The anesthetic management of ventricular septal defect (VSD) repair in a child with mitochondrial cytopathy

PURPOSE: To present the anesthetic management for the correction of a ventricular septal defect (VSD) in a patient with multiple acyl CoA dehydrogenase deficiency (glutaric aciduria type II; GAII). A review of the literature about anesthetic management of patients with mitochondrial diseases undergoing cardiopulmonary bypass (CPB) is also included. Clinical features: An 11-yr-old girl with GAII manifested as severe hypoglycemia since she was a newborn and generalized muscle weakness. She underwent open-heart surgery for VSD correction with CPB. The anesthetic management avoided inhalational anesthetics, maintained the blood sugar within normal limits and continued normothermia during CPB in order to avoid the stress of hypothermia for her abnormal mitochondria. The patient tolerated the procedure well and experienced a good recovery. CONCLUSION: The anesthetic management of patients with any mitochondrial disease requires normoglycemia, normothermia and the avoidance of metabolic stress in order to preserve energy production by the diseased mitochondria.     

Human osteoblasts' proliferative responses to strain and 17beta-estradiol are mediated by the estrogen receptor and the receptor for insulin-like growth factor I.

The mechanism by which mechanical strain and estrogen stimulate bone cell proliferation was investigated using monolayer cultures of human osteoblastic TE85 cells and female human primary (first-passage) osteoblasts (fHOBs). Both cell types showed small but statistically significant dose-dependent increases in [3H]thymidine incorporation in response to 17beta-estradiol and to a single 10-minute period of uniaxial cyclic strain (1 Hz). In both cell types, the peak response to 17beta-estradiol occurred at 10(-8) - 10(-7) M and the peak response to strain occurred at 3500 microstrain ((mu)epsilon). Both strain-related and 17beta-estradiol-related increases in [3H]thymidine incorporation were abolished by the estrogen receptor (ER) modulator ICI 182,780 (10-8 M). Tamoxifen (10(-9) - 10(-8) M) increased [3H]thymidine incorporation in both cell types but had no effect on their response to strain. In TE85 cells, tamoxifen reduced the increase in [3H]thymidine incorporation associated with 17beta-estradiol to that of tamoxifen alone but had no such effect in fHOBs. In TE85 cells, strain increased medium concentrations of insulin-like growth factor (IGF) II but not IGF-I, whereas 17beta-estradiol increased medium concentrations of IGF-I but not IGF-II. Neutralizing monoclonal antibody (MNAb) to IGF-I (3 microg/ml) blocked the effects of 17beta-estradiol and exogenous truncated IGF-I (tIGF-I; 50 ng/ml) but not those of strain or tIGF-II (50 ng/ml). Neutralizing antibody to IGF-II (3 microg/ml) blocked the effects of strain and tIGF-II but not those of 17beta-estradiol or tIGF-I. MAb aIR-3 (100 ng/ml) to the IGF-I receptor blocked the effects on [3H]thymidine incorporation of strain, tIGF-II, 17beta-estradiol, and tIGF-I. HOBs and TE85 cells, act similarly to rat primary osteoblasts and ROS 17/2.8 cells in their dose-related proliferative responses to strain and 17beta-estradiol, both of which can be blocked by the ER modulator ICI 182,780. In TE85 cells (as in rat primaries and ROS 17/2.8 cells), the response to 17beta-estradiol is mediated by IGF-I, and the response to strain is mediated by IGF-II. Human cells differ from rat cells in that tamoxifen does not block their response to strain and reduces the response to 17beta-estradiol in TE85s but not primaries. In both human cell types (unlike rat cells) the effects of strain and IGF-II as well as estradiol and IGF-I can be blocked at the IGF-I receptor.     

Hepatic reticuloendothelial function during parenteral nutrition including an MCT/LCT or LCT emulsion after liver transplantation – a double-blind study

It has been demonstrated that total parenteral nutrition (TPN) modulates the function of the hepatic reticuloendothelial system (RES). The objective of this study was to evaluate the impact of two different TPN lipid emulsions on the recovery of allograft RES function after orthotopic liver transplantation (OLTx). In a prospective, double-blind study, OLTx patients were randomly assigned to two treatment groups. Group I ( n=13) received a TPN regimen that included long-chain triglycerides (LCT). Group II ( n=9) received a TPN regimen that included a fat emulsion consisting of both medium-chain triglycerides (MCT) and LCT. At baseline, i.e., on days 2 or 3 after OLTx ( t1), before lipids for TPN were started, hepatic RES function was determined using the human serum albumin millimicrosphere technique (K-value, 1/min). A second measurement ( t2) was obtained after 7 days of TPN, including one of the study's two fat emulsions. The mean (+/- SD) K-value (1/min) was 0.48+/-0.16 in the LCT group and 0.55+/-0.28 in the MCT/LCT group at t1, and it improved to 0.62+/-0.21 in the LCT group and to 0.86+/-0.32 in the MCT/LCT group at t2. RES function recovery was significantly better in the MCT/LCT group ( P< or = 0.05). MCT/LCT emulsion appears to be the TPN fat emulsion of choice after OLTx as it seems to have less impact on hepatic RES recovery.     

