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.

An ectopic renin-secreting adrenal corticoadenoma in a child with malignant hypertension.

A previously healthy 7-year-old male presented with hypertensive emergency, hypokalemia, and elevated plasma renin activity and aldosterone levels. There was no evidence of virilization or cushingoid features. MRI of the abdomen revealed a large (5 × 5 × 3 cm) peripherally enhancing, heterogeneous mass arising from the left adrenal gland. The patient was treated for a suspected pheochromocytoma. However, his blood pressure was not responsive to alpha-blockade. Blood pressure was controlled with a calcium channel blocker and an angiotensin-converting enzyme (ACE) inhibitor. A complete surgical resection of the mass was performed. Postoperatively, his blood pressure normalized and he did not require antihypertensives. On pathological examination, the tumor tissue stained negative for chromogranin and positive for renin. The final diagnosis was renin-secreting adrenal corticoadenoma, an extremely rare adrenal tumor not previously reported in a pediatric patient. Malignant hypertension due to a renin-secreting tumor may need to be distinguished from a pheochromocytoma if alpha-adrenergic blockade is ineffective.

[Surgical treatment of a patient with occlusion of the main trunk of the renal artery of the solitary kidney].

Presented herein is a clinical case report of successful stagewise surgical treatment of a patient with atherosclerotic occlusion of the main trunk of the renal artery of the single kidney. Clinically, the patient had signs of ischaemic renal disease in the form of pronounced azotemia being characteristic of the terminal stage of renal insufficiency, as well as malignant arterial hypertension. Besides, the patient had previously endured ischaemic stroke in the vertebrobaslar basin. The patient was subjected to stagewise surgical intervention, i.e., stenting of the upper-pole renal artery followed by open operation--prosthetic repair of the left renal artery with a synthetic prosthesis. Three months thereafter, the patient underwent carotid endarterectomy and operation of transposition of the subclavian artery. The postoperative period turned out uneventful. Currently, no progression of azotemia is observed, neither are there any indications for carrying out restorative therapy of the renal function.

Use of a pulsatile perfusion pump for renal autotransplantation in a patient undergoing thoracoabdominal bypass for malignant hypertension secondary to Takayasu arteritis.

Aortorenal bypass is an effective and durable therapy for autoimmune-induced renovascular hypertension. However, when technical and patient factors preclude this option, renal autotransplantation can be a viable alternative. We present a 32-year-old woman who underwent aortobi-iliac bypass with left renal autotransplantation for malignant hypertension secondary to Takayasu arteritis. This is the first description of using machine preservation with a continuous pulsatile perfusion pump to maintain renal preservation before reimplantation. Our method proved safe to the patient and allowed for protection of the organ from prolonged warm ischemia and intraoperative hypoperfusion during a complex reconstruction.

Liver transplantation results in complete neurologic recovery from malignant hypertension secondary to fulminant hepatic failure: a case report.

BACKGROUND: Uncontrolled intracranial hypertension can lead to cerebral herniation and death in patients with acute liver failure. CASE REPORT: A 26-year-old female was admitted for acute liver failure following inadvertent acetaminophen overdose. The pH on admission was 6.9. Her neurologic status precipitously deteriorated and she was listed for liver transplantation. An intracranial pressure (ICP) monitoring catheter was inserted, which revealed a pressure >60 mmHg. After neurointensive care treatment, ICP was lowered and an emergency left lobe living donor liver transplant was performed. Intraoperative management of the ICP, which rose to 80 mmHg during the explant phase, was achieved by therapy with barbiturates and hypothermia. After surgery, hepatic function improved initially, but 7 days post transplantation the graft showed signs of acute failure. The pathology report of a liver biopsy suggested acute rejection and liver retransplantation using a deceased donor liver was then carried out. The postoperative course was uneventful and the patient recovered completely without any residual neurologic deficits. CONCLUSIONS: This case states that favourable outcomes can result from sub-optimal starting points, and that the human brain has the ability to overcome extremely adverse conditions. Critical in this effort is the role of proper neuromonitoring which helps implement the appropriate treatment measures.

Cranial decompression for the treatment of malignant intracranial hypertension after ischemic cerebral infarction: decompressive craniectomy and hinge craniotomy.

