Malignant arterial hypertension in a 2-month-old girl: Etiological diagnosis and treatment.

A 2-month-old girl presented with malignant arterial hypertension revealing bilateral renal artery stenosis secondary to neurofibromatosis type 1 (NF1). Life-supporting care was initiated immediately. High-dose peripheral vasodilator therapy induced life-threatening toxicity; vascular surgery was therefore performed. Technical difficulties due to the young age and low body weight of the patient resulted in fatal bleeding. Renovascular disease is an important cause of pediatric hypertension. NF1-associated renovascular hypertension in young pediatric patients is rare, and its highly specialized management is best delivered via a multidisciplinary approach. The long-term prognosis remains poor.

Retinopatia hipertensiva maligna secundária a lúpus eritematoso sistêmico em adolescente / Malignant hypertensive retinopathy secondary to systemic lupus erythematous in adolescent

RESUMO O lúpus eritematoso sistêmico é uma doença que pode apresentar comprometimento oftalmológico geralmente benigno, sendo as alterações mais encontradas a síndrome do olho seco e a catarata. Nos pacientes com a doença estável, o dano oftalmológico parece estar relacionado ao tratamento sistêmico a longo prazo, o que enfatiza a importância do exame oftalmológico completo de rotina. Porém, quando a doença está em franca atividade e, em especial, quando há o envolvimento renal, deve-se iniciar o tratamento precoce com corticoterapia sistêmica e com medidas de suporte, para se evitarem repercussões mais complexas, como as crises hipertensivas que podem levar ao óbito.

ABSTRACT Systemic lupus erythematosus may present ophthalmological involvement, usually benign, and the most common changes are dry eye syndrome and cataract. In patients with stable disease, ophthalmologic damage appears to be related to long-term systemic treatment, emphasizing the importance of routine complete ophthalmologic examination. However, in full-blown disease, especially when there is renal involvement, early treatment should start with systemic steroid therapy and supportive measures, to avoid major repercussions, such as hypertensive crises that may lead to death.

Hipertensión maligna y feocromocitoma: reporte de un caso / Malignant hypertension and pheochromocytoma: a case report

La hipertensión arterial maligna se define por cifras extremadamente altas de presión arterial asociadas a daño orgánico. Constituye una causa de emergencia hipertensiva donde coexisten cifras elevadas de presión arterial, con hemorragia y exudados bilaterales retinianas (retinopatía hipertensiva grado III), con o sin papiledema (retinopatía hipertensiva grado IV), asociada usualmente a lesión renal o cardíaca. En un 1% de los casos es secundaria a causas endocrinológicas, entre ellas, la más frecuente: el feocromocitoma, que clásicamente se ha caracterizado por la tríada cefalea, sudoración y palpitaciones. Sin embargo, no existe un hallazgo clínico único que tenga un valor significativo en su diagnóstico. A continuación, presentamos el caso de una paciente de 23 años con emergencia hipertensiva y masa suprarrenal asociado a retinopatía hipertensiva grado IV. (AU)

Malignant arterial hypertension is defined by extremely high levels of pressure associated with organ damage. It is a cause of hypertensive emergency and is defined by the coexistence of high blood pressure and bilateral retinal haemorrhage or exudates (grade III hypertensive retinopathy), with or without papilloedema (grade IV hypertensive retinopathy) currently associated with organ damage such as renal or cardiac failure. Around 1% of malignant arterial hypertension is secondary to endocrinological causes, including the most common: pheochromocytoma, which is classically characterized by the triad: headache, sweating and palpitations. However, there is no single clinical finding that is of significant value in its diagnosis. We now present the case of a 23-year-old patient with a hypertensive emergency, an adrenal mass associated with grade IV hypertensive retinopathy. (AU)

[Malignant hypertension and pheochromocytoma: a case report]. / Hipertensión maligna y feocromocitoma: reporte de un caso.

Malignant arterial hypertension is defined by extremely high levels of pressure associated with organ damage. It is a cause of hypertensive emergency and is defined by the coexistence of high blood pressure and bilateral retinal haemorrhage or exudates (grade III hypertensive retinopathy), with or without papilloedema (grade IV hypertensive retinopathy) currently associated with organ damage such as renal or cardiac failure. Around 1% of malignant arterial hypertension is secondary to endocrinological causes, including the most common: pheochromocytoma, which is classically characterized by the triad: headache, sweating and palpitations. However, there is no single clinical finding that is of significant value in its diagnosis. We now present the case of a 23-year-old patient with a hypertensive emergency, an adrenal mass associated with grade IV hypertensive retinopathy.

