Gender Identity in Patients with Congenital Adrenal Hyperplasia.

PubMed

Razzaghy-Azar, Maryam; Karimi, Sakineh; Shirazi, Elham

2017-07-01

Sex assignment in infancy for patients with disorder of sex development (DSD) is a challenging problem. Some of the patients with congenital adrenal hyperplasia (CAH) have DSD that may affect their gender identity. The study aimed to assess gender identity in patients with CAH. In this study, 52 patients with CAH, including 22 prepubertal children and 30 adolescents and adults, were assessed using two separate gender identity questionnaires for children and adults based on the criteria of diagnostic and statistical manual of mental disorders, 5th edition. In the children group, compatibility was seen between gender identity and rearing gender. In the adult group, there were three cases of mismatching between gender identity and sex assignment composed of two females with poor control and one male with good control with 21-hydroxylase deficiency (21-OHD). Three girls with 11-hydroxylase deficiency (11-OHD) were reared as boy. Two of them with late diagnosis at 5 and 6 years of age had pseudoprecocious puberty. Parents and children did not accept to change the gender. One of them is 36 years old now, is depressed and unsatisfied with her gender, another girl is still child and has male sexual identity. One girl with 11-OHD and early diagnosis at birth with Prader 5 virilization but with good hormonal control was changed to female gender at 12 years of age when female sexual characteristics appeared; she is 34-years-old now, married, and with two children, and she is satisfied with her gender. In patients with CAH, gender identity disorder is a rare finding. Hormonal control, social, familial, and religious beliefs have impacts on gender identity of these patients.

Gender Identity in Patients with Congenital Adrenal Hyperplasia

PubMed Central

Razzaghy-Azar, Maryam; Karimi, Sakineh; Shirazi, Elham

2017-01-01

Background Sex assignment in infancy for patients with disorder of sex development (DSD) is a challenging problem. Some of the patients with congenital adrenal hyperplasia (CAH) have DSD that may affect their gender identity. Objectives The study aimed to assess gender identity in patients with CAH. Methods In this study, 52 patients with CAH, including 22 prepubertal children and 30 adolescents and adults, were assessed using two separate gender identity questionnaires for children and adults based on the criteria of diagnostic and statistical manual of mental disorders, 5th edition. Results In the children group, compatibility was seen between gender identity and rearing gender. In the adult group, there were three cases of mismatching between gender identity and sex assignment composed of two females with poor control and one male with good control with 21-hydroxylase deficiency (21-OHD). Three girls with 11-hydroxylase deficiency (11-OHD) were reared as boy. Two of them with late diagnosis at 5 and 6 years of age had pseudoprecocious puberty. Parents and children did not accept to change the gender. One of them is 36 years old now, is depressed and unsatisfied with her gender, another girl is still child and has male sexual identity. One girl with 11-OHD and early diagnosis at birth with Prader 5 virilization but with good hormonal control was changed to female gender at 12 years of age when female sexual characteristics appeared; she is 34-years-old now, married, and with two children, and she is satisfied with her gender. Conclusions In patients with CAH, gender identity disorder is a rare finding. Hormonal control, social, familial, and religious beliefs have impacts on gender identity of these patients. PMID:29201068

The carbonic anhydrase CAH1 is an essential component of the carbon-concentrating mechanism in Nannochloropsis oceanica.

PubMed

Gee, Christopher W; Niyogi, Krishna K

2017-04-25

Aquatic photosynthetic organisms cope with low environmental CO 2 concentrations through the action of carbon-concentrating mechanisms (CCMs). Known eukaryotic CCMs consist of inorganic carbon transporters and carbonic anhydrases (and other supporting components) that culminate in elevated [CO 2 ] inside a chloroplastic Rubisco-containing structure called a pyrenoid. We set out to determine the molecular mechanisms underlying the CCM in the emerging model photosynthetic stramenopile, Nannochloropsis oceanica , a unicellular picoplanktonic alga that lacks a pyrenoid. We characterized CARBONIC ANHYDRASE 1 ( CAH1 ) as an essential component of the CCM in N. oceanica CCMP1779. We generated insertions in this gene by directed homologous recombination and found that the cah1 mutant has severe defects in growth and photosynthesis at ambient CO 2 We identified CAH1 as an α-type carbonic anhydrase, providing a biochemical role in CCM function. CAH1 was found to localize to the lumen of the epiplastid endoplasmic reticulum, with its expression regulated by the external inorganic carbon concentration at both the transcript and protein levels. Taken together, these findings show that CAH1 is an indispensable component of what may be a simple but effective and dynamic CCM in N. oceanica .

A Phase 2 Study of Continuous Subcutaneous Hydrocortisone Infusion in Adults With Congenital Adrenal Hyperplasia.

PubMed

Nella, Aikaterini A; Mallappa, Ashwini; Perritt, Ashley F; Gounden, Verena; Kumar, Parag; Sinaii, Ninet; Daley, Lori-Ann; Ling, Alexander; Liu, Chia-Ying; Soldin, Steven J; Merke, Deborah P

2016-12-01

Classic congenital adrenal hyperplasia (CAH) management remains challenging, given that supraphysiologic glucocorticoid doses are often needed to optimally suppress the ACTH-driven adrenal androgen overproduction. This study sought to approximate physiologic cortisol secretion via continuous subcutaneous hydrocortisone infusion (CSHI) and evaluate the safety and efficacy of CSHI in patients with difficult-to-treat CAH. Eight adult patients with classic CAH participated in a single-center open-label phase I-II study comparing CSHI to conventional oral glucocorticoid treatment. All patients had elevated adrenal steroids and one or more comorbidities at study entry. Assessment while receiving conventional therapy at baseline and 6 months following CSHI included: 24-hour hormonal sampling, metabolic and radiologic evaluation, health-related quality-of-life (HRQoL), and fatigue questionnaires. The ability of CSHI to approximate physiologic cortisol secretion and the percent of patients with 0700-hour 17-hydroxyprogesterone (17-OHP) ≤1200 ng/dL was measured. CSHI approximated physiologic cortisol secretion. Compared with baseline, 6 months of CSHI resulted in decreased 0700-hour and 24-hour area under the curve 17-OHP, androstenedione, ACTH, and progesterone, increased osteocalcin, c-telopeptide and lean mass, and improved HRQoL (and SF-36 Vitality Score), and fatigue. One of three amenorrheic women resumed menses. One man had reduction of testicular adrenal rest tissue. CSHI is a safe and well-tolerated modality of cortisol replacement that effectively approximates physiologic cortisol secretion in patients with classic CAH poorly controlled on conventional therapy. Improved adrenal steroid control and positive effects on HRQoL suggest that CSHI should be considered a treatment option for classic CAH. The long-term effect on established comorbidities requires further study.

UTILIZATION OF TREATABILITY AND PILOT TESTS TO PREDICT CAH BIOREMEDIATION

EPA Science Inventory

Multiple tools have been suggested to help in the design of enhanced anaerobic bioremediation systems for CAHs:
- Extensive high quality microcosm testing followed by small-scale, thoroughly observed field pilot tests (i.e., RABITT Protocol, Morse 1998)
- More limited ...

Enhanced CAH dechlorination in a low permeability, variably-saturated medium

USGS Publications Warehouse

Martin, J.P.; Sorenson, K.S.; Peterson, L.N.; Brennan, R.A.; Werth, C.J.; Sanford, R.A.; Bures, G.H.; Taylor, C.J.; ,

2002-01-01

An innovative pilot-scale field test was performed to enhance the anaerobic reductive dechlorination (ARD) of chlorinated aliphatic hydrocarbons (CAHs) in a low permeability, variably-saturated formation. The selected technology combines the use of a hydraulic fracturing (fracking) technique with enhanced bioremediation through the creation of highly-permeable sand- and electron donor-filled fractures in the low permeability matrix. Chitin was selected as the electron donor because of its unique properties as a polymeric organic material and based on the results of lab studies that indicated its ability to support ARD. The distribution and impact of chitin- and sand-filled fractures to the system was evaluated using hydrologic, geophysical, and geochemical parameters. The results indicate that, where distributed, chitin favorably impacted redox conditions and supported enhanced ARD of CAHs. These results indicate that this technology may be a viable and cost-effective approach for remediation of low-permeability, variably saturated systems.

Quality of life in adults with congenital adrenal hyperplasia relates to glucocorticoid treatment, adiposity and insulin resistance: United Kingdom Congenital adrenal Hyperplasia Adult Study Executive (CaHASE)

PubMed Central

Han, Thang S; Krone, Nils; Willis, Debbie S; Conway, Gerard S; Hahner, Stefanie; Rees, D Aled; Stimson, Roland H; Walker, Brian R; Arlt, Wiebke; Ross, Richard J

2013-01-01

Context Quality of life (QoL) has been variously reported as normal or impaired in adults with congenital adrenal hyperplasia (CAH). To explore the reasons for this discrepancy we investigated the relationship between QoL, glucocorticoid treatment and other health outcomes in CAH adults. Methods Cross-sectional analysis of 151 adults with 21-hydroxylase deficiency aged 18–69 years in whom QoL (assessed using the Short Form Health Survey), glucocorticoid regimen, anthropometric and metabolic measures were recorded. Relationships were examined between QoL, type of glucocorticoid (hydrocortisone, prednisolone and dexamethasone) and dose of glucocorticoid expressed as prednisolone dose equivalent (PreDEq). QoL was expressed as z-scores calculated from matched controls (14 430 subjects from UK population). Principal components analysis (PCA) was undertaken to identify clusters of associated clinical and biochemical features and the principal component (PC) scores used in regression analysis as predictor of QoL. Results QoL scores were associated with type of glucocorticoid treatment for vitality (P=0.002) and mental health (P=0.011), with higher z-scores indicating better QoL in patients on hydrocortisone monotherapy (P<0.05). QoL did not relate to PreDEq or mutation severity. PCA identified three PCs (PC1, disease control; PC2, adiposity and insulin resistance and PC3, blood pressure and mutations) that explained 61% of the variance in observed variables. Stepwise multiple regression analysis demonstrated that PC2, reflecting adiposity and insulin resistance (waist circumference, serum triglycerides, homeostasis model assessment of insulin resistance and HDL-cholesterol), related to QoL scores, specifically impaired physical functioning, bodily pain, general health, Physical Component Summary Score (P<0.001) and vitality (P=0.002). Conclusions Increased adiposity, insulin resistance and use of prednisolone or dexamethasone are associated with impaired QoL in adults

A Phase 2 Study of Continuous Subcutaneous Hydrocortisone Infusion in Adults With Congenital Adrenal Hyperplasia

PubMed Central

Mallappa, Ashwini; Perritt, Ashley F.; Gounden, Verena; Kumar, Parag; Sinaii, Ninet; Daley, Lori-Ann; Ling, Alexander; Liu, Chia-Ying; Soldin, Steven J.; Merke, Deborah P.

2016-01-01

Context: Classic congenital adrenal hyperplasia (CAH) management remains challenging, given that supraphysiologic glucocorticoid doses are often needed to optimally suppress the ACTH-driven adrenal androgen overproduction. Objective: This study sought to approximate physiologic cortisol secretion via continuous subcutaneous hydrocortisone infusion (CSHI) and evaluate the safety and efficacy of CSHI in patients with difficult-to-treat CAH. Design: Eight adult patients with classic CAH participated in a single-center open-label phase I–II study comparing CSHI to conventional oral glucocorticoid treatment. All patients had elevated adrenal steroids and one or more comorbidities at study entry. Assessment while receiving conventional therapy at baseline and 6 months following CSHI included: 24-hour hormonal sampling, metabolic and radiologic evaluation, health-related quality-of-life (HRQoL), and fatigue questionnaires. Main Outcome Measures: The ability of CSHI to approximate physiologic cortisol secretion and the percent of patients with 0700-hour 17-hydroxyprogesterone (17-OHP) ≤1200 ng/dL was measured. Results: CSHI approximated physiologic cortisol secretion. Compared with baseline, 6 months of CSHI resulted in decreased 0700-hour and 24-hour area under the curve 17-OHP, androstenedione, ACTH, and progesterone, increased osteocalcin, c-telopeptide and lean mass, and improved HRQoL (and SF-36 Vitality Score), and fatigue. One of three amenorrheic women resumed menses. One man had reduction of testicular adrenal rest tissue. Conclusions: CSHI is a safe and well-tolerated modality of cortisol replacement that effectively approximates physiologic cortisol secretion in patients with classic CAH poorly controlled on conventional therapy. Improved adrenal steroid control and positive effects on HRQoL suggest that CSHI should be considered a treatment option for classic CAH. The long-term effect on established comorbidities requires further study. PMID:27680873

Reassigning the CaH+ 11Σ → 21Σ vibronic transition with CaD+

NASA Astrophysics Data System (ADS)

Condoluci, J.; Janardan, S.; Calvin, A. T.; Rugango, R.; Shu, G.; Sherrill, C. D.; Brown, K. R.

2017-12-01

We observe vibronic transitions in CaD+ between the 11Σ and 21Σ electronic states by resonance enhanced multiphoton photodissociation spectroscopy in a Coulomb crystal. The vibronic transitions are compared with previous measurements on CaH+. The result is a revised assignment of the CaH+ vibronic levels and a disagreement with multi-state-complete-active-space second-order perturbation theory theoretical calculations by approximately 700 cm-1. Updated high-level coupled-cluster calculations that include core-valence correlations reduce the disagreement between theory and experiment to 300 cm-1.

UTILIZATION OF TREATABILITY AND PILOT TESTS TO PREDICT CAH BIOREMEDIATION (Battelle)

EPA Science Inventory

Multiple tools have been suggested to help in the design of enhanced anaerobic bioremediation systems for CAHs:
Extensive high quality microcosm testing followed by small-scale, thoroughly observed, induced flow field pilot tests (i.e. RABITT Protocol, Morse 1998)
More...

Rapid steroid hormone quantification for congenital adrenal hyperplasia (CAH) in dried blood spots using UPLC liquid chromatography-tandem mass spectrometry.

PubMed

Janzen, Nils; Sander, Stefanie; Terhardt, Michael; Steuerwald, Ulrike; Peter, Michael; Das, Anibh M; Sander, Johannes

2011-12-11

Newborn screening for congenital adrenal hyperplasia (CAH) is usually done by quantifying 17α-hydroxyprogesterone using immunoassay. However, this test produces high rates of false positive results caused by cross reacting steroids. Therefore we have developed a selective and specific method with a short run time (1.25 min) for quantification of 17α-hydroxyprogesterone, 21-deoxycortisol, 11-deoxycortisol, 11-deoxycorticosterone and cortisol from dried blood spots. The extraction procedure is very simple and steroid separation is ensured on a BEH C18 column and an ultra-performance liquid chromatography-tandem mass spectrometry (UPLC-MS/MS). Analysis was done in positive ionization mode (ESI+) and recorded in multiple reaction monitoring mode (MRM). The method gave linear results for all steroids over a range of 5-200 (cortisol: 12.5-500)nmol/L with coefficients of regression >0.992. Absolute recovery was >64.1%. Across the analytical range the inter-assay coefficient of variation (CV) was <3%. Newborn blood samples of patients with confirmed 21-CAH and 11-CAH could clearly be distinguished from samples of unaffected newborns falsely positive on immunoassay. The method is not influenced by cross reactions as found on immunoassay. Analysis of dried blood spots shows that this method is sensitive and fast enough to allow rapid analysis and can therefore improve the newborn screening program. Copyright © 2011 Elsevier Inc. All rights reserved.

A Defined and Flexible Pocket Explains Aryl Substrate Promiscuity by the Cahuitamycin Starter Unit Activating Enzyme CahJ.

PubMed

Sherman, David H; Tripathi, Ashootosh; Park, Sung Ryeol; Sikkema, Andrew; Cho, Hyo Je; Wu, Jianfeng; Lee, Brian; Xi, Chuanwu; Smith, Janet L

2018-05-09

Cahuitamycins are biofilm inhibitors biosynthesized by a convergent NRPS pathway. Previous genetic analysis indicated that a discrete enzyme, CahJ, serves as a gatekeeper for cahuitamycin structural diversification. Herein, the CahJ protein was probed structurally, functionally and through mutasynthesis. This analysis enabled production of a new cahuitamycin congener through targeted precursor incorporation. © 2018 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim.

Gender role across development in adult women with congenital adrenal hyperplasia due to 21-hydroxylase deficiency.

PubMed

Long, Dominique N; Wisniewski, Amy B; Migeon, Claude J

2004-10-01

This study evaluated the degree of femininity and masculinity at different developmental stages in a group of adult women, some of whom were exposed to elevated prenatal adrenal androgens as a result of congenital adrenal hyperplasia (CAH) due to 21 hydroxylase (21-OH) deficiency. Women who had presented to the Johns Hopkins Hospital Pediatric Endocrine Clinic for treatment of CAH due to 21-OH deficiency were included. The control group consisted of sisters of CAH participants and women referred for evaluation of polycystic ovary syndrome. Study participants were given a questionnaire asking them to indicate their degree of masculinity and femininity during childhood, adolescence, and adulthood. In addition, participants were asked questions related to their play behavior during childhood, including playmate preferences, toy preferences, and admiration of male or female characters during fantasy play. Across participant groups, self-reported femininity decreased in a dose response manner, according to prenatal androgen exposure. For all groups, femininity increased through developmental stages. Women with salt-losing CAH remained less feminine than controls into adulthood. Conversely, self-reported masculinity increased in a dose-response manner, according to prenatal androgen exposure, across participant groups. Women with CAH showed a decrease in masculinity across developmental stages, such that by adulthood, there were no significant differences in masculinity between controls and the women with CAH. Women with salt-losing CAH were more likely to recall preferences for boy playmates, male-typical toys, and admiration for male characters during childhood than other study participants. Our data support the effect of both prenatal androgen exposure and socialization on gender role behavior in adult women with CAH due to 21-OH deficiency.

Patient Bypass Behavior and Critical Access Hospitals: Implications for Patient Retention

ERIC Educational Resources Information Center

Liu, Jiexin (Jason); Bellamy, Gail R.; McCormick, Melissa

2007-01-01

Purpose: To assess the extent of bypass for inpatient care among patients living in Critical Access Hospital (CAH) service areas, and to determine factors associated with bypass, the reasons for bypass, and what CAHs can do to retain patients locally. Methods: Six hundred and forty-seven subjects, aged 18 years and older, who had been admitted to…

Quality of Care and Patient Outcomes in Critical Access Hospitals

PubMed Central

Joynt, Karen E.; Harris, Yael; Orav, E. John; Jha, Ashish K.

2012-01-01

Context Critical Access Hospitals (CAHs) play a crucial role in the nation’s rural safety net. Current policy efforts have focused primarily on helping these small, isolated hospitals remain financially viable to ensure access for Americans living in rural areas. However, we know little about the quality of care they provide, or the outcomes their patients achieve. Objective To examine the quality of care and patient outcomes at CAHs, and to understand why patterns of care might differ for CAHs versus non-CAHs. Design Retrospective analysis of national data from Medicare and other sources. Setting U.S. hospitals. Patients Medicare fee-for-service beneficiaries with acute myocardial infarction (AMI), congestive heart failure (CHF), and pneumonia, discharged in 2008–2009. Main Outcome Measures Clinical capabilities, performance on processes of care, and 30-day mortality rates. Results Compared to other hospitals, CAHs were less likely to have intensive care units (30.0% versus 74.4%, p<0.001), cardiac catheterization capabilities (0.5% versus 47.7%, p<0.001), and at least basic electronic health records (4.6% versus 9.9%, p<0.001). CAHs had lower performance on process measures than non-CAHs for all three conditions examined (Hospital Quality Alliance summary score for AMI 91.0% versus 97.8%, for CHF, 80.6% versus 93.5%, and for pneumonia 89.3% versus 93.7%, p<0.001 for each). Patients admitted to a CAH had higher 30-day mortality rates for each condition than those admitted to non-CAHs (for AMI, 23.5% versus 16.2%, Odds Ratio (OR) 1.70 (95% confidence interval 1.61, 1.80), p<0.001; for CHF, 13.4% versus 10.9%, OR 1.28 (1.23, 1.32), p<0.001; and for pneumonia 14.1% versus 12.1%, OR 1.20 (1.16, 1.24) p<0.001). Conclusions Care in CAHs, compared with non-CAHs, is associated with worse processes of care and higher mortality rates. PMID:21730240

Transition of Care from Childhood to Adulthood: Congenital Adrenal Hyperplasia.

PubMed

Bachelot, Anne

2018-01-01

Deficiency of the 21-hydroxylase enzyme is the most common form of congenital adrenal hyperplasia (CAH), accounting for more than 95% of the cases. With the advent of newborn screening and hormone replacement therapy, most children with CAH survive into adulthood. Adolescents and adults with CAH experience a number of complications, including short stature, obesity, infertility, impaired bone mineral density, and reduced quality of life. Transition from pediatric to adult care and management of long-term complications are challenging for both patients and practitioners. In adulthood, the aims of the medical treatment are to substitute cortisol and, when necessary, aldosterone deficiency, to ensure normal fertility, and to avoid the long-term consequences of glucocorticoid use on bone, metabolism, and cardiovascular risk. Recent data suggest that poor health status is likely to begin in adolescence and persist into adulthood, highlighting the importance of this time period in a patient's endocrine care. During transition from pediatric to adult specific care, a shift in treatment goals is thus needed. Successful transition from pediatric to adult health care requires a regular follow-up of patients by a multidisciplinary team including pediatric endocrinologists, urologists, gynecologists, psychiatrists, and adult endocrinologists. All of this could be included in a specific therapeutic education program regarding transition and/or CAH. © 2018 S. Karger AG, Basel.

Community-acquired hypokalemia in elderly patients: related factors and clinical outcomes.

PubMed

Bardak, S; Turgutalp, K; Koyuncu, M B; Harı, H; Helvacı, I; Ovla, D; Horoz, M; Demir, S; Kıykım, A

2017-03-01

Electrolyte imbalance is a common problem affecting the elderly. Increased number of comorbidities and frequent use of drugs may contribute to increased risk of hypokalemia in the elderly. This study was performed to investigate the prevalence of community-acquired hypokalemia (CAH), risk factors for its development, related factors with hypokalemia, and morbidities and all-cause mortality rates (MR) of CAH in the elderly patients. Total of 36,361 patients aged above 65 years were screened retrospectively. Group 1 consisted of 269 elderly patients with potassium level ≤3.5 mmol/L, and group 2 (control group) consisted of 182 subjects with potassium level between 3.6 and 5.5 mmol/L. Etiologic factors of CAH, presence of comorbidities, duration of hospital stay, hospital cost, and clinical outcomes were recorded. Prevalence of hypokalemia was found 3.24% in patients aged above 65 years. Duration of hospital stay, presence of ≥2 comorbid diseases, hospital cost, and MR were significantly higher in group 1 compared to group 2 (p < 0.001 for all). Loop diuretics, hydrochlorothiazides, beta agonists, inadequate oral intake, and female gender were all independent risk factors for CAH in elderly patients. Patients with ≥2 comorbid diseases were found to have greater risk of hypokalemia than the patients with <2 comorbidities. Length of hospital stay, hospital cost, and MR were higher in elderly with CAH. Female gender, hydrochlorothiazides, loop diuretics, and ≥2 comorbid diseases are the leading risk factors associated with CAH in elderly.

Growth and development in children with classic congenital adrenal hyperplasia.

PubMed

Bonfig, Walter

2017-02-01

Final height outcome in classic congenital adrenal hyperplasia (CAH) has been of interest for many years. With analysis of growth patterns and used glucocorticoid regimens, enhanced treatment strategies have been developed and are still under development. Most of the current reports on final height outcome are confirmative of previous results. Final height data is still reported in cohorts that were diagnosed clinically and not by newborn screening. Clinical diagnosis of CAH leads to delayed diagnosis especially of simple virilizing CAH with significantly advanced bone age resulting in early pubertal development and reduced final height. In contrast salt-wasting CAH is diagnosed at an earlier stage in most cases resulting in better final height outcome in some cohorts. Nevertheless, final height outcome in patients with CAH treated with glucocorticoids is lower than the population norm and also at the lower end of genetic potential. Achievement of regular adult height is still a challenge with conventional glucocorticoid treatment in patients with CAH, which is why new hydrocortisone formulations and new treatment options for CAH are underway.

42 CFR 409.68 - Guarantee of payment for inpatient hospital or inpatient CAH services furnished before...

Code of Federal Regulations, 2010 CFR

2010-10-01

... HOSPITAL INSURANCE BENEFITS Scope of Hospital Insurance Benefits § 409.68 Guarantee of payment for... 42 Public Health 2 2010-10-01 2010-10-01 false Guarantee of payment for inpatient hospital or inpatient CAH services furnished before notification of exhaustion of benefits. 409.68 Section 409.68 Public...

Field demonstration and evaluation of the Passive Flux Meter on a CAH groundwater plume.

PubMed

Verreydt, G; Annable, M D; Kaskassian, S; Van Keer, I; Bronders, J; Diels, L; Vanderauwera, P

2013-07-01

This study comprises the first application of the Passive Flux Meter (PFM) for the measurement of chlorinated aliphatic hydrocarbon (CAH) mass fluxes and Darcy water fluxes in groundwater at a European field site. The PFM was originally developed and applied to measurements near source zones. The focus of the PFM is extended from near source to plume zones. For this purpose, 48 PFMs of 1.4 m length were constructed and installed in eight different monitoring wells in the source and plume zone of a CAH-contaminated field site located in France. The PFMs were retrieved, sampled, and analyzed after 3 to 11 weeks of exposure time, depending on the expected contaminant flux. PFM evaluation criteria include analytical, technical, and practical aspects as well as conditions and applicability. PFM flux data were compared with so-called traditional soil and groundwater concentration data obtained using active sampling methods. The PFMs deliver reasonable results for source as well as plume zones. The limiting factor in the PFM applicability is the exposure time together with the groundwater flux. Measured groundwater velocities at the field site range from 2 to 41 cm/day. Measured contaminant flux data raise up to 13 g/m(2)/day for perchloroethylene in the plume zone. Calculated PFM flux averaged concentration data and traditional concentration data were of similar magnitude for most wells. However, both datasets need to be compared with reservation because of the different sampling nature and time. Two important issues are the PFM tracer loss during installation/extraction and the deviation of the groundwater flow field when passing the monitoring well and PFM. The demonstration of the PFM at a CAH-contaminated field site in Europe confirmed the efficiency of the flux measurement technique for source as well as plume zones. The PFM can be applied without concerns in monitoring wells with European standards. The acquired flux data are of great value for the purpose of site

42 CFR 410.27 - Therapeutic outpatient hospital or CAH services and supplies incident to a physician's or...

Code of Federal Regulations, 2013 CFR

2013-10-01

... 42 Public Health 2 2013-10-01 2013-10-01 false Therapeutic outpatient hospital or CAH services and supplies incident to a physician's or nonphysician practitioner's service: Conditions. 410.27 Section 410.27 Public Health CENTERS FOR MEDICARE & MEDICAID SERVICES, DEPARTMENT OF HEALTH AND HUMAN SERVICES...

42 CFR 410.27 - Therapeutic outpatient hospital or CAH services and supplies incident to a physician's or...

Code of Federal Regulations, 2012 CFR

2012-10-01

... 42 Public Health 2 2012-10-01 2012-10-01 false Therapeutic outpatient hospital or CAH services and supplies incident to a physician's or nonphysician practitioner's service: Conditions. 410.27 Section 410.27 Public Health CENTERS FOR MEDICARE & MEDICAID SERVICES, DEPARTMENT OF HEALTH AND HUMAN SERVICES...

