis of HC. Prevalences were estimated, odds ratio and logistic regression models were calculated, with confidence levels of 95%. Results: The prevalence of HC was 1.7%. 59.4% were women, aged 66.34 ± 13.8 years, 88.7% had a history of hypertension. The most frequent chronic treatment was angiotensin II receptor blockers (ARB) (52.9%). The most common HC was HE (54.6%). BP levels were higher in the HE group (p < 0.001). Headache was the most frequent symptom (30.0%). Chronic use of angiotesin-converting enzyme (ACE) inhibitors was associated with a lower probability of presenting with HE (68%). Chronic kidney disease was associated with an increased probability of HU (OR = 2.77; CI 95% = 1.31-5.87), coronary heart disease and cerebrovascular disease with an increased probability of HE (OR = 3.11; CI 95% = 1.31-5.87). CI 95 = 1.15-8.45 and OR = 4.4; CI 95% = 1.69-11.58). The most used therapy for HU and HP was losartan (47.56 and 43.14%), for HE labetalol (48.75%). Conclusions: Knowing the frequency and association of HC allows to improve the medical assessment, the use of tests, their treatment and prognosis. [ABSTRACT FROM AUTHOR]

This report presented the longitudinal optical coherence tomography angiography (OCTA) findings in a patient with malignant hypertension choroidopathy. An 87-year-old woman presented with acute unilateral central vision loss in the setting of hypertensive emergency. Spectral domain optical coherence tomography showed massive serous macular detachment. OCTA revealed extensive flow loss in the neuroretina and choriocapillaris. With blood pressure (BP) normalization and without any ocular intervention, visual acuity recovered on subsequent visits. Flow loss in the neuroretinal capillaries persisted, but significant improvement in the perfusion of the choriocapillaris was observed. This case demonstrates extensive choriocapillaris flow loss in the acute phase of malignant hypertension, and a temporal relationship between BP normalization, improvement of choriocapillaris perfusion, and decrease of subretinal fluid, providing additional insight into the pathophysiology of this life- and sight-threatening systemic condition.

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.

Ege Sinan Torun,1 Nevzat Koca,2 Yasemin Yalç&inodot;nkaya,2 Bahar Art&inodot;m Esen,2 Ahmet Gül,2 Murat &Idot;nanç2 1Division of Rheumatology, Department of Internal Medicine, Prof. Dr. Cemil Ta&scedil;c&inodot;o&gbreve;lu City Hospital, Istanbul, Turkey; 2Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University, Istanbul, TurkeyCorrespondence: Ege Sinan Torun, Prof. Dr. Cemil Ta&scedil;c&inodot;o&gbreve;lu City Hospital, Kaptan Pa&scedil;a, SSK Okmeydan&inodot; Hst. No: 25, &Scedil;i&scedil;li, &Idot;stanbul, 34384, Turkey, Tel +90-537 229 17 99, Email egesinantorun@hotmail.comAbstract: A 20 year old woman presented with right arm pain. Pulses of right upper extremity were weak, acute phase reactants were elevated and MR angiography demonstrated total occlusion of subclavian artery and right axillary artery with collaterals. The diagnosis was Takayasu arteritis and she was treated with prednisolone, azathioprine and acetylsalicylic acid. During follow up, azathioprine was switched to methotrexate. Three years later, patient presented with elevated blood pressure. CT angiography demonstrated reduced calibration of the aorta and almost total occlusion of the lumen of proximal parts of left and right renal arteries. C-reactive protein was elevated. Steroid dose was increased, methotrexate was discontinued and IV tocilizumab and antihypertensive medications were initiated. One month later, she presented to emergency department with elevated blood pressure and blurred vision in the left eye. Fundoscopic examination revealed bilateral grade 3 hypertensive retinopathy and serous detachment of retina in the left eye. Laboratory results revealed normal CRP, elevated creatinine, elevated lactate dehydrogenase, thrombocytopenia, low hemoglobin and low haptoglobin. Peripheral blood smear revealed 2– 3 schistocytes in every field. She was admitted to rheumatology department with the diagnosis of thrombotic microangiopathy secondary to malignant hypertension. IV tocilizumab was administered, and methylprednisolone was maintained at a dose of 20 mg/day. Despite treatment with maximum dose of six antihypertensive medications, her blood pressure was not controlled adequately and she became hypervolemic. After undergoing ultrafiltration, balloon dilation was performed in the left renal artery, and a stent was placed there. After stent placement, creatinine and platelet count normalized, hemoglobin increased and hypertension was controlled. In this case, malignant hypertension which was triggered by bilateral renal artery stenosis due to Takayasu arteritis had caused acute kidney injury and advanced stage hypertensive retinopathy. In addition, unlike other Takayasu arteritis cases with malignant hypertension, thrombotic microangiopathy was also detected.Keywords: Takayasu arteritis, malignant hypertension, thrombotic microangiopathy

