Bilateral pheochromocytomas with von Hippel-Lindau's disease: a case report

A case of bilateral pheochromocytomas with von Hippel-Lindau's disease (VHL) is reported. A 46-year-old woman who had hyperglycemia was admitted to our hospital because of abdominal tumors. Her elder sister and niece had been diagnosed as VHL. Ultrasonography, CT, and MRI revealed bilateral adrenal tumors. Noradrenaline levels in serum and urine were elevated and 131I-MIBG scintigraphy showed accumulation in bilateral adrenal glands. Moreover, she had bilateral renal cysts and cerebellar hemangioblastoma. Bilateral adrenalectomies were performed and pathological diagnosis was pheochromocytoma. This is the seventh case of bilateral pheochromocytomas with VHL reported in Japan.     

A renal cause for massive retroperitoneal hemorrhage--renal angiomyolipoma

In any case of intra-abdominal trauma or hemorrhage a preoperative IVP is essential to determine the renal status. Preoperative catheter occlusion of the renal artery in these vascular tumors may be attempted to decrease blood loss. Stigmas of tuberous sclerosis should be sought in patients with vascular renal tumors, especially if they are bilateral. Conservation of renal tissue in tuberous sclerotic patients harboring renal angiomyolipomas is important.     

Obstructive renal insufficiency caused by amoxicillin crystalluria

A 76-year-old woman was admitted to the ICU for a meningitis with rhombencephalitis due to Listeria monocytogenes. The treatment included amoxicillin (250 mg.kg-1.day-1) and gentamicin (3 mg.kg-1.day-1 over 6 days). Neurological outcome was favourable. However at the 14th day, an acute renal failure occurred, following macroscopic haematuria and milkiness urine. CT scan and sonography confirmed the diagnosis of obstructive renal failure with bilateral ureteral obstruction. Crystalluria caused by amoxicillin was suspected. Endoscopic ureteral insertion of double-J catheters permitted the recovery of a normal renal function.     

pH measurement: a comparison of results using glass membrane electrodes vs solid-state electrodes as a function of solution composition

We report a case of bilateral renal cell cancer and multiple lung metastasis who was first treated with left radical nephrectomy and nephron-sparing surgery of the right kidney. Consecutive interleukin-2 administration achieved partial response in lung disease and residual tumors were surgically removed. The disease-free state has continued for 33 months.     

Adenoviral-mediated interferon-gamma gene therapy augments pulmonary host defense of ethanol-treated rats

A case of anuric acute renal failure due to bilateral renal artery obstruction in a 42 year-old man is presented. The obstruction was caused by bilateral thrombosis secondary to arteritis. Autopsy showed granulomatous and necrotizing vasculitis in both main renal arteries. Scarring and also necrotizing vasculitis were found from interlobular to arcuate renal arteries. The present case indicates that vasculitis should be considered when there is renal artery obstruction in young patients.     

Tuberous sclerosis associated with renal angiomyolipoma, pulmonary lymphangioleiomyomatosis and subungual fibroma: report of a case

We report a case of tuberous sclerosis associated with bilateral renal angiomyolipomas (AMLs), pulmonary lymphangioleiomyomatosis (LAM) and subungual fibroma of hands and feet. A 42-year-old woman who was diagnosed as tuberous sclerosis at the age of 18 complained of left flank pain and abdominal fullness. Bilateral renal AMLs were pointed out when complete examinations were performed for hypertension at the age of 32. She suffered from severe left flank pain and abdominal distension due to the left renal tumor. Left nephrectomy and excision of the renal hilar tumor were performed. The left renal tumor weighed 1120 g, the perirenal space was filled with the tumor. histopathological diagnosis of the left renal tumor and renal hilar tumor was AML. In our case, bilateral pneumothorax appeared, and chest CT scan revealed bilateral multiple pulmonary cysts. Histopathological diagnosis of pulmonary cysts was LAM. Other complications of our case are intracranial calcification and adenoma sebaceum.     

