[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.

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.

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.

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.

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.

Malignant hypertension and uremia in scleroderma: efficacy of nephrectomy and hemodialysis.

Two cases of scleroderma are presented in which malignant hypertension developed abruptly, accompanied by rapidly progressive renal failure. The malignant hypertension was associated with high plasma renin levels and like other forms of hyperrenninemic hypertension and uremia, was refractory to both antihypertensive medication and extracellular fluid volume control with hemodialysis. Blood pressures became controllable only after bilateral nephrectomy was performed, and in each case resulted in a reversal of a rapidly progressive downhill course. Though both patients had multisystem involvement at the onset of renal failure, the non-renal manifestations of scleroderma have not progressed rapidly. Consequently, when patients with scleroderma develop malignant hypertension and uremia, aggressive therapy with dialysis and nephrectomy may significantly prolong survival.

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.

Malignant hypertension revisited: the role of bilateral nephrectomy.

Hypertension is the leading cause of renal failure in this dialysis and transplant center. When malignant hypertension is encountered, the symptom complex of cachexia and failure to thrive highlights its clinical presentation. The courses of 32 black renal hypertensive patients studied retrospectively demonstrated this symptom complex as well as prolongation of survival, when bilateral nephrectomy and renal transplantation were used as definitive treatment.

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.

[Aspects of hypertension of endocrine and adrenal origin]. / Nekotorye aspekty gipertenzii éndokrinno-nadpochechnikovogo geneza.

The authors discuss the results of observation of 269 patients with adrenal arterial hypertension 247 of whom underwent operation. The malignant hypertension syndrome developed in 39% of the patients. It is presumed that its development was caused by the combination of adrenal and renal affections as well as by specific hormonal shifts: relative norepinephrine hyperproduction and reduced intensity of catecholamine metabolism. In all groups of the patients with the malignant hypertension syndrome, a higher daily excretion of aldosterone and increased blood renin activity were also found. The character of the drug therapy and the late results of surgical treatment are discussed. A new variant of hyperaldosteronism, dexamethasone-inhibited form of aldosteroma, is described.

[Hypotensive effect of nephrectomy in malignant renal hypertension and the function of the remaining kidney at late follow-up]. / Gipotenzivnyi éffekt nefréktomii pri zlokachestvennoi pochechnoi gipertonii i funktsional'noe sostoianie ostavsheisia pochki v otdalennye sroki nabliudeniia.

The long-term results of nephrectomy were evaluated in 27 patients with malignant renal hypertension. The hypotensive effect of nephrectomy, function of the remaining kidney, central hemodynamics, and peripheral blood renin were investigated. It was found that hypertension remitted in subjects with an unaffected remaining kidney which completely compensated for the function of the removed kidney and maintained blood pressure within normal. Nephrectomy performed during malignant arterial hypertension that developed because of unilateral pyelonephritis and unilateral stenosing of the renal artery with a sufficient total renal function before surgery led to a prolonged remission of arterial hypertension and reverse development of the symptoms of its malignancy. Hypertension did not remit during chronic pyelonephritis of a single kidney. Renal function was substantially decreased as compared with the control group.

[Retinal function after malignant hypertension in patients with transplanted kidneys]. / Czynnosc siatkówki po przebytym nadcisnieniu zlosliwym u chorych z przeszczepionymi nerkami.

Ophthalmological examinations were carried out in 42 patients (aged 16-45 years) after kidney transplantation in whom malignant hypertension was discovered before transplantation. The first funduscopy revealed pathological changes of various type and grade (I-IV), in 17 patients the visual acuity was lowered and in 18 the ERG record was pathological (residual or extinguished). The control examinations performed in 19 patients after 1-5 years showed that the changes typical for malignant hypertension receded in 7; the fundus of the rest of the patients was the same as before. The lack of improvement in the ERG record points to a permanent lesion of the retinal activity after passed malignant hypertension in spite of normalization of the arterial pressure.

[Diversion of the venous blood from the adrenal gland and the kidney into the portal system in the treatment of malignant arterial hypertension]. / Otvedenie venoznoi krovi ot nadpochechnika i pochki v vorotnuiu sistemu pri lechenii zlokachestvennoi arterial'noi gipertensii.

A method of the portalization of the adrenal and renal blood has been devised in the experiment on 47 cadavers. The method consists in the formation of the left renoportal venous anastomosis combined with contralateral adrenalectomy. The method was successfully applied in the clinic upon 9 patients with severe and malignant arterial hypertension of various etiology. The technique of the operation is described. A detailed description of one observation of a successful use of the method, applied in malignant clinical course of arterial hypertension, illustrates the effectiveness of this operation. The indications for this operation are set forth.

[Non-extirpation methods of suppressing adrenal gland hyperfunction in severe course of arterial hypertension]. / Neekstirpatsionnye metody podavleniia giperfunktsii nadpochechnikov pri tiazhelom techenii arterial'noi gipertenzii.

The authors report on using cryodestruction of the adrenals in 30 patients. Four patients died during the postoperative period. The operation of cryodestruction of the adrenals is considered by the authors to be technically simpler than resection of the adrenals.

[Emergency adrenalectomy in pheochromocytoma]. / Neotlozhnaia suprarenéktomiia pri feokhromotsitome.

Data concerned with two patients with pheochromocytoma are presented which convince the authors that urgent operation (a removal of the tumor) is expedient in uncontrolled catecholamine crisis.

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.

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.