Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0012739 (disseminated intravascular coagulation)
8,673 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Urea Cycle Disorders (UCD) is an inborn error of urea synthesis in which ammonium and other nitrogenous precursors of urea accumulate leading to episodic coma and a high mortality rate. Therapy with peritoneal dialysis, essential amino acids or their nitrogen-free analogues has increased survival. The authors report 5 cases of urea cycle disorders, all of whom developed and were rescued from hyperammonemic coma. However, the eventual outcome was quite variable. Argininosuccinate lyase deficiency (ALD) Case 1. A 2 month old male infant, a product of a consanguineous marriage (Suphanburi province); developed poor feeding on day 7, lethargy, convulsion, hepatomegaly and respiratory alkalosis leading to respiratory failure and coma. Hyperammonemia, elevation of glutamic acid and argininosuccinic acid and its anhydrides confirmed the diagnosis of ALD. He is now 9 years old and severely retarded. Case 2. A male infant with history of lethargy, poor feeding on day 3, treated as sepsis and required respiratory support for 6 days; subsequently readmitted at age 2 weeks with vomitting, lethargy, seizure activity and hyperammonemia, and was treated by a local pediatrician in Songkhla province. There was a history of parental consanguinity and he was referred to Siriraj Hospital on day 64 with severe essential amino acid deficiency and acrodermatitis enteropathica with markedly elevated plasma citrulline level. In spite of aggressive treatment; the patient developed sepsis and he expired on day 78. Ornithine transcarbamylase deficiency (OTC) Case 3. An eleven-month-old male infant, the product of a non-consanguineous marriage, developed neonatal onset of hyperammonemia on day 5 after poor feeding, lethargy, hypothermia, seizure, apnea and coma. He was rescued from neonatal hyperammonemic coma on day 9 after aggressive treatment, but expired at eleven months of age after overwhelming sepsis. Case 4. A male infant, sibling of case 3 was referred to Siriraj Hospital on day 8 with hyperammonemia and coma. In spite of intensive genetic counseling given after the birth of their first child with OTC, the couple chose to have another baby without informing any physician. The baby developed vomiting and lethargy on day 2; subsequently hyperammonemia was noted. In spite of aggressive treatment given; hepatic dysfunction, renal failure and disseminated intravascular coagulation defects occurred on day 15. He expired on day 18 after parental permission for discontinuation of all treatment. Argininosuccinate synthetase deficiency (ASS) or Citrullinemia. Case 5. A seven week old female infant, the product of a consanguineous marriage and of Pakistani ethnic origin; developed intermittent vomiting from day 6. Initial diagnoses included ruminations, sepsis and pyloric stenosis for which she was operated on (day 30); however, vomiting continued; subsequently seizures, hyperammonemic coma developed and she was rescued from hyperammonemic coma within 30 hours. Significant elevations of citrulline and L-glutamine were demonstrated. She was discharged in excellent condition to her home in Dubai, the United Arab Emirates.
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PMID:Urea cycle disorders in Thai infants: a report of 5 cases. 1240 52

The case of a healthy 31-year-old woman in the 40th week of second pregnancy is presented. During preparation for an emergency caesarean section, she developed an amniotic fluid embolism (AFE) with unusual and unique features. The acute onset of disease with cardiorespiratory failure with hypotension, tachycardia, cyanosis, respiratory disturbances and loss of consciousness, suggested at first a pulmonary thromboembolism, but the appearance of convulsions led to the diagnosis of AFE. The patient died after 5 days due to an untreatable brain edema. At autopsy, AFE with the usually associated disseminated intravascular coagulation was found in the lungs, brain, left adrenal gland, kidneys, liver and heart. Eosinophilic inflammatory infiltrates were found in the lungs, hepatic portal fields and especially in the heart, suggesting a specific hypersensitivity reaction to fetal antigens. Moreover, intravascular accumulation of macrophages in the lungs also favored a non-specific immune reaction to amniotic fluid constituents.
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PMID:Amniotic fluid embolism with involvement of the brain, lungs, adrenal glands, and heart. 1273 31