High-dose glucocorticoids in multiple sclerosis patients exert direct effects on the kidney and skeleton

Radical nephrectomy was performed in 30 cases of renal cell carcinoma in which CT scan and angiography findings had indicated the possible suitability of enucleation. The lesions were then enucleated from the operative specimens and assessed histologically to find the feasibility of tumor enucleation and to determine its indications. Histological investigation demonstrated that the tumor was completely surrounded by the pseudocapsule in only 17 cases, and even in such tumors the thickness of the pseudocapsule was not always regular and sometimes tumor invasion was seen in the pseudocapsule. It is therefore advisable to resect the tumor with no less than several mm of surrounding renal tissue when the pseudocapsule can not be clearly distinguished. Success of the enucleation was confirmed histologically in 22 out of 30 cases (73.3%). The factors related to failure of the enucleation were invasion throughout the renal capsule in 2, tumor exposure on the resected surface in 5, residual tumor in the tumor bed in 2 and venous invasion in 6 cases. Satellite tumors were not detected in any case. Favorable results were obtained in low grade, low stage tumors less than 6 cm in diameter and clearly distinct from normal renal tissue, therefore such cases were thought to be appropriate indications for enucleation. Although we selected the cases thought to be appropriate for enucleation by imaging techniques preoperatively, success was not obtained in all cases. Enucleation is a useful and recommendable operative technique for bilateral renal cell carcinoma or renal cell carcinoma in a solitary kidney.(ABSTRACT TRUNCATED AT 250 WORDS)     

Posttraumatic acute renal insufficiency

Acute renal insufficiency is a severe, but most frequent reversible illness followed by sudden onset, oliguria or anuria of indefinite duration, by rapid increase in decomposition products of protein catabolism in serum, by acidosis and fluid balance and electrolytes disorder. The aetiologic factors of acute renal insufficiency are various. A very significant aetiological factor in the appearance of acute renal insufficiency is a trauma caused by any kind or type of weapons, arms or instruments [1-5, 6, 9-13, 15]. Of a total number of injured persons who were treated in our institution (4,086 injured persons), 251 (6.14 percent) were with acute renal insufficiency, and of that number with all signs and symptoms of acute renal insufficiency 37 (0.9 percent) were treated with haemodialysis. Of the number of dialysed patients 30 (80 percent) patients had oliguric form of acute renal insufficiency and 7 (19 percent) were with non oliguric form of acute renal insufficiency. The most frequent injuries were to abdomen and then to extremities, liver, chest and kidneys. The smallest percentage concerned isolated injuries in extremities. According to a pathogenic mortality mechanism, the highest mortality was in patients with haemorrhagic syndrome and in septic condition, and the minimal in patients with other syndromes, such as crush syndrome, etc. In 25 (68 percent) patients acute renal insufficiency was associated with haemorrhagic syndrome, in 7 (18.9 percent) with crush syndrome and in 5 (13.5 percent) with septic condition. In 36 (97 percent) patients haemodialysis was performed and in 1 (3 percent) subject peritoneal dialysis. The reason for such a small number of peritoneal dialysis are severe injuries to abdomen and chest, since this type of dialysis could not be performed for technical reasons. In 27 (73 percent) patients haemodialysis was performed as a type of intermittent heparinization. In 5 (14 percent) patients heparinization was a type of continual heparinization. Thanks to prompt haemodialysis together with medical therapy and surgical treatment, the mortality rate in our patients was lower in comparison to mortality rate in other centres (Table 3). The main causes of acute renal insufficiency in our patients were: Acute tubular nercosis, peripheral blood flow insufficiency (hypovolaemia, cardiovascular failure), and postrenal insufficiency (excretory obstruction, intrarenal obstruction, urinary organ ruptures, haemorrhagic shock) and the underlaying kidney disease. Acute renal insufficiency can be divided into acute renal insufficiency, primary parenchymal renal insufficiency and postrenal azotaemia [1-6, 9, 12, 13]. During the therapy of these patients it is important to evaluate the dehydration degree of patients by clinical and laboratory parameters. In case of hypovolaemia the complete compensation of fluid should consist of infusion together with administration of diuretics. The central venous pressure should be maintained at the values in a range from 6 to 8 cm H2O. In case of oliguric acute renal insufficiency the fluid intake should be equal to diuresis plus every other loss of fluids. Diet should be high-caloric with carbohydrates in the amount of 100 mg, and that amount should be given three to four times daily (both parenterally and orally) together with restriction of potassium intake due to a well known effect of potassium on myocardium function. Dosage of drugs which are eliminated via kidney should be managed promptly by parenteral administration of antibiotic agents [7, 8, 13-16]. Haemodialysis should be started at the very beginning of the patients admission to the hospital and should be associated with anticoagulant therapy for avoiding haemorrhages. Thanks to haemodialysis performed in time, the mortality rate in our patients was reduced in comparison to health centres where haemodialysis was delayed. Thanks to such treatment of patients with many severe injuries in whom the mortality rate is usuall     