42 CFR 410.27 - Therapeutic outpatient hospital or CAH services and supplies incident to a physician's or...

Code of Federal Regulations, 2014 CFR

2014-10-01

... 42 Public Health 2 2014-10-01 2014-10-01 false Therapeutic outpatient hospital or CAH services and supplies incident to a physician's or nonphysician practitioner's service: Conditions. 410.27 Section 410.27 Public Health CENTERS FOR MEDICARE & MEDICAID SERVICES, DEPARTMENT OF HEALTH AND HUMAN SERVICES...

Ehlers-Danlos Syndrome Caused by Biallelic TNXB Variants in Patients with Congenital Adrenal Hyperplasia.

PubMed

Chen, Wuyan; Perritt, Ashley F; Morissette, Rachel; Dreiling, Jennifer L; Bohn, Markus-Frederik; Mallappa, Ashwini; Xu, Zhi; Quezado, Martha; Merke, Deborah P

2016-09-01

Some variants that cause autosomal-recessive congenital adrenal hyperplasia (CAH) also cause hypermobility type Ehlers-Danlos syndrome (EDS) due to the monoallelic presence of a chimera disrupting two flanking genes: CYP21A2, encoding 21-hydroxylase, necessary for cortisol and aldosterone biosynthesis, and TNXB, encoding tenascin-X, an extracellular matrix protein. Two types of CAH tenascin-X (CAH-X) chimeras have been described with a total deletion of CYP21A2 and characteristic TNXB variants. CAH-X CH-1 has a TNXB exon 35 120-bp deletion resulting in haploinsufficiency, and CAH-X CH-2 has a TNXB exon 40 c.12174C>G (p.Cys4058Trp) variant resulting in a dominant-negative effect. We present here three patients with biallelic CAH-X and identify a novel dominant-negative chimera termed CAH-X CH-3. Compared with monoallelic CAH-X, biallelic CAH-X results in a more severe phenotype with skin features characteristic of classical EDS. We present evidence for disrupted tenascin-X function and computational data linking the type of TNXB variant to disease severity. © 2016 WILEY PERIODICALS, INC.

Community-acquired hyperkalemia in elderly patients: risk factors and clinical outcomes.

PubMed

Turgutalp, Kenan; Bardak, Simge; Helvacı, Ilter; İşgüzar, Gizem; Payas, Ezgi; Demir, Serap; Kıykım, Ahmet

2016-10-01

Although the risk and related factors of hyperkalemia developed in the hospital are known in elderly, risk and related factors of community-acquired hyperkalemia (CAH) in this population are not well known. This study was performed to investigate the risk of CAH in elderly and evaluate the related factors and clinical outcomes. Study design, setting and participants, intervention: Patients (aged ≥65 years) with hyperkalemia were screened. Group 1 (young-old); 65-74 years/old, Group 2 (middle-old); 75-84 years/old, Group 3 (oldest-old); ≥85 years/old, and Group 4 (control group); ≥65 years/old (normal serum potassium levels). The relation between CAH and hospital expenses (HE), the number of comorbid diseases (NCD), and all-cause of mortality rates (MR) were evaluated. We also investigated whether drugs, sex, and NCD are risk factors for the development of CAH. There was a positive correlation between serum potassium levels and length of hospital stay, MR, HE, and NCD (p < 0.001). Risk factors for CAH were the use of non-steroidal-anti inflammatory drugs (NSAIDs) (Odds Ratio [OR]: 2.679), spironolactone (OR: 2.530), and angiotensin converting enzyme inhibitors (ACEI) (OR: 2.242), angiotensin receptor blockers (ARB) (OR: 2.679), ≥2 comorbid diseases (OR: 2.221), female gender (OR: 2.112), and renal injury (OR: 5.55). CAH risk was found to be increased 30.03 times when any of ACEI, ARB, NSAIDs, or spironolactone is given to a patient with a renal injury. Use of NSAIDs, ACEI, ARB, spironolactone and increased NCD are all independent risk factors for CAH in the elderly, especially in patients with kidney diseases.

42 CFR 409.12 - Nursing and related services, medical social services; use of hospital or CAH facilities.

Code of Federal Regulations, 2013 CFR

2013-10-01

... 42 Public Health 2 2013-10-01 2013-10-01 false Nursing and related services, medical social... Inpatient Hospital Services and Inpatient Critical Access Hospital Services § 409.12 Nursing and related... (b) of this section, Medicare pays for nursing and related services, use of hospital or CAH...

42 CFR 409.12 - Nursing and related services, medical social services; use of hospital or CAH facilities.

Code of Federal Regulations, 2010 CFR

2010-10-01

... 42 Public Health 2 2010-10-01 2010-10-01 false Nursing and related services, medical social... Inpatient Hospital Services and Inpatient Critical Access Hospital Services § 409.12 Nursing and related... (b) of this section, Medicare pays for nursing and related services, use of hospital or CAH...

42 CFR 409.12 - Nursing and related services, medical social services; use of hospital or CAH facilities.

Code of Federal Regulations, 2012 CFR

2012-10-01

... 42 Public Health 2 2012-10-01 2012-10-01 false Nursing and related services, medical social... Inpatient Hospital Services and Inpatient Critical Access Hospital Services § 409.12 Nursing and related... (b) of this section, Medicare pays for nursing and related services, use of hospital or CAH...

42 CFR 409.12 - Nursing and related services, medical social services; use of hospital or CAH facilities.

Code of Federal Regulations, 2011 CFR

2011-10-01

... 42 Public Health 2 2011-10-01 2011-10-01 false Nursing and related services, medical social... Inpatient Hospital Services and Inpatient Critical Access Hospital Services § 409.12 Nursing and related... (b) of this section, Medicare pays for nursing and related services, use of hospital or CAH...

42 CFR 409.12 - Nursing and related services, medical social services; use of hospital or CAH facilities.

Code of Federal Regulations, 2014 CFR

2014-10-01

... 42 Public Health 2 2014-10-01 2014-10-01 false Nursing and related services, medical social... Inpatient Hospital Services and Inpatient Critical Access Hospital Services § 409.12 Nursing and related... (b) of this section, Medicare pays for nursing and related services, use of hospital or CAH...

Unusual phenotype of congenital adrenal hyperplasia (CAH) with a novel mutation of the CYP21A2 gene.

PubMed

Raisingani, Manish; Contreras, Maria F; Prasad, Kris; Pappas, John G; Kluge, Michelle L; Shah, Bina; David, Raphael

2016-07-01

Gonadotropin independent sexual precocity (SP) may be due to congenital adrenal hyperplasia (CAH), and its timing usually depends on the type of mutation in the CYP21A2 gene. Compound heterozygotes are common and express phenotypes of varying severity. The objective of this case report was to investigate the hormonal pattern and unusual genetic profile in a 7-year-old boy who presented with pubic hair, acne, an enlarged phallus, slightly increased testicular volume and advanced bone age. Clinical, hormonal and genetic studies were undertaken in the patient as well as his parents. We found elevated serum 17-hydroxyprogesterone (17-OHP) and androstenedione that were suppressed with dexamethasone, and elevated testosterone that actually rose after giving dexamethasone, indicating activity of the hypothalamic-pituitary-gonadal (HPG) axis. An initial search for common mutations was negative, but a more detailed genetic analysis of the CYP21A2 gene revealed two mutations including R341W, a non-classical mutation inherited from his mother, and g.823G>A, a novel not previously reported consensus donor splice site mutation inherited from his father, which is predicted to be salt wasting. However, the child had a normal plasma renin activity. He was effectively treated with low-dose dexamethasone and a GnRH agonist. His father was an unaffected carrier, but his mother had evidence of mild non-classical CAH. In a male child presenting with gonadotropin independent SP it is important to investigate adrenal function with respect to the androgen profile, and to carry out appropriate genetic studies.

Guidelines for the Development of Comprehensive Care Centers for Congenital Adrenal Hyperplasia: Guidance from the CARES Foundation Initiative

PubMed Central

Auchus, Richard J.; Witchel, Selma Feldman; Leight, Kelly R.; Aisenberg, Javier; Azziz, Ricardo; Bachega, Tânia A.; Baker, Linda A.; Baratz, Arlene B.; Baskin, Laurence S.; Berenbaum, Sheri A.; Breault, David T.; Cerame, Barbara I.; Conway, Gerard S.; Eugster, Erica A.; Fracassa, Stephanie; Gearhart, John P.; Geffner, Mitchell E.; Harris, Katharine B.; Hurwitz, Richard S.; Katz, Aviva L.; Kalro, Brinda N.; Lee, Peter A.; Alger Lin, Gretchen; Loechner, Karen J.; Marshall, Ian; Merke, Deborah P.; Migeon, Claude J.; Miller, Walter L.; Nenadovich, Tamara L.; Oberfield, Sharon E.; Pass, Kenneth A.; Poppas, Dix P.; Lloyd-Puryear, Michele A.; Quigley, Charmian A.; Riepe, Felix G.; Rink, Richard C.; Rivkees, Scott A.; Sandberg, David E.; Schaeffer, Traci L.; Schlussel, Richard N.; Schneck, Francis X.; Seely, Ellen W.; Snyder, Diane; Speiser, Phyllis W.; Therrell, Bradford L.; VanRyzin, Carol; Vogiatzi, Maria G.; Wajnrajch, Michael P.; White, Perrin C.; Zuckerman, Alan E.

2010-01-01

Patients with rare and complex diseases such as congenital adrenal hyperplasia (CAH) often receive fragmented and inadequate care unless efforts are coordinated among providers. Translating the concepts of the medical home and comprehensive health care for individuals with CAH offers many benefits for the affected individuals and their families. This manuscript represents the recommendations of a 1.5 day meeting held in September 2009 to discuss the ideal goals for comprehensive care centers for newborns, infants, children, adolescents, and adults with CAH. Participants included pediatric endocrinologists, internal medicine and reproductive endocrinologists, pediatric urologists, pediatric surgeons, psychologists, and pediatric endocrine nurse educators. One unique aspect of this meeting was the active participation of individuals personally affected by CAH as patients or parents of patients. Representatives of Health Research and Services Administration (HRSA), New York-Mid-Atlantic Consortium for Genetics and Newborn Screening Services (NYMAC), and National Newborn Screening and Genetics Resource Center (NNSGRC) also participated. Thus, this document should serve as a “roadmap” for the development phases of comprehensive care centers (CCC) for individuals and families affected by CAH. PMID:21274448

Laser Cooling the Diatomic Molecule CaH

NASA Astrophysics Data System (ADS)

Velasquez, Joe, III; Di Rosa, Michael

2014-06-01

To laser-cool a species, a closed (or nearly closed) cycle is required to dissipate translational energy through many directed laser-photon absorption and subsequent randomly-directed spontaneous emission events. Many atoms lend themselves to such a closed-loop cooling cycle. Attaining laser-cooled molecular species is challenging because of their inherently complex internal structure, yet laser-cooling molecules could lead to studies in interesting chemical dynamics among other applications. Typically, laser-cooled atoms are assembled into molecules through photoassociation or Feschbach resonance. CaH is one of a few molecules whose internal structure is quite atom-like, allowing a nearly closed cycle without the need for many repumping lasers. We will also present our work-to-date on laser cooling this molecule. We employ traditional pulsed atomic/molecular beam techniques with a laser vaporization source to generate species with well-defined translational energies over a narrow range of velocity. In this way, we can apply laser-cooling to most species in the beam along a single dimension (the beam's axis). This project is funded by the LDRD program of the Los Alamos National Laboratory.

Transformation rules and degradation of CAHs by Fentonlike oxidation in growth ring of water distribution network-A review

NASA Astrophysics Data System (ADS)

Zhong, D.; Ma, W. C.; Jiang, X. Q.; Yuan, Y. X.; Yuan, Y.; Wang, Z. Q.; Fang, T. T.; Huang, W. Y.

2017-08-01

Chlorinated hydrocarbons are widely used as organic solvent and chemical raw materials. After treatment, water polluted with trichloroethylene (TCE)/tetrachloroethylene (PCE) can reach the water quality requirements, while water with trace amounts of TCE/PCE is still harmful to humans, which will cause cancers. Water distribution network is an extremely complicated system, in which adsorption, desorption, flocculation, movement, transformation and reduction will occur, leading to changes of TCE/PCE concentrations and products. Therefore, it is important to investigate the transformation rules of TCE/PCE in water distribution network. What’s more, growth-ring, including drinking water pipes deposits, can act as catalysts in Fenton-like reagent (H2O2). This review summarizes the status of transformation rules of CAHs in water distribution network. It also evaluates the effectiveness and fruit of CAHs degradation by Fenton-like reagent based on growth-ring. This review is important in solving the potential safety problems caused by TCE/PCE in water distribution network.

Should we question early feminizing genitoplasty for patients with congenital adrenal hyperplasia and XX karyotype?

PubMed

Binet, A; Lardy, H; Geslin, D; Francois-Fiquet, C; Poli-Merol, M L

2016-03-01

There is a wide difference of opinion between the medical-surgical community and advocacy group regarding Disorders of Sexual Development (DSD) secondary to congenital adrenal hyperplasia (CAH) being ranked in the intersex category. This rupture is even more evident when the issue of genitoplasty is brought up. For physicians it is obvious and unequivocal that a person with CAH and an XX karyotype has a female gender identity, whereas associations tend to rank persons with CAH in the intersex category and advocate holding-off on surgical management. A retrospective case study vs. control group, spanning over 40years, included 21 patients who were treated in 3 different centers. Each patient and their parents were contacted independently and interviewed regarding interpersonal relationships, psychological impact of genitoplasty, gender identity and opinion on optimal care management for this disorder. Three couples controls (parent-child) per CAH patients were used and matched according to age, sex assigned at birth and ethnic origin. Sex assigned at birth seemed to concord with the gender identity perceived by the patients in 85.7% of cases. In fact, 89.7% of patients and 100% of parents felt that feminizing genitoplasty should be performed within the first year of life. There is however a significant difference compared to controls who felt that surgical management should occur later on in life. No difference was highlighted during childhood regarding parents-child relationships or social integration. However, during adolescence, the parents-child relationship tended to be significantly more painful for the CAH group. Integrating their parenting role was significantly harder for patients in the CAH-DSD group. In the population of CAH-DSD patients who had genitoplasty the level of sexual fulfillment was not lower to the one reported by the control group. Female sex assignment seems legitimate according to this study and the development of gender identity in these

Laser Spectroscopic Study of CaH in the B^2σ^+ and D^2σ^+ States

NASA Astrophysics Data System (ADS)

Watanabe, Kyohei; Uchida, Kanako; Kobayashi, Kaori; Matsushima, Fusakazu; Moriwaki, Yoshiki

2015-06-01

Calcium hydride is one of the abundant molecules in the stellar environment, and is considered as a probe of stellar analysis. Ab initio calculations have shown that the electronic excited states of CaH have complex potential curves. It is suggested that the B^2σ^+ state has an interesting double minimum potential due to the avoided crossing. Such a potential leads to drastic change of the rotational constants when the vibrational energy level goes across the potential barrier. Spectroscopic studies on CaH began in the 1920's, and many studies have been carried out since then. Bell et al. extensively assigned the D^2σ^+-X^2σ^+ bands in the UV region. Bernath's group has observed transitions in the IR and visible regions and identified their upper states as the A^2σ^+, B^2σ^+ and E^2σ^+ states. We have carried out a laser induced fluorescence (LIF) study in the UV region between 360 and 430 nm. We have produced CaH by using laser ablation of a calcium target in a hydrogen gas environment, then molecules have been excited by a second harmonic pulse of dye laser and the fluorescence from molecules have been detected through a monochromator. Detection of the D^2σ^+-X^2σ^+ bands already identified by Bell et al. indicates the production of CaH. In addition, many other bands have been also found and a few bands have been assigned by using the combination differences, the lower state of these bands have been confirmed to the vibrational ground state of X^2σ^+ state. We have tentatively assigned these bands as the B^2σ^+ -X^2σ^+ transition. We will discuss the assignment of these bands, together with the rotational constants comparing with those calculated from the ab initio potential. B. Barbuy, R. P. Schiavon, J. Gregorio-Hetem, P. D. Singh C. Batalha , Astron. Astrophys. Sippl. Ser. 101, 409 (1993). P. F. Weck and P. C .Stabcil, J. Chem. Phys. {118}, 9997 (2003). R. S. Mulliken, Phys. Rev. {25}, 509 (1925). G. D. Bell, M, Herman, J. W. C. Johns, and E. R

Growth hormone therapy alone or in combination with gonadotropin-releasing hormone analog therapy to improve the height deficit in children with congenital adrenal hyperplasia.

PubMed

Quintos, J B; Vogiatzi, M G; Harbison, M D; New, M I

2001-04-01

Short stature in the adult patient with congenital adrenal hyperplasia (CAH) is commonly seen, even among patients in excellent adrenal control during childhood and puberty. In this study we examine the effect of GH therapy on height prediction in children with both CAH and compromised height prediction. Leuprolide acetate, a GnRH analog (GnRHa), was given to patients with evidence of early puberty. GH (n = 12) or the combination of GH and GnRHa (n = 8) was administered to 20 patients with CAH while they continued therapy with glucocorticoids. Each patient in the treatment group was matched according to age, sex, bone age, puberty, and type of CAH with another CAH patient treated only with glucocorticoid replacement. The match was made at the start of GH treatment. Of the 20 patients, 12 have completed 2 yr of therapy. After 1 yr of GH or combination GH and GnRHa therapy, the mean growth rate increased from 5 +/- 1.9 to 7.8 +/- 1.6 cm/yr vs. 5.4 +/- 1.7 to 5 +/- 2 cm/yr in the group not receiving GH (P < 0.0001). During the second year of treatment, the mean growth rate was 6 +/- 1.6 vs. 4.2 +/- 2.1 cm/yr in the group not receiving GH (P < 0.001). The height SD score for chronological age in the treatment group at the end of 1 and 2 yr of treatment improved significantly more than the nontreatment group (P < 0.01). A similar improvement in the height SD score for bone age was found in the treatment group after 1 (-1.4 +/- 0.9 vs. -1.7 +/- 0.9; P < 0.0001) and 2 yr of therapy (-0.67 +/- 0.68 vs. -1.7 +/- 1.2; P < 0.0004). The mean predicted adult height improved from 159 +/- 11 (baseline) to 170 +/- 7.5 cm (after 2 yr of therapy) closely approximating target height (173 +/- 8 cm). All patients continued the hydrocortisone treatment. In patients with CAH and compromised height prediction, treatment with GH or the combination of GH and GnRHa results in an improvement of growth rate and height prediction and a reduction in height deficit for bone age.

Einstein A coefficients for rovibronic lines of the A2Π → X2Σ+ and B2Σ+ → X2Σ+ transitions of CaH and CaD

NASA Astrophysics Data System (ADS)

Alavi, S. Fatemeh; Shayesteh, Alireza

2018-02-01

Calcium monohydride is an important diatomic molecule appearing in the spectra of sunspots and M dwarfs. We report complete line lists with Einstein A coefficients for the A2Π-X2Σ+ and B2Σ+-X2Σ+ electronic transitions of CaH and CaD radicals. The most recent ab initio transition dipole moments and potential energy curves were used for the calculation of vibronic band intensities, taking the Herman-Wallis effect into account, and the rotational line strengths were calculated using the PGOPHER program of Western. For the A2Π and B2Σ+ excited states of CaH and CaD, new off-diagonal electronic matrix elements were included in the Hamiltonian matrix, and new sets of spectroscopic constants were determined in order to accurately reproduce the line positions and relative intensities of the observed branches in laboratory spectra. For both CaH and CaD isotopologues, Einstein A coefficients were calculated for all possible rovibronic transitions from the v΄ = 0-3 vibrational levels of the A2Π state and the v΄ = 0-2 vibrational levels of the B2Σ+ state to the v″ = 0-4 vibrational levels of the X2Σ+ ground state. The line lists and intensities reported here can be used to accurately determine the amounts of CaH and CaD in stellar environments.

Structure and stability of CaH 2 surfaces: on the possibility of electron-rich surfaces in metal hydrides for catalysis

DOE Office of Scientific and Technical Information (OSTI.GOV)

Ong, Phuong-Vu; Johnson, Lewis E.; Hosono, Hideo

Structure, thermodynamic stability, and electronic properties of CaH 2 surfaces in (001),(110), and (111) crystallographic orientations are investigated using ab initio modeling. We show that stoichiometric surfaces terminated with a hydrogen atomic plane are the most energetically favorable and discuss properties of hydrogen vacancies (VH) at these surfaces. The average calculated work function of the most stable pristine surfaces (~5.2 eV) is in agree-ment with experimental data for powder samples. Neutral hydrogen vacancies host localized electrons and induce defect states in the band gap, thereby shifting the effective work function to much lower values of ~2.7 eV. Surface VH aremore » predicted to aggregate into dimers and form electron-rich centers (e -)Ca 2+(e -) stable to over 800 K. These results suggest that hydrogen-deficient surfaces of CaH 2 can host a large concentration of localized electrons and, thus, give rise to new catalytic functionalities involving electron transfer between the surface, catalysts supported on it, and reacting species.« less

DECHLORINATION OF PCBS, CAHS, HERBICIDES AND PESTICIDES NEAT AND IN SOILS AT 25&DEG;C USING NA/NH3. (R829421E01)

EPA Science Inventory

Na/NH₃ reductions have been used to dehalogenate polychlorinated biphenyls (PCBs), chlorinated aliphatic hydrocarbons (CAHs) and pesticides at diffusion controlled rates at room temperature in model compound studies in both dry NH₃ and when water was adde...

Gender of rearing and psychosocial aspect in 46 XX congenital adrenal hyperplasia

PubMed Central

Gangaher, Arushi; Jyotsna, Viveka P.; Chauhan, Vasundhera; John, Jomimol; Mehta, Manju

2016-01-01

Background: In congenital adrenal hyperplasia (CAH) with ambiguous genitalia, assigning gender of rearing can be complex, especially If genitalia is highly virilized. Apart from karyotype, prenatal androgen exposure, patient's gender orientation, sociocultural, and parental influences play a role. The aim of this study was to assess gender dysphoria and psychosocial issues in patients of CAH raised as males and females. Materials and Methods: This is a cross-sectional study that includes patients (old and new) with CAH who were treated by us in the last 6 months. A semi-structured interview proforma was used to elicit history and psychosocial background of the patients. The clinical and biochemical details were noted. For psychological analysis, patients were screened for gender dysphoria using Parent Report Gender Identity Questionnaire for children <12 years and Gender Identity/Gender Dysphoria Questionnaire for Adolescents and Adults. Results: We analyzed 22 46 XX CAH patients among which, 3 were reared as males and 19 as females. Among the 19 patients reared as females, 17 patients showed no gender dysphoria. Two patients revealed gender dysphoria as indicated by their marginally low scores on the gender dysphoria assessment. However, in view of current literature and the age groups of the patients, behavior of the 6-year-old patient can be best understood as being tomboyish. Gender dysphoria in the 22-year-old can be explained by the dominance of psychosocial factors and not hormones alone. Among the three patients reared as males, two prepubertal were satisfied with their male gender identity. The third patient, aged 32 years, had gender dysphoria when reared as a male that resolved when gender was reassigned as female and feminizing surgery was done. Conclusion: Gender assignment in 46 XX CAH is guided by factors such as degree of virilization of genitalia, gender orientation, patient involvement, sociocultural, and parental influences. PMID:27867895

Gender of rearing and psychosocial aspect in 46 XX congenital adrenal hyperplasia.

PubMed

Gangaher, Arushi; Jyotsna, Viveka P; Chauhan, Vasundhera; John, Jomimol; Mehta, Manju

2016-01-01

In congenital adrenal hyperplasia (CAH) with ambiguous genitalia, assigning gender of rearing can be complex, especially If genitalia is highly virilized. Apart from karyotype, prenatal androgen exposure, patient's gender orientation, sociocultural, and parental influences play a role. The aim of this study was to assess gender dysphoria and psychosocial issues in patients of CAH raised as males and females. This is a cross-sectional study that includes patients (old and new) with CAH who were treated by us in the last 6 months. A semi-structured interview proforma was used to elicit history and psychosocial background of the patients. The clinical and biochemical details were noted. For psychological analysis, patients were screened for gender dysphoria using Parent Report Gender Identity Questionnaire for children <12 years and Gender Identity/Gender Dysphoria Questionnaire for Adolescents and Adults. We analyzed 22 46 XX CAH patients among which, 3 were reared as males and 19 as females. Among the 19 patients reared as females, 17 patients showed no gender dysphoria. Two patients revealed gender dysphoria as indicated by their marginally low scores on the gender dysphoria assessment. However, in view of current literature and the age groups of the patients, behavior of the 6-year-old patient can be best understood as being tomboyish. Gender dysphoria in the 22-year-old can be explained by the dominance of psychosocial factors and not hormones alone. Among the three patients reared as males, two prepubertal were satisfied with their male gender identity. The third patient, aged 32 years, had gender dysphoria when reared as a male that resolved when gender was reassigned as female and feminizing surgery was done. Gender assignment in 46 XX CAH is guided by factors such as degree of virilization of genitalia, gender orientation, patient involvement, sociocultural, and parental influences.

Emergency Department Telemedicine Is Used for More Severely Injured Rural Trauma Patients, but Does Not Decrease Transfer: A Cohort Study.

PubMed

Mohr, Nicholas M; Harland, Karisa K; Chrischilles, Elizabeth A; Bell, Amanda; Shane, Dan M; Ward, Marcia M

2017-02-01

Traumatic injury is a leading cause of death in the United States, and rural populations are at increased risk of injury and death. Rural residents have limited access to trauma care, and telemedicine has been proposed as one strategy to improve the provision of trauma care locally. The objective of this study was to describe patient-level factors associated with telemedicine consultation in North Dakota critical-access hospital (CAH) emergency departments (EDs) and to measure the association between telemedicine consultation and interhospital transfer. Observational cohort study of all adult (age ≥ 18 years) trauma patients treated in North Dakota CAH EDs with an active telemedicine subscription between 2008 and 2014. Trauma cases were identified from the North Dakota Trauma Registry, and telemedicine-enabled care was determined using a probabilistic linking algorithm with the call records of the predominant telemedicine network in North Dakota. Multivariable generalized estimating equations were used to identify factors associated with telemedicine consultation and to measure the association between telemedicine consultation and interhospital transfer, adjusting for patient, injury, and hospital factors. Of the 9,281 North Dakota trauma patients seen in CAHs, 2,837 were treated in an ED with an active telemedicine subscription. Telemedicine was consulted for 11% of all trauma patients in telemedicine-capable EDs. Factors associated with telemedicine consultation included higher Injury Severity Score, penetrating injuries, burns, hypotension, tachycardia, and ambulance transport. Adjusting for severity of illness, injury mechanism, and type of injury, telemedicine use was not associated with interhospital transfer (adjusted odds ratio = 1.28, 95% confidence interval = 0.94 to 1.75). Emergency department-based telemedicine consultation is requested for the most severely injured rural trauma patients, especially with those with penetrating trauma, burns

Shorter survival in adolescent and young adult patients, compared to adult patients, with stage IV colorectal cancer in Japan.

