1/70. Solitary renal myofibromatosis: an unusual cause of infantile hypertension.INTRODUCTION: Renovascular disease accounts for the vast majority of cases of infantile hypertension with complications resulting from umbilical arterial catheterization predominating in the neonatal period and fibrodysplastic lesions of the renal artery predominating outside the neonatal period. We report a previously undescribed cause of renovascular hypertension: solitary renal myofibromatosis. CASE REPORT: A 9-month-old male infant was transported to the intensive care unit at Children's Hospital in Denver, colorado, for evaluation and treatment of a dilated cardiomyopathy and severe systemic hypertension. The child was full-term with no perinatal problems. Specifically, the child never required umbilical arterial catheterization. He was well until 6 months of age when his parents noted poor weight gain. At 9 months of age, he was evaluated at the referral hospital for failure to thrive. On examination he was noted to have a blood pressure of 170/110 mm Hg, but no other abnormalities. A chest radiograph showed cardiomegaly. Laboratory studies demonstrated normal electrolytes, blood urea nitrogen, and creatinine. However, urinalysis demonstrated 4 protein without red blood cells. An echocardiogram showed severe left ventricular dilatation with an ejection fraction of 16%. On admission the child was noted to be cachectic. His vital signs, including blood pressure, were normal for age. The physical examination was unremarkable. serum electrolytes, blood urea nitrogen, and creatinine were normal. Echocardiographic studies suggested a dilated hypertrophic cardiomyopathy. He was started on digoxin and captopril. Subsequently, he demonstrated episodic hypertension ranging from 170/90 to 220/130 mm Hg. A repeat echocardiogram 24 hours after admission demonstrated a purely hypertrophic cardiomyopathy. verapamil and nifedipine were added to the treatment regimen in an effort to better control the blood pressure without success. urine and blood for catecholamines and plasma renin activity, respectively, were sent and treatment with phentolamine instituted because of a possible pheochromocytoma. A spiral abdominal computerized tomographic scan revealed a markedly abnormal right kidney with linear streaky areas of calcification around the hilum and also an area of nonenhancement in the posterior upper pole. The adrenals and the left kidney were normal. Doppler ultrasound revealed a decrease in right renal arterial flow. The urinary catecholamines were normal and surgery was scheduled after the blood pressure was brought under control by medical treatment. At surgery, tumorous tissue and thrombosis of the renal artery were found in the right upper pole. A right nephrectomy was performed. Pathologic examination of the kidney showed the presence of a diffuse spindle cell proliferation in the interstitium of the kidney. The angiogenic/angiocentric character of the proliferation was demonstrated in several large renal vessels. The lumen of most vessels was narrowed and some vessels were totally occluded with recanalization and dystrophic calcifications observed. Immunostaining of the tumor demonstrated strong desmin and vimentin positivity and minimal actin positivity in the spindle cells. Mitotic activity was not noted in the spindle cell process. These pathologic changes were consistent with a diagnosis of infantile myofibromatosis (IM). The child's preoperative plasma renin activity was 50 712 ng/dL/h (reference range, 235-3700 ng/dL/h). DISCUSSION: The causes of systemic hypertension in infancy are many although renal causes are by far the most common. Renal arterial stenosis or thrombosis accounts for 10% to 24% of cases of infantile hypertension. renal artery thrombosis is usually a consequence of umbilical arterial catheterization, which can also lead to embolization of the renal artery. renal artery stenosis may result from fibrodysplastic lesions (74%), abdominal aortitis (9%), a complication of renal transplantation (5%), and ren- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
2/70. Leukocytoclastic vasculitis in a child with epidermolysis bullosa simplex.A 10-year-old boy with epidermolysis bullosa simplex (Weber-Cockayne variant) together with leukocytoclastic vasculitis is presented. He was admitted to the hospital with the provisional diagnoses of infected epidermolysis bullosa simplex or drug eruption. On the sixth day of hospitalization he developed palpable purpura, abdominal pain and bloody diarrhea, together with hematuria and proteinuria. A generalized tonic-clonic convulsion, changes in mental status, fluctuations in arterial blood pressure and intractable pain in his extremities occurred during the course of hospitalization. Systemic pulse steroid therapy, antibiotics, and antihypertensive and anticonvulsive drugs were given. On the 30th day of hospitalization, a skin graft was performed to replace a large tissue defect on his left hand. Despite high dose steroid therapy, his hematuria, proteinuria and hypertension continued after his discharge, suggesting a steroid-resistant renal pathology, such as focal glomerulosclerosis, that occurred secondary to leukocytoclastic vasculitis.- - - - - - - - - - ranking = 0.25keywords = pressure (Clic here for more details about this article) |
