Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0033687 (proteinuria)
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Autosomal dominant polycystic kidney disease (ADPKD) is the commonest hereditary nephropathy. We collected 92 cases in VGH. Diagnosis was confirmed by intravenous pyelogram, renal sonogram, or renal CAT scan. The incidence of having positive family history was just only 28.3%. Patients were diagnosed at the mean age of 54 +/- 11 years (26-74 years). The common clinical findings were hypertension (73.9%), abdominal mass, proteinuria, anemia, azotemia, abdominal or back pain and pyuria in orders. Hypertension might present in the early stage with normal renal function (near 40%). Polycystic liver was the major extrarenal lesion (57.6%), but the incidence of abnormal liver function was only 10.1%. Enlarged kidneys were not always palpable, even at end stage of renal function (mean age 56 +/- 9 years, 89.4% kidney palpable). Patient's urine amount was usually nonoliguric, even in uremic stage (82.9%). Sepsis was the first cause of death. Cardiovascular disease and uremia were followed in sequence. Their expired mean age was 61 +/- 7 years (53-74 years).
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PMID:[Autosomal dominant polycystic kidney disease clinical analysis in VGH--Taipei]. 217 45

Factors affecting the progression of autosomal dominant adult type polycystic kidney disease were analysed in 27 cases. The patients ages ranged from 10 to 74 (mean 44) years old and the serum creatinine values were within the normal limits except two cases, in which the values were 2.4 mg/dl and 2.1. They were followed for from 2 years to 12 years (mean 5.6 years). During the followup period, 6 cases showed elevation of the serum creatinine values and hemodialysis was necessary in 4 cases. There was a tendency of higher morbidity rate of hypertension, proteinuria, hematuria and pyuria in the cases with decreased renal function. These factors may have participated in the progression of polycystic kidney disease. Cystic fluid analysis was performed by percutaneous puncture of more than hundred cysts in 27 cases. The results showed that the cystic fluid components of most cysts of the well functioning kidneys were similar to those of serum values: so-called proximal cysts. On the other hand, in the cases with decreased renal function, there were many cysts with lower sodium concentration and higher creatinine values: so-called distal cysts. The results suggest that the existence of so-called distal cysts may indicate poorer prognosis. DMSA renoscintigraphy was useful for followup polycystic kidney patients because of the uptake of the radionuclide was decreased before rising the serum creatinine value. In 6 cases, the cysts were instilled with 95% ethanol. Followup ultrasonography and DMSA renoscintigraphy revealed a marked reduction of the cystic size and an improvement of DMSA uptake.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Investigation of factors affecting the progression of polycystic kidney disease and effects of percutaneous reduction of cystic size]. 260 Dec 21

A 6-year-old girl with cerebral palsy developed conscious disturbance and generalized convulsion after one-hour hot herb drug bath. Physical examination on admission revealed rectal temperature 41 degrees C, hot skin, respiration 46/min, regular heart beat 98/min, BP 130/60 mmHg, Glascow coma scale 4 (E2M1V1), soft and flat abdomen, no hepatosplenomegaly, no skin rash, no focal neurological sign, increased generalized muscle ton. Laboratory data showed CBC: WBC 20400 cumm (Neutrophils 31%, Lymphocytes 69%), Hb 11.6gm%, ESR 11 mm/hr, arterial blood gas: PH 7.077, PO2 43mmHg, PCO2 57.1mmHg, HCO3- 16 mEq/L, BE-11.5mEq/L, serum sodium 143 mEq./L, potassium 5.2 mEq/L, chloride 101 mEq/L, free calcium ion 3.8mg%, GOT 63IU/L, GPT 263 IU/L, amylase 193 IU/L, alkaline phosphatase 388 IU/L, LDH 1245 IU/L, CPK 677 IU/L, total bilirubin 0.8 mg/dl, direct type 0.1 mg/dl, BUN 18 mg/dl, Glucose 35 mg/dl. Urinalysis revealed proteinuria( ) trace hematuria and pyuria, but no cast. Lumbar puncture is within normal limits. Bacteriology including blood and CSF are normal. Multiple organ failure was noted at that time. Intensive cooling methods were performed including central and peripheral cooling. We used luminal and valium to control the seizure. Condition didn't improve. Afterwards cardiopulmonary arrest developed. Patient expired 8 hours after admission despite of resuscitation. Heat stroke in infancy and childhood is different from that in adulthood. The predisposing factors are high ambient temperature, dehydration, very young baby, sweat gland dysfunction, or ectodermal dysplasia. Definition of heat stroke includes 1) rectal temperature above 41 degrees C, 2) behavioral change, 3) warm skin, wet or dry.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Status epilepticus induced by prolonged immersion in hot herb bath: report of one case]. 263 19

Twenty-seven patients presenting to the Royal Melbourne Hospital between 1968 and 1988 with mesangiocapillary glomerulonephritis type II with intramembranous dense deposits (dense-deposit disease, DDD) are analyzed. Patients were divided into two groups on the basis of whether renal function deteriorated (14 patients) or remained stable (13 patients). At presentation or during the course of the disease, heavy proteinuria, macroscopic hematuria, and high quantitative urinary red cell or white cell counts characterized patients with progressive disease. Patients with crescents on their initial renal biopsy or with large numbers of polymorphs in glomerular capillaries corresponding with sterile pyuria were more likely to have deterioration of renal function. The average time from onset of symptoms to development of end-stage renal disease was over 16 years. The patient's clinical course could not be anticipated by serum complement profiles, the presence of C3 nephritic factor, or partial lipodystrophy. Pregnancy did not affect the course of the disease. Six patients underwent renal transplantation and the disease recurred on renal biopsy in four. However, only two individuals lost renal allografts due to recurrent DDD.
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PMID:Mesangiocapillary glomerulonephritis type II (dense-deposit disease): clinical features of progressive disease. 265 60

