Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0033687 (proteinuria)
 24,015 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We performed a computerized search on sulfadiazine-associated nephrotoxicity reported in human immunodefiency virus (HIV)-infected patients in the international literature. Including an original case report, we summarized 35 acquired immunodefiency syndrome (AIDS) patients from 1987 to 1995 in an analysis comparing their features to historical HIV-negative controls from the 1940s and 1950s. Likely due to a high prevalence of potential risk factors, incidence of sulfadiazine-associated renal impairment was 1.9%-7.5% in AIDS patients and 1%-4% in HIV-negative controls. Its occurrence appeared to be delayed in HIV-infected patients with a median of about 3 weeks of medication compared with about 10 days in HIV-negative subjects. Correspondingly, the cumulative sulfadiazine dose at manifestation doubled in AIDS patients with a median of 84 g versus 40 g in controls. Patients usually presented with flank or lumbar pain, oliguria, and (macro) hematuria. Urinalysis showed typical "sheaves of wheat" crystalluria, erythrozyturia, and, less commonly, leukozyturia and proteinuria. Echogenic (mostly peripelvic) densities, renal stones, and hydronephrosis are frequent findings on ultrasound examination, whereas X-ray examination possesses a low diagnostic sensitivity. The principle aim of therapy in these patients is to (re)institute the physicochemical urinary solubility of sulfadiazine and its metabolites. For this purpose, forced rehydration and, most importantly, urine alkalinization proved to be effective measures without an absolute need to withhold the drug. Provided prophylactic and therapeutic recommendations are complied with, outcome of this drug-related side effect is usually excellent, and rare relapses will similarly respond well.
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PMID:Sulfadiazine-associated nephrotoxicity in patients with the acquired immunodeficiency syndrome. 869 59

We encountered a patient with megaureter-megacystis syndrome showing a giant bladder and dilated ureters with marked reflux, which is very rare; to our knowledge, only 2 patients have been reported in Japan. The patient was a 4-year-old boy, who showed inborn polyposia and polyuria, and proteinuria at the age of 1 year. He visited the pediatric department in our hospital complaining of cold-like symptoms, stomachache and diarrhea. Urinary infection and kidney dysfunction were observed, and the patient was hospitalized for close examination. Bilateral pyelocaliceal hydronephrosis was detected by ultrasonography, and the patient was referred to our department. CT revealed bilateral hydronephrosis (right atrophic kidney), hydroureters and megacystis. Bilateral grade V vesicoureteral reflux, an increase in the bladder volume (> 300 ml), and urination without residual urine were noted by voiding cystourethrography. Uroflowmetry revealed that maximum flow rate was 21.6 ml/sec, voided volume was 110 ml, and residual volume was 24 ml. From these examinations, the patient was diagnosed as having megaureter-megacystis syndrome, and underwent antireflux operation of the bilateral ureters using Cohen's procedures.
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PMID:[The so-called megaureter-megacystis syndrome: a case report]. 1051 89

The shortage of donor kidneys available for transplantation has resulted in an expansion of the criteria used for donor selection. The use of cadaveric pediatric kidneys has been suggested as a means to overcome this shortage, but is debated because of technical complications and an increased incidence of functional allograft impairment. We examined the records of all adult patients receiving renal allograft transplants from January, 1983 through December 1994, to determine whether kidneys from pediatric donors can be used safely for transplantation. During the study period, 204 adult patients received kidney transplants. The patients were divided into three groups by donor age: group 1, 26 months to 7 years old (n = 12); group 2, 7 to 14 years old (n = 19); and group 3, adult donors (n = 173). Cyclosporine- and/or azathioprine-based immunosuppression regimens were used. The three groups did not differ from one anther with respect to recipient age, recipient sex, primary renal disease, human leukocyte antigen (HLA) mismatching, or the proportion of recipients with previous transplants. The graft survival rates at 1 and 3 years, respectively, were 75% and 75% in group 1,84% and 79% in group 2, and 85% and 72% in group 3 (p > 0.05). The patient survival rates at 1 and 3 years, respectively, were 75% and 75% in group 1,95% and 90% in group 2, and 90% and 83% in group 3 (p > 0.05). The frequency of fixed proteinuria (> or = 0.8 gm/day) in the first 3 years was higher in the two pediatric groups (50%) than in the adult group (28%) (p < 0.05). The frequency of surgical complications was higher with pediatric grafts (group 1 vs group 3: 67% vs 24%, p < 0.005). In particular, group 1 patients had much higher rates of lymphocele (50% vs 8%), renal graft artery stenosis (33% vs 11%), and hydronephrosis (33% vs 9%) than group 3. We conclude that pediatric donor kidneys can be used with reasonable safety, although surgical complications remain a major problem.
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PMID:Long-term results with pediatric kidney transplants in adult recipients. 1063 19