PubMed

Shida, Dai; Ahiko, Yuka; Tanabe, Taro; Yoshida, Takefumi; Tsukamoto, Shunsuke; Ochiai, Hiroki; Takashima, Atsuo; Boku, Narikazu; Kanemitsu, Yukihide

2018-03-27

The incidence of colorectal cancer in adolescent and young adult patients is increasing. However, survival and clinical features of young patients, especially those with stage IV disease, relative to adult patients remain unclear. This retrospective single-institution cohort study was conducted at a tertiary care cancer center. Subjects were 861 consecutive patients who were diagnosed with stage IV colorectal cancer at the age of 15 to 74 years and who were referred to the division of surgery or gastrointestinal oncology at the National Cancer Center Hospital from 1999 to 2013. Overall survival (OS) was investigated and clinicopathological variables were analyzed for prognostic significance. Of these, 66 (8%) were adolescent and young adult patients and 795 (92%) were adult patients. Median survival time was 13.6 months in adolescent and young adult patients and 22.4 months in adult patients, and 5-year OS rates were 17.3% and 20.3%, respectively, indicating significant worse prognosis of adolescent and young adult patients (p = 0.042). However, age itself was not an independent factor associated with prognosis by multivariate analysis. When compared with adult patients, adolescent and young adult patients consisted of higher proportion of the patients who did not undergo resection of primary tumor, which was an independent factor associated with poor prognosis in multivariate analysis. In patients who did not undergo resection (n = 349), OS of adolescent and young adult patients were significantly worse (p = 0.033). Prognoses were worse in adolescent and young adult patients with stage IV colorectal cancer compared to adult patients in Japan, due to a higher proportion of patients who did not undergo resection with more advanced and severe disease, but not due to age itself.

Lipoid congenital adrenal hyperplasia due to STAR mutations in a Caucasian patient.

PubMed

Kaur, Jasmeet; Casas, Luis; Bose, Himangshu S

2016-01-01

Lipoid congenital adrenal hyperplasia (lipoid CAH), the most severe form of CAH, is most commonly caused by mutations in steroidogenic acute regulatory protein (STAR), which is required for the movement of cholesterol from the outer to the inner mitochondrial membranes to synthesize pregnenolone. This study was performed to evaluate whether the salt-losing crisis and the adrenal inactivity experienced by a Scandinavian infant is due to a de novo STAR mutation. The study was conducted at the University of North Dakota, the Mercer University School of Medicine and the Memorial University Medical Center to identify the cause of this disease. The patient was admitted to a pediatric endocrinologist at the Sanford Health Center for salt-losing crisis and possible adrenal failure. Lipoid CAH is an autosomal recessive disease, we identified two de novo heterozygous mutations (STAR c.444C>A (STAR p.N148K) and STAR c.557C>T (STAR p.R193X)) in the STAR gene, causing lipoid CAH. New onset lipoid CAH can occur through de novo mutations and is not restricted to any specific region of the world. This Scandinavian family was of Norwegian descent and had lipoid CAH due to a mutation in S TAR exons 4 and 5. Overexpression of the STAR p.N148K mutant in nonsteroidogenic COS-1 cells supplemented with an electron transport system showed activity similar to the background level, which was ∼10% of that observed with wild-type (WT) STAR. Protein-folding analysis showed that the finger printing of the STAR p.N148K mutant is also different from the WT protein. Inherited STAR mutations may be more prevalent in some geographical areas but not necessarily restricted to those regions. STAR mutations cause lipoid CAH.This is a pure population from a caucasian family.Mutation ablated STAR activity.The mutation resulted in loosely folded conformation of STAR.

Complete virilization in congenital adrenal hyperplasia: clinical course, medical management and disease-related complications.

PubMed

Woelfle, J; Hoepffner, W; Sippell, W G; Brämswig, J H; Heidemann, P; Deiss, D; Bökenkamp, A; Roth, C; Irle, U; Wollmann, H A; Zachmann, M; Kubini, K; Albers, N

2002-02-01

adult patients. Patients tended to follow height percentiles of genetic females. One pubertal patient was suicidal due to short stature. (4) Central precocious puberty (CPP): prolonged exposition to adrenal androgens led to CPP in one patient. He was treated with GnRH analogues until gonadectomy. Patients with CAH and complete virilization have a high risk of being diagnosed late. There are major problems and uncertainties of the patients' families and the treating physicians concerning gender assignment. Gender identity is disturbed in some patients. In addition, multiple surgical procedures are necessary and short stature as well as central precocious puberty might be important to avoid late sequelae. While some surgical interventions are probably unavoidable, most of these issues could be resolved with an early diagnosis. Thus, especially for these patients, a neonatal screening programme for CAH would be of paramount importance.

Neutron production at 0° from the 40Ca+H reaction at Elab=357A and 565A MeV

NASA Astrophysics Data System (ADS)

Tuvè, C.; Albergo, S.; Boemi, D.; Caccia, Z.; Chen, C.-X.; Costa, S.; Crawford, H. J.; Cronqvist, M.; Engelage, J.; Greiner, L.; Guzik, T. G.; Insolia, A.; Knott, C. N.; Lindstrom, P. J.; Mitchell, J. W.; Potenza, R.; Reito, S.; Romanski, J.; Russo, G. V.; Soutoul, A.; Testard, O.; Tull, C. E.; Waddington, C. J.; Webber, W. R.; Wefel, J. P.

1997-08-01

Neutrons produced in the 40Ca+H reaction at Elab=357A and 565A MeV have been detected using a three-module version of the multifunctional neutron spectrometer MUFFINS. The detector covered a narrow angular range around the beam in the forward direction (0°-3.2°). Semi-inclusive neutron production cross sections, at the two energies, are reported together with neutron energy spectra, angular, rapidity, and transverse momentum distributions. Comparison with a Boltzmann-Nordheim-Vlasov approach + phase space coalescence model is discussed.

Ca(AlH4)2, CaAlH5, and CaH2+6LiBH4: Calculated dehydrogenation enthalpy, including zero point energy, and the structure of the phonon spectra.

PubMed

Marashdeh, Ali; Frankcombe, Terry J

2008-06-21

The dehydrogenation enthalpies of Ca(AlH(4))(2), CaAlH(5), and CaH(2)+6LiBH(4) have been calculated using density functional theory calculations at the generalized gradient approximation level. Harmonic phonon zero point energy (ZPE) corrections have been included using Parlinski's direct method. The dehydrogenation of Ca(AlH(4))(2) is exothermic, indicating a metastable hydride. Calculations for CaAlH(5) including ZPE effects indicate that it is not stable enough for a hydrogen storage system operating near ambient conditions. The destabilized combination of LiBH(4) with CaH(2) is a promising system after ZPE-corrected enthalpy calculations. The calculations confirm that including ZPE effects in the harmonic approximation for the dehydrogenation of Ca(AlH(4))(2), CaAlH(5), and CaH(2)+6LiBH(4) has a significant effect on the calculated reaction enthalpy. The contribution of ZPE to the dehydrogenation enthalpies of Ca(AlH(4))(2) and CaAlH(5) calculated by the direct method phonon analysis was compared to that calculated by the frozen-phonon method. The crystal structure of CaAlH(5) is presented in the more useful standard setting of P2(1)c symmetry and the phonon density of states of CaAlH(5), significantly different to other common complex metal hydrides, is rationalized.

Ca(AlH4)2, CaAlH5, and CaH2+6LiBH4: Calculated dehydrogenation enthalpy, including zero point energy, and the structure of the phonon spectra

NASA Astrophysics Data System (ADS)

Marashdeh, Ali; Frankcombe, Terry J.

2008-06-01

The dehydrogenation enthalpies of Ca(AlH4)2, CaAlH5, and CaH2+6LiBH4 have been calculated using density functional theory calculations at the generalized gradient approximation level. Harmonic phonon zero point energy (ZPE) corrections have been included using Parlinski's direct method. The dehydrogenation of Ca(AlH4)2 is exothermic, indicating a metastable hydride. Calculations for CaAlH5 including ZPE effects indicate that it is not stable enough for a hydrogen storage system operating near ambient conditions. The destabilized combination of LiBH4 with CaH2 is a promising system after ZPE-corrected enthalpy calculations. The calculations confirm that including ZPE effects in the harmonic approximation for the dehydrogenation of Ca(AlH4)2, CaAlH5, and CaH2+6LiBH4 has a significant effect on the calculated reaction enthalpy. The contribution of ZPE to the dehydrogenation enthalpies of Ca(AlH4)2 and CaAlH5 calculated by the direct method phonon analysis was compared to that calculated by the frozen-phonon method. The crystal structure of CaAlH5 is presented in the more useful standard setting of P21/c symmetry and the phonon density of states of CaAlH5, significantly different to other common complex metal hydrides, is rationalized.

Syndrome-Related Stigma in the General Social Environment as Reported by Women with Classical Congenital Adrenal Hyperplasia.

PubMed

Meyer-Bahlburg, Heino F L; Reyes-Portillo, Jazmin A; Khuri, Jananne; Ehrhardt, Anke A; New, Maria I

2017-02-01

Stigma defined as "undesired differentness" (Goffman, 1963) and subtyped as "experienced" or "enacted," "anticipated," and "internalized" has been documented for patients with diverse chronic diseases. However, no systematic data exist on the association of stigma with somatic intersexuality. The current report concerns women with classical congenital adrenal hyperplasia (CAH), the most prevalent intersex syndrome, and provides descriptive data on CAH-related stigma as experienced in the general social environment (excluding medical settings and romantic/sexual partners) during childhood, adolescence, and adulthood. A total of 62 adult women with classical CAH [41 with the salt-wasting (SW) variant and 21 with the simple-virilizing (SV) variant] underwent a qualitative retrospective interview, which focused on the impact of CAH and its medical treatment on many aspects of women's lives. Deductive content analysis was performed on the transcribed texts. The women's accounts of CAH-related stigma were identified and excerpted as vignettes, and the vignettes categorized according to social context, stigma type, and the associated features of the CAH condition. Nearly two-thirds of women with either variant of CAH provided stigma vignettes. The vignettes included all three stigma types, and most involved some somatic or behavioral feature related to sex or gender. Stigma situations were reported for all ages and all social contexts of everyday life: family, peers, colleagues at work, strangers, and the media. We conclude that there is a need for systematic documentation of stigma in intersexuality as a basis for the development of improved approaches to prevention and intervention.

Molecular defects of the CYP21A2 gene in Greek-Cypriot patients with congenital adrenal hyperplasia.

PubMed

Skordis, Nicos; Kyriakou, Andreas; Tardy, Véronique; Ioannou, Yiannis S; Varvaresou, Athanasia; Dracopoulou-Vabouli, Maria; Patsalis, Philippos C; Shammas, Christos; Neocleous, Vassos; Phylactou, Leonidas A

2011-01-01

To determine the mutations in the CYP21A2 gene in Greek-Cypriots with congenital adrenal hyperplasia (CAH) and attempt a genotype-phenotype correlation. Molecular analysis was performed by multiplex ligation-dependent probe amplification and direct sequencing of PCR products of the CYP21A2 gene in 32 CAH patients. The most frequent genetic defect in the classic salt-wasting and simple virilizing forms was the IVS2-13A/C>G (55%) mutation, followed by Large lesion (20%) and in the non-classical form, the p.V281L (79.5%). Genotypes were categorized in 4 mutation groups (null, A, B and C). All 3 patients in the null group manifested the salt-wasting form and all 6 patients in mutation group A presented with the classical form. One patient in group B had the simple virilizing form and 22 patients in group C exhibited the non-classical form. The spectrum of mutations of the CYP21A2 gene in our population is comparable to the most common reported in similar ethnic groups. The knowledge of the ethnic specificity of the CYP21A2 mutations represents a valuable diagnostic tool for all forms of CAH. Copyright © 2010 S. Karger AG, Basel.

Complement component 4 copy number variation and CYP21A2 genotype associations in patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency.

PubMed

Chen, Wuyan; Xu, Zhi; Nishitani, Miki; Van Ryzin, Carol; McDonnell, Nazli B; Merke, Deborah P

2012-12-01

Congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency (21-OHD) is an autosomal recessive disorder of cortisol biosynthesis caused by CYP21A2 mutations. An increase in gene copy number variation (CNV) exists at the CYP21A2 locus. CNV of C4, a neighboring gene that encodes complement component 4, is associated with autoimmune disease susceptibility. In this study, we performed comprehensive genetic analysis of the RP-C4-CYP21-TNX (RCCX) region in 127 unrelated 21-OHD patients (100 classic, 27 nonclassic). C4 copy number was determined by Southern blot. C4 CNV and serum C4 levels were evaluated in relation to CYP21A2 mutations and relevant phenotypes. We found that the most common CYP21A2 mutation associated with the nonclassic form of CAH, V281L, was associated with high C4 copy number (p = 7.13 × 10(-16)). Large CYP21A2 deletion, a common mutation associated with the classic form of CAH, was associated with low C4 copy number (p = 1.61 × 10(-14)). Monomodular RCCX with a short C4 gene, a risk factor for autoimmune disease, was significantly less frequent in CAH patients compared to population estimates (2.8 vs. 10.6 %; p = 1.08 × 10(-4)). In conclusion, CAH patients have increased C4 CNV, with mutation-specific associations that may be protective for autoimmune disease. The study of CYP21A2 in relation to neighboring genes provides insight into the genetics of CNV hotspots, an important determinant of human health.

Endoplasmic reticulum stress in complex atypical hyperplasia as a possible predictor of occult carcinoma and progestin response.

PubMed

Tierney, Katherine E; Ji, Lingyun; Dralla, Shannon S; Yoo, Eunjeong; Yessaian, Annie; Pham, Huyen Q; Roman, Lynda; Sposto, Richard; Mhawech-Fauceglia, Paulette; Lin, Yvonne G

2016-12-01

Glucose-regulated protein (GRP)-78, the key regulator of endoplasmic reticulum (ER) stress, is associated with endometrial cancer (EC) development and progression. However, its role in the continuum from complex atypical hyperplasia (CAH) to EC is unknown and the focus of this study. 252 formalin-fixed, paraffin-embedded endometrial biopsies from patients with CAH diagnosed between 2003 and 2011 were evaluated for GRP78 expression by immunohistochemistry. Expression was also evaluated in subsequent biopsies from those patients treated with progestins. Differences in GRP78 expression were assessed using standard statistical methods. GRP78 expression was undetectable in 45(18%) patients with CAH, while 120(48%) CAH cases showed moderate/strong expression. Among women who ultimately underwent hysterectomy for CAH (n=134), 54(40%) had occult EC while 57(43%) had persistent CAH. Those with occult EC upon hysterectomy had significantly stronger GRP78 expression than those who did not have occult EC (p=0.007). Greater GRP78 expression within CAH remained independently associated with the presence of an occult EC (p=0.017). Thirty-four of 54 (63%) patients with occult EC had moderate/strong GRP78 expression compared to 36 of 80 (45%) patients with persistent CAH, benign or non-atypical hyperplastic endometrium. In those treated with progestins, samples with persistent CAH and EC were more likely to have high levels of GRP78 expression in the initial biopsies than those who responded (p=0.014). Increased GRP78 expression in untreated CAH correlates with the presence of an occult EC. In addition, CAH specimens with greater GRP78 expression may identify patients who are less likely to respond to progestin therapy. Copyright © 2016 Elsevier Inc. All rights reserved.

Sleep Disorders in Adult Sickle Cell Patients

PubMed Central

Sharma, Sunil; Efird, Jimmy T.; Knupp, Charles; Kadali, Renuka; Liles, Darla; Shiue, Kristin; Boettger, Peter; Quan, Stuart F.

2015-01-01

Study Objectives: While sleep apnea has been studied in children with sickle cell disease (SCD), little is known about sleep disorders in adult sickle cell patients. The objective of this study was to evaluate sleep disordered breathing and its polysomnographic characteristics in adult patients with sickle cell disease. Methods: The analysis cohort included 32 consecutive adult SCD patients who underwent a comprehensive sleep evaluation and overnight polysomnography in an accredited sleep center after reporting symptoms suggesting disordered sleep or an Epworth Sleepiness Scale score ≥ 10. Epworth score, sleep parameters, comorbid conditions, and narcotic use were reviewed and compared in patients with and without sleep disordered breathing. SCD complication rates in the two groups also were compared. Results: In adult SCD patients who underwent overnight polysomnography, we report a high prevalence (44%) of sleep disordered breathing. Disease severity was mild to moderate (mean apnea-hypopnea index = 17/h (95% CI: 10–24/h). Concomitant sleep disorders, including insomnia complaints (57%) and delayed sleep-phase syndrome (57%), also were common in this population. In this limited cohort, we did not find increased SCD complications associated with sleep disordered breathing in adult patients with sickle cell disease. Conclusions: A high burden of sleep disordered breathing and other sleep-related complaints were identified in the adult sickle cell population. Our results provide important information on this unique population. Citation: Sharma S, Efird JT, Knupp C, Kadali R, Liles D, Shiue K, Boettger P, Quan SF. Sleep disorders in adult sickle cell patients. J Clin Sleep Med 2015;11(3):219–223. PMID:25515282

Recommendations for Treatment of Nonclassic Congenital Adrenal Hyperplasia (NCCAH): an Update

PubMed Central

Trapp, Christine M.; Oberfield, Sharon E.

2013-01-01

Congenital adrenal hyperplasia (CAH) is a family of autosomal recessive disorders. 21-hydroxylase deficiency, in which there are mutations in CYP21A2 (the gene encoding the adrenal 21-hydroxylase enzyme), is the most common form (90%) of CAH. In classic CAH there is impaired cortisol production with diagnostic increased levels of 17-OH progesterone. Excess androgen production results in virilization and in the newborn female may cause development of ambiguous external genitalia. Three-fourths of patients with classic CAH also have aldosterone insufficiency, which can result in salt-wasting; in infancy this manifests as shock, hyponatremia and hyperkalemia. CAH has a reported incidence of 1:10,000–1:20,000 births although there is an increased prevalence in certain ethnic groups. Nonclassic CAH (NCCAH) is a less severe form of the disorder, in which there is 20–50% of 21-hydroxylase enzyme activity (vs. 0–5% in classic CAH) and no salt wasting. The degree of symptoms related to androgen excess is variable and may be progressive with age, although some individuals are asymptomatic. NCCAH has an incidence of 1:1000–1:2000 births (0.1–0.2% prevalence) in the White population; an even higher prevalence is noted in certain ethnic groups such as Ashkenazi Jews (1–2%). As many as two-thirds of persons with NCCAH are compound heterozygotes and carry a severe and mild mutation on different alleles. This paper discusses the genetics of NCCAH, along with its variable phenotypic expression, and reviews the clinical course in untreated patients, which includes rapid early childhood growth, advanced skeletal age, premature adrenarche, acne, impaired reproductive function in both sexes and hirsutism as well as menstrual disorders in females. Finally, it addresses treatment with glucocorticoids vs. and other alternatives, particularly with respect to long term issues such as adult metabolic disease including insulin resistance, cardiovascular disease, metabolic syndrome

42 CFR 485.641 - Condition of participation: Periodic evaluation and quality assurance review.

Code of Federal Regulations, 2013 CFR

2013-10-01

... physicians and practitioners providing telemedicine services to the CAH's patients under a written agreement... physicians and practitioners providing telemedicine services to the CAH's patients under a written agreement between the CAH and a distant-site telemedicine entity, one of the entities listed in paragraphs (b)(4)(i...

42 CFR 485.641 - Condition of participation: Periodic evaluation and quality assurance review.

Code of Federal Regulations, 2014 CFR

2014-10-01

... physicians and practitioners providing telemedicine services to the CAH's patients under a written agreement... physicians and practitioners providing telemedicine services to the CAH's patients under a written agreement between the CAH and a distant-site telemedicine entity, one of the entities listed in paragraphs (b)(4)(i...

42 CFR 485.641 - Condition of participation: Periodic evaluation and quality assurance review.

Code of Federal Regulations, 2012 CFR

2012-10-01

... physicians and practitioners providing telemedicine services to the CAH's patients under a written agreement... physicians and practitioners providing telemedicine services to the CAH's patients under a written agreement between the CAH and a distant-site telemedicine entity, one of the entities listed in paragraphs (b)(4)(i...

42 CFR 485.641 - Condition of participation: Periodic evaluation and quality assurance review.

Code of Federal Regulations, 2011 CFR

2011-10-01

... physicians and practitioners providing telemedicine services to the CAH's patients under a written agreement... physicians and practitioners providing telemedicine services to the CAH's patients under a written agreement between the CAH and a distant-site telemedicine entity, one of the entities listed in paragraphs (b)(4)(i...

Diagnosis and management of classical congenital adrenal hyperplasia.

PubMed

Marumudi, Eunice; Khadgawat, Rajesh; Surana, Vineet; Shabir, Iram; Joseph, Angela; Ammini, Ariachery C

2013-08-01

Congenital adrenal hyperplasia (CAH) is among the most common genetic disorders. Deficiency of adrenal steroid 21-hydroxylase deficiency due to mutations in the CYP21A2 gene accounts for about 95% cases of CAH. This disorder manifests with androgen excess with or without salt wasting. It also is a potentially life threatening disorder; neonatal screening with 17-hydroxyprogesterone measurement can diagnose the condition in asymptomatic children. Carefully monitored therapy with glucocorticoid and mineralocorticoid supplementation will ensure optimal growth and development for children with CAH. Genital surgery may be required for girls with CAH. Continued care is required for individuals with CAH as adults to prevent long-term adverse consequences of the disease, including infertility, metabolic syndrome and osteoporosis. Copyright © 2013 Elsevier Inc. All rights reserved.

Reproductive and oncologic outcomes after progestin therapy for endometrial complex atypical hyperplasia or carcinoma.

PubMed

Kudesia, Rashmi; Singer, Tomer; Caputo, Thomas A; Holcomb, Kevin Michael; Kligman, Isaac; Rosenwaks, Zev; Gupta, Divya

2014-03-01

This study evaluated fertility and oncological outcomes in women with complex atypical hyperplasia (CAH) or nonmyoinvasive grade 1 endometrioid endometrial carcinoma (EM) who desired fertility-sparing therapy. The retrospective cohort study included women younger than 45 years with CAH or EM who desired fertility-sparing treatment at our institution. Only patients for whom both oncological treatment and pregnancy outcomes were available were included. Statistical analyses were performed using a Fisher exact test, Pearson χ(2) test, and Spearman rank correlation test, as appropriate. Seventy-five patients were identified, and 23 (13 CAH, 10 EM) met the inclusion criteria. All 23 patients had at least 1 prior pregnancy. Treatment was split between oral progesterone only (38.5% CAH, 40% EM), levonorgestrel intrauterine device only (30.8% CAH, 20% EM), and both (30.8% CAH, 40% EM). After a median follow-up of 13 months (range, 3-74 months), 9 patients (46.2% CAH, 30% EM, P = .39) had persistent/progressive disease. Eight patients (30.8% CAH, 40% EM, P = .69) ultimately had a hysterectomy, and 3 of these (13.0%) were found to have persistent/progressive disease. Median time from diagnosis to hysterectomy was 13 months (range, 4-56 months). Fourteen of the 23 patients utilized assisted reproductive techniques (60.9%); 12 underwent IVF and 2 chose a gestation carrier. Seven clinical intrauterine pregnancies (30.4%) resulting in 6 live births (26.1%) were found in the entire cohort. Fertility-sparing treatment for CAH and grade 1 endometrial cancer is feasible with progestin therapy and leads to clinically meaningful rates of pregnancy in young women who desire fertility. Copyright © 2014 Mosby, Inc. All rights reserved.

Adrenal rest tissue in gonads of patients with classical congenital adrenal hyperplasia: multicenter study of 45 French male patients.

PubMed

Pierre, Peggy; Despert, François; Tranquart, François; Coutant, Régis; Tardy, Véronique; Kerlan, Véronique; Sonnet, Emmanuel; Baron, Sabine; Lorcy, Yannick; Emy, Philippe; Delavierre, Dominique; Monceaux, Françoise; Morel, Yves; Lecomte, Pierre

2012-12-01

Several cases of testicular adrenal rest tumours have been reported in men with congenital adrenal hyperplasia (CAH) due to the classical form of 21-hydroxylase deficiency but the prevalence has not been established. The aims of this report were to evaluate the frequency of testicular adrenal rest tissue in this population in a retrospective multicentre study involving eight endocrinology centres, and to determine whether treatment or genetic background had an impact on the occurrence of adrenal rest tissue. Testicular adrenal rest tissue (TART) was sought clinically and with ultrasound examination in forty-five males with CAH due to the classical form of 21-hydroxylase deficiency. When the diagnosis of testicular adrenal rest tumours was sought, good observance of treatment was judged on biological concentrations of 17-hydroxyprogesterone (17OHP), delta4-androstenedione, active renin and testosterone. The results of affected and non-affected subjects were compared. TART was detected in none of the 18 subjects aged 1 to 15years but was detected in 14 of the 27 subjects aged more than 15years. Five patients with an abnormal echography result had no clinical signs. Therapeutic control evaluated at diagnosis of TART seemed less effective when diagnosis was made in patients with adrenal rest tissue compared to TART-free subjects. Various genotypes were observed in patients with or without TART. Due to the high prevalence of TART in classical CAH and the delayed clinical diagnosis, testicular ultrasonography must be performed before puberty and thereafter regularly during adulthood even if the clinical examination is normal. Copyright © 2012 Elsevier Masson SAS. All rights reserved.

[Effects of pathological assessment of endometrial tissue in fertility-sparing treatment with progestin for endometrial carcinoma of stage I a and complex atypical hyperplasia].

PubMed

Gong, Qinglin; Chen, Xiaoduan; Xie, Xing

2014-09-01

To assess the efficacy and pathological change of fertility-sparing treatment with progestin for endometrial carcinoma (EC) of stage I a and complex atypical hyperplasia (CAH) and to observe the prognosis of the treatment. Nine EC patients of stage I a and 21 CAH patients aged under 40 years who desired childbearing and retaining their fertility were enrolled into this study. All patients were given a daily oral high-dose of progestin with duration of treatment ranging from 6 to 9 months. Diagnostic curettage was performed every 3 months as a modality for seeing the histologic change of neoplastic tissues and endometrial tissue. A careful and long- term follow- up is necessary for patients with complete response (CR). During the first period of fertility-sparing management, according to histologic change, 5 EC patients and 18 CAH patients showed CR with no evidence of endometrial adenocarcinoma or hyperplasia, 2 EC patients and 2 CAH patients showed partial response with a regression to complex or simple hyperplasia without atypia, 2 EC patients and 1 CAH patient showed stable disease or progressive disease. Accordingly, a total of 26 patients showed CR (26 of 30 patients). The median time to CR was 6 months (range, 3 to 21 months) of progestin treatment. The median follow-up time was 55.5 months (range, 24 to 104 months) and all patients were alive. During follow-up, among the 26 patients with CR, 3 of 6 EC patients achieved CR recurred disease after a median time interval of 10 months (range, 6 to 51 months), 7 of 20 CAH patients achieved CR had recurrent disease after a median time interval of 12 months (range, 6 to 55 months). Four of 7 CAH with recurrent disease achieved CR to progestin re-treatment. Eight of 26 patients achieved CR continued a further 3 or 6 months of consolidation therapy, 3 of them had recurrent disease, the remaining 18 stopped progesterone treatment after CR and 7 patients had recurrent disease; there was no significant statistical

Neuropsychological assessment in prepubertal patients with congenital adrenal hyperplasia: preliminary study.

PubMed

Somajni, F; Sovera, V; Albizzati, A; Russo, G; Peroni, P; Seragni, G; Lenti, C

2011-02-01

Individuals with congenital adrenal hyperplasia (CAH) provide a test population for the theory that elevated testosterone levels alter pre-/perinatal brain development. Seven prepuberal girls with CAH and seven matched controls has been submitted to a neuropsychological evaluation. We measured abilities where gender differences repeatedly has been observed or that had earlier shown differences between CAH subjects and controls. The following cognitive functions were tested: general intelligence, attention, verbal and non-verbal abilities, cerebral dominance for verbal and non-verbal material, frontal functions, peripheral dominance and motor fluency. Since several animal studies shown hippocampal morphological changes induced by prolonged hydrocortisone exposure, we also investigated memory functions. No differences were recorded between two groups on those abilities that are not sexually dimorphic. The mean general intelligence level of the patients was significantly lower than the controls', in agreement with previous studies. The verbal and non-verbal tasks revealed an age-related male-like pattern (i.e., verbal disadvantage) and an inversion of the hemispheric dominances. The latter observation was supported by a right-to-left shift of the peripheral dominances. The patients memory performances were all inferior to the controls'. The results are discussed in the light of possible hormonal influences. Our main findings support the hypothesis that elevated pre-/perinatal androgen exposure can influence some cognitive pattern of specific sexual dimorphic abilities in prepubertal subjects.

