Calcific uremic arteriopathy (CUA), termed calciphylaxis, is a rare but highly fatal clinical syndrome. There is no clearly laboratory diagnostic criteria for CUA. The medium and small arterial calcification and microthrombosis discovered by skin biopsy, radiologic imaging，bone scan and the evidence of activation of the bone morphogenetic protein signal (BMPs) transduction pathway are useful for early diagnosis of this disease. The common therapies (including intravenous sodium thiosulfate (STS) and bisphosphonates, hyperbaric oxygen therapy and other symptomatic supports) are used for the management of wounds, pain, nutrition, dialysis and so on. Controlling the chronic kidney disease-mineral and bone disorder (CKD-MBD) and some complications of dialysis and drugs (such as warfarin, active vitamin D) can prevent CUA. However, CUA patients still have poor prognosis and high mortality. Since some patients progress rapidly, it is of great importance to make early diagnosis and provide effective treatments with multidisciplinary management.
Calciphylaxis ; diagnosis ; prevention & control ; therapy ; Early Diagnosis ; Humans ; Renal Dialysis ; Uremia ; Warfarin

Two autopsy cases of polycystic disease of kidney were reported, both female,31 and 61 years of age: gravida 2,and 7 respectively. The incidence among autopsies was 0.3%.Both cases were accompanied with congenital polycysts in liver. The blood pressure of the cases was slightly or moderately elevated and hearts were moderately hpertropic. Renal function were not considerably impaired. Clinically the correct diagnosis were subarachnoidal hemorrhage(by rupture of aneurism) in 1st case and uremia after right nephrectomy in 2nd case. Etiology of the condition was discussed.
Autopsy* ; Blood Pressure ; Diagnosis ; Heart ; Incidence ; Kidney ; Liver ; Nephrectomy ; Rupture ; Uremia

A case of Wegener's Granulomatosis of 35 year old Korean male having 38 months duration with typical clinical symptoms and coular manifestations had been treated and observed in our clinic form April to November 21, 1958. The diagnosis was confirmed by the histopathological studies on biopsy specimen and by postmortem examination. The ultimate cause of the death in this case was thought to be massive cerebral hemorrhages as a result of systemic involvement of necrotizing angitis which is one of the characteristic features of this disease. Uremia and hypertension were not observed in this case to the end of the course. There is no report dealing with Wegener's Granulomatosis in Korean literatures to the best of our knowledge at this moment.
Adult ; Autopsy ; Biopsy ; Cerebral Hemorrhage ; Diagnosis ; Humans ; Hypertension ; Male ; Uremia ; Wegener Granulomatosis*

Uremic encephalopathy is a well-known disease with typical MR findings including bilateral vasogenic or cytotoxic edema at the cerebral cortex or basal ganglia. Involvement of the basal ganglia has been very rarely reported, typically occurring in uremic-diabetic patients. We recently treated a patient who had non-diabetic uremic encephalopathy with an atypical lesion distribution involving the supratentorial white matter, without cortical or basal ganglia involvement. To the best of our knowledge, this is only the second reported case of non-diabetic uremic encephalopathy with atypical MR findings.
Adult ; Brain Diseases, Metabolic/*diagnosis ; *Diffusion Magnetic Resonance Imaging ; Humans ; Male ; Uremia/*complications

Although ultrasonography is regarded as the gold standard in the diagnosis of obstructive nephropathy, dilatation is sometimes not observed by ultrasonography. We report upon a case of minimally dilated obstructive nephropathy due to an ureter stone in a kidney donor with volume depletion. A 54-year-old man was admitted due to anuria and abdominal pain of 2 days duration. Ten years previously, his right kidney was donated for transplantation, and one month before admission, he abstained from all food except water and salt, for 30 days for religious reasons. He had lost 8 kg of body weight. On admission, he had clinical signs of volume depletion, i.e., a dehydrated tongue and decreased skin turgor. Laboratory data confirmed severe renal failure, his blood urea nitrogen level was 107.3 mg/dL, and his serum creatinine 16.5 mg/dL. The plain X-ray was unremarkable and ultrasonography showed only minimal dilatation of the renal collecting system. On follow-up ultrasonography, performed on the 5th hospital day, the dilatation of the collecting system had slightly progressed and a small stone was found at ureter orifice by cystoscopy. Removal of stone initiated dramatic diuresis with a rapid return of renal function to normal by the third day.
Anuria/*etiology ; Diagnosis, Differential ; Human ; Male ; Middle Aged ; Nephrosis, Lipoid/complications/*ultrasonography ; *Tissue Donors ; Uremia/*diagnosis ; Ureteral Calculi/*complications

