1/162. Renal granulomatous sarcoidosis in childhood: a report of 11 cases and a review of the literature.We analysed retrospectively 11 children with renal granulomatous sarcoidosis confirmed by renal histology in order to describe the course and prognosis of the disease. Symptomatic sarcoidosis was diagnosed at a mean age of 10.1 years. Nine children had renal involvement at the time of diagnosis. In the course of the disease, nine patients developed renal failure and mild proteinuria, seven had transient sterile leukocyturia, four showed microscopic haematuria, seven had a urinary concentrating defect, and enlarged kidneys were seen in three patients. One child had hypercalcaemia and hypercalciuria, none had hypertension. light microscopy of the kidney showed interstitial infiltration by mononuclear cells in all children, interstitial fibrosis in nine patients, epithelioid granulomas in seven, tubular involvement in eight, and mild glomerular involvement in seven patients. Renal immunofluorescence was negative. Ten children received prednisone for 1-11 years. After a mean follow up of 5.5 years, three patients had entered end-stage renal failure and one had chronic insufficiency after interruption of medical supervision and prednisone therapy. CONCLUSION: Renal failure, proteinuria, leukocyturia, haematuria, and concentration defect are the prominent features of renal granulomatous sarcoidosis in children. Steroid therapy, adjusted according to disease activity, may prevent end-stage renal failure.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
2/162. 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 = 11.379386279075keywords = renovascular, hypertension (Clic here for more details about this article) |
3/162. Negative captopril renography on patients with renin mediated hypertension due to page kidney and reninoma.Through a mechanism similar to renal artery stenosis, patients with reninoma and page kidney also suffered from renin mediated hypertension. captopril renograms performed on our patients with the latter two conditions, however, did not yield diagnostic findings. Therefore, equivocal or negative captopril renography cannot serve to rule out conditions with elevated renin other than renal artery stenosis.- - - - - - - - - - ranking = 5keywords = hypertension (Clic here for more details about this article) |
4/162. Hereditary renal adysplasia in a three generations family.Renal agenesia is one of the more common urinary malformations. Renal agenesia can be unilateral, more frequently, or bilateral. This malformation can be isolated or present with other urinary and/or extra urinary anomalies. We report a family with renal agenesia. The proband was a fetus. Ultrasonographic examination at 15 weeks of gestation showed a left renal agenesia and a right multicystic kidney, absence of bladder and oligohydramnios. The same features were found at 19 weeks of gestation. The couple asked for termination of pregnancy. On pathologic examination the absence of left kidney was confirmed whereas the right kidney which measured 3.5 cm was filled with numerous cysts of 0.2 cm to 1 cm. of diameter and fibrosis. According to the Potter's classification these images are characteristic of a dysplasia type II. There was no hepatic fibrosis. family history revealed that the mother is in good health, she had previously a normal son. The father had a unilateral renal agenesia which was diagnosed after he had arterial hypertension when he was 25-years-old. The paternal grand father and his brother had unilateral renal agenesia which was shown by screening. This family shows that renal agenesia can be autosomal dominantly inherited and that the expressivity of this anomaly is variable.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
5/162. Medically resistant neonatal hypertension: revisiting the surgical causes.OBJECTIVE: To present the importance of searching for the surgical causes of pharmacologically resistant hypertension in the neonatal population. STUDY DESIGN: A case report and discussion are provided. RESULTS: Severe hypertension in the neonatal period is uncommon and almost always has a secondary cause. Although a majority of hypertensive neonates can be successfully managed with medical therapy, some cases are resistant to pharmacological treatment. We report three hypertensive neonates who failed to respond to intensive multidrug therapy. This led to further evaluation and identification of obstructive uropathies in two neonates and renovascular disease that necessitated surgical intervention. Subsequently, all patients had prompt resolution of hypertension and normalization of renal function. All are now off antihypertensive medications and have normal renal function at 12 months of follow-up. CONCLUSION: Our report exemplifies the importance of the consideration of surgical etiologies for differential diagnosis in neonates with severe hypertension that is unresponsive to pharmacological therapy. early diagnosis and prudent management of these etiologies reduce morbidity and mortality and preserve of renal function.- - - - - - - - - - ranking = 9.3793862790754keywords = renovascular, hypertension (Clic here for more details about this article) |
6/162. Marsupialization for bilateral pararenal lymphatic cysts.A case of bilateral pararenal lymphatic cysts associated with hypertension is described. The cysts surrounded the renal parenchyma and were located underneath the renal capsule. No vascular pathology explaining the cause of hypertension was diagnosed by radiological techniques. hypertension improved following bilateral marsupialization.- - - - - - - - - - ranking = 2keywords = hypertension (Clic here for more details about this article) |
7/162. 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 = 1keywords = hypertension (Clic here for more details about this article) |
8/162. Spontaneous renal artery dissection revealed by helical CT angiography.Spontaneous renal artery dissection is rare and may be misdiagnosed because its clinical presentation is confusing. Diagnosis is usually made by intra-arterial angiography. We report a case where a spontaneous renal artery dissection was initially misdiagnosed as a renal abscess. Diagnosis was made later with helical CT for suspicion of renal artery stenosis in a patient with recent onset of hypertension. This unusual case shows that helical CT can be useful for non-invasive diagnosis of renal artery dissection.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
9/162. Nephroptosis: the Tc-99m glucoheptonate scan as a diagnostic method.A 40-year-old man was examined because he was a potential "healthy" renal donor. However, the routine work-up before surgery revealed hypertension, although there was no family history of this condition. The patient was examined to exclude secondary causes of hypertension. Tc-99m glucoheptonate renal imaging showed nephroptosis of the right kidney when the patient was standing, and this may have been the cause of the hypertension.- - - - - - - - - - ranking = 3keywords = hypertension (Clic here for more details about this article) |
10/162. Corticotropin-independent Cushing's syndrome caused by an ectopic adrenal adenoma.Although nonsecreting suprarenal embryonic remnants are frequently found in the urogenital tract, adenomatous transformation resulting in glucocorticoid excess is a rare phenomenon. We report a case of a 63-yr-old woman that presented with new-onset hirsutism, facial plethora, hypertension, centripetal obesity, and a proximal myopathy. The 24-h urinary free cortisol excretion rate was elevated, and the serum ACTH level was suppressed. The patient failed an overnight and low dose dexamethasone suppression test and did not respond to CRH stimulation. In light of the undetectable baseline morning ACTH levels and the blunt response to CRH, the diagnosis of corticotropin-independent Cushing's syndrome was made. Imaging studies revealed normal adrenal glands and enlargement of a left pararenal nodule incidentally observed 4 yr before the onset of symptoms. Dramatic resolution of symptoms was observed after surgical removal of the 3.5-cm mass. Pathological exam confirmed adrenocortical adenoma in ectopic adrenal tissue. The case reported here represents the unusual circumstance in which the development of adenomatous transformation of ectopic adrenal tissue has been prospectively observed with imaging studies. It illustrates the importance of considering ectopic corticosteroid-secreting tumors in the context of corticotropin-independent Cushing's syndrome.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
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