[A study on malignant arterial hypertension: about 168 cases at the unit of nephrology-internal medecine of the University Hospital Center, Treichville, Abidjan]. / Hypertension artérielle maligne en milieu néphrologique à Abidjan: à propos de 168 cas colligés au Service de Néphrologie-Médecine Interne du Centre Hospitalier Universitaire de Treichville.

INTRODUCTION: malignant arterial hypertension (MAH) is a nosologic disorder which has not been described in Nephrology. The purpose of this study was to describe the profile of patients with MAH in the Division of Nephrology and to identify prognostic factors. METHODS: we conducted a retrospective, descriptive and analytical study from January 2013 to December 2018 in the Unit of Nephrology of the University Hospital Center in Treichville. The diagnosis of MAH was retained in patients with diastolic blood pressure (DBP) ≥ 130 mmHg, Keith Wegener grade III/IV hypertensive retinopathy, one or multiple visceral, cardiac and/or brain and/or renal diseases. RESULTS: we collected data from 168 patients. The average age of patients was 41.10 ± 14.86 years, with male predominance (sex ratio 1.54). Cardiovascular risk factors were AH (79.20%), alcohol (32.10%), tobacco (19.60%), chronic kidney disease (15.30%) and diabetes (11.30%). They were admitted with dyspnea (39.29%), hypertensive crisis (26.16%), consciousness disorders (10.12%). Clinical examination showed anemia (82.10%), lower limb edema (63.10%), acute pulmonary edema (37.50%). Arterial hypertension resulted in renal failure (95,9%), left ventricular hypertrophy (92.81%), stroke (16,67%), and cardiac and renal involvement (85%). Renal failure was chronic in 78% of cases. The causes of MAH were essential AH (56,8%), chronic glomerulonephritis (29.8%), and diabetes (6%). Outcome was favorable in 66,7% of cases and overall mortality rate was 25.6%. In multivariate analysis uremia ≥ 2g/l [OR=5,07; 95%CI = 2,39-10.75; p = 0.0001], hperkalaemia [OR = 3.50; 95% CI = 1.70 - 7.19; p = 0.001], hyponatremia [OR = 2.90; 95% CI= 1.40 - 6.03; p = 0.004], haemoglobin level < 12g/dl [OR=5,91; 95% CI=1,34-26,00; p=0,019] and end-stage renal disease [OR = 6.06; 95% CI = 2.04 - 18.18; p = 0.001] were factors associated with the occurrence of death. CONCLUSION: MAH is a consequence of poorly treated or untreated AH. It mainly affects young adults with multivisceral complications. In our Hospital, these were dominated by end-stage chronic renal disease. Hence the importance of early diagnosis and adequate management in patients with AH.

Thrombotic microangiopathy in aHUS and beyond: clinical clues from complement genetics.

Studies of complement genetics have changed the landscape of thrombotic microangiopathies (TMAs), particularly atypical haemolytic uraemic syndrome (aHUS). Knowledge of complement genetics paved the way for the design of the first specific treatment for aHUS, eculizumab, and is increasingly being used to aid decisions regarding discontinuation of anti-complement treatment in this setting. Complement genetic studies have also been used to investigate the pathogenic mechanisms that underlie other forms of HUS and provided evidence that contributed to the reclassification of pregnancy- and postpartum-associated HUS within the spectrum of complement-mediated aHUS. By contrast, complement genetics has not provided definite evidence of a link between constitutional complement dysregulation and secondary forms of HUS. Therefore, the available data do not support systematic testing of complement genes in patients with typical HUS or secondary HUS. The potential relevance of complement genetics for distinguishing the underlying mechanisms of malignant hypertension-associated TMA should be assessed with caution owing to the overlap between aHUS and other causes of malignant hypertension. In all cases, the interpretation of complement genetics results remains complex, as even complement-mediated aHUS is not a classical monogenic disease. Such interpretation requires the input of trained geneticists and experts who have a comprehensive view of complement biology.

Scleroderma renal crisis.