Isolated proteinuria (i.e. without hematuria and/or pyuria) is a frequent finding. Simple methods exist which make it easy to classify into various subtypes. Guidelines are suggested mainly to avoid useless and potentially dangerous investigations.
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PMID:[Isolated proteinuria: practical approach]. 274 15

The ability of a urinalysis reagent strip to predict the presence of formed elements in the sediment was evaluated. The sensitivity of individual biochemical analytes varies from 0.51 to 0.85; however, the combined sensitivity of positive reactions for either protein, nitrite, leukocyte esterase, and/or hemoglobin is 0.95. Leukocyte esterase activity becomes detectable at a concentration of 15 white blood cells per high-power field (WBCs/HPF). Proteinuria is nonspecifically related to pyuria and detects a minimum concentration of 6 WBCs/HPF, and the hemoglobin reaction detects 6 red blood cells/HPF. Most false negative reactions are associated with bacteriuria. A positive chemical reagent strip test can be safely and effectively used as a prerequisite for routine urine microscopic examination.
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PMID:Eliminating unnecessary urine microscopy. Results and performance characteristics of an algorithm based on chemical reagent strip testing. 230 Dec 96

Anorexia nervosa and bulimia are occurring with increased frequency among adolescents and preadolescents. To determine the range and severity of medical complications encountered in younger anorectic and bulimic patients, we reviewed the medical records of 65 adolescents and preadolescents, aged 10 to 20 years, who were observed in the Eating Disorders Clinic of the Children's Hospital at Stanford. Significant medical instability was present in the majority of our patients. A total of 55% of anorectic patients and 22% of bulimic patients required hospitalization for medical reasons during the study period. Cardiovascular abnormalities were frequent, including bradycardia, prolonged corrected QT intervals, dysrhythmias, and marked orthostatic pulse and BP instability. Hypothermia, with temperatures less than 35.5 degrees C, was common. Renal abnormalities included pyuria, hematuria, and proteinuria. Electrolyte derangements occurred in patients who vomited or purged. Hypokalemia was most common, but hypocalcemia, hypomagnesemia, and hypophosphatemia were also noted. The majority of our pediatric patients with eating disorders had evidence of physiologic derangement requiring medical intervention. The need for adolescents and preadolescents with eating disorders to receive ongoing medical monitoring in concert with psychiatric treatment and the need for therapists and medical practitioners to become familiar with the potential medical sequelae of eating disorders are underscored by our data.
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PMID:Medical complications of eating disorders in adolescents. 316 64

Idiopathic hypercalciuria (IH) in adults is recognized as a cause of urolithiasis. If IH is symptomatic, the symptoms are hematuria, renal colic, or obstructive uropathy with or without infection. In children, IH has been linked to the spectrum of urinary symptoms including hematuria, pyuria, dysuria, recurrent urinary infections, abdominal or suprapubic pain, proteinuria, and the frequency-urgency syndrome. Hematuria may appear prior to the appearance of stones, and thiazide therapy appears to prevent stone formation by decreasing urinary calcium excretion. This report describes an older adolescent with hematuria and flank pain. His urinary chemistry values were not consistently typical of IH, but a thiazide trial with withdrawal challenge was diagnostic. His case is remarkable because, though essentially an adult, his disease was typical of prepubertal disease. Adolescents with unexplained urinary symptoms should be evaluated for IH. The urinary calcium-creatinine ratio may not be elevated, and timed urinary calcium may be equivocal. In some cases a thiazide trial may be valuable and cost effective.
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PMID:Atypical idiopathic hypercalciuria in an adolescent. 318 67

Renal manifestations of sarcoidosis are rare. In addition to calcium nephropathy, granulomatous interstitial nephritis and glomerulo-nephritis (GN) account for most cases. The latter two manifestations are described in 4 patients and in a detailed review of the literature. In comparison to a nonselected population of sarcoidosis patients, granulomatous interstitial nephritis is found more frequently in male patients above 40 years of age; it is associated more frequently with other extrathoracic manifestations of sarcoidosis; and it causes renal insufficiency of varying degree, which is at least partially reversible with steroid therapy. Predominant findings are silent microhematuria, sterile pyuria, mild proteinuria and a variety of tubular functional disorders. Glomerulonephritis (39 observations) has been described with increasing frequency in sarcoidosis. Because of the well known immunological abnormalities of sarcoidosis, frequent association of sarcoidosis with GN could be expected but this association has not yet been proven statistically. Sarcoidosis-associated GN includes a variety of histological forms, viz. membranous, proliferative and sclerosing GN. Glomerulonephritis may appear before sarcoidosis. Conversely, both diseases may appear simultaneously, or GN may follow all other manifestations of sarcoidosis with a latency period of many years.
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PMID:[Renal manifestations of sarcoidosis]. 328 4

Renal involvement is well described in patients with mucocutaneous lymph node syndrome (MCLNS), or Kawasaki disease and is manifested by mild azotemia, hematuria, pyuria or cylinduria, and more often, proteinuria. Renal morphology during the acute stages of the illness has never been reported. In this paper we describe the renal histopathologic changes in a child with MCLNS. The glomerular histopathologic findings suggest immune complex damage to the kidney as a possible mechanism of nephrotoxicity in MCLNS. Presence of kidney lesions, which speak in favor of the injurious role of immune complexes in MLCNS may be relevant to the understanding of the pathogenesis of the vascular lesions that are characteristic of this disease.
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PMID:Renal histology of mucocutaneous lymph node syndrome (Kawasaki disease). 328 6


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