CASES--clinical audit; scenarios for evaluation and study

The incidence of bilateral involvement it is generally estimated to be 5% to 10%. It shows association with certain congenital anomalies and it has an increased occurrence of familial cases. The records of 9 children (5 boys, 4 girls) diagnosed at Vall d'Hebron Hospital with bilateral Wilms tumor between 1976-1995 were analyzed. Six patients had synchronous tumors and 3 had metachronous lesions. Genitourinary malformations were present in 4 children and another had hemihypertrophy. Two children were brothers. Eight patients underwent pre-operative radiation therapy and/or chemotherapy. Five patients had nephrectomy on one side (3 of them had metachronous presentation) and partial nephrectomy on the other side. The other children had bilateral partial nephrectomy or tumorectomy. Seven out of the nine patients are alive (78%). The two children who died presented with stage IV tumors and high grade malignant. One boy suffers cardiomyopathy. All survivors have normal renal function. With the proven efficacy of chemotherapy, bilateral renal salvage procedures were demonstrated to be effective in controlling disease without compromising renal function or survival. The innovative approaches developed for the treatment of bilateral Wilms tumor may influence the treatment of unilateral Wilms.     

血供丰富的乏脂肪肾脏错构瘤与肾透明细胞癌的CT鉴别诊断

Objective: The aim of our study was to investigate the feature of minimal fat renal angiomyolipoma with sufficient blood supply using CT scans and improve the diagnosis accuracy required to differentiate it from clear cell renal carcinoma.Methods: Retrospective analysis of 24 cases of post-surgery confirmed angiomyolipoma with sufficient blood supply (total of 25 tumors) in our hospital that were used for a pathological comparison study. Results: Among the 24 patients diagnosed with angiomyolipoma, nobody had bloody urine. Of the 96 patients diagnosed with clear cell renal cancer, 14 had bloody urine (14.6%). In our studied group, the size of angiomyolipomas with sufficient blood supply was between 1.5 cm×2.0 cm to 8.0cm × 10.0 cm. During CT scan analysis, twenty tumors had similar density, and five of them had higher density. Only one tumor had a few dots of calcification (4%). Adipose tissue was not visible in 9 tumors, while 16 tumors had visible dots of adipose tissue, as visualized by CT scan. Intensive scanning indicated that all of the tumors showed a strong enhancement in the renal corticomedullary phase. Twenty tumors had significant heterogeneous enhancement in the early phase, while another set of five cases had homogenous prolonged enhancement. Nineteen patients had surgery to remove the angiomyolipomas, while six patients had single side kidney removal due to misdiagnosis for renal cancer in cases where the tumor severely compromised the renal parenchyma and sinus. All 25 cases were classified as renal angiomyolipoma by pathological analysis. Within the 96 cases of clear cell renal cancer, 64 tumors had relatively low density, 29 tumors had equal density, and 3 cases had relatively higher density. Fourteen of the tumors had calcification (14.6% ), and none of them had visualized adipose tissue.Enhanced CT scans indicated that 69 cases of renal cancer showed significant enhancement in the renal corticomedullary phase, which had the abnormal pattern of"fast-in-and-fast-out". Additionally, 27 cases had slow and prolonged enhancement.Conclusion: Similar to clear cell renal carcinomas, angiomyolipomas with sufficient blood supply also appear to exhibit abnormal enhancement with a pattem of "fast-in-and-fast-out" during the early phase, which is easily misdiagnosed as renal cancer.It is difficult to differentiate them merely through CT scans; the key to differentiating them is to identify the adipose tissue within the tumor. Therefore, it is helpful to use thin-layer CT scans to locate the adipose tissue.     

