Syndrome of camptodactyly, ankyloses, facial anomalies, and pulmonary hypoplasia (Pena-Shokeir syndrome): obstetric and ultrasound aspects

Two siblings with Pena-Shokeir syndrome are described. This syndrome consists of polyhydramnios, intrauterine growth retardation, short umbilical cord, perinatal death, facial abnormalities, limb abnormalities including arthrogryposis, and lethal pulmonary hypoplasia. The mode of inheritance is most likely autosomal recessive. Prenatal diagnosis was made in the second pregnancy with ultrasound performed at 26 weeks' gestation. The roles of fetal akinesia and fetal apnea in the production of the various manifestations of the syndrome are detailed, and the possibility of early prenatal diagnosis is considered.     

Treatment of type IIa hyperlipidemia in childhood by a simplified American Heart Association diet and fiber supplementation

Thirty-six children identified as having primary type IIa hypercholesterolemia were treated with a diet restricting the intake of saturated fat to 10% of total energy and supplemented with soluble fiber for 8.1 +/- 2.4 (mean +/- SEM) months. In 14 of 36 patients first treated with the American Heart Association "Step-One" diet for 8.0 +/- 1.1 months, total cholesterol level dropped from 257.9 +/- 15.8 mg/dL to 240.6 +/- 10.9 mg/dL; the low-density lipoprotein cholesterol level dropped from 191.8 +/- 17.4 mg/dL to 175.0 +/- 11.7 mg/dL; the high-density lipoprotein cholesterol level dropped from 42.8 +/- 1.94 mg/dL to 41.6 +/- 1.68 mg/dL; and triglyceride concentration rose from 115.9 +/- 13.7 mg/dL to 128.1 +/- 14.1 mg/dL. The 36 patients treated with saturated fat-restrictive diets and supplemental soluble fiber diets had a reduction of total cholesterol levels from 249.2 +/- 7.66 mg/dL to 207.1 +/- 6.31 mg/dL and a low-density lipoprotein cholesterol level reduction from 184.7 +/- 7.55 mg/dL to 142.6 +/- 6.7 mg/dL, a reduction of 18% and 23%, respectively. There was no significant change in high-density lipoprotein cholesterol levels (46.4 +/- 1.9 mg/dL vs 44.3 +/- 2.1 mg/dL) or in triglyceride concentrations (94.2 +/- 7.43 mg/dL vs 102.2 +/- 8.45 mg/dL). In addition, the reduction in serum lipoprotein concentrations resulting from this program was significantly greater than the concentrations resulting from the American Heart Association diet alone. The different responses to these regimens suggest that a simplified diet and soluble fiber supplementation is well tolerated and reduces total and low-density lipoprotein cholesterol levels more effectively than the standard American Heart Association diet in children.     

Neonatal fat embolism and agglutination of intralipid

Pulmonary fat embolism in a preterm infant receiving Intralipid intravenously is described. Serum obtained at the time of clinical deterioration agglutinated Intralipid. This coincided with the onset of septicaemia and with a raised serum C reactive protein concentration. Subsequent clinical improvement was associated with reduction in the C reactive protein concentration and nonagglutination of Intralipid.     

The use and safety of Ibuprofen in the hemophiliac

After demonstrating initial safety of Ibuprofen administered to hemophiliacs, a 16-wk double-blind individual crossover trial was designed to test the safety and, to a more limited extent, the efficacy of 1600 mg of Ibuprofen or placebo given daily to 20 hemophiliacs with hemophiliac arthropathy. The trial was completed with no evidence of increased frequency or severity of hemophiliac bleeding episodes or clinical or laboratory evidence of bleeding secondary to Ibuprofen. There were five treatment failures, none associated with hemorrhage or lack of compliance. A benefit was obtained in reduction of early morning stiffness and pain. Ibuprofen should be considered as a safe and potentially beneficial antiinflammatory agent in the treatment of carefully monitored hemophiliacs eligible for such therapy.     

Percutaneous antegrade ureteric stent removal using a rigid alligator forceps

To evaluate the safety and efficacy of percutaneous antegrade ureteric stent removal using a rigid alligator forceps. Twenty patients were included in our study. Indications for ureteric stent insertion included stone disease (n = 7), malignancy (n = 8) and transplant anastomotic strictures (n = 5). Stent retrieval was carried out for proximal stent placement/migration in seven patients and encrustation in the remaining 13. Twenty‐two stents were successfully retrieved in 20 patients. There was one technical failure (5%). There were no major complications. We had four minor complications, which included nephrostomy site pain (n = 2), periprocedural sepsis (n = 1) and a small urinoma (n = 1). All patients settled with conservative management. Percutaneous radiologically guided antegrade ureteric stent removal with an alligator forceps is safe and effective, particularly when initial surgical removal has failed.     

Autologous blood transfusion: The benefits to the patient undergoing abdominal aortic aneurysm repair

The clinical benefits of using intraoperative autologous blood transfusion during abdominal aortic aneurysm bypass surgery become increasingly apparent when use of autologous and homologous blood transfusions is compared. That homologous blood transfusions carry some risk is widely recognized. When autologous blood is used as a sole source of blood transfusion, the risk of transmission of infectious agents and potential immunologic side effects are avoided. A prospective randomized pilot study comparing autologous and homologous blood transfusion in patients undergoing elective infrarenal abdominal aortic aneurysm bypass surgery was undertaken. The purpose of this study was to determine whether autologous blood salvaged intraoperatively may serve as an alternative to homologous blood by comparing the rate of postoperative infection and duration of hospital stay for patients receiving autologous versus homologous blood transfusions. Fifty patients undergoing abdominal aortic aneurysm bypass surgery were prospectively randomly assigned to receive either a homologous or an autologous blood transfusion, with 27 receiving a homologous blood transfusion and 23 receiving an autologous blood transfusion. The data from this study show that the length of hospital stay of patients receiving an autologous blood transfusion intraoperatively was reduced by a mean of 3 days and the risk of postoperative complications, such as a systemic inflammatory response or sepsis, was reduced by more than 50%.