Dietary intake and nutritional status in cancer patients; comparing adults and older adults.

PubMed

Gómez Valiente da Silva, Henyse; Fonseca de Andrade, Camila; Bello Moreira, Annie Seixas

2014-04-01

Evaluate the nutrient intake and nutritional status of food in cancer patients admitted to a university hospital, with comparison of adult and older adult age category. Cross-sectional study. This study involved cancer patients admitted to a hospital in 2010. Dietary habits were collected using a Brazilian food frequency questionnaire. Participants were divided in two groups: adults or older adults and in 4-cancer category: hematologic, lung, gastrointestinal and others. Body Mass Index evaluated nutritional status. A total of 86 patients with a mean age of 56.5 years, with 55% males and 42% older adults were evaluated. The older adult category had a higher frequency of being underweight (24.4% vs 16.3%, p < 0.01) and a lower frequency of being overweight (7% vs. 15.1%, p < 0.01) than adults. Both, adult and older adults had a high frequency of smoking, alcohol consumption and physical inactivity. The older adults had lower consumption of calories, intake of iron and folic acid. Inadequacy of vitamin intake was observed in both groups; respectively, 52%, 43%, 95%, 76% and 88% for Vitamin A, C, D, E and folic acid. The older adults had a higher folic acid and calcium inadequacy than the adults (97% vs 82%, p <0.01; 88% vs 72%, p < 0.01). There was no association of micronutrient intake with cancer, nor with nutritional status. The food intake, macro and micronutrients ingestion is insufficient among cancer individuals. Food intake of older adults was inferior, when compared to the adult category. There was a high prevalence of BMI excess in the adult group and a worst nutritional status in the older adult category. Copyright AULA MEDICA EDICIONES 2014. Published by AULA MEDICA. All rights reserved.

Sodium Chloride Supplementation Is Not Routinely Performed in the Majority of German and Austrian Infants with Classic Salt-Wasting Congenital Adrenal Hyperplasia and Has No Effect on Linear Growth and Hydrocortisone or Fludrocortisone Dose.

PubMed

Bonfig, Walter; Roehl, Friedhelm; Riedl, Stefan; Brämswig, Jürgen; Richter-Unruh, Annette; Fricke-Otto, Susanne; Hübner, Angela; Bettendorf, Markus; Schönau, Eckhard; Dörr, Helmut; Holl, Reinhard W; Mohnike, Klaus

2018-01-01

Sodium chloride supplementation in salt-wasting congenital adrenal hyperplasia (CAH) is generally recommended in infants, but its implementation in routine care is very heterogeneous. To evaluate oral sodium chloride supplementation, growth, and hydrocortisone and fludrocortisone dose in infants with salt-wasting CAH due to 21-hydroxylase in 311 infants from the AQUAPE CAH database. Of 358 patients with classic CAH born between 1999 and 2015, 311 patients had salt-wasting CAH (133 females, 178 males). Of these, 86 patients (27.7%) received oral sodium chloride supplementation in a mean dose of 0.9 ± 1.4 mmol/kg/day (excluding nutritional sodium content) during the first year of life. 225 patients (72.3%) were not treated with sodium chloride. The percentage of sodium chloride-supplemented patients rose from 15.2% in children born 1999-2004 to 37.5% in children born 2011-2015. Sodium chloride-supplemented and -unsupplemented infants did not significantly differ in hydrocortisone and fludrocortisone dose, target height-corrected height-SDS, and BMI-SDS during the first 2 years of life. In the AQUAPE CAH database, approximately one-third of infants with salt-wasting CAH receive sodium chloride supplementation. Sodium chloride supplementation is performed more frequently in recent years. However, salt supplementation had no influence on growth, daily fludrocortisone and hydrocortisone dose, and frequency of adrenal crisis. © 2017 S. Karger AG, Basel.

A novel homozygous mutation IVS6+5G>T in CYP11B1 gene in a Vietnamese patient with 11β-hydroxylase deficiency.

PubMed

Nguyen, Thi Phuong Mai; Nguyen, Thu Hien; Ngo, Diem Ngoc; Vu, Chi Dung; Nguyen, Thi Kim Lien; Nong, Van Hai; Nguyen, Huy Hoang

2015-07-10

Congenital adrenal hyperplasia (CAH) is an autosomal recessive disease which is characterized by a deficiency of one of the enzymes involved in the synthesis of cortisol from cholesterol by the adrenal cortex. CAH cases arising from impaired 11β-hydroxylase are the second most common form. Mutations in the CYP11B1 gene are the cause of 11β-hydroxylase deficiency. This study was performed on a patient with congenital adrenal hyperplasia and with premature development such as enlarged penis, muscle development, high blood pressure, and bone age equivalent of 5 years old at 2 years of chronological age. Biochemical tests for steroids confirmed the diagnosis of CAH. We used PCR and sequencing to screen for mutations in CYP11B1 gene. Results showed that the patient has a novel homozygous mutation of guanine (G) to thymine (T) in intron 6 (IVS6+5G>T). The analysis of this mutation by MaxEntScan boundary software indicated that this mutant could affect the gene splicing during transcription. Copyright © 2015 Elsevier B.V. All rights reserved.

Sonographic Appearance of Testicular Adrenal Rest Tumour in a Patient with Congenital Adrenal Hyperplasia.

PubMed

Deshpande, Saurabh S; Shetty, Devdas; Saifi, Shenaz

2017-01-01

Testicular adrenal rest tumours (TARTs) are benign testicular masses that are found in inadequately treated patients with congenital adrenal hyperplasia (CAH). Recognizing this association and identifying characteristic ultrasound features of TARTs is important so as to avoid misdiagnosing them as malignancies, which can lead to unnecessary interventions. We describe a case of a 9-year-old boy, with a diagnosis of CAH and precocious puberty, who was referred to our department for an ultrasound evaluation of the abdomen and scrotum. On ultrasound, there were well-defined, heterogeneous, predominantly hypoechoic, round-to-oval masses in both testes. Taking into account the presence of CAH and a typical sonographic appearance of bilateral testicular masses, a diagnosis of testicular adrenal rest tumour was made; biopsy was deferred and hormonal treatment was modified. Prompt diagnosis of testicular adrenal rest tumours is essential, as it only indicates inadequate hormonal control. Moreover, it can prevent unnecessary biopsies and orchidectomies, and can maintain fertility. TARTs have a typical imaging appearance that every radiologist must be aware of.

Sonographic Appearance of Testicular Adrenal Rest Tumour in a Patient with Congenital Adrenal Hyperplasia

PubMed Central

Shetty, Devdas; Saifi, Shenaz

2017-01-01

Summary Background Testicular adrenal rest tumours (TARTs) are benign testicular masses that are found in inadequately treated patients with congenital adrenal hyperplasia (CAH). Recognizing this association and identifying characteristic ultrasound features of TARTs is important so as to avoid misdiagnosing them as malignancies, which can lead to unnecessary interventions. Case Report We describe a case of a 9-year-old boy, with a diagnosis of CAH and precocious puberty, who was referred to our department for an ultrasound evaluation of the abdomen and scrotum. On ultrasound, there were well-defined, heterogeneous, predominantly hypoechoic, round-to-oval masses in both testes. Taking into account the presence of CAH and a typical sonographic appearance of bilateral testicular masses, a diagnosis of testicular adrenal rest tumour was made; biopsy was deferred and hormonal treatment was modified. Conclusions Prompt diagnosis of testicular adrenal rest tumours is essential, as it only indicates inadequate hormonal control. Moreover, it can prevent unnecessary biopsies and orchidectomies, and can maintain fertility. TARTs have a typical imaging appearance that every radiologist must be aware of. PMID:29662583

Study of inhaler technique in asthma patients: differences between pediatric and adult patients

PubMed Central

Manríquez, Pablo; Acuña, Ana María; Muñoz, Luis; Reyes, Alvaro

2015-01-01

Objective: Inhaler technique comprises a set of procedures for drug delivery to the respiratory system. The oral inhalation of medications is the first-line treatment for lung diseases. Using the proper inhaler technique ensures sufficient drug deposition in the distal airways, optimizing therapeutic effects and reducing side effects. The purposes of this study were to assess inhaler technique in pediatric and adult patients with asthma; to determine the most common errors in each group of patients; and to compare the results between the two groups. Methods: This was a descriptive cross-sectional study. Using a ten-step protocol, we assessed inhaler technique in 135 pediatric asthma patients and 128 adult asthma patients. Results: The most common error among the pediatric patients was failing to execute a 10-s breath-hold after inhalation, whereas the most common error among the adult patients was failing to exhale fully before using the inhaler. Conclusions: Pediatric asthma patients appear to perform most of the inhaler technique steps correctly. However, the same does not seem to be true for adult patients. PMID:26578130

Clinical Features of Adult Patients Admitted to Pediatric Wards in Japan.

PubMed

Michihata, Nobuaki; Matsui, Hiroki; Fushimi, Kiyohide; Yasunaga, Hideo

2015-10-01

Pediatricians generally need to treat adult patients who require long-term care for pediatric diseases. However, little is known about the characteristics of adult patients in pediatric wards. Using a national inpatient database, the aim of this study was to determine the clinical details of adult patients admitted to pediatric wards in Japanese acute-care hospitals. We extracted all inpatients aged ≥19 years who were admitted to pediatric departments in Japan from April 2012 to March 2013. We examined the patients' main diagnoses and the use of life-supporting home medical devices. Of 417,352 patients admitted to pediatric wards during the study period, we identified 4,729 (1.1%) adult patients. The major diagnoses of the adult patients were malignancy, congenital heart disease, epilepsy, and cerebral palsy. More than 35% of the patients with cerebral palsy had a tracheostomy tube, gastrostomy tube, home central venous alimentation, or home respirator. More than 20% of patients aged ≥40 years in pediatric wards had adult diseases, including ischemic heart diseases, cerebrovascular diseases, and adult malignancy. Many adult patients in pediatric wards had adult diseases. It is essential to establish a disease-oriented support system for adults with chronic conditions that originated in their childhood. Copyright © 2015 Society for Adolescent Health and Medicine. Published by Elsevier Inc. All rights reserved.

Prevalence of and markers for the attenuated form of congenital adrenal hyperplasia and hyperprolactinemia masquerading as polycystic ovarian disease.

PubMed

Benjamin, F; Deutsch, S; Saperstein, H; Seltzer, V L

1986-08-01

To determine the prevalence of the attenuated form of congenital adrenal hyperplasia (CAH) and hyperprolactinemia (HPPN) relative to polycystic ovarian disease (PCOD), 100 consecutive women presenting with the classic clinical features of PCOD were evaluated by basal hormonal profiles and subsequent adrenocorticotropic hormone (ACTH) stimulation tests. The study also sought biochemical markers for CAH other than ACTH stimulation. The prevalences were found to be as follows: PCOD, 65%; PCOD with HPPN, 9%; HPPN, 3%, end-organ hypersensitivity (EOH), 4%; homozygotic CAH, 4%; and heterozygotic CAH, 15%. Other than the differential response to ACTH, the only other biochemical markers observed for homozygotic CAH were significantly higher basal levels of testosterone (T) and 17 alpha-hydroxyprogesterone (17-OHP). Luteinizing hormone/follicle-stimulating hormone ratio, androstenedione, and dehydroepiandrosterone sulfate all showed no significant differences between homozygotic CAH, heterozygotic CAH, HPPN, PCOD, and EOH. This study establishes the relative prevalences of the syndromes commonly mimicking PCOD. We also conclude that the observed low incidence of CAH does not justify routine ACTH testing on all patients presenting with features of PCOD--however, our data suggest that patients with basal serum levels of T and 17-OHP greater than 50% above the upper limit of normal should undergo this dynamic test, especially if there are also certain clinical features suggestive of CAH.

Diagnosis of Adult Patients with Cystic Fibrosis.

PubMed

Nick, Jerry A; Nichols, David P

2016-03-01

The diagnosis of cystic fibrosis (CF) is being made with increasing frequency in adults. Patients with CF diagnosed in adulthood typically present with respiratory complaints, and often have recurrent or chronic airway infection. At the time of initial presentation individuals may appear to have clinical manifestation limited to a single organ, but with subclinical involvement of the respiratory tract. Adult-diagnosed patients have a good response to CF center care, and newly available cystic fibrosis transmembrane receptor-modulating therapies are promising for the treatment of residual function mutation, thus increasing the importance of the diagnosis in adults with unexplained bronchiectasis. Copyright © 2016 Elsevier Inc. All rights reserved.

Participation of family members and quality of patient care - the perspective of adult surgical patients.

PubMed

Leino-Kilpi, Helena; Gröndahl, Weronica; Katajisto, Jouko; Nurminen, Matti; Suhonen, Riitta

2016-08-01

The aim of this study is to describe the participation of family members in the care of Finnish adult surgical patients and the connection of the participation with the quality of patient care as perceived by surgical patients. The family members of adult surgical patients are important. Earlier studies vary concerning the nature of participation, its meaning and the connection of participation with patient-centred quality of care. In this study, we aim to produce new knowledge about adult surgical patients whose family members have participated in their care. This was a cross-sectional descriptive survey study. The data were collected among adult surgical patients (N = 481) before being discharged home from hospital with two instruments: the Good Nursing Care scale and the Received Knowledge of Hospital Patients. Based on the results, most adult surgical patients report that family members participate in their care. Participation was connected with received knowledge and preconditions of care, which are components of the quality of patient care. In future, testing of different solutions for improving the participation of surgical patients' family members in patient care should be implemented. Furthermore, the preconditions of family members' participation in care and the concept of participation should be analysed to emphasise the active role of family members. The results emphasised the importance of family members for the patients in surgical care. Family members' participation is connected with the quality of patient care. © 2016 John Wiley & Sons Ltd.

Asperger Syndrome: a frequent comorbidity in first diagnosed adult ADHD patients?

PubMed

Roy, Mandy; Ohlmeier, Martin D; Osterhagen, Lasse; Prox-Vagedes, Vanessa; Dillo, Wolfgang

2013-06-01

Because adult ADHD is often accompanied by psychiatric comorbidities, the diagnostic process should include a thorough investigation for comorbid disorders. Asperger-Syndrome is rarely reported in adult ADHD and commonly little attention is paid to this possible comorbidity. We investigated 53 adult ADHD-patients which visited our out patient clinic for first ADHD-diagnosis (17 females, 36 males; range of age: 18-56 years) for the frequency of a comorbid Asperger-Syndrome. Diagnosis of this autism-spectrum disorder was confirmed by applying the appropriate DSM-IV-criteria. Additionally we tested the power of the two screening-instruments "Autism-spectrum quotient" (AQ) and "Empathy quotient" (EQ) by Baron-Cohen for screening Asperger-Syndrome in adult ADHD. Eight ADHD-patients were diagnosed with a comorbid Asperger-Syndrome (15.1%). The difference in AQ- and EQ-scores between pure ADHD-patients and comorbid patients was analysed, showing significantly higher scores in AQ and significant lower scores in EQ in comorbid patients. Results show that the frequency of Asperger-Syndrome seems to be substantially increased in adult ADHD (versus the prevalence of 0.06% in the general population), indicating that investigators of adult ADHD should also be attentive to autism-spectrum disorders. Especially the AQ seems to be a potential screening instrument for Asperger-Syndrome in adult ADHD-patients.

Early maladaptive schemas in adult patients with attention deficit hyperactivity disorder.

PubMed

Philipsen, Alexandra; Lam, Alexandra P; Breit, Sigrid; Lücke, Caroline; Müller, Helge H; Matthies, Swantje

2017-06-01

The main purpose of this study was to examine whether adult patients with attention deficit hyperactivity disorder (ADHD) demonstrate sets of dysfunctional cognitive beliefs and behavioural tendencies according to Jeffrey Young's schema-focused therapy model. Sets of dysfunctional beliefs (maladaptive schemas) were assessed with the Young Schema Questionnaire (YSQ-S2) in 78 adult ADHD patients and 80 control subjects. Patients with ADHD scored significantly higher than the control group on almost all maladaptive schemas. The 'Failure', 'Defectiveness/Shame', 'Subjugation' and 'Emotional Deprivation' schemas were most pronounced in adult ADHD patients, while only 'Vulnerability to Harm or Illness' did not differ between the two groups. The schemas which were most pronounced in adult patients with ADHD correspond well with their learning histories and core symptoms. By demonstrating the existence of early maladaptive schemas in adults suffering from ADHD, this study suggests that schema theory may usefully be applied to adult ADHD therapy.

High-Dose Hook Effect in 17-Hydroxyprogesterone Assay in a Patient with 21-Hydroxylase Deficiency.

PubMed

Parlak, Mesut; Ellidağ, Hamit Yaşar; Türkkahraman, Doğa

2015-12-01

Congenital adrenal hyperplasia (CAH) describes a group of disorders characterized by enzyme defects in adrenal steroidogenesis. 21-hydroxylase deficiency (21-OHD) is the most commonly encountered form. The analysis of steroids in pediatric cases requires high-sensitivity assays. A 14-year-old Syrian girl was referred for evaluation of short stature, amenorrhea, and hirsutism. On physical examination, breast development was Tanner stage 1. She had a phallic clitoris with a single urogenital orifice. Laboratory findings revealed primary adrenal deficiency with high androgen levels and low levels of 17-hydroxyprogesterone (17-OHP), (<0.05 ng/mL) and estrogen. This unexpected result led to suspicion of a high-dose hook effect. The measurement was repeated after 1/10 dilution of serum, and a high level of 17-OHP (115.4 ng/mL) was detected with the same test-enzyme-linked immunosorbent assay (ELISA). Simple virilizing form of CAH (21-OHD) was suspected and confirmed with genetic analysis. After initiation of glucocorticoid therapy, breast development was noted along with a decrease in testosterone level and an increase in estrogen level. To our knowledge, this is the first case report of hook effect for 17-OHP immunoassay in a patient with 21-OHD. High-dose hook effect should be suspected in patients with CAH when the test results are incompatible with one another. Additionally, this case demonstrates that a high testosterone level can block aromatase activity and consequently also estrogen production and breast development.

Patient engagement in research with older adults with cancer.

PubMed

Puts, Martine T E; Sattar, Schroder; Ghodraty-Jabloo, Vida; Hsu, Tina; Fitch, Marg; Szumacher, Ewa; Ayala, Ana Patricia; Alibhai, Shabbir M H

2017-11-01

Cancer is a disease that mostly affects older adults. Older adults have been under-represented in clinical cancer research. Around the world there is a push for patient engagement on study teams as it is anticipated to improve study design, recruitment and dissemination of findings. In the current overview we examined the evidence with regard to: 1) the history of patient engagement in research and frameworks developed; 2) impact of patient engagement on patient and research outcomes; 3) use of patient engagement in geriatrics and oncology, 4) recommendations for successful engagement; and 5) gaps in the literature that should be studied further. A narrative review was conducted. Articles published in English were searched in Medline with the help of a librarian. Patient engagement has been shown to improve the conduct of studies by making the study design more relevant and feasible, and improving recruitment rates and uptake of research findings by patients. However, the best way to engage patients is not clear yet. Several resources have been developed to support researchers engaging older adults with cancer in research. While patient engagement in research seems promising to improve study outcomes, little evidence is available thus far in geriatric oncology settings. Several gaps in the literature are identified that should be further studied to determine the value of, and best approaches to, patient engagement with older adults with cancer. Copyright © 2017 Elsevier Ltd. All rights reserved.

Utilization and outcomes of inpatient surgical care at critical access hospitals in the United States.

PubMed

Gadzinski, Adam J; Dimick, Justin B; Ye, Zaojun; Miller, David C

2013-07-01

There is a growing interest in the quality and cost of care provided at Critical Access Hospitals (CAHs), a predominant source of care for many rural populations in the United States. To evaluate utilization, outcomes, and costs of inpatient surgery performed at CAHs. A retrospective cohort study of patients undergoing inpatient surgery from 2005 through 2009 at CAHs or non-CAHs was performed using data from the Nationwide Inpatient Sample and American Hospital Association. The CAH status of the admitting hospital. In-hospital mortality, prolonged length of stay, and total hospital costs. Among the 1283 CAHs and 3612 non-CAHs reporting to the American Hospital Association, 34.8% and 36.4%, respectively, had at least 1 year of data in the Nationwide Inpatient Sample. General surgical, gynecologic, and orthopedic procedures composed 95.8% of inpatient cases at CAHs vs 77.3% at non-CAHs (P < .001). For 8 common procedures examined (appendectomy, cholecystectomy, colorectal cancer resection, cesarean delivery, hysterectomy, knee replacement, hip replacement, and hip fracture repair), mortality was equivalent between CAHs and non-CAHs (P > .05 for all), with the exception that Medicare beneficiaries undergoing hip fracture repair in CAHs had a higher risk of in-hospital death (adjusted odds ratio = 1.37; 95% CI, 1.01-1.87). However, despite shorter hospital stays (P ≤ .001 for 4 procedures), costs at CAHs were 9.9% to 30.1% higher (P < .001 for all 8 procedures). In-hospital mortality for common low-risk procedures is indistinguishable between CAHs and non-CAHs. Although our findings suggest the potential for cost savings, changes in payment policy for CAHs could diminish access to essential surgical care for rural populations.

Psychiatric characterization of children with genetic causes of hyperandrogenism.

PubMed

Mueller, Sven C; Ng, Pamela; Sinaii, Ninet; Leschek, Ellen W; Green-Golan, Liza; VanRyzin, Carol; Ernst, Monique; Merke, Deborah P

2010-11-01

Very little is known about the mental health status in children with genetic causes of hyperandrogenism. This study sought to characterize psychiatric morbidity in this group. Children (8-18 years) with the diagnosis of classic congenital adrenal hyperplasia (CAH) or familial male precocious puberty (FMPP) underwent a semi-structured psychiatric interview, the Kiddie Schedule for Affective Disorders and Schizophrenia-Present and Lifetime Version. According to sex and the literature, incidence of identified psychopathology was compared between the two endocrinological groups. We evaluated 72 patients: 54 CAH (21 females) and 18 FMPP. Twenty-four (44.4%) CAH patients and 10 (55.6%) FMPP patients met the criteria for at least one lifetime psychiatric diagnosis. Attention-deficit hyperactivity disorder (ADHD) was present in 18.2% of CAH males, 44.4% of FMPP males, and one case (4.8%) in CAH females. A high rate of anxiety disorders was also found in all the three groups (17-21%). Relative to females with CAH, the FMPP patients exhibited higher rates of ADHD. Age at diagnosis and the treatment modalities were not associated with psychopathology. Rates of psychiatric disorder, specifically ADHD and anxiety disorders, were higher than in the general population. Although anxiety disorders may occur at an increased rate in children with chronic illness, androgens may contribute to higher risk for psychopathology in pediatric patients with genetic cause of excess androgen. Early diagnosis and treatment of childhood hyperandrogenism is essential for optimal development. The results suggest that assessment for psychiatric disorders should be part of the routine evaluation of these patients.

Assessment of motivation and psychological characteristics of adult orthodontic patients.

PubMed

Pabari, Sona; Moles, David R; Cunningham, Susan J

2011-12-01

In recent years, the demand for adult orthodontic treatment has grown rapidly; yet there is a paucity of information on this subgroup of patients. It is well known that understanding the psychological characteristics and motives of any patient is fundamental and that these factors might affect patient satisfaction and adherence with treatment. There is therefore a need for clinicians to improve their understanding of this subgroup to enhance the patient's experience of treatment delivery and to increase the potential for a successful treatment outcome. The aim of this study was to develop a measure for the assessment of motivating factors and psychological characteristics of adults seeking orthodontic treatment. This study involved the qualitative development of a valid patient-centered questionnaire to assess motivating factors for adults seeking orthodontic treatment. This was achieved through semi-structured in-depth interviews; key themes were identified and used to construct a questionnaire assessing motivation for treatment. This was then combined with 3 previously validated questionnaires to measure self-esteem, anxiety or depression, and body image and facial body image. The questionnaire was distributed to 172 adult orthodontic patients at different stages of treatment in a large teaching hospital in the United Kingdom. In addition, the self-esteem, body image, and facial body image scores were compared with data on orthognathic patients from the same hospital and with data from members of the general public. Desire to straighten the teeth and improve the smile were the key motivating factors for the adult group studied. Other motives included to improve the bite, improve facial appearance, and close (dental) spacing. With respect to the psychological characteristics of self-esteem, body image, and facial body image, the adult orthodontic group was comparable with the general public. However, differences were noted when comparing data from the adult

[Qualitative research on pain experiences of adult burn patients].

PubMed

Li, L; Pan, Q; Xu, L; Lin, R Q; Dai, J X; Chen, Z H

2018-03-20

Objective: To explore the pain experiences of adult burn patients so as to lay foundation for practical analgesic measures. Methods: Using phenomenological method in qualitative research, semi-structured interviews were conducted on 12 adult burn patients hospitalized in our burn units from May to November 2015, aiming at pain experiences from immediately after burns to 3 to 7 months after being discharged from hospital. Then the Colaizzi's analysis method was applied to analyze, induce, and refine themes of interview data. Results: After analysis, pain experiences of adult burn patients were generalized into 6 themes: deep pain experiences, heavy psychological burden, limited daily life, poor assessment and treatment of pain, different attributions of pain, and different ways of coping of pain. Conclusions: Burn pain brings harm to the patients' physiology, mentality, and daily life. Nevertheless, pain processing modes of medical staff and patients themselves are the key factors affecting patients' pain experiences. Therefore, according to the deficiency of current situation of pain management, the targeted analgesic intervention measures should be carried out from the perspectives of medical staff and patients.

Developmental deficits in adult patients with arteriovenous malformations.

PubMed

Lazar, R M; Connaire, K; Marshall, R S; Pile-Spellman, J; Hacein-Bey, L; Solomon, R A; Sisti, M B; Young, W L; Mohr, J P

1999-01-01

Cerebral arteriovenous malformations (AVMs) are congenital masses of arteries and veins that appear to undergo an unclear "maturation" for many years. Using structured interviews, we compared developmental history of adult patients with AVM with a comparison group of patients with cerebral tumor or aneurysm. To determine whether a remote history of developmental abnormality in adult patients with AVM might be an early marker of cerebral status. Adult patients with AVM and a comparison group of patients with cerebral aneurysm or low-grade tumor participated in a survey. Urban medical school-based tertiary care center. Forty-four randomly selected patients with AVM from the Columbia-Presbyterian AVM Database. There were 32 comparison patients:15 randomly chosen patients from the institution's Cerebral Aneurysm Database and all 17 patients who underwent a biopsy from 1990 to 1995 with a diagnosis of low-grade tumor and who could be contacted. A brief, structured interview adapted from the Centers for Disease Control and Prevention for its 1994 study of the prevalence of learning disabilities in American children. We defined the positive occurrence of a condition as an affirmative answer to the question, " Did have (condition) during his/her school-age years?" Each patient was also asked if there had been any problems in the following skill areas: reading, writing, listening, speaking, attention, impulsivity, organization, mathematics, or drawing. The AVM size was calculated on the angiographic film by measuring its longest diameter in any dimension. Patients with AVM were significantly more likely to report a positive occurrence to any survey question (P<.05). Two thirds of all patients with AVM (66%) reported at least 1 skill difficulty during their school years, significantly more than the comparison group (P<.001). Neither the maximum AVM diameter nor the occurrence of hemorrhage as an adult differed between patients with AVM with and without early skill difficulty

Systematic review of surgical treatment techniques for adult and pediatric patients with pectus excavatum

PubMed Central

2014-01-01

This compares outcome measures of current pectus excavatum (PEx) treatments, namely the Nuss and Ravitch procedures, in pediatric and adult patients. Original investigations that stratified PEx patients based on current treatment and age (pediatric = 0–21; adult 17–99) were considered for inclusion. Outcome measures were: operation duration, analgesia duration, blood loss, length of stay (LOS), outcome ratings, complications, and percentage requiring reoperations. Adult implant patients (18.8%) had higher reoperation rates than adult Nuss or Ravitch patients (5.3% and 3.3% respectively). Adult Nuss patients had longer LOS (7.3 days), more strut/bar displacement (6.1%), and more epidural analgesia (3 days) than adult Ravitch patients (2.9 days, 0%, 0 days). Excluding pectus bar and strut displacements, pediatric and adult Nuss patients tended to have higher complication rates (pediatric - 38%; adult - 21%) compared to pediatric and adult Ravitch patients (12.5%; 8%). Pediatric Ravitch patients clearly had more strut displacements than adult Ravitch patients (0% and 6.4% respectively). These results suggest significantly better results in common PEx surgical repair techniques (i.e. Nuss and Ravitch) than uncommon techniques (i.e. Implants and Robicsek). The results suggest slightly better outcomes in pediatric Nuss procedure patients as compared with all other groups. We recommend that symptomatic pediatric patients with uncomplicated PEx receive the Nuss procedure. We suggest that adult patients receive the Nuss or Ravitch procedure, even though the long-term complication rates of the adult Nuss procedure require more investigation. PMID:24506826

Use of Critical Access Hospital Emergency Rooms by Patients with Mental Health Symptoms

ERIC Educational Resources Information Center

Hartley, David; Ziller, Erika C.; Loux, Stephenie L.; Gale, John A.; Lambert, David; Yousefian, Anush E.

