Propofol and Propofol–Ketamine in Pediatric Patients Undergoing Cardiac Catheterization

We investigated the effects of propofol and propofol–ketamine on hemodynamics, sedation level, and recovery period in pediatric patients undergoing cardiac catheterization. We performed a prospective, randomized, double-blind study. The study included 60 American Society of Anesthesiologists physical status II or III (age range, 1 month–13 years) undergoing cardiac catheterization for evaluation of congenital heart disease. Propofol and ketamine were prepared in 5% glucose solution to a final concentration of 5 and 1 mg/ml, respectively; similar injectors containing 5% glucose solution only were prepared. Fentanyl (1 μg/kg) and propofol (1.5 mg/kg) were given to both groups. Then, group 1 received 0.5 ml/kg of 5% glucose and group 2 0.5 ml/kg of ketamine solution by an anesthesiologist who was unaware of the groups of patients. Local anesthesia with 1% lidocaine was administered before intervention in all patients. The noninvasively measured mean arterial pressure, heart rate, respiratory rate, and peripheral oxygen saturation were recorded at the baseline, following drug administration, at 3, 5, 10, 15, 20, and 30 minutes and then at 15-minute intervals until the end of the procedure. Additional drug and fentanyl requirements to maintain a sedation level of 4 or 5 were recorded. After the procedure, the time to a Steward recovery score of 6 and adverse effects in the first 24 hours were recorded. The number of patients with more than a 20% decrease in mean arterial pressure was 11 in group 1 and 3 in group 2 (p < 0.05). The number of patients who experienced more than a 20% decrease in heart rate was 12 in group 1 and 5 in group 2 (p = 0.054). Ten patients in group 1 and 3 patients in group 2 required additional fentanyl doses (p = 0.057). The number of additional propofol doses was lower in group 2 (p < 0.05). Propofol combined with low-dose ketamine preserves mean arterial pressure better without affecting the recovery and thus is a good option in pediatric patients undergoing cardiac catheterization.     

Asymmetric crying facies associated with congenital hypoparathyroidism and 22q11 deletion

Asymmetric crying facies is caused by congenital hypoplasia or agenesis of the depressor anguli oris muscle. Associations of this facial defect with major congenital anomalies have been reported, most commonly in the cardiovascular system and less frequently involving the genitourinary, musculoskeletal, cervicofacial, respiratory, and rarely, the endocrine system. CATCH 22 is a medical acronym for cardiac defects, abnormal facies, thymic hypoplasia, cleft palate and hypocalcemia and a variable deletion on chromosome 22q11. The deletion within chromosme region of 22q11 may occur in patients with dysmorphologic and cardiological syndromes: DiGeorge syndrome, velo-cardiofacial syndrome and conotruncal anomaly face syndrome. We report a newborn infant who had asymmetric crying facies associated with congenital hypoparathyroidism, severe neonatal hypocalcemia and tetralogy of Fallot. Genetic confirmation of chromosome 22q11 deletion was made.     

The relationship between preprocedural platelet size and subsequent in-stent restenosis

OBJECTIVE: Elevated mean platelet volume predicts restenosis after percutaneous transluminal coronary angioplasty but its effect on the development of in-stent restenosis is not known. We assessed the effect of mean platelet volume measured before coronary stent implantation for stable angina pectoris on subsequent development of in-stent restenosis. METHODS AND RESULTS: We retrospectively analysed the data of 60 patients who had stent implantation on one native coronary artery for stable angina pectoris and control angiographies for clinically suspected restenosis within 6 months. Mean platelet volume was measured by auto analyzer one day before stent implantation. Clinical and demographic data and laboratory results were obtained from the hospital charts of the patients. In-stent restenosis was evaluated visually from control angiograms. Angiographic in-stent restenosis was present in 35 (58%) of 60 patients and 25 (42%) patients had no restenosis. Mean platelet volume in the in-stent restenosis group was 8.28 +/- 0.71 fl compared to 7.63 +/- 0.74 fl in the no-restenosis group (p = 0.001). There was a positive correlation between preprocedural mean platelet volume and development of in-stent restenosis (r = 0.44; p < 0.001). A mean platelet volume value of > or = 8.4 fl was associated with an odds ratio of 16.0 for development of in-stent restenosis, with high specificity and positive predictivity but poor sensitivity and negative predictivity (96%, 93%, 40% and 53%, respectively). CONCLUSIONS: Mean platelet volume measured before stent implantation is correlated with subsequent development of in-stent restenosis. If preprocedural mean platelet volume is greater than 8.4 fl, in-stent restenosis is more probable to occur.     

Clinical and microscopic hair features of griscelli syndrome associated with asymmetric crying facies in an infant.

Griscelli syndrome (GS) is a rare autosomal recessive disorder characterized by immunodeficiency and partial albinism. Asymmetric crying facies (ACF) is caused by congenital hypoplasia or agenesis of the depressor anguli oris muscle. Asymmetric crying facies may be isolated or it may be associated with various anomalies. We report a 3-month-old Turkish boy who had clinical and microscopic hair features of GS associated with ACF. To the best of our knowledge, this is the 1st case of this association to be reported in the literature.     

Treatment of refractory peripheral T-cell lymphoma with denileukin diftitox (ONTAK)

Peripheral T-cell lymphomas (PTCLs) are an uncommon and heterogeneous group of well-differentiated, post-thymic T-cell malignancies that can present in the skin as cutaneous T-cell lymphomas. In general, their prognosis is poor, and specific therapy is not well defined. We report the successful treatment of a patient with relapsed, refractory PTCL who after failing 13 standard single and multiple chemotherapy regimens and experimental agents had a dramatic prolonged response to diftitoxin denileukin (ONTAK). This fusion protein, composed of diphtheria toxin coupled to interleukin-2, is approved for cutaneous T-cell lymphomas, including mycosis fungoides, and should be considered for treatment of the rare subset of peripheral T-cell lymphomas.     

Cytochrome P450 2E1 polymorphism and nasopharyngeal carcinoma development in Thailand: a correlative study

Background  

Nasopharyngeal carcinoma (NPC) is a rare tumor in most parts of the world but occurs at relatively high frequency among people of Chinese descent. The cytochrome P450 2E1 enzyme (CYP2E1) is responsible for the metabolic activation of nitrosamines, and has been shown to be a susceptibility gene for NPC development in Taiwan [RR = 2.6; 95%CI = 1.2-5.7]. Since there has been only one report of this link, it was decided to investigate the susceptibility of CYP2E1 to NPC development in other populations. Therefore, the correlation between the RsaI polymorphism of this gene and NPC was studied in-patients including Thai and Chinese in Thailand. The present study comprised 217 cases diagnosed with NPC and 297 healthy controls.     

Giant right atrial thrombus in premature newborn

Pediatric guidelines for treatment options of right atrial thrombosis in newborn are quite limited. Herein we present a case with giant atrial thrombosis resulting from umbilical venous catheter and intend to discuss the therapy in the area of current literature on right atrial thrombus in newborn and children.