Objective: Overweight and obesity is a risk factor for hypertension. Malignant hypertension (MHT) is the most severe form of hypertension, and thrombotic microangiopathy (TMA), one of its complications, has been linked to significant renal outcomes. However, the impact of overweight and obesity on renal prognosis in MHT patients with TMA is not well understood., Methods: This was a prospective cohort enrolled 288 MHT patients with renal TMA from 2008 to 2023. The clinical and histopathological characteristics were recorded based on body mass index (BMI, < 25 and ≥ 25 kg/m 2 ). The outcome was the incidence of kidney failure. The associations of BMI with kidney failure were examined in logistic regression models., Results: Among 288 patients, 180 (62.5%) progressed to kidney failure, including 113 (68.5%) patients with BMI < 25 kg/m 2 . Participants with obesity had higher levels of hemoglobin, estimated glomerular filtration rate and C3, but lower levels of serum creatinine and IgA nephropathy. BMI ≥ 25 kg/m 2 was associated with a better outcome of kidney failure in MHT patients with TMA (odd ratios [ORs]: 0.49 [95% confidence interval (CI): 0.27-0.91], p = 0.025). Male, uric acid, onion skin lesions, and global sclerosis ratio were correlated with higher risk of kidney failure; serum albumin and treatment with renin-angiotensin system blockers were related to lower risk of kidney failure., Conclusions: In MHT patients with renal TMA, normal-weight rather than overweight was found to associate with a worse renal prognosis. Management efforts for MHT may be directed toward controlling body weight within a reasonable range for patients., (© 2024 The Author(s). Journal of Clinical Laboratory Analysis published by Wiley Periodicals LLC.)

A tricenarian female with a past medical history of status epilepticus secondary to posterior reversible encephalopathy syndrome (PRES) of unknown etiology presented with a 2-week history of double vision, dizziness, elevated blood pressure, and altered mental status. On hospital day 2, she experienced status epilepticus, during which her blood pressure rose to 240/160 from her baseline of around 140/90. The patient was subsequently intubated for airway protection and transferred to the intensive care unit, where she was started on a nicardipine drip. Due to her history of thrombotic microangiopathy, empiric treatment with plasma exchange and prednisone was started but discontinued when ADAMTS13 came back negative. Urine metanephrines also were found to be negative. Computed tomography angiography of the abdomen showed left renal artery stenosis and stent was placed. Remarkably, over the coming days, her blood pressure normalized, and her neurologic symptoms significantly improved. As a result, antihypertensive medications were titrated down, and the patient was finally provided with a cause of her repetitive, life-threatening episodes of PRES.

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.

Malignant hypertension (MH) is characterized by severe hypertension (usually grade 3) associated with fundoscopic changes (flame hemorrhages and/or papilledema), microangiopathy and disseminated intravascular coagulation. In addition encephalopathy, acute heart failure and acute deterioration in renal function may be present. The term “malignant” reflects the very poor prognosis for this condition if untreated. When severe hypertension is associated with hypertension-mediated organ damage (HMOD) a life-threatening situation that requires immediate but careful intervention occurs (hypertensive emergency). In the last few years an increase in the number of patients with malignant hypertension has been observed, especially among those patients with black ethnicity. Limited access to treatment and the poor adherence to anti-hypertensive therapy may contribute to the development of hypertensive emergencies. It is considered appropriate to study patients in order to rule out thrombotic thrombocytopenic purpura and hemolytic uremic syndrome. In fact, the microvascular damage caused by malignant hypertension can favor intravascular hemolysis like Thrombotic Microangiopathies (TMs). TMs may present in three different clinical conditions: typical hemolytic uremic syndrome (HUS), atypical hemolytic uremic syndrome (aHUS) and thrombotic thrombocytopenic purpura (TTP). TMs can arise in the context of other pathological processes, including malignant hypertension.

Tuberculosis is common in chronic kidney disease (CKD). There are reports of adults with CKD experiencing hypertensive crises and an increase in dosages and number of antihypertensive drugs after starting antitubercular therapy (ATT). Studies have demonstrated that rifampicin could increase the metabolism of beta-blockers, calcium channel blockers, and prazosin. There are no studies or reports of worsening blood pressure (BP) control in children with CKD after starting ATT. Here, we report three cases of children who developed hypertensive crises after starting ATT. All patients presented in emergency with the acute severe rise of BP with breathlessness and or visual blurring. All of them showed retinal changes suggestive of malignant hypertension. They needed parenteral therapy to control BP. They also needed the escalation of antihypertensives and the addition of other drugs. One patient needed a withdrawal of rifampicin. It is suggested that children with CKD should be monitored for BP control after instituting ATT.