Reassessment of CD62L as a marker of pre-effector T cells in the tumor draining lymph nodes of head and neck cancer patients.

OBJECTIVES: CD62L was evaluated as a determinant of human pre-effector T cells. STUDY DESIGN AND SETTING: Phenotype and cytokine secretion profiles of CD62L cells were determined based on activation status. RESULTS: CD62L(Low) T cells demonstrated significantly higher secretion of interleukin (IL)-10 and interferon (IFN)-gamma than did CD62L(High) T cells. After activation, the majority of cells expressed high levels of the CD62L surface marker. Postactivation levels of IL-10 production remained elevated or unchanged. In a murine B16 melanoma model, freshly isolated CD62L(Low) tumor draining lymph nodes (TDLN) T cells showed increased secretion of IL-2 and IL-4 but not of IL-10 or IFN-gamma. The surface expression of CD62L and cytokine secretion patterns were maintained after activation with concomitant increases in IL-10. CONCLUSION: Our results provide evidence that CD62L(Low) T cells in TDLNs of progressively growing squamous cell carcinoma of the head and neck differ phenotypically and functionally from those of mouse origin. SIGNIFICANCE: Characterization of this human CD62L(Low) T cell population provides initial insight regarding novel surface markers in TDLN T cells that might correlate with antitumor reactivity.     

Intrapartum-related birth asphyxia in South Africa--lessons from the first national perinatal care survey.

BACKGROUND: The recent amalgamation of data by users of the Perinatal Problem Identification Programme (PPIP) throughout South Africa has culminated in the publication of the Saving Babies report. OBJECTIVES: To determine the absolute rate of death from intrapartum-related birth asphyxia, and the contribution of intrapartum-related asphyxia to total perinatal mortality in South African hospitals, and to identify the primary obstetric causes and avoidable factors for these deaths. METHODS: The amalgamated PPIP data for the year 2000 were obtained from 27 state hospitals (6 metropolitan, 12 town and 9 rural) in South Africa. In PPIP-based audit, all perinatal deaths are assigned primary obstetric causes and avoidable factors, and these elements were obtained for all deaths resulting from intrapartum-related birth asphyxia. RESULTS: There were 123,508 births in the hospitals surveyed, with 4,142 perinatal deaths among infants > or = 1,000 g, giving a perinatal mortality rate of 33.5/1,000 births. The perinatal mortality rate from intrapartum-related birth asphyxia was 4.8/1,000 births. The most frequent avoidable factors were delay by mothers in seeking attention during labour (36.6%), signs of fetal distress interpreted incorrectly (24.9%), inadequate fetal monitoring (18.0%) and no response to poor progress in labour (7.0%). The perinatal mortality rates for metropolitan, town, and rural areas were 30.0, 39.4 and 30.9/1,000 births respectively. The contribution of intrapartum-related birth asphyxia to perinatal mortality in these areas was 10.8%, 16.7% and 26.4% respectively. CONCLUSION: The high rates of perinatal death from intrapartum-related birth asphyxia in South Africa are typical of those in underdeveloped countries, with the most serious deficiencies in rural areas. Most of these deaths are avoidable and the reduction of these rates presents an important challenge to providers of perinatal care in this country. Areas worthy of research and action include provision of mothers' waiting facilities in rural regions, improvements in fetal monitoring, partogram-based labour management, and the establishment of midwifery staffing norms for South African labour units.     

Photodynamic therapy for choroidal neovascular membrane secondary to optic nerve drusen.

The authors describe the use of photodynamic therapy with verteporfin for subfoveal choroidal neovascular membrane secondary to optic nerve drusen. A 28-year-old woman had a peripapillary choroidal neovascular membrane secondary to optic nerve drusen with significant metamorphopsia. Photodynamic therapy using verteporfin was performed. Visual acuity improved to 20/20 with resolution of metamorphopsia and absence of leakage on fluorescein angiography a few weeks after verteporfin therapy. The patient's condition remained stable for 16 months with 20/20 vision. Photodynamic therapy with verteporfin may be a useful treatment option in patients with choroidal neovascular membranes secondary to optic nerve drusen.     

Changes in Marijuana Use Over the Transition Into Marriage

Reductions in substance use tend to coincide with marriage, as both may occur during emerging adulthood. During the transition to marriage, it is possible that one's spouse may be the influence that causes the reduction in substance use. Data on participants (N = 471 couples) for this report are taken from a longitudinal study of early marriage. The objective of the current analysis is to determine if having a spouse who uses marijuana is associated with a greater likelihood of one's own use. Additionally, we are interested in spousal influence and cessation. The findings support spousal influence. Husbands are more likely to start using marijuana if their wives use marijuana, but the reverse is not true. Husbands also are more likely to stop using if their spouses do not use. During the transition into marriage, the drug use of one spouse does affect the other. However, this influence appears to be unidirectional, with wives influencing their husbands more often.