Hemolytic-uremic syndrome (HUS) is the most common cause of acute renal failure in young children. It is classically characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia, and uremia. Further, not only is intussusception one of the differential diagnoses of HUS but it may also become a complication during disease progression. We report a case of HUS preceded by intussusception in a previously healthy 17-month-old boy. The patient presented at the emergency department with bloody stools that developed the day after reduction of intussusception. HUS was diagnosed 4 days after the reduction of intussusception. The patient was provided only supportive care and his laboratory test findings were normal at discharge.
Acute Kidney Injury ; Anemia, Hemolytic ; Child ; Diagnosis, Differential ; Disease Progression ; Emergencies ; Hemolytic-Uremic Syndrome ; Humans ; Infant ; Intussusception ; Thrombocytopenia ; Uremia

Asphyxiating thoracic dysplasia(ATD;Jeunes's syndrome) is a rare variety of short limb dwarfism. It is characterized by an extremely small thorax when compared to the ab-dominal circumference, which frequently results in respiratory distress. Other anomalies as-sociated with Jeune's syndrome are pelvic bone malformations and renal dysplasia. It was first described and namely by Jeune et al. in 1954. Jeune's syndrome is an autosomal rece-ssive trait and has a 25% recurrence risk. These patients died at early age due to respirat-ory insufficiency. Death due to uremia has occurred in number of children surviving infan-cy, following progressive renal failure, hypertension and hepatic failure. About 50 cases have been reported in the world literature. We experienced a case of small thorax with short limb dwarfism on antenatal ultraso- und examination and then the baby was delivered by cesarean section. The diagnosis was confirmed to Asphyxiating thoracic dysplasia by clinical features, radiological findings and pathological findings. We reported a case of Asphyxiating thoracic dysplasia with review of literatures.
Cesarean Section ; Child ; Diagnosis ; Dwarfism ; Extremities ; Female ; Humans ; Hypertension ; Liver Failure ; Pelvic Bones ; Pregnancy ; Recurrence ; Renal Insufficiency ; Thorax ; Uremia

PURPOSE: Metastasis from primary malignancies anywhere in the body can spread to the retroperitoneum and lead to ureteral obstruction. We evaluated the correlation between pathologic stages of stomach cancer and ureteral obstruction, and their urologic manifestations. MATERIALS AND METHODS: Ten patients who had ureteral obstructions caused by stomach cancer were retrospectively analysed. Medical records associated with stomach cancer, urologic symptoms and signs, and methods of urinary diversion were reviewed. RESULTS: Pathologic stages of stomach cancer(UICC & AJC classification) at the time of primary surgery were stage III in 1 and IV in 8 patients. Mean time inteval between the diagnosis of stomach cancer and subsequent ureteral obstruction was 26.6 months(range: 2months-10years) and ureteral obstruction of 6 patients(60.0%) was discovered within 2 years. Urologic symptoms and signs were gross or microscopic hematuria(50.0%), elevated Cr(40.0%) and flank pain(40.0%). Nine out of 10 patients had one of these findings. Ureteral involvements were bilateral in 7(70.0%) and unilateral in 3 patients(30.0%). Sites of ureteral obstruction were upper ureter in 5 and midureter in 5 patients. 14 out of 17 obstructed kidneys were managed by double-J stent(7 kidneys) or percutaneous nephrostomy(7 kidneys). CONCLUSIONS: We believe that we should take into account the possibility of ureteral obstruction by direct invasion or lymph node metastasis in patients who had advanced stomach cancer. If such patients show hematuria, uremia or flank pain, secondary ureteral obstruction should be suspected. And malignant ureteral obstruction should be detected and managed as early as possible to preserve renal function.
Diagnosis ; Flank Pain ; Hematuria ; Humans ; Kidney ; Lymph Nodes ; Medical Records ; Neoplasm Metastasis ; Retrospective Studies ; Stomach Neoplasms* ; Stomach* ; Uremia ; Ureter* ; Ureteral Obstruction* ; Urinary Diversion

Uremic pleuritis is a fibrinous pleuritis of unknown pathogenesis in patients with chronic kidney disease. Although it responds to regular dialysis or repeated thoracentesis, cases that are refractory to those therapies have been reported. We report a case of uremic pleuritis which showed marked improvement following corticosteroid therapy. The effusion was exudate, and negative in cytology and microbiology. Pleural biopsy revealed chronic inflammation with fibrosis. The pleural effusion did not respond to chest tube drainage and continuance of hemodialysis. With a diagnosis of refractory uremic pleuritis, we started methylprednisolone. The pleural effusion responded to the treatment and resolved without complication.
Biopsy ; Chest Tubes ; Diagnosis ; Dialysis ; Drainage ; Exudates and Transudates ; Fibrin ; Fibrosis ; Humans ; Inflammation ; Methylprednisolone ; Pleural Effusion ; Pleurisy* ; Renal Dialysis ; Renal Insufficiency, Chronic ; Thoracentesis ; Uremia

