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)

The course of disease of a patient with membranoproliferative glomerulonephritis and partial lipodystrophy is described. The case is further characterized by a deficiency of C3 and C3- activator, by normal values of C4, by evidence of the nephritogenic factor, by raised fibrin degradation products and by an unselective proteinuria. The course of the glomerulonephritis runs parallel to a pronounced susceptibility to infection (at first varicella, tonsillitis and measles, later pneumonia, meningitis, encephalitis and hepatitis). On account of a nephrotic syndrome and an initative impairment of the renal function, a cytostatic treatment was begun, which although raising the C3 level did not influence the further course of the disease. As the patient has a healthy identical twin sister without lipodystrophy, who shows no reduction in C3 and no nephritogenic factor, this case proves that these diseases are acquired and not genetically determined.
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PMID:Membranoproliferative glomerulonephritis with partial lipodystrophy: discordant occurrence in identical twins. 12 86

A case of systemic lupus erythematosus (SLE) with benign intracranial hypertension (BIH) is reported. A 41-year-old male with a history of SLE starting in 1982 was admitted to our hospital in December 1989 because of headache and vertigo. Laboratory examinations on admission showed proteinuria, mild anemia, and positive antinuclear and anti-Sm antibodies. No abnormal findings except high pressure of 350 mmH2O were observed in his cerebrospinal fluid (CSF). Fundoscopic examinations showed marked bilateral papilledema and retinal bleeding. Brain CT, MRI and angiography revealed diffuse brain edema without space occupying lesion and cerebrovascular diseases. Because there were no diseases such as endocrinological disorders, severe anemia, and no history of the administration of drugs which might cause intracranial hypertension, the diagnosis of BIH was made. Subsequently, he was treated with intravenous methylprednisolone therapy and osmotic diuretics and his clinical symptoms and pressure of CSF gradually improved. The decrease of CSF adsorption was observed with RI cisternography in our case. Psychosis, seizures and meningitis are common CNS manifestations in SLE patients. But BIH is very rare and its cause is unclear. Only 17 cases of SLE with BIH have been reported. The pathogenesis and treatment of BIH in SLE patients were discussed in this paper.
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PMID:[Systemic lupus erythematosus associated with benign intracranial hypertension: a case report]. 160 19

We have retrospectively evaluated 24 sepsis episodes caused by viridans streptococci in 23 neutropenic children during a 21 months period at the Pediatric Hematology Unit of St. Louis Hospital. The underlying malignancies included acute lymphoblastic leukemia, acute non lymphoblastic leukemia, aplastic anemia and solid tumor. In 17 children neutropenia, defined as a neutrophil count of less than 500 per cubic millimeter, was caused by cytotoxic chemotherapy. For 6 other children neutropenia was consequential to pretransplant treatment regimen for autologous bone marrow transplantation including cytotoxic chemotherapy and total body irradiation. All patients had a silicone rubber atrial catheter. In 9 patients sepsis was associated only with fever for less than 48 hours. In 5 other children fever was prolonged more than 72 hours in spite of specific antimicrobial therapy. No other organism was isolated. In 10 patients, however, the infectious syndrome was severe and the features included cardiac failure (7 patients), pneumonia (7 patients) resembling adult respiratory distress syndrome, encephalopathy (3 patients) without meningitis and proteinuria, 7 of these patients needed a management in a pediatric intensive care unit and 2 died in spite of adapted antibiotics. Streptococci were isolated in blood cultures in 23 children.
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PMID:[Frequency and severity of systemic infections caused by Streptococcus mitis and sanguis II in neutropenic children]. 278 Jan 2

The predictive value of a number of clinical and laboratory variables for the mortality of 148 patients with systemic lupus erythematosus (SLE) with a mean observation period of 8 years and a 10-year-survival of 80 per cent was calculated by means of differentiated survival rate analyses and stepwise regression analyses. The predictive power of several variables increased if the calculations were based on deaths caused by SLE rather than on the total mortality rate. The survival rate decreased after 1973 because a diagnosis of SLE was made in some patients with terminal disease who would have remained without a diagnosis before that time. The causes of death and the treatment were identical before and after 1973. The presence of a high number of diagnostic ARA criteria within the first year of observation was a predictor of decreased survival. Severe but non-fatal infections (meningitis, septicemia, pneumonia) significantly reduced the survival rate. Patients with proteinuria and azotemia, within the first 2 years of observation, had a 10-year-survival of 70 per cent. The survival of patients with CNS manifestations was not significantly reduced. The butterfly rash and the presence of lymphopenia were predictors of decreased survival, whereas the presence of DNA antibodies had no predictive value for survival.
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PMID:Systemic lupus erythematosus. Follow-up study of 148 patients. II: Predictive factors of importance for course and outcome. 358 95