OBJECT: Recent randomized trials have demonstrated a positive role (improved survival) in patients treated with cranial decompression for malignant cerebral infarction. However, many variables regarding operative decompression in this setting remain to be determined. Hinge craniotomy is an alternative to decompressive craniectomy, but its role in space-occupying cerebral infarctions has not been delineated. The objective of this study was to compare the authors' experiences with these 2 procedures in the management of space-occupying cerebral infarctions to determine the efficacy of each. METHODS: The authors conducted a retrospective review of 28 cases involving patients who underwent cranial decompression (hinge craniotomy in 9 cases, decompressive craniectomy in 19) for treatment of malignant intracranial hypertension after ischemic cerebral infarction. RESULTS: No significant differences were identified in baseline demographics, neurological examination, or Rotterdam score between the hinge craniotomy and decompressive craniectomy groups. Both treatments resulted in adequate control of intracranial pressure (ICP). The need for reoperation for persistent intracranial hypertension and duration of mechanical ventilation and intensive care unit stay were similar. Hospital survival was significantly higher in the decompressive craniectomy group (89% vs 56%), whereas long-term functional outcome was better in the hinge craniotomy group. Cranial defect size was comparable in the 2 groups. Postoperative imaging revealed a higher rate of subarachnoid hemorrhage, contusion/hematoma progression, and subdural effusions/hygromas after decompressive craniectomy. The requirement for cranial revision in survivors was higher for patients undergoing decompressive craniectomy (100%) than those undergoing hinge craniotomy (20%). CONCLUSIONS: Hinge craniotomy appears to be at least as good as decompressive craniectomy in providing postoperative ICP control at a similar therapeutic index. Although the in-hospital mortality was higher in patients treated with hinge craniotomy, that procedure resulted in superior long-term functional outcomes and may help limit postoperative complications.

Immunosuppression and sebaceous tumors: a confirmed diagnosis of Muir-Torre syndrome unmasked by immunosuppressive therapy.

Muir-Torre syndrome (MTS) is an autosomal dominant genodermatosis caused by mutations in the DNA mismatch repair genes MLH1 and MSH2. This case describes a patient with an extensive family history of colon cancer who experienced the onset of multiple sebaceous adenomas and carcinomas after undergoing kidney transplantation and receiving immunosuppressive therapy. The finding of deficient MSH2 expression in the immunohistochemical analysis of a sebaceous carcinoma prompted genetic testing for a systemic mutation in the mismatch repair gene. A systemic mutation of the MSH2 gene was detected and, despite the absence of a visceral malignancy, the diagnosis of MTS was made. Immunosuppression has previously been thought to play a possible role in unmasking a latent MTS phenotype in transplant recipients, but systemic mutations have not previously been analyzed. The relationship between immunosuppression and sebaceous tumors with the possibility of unmasking a MTS phenotype in transplant recipients is discussed.

Renal manifestations in toddlers with Takayasu's arteritis and malignant hypertension.

Three children under the age of 3 years presented with malignant hypertension, proteinuria, and acute kidney injury. Takayasu's arteritis was diagnosed on the basis of clinical symptoms of weight loss and low grade fever in conjunction with elevated sedimentation rate and radiographic evidence of aortic and renal artery stenosis. One patient had a renal biopsy which showed arteriolar sclerosis and focal glomerulosclerosis. All three patients required multiple antihypertensive agents, ultimately including angiotensin receptor blockers and/or angiotensin converting enzyme inhibitors. The vasculitis was treated with pulse corticosteroids followed by cyclophosphamide in one patient and mycophenolate mofetil as maintenance therapy in all. Follow-up has ranged from 2 to 8 years. Although global renal function has normalized in each patient, two have unilateral non-function of one kidney. The last patient has persistent aortic and renal artery stenosis with complex collateralization requiring ongoing medical and anticipated surgical management.

[Refractory arterial hypertension]. / Hipertensiunea arteriala rezistenta la tratament.

The present review synthesizes "the state of the art" concerning resistant (refractory) arterial hypertension (RAH). Definition, epidemiology, frequent risk factors, clinical conditions and causes of therapeutic resistance of RAH are revisited. Pathogenic hypothesis and related therapeutic approaches are explored. The paper lists current and new pharmacological therapies of RAH and introduces the latest interventional approach (the Rheos BHT system) to this form of arterial hypertension.

Malignant hypertension with intestinal ischemia secondary to juxtaglomerular cell tumor.