The term dilated(congestive) cardiomyopathy refers to a variety of cardiac disorders that have in common ventricular dilation and reduced myocardial contractility, and is derived from the common late clinical manifestation of congestive heart failure. Since echocardiography can evaluate several aspects of anatomical structures and cardiac function, it has provide a valuable contribution to the understanding of ventricular function and diagnosis of dilated cardiomyopathy. The purpose of this study is to perform the echocardiographic evaluation of left ventricular function in 44 patients with dilated cardiomyopathy and to compare the patients with normal individuals. The patients, 24 males and 20 females, had a mean age of 53.0 and body surface area of 1.61m2. Normal individuals as a control group, 10 males and 10 females, had a mean age of 47.2 and body surface area of 1.67m2. Among 44 patients with dilated cardiomyopathy, 22 cases had a history of heart failure and in 15 cases systolic murmur was heard at the apex. The cardiothoracic ratio was 0.7+/-0.1 on chest X-ray which showed significant difference when compared to normal control group, being 0.4+/-0.1(p<0.01) One case showed left ventricular thrombus and in 8 cases small mounts of pericardial effusion were noted. Among the 44 patients two had uremia, one had a history of myocarditis, one patient was young female whose illness began during the peripartum period and one had a diabetes mellitus. Results obtained were as follows; 1) Mitral valve echocardiogram (1) DE amplitude and EF slope of mitral valve(16.5+/-3.3mm and 83.5+/-3.65mm/sec respectively) showed significant decrease when compared to normal control group (20.1+/-3.0 mm and 102.4+/-28.9 mm/sec respectively) (p<0.01 and p<0.05). (2) EPSS (25.5+/-7.6mm) and the distance between posterior cusp of mitral valve and left ventricular posterior wall (9.1+/-2.1mm) showed significant increase (p<0.01) when compared to normal control group (7.1+/-2.1mm and 5.9+/-1.2mm). The ratio of the separation of two mitral leaflets to the left ventrticular diastolic dimension (0.4+/-0.1) showed significant showed significant decrease in patients when compared to normal control group (p<0.01) (3) Among 44 patients with dilated cardiomyopathy, 12 cases showed B-notch in mitral valve. 2) Echocardiographic findings of aortic valve. Left atrial size was markedly increased (37.7+/-7.5mm) when compared to nomal control group (29.1+/-4.9) (p<0.01). The ratio of left atrial size to aortic dimension was 1.3+/-0.3, showing significant increase when compared to normal control group (0.99+/-0.3) (<0.01). And 4 cases showed systolic notch in aortic valves. 3) Echocardiographic findings of left ventrcle and ventricular septum. (1) Left ventricular diastolic and systolic dimensions (41.8+/-7.8mm/m2 and 36.7+/-7.2mm/m2) were markedly increased in patient with dilated cardiomyopathy, when compared to normal control group (30.4+/-2.6mm/m2 and 11.4+/-3.2mm/m2) (p<0.1, both). Left ventricular diastolic and systolic volume (181.5+/-91.0ml/m2 and 136.6+/-69.7ml/m2) were also significantly higher in patients with dilated cardiomyopathy when compared to normal control group (79.1+/-23.8mm/m2 and 19.4+/-2.5ml/m2) (p<0.01, both). (2) Ejection fraction (0.32+/-0.10) percent fractional shortening (12.3+/-4.6) and mVcf (0.5+/-0.2 cric/sec) showed significant decrease in patients when compared to normal control group (0.74+/-0.05, 34.6+/-6.7 and 1.4+/-0.2) (p<0.01, all). Thus, We could find the reduced myocardial contractility in dilated cardiomyopathy. (3) Systolic amplitudes of venticular septum (LSa) and ventricular posterior wall (Ena) were markedly decreased (p<0.01). The sum of both values (LSa+ENa) also showed significant decrease (12.0+/-4.2mm) when compared to normal control group (p<0.01) (4) Thickness of ventricular septum and ventricular posterior wall showed slight increase in patients, but there was no significant changes when compared to normal group. 4) Systolic time interval Echocardiograms showed significant increase in ratio of preejection period to ejection time, when compared to normal group (p<0.01). Thus, the author observed that in dilated cardiomyopathy the contractility of left ventricle was significantly reduced and the left ventricular diameter and diastolic volume of left ventricle showed marked increase. And also the decreased motions of ventricular septum and posterior wall of left ventricle were noted.
Aortic Valve ; Body Surface Area ; Cardiomyopathies ; Cardiomyopathy, Dilated* ; Diabetes Mellitus ; Diagnosis ; Echocardiography* ; Female ; Heart Failure ; Heart Ventricles ; Humans ; Male ; Mitral Valve ; Myocarditis ; Pericardial Effusion ; Peripartum Period ; Systole ; Systolic Murmurs ; Thorax ; Thrombosis ; Uremia ; Ventricular Function ; Ventricular Function, Left* ; Ventricular Septum