1/25. 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 = physical examination, physical (Clic here for more details about this article) |
2/25. Renal infarction: an uncommon mimic presenting with flank pain.A 39-year-old woman arrived to the emergency department complaining of a constant, progressive, left flank pain, with no beneficial effect from spasmolytic and nonsteroidal antiinflammatory drugs. Two years before, she suffered another episode of right flank pain and stranguria, but instrumental examinations (ultrasonography, urography) remained negative. Besides a mild tenderness in the left flank, physical examination was normal. Blood chemistry panel showed leukocytosis (17.2 x 10(3) mL, neutrophils 82.8%) and a slight increase of serum lactate dehydrogenase (LDH) (543 U/L versus 230 to 460 U/L). Urinanalysis showed a slight hemoglobinuria (0.5 mg/dL), and sediment contained some red cells and leukocytes. Diagnostic examinations (ultrasonography, computed tomography) showed a left renal nonhomogeneous space-occupying lesion, orientative for renal malignancy. She was transferred to the urology department and operated. Both intraoperatory and histological diagnosis was ischemic infarction and, after exclusion of all possible underlying causes, final diagnosis was idiopathic renal infarction. Diagnostic procedures and literature reports are discussed.- - - - - - - - - - ranking = 1keywords = physical examination, physical (Clic here for more details about this article) |
3/25. Huge renal arteriovenous malformation mimicking simple parapelvic cyst.The presenting symptoms of renal arteriovenous malformations are usually gross hematuria and hypertension. Herein we present an unusual case of huge renal arteriovenous malformation without these signs, but with ultrasound picture mimicking simple parapelvic cyst. Other imaging test, including Duplex ultrasound, computerized tomography and aortography, demonstrated that vascular lesion. We suggest that Duplex ultrasound should accompany the routine renal ultrasound in order not to miss such cases, especially when the physical examination suggests intra-abdominal vascular lesion or bleeding.- - - - - - - - - - ranking = 1keywords = physical examination, physical (Clic here for more details about this article) |
4/25. Perinephric abscesses in the neurologically impaired.STUDY DESIGN: Retrospective chart review. OBJECTIVES: To document the occurrence and management of large perinephric abscesses in neurologically impaired patients at high risk for this infectious complication. SETTING: US veterans Affairs hospital. methods: The records, radiographs, operative findings and outcomes of all patients who presented with perinephric abscesses evident on physical exam within the last 5 years were reviewed. RESULTS: Four patients presented with large perinephric abscesses evident on physical examination. All had severe neurologic impairment with high sensory levels; three had spinal cord injuries, one had advanced multiple sclerosis. All had neurogenic bladders and recurrent urinary tract infections. The diagnosis was made through a combination of history, physical examination and computed tomography (CT) examination. All were found to have upper tract obstruction. All were managed with immediate abscess drainage and three had elective nephrectomy once the infection had resolved. No patients died of their perinephric abscess. CONCLUSIONS: These four cases illustrate that although advances in antibiotics, imaging and percutaneous management have improved the speed of diagnosis and reduced the mortality in patients with perinephric abscesses, the neurologically impaired population continues to remain at significant risk for the development and the delayed diagnosis of these morbid renal infections.- - - - - - - - - - ranking = 2.0616365404135keywords = physical examination, physical (Clic here for more details about this article) |
5/25. Secondary amyloidosis in Castleman's disease: review of the literature and report of a case.It is quite rare to diagnose secondary amyloidosis during the course of Castleman's disease (CD). A 51-year-old female who complained of fatigue, weight loss, and fever was diagnosed with CD -- plasma cell type -- in our hospital in 1993. One year after diagnosis, she developed nephrotic syndrome, the etiology of which was found to be secondary amyloidosis based on renal biopsy. As the patient rejected therapy, she was discharged after only symptomatic treatment. At her last follow-up in March 2001, she had no complaints; physical examination, blood chemistries, and urinalysis were normal. Abdominopelvic tomography revealed no lymphadenopathy in the abdomen, which had been previously present. We could identify 17 other cases of CD with secondary amyloidosis in the literature. Ours is the 18th such case and the 2nd case of multicentric CD leading to amyloidosis. This case also shows that CD might sometimes run a relatively benign course being cured with no therapy, whereas it might have a rapidly fatal downhill course -- even with therapy -- in others. Still, effective treatment strategies need to be developed.- - - - - - - - - - ranking = 1keywords = physical examination, physical (Clic here for more details about this article) |
6/25. Cases from the osler medical service at Johns Hopkins University.PRESENTING FEATURES: A 29-year-old woman with a history of rheumatic heart disease and one episode of endocarditis as an adolescent was admitted to the hospital after 1 week of headache, fever, and myalgia. Her past medical history was otherwise unremarkable and did not include illicit drug use. On physical examination, she had a previously noted 3/6 holosystolic murmur at the apex, which radiated to her back; a previously noted 1/4 diastolic murmur at the right upper sternal border; diminished strength in her right upper extremity; multiple painful erythematous nodules on her fingers (Figure 1); and red streaks under her nails (Figure 2). magnetic resonance imaging of the brain demonstrated multiple lesions; the largest was in the right frontal lobe with associated hemorrhage (Figure 3).What is the diagnosis?- - - - - - - - - - ranking = 1keywords = physical examination, physical (Clic here for more details about this article) |