Scleroderma renal crisis (SRC) is a rare but life-threatening complication of systemic sclerosis (SSc) characterized by malignant hypertension and acute kidney injury. Historically, SRC was the leading cause of death in SSc. However, with the advent of angiotensin converting enzyme (ACE) inhibitors, mortality rates have decreased significantly. Nevertheless, one-year outcomes remain poor, with over 30% mortality and 25% of patients remaining dialysis-dependent. There is an urgent need to improve early recognition and treatment, and to identify novel treatments to improve outcomes of SRC. In this chapter, the clinical features, classification, pathophysiology, differential diagnosis, management and outcomes of SRC are presented. Specific issues relating to pregnancy, prophylactic ACE inhibition and management of essential hypertension are also discussed.

Elevated Plasma Renin Activity Caused by Accelerated-malignant Hypertension in a Patient with Aldosterone-producing Adenoma Complicated with Renal Insufficiency.

During the malignant phase of hypertension in patients with primary aldosteronism complicated with severe renal failure, the plasma renin activity may markedly increase with a false negative screening result for primary aldosteronism, thus potentially leading to a missed diagnosis of primary aldosteronism. We herein report the case of 37-year-old man who presented with accelerated-malignant hypertension complicated with severe renal insufficiency. The plasma renin activity was markedly increased in the malignant phase of hypertension, which were atypical results for primary aldosteronism. However, a plain abdominal computed tomography scan revealed a left adrenal nodule, which was diagnosed as aldosterone-producing adenoma by adrenal venous sampling.

Generation of a Core Set of Items to Develop Classification Criteria for Scleroderma Renal Crisis Using Consensus Methodology.

OBJECTIVE: To generate a core set of items to develop classification criteria for scleroderma renal crisis (SRC) using consensus methodology. METHODS: An international, multidisciplinary panel of experts was invited to participate in a 3-round Delphi exercise developed using a survey based on items identified by a scoping review. In round 1, participants were asked to identify omissions and clarify ambiguities regarding the items in the survey. In round 2, participants were asked to rate the validity and feasibility of the items using Likert-type scales ranging from 1 to 9 (where 1 = very invalid/unfeasible, 5 = uncertain, and 9 = very valid/feasible). In round 3, participants reviewed the results and comments from round 2 and were asked to provide final ratings. Items rated as highly valid and feasible (median scores ≥7 for each) in round 3 were selected as the provisional core set of items. A consensus meeting using a nominal group technique was conducted to further reduce the core set of items. RESULTS: Ninety-nine experts from 16 countries participated in the Delphi exercise. Of the 31 items in the survey, consensus was achieved on 13, in the categories hypertension, renal insufficiency, proteinuria, and hemolysis. Eleven experts took part in the nominal group technique discussion, where consensus was achieved in 5 domains: blood pressure, acute kidney injury, microangiopathic hemolytic anemia, target organ dysfunction, and renal histopathology. CONCLUSION: A core set of items that characterize SRC was identified using consensus methodology. This core set will be used in future data-driven phases of this project to develop classification criteria for SRC.

Anticentromere Antibody-positive Scleroderma Renal Crisis Requiring Dialysis.

A 70-year-old man with prior Raynaud's phenomena developed hypertension and renal insufficiency. Raynaud's phenomena, finger skin thickening, interstitial lung disease, and positive anticentromere antibody findings indicated systemic sclerosis (SSc). Based on the presence of SSc, severe hypertension with rapidly progressive renal failure, and proliferative and obliterative arteriolar vasculopathy, scleroderma renal crisis (SRC) was diagnosed. Despite good blood pressure control with antihypertensive drugs, hemodialysis was initiated and could not be withdrawn owing to unimproved renal dysfunction. Although SRC in anticentromere antibody-positive limited cutaneous SSc is extremely rare, some patients may develop SRC, and their renal prognosis may be poor.

'Malignant' hypertension from hyperaldosteronism: a case report.

Adrenocortical carcinomas (ACC) are rare with an incidence of 0.7-2 per million population per year and account for only 0.05%-2% of all malignant tumors. While majority of the functional ACC present as Cushing syndrome, recurrent hyperaldosteronism from metastatic ACC is exceedingly rare. We describe a 67-year old female presented with hypertensive urgency & hypokalemia as a result of hyperaldosteronism from an 8-cm right ACC. She underwent a radical right nephrectomy with adrenalectomy that normalized her blood pressure. However, a few years later she presented again with resistant hypertension from hyperaldosteronism, raising the suspicion of recurrence of ACC. A contrast-enhanced CT scan showed a normal left adrenal gland but revealed pulmonary metastases of ACC based on a lung biopsy. Chemotherapy was complicated with side effects leading to refusal of further chemotherapy, henceforth requiring high dose of spironolactone for blood pressure control. Despite curative surgery, metastatic functional ACC should be considered in patients presenting with secondary hypertension from recurrent hyperaldosteronism, due to its high recurrence rate. Besides standard cancer surveillance after a curative surgery, meticulous monitoring of blood pressure is a simple yet crucial way to detect cancer recurrence early.

