Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002878 (hemolytic anemia)
 7,530 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Classic hemophilia or factor VIII deficiency is a recessive, sex-linked bleeding diathesis. The primary clinical problem is hemorrhage, which can be severe and often life threatening, even in the presence of only minor trauma. In the past this inadequate hemostasis has been treated with transfusions of cryoprecipitate, fresh frozen plasma, or commercially prepared factor VIII concentrate. Unfortunately, such treatment carries with it a number of risks, including the development of hepatitis B or hemolytic anemia and the formation of anti-factor VIII antibodies. Because of hemorrhage severity and the risks of conventional treatment, elective surgery in general and oral surgery in particular have often been neglected in patients with hemophilia. This article reviews a drug, 1-desamino-8-d-arginine (DDAVP), heretofore not discussed in the dental literature, and reports on its use in conjunction with epsilon-aminocaproic acid (EACA), a synthetic antifibrinolytic agent, in the surgical dental treatment of a patient with hemophilia A. The results suggest that certain dental surgical procedures can be performed in the presence of subclinical and mild hemophilia without conventional factor VIII replacement therapy with its associated costs and risks.
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PMID:DDAVP: review of indications for its use in the treatment of factor VIII deficiency and report of a case. 622 10

Thrombotic thrombocytopenic purpura (TTP) is a rare and often fatal disorder characterized by thrombocytopenia, microangiopathic hemolytic anemia, mental status changes, and renal dysfunction. Central diabetes insipidus (CDI) is a rare disease due to anatomic lesions of the hypothalamicpituitary system associated with various underlying diseases, or trauma. We present an unusual case of TTP and CDI in a 47 year-old African American female who was admitted to our hospital with crampy abdominal pain and nausea. The patient had tachycardia, fever and hypotension. The patient subsequently became confused, developed seizures, and her renal function deteriorated. Bone marrow analysis showed adequate megakaryocytes while a peripheral smear revealed severe thrombocytopenia, polychromasia and schistocytes. The diagnosis of thrombotic thrombocytopenic purpura (TTP) was made and plasmapharesis initiated. Over the next few days, the patient developed severe polyuria with a rise in serum sodium. Central diabetes insipidus was diagnosed and DDAVP (desmopressin acetate, 1-deamino-8-D-arginine vasopressin) was given. However, DDAVP was stopped several times due to worsening thrombocytopenia. Renal function worsened and the patient expired. A review of the literature revealed only one case of report of TTP and central diabetes insipidus. Our case was the only one reporting the use of DDAVP in such a setting.
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PMID:Unique case of thrombotic thrombocytopenic purpura and diabetes insipidus. 1641 83

Gastroenteritis due to Escherichia coli O157:H7 occurs in young children and is associated with consumption of under cooked beef. Approximately 5-10% of patients will develop hemolytic uremic syndrome (HUS): renal failure, microangiopathic hemolytic anemia, and thrombocytopenia. A 6-year-old boy was admitted with abdominal pain, guaiac positive stool, decreased urine output and elevated creatinine levels. Hemodialysis was initiated upon rapid progression to anuria. On hospital day # 5 he developed acute abdominal pain, which was different from his initial assessment. Exam revealed focal tenderness in the right lower quadrant with localized guarding and rebound. Ultrasound demonstrated a dilated, fluid filled tubular structure in the RLQ concerning for appendicitis. Based on these findings the patient was taken to the operating room for a laparoscopic appendectomy. The patient had undergone dialysis the previous day and was preoperatively treated with DDAVP to minimize the risk of bleeding. The procedure occurred without complication and final pathology confirmed acute appendicitis. This case highlights the unique clinical scenario in which patients with HUS require operative intervention. Surgical procedures can be performed on these patients, however, all precautions should be taken to minimize the risk of bleeding, including the use of preoperative DDAVP.
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PMID:Acute appendicitis in a patient with hemolytic uremic syndrome: an unusual clinical scenario. 1741 Mar 68