7/25. copper deficiency anemia and nephrosis in zinc-toxicity: a case report.zinc is a ubiquitous element that is essential for normal enzymatic function in multiple metabolic pathways. Chronic excessive zinc ingestion causes severe reversible anemia in humans. In animals, zinc toxicity leads to anemia as well as physiologic and morphologic damage to the pancreas, kidneys, and often, multisystem failure and death. In this case, a young female ingested approximately 2000 mg of zinc gluconate daily for 12 months. She subsequently developed anemia consistent with zinc-induced copper deficiency and severe nephrosis. After cessation of zinc ingestion, her anemia and nephrosis resolved. This case study underscores the importance of an accurate and thorough investigation of nutritional supplements during the history and physical examination. Given the promulgation of zinc for the treatment of skin disorders and the common cold, along with the commercialization of nutritional supplements, unimpeded by regulatory guidelines, it is imperative that primary care physicians be attuned to the potentially dangerous consequences of excessive zinc ingestion.- - - - - - - - - - ranking = 1keywords = physical examination, physical (Clic here for more details about this article) |
8/25. Fine-needle aspiration cytology of bilateral renal malakoplakia.Isolated bilateral renal malakoplakia in the absence of concomitant involvement of the urinary tract is a rare occurrence. We report imaging, cytologic, and histologic findings of such a case diagnosed initially by fine-needle aspiration (FNA) cytology. A 26-yr-old female presented with pain in the left flank, fever, anorexia, and weight loss for the past 2 mo. A left lumbar lump was palpable on physical examination. Imaging studies showed an enlarged nonfunctioning left kidney and a small lesion in the right kidney with preserved function. FNA from both kidneys yielded purulent material positive for E. coli on culture. The smears were inflammatory, with a predominance of neutrophilic polymorphs and numerous histiocytes along with some intracellular and extracellular Michaelis-Guttman bodies, which were highlighted with the use of a PAS stain. histology of the nephrectomy specimen showed ill-defined nodules, composed of foamy histiocytes intermingled with neutrophils, plasma cells, and many variably sized concentric laminated bodies. The right-side lesion resolved with the use of broad-spectrum antibiotics in conjunction with ascorbic acid and bethanecol. When imaging studies are suggestive of a chronic inflammatory process, renal malakoplakia must always be considered in the differential diagnosis even if aspirated material shows a predominance of polymorphonuclear leukocytes. The use of special stains like PAS, Von Kossa, and Perl's helps in reaching the correct diagnosis.- - - - - - - - - - ranking = 1keywords = physical examination, physical (Clic here for more details about this article) |
9/25. Nephrogenic systemic fibrosis among liver transplant recipients: a single institution experience and topic update.Nephrogenic systemic fibrosis (NSF) is a recently characterized systemic fibrosing disorder developing in the setting of renal insufficiency. NSF's rapidly progressive nature resulting in disability within weeks of onset makes early diagnosis important. Two reports of NSF after liver transplantation are known of. We present three cases of NSF developing within a few months after liver transplantation and review the current literature. Loss of regulatory control of the circulating fibrocyte, its aberrant recruitment, in a milieu of renal failure and a recent vascular procedure appear important in its development. Known current therapies lack consistent efficacy. Only an improvement in renal function has the greatest likelihood of NSF's resolution. Delayed recognition may pose a significant barrier to functional recovery in the ubiquitously deconditioned liver transplant patient. Early recognition and implementation of aggressive physical therapy appear to have the greatest impact on halting its progression.- - - - - - - - - - ranking = 0.06163654041352keywords = physical (Clic here for more details about this article) |
10/25. A complex case of renal amyloidosis with a rare co-occurrence of 2 mutations in separate hereditary periodic fever syndrome-related genes.A 41 year-old male was admitted because of nephrotic syndrome associated with renal impairment and arterial hypertension. Renal biopsy showed a complete subverting of renal architecture with eosinophilic, amorphous deposits which stained positive for congo red and were positive for antibodies against AA-amyloid. abdominal fat pad aspirate confirmed the diagnosis of AA amyloidosis. Despite high values of serum amyloid A (SAA), surprisingly medical history, physical examination and all tests failed to identify any underlying inflammatory disease, even asymptomatic, at presentation and during the whole follow-up period. The patient carried a mutation (Glu148Gln) in the MEFV gene, and a mutation (Arg92Gln) in the TNFRSF1A gene, both in heterozygosity. The patient has never complained of the typical features of the familial mediterranean fever or of the TNF receptor-associated periodic syndrome. The patient's father carried the same mutations. His father's medical history was unremarkable; renal tests, acute-phase reactants and SAA were normal. During a trial with colchicine (while the patient was also taking atorvastatin) SAA decreased, renal function continued to deteriorate and proteinuria remained high; no cardiac involvement was detected. Six months later our patient developed rhabdomyolysis, thus accelerating the decline of renal function and requiring the start of dialysis.- - - - - - - - - - ranking = 1keywords = physical examination, physical (Clic here for more details about this article) |
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