1/20. Heavy chain deposition disease: the disease spectrum.A 45-year-old white woman was found to have microscopic hematuria during her annual physical examination. After a negative urologic workup, she returned 5 months later with nephrotic syndrome, renal insufficiency, and hypocomplementemia. Renal biopsy showed a nodular sclerosing glomerulopathy that could not be further characterized because of inadequate tissue for immunofluorescence. The patient returned 8 months later with chronic renal failure. A repeat renal biopsy showed deposits composed of immunoglobulin g (IgG) heavy chain and complement components C3 and C1 along glomerular, tubular, and vascular basement membranes, with negativity for kappa and lambda light chains, findings consistent with heavy chain deposition disease (HCDD). The heavy chain subclass was exclusively IgG3. Staining with monoclonal antibodies to epitopes of the constant domains of IgG heavy chain showed a CH1 deletion, indicating a truncated heavy chain. On review of the previously reported cases of HCDD, common clinical presentations include nephrotic syndrome, renal insufficiency, hematuria, and, in some cases, hypocomplementemia. In most patients, the hematologic disorder is mild, without overt myeloma. light microscopy shows a nodular sclerosing glomerulopathy, and heavy chain deposits are detectable within basement membranes throughout the kidney by immunofluorescence and electron microscopy. There is no effective treatment for this condition, and virtually all patients progress to chronic renal failure.- - - - - - - - - - ranking = 1keywords = physical examination, physical (Clic here for more details about this article) |
2/20. A child with spider bite and glomerulonephritis: a diagnostic challenge.A previously healthy 7-year-old white boy presented to St. Louis Children's Hospital with a 1-day history of headache, malaise, temperature of 38.7 degrees C, and a progressively erythematous, tender calf with central dusky purpura. On the morning of admission, his mother noticed a 2-mm crust on the patient's right calf with a 3-cm x 3-cm area of surrounding erythema. No history of recent trauma or bite was obtained. He had suffered two episodes of nonbloody, nonbilious emesis during the last day. In addition, over the previous 12 h, he presented brown urine without dysuria. His mother and brother had suffered from gastroenteritis over the previous week without bloody diarrhea. On initial physical examination, there was a 6-cm x 11-cm macular tender purpuric plaque with a central punctum on the right inner calf, which was warm and tender to the touch, with erythematous streaking towards the popliteal fossa (Fig. 1). The inguinal area was also erythematous with tender lymphadenopathy and induration, but without fluctuance. Laboratory studies included an elevated white blood cell count of 20, 800/microL with 6% bands, 86% segs, and 7% lymphocytes, hemoglobin of 12.5 g/dL, hematocrit of 35.1%, and platelets of 282,000/microL. The prothrombin time/activated partial tissue thromboplastin was 10. 4/28.0 s (normal PT, 9.3-12.3 s; normal PTT, 21.3-33.7 s) and fibrinogen was 558 mg/dL (normal, 192-379 mg/dL). urinalysis showed 1 protein, 8-10 white blood cells, too numerous to count red blood cells, and no hemoglobinuria. His electrolytes, blood urea nitrogen (BUN), and creatine were normal. The urine culture was negative. Blood culture after 24 h showed one out of two bottles of coagulase negative staphylococcus epidermidis. The patient's physical examination was highly suggestive of a brown recluse spider bite with surrounding purpura. Over the next 2 days, the surrounding rim of erythema expanded. The skin within the plaque cleared and peeled at the periphery. The coagulase negative staphylococci in the blood culture were considered to be a contaminant. cefotaxime and oxacillin were given intravenously. His leg was elevated and cooled with ice packs. The patient's fever resolved within 24 h. The lesion became less erythematous and nontender with decreased warmth and lymphadenopathy. The child was discharged on Duricef for 10 days. Because the patient experienced hematuria rather than hemoglobinuria, nephritis was suggested. In this case, poststreptococcal glomerulonephritis was the most likely cause. His anti-streptolysin-O titer was elevated at 400 U (normal, <200 U) and C3 was 21.4 mg/dL (normal, 83-177 mg/dL). His urine lightened to yellow-brown in color. His blood pressure was normal. Renal ultrasound showed severe left hydronephrosis with cortical atrophy, probably secondary to chronic/congenital ureteropelvic junction obstruction. His right kidney was normal.- - - - - - - - - - ranking = 2keywords = physical examination, physical (Clic here for more details about this article) |