A rare case of botryoid Wilms tumor is presented. The main clinical manifestations were persistent low-grade fever, malaise, and proteinuria associated with microhematuria. Ultrasonography revealed an echogenic mass in the right kidney, and a contrast-enhanced mass was found in the dilated collecting system by contrast-enhanced computed tomography. The surgically resected tumor was a polypoid, light-yellow, glistening mass that occupied a large part of the renal pelvis and originated from the pelvicaliceal wall. Part of the tumor extended to the proximal ureter, resulting in hydronephrosis in the involved kidney. No parenchymal lesion was observed. Microscopic examination revealed epithelial, stromal, and blastemal components, which indicated Wilms tumor. Infection had occurred in the hydronephrotic kidney, which presumably had caused the major presenting symptoms. The prognosis of our patient and previously reported cases of botryoid Wilms tumor was good compared with that of typical Wilms tumor, since the botryoid type can be detected at an early stage.
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PMID:Botryoid Wilms tumor: case report and review of literature. 1065 34

Chronic idiopathic intestinal pseudo-obstruction (CIIP) is a rare heterogeneous clinical syndrome characterized by recurrent episodes of symptoms and signs of intestinal obstruction in the absence of a mechanically obstructing lesion. Dilatation of other viscera, such as the renal pelvis, ureter or urinary bladder, is identified in a minority of patients. We report the cases of two patients with CIIP presenting with abdominal fullness and constipation. Radiologic examination of the first patient revealed dilatation of the esophagus, stomach, duodenum and bowel loops up to the ascending colon. The nerve conduction velocity study of the right extremities revealed polyneuropathy and urinary bladder manometry revealed poor sensation. The patient had been admitted to our hospital three times for symptomatic relief within the prior six months. During the last admission, his symptoms persisted without response to medical treatment. Soon after discharge, the patient underwent surgery at another hospital and died of nutritional problems. The second patient was transferred to our hospital after an exploratory laparotomy was performed one month earlier. A radiographic examination revealed distention of the stomach, duodenum, small intestine and ascending colon, as well as bilateral hydronephrosis. Rheumatologic examination revealed no evidence of autoimmune disorder. The patient also had heavy proteinuria due to minimal change disease that was proven by renal biopsy. After receiving prokinetic, cathartic and corticosteroid medication for kidney disease, symptoms improved, but hydronephrosis persisted.
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PMID:Chronic idiopathic intestinal pseudo-obstruction. 1074 24

We have used homologous recombination to disrupt the mouse gene coding for the NaK2Cl cotransporter (NKCC2) expressed in kidney epithelial cells of the thick ascending limb and macula densa. This gene is one of several that when mutated causes Bartter's syndrome in humans, a syndrome characterized by severe polyuria and electrolyte imbalance. Homozygous NKCC2-/- pups were born in expected numbers and appeared normal. However, by day 1 they showed signs of extracellular volume depletion (hematocrit 51%; wild type 37%). They subsequently failed to thrive. By day 7, they were small and markedly dehydrated and exhibited renal insufficiency, high plasma potassium, metabolic acidosis, hydronephrosis of varying severity, and high plasma renin concentrations. None survived to weaning. Treatment of -/- pups with indomethacin from day 1 prevented growth retardation and 10% treated for 3 weeks survived, although as adults they exhibited severe polyuria (10 ml/day), extreme hydronephrosis, low plasma potassium, high blood pH, hypercalciuria, and proteinuria. Wild-type mice treated with furosemide, an inhibitor of NaK2Cl cotransporters, have a phenotype similar to the indomethacin-rescued -/- adults except that hydronephrosis was mild. The polyuria, hypercalciuria, and proteinuria of the -/- adults and furosemide-treated wild-type mice were unresponsive to inhibitors of the renin angiotensin system, vasopressin, and further indomethacin. Thus absence of NKCC2 in the mouse causes polyuria that is not compensated elsewhere in the nephron. The NKCC2 mutant animals should be valuable for uncovering new pathophysiologic and therapeutic aspects of genetic disturbances in water and electrolyte recovery by the kidney.
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PMID:Uncompensated polyuria in a mouse model of Bartter's syndrome. 1077 55

Corticosteroids were successfully used to treat a 66-year-old man with retroperitoneal fibrosis (RPF) and previously diagnosed membranous nephropathy. Proteinuria was noted at the age of 51 years, and membranous nephropathy was diagnosed by renal biopsy. Ten years later, he presented with right hydronephrosis and renal dysfunction, and was diagnosed as having RPF based on the typical diagnostic imaging findings. Steroid therapy was successful, resulting in improvement of the hydronephrosis and renal function. The hydronephrosis recurred three years later, but corticosteroids were again effective in improving ureteral obstruction. This was a rare case of recurrent RPF with membranous nephropathy in which steroid therapy was effective in treating pleural effusion and hypergammaglobulinemia during the clinical course. This case suggests that an immunological disorder is involved in the pathogenesis of RPF.
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PMID:Retroperitoneal fibrosis associated with membranous nephropathy effectively treated with steroids. 1183 85

