1/1088. Solitary hepatic hemangioma in a newborn infant complicated by cardiac failure, consumption coagulopathy, microangiopathic hemolytic anemia, and obstructive jaundice. Case report and review of the literature. A newborn infant with a large hepatic hemangioma developed congestive heart failure, consumption coagulopathy, microangiopathic hemolytic anemia, and obstructive jaundice. The patient was mildly heparinized (250 units per kg and day) and underwent successful resection of the tumor without lobectomy at the age of 3 days. blood volume increased from 93.9 ml/kg at the age of 5 h to 124.2 ml/kg prior to surgery. Red-cell mass simultaneously decreased from 53.8 to 39.4 ml/kg. The increase of blood volume is explained by congestive heart failure, the decrease of red-cell mass by intravascular coagulation within the tumor resulting in formation of thrombi and microangiopathic hemolytic anemia. A review of the literature on infants with symptoms caused by an intrahepatic hemangioma during the first month of life confirms that surgical intervention is the treatment of choice for infants with giant solitary hemangioma of the liver. ( info) |
2/1088. Splenic hemangiosarcoma with massive calcification. We present a case of large splenic hemangiosarcoma in a 40-year-old man associated with consumptive coagulopathy. Computed tomography showed radial calcification within the splenic tumor. On magnetic resonance imaging, T2 shortening represented a meshwork of calcification and surrounding fibrosis. The possibility of hemangiosarcoma should be considered when a large splenic tumor with massive calcification is noted. ( info) |
| disseminated intravascular coagulation is a complex hemostatic imbalance associated with many disease states. The potentially lethal systemic consequences of this disease mandate that the podiatric physician obtain a complete detailed history in addition to proceeding with appropriate consultations from other specialties. If haste is utilized and surgical intervention is undertaken without careful consideration, a potentially fatal situation may ensue. A comprehensive case report highlighting the pedal presentation and complications, and an overview of this disease process are presented. ( info) |
| A 90-year-old male, with impending rupture of an isolated internal iliac artery aneurysm (IIAA) complicated with disseminated intravascular coagulation (DIC) was successfully treated with transcatheter arterial embolization (TAE). After TAE, enlargement of the aneurysm was arrested and coagulation-fibrinolytic abnormalities induced by DIC improved without severe complications. Although IIAA is relatively rare, the post-operative mortality of patients with ruptures is reportedly high. We assessed the usefulness of this procedure for impending rupture of IIAA, especially for patients in high risk groups. ( info) |
5/1088. Compound heterozygosity for one novel and one recurrent mutation in a Thai patient with severe protein s deficiency. Homozygous or compound heterozygous protein S (PS) deficiency is a very rare disorder in the anticoagulant system, that can lead to life-threatening thrombotic complications shortly after birth. This report describes the results of the genetic analysis of the PROS 1 genes in a Thai girl patient. She was reported in 1990 as the first case with homozygous PS deficiency and neonatal purpura fulminans. In the present report, we identified the mutations in this patient by direct sequencing of PCR products representing all 15 exons of the PROS 1 gene and their flanking intronic regions. The patient turned out to be compound heterozygous for two null mutations. One allele contained a novel sequence variation, an A-insertion in an A5-tract covering codon 146 and 147, that results in a frameshift and a stop codon (TAA) at position 155. The other allele contained a nonsense mutation in exon 12 by a transition at codon 410 CGA (Arg) to TGA (stop). Cosegregation of PS deficiency with these two genetic defects was observed in her family. ( info) |
| Acute disseminated intravascular coagulation is a common and potentially serious complication of many clinical conditions, including trauma. We report two cases in which this condition occurred after routine elective primary total hip replacement. ( info) |
7/1088. hemolysis associated with 25% human albumin diluted with sterile water--united states, 1994-1998. Since 1994, a shortage of 5% human albumin, a product used off-label during therapeutic plasma exchange (TPE), has existed in the united states. Because of this shortage, hospital pharmacists may prepare 5% solution of human albumin by diluting 25% human albumin with 0.9% NaCl or, when sodium load is a concern, 5% dextrose. However, if sterile water alone is used as the diluent, the osmolarity (tonicity) of the albumin solution is reduced and may cause hemolysis in recipients. This report describes two of 10 episodes of hemolysis (one fatal) among persons who received 25% human albumin diluted with sterile water and emphasizes that sterile water alone should not be used to dilute albumin. ( info) |
8/1088. Torted ovarian cyst with lethal bleeding diathesis in an infant. We report a 9-month-old infant with a torted ovarian cyst who presented with an acute consumptive coagulopathy (CC) with lethal outcome. That ischemic tissue can act as a trigger for a CC is well-known, but we did not find any report of a torted ovarian cyst causing a coagulopathy in the pediatric literature. This potential complication constitutes one more reason for the prompt surgical removal of torted ovarian cysts in infants. ( info) |
9/1088. Fatal multi-organ failure after suicidal overdose with MDMA, 'ecstasy': case report and review of the literature. A 53-year-old prisoner died of multiorgan failure after a suicidal overdose with 3,4-methylenedeoxymethamphetamine (MDMA, 'Ecstasy'). Twelve hours after ingestion of MDMA, the patient became severely hyperthermic (107.2 degrees F) with evidence of rhabdomyolysis. He subsequently developed acute respiratory distress syndrome (ARDS), disseminated intravascular coagulopathy (DIC) and acute renal failure. At autopsy, plasma concentration of MDMA was 3.05 mg/L. This case shows that MDMA is still abused in our community and clinicians should know the symptoms of MDMA intoxication. In particular, MDMA should be considered when patients have symptoms or signs of increased sympathetic activity. The pathophysiology and treatment of MDMA-induced hyperthermia are discussed. ( info) |
| disseminated intravascular coagulation (DIC) was first observed clinically in a case of sepsis following severe trauma. It was postulated that the observed clotting defect and bleeding were due to the using up of clotting factors in an episode of intravascular clotting. It was also postulated that the multiple organ failure observed was due to obstruction of the microcirculation of the organs by microclots. Evidence for this process was worked out in many animal studies. It was then postulated that if these microclots could be lysed before organ necrosis was produced, organ failure could be prevented. This prevention was shown to be possible in animals. It was then tried in humans using plasminogen activators, and the approach was found to be effective. Using a low dose of plasminogen activator over a 24-hour period caused no changes in the coagulation profile or bleeding. ( info) |