Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0033687 (proteinuria)
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HELLP syndrome continues to be a clinical entity of difficult diagnosis. Weinstein first defined it in 1982 giving the practicing obstetrician a sequence of useful initials (H = hemolysis; EL = elevated liver enzymes; LP = low platelets). Since then a lot has been written and it has become clear that the syndrome is a form of severe preeclampsia. The American College of Obstetrics and Gynecology does not include HELLP in the description of severe pre-eclampsia as such but does accept each of its components as being part of severe pre-eclampsia. The case presented deals with a 33 year old white female, admitted at 27 weeks gestation with nausea, epigastric pain resembling acute abdomen, nose bleeding and mild hypertension. The analysis revealed an abnormal liver profile with elevated GOT, GPT and LDH, heavy proteinuria (14.4 g/day), decreased platelet count (92000/mm3) and elevated total bilirubin. Pregnancy was terminated by cesarean section 24 hours after admission because the patient's condition was deteriorating. Obviously in pre-eclampsia/eclampsia there is a systematic injury to all tissues. Proof of this is the hypertension as a consequence of vascular spasm and proteinuria due to glomerular injury. In HELLP the sequence of events is probably altered; hepatic injury precedes vascular and renal injury of conventional preeclampsia. The syndrome results from many clinical and pathological symptoms derived from endothelial microvascular injury which determine a rapid platelet activation causing vascular spasm, platelet aggregation and further endothelial injury through a feedback mechanism.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Massive proteinuria and HELLP syndrome]. 130 8

This report is on a 35-year-old II-para (status post-Caesarean Section due to breech presentation, at that time normal pregnancy) progress, who was hospitalized with hypertension and proteinuria during the 40th week of pregnancy. Both symptoms occurred initially three days before hospitalization. Blood pressure was within the high normal range (140/90 mmHg) as a result of medication with Dihydralazine (50 mg/die). After induction of labour with prostaglandin (PGE2), the patient delivered normally, and the highest blood pressure measured was 140/90 mmHg, following a subsequent curettage under general anaesthesia, which had to be performed due to incomplete deliver of the placenta. Two hours post delivery, sudden epigastric pain occurred, followed by nausea and vomiting. Blood chemistry showed the development of a severe post-partal HELLP-Syndrome with acute renal failure. The case demonstrates, that the life threatening picture of the HELLP-Syndrome may develop without preexistent severe hypertension or proteinuria. For this reason a post-delivery screening of blood chemistry should be mandatory in cases of severe epigastric or right-upper-quadrant pain.
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PMID:[HELLP syndrome--postpartum]. 174 78

Ten women with severe pre-eclampsia, i.e. a blood pressure greater than or equal to 150/110 mmHg or 140/90 mmHg and proteinuria greater than 3 g/24 h were, after initial antihypertensive treatment, centrally monitored with a pulmonary artery catheter (Swan-Ganz). All had been normotensive in early pregnancy. Mean age was 29 years (range 23-37). Mean gestational age upon admission was 29 weeks (range 23-36) and 7 of the women were nulliparous. Nine of the 10 patients had subjective symptoms, e.g. headache and/or epigastric pain. All were considered in need of intensive care. Two patients were found to have an abnormal coagulation and liver function. All patients had normal serum creatinine values despite proteinuria. Hypertension was treated with dihydralazine and/or labetalol. Volume substitution was carried out with plasma and albumin. The women could be divided into two groups: 5 patients where progress of the disease despite therapy led to delivery within 24 h, and 5 patients whose diastolic blood pressure could be stabilized around 100 mmHg after treatment and pregnancy could be prolonged by 5-13 days. Common for all patients was a hyperkinetic circulation with an increased cardiac output despite a variety of central pressures. Invasive monitoring of central pressures with a Swan-Ganz catheter demonstrated that the clinical status could be stabilized and the pregnancy prolonged in 5 of the 10 women with severe pre-eclampsia. The variety of the central hemodynamic values illustrates clearly that treatment has to be individualized regarding antihypertensive medication, fluids and diuretics.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Hemodynamic measurements with Swan-Ganz catheter in women with severe proteinuric gestational hypertension (pre-eclampsia). 192 95