In patients with systemic lupus erythematosus(SLE), interstitial cystitis(lupus cystitis) is an uncommon, but important manifestation. We report two Japanese patients with lupus cystitis. Case 1 was a 49-year-old woman diagnosed as having rheumatoid arthritis and membranous nephropathy. She was treated with prednisolone(5 mg daily). Case 2 was a 41-year-old woman also diagnosed as having rheumatoid arthritis previously and treated with a non-steroidal anti-inflammatory drug. Both cases presented abdominal pain, vomiting, dysuria and frequency of micturition. We diagnosed these cases as SLE on the basis of arthritis, renal disorder(proteinuria and hematuria), and positive antinuclear and anti-dsDNA antibodies. In addition, bilateral hydronephrosis was found in both cases. Thus, they were also diagnosed as probable lupus cystitis. The patients were treated with one cycle of methylprednisolone pulse therapy. Thereafter they were treated with 60 mg/day of prednisolone and their symptoms resolved promptly. Furthermore, no abnormal finding was found by abdominal ultrasonography and/or the intravenous pyelogram after therapy. Renal biopsies were performed and both cases showed lupus glomerulopathy (case 1: WHO class Vb, case II: WHO class IVb). Abdominal pain and/or dysuria, which is common in SLE patients, requires further examinations to evaluate the lupus cystitis.
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PMID:[Two cases of lupus cystitis complicated by lupus nephritis treated successfully with steroid therapy]. 1473 94

The acute renal failure is a grave pathology, of rapid establishment and relatively frequent in the hospital environment. We can describe three etiological groupS, which are responsible for it, amongst which are emphasized the pre-renal reasons. The obstructive pathology, of minor incidence, increases with the age. It is described the case of a 67-yr-old patient who was admitted in the Nephrology Service because of abrupt decline of the renal function. Among the initial symptoms, he presented arterial hypertension (190/90) and preserved diuresis. Blood analysis: urea 199 mg/dl, creatinine 7.7 mg/dl, without proteinuria. Sonography reported a bilateral ureteral hydronephrosis with simple cyst of possible ischemic origin. In view of the absence of previous biochemical data of renal failure, we considered possible reasons which start with an acute pattern. In initial evaluation, pre-renal etiology was not seen (high blood pressure, right cardiac systole function). The absence of prostatic syndrome and sonography discovery did not justify a diagnosis of urinary tract obstruction. Finally, abdominal-pelvic scan showed a periaortic retroperitoneal mass which included both ureters and appeared to trigger the obstruction. Combined efforts were pursued with the Urology Service, which implanted a bilateral "double J" catheter and later operated surgically on the patient, carrying out an alternating ureterolysis of both ureters. The biopsy manifested a retroperitoneal fibrosis, and the renogram showed a residual renal function of 20% in the right kidney and 80% in the left kidney. Due to the failure of the previous measures and as a last therapeutic recourse when one year had passed from the diagnosis, a continuous regimen with tamoxifen (anti-estrogen drug) in dose of 20 mg/dl each 12 hours was started, which began a progressive remission in the size of the observed mass by scan (CT) and magnetic resonance (MR). The treatment was completed during 12 months and in this time, the levels of blood urea nitrogen and creatinine were reduced gradually too. Finally, at the end of the treatment, the magnetic resonance demonstrate the complete disappearance of the fibrosis.
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PMID:[Acute obstructive renal failure secondary to retroperitoneal mass]. 1521 69

We report a case of non-Hodgkin lymphoma (NHL) with acute renal failure. A 62-year-old man was admitted to our hospital on March 8, 2002 with leg edema and dyspnea on effort. About 3 weeks before admission, he was found to have slightly high serum creatinine (Cr) and high lactate dehydrogenase (LDH) levels by another home doctor. Physical examination revealed anemic conjunctivae and leg edema, but the urinary volume was preserved. Blood examination showed high BUN (64 mg/dl) and Cr levels (3.91 mg/dl). Urinary analysis showed proteinuria (1.05 g/day) and high BMG (14,434/microg/day) and NAG (4.55 U/day) levels, suggesting severe tubulointerstitial injury. On ultrasonography of the kidney, the bilateral kidneys showed marked swelling without hydronephrosis. To investigate the genesis of renal failure, we performed a renal biopsy. The specimen showed normal glomeruli, but a large number of cells infiltrated in the tubulointerstitial area with normal tubulointerstitial structure. The cells stained positively with anti-leukocyte antigen and L26 (B cell marker), and negatively with cytokeratin and UCHL-1 (T cell marker). These findings indicate that the interstitial cells were non-Hodgkin lymphoma with B cell diffuse large cells. Chemotherapy was performed with VAD (vincristine sulfate, doxorubicin hydrochloride, dexamethasone) considering his renal dysfunction. To avoid tumor lysis syndrome after chemotherapy, hemodialysis was performed on days 1-4 after the initiation of chemotherapy. After a series of chemotherapy, the urinary volume increased and serum Cr levels decreased to 2 mg/dl. After additional therapy with 4 courses of CHOP, he improved and was discharged on day 180 after admission.
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PMID:[Case of non-Hodgkin lymphoma with acute renal failure successfully treated with chemotherapy]. 1564 40


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