3/70. smoking--a renal risk factor.One of the most important tasks of clinical and experimental nephrology is to identify the risk factors of progression of renal failure. A major renal risk factor which has not been sufficiently acknowledged despite increasing evidence is cigarette smoking. Diabetologists were the first to recognize the adverse effects of smoking on the kidney: both in type 1 and in type 2 diabetes smoking (i) increases the risk of development of nephropathy and (ii) nearly doubles the rate of progression to end-stage renal failure. Until recently it was not known whether smoking also increases the risk to progress to end-stage renal failure in patients with primary renal disease. A retrospective multicenter European case-control study showed that smoking is an independent risk factor for end-stage renal failure in patients with inflammatory and noninflammatory renal disease, i.e. IgA glomerulonephritis and polycystic kidney disease. The pathogenesis of the smoking-related renal damage is largely unknown. The intermittent increase in blood pressure during smoking seems to play a major role in causing renal damage, but further potential pathomechanisms are presumably also operative. smoking as a renal risk factor is of great interest to diabetologists as well as nephrologists, but unfortunately so far this information has had little impact on patient management. The present article reviews the current knowledge about the renal risks of smoking and discusses the potential mechanisms of smoking-mediated renal injury.- - - - - - - - - - ranking = 0.25keywords = pressure (Clic here for more details about this article) |
4/70. hypertension, renal vein thrombosis and renal failure (occurring in a patient on an oral contraceptive agent).A case of accelerated hypertension leading to renal failure in a young woman taking an oral contraceptive agent is described. During the course of her disease the left kidney was documented to decrease in size. Renal vein plasma renin activity was found to be elevated on the left in the absence of renal artery stenosis. Left nephrectomy, prompted by continuing poor blood pressure control, resulted in amelioration of the hypertension. Left renal vein thrombosis was found at surgery. It is suggested that renal vein thrombosis was a contributing factor to this patient's accelerated hypertension and may represent an unusual thrombotic complication of oral contraceptives.- - - - - - - - - - ranking = 0.25keywords = pressure (Clic here for more details about this article) |
5/70. Remission achieved in chronic nephropathy by a multidrug approach targeted at urinary protein excretion.Regardless of the pattern of renal involvement, increased urinary protein excretion rate is the best independent predictor of progression of chronic nephropathies and short-term reduction in proteinuria has been reported to be renoprotective in the long term. Despite such evidence, however, the therapeutic target in renoprotection is almost exclusively on blood pressure control. We report the clinical course of a patient with chronic nephropathy after the institution of a multidrug treatment titrated against urinary protein excretion to achieve renoprotection. The present findings indicate that adjusting renoprotective therapy according to the decline in protein excretion in a multidrug strategy may stabilize or even reverse renal disease progression. This approach should be formally explored in prospective studies.- - - - - - - - - - ranking = 0.25keywords = pressure (Clic here for more details about this article) |
6/70. Electrocardiographic QT prolongation and sudden death in renal hypokalemic alkalosis.A 3-year-old boy was found to have a mixed tubulopathy with hypokalemia (1.9 mmol/l), alkalosis (blood pH 7.51, plasma carbon dioxide pressure 46 mm Hg, plasma bicarbonate 35.7 mmol/l) and hypophosphatemia (1.21 mmol/l). An electrocardiogram disclosed a prolonged heart rate corrected QT interval of 490 ms. The boy was put on potassium chloride, phosphate and nonsteroidal anti-inflammatory agents. With this treatment plasma phosphate normalized and plasma potassium increased up to 3.0-3.3 mmol/l. Three years later the child, who did not have history of gastroesophageal reflux or epileptic convulsions, suddenly died during sleep. The cause of death could not be determined through gross examination of the body. The history of hypokalemia, the QT-prolongation, the sudden death and the failure to assess the cause of death through gross examination of the body suggest that death was caused by an arrhythmia secondary to hypokalemia.- - - - - - - - - - ranking = 0.25keywords = pressure (Clic here for more details about this article) |