2007-01-01

Context: National data demonstrate that mental health (MH) visits to the emergency room (ER) comprise a small, but not inconsequential, proportion of all visits; however, we lack a rural picture of this issue. Purpose: This study investigates the use of critical access hospital (CAH) ERs by patients with MH problems to understand the role these…

Stigma Associated with Classical Congenital Adrenal Hyperplasia in Women's Sexual Lives.

PubMed

Meyer-Bahlburg, Heino F L; Khuri, Jananne; Reyes-Portillo, Jazmin; Ehrhardt, Anke A; New, Maria I

2018-05-01

The risk of intersex-related stigma often serves as social indication for "corrective" genital surgery, but has not been comprehensively documented. In preparation for the development of an intersex-specific stigma assessment tool, this qualitative project aimed to explore stigma in girls and women with classical congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency. As part of a comprehensive follow-up project, 62 adult women with classical CAH (age range 18-51 years) took part in an open-ended retrospective interview focusing on the impact of CAH and its treatment on various aspects of girls' and women's lives. Deductive qualitative content analysis (Patton, 2014) of de-identified transcripts involved categorization of three types of stigma: experienced, anticipated, and internalized. Two-fifths of the participants reported CAH-related stigma in romantic/sexual situations. Stigma enactment by romantic partners occurred in reaction to both genital and non-genital sex-atypical features of CAH and sometimes included explicit questioning of the women's true gender. Stigma anticipation by the women and their related avoidance of nudity, genital exposure, and romantic involvement altogether were frequent. Internalization of stigma occurred as well. In conclusion, the data suggest that many women with CAH experience, anticipate, and/or internalize intersex-related stigma in the context of their romantic/sexual lives.

Lifetime Increased Risk of Adult Onset Atopic Dermatitis in Adolescent and Adult Patients with Food Allergy.

PubMed

Yu, Hsu-Sheng; Tu, Hung-Pin; Hong, Chien-Hui; Lee, Chih-Hung

2016-12-27

Food allergy can result in life-threatening anaphylaxis. Atopic dermatitis (AD) causes intense itching and impaired quality of life. Previous studies have shown that patients with classical early-onset AD tend to develop food allergy and that 10% of adults with food allergies have concomitant AD. However, it is not known whether late-onset food allergy leads to adult-onset AD, a recently recognized disease entity. Using an initial cohort of one-million subjects, this study retrospectively followed-up 2851 patients with food allergy (age > 12 years) for 14 years and compared them with 11,404 matched controls. While 2.8% (81) of the 2851 food allergy patients developed AD, only 2.0% (227) of the 11,404 controls developed AD. Multivariate regression analysis showed that food allergy patients were more likely to develop AD (adjusted hazard ratio = 2.49, p < 0.0001). Controls had a 1.99% risk of developing AD, while food allergy patients had a significantly higher risk (7.18% and 3.46% for patients with ≥3 and <3 food allergy claims, respectively) of developing adult-onset AD. This is the first study to describe the chronological and dose-dependent associations between food allergy in adolescence and the development of adult-onset AD.

Male gender identity in children with 46,XX DSD with congenital adrenal hyperplasia after delayed presentation in mid-childhood.

PubMed

Chowdhury, Tanvir Kabir; Laila, Kamrun; Hutson, John M; Banu, Tahmina

2015-12-01

Girls with congenital adrenal hyperplasia (CAH) diagnosed at birth have some masculine behaviors but rarely convert to male gender. In developing countries, however, diagnosis and treatment (with secondary androgen suppression) are delayed. We aimed to assess effect of delayed treatment of CAH on gender identity. As part of a cross-sectional, case-control study of children with disorders of sex development (DSD), there were 11 patients with CAH. Patients and caregivers answered a questionnaire about gender identity, and behavior was assessed by observing toy play. Patients were examined for Prader score and gender identity. Of 11 CAH patients initially raised as girls, 3 (27%) had converted to male gender at presentation (5, 9, 9years) (Prader 3, 4, 4). Of the remaining 8 patients, one 4-year-old (Prader 2) had a male gender identity score. The remaining girls (2-13years, mean 8.1) (Prader 1-3) had gender identity scores in the female range. One third (4/11) of CAH patients presenting in mid-childhood had male gender identity scores, and ¾ had assumed male gender role. Although social and cultural factors are important in developing countries, this result suggests that delayed treatment may trigger male gender identity, and delayed female genital surgery may be unwise. Copyright © 2015 Elsevier Inc. All rights reserved.

Adult Patients' Experiences of Nursing Care Dependence.

PubMed

Piredda, Michela; Matarese, Maria; Mastroianni, Chiara; D'Angelo, Daniela; Hammer, Marilyn J; De Marinis, Maria Grazia

2015-09-01

Care dependence can be associated with suffering and humiliation. Nurses' awareness of patients' perception of care dependence is crucial to enable them in helping the dependent persons. This study aimed to describe adult patients' experience of nursing care dependence. A metasynthesis was conducted to integrate qualitative findings from 18 studies published through December 2014 on adult patients' experiences of care dependency. Procedures included the Johanna Briggs Institute approach for data extraction, quality appraisal, and integration of findings. The experience of dependence revealed the concept of the embodied person, particularly in relation to care of the physical body. The relationship between the individual and nurses within the context of care had a major impact for dependent patients. When the care relation was perceived as positive, the experience led to the development of the person in finding new balances in life, but when it was perceived as negative, it increased patient' suffering. Care dependence is manifested mostly as bodily dependence and is consistent with its relational nature. The nurse-patient relationship is important to the dependent patients' experience. A greater understanding of patients' experiences of dependence is crucial to enable nurses in improving care and decreasing patient suffering. © 2015 Sigma Theta Tau International.

Comparing catheter-related bloodstream infections in pediatric and adult cancer patients.

PubMed

Zakhour, Ramia; Hachem, Ray; Alawami, Hussain M; Jiang, Ying; Michael, Majd; Chaftari, Anne-Marie; Raad, Issam

2017-10-01

Central venous catheters (CVCs) are essential to treatment of children with cancer. There are no studies comparing catheter-related bloodstream infections (CRBSIs) in pediatric cancer patients to those in adults, although current guidelines for management of CRBSI do not give separate guidelines for the pediatric population. In this study, we compared CRBSIs in both the pediatric and adult cancer population. We retrospectively reviewed the electronic medical records of 92 pediatric and 156 adult patients with CRBSI cared for at MD Anderson Cancer Center between September 2005 and March 2014. We evaluated 248 patients with CRBSI. There was a significant difference in etiology of CRBSI between pediatric and adult patients (P = 0.002), with the former having less Gram-negative organisms (27 vs. 46%) and more polymicrobial infections (10 vs. 1%, P = 0.003). Pediatric patients had less hematologic malignancies (58 vs. 74%) and less neutropenia at presentation (40 vs. 54%) when compared with adult patients. Peripheral blood cultures were available in only 43% of pediatric cases. CVC was removed in 64% of pediatric cases versus 88% of adult cases (P < 0.0001). We found higher rates of Gram-negative organisms in adults and higher rates of polymicrobial in children. Because of the low rates of peripheral blood cultures and the low rates of CVC removal, CRBSI diagnosis could be challenging in pediatrics. A modified CRBSI definition relying more on clinical criteria may be warranted. © 2017 Wiley Periodicals, Inc.

Integrated evaluation of the performance of a more than seven year old permeable reactive barrier at a site contaminated with chlorinated aliphatic hydrocarbons (CAHs)

NASA Astrophysics Data System (ADS)

Muchitsch, Nanna; Van Nooten, Thomas; Bastiaens, Leen; Kjeldsen, Peter

2011-11-01

An important issue of concern for permeable reactive iron barriers is the long-term efficiency of the barriers due to the long operational periods required. Mineral precipitation resulting from the anaerobic corrosion of the iron filings and bacteria present in the barrier may play an important role in the long-term performance. An integrated study was performed on the Vapokon permeable reactive barrier (PRB) in Denmark by groundwater and iron core sample characterization. The detailed field groundwater sampling carried out from more than 75 well screens up and downstream the barrier showed a very efficient removal (> 99%) for the most important CAHs (PCE, TCE and 1,1,1-TCA). However, significant formation of cis-DCE within the PRB resulted in an overall insufficient efficiency for cis-DCE removal. The detailed analysis of the upstream groundwater revealed a very heterogeneous spatial distribution of contaminant loading into the PRB, which resulted in that only about a quarter of the barrier system is treating significant loads of CAHs. Laboratory batch experiments using contaminated groundwater from the site and iron material from the core samples revealed that the aged iron material performed equally well as virgin granular iron of the same type based on determined degradation rates despite that parts of the cored iron material were covered by mineral precipitates (especially iron sulfides, carbonate green rust and aragonite). The PCR analysis performed on the iron core samples indicated the presence of a microbial consortium in the barrier. A wide range of species were identified including sulfate and iron reducing bacteria, together with Dehalococcoides and Desulfuromonas species indicating microbial reductive dehalogenation potential. The microbes had a profound effect on the performance of the barrier, as indicated by significant degradation of dichloromethane (which is typically unaffected by zero valent iron) within the barrier.

Neuropsychological Assessment of Adult Patients with Shunted Hydrocephalus

PubMed Central

Bakar, Emel Erdogan

2010-01-01

Objective This study is planned to determine the neurocognitive difficulties of hydrocephalic adults. Methods The research group contained healthy adults (control group, n : 15), and hydrocephalic adults (n : 15). Hydrocephalic group consisted of patients with idiopathic aquaduct stenosis and post-meningitis hydrocephalus. All patients were followed with shunted hydrocephalus and not gone to shunt revision during last two years. They were chosen from either asymptomatic or had only minor symptoms without motor and sensorineural deficit. A neuropsychological test battery (Raven Standart Progressive Matrices, Bender-Gestalt Test, Cancellation Test, Clock Drawing Test, Facial Recognition Test, Line Orientation Test, Serial Digit Learning Test, Stroop Color Word Interference Test-TBAG Form, Verbal Fluency Test, Verbal Fluency Test, Visual-Aural Digit Span Test-B) was applied to all groups. Results Neuropsychological assessment of hydrocephalic patients demonstrated that they had poor performance on visual, semantic and working memory, visuoconstructive and frontal functions, reading, attention, motor coordination and executive function of parietal lobe which related with complex and perseverative behaviour. Eventually, these patients had significant impairment on the neurocognitive functions of their frontal, parietal and temporal lobes. On the other hand, the statistical analyses performed on demographic data showed that the aetiology of the hydrocephalus, age, sex and localization of the shunt (frontal or posterior parietal) did not affect the test results. Conclusion This prospective study showed that adult patients with hydrocephalus have serious neuropsychological problems which might be directly caused by the hydrocephalus; and these problems may cause serious adaptive difficulties in their social, cultural, behavioral and academic life. PMID:20379471

The Adult Diabetic Patient: An Education Challenge

DTIC Science & Technology

1993-05-01

finding that he/she, too, must care for sicker patients. To better prepare these patients for life after discharge, patient education must be initiated as...admitted, patient education often begins at the physicians’ office. This paper explores diabetes mellitus in relation to concepts of self-care and adult...betting foj.L eduuation and iio.w, wore ofteni, patient education and follow-up sercvices- a:leL beiny p~rovided on ani outpatient bcdtsis" (p. 36) . Thet

Neutron production in coincidence with fragments from the 4Ca+H reactions at Elab=357 and 565 A MeV

NASA Astrophysics Data System (ADS)

Tuvà, C.; Albergo, S.; Boemi, D.; Caccia, Z.; Chen, C.-X.; Costa, S.; Crawford, H. J.; Cronqvist, M.; Engelage, J.; Greiner, L.; Guzik, T. G.; Insolia, A.; Knott, C. N.; Lindstrom, P. J.; Mitchell, J. W.; Potenza, R.; Russo, G. V.; Soutoul, A.; Testard, O.; Tricomi, A.; Tull, C. E.; Waddington, C. J.; Webber, W. R.; Wefel, J. P.

2000-04-01

In the frame of the Transport Collaboration neutrons in coincidence with charged fragments produced in the 40Ca+H reaction at Elab=357 and 565 A MeV have been measured at the Heavy Ion Spectrometer System (HISS) facility of the Lawrence Berkeley National Laboratory, using the multifunctional neutron spectrometer MUFFINS. The detector covered a narrow angular range about the beam in the forward direction (0°-3.2°). In this contribution we report absolute neutron production cross sections in coincidence with charged fragments (10⩽Z⩽20). The neutron multiplicities have been estimated from the comparison between the neutron cross sections, in coincidence with the fragments, and the elemental cross sections. We have found evidence for a pre-equilibrium emission of prompt neutrons in superposition to a `slower' deexcitation of the equilibrated remnant by emission of nucleons and fragments, as already seen in the inclusive rapidity distributions.

Spine Surgery Outcomes in Elderly Patients Versus General Adult Patients in the United States: A MarketScan Analysis.

PubMed

Lagman, Carlito; Ugiliweneza, Beatrice; Boakye, Maxwell; Drazin, Doniel

2017-07-01

To compare spine surgery outcomes in elderly patients (80-103 years old) versus general adult patients (18-79 years-old) in the United States. Truven Health Analytics MarketScan Research Databases (2000-2012) were queried. Patients with a diagnosis of degenerative disease of the spine without concurrent spinal stenosis, spinal stenosis without concurrent degenerative disease, or degenerative disease with concurrent spinal stenosis and who had undergone decompression without fusion, fusion without decompression, or decompression with fusion procedures were included. Indirect outcome measures included length of stay, in-hospital mortality, in-hospital and 30-day complications, and discharge disposition. Patients (N = 155,720) were divided into elderly (n = 10,232; 6.57%) and general adult (n = 145,488; 93.4%) populations. Mean length of stay was longer in elderly patients versus general adult patients (3.62 days vs. 3.11 days; P < 0.0001). In-hospital mortality was more common in elderly patients versus general adult patients (0.31% vs. 0.06%; P < 0.0001). In-hospital and 30-day complications were more common in elderly patients versus general adult patients (11.3% vs. 7.15% and 17.8% vs. 12.6%; P < 0.0001). Nonroutine discharge was more common in elderly patients versus general adult patients (33.7% vs. 16.2%; P < 0.0001). Our results revealed significantly longer hospital stays, more in-hospital mortalities, and more in-hospital and 30-day complications after decompression without fusion, fusion without decompression, or decompression with fusion procedures in elderly patients. Copyright © 2017 Elsevier Inc. All rights reserved.

Pre-adult versus adult onset major depressive disorder in a naturalistic patient sample: the Leiden Routine Outcome Monitoring Study.

PubMed

van Noorden, M S; Minkenberg, S E; Giltay, E J; den Hollander-Gijsman, M E; van Rood, Y R; van der Wee, N J; Zitman, F G

2011-07-01

Pre-adult onset of major depressive disorder (MDD) may predict a more severe phenotype of depression. As data from naturalistic psychiatric specialty care settings are scarce, we examined phenotypic differences between pre-adult and adult onset MDD in a large sample of consecutive out-patients. Altogether, 1552 out-patients, mean age 39.2 ± 11.6 years, were diagnosed with current MDD on the Mini-International Neuropsychiatric Interview Plus diagnostic interview as part of the usual diagnostic procedure. A total of 1105 patients (71.2%) had complete data on all variables of interest. Pre-adult onset of MDD was defined as having experienced the signs and symptoms of a first major depressive episode before the age of 18 years. Patients were stratified according to the age at interview (20-40/40-65 years). Correlates of pre-adult onset were analysed using logistic regression models adjusted for age, age squared and gender. Univariate analyses showed that pre-adult onset of MDD had a distinct set of demographic (e.g. less frequently living alone) and clinical correlates (more co-morbid DSM-IV - Text Revision diagnoses, more social phobia, more suicidality). In the multivariate model, we found an independent association only for a history of suicide attempts [odds ratio (OR) 3.15, 95% confidence intervals (CI) 1.97-5.05] and current suicidal thoughts (OR 1.81, 95% CI 1.26-2.60) in patients with pre-adult versus adult onset MDD. Pre-adult onset of MDD is associated with more suicidality than adult onset MDD. Age of onset of depression is an easy to ascertain characteristic that may help clinicians in weighing suicide risk.

Ab interno trabeculectomy in the adult patient.

PubMed

SooHoo, Jeffrey R; Seibold, Leonard K; Kahook, Malik Y

2015-01-01

Glaucoma is a potentially blinding disease that affects millions of people worldwide. The mainstay of treatment is lowering of intraocular pressure (IOP) through the use of medications, laser and/or incisional surgery. The trabecular meshwork (TM) is thought to be the site of significant resistance to aqueous outflow in open angle glaucoma. Theoretically, an incision through TM or TM removal should decrease this resistance and lead to a significant reduction in IOP. This approach, commonly referred to as goniotomy or trabeculotomy, has been validated in the pediatric population and has been associated with long-term IOP control. In adults, however, removal of TM tissue has been historically associated with more limited and short-lived success. More recent evidence, reveals that even adult patients may benefit significantly from removal of diseased TM tissue and can lead to a significant reduction in IOP that is long-lasting and safe. In this review, we discuss current evidence and techniques for ab interno trabeculectomy using various devices in the adult patient.

Children with classic congenital adrenal hyperplasia have elevated serum leptin concentrations and insulin resistance: potential clinical implications.

PubMed

Charmandari, Evangelia; Weise, Martina; Bornstein, Stefan R; Eisenhofer, Graeme; Keil, Margaret F; Chrousos, George P; Merke, Deborah P

2002-05-01

Leptin is secreted by the white adipose tissue and modulates energy homeostasis. Nutritional, neural, neuroendocrine, paracrine, and autocrine factors, including the sympathetic nervous system and the adrenal medulla, have been implicated in the regulation of leptin secretion. Classic congenital adrenal hyperplasia (CAH) is characterized by a defect in cortisol and aldosterone secretion, impaired development and function of the adrenal medulla, and adrenal hyperandrogenism. To examine leptin secretion in patients with classic CAH in relation to their adrenomedullary function and insulin and androgen secretion, we studied 18 children with classic CAH (12 boys and 6 girls; age range 2-12 yr) and 28 normal children (16 boys and 12 girls; age range 5-12 yr) matched for body mass index (BMI). Serum leptin concentrations were significantly higher in patients with CAH than in control subjects (8.1 +/- 2.0 vs. 2.5 +/- 0.6 ng/ml, P = 0.01), and this difference persisted when leptin values were corrected for BMI. When compared with their normal counterparts, children with CAH had significantly lower plasma epinephrine (7.1 +/- 1.3 vs. 50.0 +/- 4.2, P < 0.001) and free metanephrine concentrations (18.4 +/- 2.4 vs. 46.5 +/- 4.0, P < 0.001) and higher fasting serum insulin (10.6 +/- 1.4 vs. 3.2 +/- 0.2 microU/ml, P < 0.001) and testosterone (23.7 +/- 5.3 vs. 4.6 +/- 0.5 ng/dl, P = 0.003) concentrations. Insulin resistance determined by the homeostasis model assessment method was significantly greater in children with classic CAH than in normal children (2.2 +/- 0.3 vs. 0.7 +/- 0.04, P < 0.001). Leptin concentrations were significantly and negatively correlated with epinephrine (r = -0.50, P = 0.001) and free metanephrine (r = -0.48, P = 0.002) concentrations. Stepwise multiple linear regression analysis indicated that serum leptin concentrations were best predicted by BMI in both patients and controls. Gender predicted serum leptin concentrations in controls but not in patients

Animal Models of Cancer-Associated Hypercalcemia

PubMed Central

Kohart, Nicole A.; Elshafae, Said M.; Breitbach, Justin T.; Rosol, Thomas J.

2017-01-01

Cancer-associated hypercalcemia (CAH) is a frequently-occurring paraneoplastic syndrome that contributes to substantial patient morbidity and occurs in both humans and animals. Patients with CAH are often characterized by markedly elevated serum calcium concentrations that result in a range of clinical symptoms involving the nervous, gastrointestinal and urinary systems. CAH is caused by two principle mechanisms; humorally-mediated and/or through local osteolytic bone metastasis resulting in excessive calcium release from resorbed bone. Humoral hypercalcemia of malignancy (HHM) is the most common mechanism and is due to the production and release of tumor-associated cytokines and humoral factors, such as parathyroid hormone-related protein (PTHrP), that act at distant sites to increase serum calcium concentrations. Local osteolytic hypercalcemia (LOH) occurs when primary or metastatic bone tumors act locally by releasing factors that stimulate osteoclast activity and bone resorption. LOH is a less frequent cause of CAH and in some cases can induce hypercalcemia in concert with HHM. Rarely, ectopic production of parathyroid hormone has been described. PTHrP-mediated hypercalcemia is the most common mechanism of CAH in human and canine malignancies and is recognized in other domestic species. Spontaneous and experimentally-induced animal models have been developed to study the mechanisms of CAH. These models have been essential for the evaluation of novel approaches and adjuvant therapies to manage CAH. This review will highlight the comparative aspects of CAH in humans and animals with a discussion of the available animal models used to study the pathogenesis of this important clinical syndrome. PMID:29056680

[Bacterial parotitis in an immunocompromised patient in adult ICU].

PubMed

Vassal, O; Bernet, C; Wallet, F; Friggeri, A; Piriou, V

2013-09-01

Bacterial parotitis is a common childhood disease with a favorable outcome. Staphylococcus aureus is the most frequently involved pathogen. Clinical presentation in adult patients can be misleading, Onset occurs in patients with multiple comorbidities, making diagnosis difficult--particularly in ICU. Different pathogens are found in adults with worse outcomes observed. We report here the case of a critically ill patient and discuss diagnosis and management of bacterial parotitis. Copyright © 2013 Société française d’anesthésie et de réanimation (Sfar). Published by Elsevier SAS. All rights reserved.

Comparative characteristics of primary hyperparathyroidism in pediatric and young adult patients.

PubMed

Nicholson, Kristina J; McCoy, Kelly L; Witchel, Selma F; Stang, Michael T; Carty, Sally E; Yip, Linwah

2016-10-01

Primary hyperparathyroidism is rare in pediatric patients. Our study aim was to compare primary hyperparathyroidism in pediatric (<19 years) and young adult (19-29 years) patients. A prospectively collected database from a single, high-volume institution was queried for all patients age <30 years who had initial parathyroidectomy for primary hyperparathyroidism yielding 126/4,546 (2.7%) primary hyperparathyroidism patients representing 39 pediatric and 87 young adult patients. Presenting symptoms, operative data, and postoperative course were compared for patients age 0-19 years and 20-29 years. Sporadic primary hyperparathyroidism was present in 81.7% and occurred less often in pediatric patients than young adult patients (74.4% vs 86.2%, P = .12). Among patients with hereditary primary hyperparathyroidism, multiple endocrine neoplasia type 1 was the most common type. Multiglandular disease was common in both pediatric (30.7%) and young adult (21.8%) patients. Following parathyroidectomy, 3 (2.3%) patients had permanent hypoparathyroidism and none had permanent recurrent laryngeal nerve paralysis. Biochemical cure at 6 months was equally likely in pediatric and young adult patients (97.1% vs 93.6%, P = .44) with comparable follow-up (78.4 months vs 69.1 months, P = .66) and rates of recurrent disease (5.9% vs 10.3%, P = .46). Recurrence was due to multiple endocrine neoplasia 1-related primary hyperparathyroidism in all cases. Although primary hyperparathyroidism is sporadic in most patients <19 years, they are more likely to have multiple endocrine neoplasia type 1-associated primary hyperparathyroidism (23%). Parathyroidectomy for primary hyperparathyroidism can be performed safely in pediatric patients with a high rate of cure. Follow-up for patients with hereditary disease is necessary. Copyright © 2016 Elsevier Inc. All rights reserved.

Personality Traits and Common Psychiatric Conditions in Adult Patients with Acne Vulgaris

PubMed Central

Çölgeçen, Emine

2015-01-01

Background We believe that instances of neuroticism and common psychiatric disorders are higher in adults with acne vulgaris than the normal population. Objective Instances of acne in adults have been increasing in frequency in recent years. The aim of this study was to investigate personality traits and common psychiatric conditions in patients with adult acne vulgaris. Methods Patients who visited the dermatology outpatient clinic at Bozok University Medical School with a complaint of acne and who volunteered for this study were included. The Symptom Checklist 90-Revised (SCL 90-R) Global Symptom Index (GSI), somatization, depression, and anxiety subscales and the Eysenck Personality Questionnaire-Revised Short Form (EPQ-RSF) were administered to 40 patients who fulfilled the inclusion criteria before treatment. The results were compared with those of a control group. Results Of the 40 patients included in this study, 34 were female and 6 were male. The GSI and the somatization, depression, and anxiety subscales of the SCL 90-R were evaluated. Patients with adult acne had statistically significant higher scores than the control group on all of these subscales. In addition, patients with adult acne had statistically significantly higher scores on the neuroticism subscale of the EPQ-RSF. Conclusion Our results show that common psychiatric conditions are frequent in adult patients with acne. More importantly, neurotic personality characteristics are observed more frequently in these patients. These findings suggest that acne in adults is a disorder that has both medical and psychosomatic characteristics and requires a multi-disciplinary approach. PMID:25673931

Transient receptor potential melastatin 8 gene polymorphism is associated with cold-induced airway hyperresponsiveness in bronchial asthma.