Case report: acute renal vein thrombosis in patients with spinal cord injury and secondary amyloidosis

Chronic spinal cord injury, when complicated by chronic suppurative infections, has replaced chronic tuberculosis as a leading cause of secondary amyloidosis. Renal involvement with secondary amyloidosis is characterized by the presence of nephrotic range proteinuria and an increased incidence of renal vein thrombosis. Two cases of acute renal vein thrombosis associated with secondary amyloidosis in patients with spinal cord injury are presented. In both cases, a past history of extensive decubitus ulcerations and urinary tract infections preceded the development of nephrotic range proteinuria. In case 1, nonoliguric acute renal failure occurred after the development of acute bilateral renal vein thrombosis. The patient declined dialytic therapy and expired with uremia. In case 2, worsening renal function and increased proteinuria resulted after the development of acute unilateral renal vein thrombosis. These cases include the clinical and anatomic findings of acute renal vein thrombosis that occur as a complication of secondary amyloidosis. Acute renal vein thrombosis should be considered whenever an acute change in renal function or increase in proteinuria is noted in this setting.     

Bilateral Sertoli cell tumor of testes with feminizing and masculinizing activity occurring in a child

A case of an unusual, histologically atypical, bilateral Sertoli cell tumor of testis, in association with masculinization and feminization effects, that occurring in a 5-year-old boy, is presented. Although Sertoli cell tumors are not infrequent, this case is morphologically and endocrinologically unique.     

Patients with chronic renal insufficiency in hemodialysis: nurses educational activities

The aim of this study is to contribute to the knowledge of the nurse's educative activity with chronic renal patients undergoing an hemodialytic treatment, providing an improvement in the patient's quality of life and using a model called "awareness education". The subjects of this study were eight chronic renal patients--four female patients and four male patients whose age varied from 17 to 63 years old. The methodological approach was the research-action and data collection about significant situations was carried out through participant observation. Data interpretation was related to the referential proposed by PAULO FREIRE, i.e. the analysis of the generating themes. Data were selected and encoded in six generating topics: Chronic Renal Failure (IRC), Causes of IRC, Hemodialytic Treatment, Chronic Renal Patient's Limitations and Possibilities when undergoing an Hemodialytic Treatment, Renal Transplantation and Family Support. The Discussion Groups were developed according to the Pedagogical Method by PAULO FREIRE. After that stage, satisfactory changes in the chronic renal patients' quality of life who participated in the teaching-learning process have been observed. This has enabled us to conclude that there has been a development of the na?ve consciousness for the criticism about their situation, owing to some changes in their reality.     

The use of bilateral adult renal allografts - a method to optimize function from donor kidneys with suboptimal nephron mass

Alternatives to traditional organ donor usage has allowed expansion of the organ donor pool to help compensate for the increasing disparity between recipients and donors. The use of bilateral adult renal transplants is a novel idea to salvage older donor kidneys with suboptimal nephron mass that would otherwise be destined for discard. Ten renal transplants were performed utilizing both kidneys from adult cadaver donors with diminished nephron mass determined by calculated glomerular filtration rate or biopsy evidence of significant glomerulosclerosis (>10%). Nine of ten (90%) recipients have satisfactory renal function at a mean follow-up of 7 months. The single case of graft failure was due to documented medical non-compliance. Mean serum creatinine at 6 months was 1.5 mg/dl. Mean measured creatinine clearance was 43.2+/-3.4. These preliminary findings suggest that the use of bilateral renal transplants provide satisfactory early function and allows salvage of older donor kidneys with suboptimal nephron mass.     

Primary osteosarcoma of the spermatic cord with synchronous bilateral renal cell carcinoma

Primary extraskeletal osteosarcoma of the spermatic cord is an extremely rare tumor with only two other cases being reported in the world literature. We describe a patient with this lesion who also had concomitant bilateral renal cell carcinoma. The management of this case and a review of this subject are presented.     

