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.

Malignant Hypertension Complicated with Necrotizing Pancreatitis After Starting Treatment: A Case Report.

BACKGROUND Malignant hypertension (MHT), one of the severest forms of hypertension, can have deleterious effects on various organs, such as renal failure, retinopathy, and encephalopathy. These types of organ damage are common complications of MHT, but in several previous cases, damage to other organs, such as the gastrointestinal tract or pancreas, resulting from small vessel lesions, has also been reported, and these cases have had severe clinical outcomes and a poor prognosis. CASE REPORT A 32-year-old male patient with untreated hypertension of a 5-year duration presented with breathlessness and edema. His blood pressure was 220/144 mmHg, and he had renal dysfunction, congestive heart failure, and hypertensive retinopathy. He immediately received treatment, including antihypertensive agents and intermittent hemodialysis, but experienced epigastric pain for several days. A cystic lesion appeared in the pancreatic head, and his serum pancreatic enzymes were elevated. Based on these findings, acute pancreatitis with a cystic lesion was diagnosed. He first received fluid management, pain control, and parenteral nutrition but experienced 2 relapses. Finally, he received transpapillary endoscopic drainage for the cystic lesion with suspected walled-off necrosis. Thereafter, his symptoms improved. CONCLUSIONS The present case of MHT is the first to demonstrate acute necrotizing pancreatitis and it illustrates the difficulty of treatment. Therefore, if a patient with MHT presents with abdominal pain, a thorough workup, including contrast-enhanced computed tomography, should be performed to rule out significant organ involvement.

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.

Hypertensive emergencies and urgencies in emergency departments: a systematic review and meta-analysis.

OBJECTIVES: The prevalence of hypertensive emergencies and urgencies and of acute hypertension-mediated organ damage (aHMOD) in emergency departments is unknown. Moreover, the predictive value of symptoms, blood pressure (BP) levels and cardiovascular risk factors to suspect the presence of aHMOD is still unclear. The aim of this study was to investigate the prevalence of hypertensive emergencies and hypertensive urgencies in emergency departments and of the relative frequency of subtypes of aHMOD, as well as to assess the clinical variables associated with aHMOD. METHODS: We conducted a systematic literature search on PubMed, OVID, and Web of Science from their inception to 22 August 2019. Two independent investigators extracted study-level data for a random-effects meta-analysis. RESULTS: Eight studies were analysed, including 1970 hypertensive emergencies and 4983 hypertensive urgencies. The prevalence of hypertensive emergencies and hypertensive urgencies was 0.3 and 0.9%, respectively [odds ratio for hypertensive urgencies vs. hypertensive emergencies 2.5 (1.4-4.3)]. Pulmonary oedema/heart failure was the most frequent subtype of aHMOD (32%), followed by ischemic stroke (29%), acute coronary syndrome (18%), haemorrhagic stroke (11%), acute aortic syndrome (2%) and hypertensive encephalopathy (2%). No clinically meaningful difference was found for BP levels at presentations. Hypertensive urgency patients were younger than hypertensive emergency patients by 5.4 years and more often complained of nonspecific symptoms and/or headache, whereas specific symptoms were more frequent among hypertensive emergency patients. CONCLUSION: Hypertensive emergencies and hypertensive urgencies are a frequent cause of access to emergency departments, with hypertensive urgencies being significantly more common. BP levels alone do not reliably predict the presence of aHMOD, which should be suspected according to the presenting signs and symptoms.

A Case of Profound Hypothyroidism Presenting with Hypertensive Emergency and Large Amount of Pericardial Effusion.

BACKGROUND Thyroid function is closely related to the cardiovascular system. Pericardial effusion is a well-known complication of hypothyroidism. It is common for massive pericardial effusion to progress to tamponed heart with hypotension, but not high blood pressure. CASE REPORT A 46-year-old woman presented to the hospital with dysarthria and left-side weakness of the upper limb which had started 30 minutes before her arrival at the hospital. The patient showed hypertensive emergency (213/124 mmHg) with intracerebral hemorrhage. Further evaluation for high blood pressure and transthoracic echocardiography demonstrated the presence of a large amount of pericardial effusion, and urgent pericardiocentesis was performed. The laboratory examination showed elevated thyroid-stimulating hormone and decreased free thyroxine level, leading to a diagnosis of primary hypothyroidism. The administration of current medications was maintained, including thyroid hormone replacement and anti-hypertensive drugs. CONCLUSIONS A rare case of profound hypothyroidism presenting with hypertensive crisis and massive pericardial effusion is described in this report.