PubMed

Naumov, Denis E; Perelman, Juliy M; Kolosov, Victor P; Potapova, Tatyana A; Maksimov, Vladimir N; Zhou, Xiangdong

2015-11-01

Cold-induced airway hyperresponsiveness (CAH) is common in bronchial asthma (BA) patients and represents a problem for those living in cold climate. Transient receptor potential melastatin 8 (TRPM8) channel is the main cold temperature sensor in humans that could mediate cold response in asthmatics with CAH. No associations between TRPM8 gene polymorphisms and CAH have been reported. The present study involved 123 BA patients. CAH was assessed by 3-min isocapnic (5% CO2 ) cold air (-20°C) hyperventilation challenge. The c.750G > C (rs11562975), c.1256G > A (rs7593557), c.3048C > T (rs11563208) and c.3174C > G (rs11563071) polymorphisms of TRPM8 gene were genotyped by allele-specific polymerase chain reaction (PCR) and PCR with subsequent restriction fragment length polymorphism analysis. GC genotype and C allele carriers of the c.750G > C (rs11562975) polymorphism were more frequently observed to exhibit CAH. The estimated odds ratio for the GC genotype was 3.73 95%CI (1.48; 9.37), P = 0.005. Furthermore, GC heterozygotes had a prominent decrease in forced expiratory volume in 1 s after the challenge as compared to GG homozygotes (-12% (-16; -8.1) vs -6.45% (-11; -2.1), P < 0.001). GC carriers also had a marked reduction in other spirometric parameters. The GC variant of the TRPM8:c.750G > C (rs11562975) polymorphism is associated with CAH in patients with BA, which suggests a potential role of TRPM8 in CAH development. © 2015 Asian Pacific Society of Respirology.

[Treatment of Adult Schizophrenic Patients With Depot Antipsychotics].

PubMed

Jaramillo González, Luis Eduardo; Gómez Restrepo, Carlos; García Valencia, Jenny; de la Hoz Bradford, Ana María; Ávila-Guerra, Mauricio; Bohórquez Peñaranda, Adriana

2014-01-01

To determine the indications of long-acting antipsychotic injection and what its effectiveness and safety in adult patients with schizophrenia during the treatment maintenance phase. A clinical practice guideline was elaborated under the parameters of the Methodological Guide of the Ministerio de Salud y Protección Social to identify, synthesize and evaluate the evidence and make recommendations about the treatment and follow-up of adult patients with schizophrenia. The evidence of NICE guide 82 was adopted and updated. The evidence was presented to the Guideline Developing Group and recommendations, employing the GRADE system, were produced. The literature review shows that the evidence has moderate to low quality. 8 articles were used. The risk of relapse was lower with depot risperidone and paliperidone palmitate when compared with placebo. For the risk of hospitalizations comparing depot antipsychotics (APD) versus oral AP, the result is inconclusive. Globally the second-generation APD had a lower risk of discontinuation when compared with placebo. The second generation AP had higher risk of extrapyramidal syndromes than placebo, as in the use of antiparkinsonian. The comparison of second-generation AP injections versus placebo showed an increased risk of early weight gain. The use of depot antipsychotics in the maintenance phase of adult patients diagnosed with schizophrenia is recommended if there is no adherence to oral antipsychotics as the patient's preference. It is not recommended depot antipsychotics in the acute phase of schizophrenia in adults. Copyright © 2014 Asociación Colombiana de Psiquiatría. Publicado por Elsevier España. All rights reserved.

Posterior nasal septal abscess in a healthy adult patient.

PubMed

George, A; Smith, W K; Kumar, S; Pfleiderer, A G

2008-12-01

We report an extremely rare case of bilateral posterior nasal septal abscess in an otherwise healthy adult patient. Case report and a review of the world literature concerning atraumatic nasal septal abscess and its management. The development of an atraumatic nasal septal abscess is rare, but it has been reported in association with acute sinusitis, in patients with poor immunity and in children. The presentation, clinical course and treatment are discussed in the presented patient. To our knowledge, this is the first report in the world literature of a bilateral posterior septal abscess associated with acute sinusitis in an otherwise healthy adult.

Pyridoxine deficiency in adult patients with status epilepticus.

PubMed

Dave, Hina N; Eugene Ramsay, Richard; Khan, Fawad; Sabharwal, Vivek; Irland, Megan

2015-11-01

An 8-year-old girl treated at our facility for superrefractory status epilepticus was found to have a low pyridoxine level at 5 μg/L. After starting pyridoxine supplementation, improvement in the EEG for a 24-hour period was seen. We decided to look at the pyridoxine levels in adult patients admitted with status epilepticus. We reviewed the records on patients admitted to the neurological ICU for status epilepticus (SE). Eighty-one adult patients were identified with documented pyridoxine levels. For comparison purposes, we looked at pyridoxine levels in outpatients with epilepsy (n=132). Reported normal pyridoxine range is >10 ng/mL. All but six patients admitted for SE had low normal or undetectable pyridoxine levels. A selective pyridoxine deficiency was seen in 94% of patients with status epilepticus (compared to 39.4% in the outpatients) which leads us to believe that there is a relationship between status epilepticus and pyridoxine levels. Copyright © 2015 Elsevier Inc. All rights reserved.

Congenital Adrenal Hyperplasia: Review from a Surgeon’s Perspective in the Beginning of the Twenty-First Century

PubMed Central

Piaggio, Lisandro Ariel

2013-01-01

Congenital adrenal hyperplasia (CAH) most commonly due to 21-hydroxylase deficiency is the most common type of disorder of sex development. This review will focus on CAH addressing historical and current surgical techniques with their anatomical foundations, with special attention to long-term results and outcomes on sexual function, patient satisfaction, patient attitude toward surgery, and ongoing controversies in management of these patients. PMID:24400298

Ab Interno Trabeculectomy in the Adult Patient

PubMed Central

SooHoo, Jeffrey R.; Seibold, Leonard K.; Kahook, Malik Y.

2015-01-01

Glaucoma is a potentially blinding disease that affects millions of people worldwide. The mainstay of treatment is lowering of intraocular pressure (IOP) through the use of medications, laser and/or incisional surgery. The trabecular meshwork (TM) is thought to be the site of significant resistance to aqueous outflow in open angle glaucoma. Theoretically, an incision through TM or TM removal should decrease this resistance and lead to a significant reduction in IOP. This approach, commonly referred to as goniotomy or trabeculotomy, has been validated in the pediatric population and has been associated with long-term IOP control. In adults, however, removal of TM tissue has been historically associated with more limited and short-lived success. More recent evidence, reveals that even adult patients may benefit significantly from removal of diseased TM tissue and can lead to a significant reduction in IOP that is long-lasting and safe. In this review, we discuss current evidence and techniques for ab interno trabeculectomy using various devices in the adult patient. PMID:25624670

High-flow nasal cannula therapy for adult patients

PubMed Central

Zhang, Jian; Lin, Ling; Pan, Konghan; Zhou, Jiancang

2016-01-01

High-flow nasal cannula (HFNC) oxygen therapy has several physiological advantages over traditional oxygen therapy devices, including decreased nasopharyngeal resistance, washing out of the nasopharyngeal dead space, generation of positive pressure in the pharynx, increasing alveolar recruitment in the lungs, humidification of the airways, increased fraction of inspired oxygen and improved mucociliary clearance. Recently, the use of HFNC in treating adult critical illness patients has significantly increased, and it is now being used in many patients with a range of different disease conditions. However, there are no established guidelines to direct the safe and effective use of HFNC for these patients. This review article summarizes the available published literature on the positive physiological effects, mechanisms of action, and the clinical applications of HFNC, compared with traditional oxygen therapy devices. The available literature suggests that HFNC oxygen therapy is an effective modality for the early treatment of critically adult patients. PMID:27698207

Lifestyle Habits among Adult Congenital Heart Disease Patients in Malta.

PubMed

Caruana, Maryanne; Grech, Victor

2016-07-01

Adult congenital heart disease patients are prone to health risk behaviors like their peers. Such behaviors can have serious implications, particularly in patients with complex congenital lesions. We investigated patterns in tobacco smoking, alcohol consumption, substance misuse, dental reviews, and physical activity in Maltese patients. A questionnaire modeled on the 2008 European Health Interview Survey was offered to consecutive adult congenital heart disease outpatients, aged 16 years and over, between May 2013 and May 2014. Foreigners and patients with learning difficulties or cognitive impairment were excluded. Data on these habits was compared with that from 372 age-matched and sex-matched 2008 survey responders (general population cohort). There were 125 patient responders (65 males; mean age 30.64, SD 12.80 years). Adult congenital heart disease patients smoked less (patients 18.4%, general 32.0% P = .004) and had more regular dental reviews (patients 60.9%, general 45.2% P = .003) but were less physically active than the general population cohort (patients 30.6%, general 62.9% P < .001). The difference in smoking was only significant in patients born after the mid-1970s (younger) and females, while dental reviews were only better in younger and male patients. Male patients consumed alcohol more frequently (38.1%) than general population males (23.3%, P = .02). In the patient cohort, smoking was more likely with milder congenital lesions (OR 4.689, P = .007), substance misuse more likely in males (OR 5.703, P = .036) and with milder lesions (OR 5.405, P = .030) and alcohol consumption more likely in males (OR 11.697, P < .001). Although there is an overall lower uptake of some unhealthy habits by Maltese adult congenital heart disease patients, this is not consistent between sexes or age groups. Male and older patients and those with milder lesions might need more reinforcement of the advice given regarding lifestyle habits

General practitioners' use of caries-preventive agents in adult patients versus pediatric patients: findings from the dental practice-based research network.

PubMed

Riley, Joseph L; Gordan, Valeria V; Rindal, D Brad; Fellows, Jeffrey L; Williams, O Dale; Ritchie, Lloyd K; Gilbert, Gregg H

2010-06-01

In this study, the authors tested the frequency of dentists' recommendations for and use of caries-preventive agents for children as compared with adults. The authors surveyed 467 general dentists in the Dental Practice-Based Research Network who practice within the United States and treat both pediatric and adult patients. They asked dentists to identify the percentage of their patients for whom they had administered or recommended dental sealants, in-office and at-home fluoride, chlorhexidine rinse and xylitol gum. Dentists were less likely to provide adult patients than pediatric patients with in-office caries-preventive agents. However, the rate at which they recommended at-home preventive regimens for the two groups of patients was similar. Dentists with a conservative approach to caries treatment were the most likely to use and recommend the use of caries-preventive agents at similar rates in adults as in children. In addition, dentists in practices with a greater number of patients who had dental insurance were significantly more likely to provide in-office fluoride or sealants to adult patients than to pediatric patients. General dentists use in-office caries-preventive agents more commonly with their pediatric patients than with their adult patients. General dentists should consider providing additional in-office caries-preventive agents for their adult patients who are at increased risk of experiencing dental caries.

Comparative clinical outcomes between pediatric and young adult dialysis patients.

PubMed

Atkinson, Meredith A; Lestz, Rachel M; Fivush, Barbara A; Silverstein, Douglas M

2011-12-01

Published data on the comparative achievement of The Kidney Disease Dialysis Outcome Quality Initiative (KDOQI) recommended clinical performance targets between children and young adults on dialysis are scarce. To characterize the achievement of KDOQI targets among children (<18 years) and young adults (18-24 years) with prevalent end stage renal disease (ESRD), we performed a cross-sectional analysis of data collected by the Mid-Atlantic Renal Coalition, in conjunction with the 2007 and 2008 ESRD Clinical Performance Measures Projects. Data on all enrolled pediatric dialysis patients, categorized into three age groups (0-8, 9-12, 13-17 years), and on a random sample of 5% of patients ≥ 18 years in ESRD Network 5 were examined for two study periods: hemodialysis (HD) data were collected from October to December 2006 and from October to December 2007 and peritoneal dialysis (PD) data were collected from October 2006 to March 2007 and from October 2007 to March 2008. In total, 114 unique patients were enrolled the study, of whom 41.2% (47/114) were on HD and 58.8% (67/114) on PD. Compared to the pediatric patients, young adults were less likely to achieve the KDOQI recommended serum phosphorus levels and serum calcium × phosphorus product values, with less than one-quarter demonstrating values at or below each goal. Multivariate analysis revealed that both young adults and 13- to 17-year-olds were less likely to achieve target values for phosphorus [young adults: odds ratio (OR) 0.04, 95% confidence interval (95% CI) 0.01-0.19, p < 0.001; 13- to 17-year-olds: OR 0.17, 95% CI 0.04-0.77, p = 0.02] and calcium × phosphorus product (young adults: OR 0.01, 95% CI 0.002-0.09, p < 0.001; 13- to 17-year-olds: OR 0.09, 95% CI 0.02-0.56, p = 0.01) than younger children. In summary, there are significant differences in clinical indices between pediatric and young adult ESRD patients.

Patient Portal Use and Experience Among Older Adults: Systematic Review

PubMed Central

2017-01-01

Background The older adult population (65 years or older) in the United States is growing, and it is important for communities to consider ways to support the aging population. Patient portals and electronic personal health records (ePHRs) are technologies that could better serve populations with the highest health care needs, such as older adults. Objective The aim of this study was to assess the existing research landscape related to patient portal and ePHR use and experience among older adults and to understand the benefits and barriers to older adults’ use and adoption of patient portals and ePHRs. Methods We searched six pertinent bibliographic databases for papers, published from 2006 to 2016 and written in English, that focused on adults 60 years or older and their use of or experience with patient portals or ePHRs. We adapted preferred reporting items for systematic reviews and meta-analyses (PRISMA) guidelines to review papers based on exclusion and inclusion criteria. We then applied thematic analysis to identify key themes around use, experience, and adoption. Results We retrieved 199 papers after an initial screening and removal of duplicate papers. Then we applied an inclusion and exclusion criteria, resulting in a final set of 17 papers that focused on 15 separate projects. The majority of papers described studies involving qualitative research, including interviews and focus groups. They looked at the experience and use of ePHRs and patient portals. Overall, we found 2 main barriers to use: (1) privacy and security and (2) access to and ability to use technology and the Internet. We found 2 facilitators: (1) technical assistance and (2) family and provider advice. We also reported on older adults’ experience, including satisfaction with the system and improvement of the quality of their health care. Several studies captured features that older adults wanted from these systems such as further assistance managing health-related tasks and contextual

Alpha-1-antitrypsin phenotypes in adult liver disease patients

PubMed Central

Alempijevic, Tamara; Milutinovic, Aleksandra Sokic; Kovacevic, Nada

2009-01-01

Alpha-1-antitrypsin (AAT) is an important serine protease inhibitor in humans. Hereditary alpha-1-antitrypsin deficiency (AATD) affects lungs and liver. Liver disease caused by AATD in paediatric patients has been previously well documented. However, the association of liver disease with alpha-1-antitrypsin gene polymorphisms in adults is less clear. Therefore, we aimed to study AAT polymorphisms in adults with liver disease. We performed a case-control study. AAT polymorphisms were investigated by isoelectric focusing in 61 patients with liver cirrhosis and 9 patients with hepatocellular carcinoma. The control group consisted of 218 healthy blood donors. A significant deviation of observed and expected frequency of AAT phenotypes from Hardy-Weinberg equilibrium (chi-square = 34.77, df 11, P = 0.000) in the patient group was caused by a higher than expected frequency of Pi ZZ homozygotes (f = 0.0143 and f = 0.0005, respectively, P = 0.000). In addition, Pi M homozygotes were more frequent in patients than in controls (63% and 46%, respectively, P = 0.025). Our study results show that Pi ZZ homozygosity in adults could be associated with severe liver disease. Presence of Pi M homozygosity could be associated with liver disease via some mechanism different from Z allele-induced liver damage through accumulation of AAT polymers. PMID:19961268

Minimal and moderate oral sedation in the adult special needs patient.

PubMed

Coke, John M; Edwards, Michael D

2009-04-01

Oral minimal/moderate sedation can be an effective tool to aid in the dental management of adult special needs patients. Specific sedative drugs must be chosen by the dentist that can be used safely and effectively on these patients. This article focuses on a select number of these drugs, specific medical and pharmacologic challenges presented by adult special needs patients, and techniques to safely administer oral minimal and moderate sedation.

42 CFR 485.616 - Condition of participation: Agreements.

Code of Federal Regulations, 2011 CFR

2011-10-01

...: Agreements for credentialing and privileging of telemedicine physicians and practitioners. (1) The governing body of the CAH must ensure that, when telemedicine services are furnished to the CAH's patients... to its physicians or practitioners providing telemedicine services: (i) Determine, in accordance with...

42 CFR 485.616 - Condition of participation: Agreements.

Code of Federal Regulations, 2014 CFR

2014-10-01

...: Agreements for credentialing and privileging of telemedicine physicians and practitioners. (1) The governing body of the CAH must ensure that, when telemedicine services are furnished to the CAH's patients... to its physicians or practitioners providing telemedicine services: (i) Determine, in accordance with...

42 CFR 485.616 - Condition of participation: Agreements.

Code of Federal Regulations, 2012 CFR

2012-10-01

...: Agreements for credentialing and privileging of telemedicine physicians and practitioners. (1) The governing body of the CAH must ensure that, when telemedicine services are furnished to the CAH's patients... to its physicians or practitioners providing telemedicine services: (i) Determine, in accordance with...

42 CFR 485.616 - Condition of participation: Agreements.

Code of Federal Regulations, 2013 CFR

2013-10-01

...: Agreements for credentialing and privileging of telemedicine physicians and practitioners. (1) The governing body of the CAH must ensure that, when telemedicine services are furnished to the CAH's patients... to its physicians or practitioners providing telemedicine services: (i) Determine, in accordance with...

Experience with long-term glucocorticoid treatment in congenital adrenal hyperplasia: growth pattern compared with genetic height potential.

PubMed

Aycan, Zehra; Ocal, Gonul; Berberoglu, Merih; Cetinkaya, Ergun; Adiyaman, Pelin; Evliyaoglu, Olcay

2006-03-01

Long-term replacement treatment with high doses of steroids in congenital adrenal hyperplasia (CAH) is known to have a negative influence on growth. We evaluated the effects of long-term steroid treatment in patients with classical CAH on height development in relation to genetic height potential. Twenty-three patients with CAH (16 females, 7 males, mean age: 9.8 +/- 3.5 years) were included in this longitudinal study. The effect of steroid treatment on growth was determined by monitoring patients for 8.61 +/- 3.46 years (2-17 years) while they were treated with hydrocortisone at a mean dosage of 17.64 +/- 3.60 mg/m2/day. The height standard deviation scores (Ht-SDS), target Ht-SDS, and corrected Ht-SDS for target height was calculated for all patients. Predicted adult height according to bone age was calculated and it was determined whether height was developing according to the genetic height potential. In addition, patients were grouped as 'tight control' or 'poor control' according to their mean serum 17OH-progesterone or ACTH levels while on treatment. We evaluated whether height development was different for the tight and poor control groups. The mean chronological age of our patients at the time of the study was 9.89 +/- 3.53 years, Ht-SDS -0.77 +/- 1.57, target height (TH) 161.03 +/- 6.54 cm, TH-SDS -0.60 +/- 0.90, predicted height (PH) 157.2 +/- 11.16 cm, PH-SDS -1.1 +/- 1.69, and corrected Ht-SDS -0.75 +/- 1.14. There was no significant difference between the actual Ht-SDS and TH-SDS of our patients (p >0.05) but the corrected Ht-SDS was less than zero. Only 28.5% of our patients had normal height according to their genetic potential while 71.5% were shorter than their genetic height potential. While the Ht-SDS and corrected Ht-SDS were similar in the tight and poor metabolic control groups, the predicted height was significantly greater in the tight control group. We demonstrated that a hydrocortisone dose of 17.64 +/- 3.60 mg/m2/day in classical CAH had

The utility of presacral drainage in penetrating rectal injuries in adult and pediatric patients.

PubMed

Savoie, Kate B; Beazley, Thomas M; Cleveland, Brent; Khaneki, Sina; Markel, Troy A; Hammer, Peter M; Savage, Stephanie; Williams, Regan F

2017-11-01

With changing weaponry associated with injuries in civilian trauma, there is no clinical census on the utility of presacral drainage (PSD) in penetrating rectal injuries (PRIs), particularly in pediatric patients. Patients with PRI from July 2004-June 2014 treated at two free-standing children's hospitals and two adult level 1 trauma centers were compared by age (pediatric patients ≤16 years) and PSD. A stratified analysis was performed based on age. The primary outcome was pelvic/presacral abscess. We identified 81 patients with PRI; 19 pediatric, 62 adult. Forty patients had PSD; only three pediatric patients had a drain. Adult patients were more likely to have sustained gunshot wounds (84%), whereas pediatric patients were more likely to sustain impalement injuries (59%). Pediatric patients were more likely to have distal extraperitoneal injuries (56% versus 27% in adults, P = 0.03). PSD was more common in adult patients (59% versus 14%, P = 0.0004), African-Americans (71% versus 11% Caucasian, P < 0.01), and those sustaining gun shot wounds (63% versus 18% impalement, P < 0.01); only race remained significant in stratified analysis for both adult and pediatric patients. There were three cases of pelvic/presacral abscess, all in the adult patients (P = 0.31); one patient with PSD and two without PSD (P = 0.58). In stratified analysis, there were no differences in any infectious complication between those with and without PSD. Pelvic/presacral abscess is a rare complication of PRI, especially in pediatric patients. PSD is not associated with decreased rates of infectious complications and may not be necessary in the treatment of PRI. Copyright © 2017 Elsevier Inc. All rights reserved.

Psychotherapy Termination Practices with Older Adults: Impact of Patient and Therapist Characteristics.

PubMed

Sullivan, Daniel J; Zeff, Patricia; Zweig, Richard A

2018-02-06

The aims of this study were to survey clinicians' opinions regarding psychotherapy practices in mutual termination with a specified population (depressed older adult outpatients) and to examine the patient and therapist characteristics that may influence such practices. We surveyed psychologists' (N = 96) psychotherapy termination practices, using a hypothetical depressed older adult as a referent, to assess consensus on the appropriateness of various guidelines to termination and to examine whether these differ as a function of patient and therapist characteristics. Several practices were generally agreed to be "extremely appropriate" when terminating psychotherapy with older adults, including collaborating to determine the end date of treatment and discussing patient growth. Data also indicate that patient factors, such as personality pathology, and therapist factors, such as having an Integrative theoretical orientation were associated with differential endorsement of termination practices. Identification as a geropsychologist or working regularly with older adults were associated with a more cautious approach to termination. There is substantial consensus regarding many approaches to termination, but modifications might be appropriate depending on patient characteristics. Clinicians agree on a set of fundamental termination practices when working with older adults, but modify these based on orientation and diagnosis.

[Psychological aspects in adult congenital heart disease patients].

PubMed

Lahlou-Laforêt, Khadija

2013-03-01

Patients with congenital heart disease mostly owe their survival to a saving chirurgical intervention which confers them a survivor's identity. The relationships between parents and children are strongly influenced by these early events. The effort made by parents to allow a good quality of life to their child is often successful. However, difficulties to anticipate late complications are frequently observed in adolescents and young adult patients. In adulthood, these patients seem to discover suddenly their disease, its consequences and the necessity of long term follow-up. The coexistence of several changes, i.e adolescence, change of medical team, transfer of parental decision to the young adult own decision make this period a vulnerable way. An adapted multidisciplinary support aimed to a real transition may limit the risks of breaking off and better organize long term follow-up.

[Orofacial clinical manifestations in adult patients with variable common immunodeficiency].

PubMed

Chávez-García, Aurora Alejandra; Moreno-Alba, Miguel Ángel; Elizalde-Monroy, Martín; Segura-Méndez, Nora Hilda; Romero-Flores, Jovita; Cambray-Gutiérrez, Julio César; López-Pérez, Patricia; Del Rivero-Hernández, Leonel Gerardo

2015-01-01

Common variable immunodeficiency is the primary immunodeficiency (CVID) frequently found in adults. Its prevalence is estimated from 1:25,000 to 75,000 alive newborns; there are variations by ethnic groups, it is estimated about 50-70% in Caucasian patients. Oral cavity lesions are rarely found in adult patients with CVID, there are reports about lesions on pediatric patients mostly caused by infections. To describe the orofacial lesions (oral, maxillofacial and neck area) affecting adults with CVID. A transversal, prospective study was done in patients with CVID attended at Specialties Hospital, CMN SXXI, Mexico City. Patients where examined by the oral and maxillofacial surgeon and clinical findings were reported, then the descriptive analysis of the lesions was done. We evaluated 26 patients, 16 female and 10 males, average age of 38.6 years. In 18/26 patients we found oral lesions on 7 different types. The most frequent was minor salivary glands hiperplasia (19/26),petechiae (12/26) and herpetic ulcers (7/26). In head and neck, we found 4 different lesions, the most common was lymphadenopathy <2cm (4/26). The immunologic alterations associated to CVID favors the development of lesions mainly of infectious and probably autoinmune origin that affects the oral cavity and head and neck area.

Prevalence of vitamin D deficiency and insufficiency among adult asthmatic patients in Karachi.

PubMed

Kamran, Afshan; Alam, Syed Mahboob; Qadir, Farida

2014-11-01

Vitamin D deficiency has assumed pandemic proportions all over the world. It has been documented as a frequent problem in studies of young adults, elderly person and children in other countries, but there is no reliable data on vitamin D status of adult asthmatic patients in Pakistan. To determine the prevalence of vitamin D deficiency and insufficiency in adult asthmatic patients with moderate to severe asthma using a cross-sectional study design in Basic Medical Sciences Institute, Jinnah Postgraduate Medical Centre, Karachi.311 adult asthmatic patients with moderate to severe asthma were recruited from JPMC, tertiary care hospital in Karachi. Questionnaires were administered together demographics, height, weight, nutritional and physical activity assessment. Blood samples for vitamin D measurement were also taken. Results show high prevalence of vitamin D deficiency and insufficiency (88.10%) in adult patients with moderate to severe persistent asthma. Vitamin D deficiency and insufficiency was more frequently observed in female than in male patients.67.66% of the female patients had serum vitamin D level less than 20 ng/ml as compare to 56.1% of the male patients (p=0.01).

Adult height in patients with familial male-limited precocious puberty and the role of an aromatase inhibitor in patient management.

PubMed

Lane, Laura C; Flowers, Josephine; Johnstone, Helen; Cheetham, Tim

2018-04-25

There is little adult height data in patients with familial male-limited precocious puberty (FMPP) and no management consensus. We assessed the treatment and adult height in local patients with FMPP and those reported in the literature. Growth data were obtained on four local patients with FMPP and a search performed to obtain management details and adult height data on cases in the literature. UK (90) population standards were used to calculate standard deviation scores (SDS). Adult height data were available on 25 men with FMPP of whom 21 were treated. Median adult height SDS of patients was -1.5 SD with a mid-parental target of -0.6 SD (p=0.1). Eight patients (32%) had an adult height above the mid-parental target and seven patients (28%) had a height >2 SD below the mean. The median height SD was -0.03 in untreated patients and +0.5 SD in those receiving an aromatase inhibitor. There was no relationship between height and age at presentation. Aromatase inhibitor therapy is associated with a positive height outcome in FMPP but the outcome with and without intervention is unpredictable. Clinicians need to be cautious when counselling families about the potential height outcome in FMPP.

Medication safety infrastructure in critical-access hospitals in Florida.

PubMed

Winterstein, Almut G; Hartzema, Abraham G; Johns, Thomas E; De Leon, Jessica M; McDonald, Kathie; Henshaw, Zak; Pannell, Robert

2006-03-01

The medication safety infrastructure of critical-access hospitals (CAHs) in Florida was evaluated. Qualitative assessments, including a self-administered survey and site visits, were conducted in seven of nine CAHs between January and June 2003. The survey consisted of the Institute for Safe Medication Practices Medication Safety Self-assessment, the 2003 Joint Commission on Accreditation of Healthcare Organizations patient safety goals, health information technology (HIT) questions, and medication-use-process flow charts. On-site visits included interviews of CAH personnel who had safety responsibility and inspections of pharmacy facilities. The findings were compiled into a matrix reflecting structural and procedural components of the CAH medication safety infrastructure. The nine characteristics that emerged as targets for quality improvement (QI) were medication accessibility and storage, sterile product compounding, access to drug information, access to and utilization of patient information in medication order review, advanced safety technology, drug formularies and standardized medication protocols, safety culture, and medication reconciliation. Based on weighted importance and feasibility, QI efforts in CAHs should focus on enhancing medication order review systems, standardizing procedures for handling high-risk medications, promoting an appropriate safety culture, involvement in seamless care, and investment in HIT.