Lymphomatoid tumor infiltration of renal veins

A case of bilateral renal vein infiltration and thrombosis secondary to malignant lymphoma is presented. Although the kidney frequently is known to be involved by metastatic lymphoma, uremia is a rare terminal complication. The etiology and diagnosis of this disorder are discussed. We believe that it is important to recognize renal vein thrombosis in such patients so that treatment with radiation and/or chemotherapy may restore renal function.     

Use of prostaglandin E1 for treatment of erectile dysfunction

Metastatic tumors to the head and neck are uncommon. We report a case of renal cell carcinoma that presented as an epidermal inclusion cyst on the forehead. The forehead is an exceedingly uncommon site for renal cell metastases. We review the biology of tumor metastases and explore the pathways by which infraclavicular tumors spread to the head and neck. We then discuss the clinical appearance and management of metastatic renal cell carcinoma.     

The localization of aldosterone-producing adenoma on computed tomography--a comparative study with adrenal scintigraphy and plasma aldosterone concentration in the adrenal or renal vein

The evaluation of computed tomography (CT) for detecting aldosterone-producing adenoma in primary aldosteronism was performed by comparison with adrenal scintiscan; determination of aldosterone in adrenal or renal veins, retroperitoneal pneumography and adrenal venography was reliable for diagnosis of adrenal tumors in pheochromocytoma or Cushing's syndrome, but not so effective for small adenoma of primary aldosteronism. An abdominal CT scan was performed on six patients with primary aldosteronism, one with idiopathic hyperaldosteronism and one with glucocorticoid responsive hyperaldosteronism; in an attempt to evaluate the utility of this noninvasive procedure. Diagnosis of hyperaldosteronism was made by demonstrating the elevated plasma aldosterone concentration and aldosterone secretion rate, normal excretion rate of urinary 17-OHCS and 17-KS, and low plasma renin activity. The CT scan correctly predicted unilateral adrenal adenoma in all the patients with primary aldosteronism of which the findings were identical to those demonstrated by surgery. The diameter of these tumors ranged from 10 X 7 X 6 to 19 X 17 X 14 mm. Also the CT scan in idiopathic hyperaldosteronism and glucocorticoid responsive hyperaldosteronism showed bilateral adrenal hyperplasia and bilateral normal adrenal glands, respectively. The pathological findings in these two cases disclosed the adrenal hyperplasia of zona glomerulosa and adrenal hyperplasia of zona subglomerulosa accompanied by a normal thickness of the adrenal gland, respectively. The precision of the CT scan, adrenal scintigraphy and determination of plasma aldosterone in the adrenal or renal veins were almost equal to the diagnosis of the localization of adrenal adenoma. It is concluded that the CT scan is a noninvasive and most useful method for the localization of aldosterone-producing adenoma and helpful in distinguishing adrenal adenoma from adrenal hyperplasia.     

Prenatal maternal indomethacin use resulting in prolonged neonatal renal insufficiency

The short-term use of indomethacin has been shown to be relatively safe and effective in halting premature labor. Its use has been associated with adverse renal effects in both the fetal and newborn periods that are generally transient and resolve on discontinuation of the drug. However, limited data suggest that prolonged prenatal exposure to indomethacin may be harmful to the fetus. We report a case of prolonged severe renal dysfunction characterized by oligohydramnios and postnatal anuria, azotemia, and ultrasonographic kidney abnormalities associated with the long-term prenatal use of indomethacin. Although partial resolution was observed, a moderate decrease in renal function persists. Prenatal maternal indomethacin use represents a potential cause of renal dysfunction in the newborn infant that may be only partially reversible.     

Auricular ossification

Cefuroxime axetil has been associated with few reported adverse effects. We report a case of bilateral renal cortical necrosis in a female after receiving 7 doses over 4 treatment days. The patient presented with worsening symptoms consisting of arthralgias, pruritus, and abdominal pain. Laboratory data obtained was indicative of worsening renal failure and thrombocytopenia. The patient required hemodialysis by the third day. Kidney biopsy revealed cortical necrosis. The possible pathogenesis of cefuroxime axetil causing cortical necrosis in this case and a review of other reported cases of chemical induced renal cortical necrosis is discussed.