A comprehensive understanding of the transport and geochemical processes controlling solutes in clay-rich aquitard confining units is needed to accurately predict the long-term migration of contaminants into the subsurface. To this end, the geochemical and stable isotopic composition of porewaters in the upper 22 m of a thick, unoxidized and nonfractured clay-rich, till aquitard (Sutherland Group) was examined in detail. The aquitard is overlain by about 8 m of oxidized and fractured till (Floral Fm). Concentrations of TDS, SO4(2-), HCO3-, Cl-, Na+, Mg2+, Ca2+ and porewater deuterium were greater in the Floral Fm and decreased with depth through the aquitard. The elevated and seasonably variable solute concentrations in the oxidized Floral Fm were attributed to geochemical weathering and dynamic water movement through fractures. Good fits between measured delta2H, TDS, SO4(2-), Cl- and HCO3- profiles through the aquitard and simulated solute transport profiles were obtained by diffusion (without advection) with transport times of 4-6 ka. The deficiency of geochemical reactions affecting HCO3- and SO4(2-) in the aquitard was supported by delta13C(DIC) and delta34S(SO4) analyses. Geochemical and isotope mass balance modelling (NETPATH) indicated that diffusive mixing with minor calcite dissolution and ion exchange could account for the distribution of Na+, Ca2+ and Mg2+ in the aquitard. Results of this study further suggested that microbiological activity in the aquitard was limited. With minor exceptions, the solute and isotopic profiles, their transport and the controlling geochemical reactions in the Sutherland are similar to those determined at another clay-rich till aquitard, 160 km south of this site, suggesting that geochemical and biological processes in some clay-rich aquitards may have a minimal effect on the migration of dissolved constituents.
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PMID:Transport and geochemical controls on the distribution of solutes and stable isotopes in a thick clay-rich till aquitard, Canada. 1508 80

A 78-year-old Japanese female was admitted to our hospital because of headache. Trismus, opisthotonus and convulsion appeared in the early morning on the second hospital day. She was diagnosed as tetanus based on these symptoms and the history of trauma on her left hand 6 days ago. A piece of bamboo was extracted from her left hand. However, Clostridium tetani was not identified. Thiamylal and mechanical ventilation were initiated combined with Pancronium, Diazepam and tetanus immune globulin. Gabexate Mesilate was also added for disseminated intravascular coagulation syndrome probably associated with rhabdomyolysis. By these treatments, she was healed leaving only a disuse syndrome of her limbs.
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PMID:[A survival case of tetanus complicated with disseminated intravascular coagulation syndrome in the elderly, rescued by mechanical ventilation and administration of thiamylal]. 1571 80

HELLP syndrome is a severe complication of pre-eclampsia characterised by hemolysis, elevated liver enzymes and a low platelet count. It is associated with an increased risk of adverse outcome for both the mother and the fetus. Patients with HELLP syndrome are also at greater risk of pulmonary edema, adult respiratory distress syndrome, abruptio placentae, intracerebral hemorrhage, eclamptic convulsions, disseminated intravascular coagulation, ruptured liver hematomas and acute renal failure. Perinatal mortality is equally high. Before delivery, aggressive obstetric management is directed toward stabilization of the affected organ systems, if possible, and interruption of the pregnancy in the early phase of the accelerated disease progression. Definitive therapy is delivery. Parturients HELLP syndrome often require general anesthesia for Cesarean section delivery. The anesthetic technique is critical for these patients with a high risk of uncontrollable hypertension, bleeding and multiple organ failure. Remifentanil is increasingly used as a very short analgesic agent providing cardiovascular stability in high-risk patients. We report the management of a patient presenting in labor with HELLP syndrome, and describe the successful use of remifentanil as part of the anesthetic technique for her subsequent Cesarean section.
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PMID:General anesthesia with remifentanil for Cesarean section in a patient with HELLP syndrome. 1575 13

