Hereditary pancreatitis: An updated review in pediatrics

Hereditary Pancreatitis (HP) has emerged as a significant cause of acute, acute recurrent and chronic pancreatitis in the pediatric population. Given that it presents similarly to other causes of pancreatitis, a positive family history and/or isolation of a gene mutation are vital in its designation. Inheritance patterns remain complex, but mutations involving the PRSS1, SPINK1, CFTR and CTRC genes are commonly implicated. Since being first described in 1952, dozens of genetic alterations that modify the action of pancreatic enzymes have been identified. Among children, these variants have been isolated in more than 50% of patients with chronic pancreatitis. Recent research has noted that such mutations in PRSS1, SPINK1 and CFTR genes are also associated with a faster progression from acute pancreatitis to chronic pancreatitis. Patients with HP are at increased risk of developing diabetes mellitus, exocrine pancreatic insufficiency, and pancreatic adenocarcinoma. Management follows a multi-disciplinary approach with avoidance of triggers, surveillance of associated conditions, treatment of pancreatic insufficiency and use of endoscopic and surgical interventions for complications. With significant sequela, morbidity and a progressive nature, a thorough understanding of the etiology, pathophysiologic mechanisms, diagnostic evaluation, current management strategies and future research considerations for this evolving disease entity in pediatrics is warranted.     

Multicenter prospective validation of prehospital clinical spinal clearance criteria

BACKGROUND: Spine immobilization is one of the most frequently performed prehospital procedures. If trauma patients without significant risk for spine injury complications can be identified, spine immobilization could be selectively performed. The purpose of this study was to evaluate five prehospital clinical criteria-altered mental status, neurologic deficit, spine pain or tenderness, evidence of intoxication, or suspected extremity fracture-the absence of which identify prehospital trauma patients without a significant spine injury. METHODS: Prospectively collected emergency medical services data items included the above-listed criteria. Outcome data include spine fracture or cord injury, and also the level and management of injuries. RESULTS: A total of 295 patients with spine injuries were present in 8,975 (3.3%) cases. Spine injury was identified by the prehospital criteria in 280 of 295 (94.9%) injured patients. The criteria missed 15 patients. Thirteen of 15 had stable injuries, the majority of which were stable compression or vertebral process injuries. The remaining two would have been captured by more accurate prehospital evaluation. CONCLUSION: Absence of the study criteria may form the basis of a prehospital protocol that could be used to identify trauma patients who may safely have rigid spine immobilization withheld. Evaluation of such a protocol in practice should be performed.     

Validation of a questionnaire for measurement of beliefs about the climacteric

OBJECTIVE: To design an instrument for measuring beliefs about the social, psychological, and physiological consequences of women's climacteric stage. MATERIAL AND METHODS: The study included 340 women affiliated to Instituto de Seguridad y Servicios Sociales para los Trabajadores del Estado (Institute for Social Services and Security for State Workers, ISSSTE) (age mean = 49.46, (SD 7.92). The mean number of pregnancies in the sample was 3.75 (SD 2.57), and the mean number of born children was 3.21 (SD 2.19); 48% of women were premenopausal, 10.9% perimenopausal, and 40.6% postmenopausal. The instrument consisted of 25 items. RESULTS: A factorial analysis with Varimax rotation was carried out. Four factors were confirmed: disadvantages (alpha = 0.769), advantages (alpha = 0.839), physiological (alpha = 0.659), and psychological (alpha = 0.711). CONCLUSION: This instrument shows good internal consistency, and measures four climacteric belief groups: a) beliefs on disadvantages, b) beliefs on advantages, c) beliefs on physiological ailments, and d) beliefs on psychological symptoms. All three confirmed dimensions of the climacteric phase have been proposed in other studies.     

Outcomes of cardiac arrest in the nursing home: Destiny or futility?

Objective. To compare EMS system characteristics and outcomes between nursing home (NH) patients and out-of-hospital cardiac arrest (OHCA) patients whose arrests occurred in a residence (home).

Design. Prospective cohort study reviewing OHCA from July 1989 to December 1993. Variables were age, witnessed arrest, response intervals, automated external defibrillator (AED) use, and arrest rhythms. Outcomes were hospital admission and discharge. Pearson chi-square was used for analysis.

Setting. Suburban EMS system.

Subjects. Patients ≥ 19 years old with arrest of presumed cardiac cause, with locations at home or at a NH.

Results. 2,348 total arrests were complete for analysis, 182 at a NH and 2,166 at home. BLS and ALS response intervals were shorter for the NH patients. The NH patients were more likely to receive CPR on collapse, were older (73.1 vs 67.5 years, p < 0.001), were less likely to have had an AED used (9.9% vs 30.0%, p < 0.001), and were more likely to have an arrest bradyasystolic rhythm (74.7% vs 51.5%, p < 0.001). They were less likely to survive to hospital admission (10.4% vs 18.5%, p < 0.006) and discharge (0.0% vs 5.6%, p < 0.001).

Conclusion. During this four-and-a-half-year study period, no NH patient survived, even though % CPR was increased. Arrest rhythm is an important factor in this finding. EMS initial care for ventricular tachycardia/fibrillation NH patients, with less application of AEDs, was identified. This different response may adversely contribute to dismal NH outcome.     

Validity and reliability of the Mexican resilience measurement scale in families of children with chronic conditions

Background

The resilience to face disease is a process of positive adaptation despite the loss of health. It involves developing vitality and skills to overcome the negative effects of adversity, risks, and vulnerability caused by disease. In Mexico, the Mexican Resilience Measurement Scale (RESI-M) has been validated with a general population and has a five-factor structure. However, this scale does not allow evaluation of resilience in specific subpopulations, such as caregivers.