3/20. rhabdomyolysis triggered by an asthmatic attack in a patient with McArdle disease.We describe a patient with McArdle disease who developed rhabdomyolysis triggered by a bronchial asthmatic attack. A 64-year-old man had chronic pulmonary emphysema with asthma, and an asthmatic attack led to severe rhabdomyolysis that required continuous hemodiafiltration. After 2 years, a physical examination revealed atrophy of the extremities compared with previous examinations, especially of the intercostal muscles. During that time, he suffered two severe bronchial asthmatic attacks. His serum level of creatinine kinase remained between 4,000 and 7,000 IU/l when he did not suffer from asthmatic attacks and rhabdomyolysis had abated. Therefore, we suspected that his recent muscle atrophy was caused by asthmatic attacks, and discussed the possibility of his respiratory muscle weakness due to McArdle disease in relation to his severe bronchial asthmatic attacks as well as chronic obstructive pulmonary disease.- - - - - - - - - - ranking = 1keywords = physical examination, physical (Clic here for more details about this article) |
4/20. light chain amyloidosis of the urinary bladder. A site restricted deposition of an externally produced immunoglobulin.AIMS:To identify the amyloid protein in a patient with amyloidosis localised to the urinary bladder, and to see whether subtyping of the protein by sequence analysis increases the understanding of the selection of the urinary bladder as the site of amyloid deposition. methods:A patient with gross haematuria and a congophilic mass in his urinary bladder was evaluated further. Characterisation of the amyloid protein was performed using conventional histological and immunohistochemical methods. Determination of the N-terminal amino acid sequence of the amyloid protein was performed using protein sequencers. RESULTS:The patient's history, physical examination, and laboratory evaluation excluded the involvement of other organs, justifying a diagnosis of amyloidosis localised to the urinary bladder. Histological and immunological studies showed that the amyloid protein deposited in the urinary bladder of the patient was probably of the amyloid light chain type. No plasma cells or lymphocytes were seen in sections of the urinary bladder and lower ureter adjacent to the amyloid deposits. Molecular analysis showed the sequence NFMLTQPHSISGSPG, which assigned the amyloid protein to either the Vlambda(I) or the Vlambda(VI) immunoglobulin (Ig) light chain families. CONCLUSIONS:The findings suggest that the amyloid protein in this patient originated outside the urinary bladder. The heterogeneity of the Ig proteins in known cases of amyloidosis of the lower urinary tract suggests that the amino acid residues, which determine the Vlambda subtyping, have no major role in restricting the deposited protein to the urinary bladder.- - - - - - - - - - ranking = 1keywords = physical examination, physical (Clic here for more details about this article) |
5/20. A rare case of painless gross hematuria: primary localized AA-type amyloidosis of the urinary bladder.A 60-year-old Asian woman presented with a 1-year history of painless gross hematuria. She had no prior urologic history and no other medical problems. Her physical examination, urine cytology, and computed tomography scan were normal. cystoscopy demonstrated several punctate areas of erythema within the bladder. Biopsies revealed amyloidosis, and immunohistochemical staining of the specimens defined the process as amyloid AA (often called secondary amyloidosis). The workup for systemic conditions associated with amyloid AA was negative. This represents an unusual case of primary localized AA-type amyloidosis of the bladder.- - - - - - - - - - ranking = 1keywords = physical examination, physical (Clic here for more details about this article) |
6/20. An unusual cause of abdominal pain.A 36-year-old woman presented to the Emergency Room because of abdominal pain associated with hematuria and red blood blending to stool. On admission, the physical examination revealed abdominal tenderness and diffuse cutaneous hematoma. The laboratory findings showed abnormal clotting tests with high International Normalised Ratio (INR) and prolonged activated partial thromboplastin time. hemoperitoneum and ureteral hematoma were noted on the abdomen computed tomography. The patient confessed she had ingested difenacoum for several weeks. All the symptoms resolved with fresh frozen plasma perfusion and vitamin k.- - - - - - - - - - ranking = 1keywords = physical examination, physical (Clic here for more details about this article) |