Malignant hypertension is a well-defined condition associated with high blood pressure and acute target-organ damage. Although 95% of cases are secondary to essential hypertension, its etiological profile is broad. Juxtaglomerular cell tumor is a rare condition, with only approximately 65 cases reported to date. We describe a patient with malignant hypertension with acute renal failure and intestinal ischemia secondary to a juxtaglomerular cell tumor. We believe this is the first case of juxtaglomerular cell tumor causing malignant hypertension. The diagnostic approach and treatment are discussed.

Hyponatremic hypertensive syndrome (HHS) in an 18-month old-child presenting as malignant hypertension: a case report.

BACKGROUND: The combination of hyponatremia and renovascular hypertension is called hyponatremic hypertensive syndrome (HHS). Malignant hypertension as a presentation has been reported in adults with HHS but is rare in children. CASE PRESENTATION: An eighteen month-old male presented with drowsiness, sudden onset status epilepticus and blood pressure of 210/160. The electrolytes on admission revealed sodium of 120 mEq/L and potassium of 2.1 mEq/L. The peripheral renin activity (PRA) was 172 ng/ml/min (normal 3-11 ng/ml/min) and serum aldosterone level was 91 ng/dl (normal 4 to 16 ng/dl). Patient underwent angioplasty with no success, followed by surgical correction. Two years since the diagnosis, the blood pressure is controlled with labetolol and amlodipine (at less than sixth of the pre-operative dosages). The PRA is 2.4 ng/ml/min and aldosterone 15.5 ng/dl. The child not only had three renal arteries on left but all of them were stenosed which to best of our knowledge has not been described. CONCLUSION: As uncommon as HHS with malignant hypertension may be in adults it is under-reported in children and purpose of the case report is to raise its awareness.

Nephrectomy in severe hypertension.

We describe a 12-year-old girl who died due to hypertensive encephalopathy and a 7-year-old boy with a favorable outcome after bilateral nephrectomy. Both had end-stage renal disease. Nephrectomy should be considered as a possible treatment of severe hypertension even without end-stage renal disease, if the patient has symptoms of hypertensive encephalopathy.

The heterogeneous and delayed course of blood pressure normalization in hypertensive patients after bilateral nephrectomy with and without subsequent renal transplantation.

BACKGROUND: Controversy exists about the time course of blood pressure normalization following bilateral nephrectomy. We sought to evaluate the time course of blood pressure normalization following bilateral nephrectomy and after subsequent kidney transplantation. METHODS AND RESULTS: Clinical data from 14 hypertensive patients were retrospectively assessed. Baseline blood pressure was 175 +/- 33/109 +/- 9 mmHg. Ten patients firstly underwent unilateral nephrectomy, which resulted in a slight increase of blood pressure (185 +/- 22/110 +/- 5 mmHg). One month following bilateral nephrectomy, blood pressure was 167 +/- 23/104 +/- 17 mmHg, at 3 months 159 +/- 42/104 +/- 25 mmHg, and at 6 months 149 +/- 41/96 +/- 30 mmHg. Antihypertensive medication was necessary in 9/14 patients at a 2 year follow-up. Eight patients remained anephric (group I), 6 patients had subsequent kidney transplantation (group II). In group I, blood pressure was 159 +/- 42/93 +/- 17 mmHg and 129 +/- 34/75 +/- 14 mmHg at 3 and 6 months, respectively (p< 0.05 vs. baseline). In group II, blood pressure decreased from 188 +/- 42/ 128 +/- 46 mmHg to 167 +/- 48/113 +/- 32 mmHg at 3 months, but increased after transplantation to 186 +/- 39/118 +/- 33 mmHg. Antihypertensive medication was still necessary in 5 transplanted patients (83%) and in 3 anephric patients (38%). CONCLUSION: Adaptation of the blood pressure response following bilateral nephrectomy is a time requiring process, and long-term antihypertensive medication may still be necessary.

Extraadrenal retroperitoneal paraganglioma causing malignant hypertension and permanent visual loss in an eleven-year-old girl.

We report a secretory paraganglioma in an eleven-year-old Trinidadian girl of East Indian descent who presented with malignant hypertension, proteinuria and modest elevation of urinary vanillylmandelic acid levels. The extra-adrenal mass was surgically removed without complications. The patient has been normotensive without evidence of recurrence of disease but with persistent visual sequel two years later. This case reinforces the observation that extraadrenal retroperitoneal paragangliomas can be functionally very active and early computed tomography or other imaging procedure is necessary for rapid noninvasive evaluation, timely surgery and prevention of permanent morbidity.