Metatropic dysplasia-a skeletal dysplasia with challenging airway and other anesthetic concerns.

BACKGROUND: Metatropic dysplasia is a rare form of skeletal dysplasia requiring multiple anesthetics for surgical and imaging procedures, most of which are orthopedic procedures. We provide centralized care to patients with skeletal dysplasia at our tertiary care pediatric hospital, and we were able to collect the largest number of metatropic dysplasia patients reported to date. AIM: The aim of this retrospective study was to describe and characterize the anesthetic difficulties in this high-risk population. METHODS: Medical charts of all patients with metatropic dysplasia were reviewed to collect data, including anesthetics performed, difficulties, and complications related to the anesthetic care, co-morbid conditions, and related events. RESULTS: Twenty-three patients with metatropic dysplasia underwent 188 anesthetics with 61% of the anesthetics having been administered for orthopedic procedures. Fourteen of 23 (60.8%) progressively became difficult to intubate over the course of their care, with 12 out of 14 having undergone cervical spine fusion. These 14 patients had a total of 133 procedures. Sixty procedures (45.1%) had an airway described as difficult. Glidescope was the difficult airway tool most commonly used (68%) with flexible fiberoptic scope used 12% and Miller or Macintosh blade used 18% of the time. In addition to the airway difficulties, spinal canal narrowing or stenosis was widely prevalent, and no neuraxial anesthetic was performed in any of our patients. CONCLUSION: Difficult airway is the most common co-morbid condition present in patients with metatropic dysplasia, especially if their cervical spine has been fused. Familiarity with the difficulties involving the airway and its management is critical in safe and successful management of anesthesia in this high-risk population.

Correlates of patient portal enrollment and activation in primary care pediatrics.

OBJECTIVE: To identify the demographic, practice site, and clinical predictors of patient portal enrollment and activation among a pediatric primary care population. METHODS: We conducted a cross-sectional analysis of the primary care database of an academic children's hospital that introduced a patient portal in December 2007. RESULTS: We analyzed data for 84,015 children. Over a 4-year period, 38% enrolled in the portal; of these, 26% activated the account. The adjusted odds of portal enrollment was lower for adolescents, Medicaid recipients, low-income families, Asian or other race, and Hispanic ethnicity, and higher for patients with more office encounters, and presence of autism on the problem list. Once enrolled, the odds of portal activation [adjusted odds ratio (95% confidence interval)] was decreased for: Medicaid [0.55 (0.50-0.61)] and uninsured [0.79 (0.64-0.97)] (vs private insurance), black [0.53 (0.49-0.57)] and other [0.80 (0.71-0.91)] (vs white race), Hispanic ethnicity [0.77 (0.62-0.97)], and increased for: infant age [1.26 (1.15-1.37)] (vs school age), attendance at a resident continuity practice site [1.91 (1.23-2.97)], living further away from the practice (vs under 2 miles)[4.5-8.8 miles: 1.14 (1.02-1.29); more than 8.8 miles: 1.19 (1.07-1.33)], having more office encounters (vs 1-3) [4-7 encounters: 1.40 (1.24-1.59); 8-12 encounters: 1.58 (1.38-1.81); 13+ encounters: 2.09 (1.72-2.55)], and having 3 or more items on the problem list (vs 0) [1.19 (1.07-1.33)]. CONCLUSIONS: Sociodemographic disparities exist in patient portal enrollment/activation in primary care pediatrics. Attendance at a resident continuity practice site, living farther away from the practice, having more office encounters, and having more problem list items increased the odds of portal activation.

Independent relation of maternal prenatal factors to early childhood obesity in the offspring.