The study of serum from a patient with C2 deficiency is described. The patient had an episode of pneumococcal meningitis at 5 mo of age with seizures and transient hemiparesis and apparent purpuric skin lesions. He was first admitted to the University of Minnesota Hospitals at 10 yr of age following the discovery of proteinuria accidentally by his mother. Since then he has been admitted repeatedly to this hospital with numerous clinical findings including arthralgia, recurrent abdominal pain, proteinuria, membranous nephropathy, malar butterfly rash, seizures, personality aberrations, and recurrent fever. In June 1971, the patient developed positive DNA and DNP antibodies and positive LE cells. When the C profile was studied before and after recognition of lupus, C1q, C1s, and C4 dropped. C3 levels were elevated as were C5, C6, and C7, C3 proactivator had been reduced in the patient even before he developed lupus. Also because of a traumatic renal biopsy leading to a perirenal hematoma, he required surgery and a blood transfusion. 1 h after blood transfusion, a C2 titer of 23 hemolytic units was detected. Almost immediately levels of C3, C5, C6, and C7 dropped, C8 and C9 remained elevated. The addition of C2 from normal blood permitted dramatic activation of C3. These findings support the view that the rare deficiency in production of C2 predisposes to serious susceptibility to infection, vascular and mesenchymal disease as well as to renal disease and a lupus syndrome.
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PMID:C2 deficiency. Development of lupus erythematosus. 457 55

The protean manifestations of Legionnaires' disease are described in an analysis of 12 sporadic cases. Two forms of the disease have been delineated. One variant (Group A) consisted of six patients who had a mild form of non-progressive pneumonia with minimum extra-pulmonary involvement. Six patients (Group B) were differentiated by rapidly progressive pulmonary infiltrates, severe hypoxia and respiratory failure, plus a higher frequency of band neutrophils and extra-pulmonary manifestations. Particularly notable were evidence of severe myositis (elevated creatinine phosphokinase and lactate dehydrogenase), anaemia, and neurological findings which included alterations in the sensorium, meningitis, and convulsions. Cerebrospinal fluid (CSF) abnormalities were seen frequently in patients with neurological manifestations, and necropsy findings in one patient suggested that the Legionnaires' bacillus was capable of producing a fatal leucoencephalitis. Renal findings included haematuria, proteinuria and oliguric renal failure. Hepatic transaminases (SGPT, SGOT) were elevated in six patients and serum bilirubin was abnormal in five. Alkaline phosphatase values were normal to minimally elevated. The gastrointestinal symptoms commonly considered to be a frequent initial manifestation of Legionnaires' disease were rare in this series. Recommendations for instituting empirical therapy, based upon recognition of a clinical syndrome which should suggest the diagnosis of Legionnaires' disease, are included.
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PMID:The protean manifestations of Legionnaires' disease. 663 Oct 29

BACKGROUND: Leptospirosis (LS) and Hantavirus (HV) infection have many common clinical manifestations, including acute renal failure. In as much as clinical experience with these diseases is quite limited in urban centers, we identified cases of Leptospira and HV-induced acute renal failure and compared the clinical course and evolution of these diseases. METHODS: Patients hospitalized with acute renal failure, suspected to be caused by infection, were retrospectively studied over a 13-year period (1985-1998). Based on pertinent clinical data and positive serology, a total of 26 patients were included in the study, 17 patients with LS and 9 patients with HV infection. RESULTS: Both diseases presented as flu-like syndromes with high fever. In LS, 2 patients presented with acute meningitis and died soon after admission. Symptoms and signs from other organs began after the fifth day of illness. Jaundice occurred in 71% of LS patients but not in HV. Hemorrhagic phenomena occurred in both diseases but affected predominantly patients with icteric LS. Anuria or oliguria occurred in 76% of patients with LS and 78% with HV infection. Laboratory studies demonstrated minor transaminase elevations in all patients with LS and in 44% with HV. Hypoprothrombinemia or thrombocytopenia was uncommon, although disseminated intravascular coagulation (DIC) developed in 2 patients that had icteric Ls and major bleeding. Nephropathy was associated with haematuria in 71% with LS and all patients with HV. Proteinuria was evident in 35% of patients with LS compared to 78% with HV. Abnormal chest radiographs were seen in 24% with LS and in 33% with HV. In both diseases, aggressive supportive treatment was given, including hemodialysis or peritoneal dialysis in 4/26 patients. Of the 26 patients, 22 survived and four died. The latter all had LS, and the causes of death were meningitis and DIC with multiple organ failure. Follow-up, after 6 months, showed that renal function had returned to normal in the 22 survivors. CONCLUSION: It is important to include LS and HV infection in the differential diagnosis of acute renal failure. Both diseases present with flu-like symptoms and may be complicated by thrombotic microangiopathy with hemorrhagic phenomena and hepatic and pulmonary involvement. Jaundice should alert the physician to icteric LS, a severe disease associated with significant mortality that requires antimicrobial treatment.
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PMID:Acute renal failure caused by leptospirosis and Hantavirus infection in an urban hospital. 1206 23