Brown spider (Loxosceles genus) venom can induce dermonecrotic lesions at the bite site and systemic manifestations including fever, vomiting, convulsions, disseminated intravascular coagulation, hemolytic anemia and acute renal failure. The venom is composed of a mixture of proteins with several molecules biochemically and biologically well characterized. The mechanism by which the venom induces renal damage is unknown. By using mice exposed to Loxosceles intermedia recombinant dermonecrotic toxin (LiRecDT), we showed direct induction of renal injuries. Microscopic analysis of renal biopsies from dermonecrotic toxin-treated mice showed histological alterations including glomerular edema and tubular necrosis. Hyalinization of tubules with deposition of proteinaceous material in the tubule lumen, tubule epithelial cell vacuoles, tubular edema and epithelial cell lysis was also observed. Leukocytic infiltration was neither observed in the glomerulus nor the tubules. Renal vessels showed no sign of inflammatory response. Additionally, biochemical analyses showed such toxin-induced changes in renal function as urine alkalinization, hematuria and azotemia with elevation of blood urea nitrogen levels. Immunofluorescence with dermonecrotic toxin antibodies and confocal microscopy analysis showed deposition and direct binding of this toxin to renal intrinsic structures. By immunoblotting with a hyperimmune dermonecrotic toxin antiserum on renal lysates from toxin-treated mice, we detected a positive signal at the region of 33-35 kDa, which strengthens the idea that renal failure is directly induced by dermonecrotic toxin. Immunofluorescence reaction with dermonecrotic toxin antibodies revealed deposition and binding of this toxin directly in MDCK epithelial cells in culture. Similarly, dermonecrotic toxin treatment caused morphological alterations of MDCK cells including cytoplasmic vacuoles, blebs, evoked impaired spreading and detached cells from each other and from culture substratum. In addition, dermonecrotic toxin treatment of MDCK cells changed their viability evaluated by XTT and Neutral-Red Uptake methodologies. The present results point to brown spider dermonecrotic toxin cytotoxicity upon renal structures in vivo and renal cells in vitro and provide experimental evidence that this brown spider toxin is directly involved in nephrotoxicity evoked during Loxosceles spider venom accidents.
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PMID:Brown spider dermonecrotic toxin directly induces nephrotoxicity. 1600 84

Previous studies in our laboratories and elsewhere have shown that the fruit of Tetrapleura tetraptera (Taub) (family: Fabaceae) is widely used in African traditional medicine for the management and/or control of an array of human ailments, including schistosomiasis, asthma, epilepsy, hypertension and so on. The present study was designed to investigate the analgesic and anticonvulsant effects of Tetrapleura tetraptera (Taub) fruit aqueous extract (TTE) in mice. Morphine (MPN, 10 mg/kg i.p.), diclofenac (DIC, 100 mg/kg i.p.), phenobarbitone (20 mg/kg i.p.) and diazepam (0.5 mg/kg i.p.) were used, respectively, as reference analgesic and anticonvulsant agents for comparison. T. tetraptera fruit aqueous extract (TTE, 50-800 mg/kg i.p.) produced dose-dependent, significant (p < 0.05-0.001) analgesic effects against thermally and chemically induced pain in mice. Like the standard anticonvulsant agents (phenobarbitone and diazepam) used, T. tetraptera fruit aqueous extract (TTE, 50-800 mg/kg i.p.) significantly (p < 0.05-0.001) delayed the onset of, and antagonized, pentylenetetrazole (PTZ)-induced seizures. Aqueous extract of the fruit (TTE, 50-800 mg/kg i.p.) also profoundly antagonized picrotoxin (PCT)-induced seizures, but only partially and weakly antagonized bicuculline (BCL)-induced seizures. However, the results of this experimental animal study indicate that Tetrapleura tetraptera (Taub) fruit aqueous extract (TTE) possesses analgesic and anticonvulsant properties. These findings lend pharmacological support to the suggested folkloric uses of the plant's fruit in the management and/or control of painful, arthritic inflammatory conditions, as well as for the management and/or control of epilepsy and childhood convulsions in some tropical African countries.
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PMID:Analgesic and anticonvulsant properties of Tetrapleura tetraptera (Taub) (Fabaceae) fruit aqueous extract in mice. 1637 67