[Malignant hypertension: A bright future]. / L'hypertension artérielle maligne , un futur prometteur.

Malignant hypertension has not disappeared, it has been forgotten. Its incidence is increasing again. It considerably worsens the prognosis of young patients (35 to 55 years old on average). There might be susceptibility factors, several hypotheses are under study. New diagnostic criteria and therapeutic options have been proposed and will have to be validated. Faced with these important challenges for patients, the first prospective multicentric register on this pathology will be set up in France in September 2019.

Severe and malignant hypertension are common in primary atypical hemolytic uremic syndrome.

Malignant hypertension is listed among the causes of secondary thrombotic microangiopathy, but pathogenic mutations in complement genes have been reported in patients with hypertension-induced thrombotic microangiopathy. 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 thrombotic microangiopathy 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, thrombotic microangiopathy is uncommon among patients presenting with malignant hypertension caused by diseases other than aHUS.

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.

Malignant Hypertension with Thrombotic Microangiopathy.

A 49-year-old man with malignant hypertension, acute kidney injury and mental deterioration was referred to our hospital. We initially observed microangiopathic hemolytic anemia, thrombocytopenia and kidney damage, indicating he had thrombotic microangiopathy (TMA). We considered TMA was caused by malignant hypertension and therefore did not start plasma therapy. The French TMA reference center reported that platelet counts and serum creatine levels have high values for predicting severe ADAMTS13 deficiency. The patient fully recovered from his illness after treatment with antihypertensive drugs and intermittent hemodialysis. This case might thus be useful to understand the proper differential diagnosis and treatment of TMA.

[Cough has Many Faces]. / Husten hat viele Gesichter.

We report about a 35 year old previously apparently healthy male patient with terminal chronic renal failure due to IgA nephropathy. The initial symptoms were similar to those of a banal viral respiratory infection. Massive hypertension, orthopnea, edema and blurred vision finally led to the diagnosis of an end stage renal disease. In the renal biopsy the final stage of IgA nephropathy was detected with mostly deserted glomeruli. Hemodialysis was necessary. Among it the patient is well according to the circumstances. He is waiting for a renal transplant.

[HYPERTENSIVE CRISIS: MODERN VIEW OF THE PROBLEM AND OPTIMIZATION OF DIAGNOSTIC AND THERAPEUTIC MODALITIES].

The data collected by Burdenko Military Hospital indicate that in the 1980s hypertensive crisis (HC) occurred in roughly 30% of the patients with AH. This value fell down to 16% by 2012, with a rise in the number of uncomplicated crises from 46 to 62%. Analysis of the causes behind these changes showed that half of the patients simply experienced an elevated arterial pressure with minimal clinical symptoms. The decrease in the number of complicated cases from 54 to 39% is doubtful bearing in mind that ICD-10 gives the status of nosological entities to complications of hypertensive crisis (stroke, myocardial infarction, etc.) but not to the HC syndrome proper requiring urgent hospitalization; due to this hypertensive crisis itself tends to be disregarded and not included in statistics. HC with acute clinically significant lesions of target organs requires intensive care or resuscitation using infusion of vasodilators and loop diuretics to stabilize arterial pressure. In case of uncomplicted HC and aggravation of hypertensive disease, the medications of choice are oral short-acting ACE inhibitors and imidazoline receptor agonists.

[Hypertensive emergencies and urgencies]. / Vraies et fausses urgences hypertensives.

Hypertension is a common disease, the most common chronic disease. Hypertensive emergency is much less frequent and only affects 1 to 2 % of all hypertensive patients. The true hypertensive emergency is characterized by the serious damage of one hypertensive target organ and requires an urgent intravenous treatment. Isolated blood pressure elevation should not be regarded as a hypertensive emergency if there is no target organ damage, even if the blood pressure is very high. These situations of "false hypertensive emergency", or hypertensive urgencies, often requires an immediate treatment, but oral. Signs of visceral pain of true hypertensive emergency often are a poor general condition, severe headache, decreased visual acuity, neurological deficit of ischemic or hemorrhagic cause, confusion, dyspnea with orthopnoea revealing heart failure, angina, chest pain revealing an aortic dissection, proteinuria, acute renal failure or eclampsia. True hypertensive emergencies include several entities, namely: severe hypertension, malignant hypertension and accelerated hypertension. If malignant hypertension is not treated, the prognosis is poor with 50 % death risk in the following year.