The purpose of this study was to summarize the clinical findings in 40 dogs with systemic hypersensitivity reactions associated with the administration of potentiated sulfonamides. Dogs ranged from 6 months to 14 years of age, with a mean of 5.7 +/- 3.2 years. Spayed female dogs were overrepresented (24 of 40, or 60% of the dogs), as were Samoyeds (3 of 40; 8%) and Miniature Schnauzers (5 of 40; 13%). Mean dosages of potentiated sulfonamides were 47.0 +/- 14.9 mg/kg/d (range, 23.4-81.4 mg/kg/d). The time from the 1st administration of the drug to the onset of the clinical signs of hypersensitivity ranged from 5 to 36 days, with a mean of 12.1 +/- 5.9 days. There was no relationship between either the dosage or type of sulfonamide given and the time to the onset of the clinical signs. Fever was the most common clinical sign observed (55% of the dogs); thrombocytopenia was 2nd (54%), and hepatopathy (28%) was 3rd. Neutropenia, keratoconjunctivitis sicca (KCS), hemolytic anemia. arthropathy, uveitis, skin and mucocutaneous lesions, proteinuria, facial palsy, suspected meningitis, hypothyroidism, pancreatitis, facial edema, and pneumonitis were also observed in some patients. Of 39 dogs with adequate follow-up, 30 (77%) recovered, whereas 8 (21%) either died or were euthanized, and 1 recovered clinically but had persistent increases in alanine aminotransferase (ALT) activity. Dogs with hepatopathy generally had a poorer prognosis (46% recovery) than dogs without hepatopathy (89% recovery; P = .0035). Sixty-three percent of the dogs with thrombocytopenia recovered, compared to 90% of the dogs without thrombocytopenia (P = .042). Recovery was not associated with sex, age, breed, or type of sulfonamide administered.
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PMID:Clinical findings in 40 dogs with hypersensitivity associated with administration of potentiated sulfonamides. 1452 30

A 36-year-old primigravida with a history of temporal lobe epilepsy presented at 25 weeks of pregnancy with generalized tonic clonic seizures. The clinical picture was confused with eclampsia because of rising blood pressure and proteinuria. Clinical investigations, which included a lumbar puncture, were carried out to rule out an infective cause for the seizures. A computed tomography of the brain was performed for evidence of intracranial hemorrhage. The patient was intubated and ventilated in the intensive care unit. The labile blood pressure settled in 2 days, and the transient heavy proteinuria also resolved after 3 days. Eclampsia would have warranted operative delivery of the preterm fetus with the attendant problems of prematurity. Delivery would have been hazardous in such an acutely unwell patient. The management also would have required magnesium sulfate with its potential for toxicity. Transient proteinuria may occur in status epilepticus. The blood pressure can be labile during epileptic seizures and, in the absence of an intracranial hemorrhage, generally settles without treatment after control of the seizures. This case highlights the importance of differentiating eclampsia in a patient with known epilepsy that may also mask other disease entities such as intracranial hemorrhage, meningitis, or encephalopathy. We have also discussed the importance of various signs associated with eclampsia and their clinical significance. The differential diagnosis of seizures in pregnancy are broad as symptoms of the various disease entities including eclampsia, intracranial hemorrhage, status epilepticus, meningitis, stroke overlap creating a dilemma in an acute emergency. We present a case whereby the clinical picture of status epilepticus was confused with eclampsia because of the presence of a rising blood pressure and proteinuria.
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PMID:Proteinuria in status epilepticus or eclampsia; a diagnostic dilemma. 1949 73

Recurrent malignant gliomas (RMGs) are difficult to control, and no standard protocol has been established for their treatment. At our institute, we have often treated RMGs by tumor-selective particle radiation called boron neutron capture therapy (BNCT). However, despite the cell-selectivity of BNCT, brain radiation necrosis (BRN) may develop and cause severe neurological complications and sometimes death. This is partly due to the full-dose X-ray treatments usually given earlier in the treatment course. To overcome BRN following BNCT, recent studies have used bevacizumab (BV). We herein used extended BV treatment beginning just after BNCT to confer protection against or ameliorate BRN, and evaluated; the feasibility, efficacy, and BRN control of this combination treatment. Seven patients with RMGs (grade 3 and 4 cases) were treated with BNCT between June 2013 and May 2014, followed by successive BV treatments. They were followed-up to December 2017. Median overall survival (OS) and progression-free survival (PFS) after combination treatment were 15.1 and 5.4 months, respectively. In one case, uncontrollable brain edema occurred and ultimately led to death after BV was interrupted due to meningitis. In two other cases, symptomatic aggravation of BRN occurred after interruption of BV treatment. No BRN was observed during the observation period in the other cases. Common terminology criteria for adverse events grade 2 and 3 proteinuria occurred in two cases and necessitated the interruption of BV treatments. Boron neutron capture therapy followed by BV treatments well-prevented or well-controlled BRN with prolonged OS and acceptable incidence of adverse events in our patients with RMG.
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PMID:Boron Neutron Capture Therapy Combined with Early Successive Bevacizumab Treatments for Recurrent Malignant Gliomas - A Pilot Study. 3046 50


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