Method

This study investigated the psychometric properties of RESI-M in 446 family caregivers of children with chronic diseases. A confirmatory factor analysis (CFA) was performed, internal consistency values were calculated using Cronbach’s alpha coefficient, and mean comparisons were determined using t-tests.

Results

The expected five-factor model showed an adequate fit with the data based on a maximum likelihood test. The internal consistency for each factor ranged from .76 to .93, and the global internal consistency was .95. No average difference in RESI-M and its factors was found between women and men.

Conclusion

The RESI-M showed internal consistency and its model of five correlated factors was valid among family caregivers of children with chronic diseases.

     

Prevalence and correlates of adolescent dating violence: baseline study of a cohort of 7,960 male and female Mexican public school students

INTRODUCTION: Factors correlated with adolescent dating violence have yet to be documented in most developing countries; this study assesses the prevalence and correlates of victimization with and perpetration of dating violence among Mexican youth. METHODS: This was the baseline measurement (1998-1999) of a cohort of 7960 public school students (11-24 years) developed to explore various health behaviors in Mexican youth. Multinomial logistic regression models were constructed with adolescent dating violence as the dependent variable. RESULTS: Prevalence of dating violence victimization was 9.37% (female) and 8.57% (male) for psychological violence; 9.88% (female) and 22.71% (male) for physical violence, and 8.63% (female) and 15.15% (male) for both psychological and physical violence. Prevalence of perpetration was 4.21% (female) and 4.33% (male) for psychological violence; 20.99% (female) and 19.54% (male) for physical violence; and 7.48% (female) and 5.51% (male) for both types of violence. Factors associated with dating violence victimization for both genders included: two or more lifetime sexual partners and intra-familial violence. Higher age, alcohol use and illegal drug use were significantly associated with victimization only among girls. The following were significantly associated with perpetration of dating violence in both genders: gang membership, illegal drug use, two or more lifetime sexual partners and intra-familial violence. Higher age and alcohol use were significantly associated with perpetration only among girls. High or middle socio-economic status was associated with perpetration only in boys. CONCLUSIONS: Future research on adolescent dating violence in Mexico should further explore severity and frequency of violent behaviors, include a focus on severe dating violence and take into account the context and meaning of dating violence. A longitudinal design that allows determination of causality will also be needed in order to develop prevention strategies.     

Assessment of vascular stiffness in the internal carotid artery proximal to the carotid canal in Alzheimer’s disease using pulse wave velocity from low rank reconstructed 4D flow MRI

Clinical evidence shows vascular factors may co-occur and complicate the expression of Alzheimer’s disease (AD); yet, the pathologic mechanisms and involvement of different compartments of the vascular network are not well understood. Diseases such as arteriosclerosis diminish vascular compliance and will lead to arterial stiffness, a well-established risk factor for cardiovascular morbidity. Arterial stiffness can be assessed using pulse wave velocity (PWV); however, this is usually done from carotid-to-femoral artery ratios. To probe the brain vasculature, intracranial PWV measures would be ideal. In this study, high temporal resolution 4D flow MRI was used to assess transcranial PWV in 160 subjects including AD, mild cognitive impairment (MCI), healthy controls, and healthy subjects with apolipoprotein ɛ4 positivity (APOE4+) and parental history of AD dementia (FH+). High temporal resolution imaging was achieved by high temporal binning of retrospectively gated data using a local-low rank approach. Significantly higher transcranial PWV in AD dementia and MCI subjects was found when compared to old-age-matched controls (AD vs. old-age-matched controls: P <0.001, AD vs. MCI: P = 0.029, MCI vs. old-age-matched controls P = 0.013). Furthermore, vascular changes were found in clinically healthy middle-age adults with APOE4+ and FH+ indicating significantly higher transcranial PWV compared to controls (P <0.001).     

Functional analysis of the phenobarbital-responsive unit in rat CYP2B2

An 163-bp fragment of the rat cytochrome P450 gene, CYP2B2 has been shown to contain sequences that mediate phenobarbital (PB) responsiveness of this gene. In studies on this rat gene and the orthologous mouse gene, Cyp2b10, the minimal fragment required for near full PB responsiveness has varied from about 50 to 80 bp depending on the gene used and the number of copies of the PB responsive sequences assessed. Since there is a single copy of the CYP genes in the genome, we have evaluated deletion and block mutations across an 84-bp region of the PB responsive unit (PBRU), by in situ transfection in rat liver using single copies of the PBRU sequences. From the 5' end, deletions to -2243 retained more than 50% responsiveness to PB compared to the 163-bp fragment. The fragment -2237 to -2155 retained less than 20% responsiveness even though it contained the nuclear receptor (NR)-1, NR-2, and NF-1 motifs which are present in the core of the PBRU. From the 3' end, deletions from -2170 to -2194 eliminated PB responsiveness indicating that the 74-bp sequence from -2243 to -2170 is able to mediate full PB responsiveness. Block mutations within the NR-1 and NF-1 regions reduced responsiveness most dramatically, but did not abolish it, and mutations 3' of the NF-1 site modestly reduced responsiveness. Protein binding was not affected by mutations in the NR-1 region as assessed by DNase I footprinting in vitro but mutations within the NR-2 region reduced binding to the NF-1 site. Mutations of the 5' half or the 3' half of the bipartite NF-1 site, resulted in loss of protection of the NF-1 site and new footprints to the 3' or 5' side, respectively, of the NF-1 site. These results indicate that sequences in addition to the NR-1 and -2 and the NF-1 sites are required for full responsiveness to PB and suggest that proteins which bind to these sites may interact.