Superior sagittal sinus thrombosis (SSST) has been reported to be caused by coagulopathy following oral contraceptive therapy, DIC, infection around the sinus, compression from a tumor, infiltration of tumor, and an inherited deficiency of proteins C and S, but SSST associated with hematological malignancies and L-asparaginase (L-Asp) therapy is rare. We report a case of an adult patient with acute lymphoblastic leukemia (ALL) who developed SSST during the remission induction therapy. A 25-year-old man was admitted with left facial nerve palsy and, following bone marrow aspiration and lumbar puncture, he was diagnosed as having T-ALL with CNS involvement. He received a 1-AdVP regimen as remission induction therapy and intrathecal administration of methotrexate and cytarabine. On day 29, he had a generalized convulsion and SSST was demonstrated by imaging tests. Lymphoid malignancy (ALL in particular), the use of L-Asp, CNS involvement, and intrathecal chemotherapy might be risk factors for the occurrence SSST. When a patient with those factors develops any neurological symptoms, we should pay attention to the occurrence of SSST, as well as stroke or CNS involvement, though SSST is rare.
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PMID:[Superior sagittal sinus thrombosis during remission induction therapy for acute lymphoblastic leukemia]. 1723 72

We present a patient with Posterior Reversible Encephalopathy Syndrome (PRES). A 74-year-old woman was admitted with sepsis, which originated from erysipelas on her neck the following day. She developed respiratory obstruction due to oedema, septic shock, disseminated intravascular coagulation (DIC), acute renal failure and atrial fibrillation. She responded well to treatment and improved rapidly, despite of her serious condition. When she had almost fully recovered after 15 days, her general condition worsened, and she developed confusion, blindness and pareses. MRI showed vasogenic oedema in the parietooccipital regions of the brain and in the cerebellum, consistent with PRES. PRES is a clinical and radiological diagnosis consisting of headache, confusion, cortical blindness, convulsions and sometimes pareses. MRI of the cerebrum with diffusion-weighted imaging (DWI) and Apparent Diffusion Coefficient (ADC) map are decisive to the diagnosis, and usually shows a characteristic bilateral vasogenic oedema in the parietooccipital region. This can distinguish PRES from brain infarction, which shows a cytotoxic oedema on MRI. We discuss our patient in the light of different conditions leading to PRES, possible pathophysiological factors and treatment options.
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PMID:[An old woman with sudden pareses and blindness]. 1735 25

HELLP syndrome is a multi-organ disorder unique to pregnancy. It is characterized by hemolysis, elevated liver enzymes, and low platelets in patients with pre-eclampsia or eclampsia. In King Abdulaziz Oncology Center, Jeddah, seven patients with HELLP syndrome were admitted over a period of four years (1991-94). Retrospective analysis of data was done to study the clinical profile of HELLP syndrome. The incidence of HELLP syndrome in our institution was 1 per 2285 deliveries. One patient was Saudi and six were non-Saudis. The age range was 23 to 44 years, with a mean of 29 years. All patients were multipara. The disorder occurred between 24 to 33 weeks of gestational age, the average being 29 weeks. The most commonly encountered clinical feature was right upper quadrant/epigastric pain. Other features included nausea/vomiting, jaundice, hepatic encephalopathy, azotemia, hypotension and grand mal convulsions. All patients had severe pre-eclampsia pr eclampsia. Indirect hyperbilirubinemia was in the range of 2 to 8 mg/dL and elevated transaminases up to 229 U/L (n<40 U/L) were noted. Various degrees of peripheral thrombocytopenia (<150x10(9)/L) were present in seven patients. Four patients had elevated prothrombin and partial thromboplastin time with postive fibrinogen degradation products. Laboratory abnormalities returned to normal within 10 days following delivery. Four patients were delivered by cesarean section and three had vaginal deliveries. We had two maternal deaths (mortality 34%). One died of multi-organ failure and the other with adult respiratory distress syndrome. There was one stillbirth and the second baby died soon after birth due to prematurity (infant perinatal mortality 34%). We conclude that HELLP syndrome is rare among pregnant women in our institution. It should always be suspected in women with pre-eclampsia or eclampsia when they present with upper abdominal pain. Multipara seem to be more afflicted. Subclinical disseminated intravascular coagulation was detected in 55% of the patients. A majority of our patients presented late to the hospital.
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PMID:HELLP syndrome: Clinical profile of seven patients. 1737 23


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