Rural relevant quality measures for critical access hospitals.

PubMed

Casey, Michelle M; Moscovice, Ira; Klingner, Jill; Prasad, Shailendra

2013-01-01

To identify current and future relevant quality measures for Critical Access Hospitals (CAHs). Three criteria (patient volume, internal usefulness for quality improvement, and external usefulness for public reporting and payment reform) were used to analyze quality measures for their relevance for CAHs. A 6-member panel with expertise in rural hospital quality measurement and improvement provided input regarding the final measure selection. The relevant quality measures for CAHs include measures that are ready for reporting now and measures that need specifications to be finalized and/or a data reporting mechanism to be established. They include inpatient measures for specific medical conditions, global measures that address appropriate care across multiple medical conditions, and Emergency Department measures. All CAHs should publicly report on relevant quality measures. Acceptance of a single consolidated set of quality measures with common specifications for CAHs by all entities involved in regulation, accreditation, and payment; a phased process to implement the relevant measures; and the provision of technical assistance would help CAHs meet the challenge of reporting. © 2012 National Rural Health Association.

Unique medical issues in adult patients with mucopolysaccharidoses.

PubMed

Mitchell, John; Berger, Kenneth I; Borgo, Andrea; Braunlin, Elizabeth A; Burton, Barbara K; Ghotme, Kemel A; Kircher, Susanne G; Molter, David; Orchard, Paul J; Palmer, James; Pastores, Gregory M; Rapoport, David M; Wang, Raymond Y; White, Klane

2016-10-01

The mucopolysaccharidoses are a group of inherited metabolic diseases caused by deficiencies in enzymes involved in the sequential degradation of glycosaminoglycans (GAGs) leading to substrate accumulation in various tissues and organs. GAG accumulation can cause growth retardation and progressive damage to respiratory, cardiovascular, musculoskeletal, nervous, gastrointestinal, auditory, and visual systems. In the past, few people with severe phenotypic mucopolysaccharidosis (MPS) reached adulthood. However, better methods for diagnosis, multi-disciplinary care, and new therapies have extended lifespan, leading to an increasing number of patients surviving beyond childhood. The growing number of adult MPS patients poses significant challenges for clinicians who may not be familiar with the clinical manifestations of MPS. In addition, as new interventions have changed the natural history of these disorders, it is difficult to anticipate both the impact on life expectancy and other complications that may occur as these patients age. Because the MPS disorders are multi-organ diseases, their management requires a coordinated multi-disciplinary approach. Here we discuss the unique pattern of medical issues and multi-organ involvement in adult patients with MPS and identify the challenges that are associated with management of MPS. This review is based on information from an expert investigator meeting with MPS specialists held October 2-4, 2014 in Dublin, Ireland, as well as on current literature searches focusing on MPS and adults. Copyright © 2016 The Authors. Published by Elsevier B.V. All rights reserved.

[Eye contact in adult patients with Asperger syndrome].

PubMed

Roy, M; Wolfgang, D

2015-05-01

It is unclear if individuals with autism spectrum disorders rarely hold direct eye contact because eyes are unimportant for them, or if it is actively avoided. The aim of the current investigation was to gain a better understanding for their views on direct eye contact by exploring adult patients with Asperger syndrome. 63 adult patients with Asperger syndrome (28 females, 35 males, 21 - 62 years old) were explored about using and sensing direct eye contact by means of a standardised questionnaire. 87 % of investigated patients depict direct eye contact as being disagreeable. They describe it as arduous and distracting. Therefore they mostly actively avoid direct eye contact. The here gained knowledge about aversion towards direct eye contact in individuals with autism should lead to a stronger understanding and acceptance of this problem in the non-autistic population. © Georg Thieme Verlag KG Stuttgart · New York.

Epidemiology of adult-onset hydrocephalus: institutional experience with 2001 patients.

PubMed

Bir, Shyamal C; Patra, Devi Prasad; Maiti, Tanmoy K; Sun, Hai; Guthikonda, Bharat; Notarianni, Christina; Nanda, Anil

2016-09-01

OBJECTIVE Adult-onset hydrocephalus is not commonly discussed in the literature, especially regarding its demographic distribution. In contrast to pediatric hydrocephalus, which is related to a primary CSF pathway defect, its development in adults is often secondary to other pathologies. In this study, the authors investigated the epidemiology of adult-onset hydrocephalus as it pertains to different etiologies and in reference to age, sex, and race distributions. METHODS The authors retrospectively reviewed the clinical notes of 2001 patients with adult-onset hydrocephalus who presented to Louisiana State University Health Sciences Center within a 25-year span. Significant differences between the groups were analyzed by a chi-square test; p < 0.05 was considered significant. RESULTS The overall mean (± SEM) incidence of adult hydrocephalus in this population was 77 ± 30 per year, with a significant increase in incidence in the past decade (55 ± 3 [1990-2003] vs 102 ± 6 [2004-2015]; p < 0.0001). Hydrocephalus in a majority of the patients had a vascular etiology (45.5%) or was a result of a tumor (30.2%). The incidence of hydrocephalus in different age groups varied according to various pathologies. The incidence was significantly higher in males with normal-pressure hydrocephalus (p = 0.03) or head injury (p = 0.01) and higher in females with pseudotumor cerebri (p < 0.0001). In addition, the overall incidence of hydrocephalus was significantly higher in Caucasian patients (p = 0.0002) than in those of any other race. CONCLUSIONS Knowledge of the demographic variations in adult-onset hydrocephalus is helpful in achieving better risk stratification and better managing the disease in patients. For general applicability, these results should be validated in a large-scale meta-analysis based on a national population database.

Endoscopic Third Ventriculostomy before Posterior Fossa Tumor Surgery in Adult Patients.

PubMed

Marx, Sascha; El Damaty, Ahmed; Manwaring, Jotham; El Refaee, Ehab; Fleck, Steffen; Fritsch, Michael; Gaab, Michael R; Schroeder, H W S; Baldauf, Jörg

2018-03-01

 Obstructive hydrocephalus in patients with posterior fossa tumors is frequently seen. Treatment options include immediate tumor removal or prior cerebrospinal fluid (CSF) diversion procedures. The necessity and feasibility of an ETV in these situations has not yet been proven in adult patients.  We retrospectively reviewed our prospectively maintained database for ETVs before surgery of posterior fossa tumors in adults. The primary focus of data analyses was the question of whether the ETV was suitable to treat the acute situation of hydrocephalus without an increased rate of complications due to the special anatomical situation with a posterior fossa tumor. We also analyzed whether any further CSF diverting procedures were necessary.  A total of 40 adult patients who underwent an ETV before posterior fossa tumor surgery were analyzed. Overall, 33 patients (82.5%) had clinical signs of hydrocephalus, and all of them improved in their clinical course after ETV. Seven patients (17.5%) did not demonstrate clinical signs of hydrocephalus, but ETV was performed with prophylactic or palliative intent in six patients and one patient, respectively. No complications were observed due to ETV itself. No permanent shunting procedure was necessary in a mean follow-up of 76.5 months. Early additional CSF diverting procedures (redo ETV, external ventricular drain) were performed in five patients (12.5%).  The present series confirms the feasibility and safety of ETV before posterior fossa tumor surgery in adult patients. If patients had symptomatic hydrocephalus before tumor surgery, an ETV can be performed, followed by early elective tumor surgery. A prophylactic ETV in asymptomatic patients is not advised. Early elective tumor surgery should be performed in these patients. Georg Thieme Verlag KG Stuttgart · New York.

Manifestations and characteristics of congenital adrenal hyperplasia-associated encephalopathy.

PubMed

Abe, Yuichi; Sakai, Tetsuro; Okumura, Akihisa; Akaboshi, Shinjiro; Fukuda, Mitsumasa; Haginoya, Kazuhiro; Hamano, Shin-Ichiro; Hirano, Kouichi; Kikuchi, Kenjiro; Kubota, Masaya; Lee, Sooyoung; Maegaki, Yoshihiro; Sanefuji, Masafumi; Shimozato, Sachiko; Suzuki, Motomasa; Suzuki, Yasuhiro; Takahashi, Mitsugi; Watanabe, Kenji; Mizuguchi, Masashi; Yamanouchi, Hideo

2016-08-01

This study aimed to clarify the characteristics of acute encephalopathic episodes in patients with congenital adrenal hyperplasia (CAH), which we termed "CAH-associated encephalopathy (CAHE)." This retrospective study was conducted using a questionnaire as a nationwide survey of patients with CAH with acute encephalopathy and related episodes. Fifteen patients were recruited on the bases of clinical data that supported a diagnosis of CAHE. Fourteen patients displayed seizures at onset, and 12 patients exhibited refractory seizures. Deep coma lasting >24h was noted in 12 patients. Neuroimaging studies revealed some heterogeneous features. Diffuse or focal edematous lesions in the cerebrum, which produce high signal intensity on diffusion-weighted magnetic resonance imaging or low density on computer tomography, were found in the acute period in all 15 patients. In the chronic period, 14 patients survived, 11 of whom had some degree of neurological sequelae. Moreover, various degrees of cerebral shrinkage were observed in 11 of 14 surviving patients. Surprisingly, there were no abnormal neuroimaging findings in the basal ganglia, brainstem, and cerebellum in any patient. Our results indicated that patients with CAH have a risk of developing CAHE, and thus, they should be followed closely because not only status epilepticus or deep coma but also minor symptoms, such as fever and nausea, may lead to CAHE. Because CAHE may feature some heterogeneous encephalopathic episodes, further validation is needed to clarify its etiology. Copyright © 2016 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

Cluster Analysis on Longitudinal Data of Patients with Adult-Onset Asthma.

PubMed

Ilmarinen, Pinja; Tuomisto, Leena E; Niemelä, Onni; Tommola, Minna; Haanpää, Jussi; Kankaanranta, Hannu

Previous cluster analyses on asthma are based on cross-sectional data. To identify phenotypes of adult-onset asthma by using data from baseline (diagnostic) and 12-year follow-up visits. The Seinäjoki Adult Asthma Study is a 12-year follow-up study of patients with new-onset adult asthma. K-means cluster analysis was performed by using variables from baseline and follow-up visits on 171 patients to identify phenotypes. Five clusters were identified. Patients in cluster 1 (n = 38) were predominantly nonatopic males with moderate smoking history at baseline. At follow-up, 40% of these patients had developed persistent obstruction but the number of patients with uncontrolled asthma (5%) and rhinitis (10%) was the lowest. Cluster 2 (n = 19) was characterized by older men with heavy smoking history, poor lung function, and persistent obstruction at baseline. At follow-up, these patients were mostly uncontrolled (84%) despite daily use of inhaled corticosteroid (ICS) with add-on therapy. Cluster 3 (n = 50) consisted mostly of nonsmoking females with good lung function at diagnosis/follow-up and well-controlled/partially controlled asthma at follow-up. Cluster 4 (n = 25) had obese and symptomatic patients at baseline/follow-up. At follow-up, these patients had several comorbidities (40% psychiatric disease) and were treated daily with ICS and add-on therapy. Patients in cluster 5 (n = 39) were mostly atopic and had the earliest onset of asthma, the highest blood eosinophils, and FEV 1 reversibility at diagnosis. At follow-up, these patients used the lowest ICS dose but 56% were well controlled. Results can be used to predict outcomes of patients with adult-onset asthma and to aid in development of personalized therapy (NCT02733016 at ClinicalTrials.gov). Copyright © 2017 The Authors. Published by Elsevier Inc. All rights reserved.

Warming up Improves Speech Production in Patients with Adult Onset Myotonic Dystrophy

ERIC Educational Resources Information Center

de Swart, B.J.M.; van Engelen, B.G.M.; Maassen, B.A.M.

2007-01-01

This investigation was conducted to study whether warming up decreases myotonia (muscle stiffness) during speech production or causes adverse effects due to fatigue or exhaustion caused by intensive speech activity in patients with adult onset myotonic dystrophy. Thirty patients with adult onset myotonic dystrophy (MD) and ten healthy controls…

Clinical review of 95 patients with 46,XX disorders of sex development based on the new Chicago classification.

PubMed

Öcal, Gönül; Berberoğlu, Merih; Sıklar, Zeynep; Aycan, Zehra; Hacıhamdioglu, Bülent; Savas Erdeve, Şenay; Çamtosun, Emine; Kocaay, Pınar; Ruhi, Hatice I; Kılıç, Birim G; Tukun, Ajlan

2015-02-01

The aim of our study was to determine the etiologic distribution of 46,XX disorder of sexual development (DSD) according to the new DSD classification system and to evaluate the clinical features of this DSD subgroup in our patient cohort. The evaluation criteria and clinical findings of 95 46,XX patients were described by clinical presentation, gonadal morphology, genital anatomy, associated dysmorphic features, presence during prenatal period with/without postnatal virilization, hormonal characteristics, and presence or absence of steroidogenic defects among 319 patients with DSD. Types and ratios of each presentation of our 95 patients with 46,XX DSD were as follows: 82 had androgen excess (86.3%): (74 had classical congenital adrenal hyperplasia, 2 had CAH variant possibility of P450-oxidoreductase gene defect), 6 had disorders of ovarian development (6.3%): (1 patient had gonadal dysgenesis with virilization at birth with bilateral streak gonad, 4 patients had complete gonadal dysgenesis, and 1 patient had ovotesticular DSD) and 7 had other 46,XX DSD. Two sisters, who had 46,XX complete gonadal dysgenesis,were diagnosed with Perrault Syndrome with ovarian failure due to streak gonads and associated with sensorineural deafness. 46,XX DSD are usually derived from intrauterine virilization and CAH is the most common cause of 46,XX DSD due to fetal androgen exposure. Copyright © 2015 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.

Taiwanese adult cancer patients' reports of using complementary therapies.

PubMed

Lu, Jui-Hua; Tsay, Shiow-Luan; Sung, Su-Ching

2010-01-01

More information is needed by cancer clinicians regarding cancer patients' use of complementary and alternative medicine (CAM). In this qualitative study, in-depth interviews were used to obtain the reports of adult cancer patients regarding their use of CAM. Seven cancer patients (4 women, 3 men) who reported using CAM were recruited by snowball sampling. Content analysis was used to examine the interview transcriptions. Five themes and multiple categories were identified related to CAM use: (1) facing the challenges of cancer (I can't be defeated, need to cooperate with conventional medical treatment, rebuilding my confidence), (2) handling the physical and psychological distress of CAM use (extra loading due to the therapy, uncertainty and fear about the efficacy of CAM, being understood and supported, feeling guilty about being sick, (3) lifestyle disruption (altering social life, changing family living style), (4) having reasons for seeking other therapies (finding a way to cure the disease, boosting my immunity, improving my overall health status, and prolonging life and searching for peace of mind), and (5) unresolved practical concerns about CAM (finding an easy and effective way to practice CAM, needing CAM to be integrated into mainstream health care, and where to get the related information). Adult Taiwanese cancer patients who use CAM do experience burdens secondary to CAM use and prefer that oncology specialists be more informed about CAM. Oncology specialists who know where adult cancer patients could obtain helpful information about CAM would help to decrease the burdens that patients who use CAM experience.

Temporal Cytokine Profiles in Severely Burned Patients: A Comparison of Adults and Children

PubMed Central

Finnerty, Celeste C; Jeschke, Marc G; Herndon, David N; Gamelli, Richard; Gibran, Nicole; Klein, Matthew; Silver, Geoff; Arnoldo, Brett; Remick, Daniel; Tompkins, Ronald G

2008-01-01

A severe burn leads to hypermetabolism and catabolism resulting in compromised function and structural changes of essential organs. The release of cytokines has been implicated in this hypermetabolic response. The severity of the hypermetabolic response following burn injury increases with age, as does the mortality rate. Due to the relationship between the hypermetabolic and inflammatory responses, we sought to compare the plasma cytokine profiles following a severe burn in adults and in children. We enrolled 25 adults and 24 children who survived a flame burn covering more than 20% of total body surface area (TBSA). The concentrations of 22 cytokines were measured using the Linco multiplex array system (St. Charles, MO, USA). Large perturbations in the expression of pro- and anti-inflammatory cytokines were seen following thermal injury. During the first week following burn injury, IFN-γ, IL-10, IL-17, IL-4, IL-6, and IL-8 were detected at significantly higher levels in adults compared with children, P < 0.05. Significant differences were measured during the second week post-burn for IL-1β (higher in children) and IL-5 (higher in adults), P < 0.05. IL-18 was more abundant in children compared with adults during the third week post-burn, P < 0.05. Between post-burn d 21 and d 66, IL-1α was detected at higher concentrations in pediatric compared with adult patients, P < 0.05. Only GM-CSF expression was significantly different at all time points; it was detected at lower levels in pediatric patients, P < 0.05. Eotaxin, G-CSF, IL-13, IL-15, IP-10, MCP-1, and MIP-1α were detected at significantly different concentrations in adult compared with pediatric patients at multiple time points, P < 0.05. There were no differences in IL-12, IL-2, IL-7, or TNF levels in adult compared with pediatric burn patients at any of these time points. Following severe flame burns, the cytokine profiles in pediatric patients differ compared with those in adult patients, which may

Trauma injury in adult underweight patients

PubMed Central

Hsieh, Ching-Hua; Lai, Wei-Hung; Wu, Shao-Chun; Chen, Yi-Chun; Kuo, Pao-Jen; Hsu, Shiun-Yuan; Hsieh, Hsiao-Yun

2017-01-01

Abstract The aim of this study was to investigate and compare the injury characteristics, severity, and outcome between underweight and normal-weight patients hospitalized for the treatment of all kinds of trauma injury. This study was based on a level I trauma center Taiwan. The detailed data of 640 underweight adult trauma patients with a body mass index (BMI) of <18.5 kg/m2 and 6497 normal-weight adult patients (25 > BMI ≥ 18.5 kg/m2) were retrieved from the Trauma Registry System between January 1, 2009, and December 31, 2014. Pearson's chi-square test, Fisher's exact test, and independent Student's t-test were performed to compare the differences. Propensity score matching with logistic regression was used to evaluate the effect of underweight on mortality. Underweight patients presented a different bodily injury pattern and a significantly higher rate of admittance to the intensive care unit (ICU) than did normal-weight patients; however, no significant differences in the Glasgow Coma Scale (GCS) score, injury severity score (ISS), in-hospital mortality, and hospital length of stay were found between the two groups. However, further analysis of the patients stratified by two major injury mechanisms (motorcycle accident and fall injury) revealed that underweight patients had significantly lower GCS scores (13.8 ± 3.0 vs 14.5 ± 2.0, P = 0.020), but higher ISS (10.1 ± 6.9 vs 8.4 ± 5.9, P = 0.005), in-hospital mortality (odds ratio, 4.4; 95% confidence interval, 1.69–11.35; P = 0.006), and ICU admittance rate (24.1% vs 14.3%, P = 0.007) than normal-weight patients in the fall accident group, but not in the motorcycle accident group. However, after propensity score matching, logistic regression analysis of well-matched pairs of patients with either all trauma, motorcycle accident, or fall injury did not show a significant influence of underweight on mortality. Exploratory data analysis revealed that underweight patients

Carbamazepine and oxcarbazepine in adult patients with Dravet syndrome: Friend or foe?

PubMed

Snoeijen-Schouwenaars, F M; Veendrick, M J B M; van Mierlo, P; van Erp, G; de Louw, A J A; Kleine, B U; Schelhaas, H J; Tan, I Y

2015-07-01

In newly diagnosed patients with Dravet syndrome sodium channel blockers are usually avoided. However, in many adult patients the diagnosis was made long after the initiation of therapy. The purpose of our study was to acquire information concerning the potential risks and benefits of (ox)carba(ma)zepine withdrawal in adult patients with genetically confirmed Dravet syndrome. We identified 16 adults with Dravet syndrome, living in a tertiary care facility for people with epilepsy and an intellectual disability. We reviewed clinical history, genetic findings, the type and duration of sodium channels blockers that were used, seizure types and frequency, and the effect of a change in these medications. The study population consisted of 9 men and 7 women. Median age was 35 years (range 20-61 years). An attempt to withdraw carbamazepine (CBZ) was made in 9 patients. In 3 of these patients an increase in tonic-clonic seizures was observed. An attempt to withdraw oxcarbazepine (OXC) was made in 3 patients, leading to a complete stop in 2 patients. 3 of the 4 deaths in the withdrawal-group were related to epilepsy. In adult patients with Dravet syndrome withdrawal of CBZ or OXC is not without risks. We suggest that (ox)carba(ma)zepine withdrawal should be considered in these patients but only if there is a good reason to do so and only if they are closely monitored. Copyright © 2015 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.

Determination of a steroid profile in heel prick blood using LC-MS/MS.

PubMed

Boelen, Anita; Ruiter, An F C; Claahsen-van der Grinten, Hedi L; Endert, Erik; Ackermans, Mariette T

2016-01-01

The aim of this study was to improve the sensitivity of the congenital adrenal hyperplasia (CAH) neonatal screening by including second-tier steroid profiling on a DBS using LC-MS. We developed a method to measure the steroid profile in DBS and established gestational age-specific reference ranges of cortisol, cortisone, 11-deoxycortisol, 21-deoxycortisol, 17-hydroxyprogesterone, testosterone, Δ4-androstenedione, corticosterone and 11-deoxycorticosterone using 450 heel prick samples of neonates, participating in the Dutch Screening Program. Analyzing 92 cards with a positive CAH screening showed that only 21-deoxycortisol was 100% specific for diagnosed CAH patients. Steroid precursors can be measured in DBS and we suggest to implement the method as a second tier testing for CAH in The Netherlands.

Congenital adrenal hyperplasia with localized aggressive periodontitis and amelogenesis imperfecta.

PubMed

Ajlan, Sumaiah Abdulbaqi

2015-11-01

Congenital adrenal hyperplasia (CAH) is an inherited medical condition that implies defects in steroid biosynthesis. The dental findings of a female patient with CAH are reported. The patient suffered from severe periodontal tissue destruction, obvious enamel defects, as well as some occlusal problems. The management approach is presented and the possibility of interrelation of her dental findings with her medical condition is discussed. © 2015 Japanese Teratology Society.

Outcomes in adult pectus excavatum patients undergoing Nuss repair

PubMed Central

Ewais, MennatAllah M; Chaparala, Shivani; Uhl, Rebecca

2018-01-01

Pectus excavatum (PEx) is one of the most common congenital chest wall deformities. Depending on the severity, presentation of PEx may range from minor cosmetic issues to disabling cardiopulmonary symptoms. The effect of PEx on adult patients has not been extensively studied. Symptoms may not occur until the patient ages, and they may worsen over the years. More recent publications have implied that PEx may have significant cardiopulmonary implications and repair is of medical benefit. Adults presenting for PEx repair can undergo a successful repair with a minimally invasive “Nuss” approach. Resolution of symptoms, improved quality of life, and satisfying results are reported. PMID:29430201

Prescribing errors in adult congenital heart disease patients admitted to a pediatric cardiovascular intensive care unit.

PubMed

Echeta, Genevieve; Moffett, Brady S; Checchia, Paul; Benton, Mary Kay; Klouda, Leda; Rodriguez, Fred H; Franklin, Wayne

2014-01-01

Adults with congenital heart disease (CHD) are often cared for at pediatric hospitals. There are no data describing the incidence or type of medication prescribing errors in adult patients admitted to a pediatric cardiovascular intensive care unit (CVICU). A review of patients >18 years of age admitted to the pediatric CVICU at our institution from 2009 to 2011 occurred. A comparator group <18 years of age but >70 kg (a typical adult weight) was identified. Medication prescribing errors were determined according to a commonly used adult drug reference. An independent panel consisting of a physician specializing in the care of adult CHD patients, a nurse, and a pharmacist evaluated all errors. Medication prescribing orders were classified as appropriate, underdose, overdose, or nonstandard (dosing per weight instead of standard adult dosing), and severity of error was classified. Eighty-five adult (74 patients) and 33 pediatric admissions (32 patients) met study criteria (mean age 27.5 ± 9.4 years, 53% male vs. 14.9 ± 1.8 years, 63% male). A cardiothoracic surgical procedure occurred in 81.4% of admissions. Adult admissions weighed less than pediatric admissions (72.8 ± 22.4 kg vs. 85.6 ± 14.9 kg, P < .01) but hospital length of stay was similar. (Adult 6 days [range 1-216 days]; pediatric 5 days [Range 2-123 days], P = .52.) A total of 112 prescribing errors were identified and they occurred less often in adults (42.4% of admissions vs. 66.7% of admissions, P = .02). Adults had a lower mean number of errors (0.7 errors per adult admission vs. 1.7 errors per pediatric admission, P < .01). Prescribing errors occurred most commonly with antimicrobials (n = 27). Underdosing was the most common category of prescribing error. Most prescribing errors were determined to have not caused harm to the patient. Prescribing errors occur frequently in adult patients admitted to a pediatric CVICU but occur more often in pediatric patients of adult weight. © 2013 Wiley

Mandibular fractures: a comparative analysis between young and adult patients in the southeast region of Turkey

PubMed Central

ATILGAN, Serhat; EROL, Behçet; YAMAN, Ferhan; YILMAZ, Nezih; UCAN, Musa Can

2010-01-01

Objective The purpose of this study was to review and compare the differences between mandibular fractures in young and adult patients. Material and Methods Patients treated at the Oral and Maxillofacial Department of Dicle University during a five-year period between 2000 and 2005 were retrospectively evaluated with respect to age groups, gender, etiology, localization and type of fractures, treatment methods and complications. Result 532 patients were included in the study, 370 (70%) males and 162 (30%) females, with a total of 744 mandibular fractures. The mean age of young patients was 10, with a male-female ratio of 2:1. The mean age of adult patients was 28, with a male-female ratio of 3:1. The most common causes of injury were falls (65%) in young patients and traffic accidents (38%) in adults. The most common fracture sites were the symphysis (35%) and condyle (36%) in young patients, and the symphysis in adults (36%). Mandibular fractures were generally treated by arch bar and maxillomandibular fixation in both young (67%) and adult (39%) patients, and 43% of the adult patients were treated by open reduction and internal fixation. Conclusion There was a similar gender, monthly and type of treatment distribution in both young and adult patients in the southeast region of Turkey. However, there were differences regarding age, etiology and fracture site. These findings between young and adult patients are broadly similar to those from other studies. Analysis of small differences may be an important factor in assessing educational and socioeconomic environments. PMID:20379677

A unique haplotype of RCCX copy number variation: from the clinics of congenital adrenal hyperplasia to evolutionary genetics

PubMed Central

Doleschall, Márton; Luczay, Andrea; Koncz, Klára; Hadzsiev, Kinga; Erhardt, Éva; Szilágyi, Ágnes; Doleschall, Zoltán; Németh, Krisztina; Török, Dóra; Prohászka, Zoltán; Gereben, Balázs; Fekete, György; Gláz, Edit; Igaz, Péter; Korbonits, Márta; Tóth, Miklós; Rácz, Károly; Patócs, Attila

2017-01-01

There is a difficulty in the molecular diagnosis of congenital adrenal hyperplasia (CAH) due to the c.955C>T (p.(Q319*), formerly Q318X, rs7755898) variant of the CYP21A2 gene. Therefore, a systematic assessment of the genetic and evolutionary relationships between c.955C>T, CYP21A2 haplotypes and the RCCX copy number variation (CNV) structures, which harbor CYP21A2, was performed. In total, 389 unrelated Hungarian individuals with European ancestry (164 healthy subjects, 125 patients with non-functioning adrenal incidentaloma and 100 patients with classical CAH) as well as 34 adrenocortical tumor specimens were studied using a set of experimental and bioinformatic methods. A unique, moderately frequent (2%) haplotypic RCCX CNV structure with three repeated segments, abbreviated to LBSASB, harboring a CYP21A2 with a c.955C>T variant in the 3′-segment, and a second CYP21A2 with a specific c.*12C>T (rs150697472) variant in the middle segment occurred in all c.955C>T carriers with normal steroid levels. The second CYP21A2 was free of CAH-causing mutations and produced mRNA in the adrenal gland, confirming its functionality and ability to rescue the carriers from CAH. Neither LBSASB nor c.*12C>T occurred in classical CAH patients. However, CAH-causing CYP21A2 haplotypes with c.955C>T could be derived from the 3′-segment of LBSASB after the loss of functional CYP21A2 from the middle segment. The c.*12C>T indicated a functional CYP21A2 and could distinguish between non-pathogenic and pathogenic genomic contexts of the c.955C>T variant in the studied European population. Therefore, c.*12C>T may be suitable as a marker to avoid this genetic confound and improve the diagnosis of CAH. PMID:28401898

A unique haplotype of RCCX copy number variation: from the clinics of congenital adrenal hyperplasia to evolutionary genetics.