OBJECTIVE: To examine the independent contribution of risk factors developing during pregnancy to subsequent risk of obesity in young children. METHODS: We conducted a historical cohort study using data from electronic medical records of mothers and their 3,302 singleton offspring born between 2004 and 2007 at a community-based obstetric facility who attended a 4-year well visit at a pediatric practice network. The child's body mass index (BMI) z score at age 4 years was studied in relation to the mother's gestational weight gain, gestational diabetes mellitus, gestational hypertension or preeclampsia, and prenatal tobacco use. Institute of Medicine categories defined excess and inadequate gestational weight gain at term. Analysis of variance and multiple linear regression were used to test their independent relation to BMI. RESULTS: Mothers were white (39%), African American (46%), and of Hispanic ethnicity (11%); 46% were privately insured. The association of net gestational weight gain with the child's BMI z score was significant after adjustment for prepregnancy maternal factors (P<.001); gestational diabetes mellitus, gestational hypertension, and tobacco use were not significant in adjusted models. Children of mothers with excess gestational weight gain had a higher mean BMI z score (P<.001) but a significant association was observed only for inadequate gestational weight gain after adjusting for prepregnancy BMI and other covariates. Prepregnancy BMI (P<.001), Hispanic ethnicity (P<.001), and being married (P<.05) were independently associated with increasing BMI z score of the offspring. CONCLUSIONS: Preconception maternal factors had a greater influence on child obesity than prenatal factors. The gestational weight gain category was independently related to BMI z score of 4 year olds, but this association was significant only for mothers with inadequate gestational weight gain. LEVEL OF EVIDENCE: II.

Altered physiology of rainbow trout in response to modified energy intake combined with pulp and paper effluent exposure.

Two experiments using rainbow trout (Oncorhynchus mykiss) were conducted to examine the combined effects of energy intake as manipulated by ration and pulp and paper mill effluent exposure over either one, or two consecutive reproductive cycles. This study demonstrated that the level of energy intake affected the full range of measured parameters from energy allocation to somatic growth and the gonadal development, steroid production and hematology. Increasing ration level expectedly increased growth, condition, liver and gonad size. Female trout in the higher ration treatments produced more follicles and had larger eggs, investing the same relative proportion of total energy into ovarian development. Sex steroid levels and hematological parameters were also positively influenced by increasing ration level in males and females. By far, the most dramatic impact of reduced ration on reproduction was to substantially reduce the frequency of sexually maturing fish. The effects of effluent exposure were not as marked as those linked to ration level and typically did not manifest unless fish were exposed through two consecutive reproductive cycles. The physiological effects of pulp and paper effluent exposure observed in these experiments were not consistent between the two experiments conducted herein, nor were they consistent with previously observed impacts in similar experiments with this effluent. Effluent exposure over one reproductive cycle did not impact physiological parameters in trout. However, when effluent exposure was maintained over two reproductive cycles, a new pattern of effluent response emerged including increased condition factor in both sexes, a decrease in the potential ability of the blood of females to transport oxygen, and increased sex steroids and reproductive investment in males. Effluent was also observed to cause reduced growth in male trout over two years. The effects of ration on gonad and liver size were far more obvious and consistent when a longer exposure was employed, thus, it appears to take more than one full year for energy intake changes to be reflected in those particular physiological endpoints.

Cumulative impacts assessment along a large river, using brown bullhead catfish (Ameiurus nebulosus) populations.

The effects of point-source and diffuse discharges on resident populations of brown bullhead catfish (Ameiurus nebulosus (LeSueur, 1819)) in the Waikato River (New Zealand) were assessed at sites both upstream and downstream of point-source discharges. At each site, the population parameters, relative abundance, age structure, and individual indices, such as condition factor, organ (gonad, liver, and spleen) to somatic weight ratios, and number and size of follicles per female, were assessed. Physiological (blood), biochemical (hepatic ethoxyresorufin-O-deethylase [EROD] and plasma steroids), and other indicators (bile chemistry and liver metals) of exposure or response also were measured. No impacts on brown bullhead health were obvious at individual geothermal, municipal sewage, or thermal discharge sites or cumulatively along the river. Brown bullhead from the bleached kraft mill effluent site showed elevated levels of EROD, decreased numbers of red blood cells, increased numbers of white blood cells, and depressed levels of sex steroids. However, growth rates, condition factor, age structure, and gonadosomatic index suggest that discharges with significant heat or nutrients benefit catfish despite physiological impairment at one site. Consideration of brown bullhead population-level responses to discharges in a monitoring framework revealed three different population-level response patterns resulting from the point-source discharges.