This study reviews liver disease in toxemia of pregnancy based on 102 cases submitted to the Armed Forces Institute of Pathology. The common clinical features were right upper quadrant and epigastric pain, nausea, vomiting, and elevation of the serum transaminases. Jaundice occasionally developed. These occurred in severe preeclampsia or eclampsia and their cause was usually recognized. However, hepatic symptoms and signs did result in inappropriate diagnoses and misdirected therapy. Such confusion occurred when these were the initial problems confronting the clinician in women presenting with advanced toxemia due to poor prenatal care. They were also likely to be misleading when other more classic parameters, such as blood pressure and proteinuria, were only midly abnormal. Central nervous system complications were the common cause of death but liver disease could be partially or wholly responsible. Extensive periportal lesions, hepatic hematomas, spontaneous rupture, and infarction all contributed to hepatic injury and to morbidity. Fibrin deposition, hemorrhage, or both in the periportal areas was characteristic of the histopathology. Scanning electron microscopy validated this spectrum of change. A toxemic vasculopathy related to severe vasospasm in the hepatic arterial circulation may be responsible.
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PMID:Liver disease in toxemia of pregnancy. 378 23

A 51-year-old man with extramedullary plasmacytoma of the stomach is presented. He complained of epigastralgia. Barium meal revealed 2 submucosal tumors in the gastric body. Laboratory investigations showed neither hypergammaglobulinemia nor proteinuria. Bone X-ray examinations were not remarkable. There was tumor invasion to the pancreas and metastasis to the perigastric lymph nodes. Proximal gastrectomy was performed, and the patient died 5.5 years after the operation. Histologically, the proliferation of atypical plasma cells with eccentric nuclei was seen. Those cells invaded all layers of the stomach. The tumor cells were pyroninophilic and contained Russel bodies. Immunohistochemical study using the immunoperoxidase technique demonstrated IgG and lambda-light chains in the tumor cells.
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PMID:[A case of extramedullary plasmacytoma of the stomach]. 642 3

Renal artery thrombosis after blunt trauma presented without other injury, without external signs of trauma, and without hematuria in the case reported. Review of 65 cases from the literature showed that flank and epigastric pain and proteinuria are usually present. Renal artery thrombosis following blunt trauma has usually been diagnosed too late to salvage the kidney. Nephrectomy is performed for ileus, fever, and pain caused by the necrotic kidney, or for hypertension. Ideally, rapid diagnosis by intravenous pyelogram and arteriography and early surgical intervention should allow revascularization and renal salvage before permanent parenchymal damage has occurred. The cases reviewed showed that successful revascularization without hypertension could be achieved 12 hours after injury. Patent small collateral vessels as well as incomplete or gradual renal artery occlusion may prolong renal salvage time.
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PMID:Renal artery thrombosis following blunt trauma. 739 14

The syndrome of haemolysis, elevated liver enzymes and low platelet count (HELLP-Syndrome) is a severe form of preeclampsia and eclampsia. The clinical course is characterized by right upper quadrant and epigastric pain, hypertension, proteinuria and edema. Maternal and neonatal morbidity are high. The underlying cause for this pregnancy-related syndrome is still unclear. As soon as a reliable diagnosis is established handling of patients suffering from HELLP-Syndrome is ambivalent: Immediate termination of pregnancy, however, poses a problem at early gestational age. Therefore some authors have advocated a conservative management. At our department active management and delivery by Caesarean section as soon as possible has gained acceptance in the past 5 years. We report our experience with 23 patients over a 12 year period, and with 4 patients from the intensive care unit (N = 27). Mean gestational age was 33.5 weeks (+/- 4.8) and the mean birthweight was 1922.5 g (+/- 971.5). 19 patients were delivered by Caesarean section. Most complications were based on a delayed delivery and subsequent deterioration of maternal condition. Reduction of the time interval between establishment of diagnosis and termination of pregnancy (1980-1985-3 days; 1986-1992-12 hours) resulted in a better outcome. We recommend intensive laboratory screening and exact clinical examination since missed or delayed diagnosis as well as delayed delivery are life threatening for mother and child. Only prompt delivery yields an improvement of prognosis.
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PMID:[Obstetric management of patients with HELLP syndrome]. 836 86