7/20. Loin pain hematuria syndrome: case series.Loin pain hematuria syndrome (LPHS) is a rare condition manifested by flank pain, hematuria, and occasional low-grade fevers. Usually there are no physical findings other than hematuria. Although numerous etiological theories exist, the pathophysiology of LPHS is unknown and diagnosis remains one of exclusion. Renal autotransplantation has been suggested as an effective procedure to control the severe and debilitating pain. However, pain often recurs in the long term. This paper reviews our experiences with the diagnosis and management of this condition.- - - - - - - - - - ranking = 0.081859610095278keywords = physical (Clic here for more details about this article) |
8/20. Development of IgA nephritis following cat scratch disease in a 13-year-old boy.We describe a 13-year-old boy who presented with hematuria and intermittent low-grade proteinuria at the time when he was diagnosed with cat scratch disease. Two months before presentation, he had a negative urinalysis during a routine physical evaluation. He continued to have microscopic hematuria for the next 6 months, when he developed gross hematuria and recurrence of low-grade proteinuria. The renal biopsy showed evidence of mild/moderate IgA nephropathy. We speculate that the immunological changes associated with cat scratch disease in this patient may have triggered the development of IgA nephropathy. A history or serological evidence of infection with bartonella henselae may need to be sought in patients presenting with IgA nephritis.- - - - - - - - - - ranking = 0.081859610095278keywords = physical (Clic here for more details about this article) |
9/20. Spontaneous remission of persistent severe hematuria in an adolescent with nutcracker syndrome: seven years' observation.A Japanese boy aged 14 years presented with gross hematuria associated with mild proteinuria and was diagnosed as having nutcracker syndrome. magnetic resonance angiography (MRA) revealed significant compression of the left renal vein between the aorta and the superior mesenteric artery with collaterals. A percutaneous renal biopsy on the right kidney revealed no evidence of glomerular or interstitial changes with immune deposition. He was observed closely without any intervention thereafter. Although repeat MRA performed 4 years after our first observation disclosed the development of collateral veins, severe hematuria with an intermittent exacerbation remained unchanged. During the next 2 years, the hematuria completely subsided spontaneously. Although the etiology of spontaneous remission of the disease remains speculative, his good physical development (i.e., approximately 10 cm taller than his height at the onset) may change presumptive hemodynamic factors. These clinical observations suggest that a proportion of pubertal patients with nutcracker syndrome should be treated conservatively for a relatively long time.- - - - - - - - - - ranking = 0.081859610095278keywords = physical (Clic here for more details about this article) |
10/20. Renal cell carcinoma: case report and literature review.Renal cell carcinoma arising from epithelial cells of the renal tubule is a highly aggressive and malignant tumor in all ages. Less than 2% of cases occur in childhood, relatively in older age group. Only a few pediatric series have been presented in the English literature. Tumor is presented with characteristic findings of flank pain, gross hematuria, and palpable mass. Although one half of the patients have metastasis at the time of diagnosis, most cases are currently being incidentally detected using improved imaging techniques. The overall prognosis in children appears to be similar to that in adults. Tumor stage and complete surgical resection have been reported as the most meaningful prognostic factors for the outcome. The incidence of metastatic disease is same as in adults. The effect of chemotherapy and immunotherapy, either preoperatively or postoperatively, is unclear. Cure is the most likely consequence in localized and completely resected tumors. Here, we present an 8-year-old boy with renal cell carcinoma demonstrating only hematuria without any pathological physical examination findings. The mass was described by abdominal ultrasonography and computed tomography in the left kidney. After the left nephroureterectomy, the patient was given no therapy.- - - - - - - - - - ranking = 1keywords = physical examination, physical (Clic here for more details about this article) |
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