Ceftriaxone induced hemolysis complicated by acute renal failure.

Over the last decade, second and third generation cephalosporins have been the most common drugs causing hemolytic anemia (HA). Of these cases, 20% have been attributed to ceftriaxone. The clinical presentation of ceftriaxone-induced HA is usually abrupt with sudden onset of pallor, tachypnea, cardio-respiratory arrest and shock. Acute renal failure (ARF) has been reported in 41% of such cases with a high fatality rate. We report a pediatric patient with ARF complicating ceftriaxone-induced HA who survived. Ceftriaxone is a commonly used drug, and early recognition of HA and institution of supportive care, including dialysis is likely to improve the outcome.

Increased lymphocyte Fas expression and high incidence of common variable immunodeficiency disorder in childhood Evans' syndrome.

Evans' syndrome (ES) is characterized by autoimmune hemolytic anemia and thrombocytopenia and has been associated with immune deficiency and lymphoproliferation in some cases. Abnormalities of Fas-mediated apoptosis have been reported in various immune dysregulation disorders associated with autoimmunity and lymphoproliferation. We measured lymphocyte Fas expression and Fas-mediated T lymphocyte apoptosis in 7 children with ES, 7 with acute idiopathic thrombocytopenic purpura (ITP) and 9 with non-immune-mediated disorders. Patients with ES had higher Fas expression on peripheral blood T and B lymphocytes (P<0.001 and P=0.046, respectively) and increased Fas-mediated elimination of activated T lymphocytes compared with the control groups. While two ES patients had panhypogammaglobulinemia at testing, three more developed it later, reaching a frequency of 83%. Some children with ES have increased lymphocyte Fas expression and Fas-mediated T lymphocyte apoptosis and these may be early signs of common variable immunodeficiency disorder in ES.

Primitive neuroectodermal tumor (PNET) of the uterus in a renal allograft patient: a case report.

The incidence of malignancy after renal transplant has been reported to range from 4% to 18%. Tumors of the skin and lip tend to be the most common with non-Hodgkin lymphoma comprising 20% of all neoplasms. Primitive neuroectodermal tumors (PNET) are collectively described as being a part of the Ewing sarcoma family of tumors. PNET occur more commonly in the second decade of life, predominantly affecting Whites and Hispanics, and rarely occur in individuals of African or Asian descent. The most common primary site of involvement is along the central axis, particularly the chest (Askin tumor), but it can arise in any soft tissue. PNET also occur in the head and neck. PNET involving the cervix, urinary bladder, uterus, and vagina have been reported. We describe a case of a 15-year-old female who, 9 years after receiving a living related renal transplant, developed a post-transplant PNET of the uterus.

Bleeding and coagulation changes during spinal fusion surgery: a comparison of neuromuscular and idiopathic scoliosis patients.

OBJECTIVE: Major blood loss is common during spinal fusion surgery. We have previously demonstrated that patients with neuromuscular scoliosis have more blood loss and greater transfusion requirement than those with idiopathic scoliosis. Our objective is to study the relationships between etiology of scoliosis, blood loss, and coagulation changes in children and adolescents undergoing spinal fusion surgery. DESIGN: Prospective, observational study. SETTING: University teaching hospital. PATIENTS: A total of 25 patients, 11 with neuromuscular and 14 with idiopathic scoliosis, undergoing spinal fusion surgery. INTERVENTIONS: Blood was obtained preoperatively, 2 and 4 hrs intraoperatively, and 2 and 24 hrs postoperatively for prothrombin time, partial thromboplastin time, thrombin time, platelet count, D-dimer, factor VII and VIII activity, thrombin-antithrombin III complex, and protein induced by vitamin K absence. Changes in coagulation over time were analyzed by repeated-measures analysis of variance. Comparisons between groups were made using independent t-tests. RESULTS: Neuromuscular scoliosis patients had significantly greater blood loss than idiopathic scoliosis patients (median blood loss, 78% of total blood volume; range, 25-127% vs. 20%, 2-82%; p < .001). Prothrombin time increased over time in both groups and was higher in the neuromuscular than the idiopathic group both preoperatively and postoperatively (p < .05). Factor VII activity decreased over time in both groups (p < .001) and was lower in the neuromuscular than the idiopathic group during surgery (p < .05). No changes in partial thromboplastin time, thrombin time, or factor VIII activity were observed. D-dimers were present in both groups by 4 hrs intraoperatively. Protein induced by vitamin K absence was not detected in any patient. CONCLUSIONS: Neuromuscular scoliosis patients have more blood loss during spinal fusion surgery than idiopathic scoliosis patients. The prolongation of prothrombin time and decrease in Factor VII activity suggest activation of the extrinsic coagulation pathway. Depletion of clotting factors during scoliosis surgery occurs to a greater extent in patients with underlying neuromuscular disease.