Valsartan Effective for Malignant Hypertension after Aortic Dissection with Renal Artery Involvement.

When aortic dissections extend to the renal arteries, reductions in renal blood flow can cause marked increases in renin production. The resultant rise in angiotensin II can lead to difficult-to-control blood pressure, despite normal postdissection antihypertensive agents. We highlight a case of a postdissection patient with malignant hypertension refractory to eight different enteral antihypertensives. Angiotensin-converting enzyme inhibitors and angiotensin receptor blockers had been held due to postoperative acute kidney injury. A single dose of valsartan, administered on day 12, produced a marked drop in blood pressure, alleviation of encephalopathy, and allowed for cancellation of a planned tracheostomy. A serum renin level was found to be 50 times the normal upper limit. In patients with aortic dissection and renal artery involvement, angiotensin-modifying agents may warrant earlier administration to combat this unique cause of hypertension.

Thrombotic microangiopathy due to malignant hypertension complicated with late-onset bleeding after renal biopsy.

A 47-year-old man presented at a local ophthalmological hospital with blurred vision. He had been diagnosed with hypertensive retinopathy and renal failure and was referred to our hospital for treatment. A renal biopsy was done to evaluate pathology of high proteinuria, hematuria, and rapidly progressive glomerulonephritis. Blood pressure remained high despite antihypertensive therapy; anemia and thrombocytopenia gradually progressed. Thrombotic microangiopathy (TMA) was suspected based on red blood cell fragmentation due to hemolytic anemia, thrombocytopenia, and renal failure. However, plasma exchange resolved neither thrombocytopenia nor renal failure, and anemia gradually progressed. Backache suddenly developed 13 days later, and CT findings indicated a retroperitoneal hematoma secondary to bleeding from the kidney. Selective renal artery embolization via angiography stopped the bleeding, but the patient went into hemorrhagic shock. Pathological findings on renal biopsy were identical to those in malignant hypertension, namely an edematous membrane lining, thickened arterioles, and stenosis. We diagnosed thrombotic microangiopathy due to malignant hypertension, without decrease in activities of ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 motif) or its antibodies. Renal failure did not improve, and continuous hemodiafiltration was needed. This procedure stabilized blood pressure and improved the TMA.

[Moyamoya disease associated with kidney angiodysplasia in a child]. / Maladie de Moya-Moya associée à une angiodysplasie rénale chez un enfant.

INTRODUCTION: Moyamoya disease is a progressive, chronic occlusive vascular disease of the circle of Willis arteries leading to the development of collateral circulation to compensate the occlusion. CASE REPORT: An 11-year-old girl presented an abrupt loss of consciousness and a right-sided motor deficit. Clinical examination found hypertension with 220/120mmHg arterial blood pressure. Brain magnetic resonance imaging showed a large left hemispheric ischemic lesion. Cerebral arteriography demonstrated an abnormal anastomotic vascular network with fine arteriolar ends in several territories, with a "wisp of smoke" aspect arguing in favor of moyamoya disease. Renal arteriography revealed dysplasia of the upper polar branches of the right kidney. CONCLUSION: Moyamoya disease is a rare cause of stroke in children. Its association with renal angiodysplasia is unusual and may be responsible for malignant hypertension. Cognitive impairment and social dependence have recently been recognized as an important unresolved social issue. Affected children require medical-surgical, social, and psychological care.

Emergency Department Use among Patients with CKD: A Population-Based Analysis.