[Hypertensive urgency and emergency]. / Hypertensive Krise.

European and North-American guidelines for the diagnosis and therapy of arterial hypertension refer to hypertensive crisis as an acute and critical increase of blood pressure>180/120 mmHg. Presence of acute hypertensive target organ damage, such as stroke, myocardial infarction or heart failure, in this situation defines a "hypertensive emergency". In these patients, immediate lowering of blood pressure (about 25% within one to two hours) in an intensive care setting is mandatory to prevent further progression of target organ damage. In contrast to hypertensive emergencies, hypertensive urgencies are characterized by an acute and critical increase in blood pressure without signs or symptoms of acute hypertensive target organ damage. In these patients, blood pressure should be lowered within 24 to 48 hours in order to avoid hypertensive target organ damage. In general, hospitalization is not required, and oral antihypertensive therapy usually is sufficient. However, further and continuing outpatient care has to be ensured.

Scleroderma renal crisis.

OBJECTIVES: To discuss the pathophysiology, risk factors, clinical manifestations, diagnosis, treatment, prevention, and outcomes of scleroderma renal crisis (SRC), a serious yet potentially treatable complication of scleroderma (systemic sclerosis). METHODS: A PubMed search for articles published up until April 2014 was conducted using the following keywords: scleroderma, systemic sclerosis, scleroderma renal crisis, renal, treatment, and prognosis. Literature was carefully reviewed, and different risk factors, treatment options, prognostic factors, and survival data were assessed. RESULTS: SRC occurs in about 10% of all patients with scleroderma. It is characterized by malignant hypertension and progressive renal failure. Around 10% of SRC cases may present with normal blood pressure, termed normotensive renal crisis. The etiopathogenesis is presumed to be a series of insults to the kidneys resulting in endothelial injury, intimal proliferation, and narrowing of renal arterioles leading to decreased blood flow, hyperplasia of the juxtaglomerular apparatus, hyperreninemia, and accelerated hypertension. Risk factors include rapid skin thickening, use of certain medications such corticosteroids or cyclosporine, new-onset microangiopathic hemolytic anemia and/or thrombocytopenia, cardiac complications (pericardial effusion, congestive heart failure, and/or arrhythmias), large joint contractures, and presence of anti-RNA polymerase III antibody. Since the 1970s, with the advent of angiotensin-converting enzyme (ACE) inhibitors, mortality associated with SRC decreased from 76% to <10%. Some patients may progress to end-stage renal disease and need dialysis. Renal transplantation has improved survival, though SRC may recur in transplanted kidneys. CONCLUSIONS: More than 60 years after its initial description, SRC still remains an important cause of morbidity and mortality in scleroderma. Since the advent of ACE inhibitors, the prognosis of SRC has improved substantially. Prompt diagnosis and treatment may help prevent adverse outcomes and improve survival.

[Wasp-sting-induced pheochromozytoma crisis with stress-related cardiomyopathy (Takotsubo)]. / Wespenstichinduzierte sympathikotone Krise mit Stresskardiomyopathie (Takotsubo) bei Phäochromozytom.

This article presents the case of a patient with sudden onset of heart failure caused by transient severe left ventricular dysfunction with the typical pattern of stress-induced cardiomyopathy (takotsubo cardiomyopathy) who had wasp sting a few hours before admission in the presence of a previously asymptomatic pheochromocytoma. There seems to be correlation between the wasp-venom-induced pheochomocytoma crisis and acute onset of heart failure. Once pheocromocytoma is diagnosed, medical therapy is preferable before surgical treatment. This case demonstrates that a previously asymptomatic pheochromocytoma can become clinically relevant by catecholamine-releasing wasp venom causing stress-related cardiomyopathy and that patient history is mandatory for evaluating the cause of sudden clinical outcome.

Renal fibromuscular dysplasia with malignant hypertension cured by balloon angioplasty with stenting.

We presented a 31-year-old female patient with a history of hypertension and severe pulsing headache for about 3 months. The patient had pulsing headaches over the past 3 months with increased very high blood pressure (BP = 220/130 mmHg), sometimes with blurred vision, nausea and vomiting, with no known pathological conditions in her medical history or family background. A digital subtraction angiography confirmed tight stenosis (90%) in the middle segment of the right renal artery. Balloon angioplasty with a stent was the treatment of choice. Blood pressure dropped to normal after treatment.