A 22-year-old woman began to have the symptoms of anorexia, high fever, cough and general fatigue from June of 1997. She was admitted in our hospital on Aug. 8th, 1997 for the further detail examination because of pancytopenia and positive antinuclear antibody (ANA). Her laboratory findings and clinical symptoms were compatible with systemic lupus erythematosus (SLE) such as leukopenia, proteinuria, hypocomplementemia, positive ANA, elevated titer of autoantibodies including anti-DNA, anti-Sm, anti-RNP antibodies, polyarthralgia and photosensitivity. The administration of oral prednisolone (40 mg/day) was started on Aug. 15th, 1997 under the diagnosis of SLE. However, she had severe abdominal pain in epigastrium with elevated serum amylase, ascites and dull shape of pancreas tail by CT scan compatible with acute pancreatitis. On Aug. 18th, her general condition was worsening with fever, epigastralgia, abdominal distension, anemia, weak palpation of radial artery, hypotension, tachycardia, shallow breathing and cold sensation on both extremities as shock. In spite of steroid pulse therapy with nafamostat mesilate intraarterial infusion, her condition was not improved. The dose of 50 mg/day of cyclophosphamide was added to the regimen on Aug. 22nd. Then, gradually her condition started to be restored. Anemia, leukopenia, hypocomplementemia continued. Second steroid pulse therapy was done on Sep. 5th. After then, she became better in her clinical symptoms and laboratory data. The dose of PSL was tapered to 15 mg/day and 7.5 mg/day update of Oct. 1998 without the pseudcysts found after pancreatitis. She is a rare case who recovered from severe acute pancreatitis due to SLE itself.
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PMID:[A case of systemic lupus erythematosus associated with severe acute pancreatitis]. 1043 57

HELLP syndrome is a serious, life-threatening form of pre-eclampsia with a typical laboratory triad. The incidence of the disease is reported as being 0.17-0.85% of all live births. There has been, to date, neither reliable early recognition nor effective prevention of HELLP syndrome. As a result of endothelial dysfunction, activation of intravascular coagulation occurs with fibrin deposition in the capillaries and consecutive microcirculation disorders. The disease manifests itself on average between 32-34 weeks' gestation. HELLP syndrome will occur postpartum in up to 30% of the cases. The clinical cardinal symptom of the disease is right upper quadrant pain or epigastric pain accompanied with nausea, vomiting and malaise. In 20% of the cases with HELLP syndrome there is no hypertension and 5-15% of the pregnant patients present a low level of proteinuria or none at all. The early recognition of hemolysis is most sensitively managed by the determination of the serum haptoglobin. The increase of the aspartate transaminase (AST) and the alanine transaminase (ALT) often precedes a decrease in platelets. The course of HELLP syndrome is incalculable. It is universally agreed that a pregnancy from 32-34 weeks should be immediately delivered. Before 32-34 weeks, expectant management is generally possible in a perinatal center. The frequency for a repeated hypertensive disease in pregnancy ranges from 27% to 48%.
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PMID:HELLP syndrome. 1103 96

We report an atypical occurrence of invasive Strongyloides stercoralis infection of the stomach mucosa in an elderly female patient from Bangka Island, northwestern Indonesia. The patient presented with severe epigastric pain, edema of the legs, proteinuria and severe hypoalbuminemia. Gastric and duodenal biopsies found eggs, larval and adult forms present in the superficial mucosa with mild inflammation. The Harada-Mori filter paper culture technique revealed S. stercoralis filariform larvae and free-living adult worms, corroborating the diagnosis. The infection was associated with acute interstitial nephritis. The patient showed rapid and dramatic improvement after treatment with mebendazole.
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PMID:Strongyloidiasis with gastric mucosal invasion presenting with acute interstitial nephritis. 1712 Dec 87


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