7/70. liddle syndrome in a newborn infant.A 10-week-old female infant developed hypertension. The elevated blood pressure was associated with metabolic alkalosis and urinary chloride wastage. The family history was unremarkable. Her urinalysis, blood urea nitrogen (BUN), and serum creatinine concentrations were all normal. A renal ultrasound was normal. A technetium-99m diethylenetriaminopentoacetic acid (DTPA) renal scan with captopril showed normal blood flow bilaterally. The head ultrasound and echocardiogram were normal. Blood epinephrine, norepinephrine, catecholamines, thyroxine, and steroid levels were also normal. Treatment with various combinations of labetalol, hydralazine, captopril, methyldopa, nifedipine, and spironolactone, all at high doses, failed to control the elevated blood pressure. serum aldosterone level and peripheral plasma renin activity were low. The lack of therapeutic response to spironolactone, with a good response to amiloride and recurrence of hypertension and metabolic alkalosis after amiloride cessation that was subsequently treated with amiloride, established the diagnosis of liddle syndrome. To our knowledge, this is the youngest patient with liddle syndrome that has been reported in the literature.- - - - - - - - - - ranking = 0.5keywords = pressure (Clic here for more details about this article) |
8/70. mitomycin C-induced renal insufficiency: a case report.A 58-year-old normotensive male with normal renal function and gastric cancer underwent total gastrectomy and received adjuvant chemotherapy with mitomycin C (MMC) for 10 months. He developed anemia, hypertension, and renal function impairment 9 months after initiation of chemotherapy. kidney biopsy showed thrombotic microangiopathy with marked mesangiolysis and expansion of the subendothelial space resulting in cystic dilation of the glomerular capillaries, and cellular atypia in the tubular cells. His renal function deteriorated gradually then stabilized after treatment with plasma exchange, antihypertensive agents, and antiplatelet agents. He had no sign of tumor recurrence after 3 years of follow-up. We suggest that patients receiving MMC should have their blood pressure and renal function closely monitored for the possibility of development of drug-induced renal insufficiency.- - - - - - - - - - ranking = 0.25keywords = pressure (Clic here for more details about this article) |
9/70. Regression of hypertensive nephropathy during three years of optimal blood pressure control.Hypertensive nephropathy is among the leading causes of end-stage renal disease. Once renal function is severely impaired, the effects of strict control of blood pressure on the recovery of renal function remain elusive. Published case series suggest that optimal control of blood pressure results in regression of renal failure to some extent. In the present case of biopsy-proven hypertensive nephropathy we show that renal function can substantially improve over time if blood pressure is optimally controlled. Glomerula filtration rate continuously improved in our patient from 20 ml/min at presentation to 80 ml/min over a period of three years using a fivefold antihypertensive regimen. hypertensive retinopathy regressed from stage III to stage I, and left ventricular hypertrophy decreased from an initial septum thickness of 19 mm to 12 mm within that period of time. This case clearly illustrates that optimal control of blood pressure is mandatory in patients with pre-terminal renal failure due to hypertensive nephropathy. Such intervention can lead to a regression of hypertension-associated end-organ injury.- - - - - - - - - - ranking = 2keywords = pressure (Clic here for more details about this article) |
10/70. Inverse relationship between blood volume and blood pressure.The outcome objectives for EG were met. In order for her blood pressure to improve, cardiac output had to improve. In her case, this could only be accomplished by reducing the vascular volume through aggressive ultrafiltration. Starling's Law and Laplace's Theorem provide the key to understanding this concept, namely that an increase or decrease in cardiac output will result in a corresponding increase or increase or decrease in mean arterial pressure (Smith & Kampine, 1990, p. 93). Thus, there is an inverse relationship between blood volume and blood pressure. In this case ultrafiltration treated the cause of hypotension. Discontinuing the ultrafiltration and/or giving a saline bolus would have been an incorrect attempt to treat the effects of hypotension.- - - - - - - - - - ranking = 1.75keywords = pressure (Clic here for more details about this article) |
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