Pregnancy in Gaucher disease

An 18-year old woman with type I Gaucher disease and two uncomplicated pregnancies is described. Although she experienced one miscarriage and pregnancy was associated with exaggeration of the clinical symptoms, leading to the diagnosis of the disorder, both her 2nd and 3rd pregnancies were uneventful and deterioration of her clinical situation was not observed. The issue of criteria for risk assessment in pregnancy of type I Gaucher disease patients is addressed.     

Uterine leiomyomas in the infertile patient: preoperative localization with MR imaging versus US and hysterosalpingography

Eleven women with a history of infertility and uterine leiomyomas underwent magnetic resonance (MR) imaging of the pelvis prior to myomectomy. Nine also underwent preoperative pelvic ultrasonography (US), and ten underwent hysterosalpingography. All studies were interpreted prospectively by independent observers. With each imaging modality, the location (one of 11 anatomic segments), size, and appearance of detected uterine leiomyomas were determined and compared with surgical and histologic findings. Among the nine patients who underwent both MR and US, the sensitivity (85%) and accuracy (94%) of MR imaging for abnormal segments was significantly better than that of US (sensitivity = 69%, P = .015; accuracy = 87%, P = .043). For the ten patients who underwent both MR and hysterosalpingography, the sensitivity (91%) and accuracy (96%) of MR imaging was better than that of hysterosalpingography (sensitivity = 18%, P = .0005; accuracy = 72%, P = .0005). The specificities of the three modalities did not significantly differ (100%, 97%, and 98% for MR, US, and hysterosalpingography, respectively). These data suggest that MR imaging is superior to US or hysterosalpingography for preoperatively locating uterine leiomyomas.     

MAGNOLOL REDUCES INFARCT SIZE AND SUPPRESSES VENTRICULAR ARRHYTHMIA IN RATS SUBJECTED TO CORONARY LIGATION

1. Magnolol is an active component of Magnolia officinalis. It is 1000-times more potent than α-tocopherol in inhibiting lipid peroxidation in rat heart mitochondira. In the present study, the in vivo antiarrhythmic and anti-ischaemic effects of magnolol in coronary ligated rats were investigated. 2. Male Sprague-Dawley rats were anaesthetized with urethane. Magnolol, at dosages of 10^?7, 10^?8 and 10^?9 g/kg, was adminstered intravenously 15 min before ligation of the coronary artery. 3. The incidence and duration of ventricular tachycardia and ventricular fibrillation during 30 min coronary ligation were significantly reduced by magnolol. Ventricular arrhythmias during 10 min reperfusion after the relief of coronary ligation were also reduced. 4. In rats subjected to 4h coronary ligation, 10^?7 and 10^?8 g/kg magnolol significantly reduced infarct size. 5. We conclude that magnolol may protect the myocardium against ischaemic injury and suppress ventricular arrhythmia during ischaemia and reperfusion.     

Application of recombinant BMP-7 on persistent upper and lower limb non-unions

The purpose of this study was to evaluate the efficacy and safety of recombinant bone morphogenetic protein 7 (rhBMP-7 or OP-1) as a bone-stimulating agent in the treatment of persistent fracture non-unions. Twenty-five consecutive patients [19 males, mean age 39.4 years (range: 18-79)] with 26 fracture non-unions were treated with rhBMP-7. There were 10 tibial non-unions, eight femoral, three humeral, three ulnar, one patellar, and one clavicular non-union. The mean follow-up was 15.3 months. The mean number of operations performed prior to rhBMP-7 application was 3.2, with autologous bone graft and bone marrow injection being used in 10 cases (38.5%). Both clinical and radiological union occurred in 24 (92.3%) cases, within a mean time of 4.2 months and 5.6 months, respectively. Of the remaining two cases, one patient ultimately underwent a below knee amputation, secondary to recurrence of deep sepsis. The other patient with recalcitrant ulnar non-union although the radiological union was incomplete, declined further intervention, as he was asymptomatic. No complications or adverse effects from the use of rhBMP-7 were encountered. This study supports the view that the application of rhBMP-7 as a bone-stimulating agent is safe and a power adjunct to be considered in the surgeon's armamentarium for the treatment of these challenging clinical conditions.     

