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

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

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

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

OBJECTIVE: We examined clinical and laoratory features retrospectively in 56 patients at the start and after the chronic maintenance hemodialysis in order to provide a more complete picture of the uremic symdrome in the dialysis era. for deciding the time when chronic hemodialysis must be started. METHODS: The records of 56 patients began chronic hemodialysis treatment at the Han Yang University Hospital from 1995 august until 1997 august were reviewed retrospectively. The information gathered included demographic data, renal diagnosis, uremic symptoms, biochemical values. RESULTS: The most prevalence of manifestation of uremia was general weakness (67.9%). Unexpected fin-dings were the wide ranges of serum creatinine levels (3.5 to 19.4mg/dL) and blood urea nitrogen levels (19 to 204mg/dL), creatinine clearance rate (1.2-17.4mL/ min), and the frequency of hyponatremia (19.6%), hypo-albuminemia (69.6%), and the anion gap above 14mByL was (66.7%). There was higher potassium leve1 in diabetes mellitus patients than non-diabetes mellitus patients(6.2+/-1.6mEq/L VS. 4.9+/-1.0mEq/L,p=01). Patients laboratory values were changed after the chronic maintenance hemodialysis-Albumin(3.2+/-0.8 to 3.6+/-0.5gm/dL, p=0.01), calcium (7.6+/-1.2 to 8.7+/-1.9mg/dL, p=0.01), he-matocrit(23.0+/-4.7 to 27.7+/-4.2% , p=0.01), phosphorus (5.6+/-2.1 to 4,6+/-1.4mg/dL, p=0.01), pH (7.30+/-0,1 to 7.35+/-0.2, p>0.05), anion gap (22,0+/-11.0 to 12.1+/-8.8mg/dL, p>0.05). CONCLUSION: Finally, uremic symptoms were expressed mainly gastro-intestinal and respiratory symptoms. Chronic renal failure associated with diabetes mellitus was earlier on set of uremic symptoms and higher level of serum potassium level than other underlying diseases. Uremic symptoms and laboratory values were almost corrected but metabolic acidosis was not corrected significantly after the chronic maintenance hemodialysis.
Acid-Base Equilibrium ; Acidosis ; Blood Urea Nitrogen ; Calcium ; Creatinine ; Diabetes Mellitus ; Diagnosis ; Dialysis ; Humans ; Hydrogen-Ion Concentration ; Hyponatremia ; Kidney Failure, Chronic ; Phosphorus ; Potassium ; Prevalence ; Renal Dialysis* ; Renal Insufficiency, Chronic* ; Retrospective Studies ; Uremia