BACKGROUND AND OBJECTIVES: Although prior studies have observed high resource use among patients with CKD, there is limited exploration of emergency department use in this population and the proportion of encounters related to CKD care specifically. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: We identified all adults (≥18 years old) with eGFR<60 ml/min per 1.73 m2 (including dialysis-dependent patients) in Alberta, Canada between April 1, 2010 and March 31, 2011. Patients with CKD were linked to administrative data to capture clinical characteristics and frequency of emergency department encounters and followed until death or end of study (March 31, 2013). Within each CKD category, we calculated adjusted rates of overall emergency department use as well as rates of potentially preventable emergency department encounters (defined by four CKD-specific ambulatory care-sensitive conditions: heart failure, hyperkalemia, volume overload, and malignant hypertension). RESULTS: During mean follow-up of 2.4 years, 111,087 patients had 294,113 emergency department encounters; 64.2% of patients had category G3A CKD, and 1.6% were dialysis dependent. Adjusted rates of overall emergency department use were highest among patients with more advanced CKD; 5.8% of all emergency department encounters were for CKD-specific ambulatory care-sensitive conditions, with approximately one third resulting in hospital admission. Heart failure accounted for over 80% of all potentially preventable emergency department events among patients with categories G3A, G3B, and G4 CKD, whereas hyperkalemia accounted for almost one half (48%) of all ambulatory care-sensitive conditions among patients on dialysis. Adjusted rates of emergency department events for heart failure showed a U-shaped relationship, with the highest rates among patients with category G4 CKD. In contrast, there was a graded association between rates of emergency department use for hyperkalemia and CKD category. CONCLUSIONS: Emergency department use is high among patients with CKD, although only a small proportion of these encounters is for potentially preventable CKD-related care. Strategies to reduce emergency department use among patients with CKD will, therefore, need to target conditions other than CKD-specific ambulatory care-sensitive conditions.

Takayasu arteritis presenting with malignant hypertension; a rare manifestation of a rare disease: a case report and review of the literature.

Takayasu arteritis (TA) is a chronic inflammatory and obliterative disease of large vessels, which mainly affects the aorta and its major branches. TA can lead to renal failure and renovascular hypertension in 60% of patients; it is rare in children aged <10 years and, more rarely, it presents with malignant hypertension in the paediatric age group. Here we present a case of 9-year-old boy with TA who presented with malignant hypertension and required surgical intervention to control the blood pressure. Subsequently, his medications were titrated using 24 h ambulatory blood pressure monitoring (ABPM) and is doing well on follow-up.

[Hypertensive urgencies and emergencies]. / Urgencias y emergencias hipertensivas.

Hypertensive urgencies and emergencies are common situations in clinical practice. Hypertensive urgencies are characterized by acute elevation of blood pressure without target organ damage. Hypertensive emergencies are life-threatening situations characterized by acute elevation of blood pressure and target organ damage. The aims of blood pressure control, antihypertensive drugs to use and route of administration will depend on the presence or absence of target organ damage and individual patient characteristics. The correct diagnosis and treatment of these situations are essential for patient prognosis. © 2017 SEHLELHA. Published by Elsevier España, S.L.U. All rights reserved.

Potentially Preventable Hospitalization among Patients with CKD and High Inpatient Use.

BACKGROUND AND OBJECTIVES: Although patients with CKD are commonly hospitalized, little is known about those with frequent hospitalization and/or longer lengths of stay (high inpatient use). The objective of this study was to explore clinical characteristics, patterns of hospital use, and potentially preventable acute care encounters among patients with CKD with at least one hospitalization. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: We identified all adults with nondialysis CKD (eGFR<60 ml/min per 1.73 m2) in Alberta, Canada between January 1 and December 31, 2009, excluding those with prior kidney failure. Patients with CKD were linked to administrative data to capture clinical characteristics and frequency of hospital encounters, and they were followed until death or end of study (December 31, 2012). Patients with one or more hospital encounters were categorized into three groups: persistent high inpatient use (upper 5% of inpatient use in 2 or more years), episodic high use (upper 5% in 1 year only), or nonhigh use (lower 95% in all years). Within each group, we calculated the proportion of potentially preventable hospitalizations as defined by four CKD-specific ambulatory care sensitive conditions: heart failure, hyperkalemia, volume overload, and malignant hypertension. RESULTS: During a median follow-up of 3 years, 57,007 patients with CKD not on dialysis had 118,671 hospitalizations, of which 1.7% of patients were persistent high users, 12.3% were episodic high users, and 86.0% were nonhigh users of hospital services. Overall, 24,804 (20.9%) CKD-related ambulatory care sensitive condition encounters were observed in the cohort. The persistent and episodic high users combined (14% of the cohort) accounted for almost one half (45.5%) of the total ambulatory care sensitive condition hospitalizations, most of which were attributed to heart failure and hyperkalemia. Risk of hospitalization for any CKD-specific ambulatory care sensitive condition was higher among older patients, higher CKD stage, lower income, registered First Nations status, and those with poor attachment to primary care. CONCLUSIONS: Many hospitalizations among patients with CKD and high inpatient use are ambulatory care sensitive condition related, suggesting opportunities to improve outcomes and reduce cost by focusing on better community-based care for this population.