Discovery and development of BMPs

In the early 1970s, the isolation and identification of bone morphogenetic proteins (BMPs) was a major breakthrough for the understanding of the distinct biological events occurring during bone formation. Not surprising, since their discovery, BMPs have been perhaps one of the most intensively studied group of factors in various physiological processes. The prompt development of recombinant BMPs and various delivery methods made BMPs currently available for clinical use. Research and clinical studies on BMPs are ongoing, aiming to refine further our understanding of their activities in vivo and to optimise and expand their clinical use in humans.     

Prophylactic intravenous ondansetron and dolasetron in intrathecal morphine-induced pruritus: a randomized, double-blinded, placebo-controlled study

Pruritus is the most common side effect of intrathecal morphine for postoperative pain relief. Activation of central 5-hydroxytryptamine subtype 3 (5-HT3) receptors is one of its possible mechanisms. The role of 5-HT3 antagonists in the prevention of pruritus has not been clearly established. In a prospective, randomized, double-blind, placebo-controlled study, we evaluated the efficacy of prophylactic administration of ondansetron and dolasetron for the prevention of intrathecal morphine-induced pruritus. The patients were randomized into 3 groups to receive either 4 mg ondansetron IV (group O, n = 35), 12.5 mg dolasetron IV (group D, n = 35) or 5 mL placebo (group P, n = 35) 30 min before administration of spinal anesthesia with 10 to 17.5 mg of 0.5% hyperbaric bupivacaine and 0.25 mg of morphine for urologic, orthopedic, or vascular surgery. Patients were evaluated for incidence and severity of pruritus at arrival to the postanesthesia care unit and at 2, 4, 8, and 24 h postoperatively. The incidence and severity of pruritus was significantly less frequent in the ondansetron and dolasetron groups compared with placebo (34%, 20%, and 66% respectively, P < 0.01). Patients who received 5-HT3 antagonist reported significantly less total severity of pruritus compared with placebo during the first 8 h and the severe pruritus was observed only in patients within P group (P group: 4 of 35; 11%, O or D group: 0 of 35; 0%, P < 0.05). We conclude that the prophylactic use of ondansetron and dolasetron helps to reduce the incidence and severity of intrathecal morphine-induced pruritus.     

Extrapulmonary Tuberculosis in Human Immunodificiency Virus Infection

Background

In view of increase in incidence of exptrapulmonary tuberculosis after the epidemic of human immunodeficiency virus infection, the clinical profile of extrapulmonary tuberculosis in patients with HIV infection was studied.

Method

The study population comprised patients of HIV infection with extrapulmonary tuberculosis. Work up included history, clinical examination, sputum for acid fast bacilli, chext X-ray, ultrasonography (USG) abdomen, fine needle aspiration cytology(FNAC), transbronchial needle aspiration (TBNA) and computed tomography of chest.

Results

There were 50 cases, all males with mean age of 35 years. 24(48%) were without pulmonary tuberculosis and 26(52%) had pulmonary tuberculosis. 41(82%) had disseminated disease and 9(18%) involve one site. Fever and weight loss were the most frequent symptoms (79% and 58% respectively) in cases without pulmonary tuberculosis. The most frequent extrapulmonary site was lymph node in 46(92%), followed by spleen in 13(26%), pleura 9(18%), miliary 7(14%) and hepatic 1(2%). The diagnosis was confirmed by invasive methods in 30 out of 50(60%) cases [FNAC in 23(88%), TBNA in 2(25%) and pleural biopsy in 5(55%)].

Conclusion

In HIV infected patients, the most common extrapulmonary site is lymph mode followed by spleen.Key Words: Extrapulmonary TB, HIV Infection     

Chitotriosidase in neonates with fungal and bacterial infections

Increased plasma and/or urine chitotriosidase activity was found in neonates with fungal infection changing in parallel with their clinical condition. Increased levels were also found in neonates with bacterial infection. Chitotriosidase activity increase is not a response specific to fungi, but serial assays could monitor the course of neonatal fungal infection.