PubMed

Doleschall, Márton; Luczay, Andrea; Koncz, Klára; Hadzsiev, Kinga; Erhardt, Éva; Szilágyi, Ágnes; Doleschall, Zoltán; Németh, Krisztina; Török, Dóra; Prohászka, Zoltán; Gereben, Balázs; Fekete, György; Gláz, Edit; Igaz, Péter; Korbonits, Márta; Tóth, Miklós; Rácz, Károly; Patócs, Attila

2017-06-01

There is a difficulty in the molecular diagnosis of congenital adrenal hyperplasia (CAH) due to the c.955C>T (p.(Q319*), formerly Q318X, rs7755898) variant of the CYP21A2 gene. Therefore, a systematic assessment of the genetic and evolutionary relationships between c.955C>T, CYP21A2 haplotypes and the RCCX copy number variation (CNV) structures, which harbor CYP21A2, was performed. In total, 389 unrelated Hungarian individuals with European ancestry (164 healthy subjects, 125 patients with non-functioning adrenal incidentaloma and 100 patients with classical CAH) as well as 34 adrenocortical tumor specimens were studied using a set of experimental and bioinformatic methods. A unique, moderately frequent (2%) haplotypic RCCX CNV structure with three repeated segments, abbreviated to LBSASB, harboring a CYP21A2 with a c.955C>T variant in the 3'-segment, and a second CYP21A2 with a specific c.*12C>T (rs150697472) variant in the middle segment occurred in all c.955C>T carriers with normal steroid levels. The second CYP21A2 was free of CAH-causing mutations and produced mRNA in the adrenal gland, confirming its functionality and ability to rescue the carriers from CAH. Neither LBSASB nor c.*12C>T occurred in classical CAH patients. However, CAH-causing CYP21A2 haplotypes with c.955C>T could be derived from the 3'-segment of LBSASB after the loss of functional CYP21A2 from the middle segment. The c.*12C>T indicated a functional CYP21A2 and could distinguish between non-pathogenic and pathogenic genomic contexts of the c.955C>T variant in the studied European population. Therefore, c.*12C>T may be suitable as a marker to avoid this genetic confound and improve the diagnosis of CAH.

Transition of adolescent and young adult patients with childhood-onset chronic kidney disease from pediatric to adult renal services: a nationwide survey in Japan.

PubMed

Hattori, Motoshi; Iwano, Masayuki; Sako, Mayumi; Honda, Masataka; Okada, Hirokazu; Akioka, Yuko; Ashida, Akira; Kawasaki, Yukihiko; Kiyomoto, Hideyasu; Terada, Yoshio; Hirano, Daishi; Fujieda, Mikiya; Fujimoto, Shouichi; Masaki, Takao; Maruyama, Shoichi; Mastuo, Seiich

2016-12-01

Transition of adolescent and young adult (AYA) patients with childhood-onset chronic kidney diseases (C-CKD) from pediatric to adult renal services has received increasing attention. However, information on transition of Japanese patients with C-CKD is limited. The Transition Medicine Working Group, in collaboration with the Japanese Society for Nephrology, the Japanese Society for Pediatric Nephrology and the Japanese Society of Pediatric Urology, conducted a retrospective cross-sectional study in 2014 on issues concerning the transition of Japanese patients with C-CKD. Few institutions in Japan had transition programs and/or transition coordinators for patients with C-CKD. Refusal to transfer by patients or their families, lack of concern about transition and inability to decide on transfer were common reasons for non-transfer of patients still followed by pediatric renal services. Around 25 % of patients who had ended or interrupted follow-up by pediatric renal services presented to adult renal services because of symptoms associated with C-CKD. Patients with various types of childhood-onset nephrourological diseases were transferred from pediatric to adult renal services. IgA nephropathy, minimal change nephrotic syndrome and congenital anomalies of the kidney and urinary tract were the most frequent primary kidney diseases in adult patients with C-CKD. These survey results indicate the need for introduction of transitional care for Japanese AYA patients with C-CKD. Consensus guidelines for the optimal clinical management of AYA patients with C-CKD are required to ensure the continuity of care from child to adult renal services.

Immunophenotypic analysis of adult patients with T-cell lymphoblastic lymphoma treated with hyper-CVAD.

PubMed

Kato, Harumi; Yamamoto, Kazuhito; Kodaira, Takeshi; Higuchi, Yusuke; Yamamoto, Hideyuki; Saito, Toko; Taji, Hirofumi; Yatabe, Yasushi; Nakamura, Shigeo; Kinoshita, Tomohiro

2018-03-01

Immunophenotype is an important prognostic factor for childhood and adult T-cell acute lymphoblastic leukemia. However, immunophenotypic data from adult patients with T-cell lymphoblastic lymphoma (T-LBL) are scarcely available. Subjects were unselected adult patients with T-LBL who were treated with intensive chemotherapy. Immunophenotyping of tumor cells was performed according to standard techniques. A total of eight patients with a median age of 31 years were analyzed who received hyper-CVAD treatment for LBL. Immunophenotypic analysis showed that the most common tumor type was cortical T-cell type [early T (n = 2), cortical T (n = 4), and medullary T (n = 2)]. Two patients diagnosed with early T-cell type had early disease progression. Assessment of T-cell differentiation stages in malignant T lymphoblasts would be important in choosing treatment strategies for adult patients with T-LBL.

Use of Patient Portals for Personal Health Information Management: The Older Adult Perspective

PubMed Central

Turner, Anne M.; Osterhage, Katie; Hartzler, Andrea; Joe, Jonathan; Lin, Lorelei; Kanagat, Natasha; Demiris, George

2015-01-01

The personal health information management (PHIM) practices and needs of older adults are poorly understood. We describe initial results from the UW SOARING project (Studying Older Adults & Researching Information Needs and Goals), a participatory design investigation of PHIM in older adults (60 years and older). We conducted in-depth interviews with older adults (n=74) living in a variety of residential settings about their management of personal health information. A surprising 20% of participants report using patient portals and another 16% reported prior use or anticipated use of portals in the future. Participants cite ease of access to health information and direct communication with providers as valuable portal features. Barriers to the use of patient portals include a general lack of computer proficiency, high internet costs and security concerns. Design features based on consideration of needs and practices of older adults will facilitate appeal and maximize usability; both are elements critical to adoption of tools such as patient portals that can support older adults and PHIM. PMID:26958263

Extracorporeal respiratory support in adult patients

PubMed Central

Romano, Thiago Gomes; Mendes, Pedro Vitale; Park, Marcelo; Costa, Eduardo Leite Vieira

2017-01-01

ABSTRACT In patients with severe respiratory failure, either hypoxemic or hypercapnic, life support with mechanical ventilation alone can be insufficient to meet their needs, especially if one tries to avoid ventilator settings that can cause injury to the lungs. In those patients, extracorporeal membrane oxygenation (ECMO), which is also very effective in removing carbon dioxide from the blood, can provide life support, allowing the application of protective lung ventilation. In this review article, we aim to explore some of the most relevant aspects of using ECMO for respiratory support. We discuss the history of respiratory support using ECMO in adults, as well as the clinical evidence; costs; indications; installation of the equipment; ventilator settings; daily care of the patient and the system; common troubleshooting; weaning; and discontinuation. PMID:28380189

Outcomes of interfacility critical care adult patient transport: a systematic review

PubMed Central

Fan, Eddy; MacDonald, Russell D; Adhikari, Neill KJ; Scales, Damon C; Wax, Randy S; Stewart, Thomas E; Ferguson, Niall D

2006-01-01

Introduction We aimed to determine the adverse events and important prognostic factors associated with interfacility transport of intubated and mechanically ventilated adult patients. Methods We performed a systematic review of MEDLINE, CENTRAL, EMBASE, CINAHL, HEALTHSTAR, and Web of Science (from inception until 10 January 2005) for all clinical studies describing the incidence and predictors of adverse events in intubated and mechanically ventilated adult patients undergoing interfacility transport. The bibliographies of selected articles were also examined. Results Five studies (245 patients) met the inclusion criteria. All were case-series and two were prospective in design. Due to the paucity of studies and significant heterogeneity in study population, outcome events, and results, we synthesized data in a qualitative manner. Pre-transport severity of illness was reported in only one study. The most common indication for transport was a need for investigations and/or specialist care (three studies, 220 patients). Transport modalities included air (fixed or rotor wing; 66% of patients) and ground (31%) ambulance, and commercial aircraft (3%). Transport teams included a physician in three studies (220 patients). Death during transfer was rare (n = 1). No other adverse events or significant therapeutic interventions during transport were reported. One study reported a 19% (28/145) incidence of respiratory alkalosis on arrival and another study documented a 30% overall intensive care unit mortality, while no adverse events or outcomes were reported after arrival in the three other studies. Conclusion Insufficient data exist to draw firm conclusions regarding the mortality, morbidity, or risk factors associated with the interfacility transport of intubated and mechanically ventilated adult patients. Further study is required to define the risks and benefits of interfacility transfer in this patient population. Such information is important for the planning and

CARES Foundation

MedlinePlus

... CAH) seeking both male and female adults, aged 18-80. CLICK HERE FOR MORE INFORMATION AND STUDY ... Hyperplasia” for females between the ages of 7-18 years old. CLICK HERE FOR MORE INFORMATION AND ...

Clinical characteristics of adult patients with tics and/or Tourette's syndrome.

PubMed

Ohta, Masataka; Kano, Yukiko

2003-12-01

This study was conducted to describe the natural course of tic disorders over a long period of time in Japanese adults patients with Tourette's syndrome (TS) in terms of symptomatology. An extensive literature on TS cases was reviewed selectively and 31 TS patients (mean age: 31.4 years; sex: 28 males and 3 females) at our outpatient clinic were examined. The mean follow-up period of the patients was 7.6 years (SD: 8.1; 0 to 26). All the data available for this study, including medical charts, were examined systematically by two experienced child psychiatrists. The adult patients with tic disorders could be classified into the four groups: group A - tics only, group B - tics + comorbidities, group C - comorbidities only and group D - sub-clinical (remission) cases. Our 31 subjects consisted of 10 patients (32.3%) for group A, 14 (45.2%) for group B, 7 (22.6%) for group C, and 0 for group D. Further investigation into the natural course and clinical characteristics of adult TS needs to be done in order to acquire a better understanding of the broad spectrum of TS and to make improvements to the treatment for this illness.

Characterizing Pelvic Organ Prolapse in Adult Spina Bifida Patients.

PubMed

Liu, Joceline S; Vo, Amanda X; Doolittle, Johnathan; Hamoui, Nabeel; Lewicky-Gaupp, Christina; Kielb, Stephanie J

2016-11-01

To report the distribution of pelvic organ prolapse (POP) stages in adult spina bifida (SB) patients. The severity of POP in the SB population has not been previously reported. Retrospective review of SB patients ≥18 years with a documented POP quantification examination between 2006 and 2014 were included. Patient demographics, gestation, parity, POP quantification examinations and prolapse symptoms were obtained. Thirty-three SB patients were identified with a mean age of 33.2 years. Five patients (15.2%) had stage 0 prolapse, 12 (36.4%) had stage 1, 12 (36.4%) had stage 2, 3 (9.1%) had stage 3, and 1 (3.0%) had stage 4. Of the 16 patients with advanced POP (stage 2 prolapse or greater), only 6 patients (37.5%) reported symptoms related to POP. All 6 symptomatic patients endorsed sensation of a vaginal bulge. Two of the 6 patients also reported dyspareunia. Additionally, 1 patient with advanced POP presented with vaginal bulge, noted by a caregiver, and cervical bleeding, but was otherwise asymptomatic. Twenty-four patients (72.7%) were nulliparous, and 12 of the 24 nulliparous patients (50%) demonstrated prolapse. Despite young age and frequent nulliparity, patients with SB are more likely to have POP than the general population. Additionally, the majority of SB patients with prolapse are asymptomatic. Assessment of pelvic organ prolapse should be included in the evaluation of adult SB females due to the low rate of symptoms even in the setting of advanced stage prolapse and potential impact on both urinary and bowel function. Copyright © 2016 Elsevier Inc. All rights reserved.

Assessing Quality of Life in Older Adult Patients with Skin Disorders

PubMed Central

Farage, Miranda A.; Miller, Kenneth W.; Sherman, Susan N.; Tsevat, Joel

2012-01-01

Significance for Public Health The global population is aging. In the industrial world, adults over 65 outnumber children and comprise almost 20% of the population in some countries. Older adults experience a number of skin diseases and disorders that substantially affect their quality of life. Opportunity exists for developing and validating health-related quality of life (HRQoL) measures specifically for dermatological conditions most pertinent to older patients. Older adults experience a number of skin diseases and disorders that substantially affect quality of life. In the last two decades, a number of instruments have been developed for use among general dermatology patients to assess the effects of treatment and disease progression, perceptions of well-being, and the value that patients place on their dermatologic state of health. This chapter reviews some health-related quality of life (HRQoL) (HRQoL) measures developed and validated specifically for dermatological conditions. However, opportunity exists for developing and validating HRQoL measures specifically for dermatological conditions most pertinent to older patients. PMID:22980159

The optimal blood glucose level for critically ill adult patients.

PubMed

Lv, Shaoning; Ross, Paul; Tori, Kathleen

2017-09-01

Glycaemic control is recognized as one of the important aspects in managing critically ill patients. Both hyperglycaemia and hypoglycaemia independently increase the risk of patient mortality. Hence, the identification of optimal glycaemic control is of paramount importance in the management of critically ill patients. The aim of this literature review is to examine the current status of glycaemic control in critically ill adult patients. This literature review will focus on randomized controlled trials comparing intensive insulin therapy to conventional insulin therapy, with an objective to identify optimal blood glucose level targets for critically ill adult patients. A literature review was conducted to identify large randomized controlled trials for the optimal targeted blood glucose level for critically ill adult patients published since 2000. A total of eight studies fulfilled the selection criteria of this review. With current human and technology resources, the results of the studies support commencing glycaemic control once the blood glucose level of critically ill patients reaches 10 mmol/L and maintaining this level between 8 mmol/L and 10 mmol/L. This literature review provides a recommendation for targeting the optimal blood glucose level for critically ill patients within moderate blood glucose level target range (8-10 mmol/L). The need for uniformed glucometrics for unbiased reporting and further research for optimal blood glucose target is required, especially in light of new technological advancements in closed-loop insulin delivery and monitoring devices. This literature review has revealed a need to call for consensus in the measurement and reporting of glycaemic control using standardized glucometrics. © 2017 British Association of Critical Care Nurses.

Gender role behavior, sexuality, and psychosocial adaptation in women with congenital adrenal hyperplasia due to CYP21A2 deficiency.

PubMed

Frisén, Louise; Nordenström, Anna; Falhammar, Henrik; Filipsson, Helena; Holmdahl, Gundela; Janson, Per Olof; Thorén, Marja; Hagenfeldt, Kerstin; Möller, Anders; Nordenskjöld, Agneta

2009-09-01

Gender-atypical behavior has been described in young girls as well as in women with congenital adrenal hyperplasia (CAH) due to a CYP21A2 deficiency. The aim of the study was to assess health-related, psychosexual, and psychosocial parameters and correlate the results to CYP21A2 genotype. Sixty-two Swedish women with CAH and age-matched controls completed a 120-item questionnaire and a validated quality of life instrument [psychological general well-being (PGWB) formula] to identify psychosexual and psychosocial parameters. The patients were divided into four CYP21A2 genotype groups. The women with CAH held more male-dominant occupations (30%) compared to controls (13%) (P = 0.04), especially those in the null genotype group (55%) (P = 0.006). They also reported a greater interest in rough sports (74%) compared to controls (50%) (P = 0.007). Eight women with CAH (14%) reported a prime interest in motor vehicles, compared to none of the controls (P = 0.002). Non-heterosexual orientation was reported by 19% of women with CAH (P = 0.005), 50% in the null genotype group (P = 0.0001), 30% in I2 splice (NS), and 5% in I172N (NS). PGWB total score did not differ between patients and controls. We identified increased gender-atypical behavior in women with CAH that could be correlated to the CYP21A2 genotype. This speaks in favor of dose-dependent effects of prenatal androgens on the development of higher brain functions. The impact of the disease on upbringing and interpersonal relationships did not correlate with disease severity, indicating that other factors, such as coping strategies, are important for psychosocial adaptation. This illustrates the need for psychological support to parents and patients.

Intensive Care in Critical Access Hospitals

ERIC Educational Resources Information Center

Freeman, Victoria A.; Walsh, Joan; Rudolf, Matthew; Slifkin, Rebecca T.; Skinner, Asheley Cockrell

2007-01-01

Context: Although critical access hospitals (CAHs) have limitations on number of acute care beds and average length of stay, some of them provide intensive care unit (ICU) services. Purpose: To describe the facilities, equipment, and staffing used by CAHs for intensive care, the types of patients receiving ICU care, and the perceived impact of…

Approximate Quantification in Young, Healthy Older Adults', and Alzheimer Patients

ERIC Educational Resources Information Center

Gandini, Delphine; Lemaire, Patrick; Michel, Bernard Francois

2009-01-01

Forty young adults, 40 healthy older adults, and 39 probable AD patients were asked to estimate small (e.g., 25) and large (e.g., 60) collections of dots in a choice condition and in two no-choice conditions. Participants could choose between benchmark and anchoring strategies on each collection of dots in the choice condition and were required to…

Fungal infections in adult patients on extracorporeal life support.

PubMed

Cavayas, Yiorgos Alexandros; Yusuff, Hakeem; Porter, Richard

2018-04-17

Patients on extracorporeal membrane oxygenation (ECMO) are often among the most severely ill in the intensive care unit. They are often receiving broad-spectrum antibiotics; they have multiple entry points for pathogens; and their immune system is impaired by blood circuit interaction. These factors are thought to predispose them to fungal infections. We thus aimed to evaluate the prevalence, risk factors, and prognosis of fungal infections in adults on ECMO. We conducted a retrospective cohort study using the Extracorporeal Life Support Organization registry, which compiles data on ECMO use from hundreds of international centers. We included all adult patients from 2006 to 2016 on any mode of ECMO with either a diagnosis of fungal infection or a positive fungal culture. Our study comprised 2129 adult patients (10.8%) with fungal colonization or infection. Aspergillus involvement (colonization or infection) was present in 272 patients (1.4%), of whom 35.7% survived to hospital discharge. There were 245 patients (1.2%) with Candida invasive bloodstream infection, with 35.9% survival. Risk factors for Aspergillus involvement included solid organ transplant (OR 1.83; p = 0.008), respiratory support (OR 2.75; p < 0.001), and influenza infection (OR 2.48; p < 0.001). Risk factors for candidemia included sepsis (OR 1.60; p = 0.005) and renal replacement therapy (OR 1.55; p = 0.007). In multivariable analysis, Aspergillus involvement (OR 0.40; p < 0.001) and candidemia (OR 0.47; p < 0.001) were both independently associated with decreased survival. The prevalence of Aspergillus involvement and Candida invasive bloodstream infection were not higher in patients on ECMO than what has been reported in the general intensive care population. Both were independently associated with a reduced survival. Aspergillus involvement was strongly associated with ECMO for respiratory support and influenza.

Molecular Diagnostic Experience of Whole-Exome Sequencing in Adult Patients

PubMed Central

Posey, Jennifer E.; Rosenfeld, Jill A.; James, Regis A.; Bainbridge, Matthew; Niu, Zhiyv; Wang, Xia; Dhar, Shweta; Wiszniewski, Wojciech; Akdemir, Zeynep H.C.; Gambin, Tomasz; Xia, Fan; Person, Richard E.; Walkiewicz, Magdalena; Shaw, Chad A.; Sutton, V. Reid; Beaudet, Arthur L.; Muzny, Donna; Eng, Christine M.; Yang, Yaping; Gibbs, Richard A.; Lupski, James R.; Boerwinkle, Eric; Plon, Sharon E.

2015-01-01

Purpose Whole exome sequencing (WES) is increasingly used as a diagnostic tool in medicine, but prior reports focus on predominantly pediatric cohorts with neurologic or developmental disorders. We describe the diagnostic yield and characteristics of whole exome sequencing in adults. Methods We performed a retrospective analysis of consecutive WES reports for adults from a diagnostic laboratory. Phenotype composition was determined using Human Phenotype Ontology terms. Results Molecular diagnoses were reported for 17.5% (85/486) of adults, lower than a primarily pediatric population (25.2%; p=0.0003); the diagnostic rate was higher (23.9%) in those 18–30 years of age compared to patients over 30 years (10.4%; p=0.0001). Dual Mendelian diagnoses contributed to 7% of diagnoses, revealing blended phenotypes. Diagnoses were more frequent among individuals with abnormalities of the nervous system, skeletal system, head/neck, and growth. Diagnostic rate was independent of family history information, and de novo mutations contributed to 61.4% of autosomal dominant diagnoses. Conclusion Early WES experience in adults demonstrates molecular diagnoses in a substantial proportion of patients, informing clinical management, recurrence risk and recommendations for relatives. A positive family history was not predictive, consistent with molecular diagnoses often revealed by de novo events, informing the Mendelian basis of genetic disease in adults. PMID:26633545

Effects of neurofeedback on adult patients with psychiatric disorders in a naturalistic setting.

PubMed

Cheon, Eun-Jin; Koo, Bon-Hoon; Seo, Wan-Seok; Lee, Jun-Yeob; Choi, Joong-Hyeon; Song, Shin-Ho

2015-03-01

Few well-controlled studies have considered neurofeedback treatment in adult psychiatric patients. In this regard, the present study investigates the characteristics and effects of neurofeedback on adult psychiatric patients in a naturalistic setting. A total of 77 adult patients with psychiatric disorders participated in this study. Demographic data and neurofeedback states were retrospectively analyzed, and the effects of neurofeedback were evaluated using clinical global impression (CGI) and subjective self-rating scales. Depressive disorders were the most common psychiatric disorders (19; 24.7 %), followed by anxiety disorders (18; 23.4 %). A total of 69 patients (89.6 %) took medicine, and the average frequency of neurofeedback was 17.39 ± 16.64. Neurofeedback was applied to a total of 39 patients (50.6 %) more than 10 times, and 48 patients (62.3 %) received both β/SMR and α/θ training. The discontinuation rate was 33.8 % (26 patients). There was significant difference between pretreatment and posttreatment CGI scores (<.001), and the self-rating scale also showed significant differences in depressive symptoms, anxiety, and inattention (<.001). This is a naturalistic study in a clinical setting, and has several limitations, including the absence of a control group and a heterogenous sample. Despite these limitations, the study demonstrates the potential of neurofeedback as an effective complimentary treatment for adult patients with psychiatric disorders.

Upper and Lower Urinary Tract Outcomes in Adult Myelomeningocele Patients: A Systematic Review

PubMed Central

Veenboer, Paul W.; Bosch, J. L. H. Ruud; van Asbeck, Floris W. A.; de Kort, Laetitia M. O.

2012-01-01

Background The introduction of sophisticated treatment of bladder dysfunction and hydrocephalus allows the majority of SB patients to survive into adulthood. However, no systematic review on urological outcome in adult SB patients is available and no follow-up schemes exist. Objectives To systematically summarize the evidence on outcome of urinary tract functioning in adult SB patients. Methods A literature search in PubMed and Embase databases was done. Only papers published in the last 25 years describing patients with open SB with a mean age >18 years were included. We focused on finding differences in the treatment strategies, e.g., clean intermittent catheterization and antimuscarinic drugs versus early urinary diversion, with regard to long-term renal and bladder outcomes. Results A total of 13 articles and 5 meeting abstracts on urinary tract status of adult SB patients were found describing a total of 1564 patients with a mean age of 26.1 years (range 3–74 years, with a few patients <18 years). All were retrospective cohort studies with relatively small and heterogeneous samples with inconsistent reporting of outcome; this precluded the pooling of data and meta-analysis. Total continence was achieved in 449/1192 (37.7%; range 8–85%) patients. Neurological level of the lesion and hydrocephalus were associated with incontinence. Renal function was studied in 1128 adult patients. In 290/1128 (25.7%; range 3–81.8%) patients some degree of renal damage was found and end-stage renal disease was seen in 12/958 (1.3%) patients. Detrusor-sphincter dyssynergy and detrusor-overactivity acted as adverse prognostic factors for the development of renal damage. Conclusions These findings should outline follow-up schedules for SB patients, which do not yet exist. Since renal and bladder deterioration continues beyond adolescence, follow-up of these individuals is needed. We recommend standardization in reporting the outcome of urinary tract function in adult SB

Assembling a Functional Clitoris and Vulva from a Pseudo-Penis: A Surgical Technique for an Adult Woman with Congenital Adrenal Hyperplasia.

PubMed

Tjalma, Wiebren A A

2017-06-01

Congenital adrenal hyperplasia (CAH) is associated with a genital deformation that might cause a negative body image. The genital ambiguity is generally "corrected" surgically during early infancy. The advantage is a psychological benefit. The disadvantages are multiple surgical procedures and the loss of orgasm. A 22-year-old woman with CAH consulted for genital reconstructive surgery. She had a pseudopenis of 4 cm and could achieve an orgasm by masturbating. During surgery, the penis was dismantled and with the preserved glans penis and the corpora cavernosa, a clitoris and vestibules were constructed, respectively. On the basis of the anamneses during the follow-up, she had a functional vagina and could still achieve orgasms. Genital correction surgery for CAH at an older age was easier, could be done in 1 step, and enabled the preservation of orgasm. Copyright © 2016 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.

Meaningful use of health information technology by rural hospitals.

PubMed

McCullough, Jeffrey; Casey, Michelle; Moscovice, Ira; Burlew, Michele

2011-01-01

This study examines the current status of meaningful use of health information technology (IT) in Critical Access Hospitals (CAHs), other rural, and urban US hospitals, and it discusses the potential role of Medicare payment incentives and disincentives in encouraging CAHs and other rural hospitals to achieve meaningful use. Data from the American Hospital Association (AHA) Annual Survey IT Supplement were analyzed, using t tests and probit regressions to assess whether implementation rates in CAHs and other rural hospitals are significantly different from rates in urban hospitals. Of the many measures we examined, only 4 have been met by a majority of rural hospitals: electronic recording of patient demographics and electronic access to lab reports, radiology reports, and radiology images. Meaningful use is even less prevalent among CAHs. We also find that rural hospitals lag behind urban institutions in nearly every measure of meaningful use. These differences are particularly large and significant for CAHs. The meaningful use incentive system creates many challenges for CAHs. First, investments are evaluated and subsidies determined after adoption. Thus, CAHs must accept financial risk when adopting health IT; this may be particularly important for large expenditures. Second, the subsidies may be low for relatively small expenditures. Third, since the subsidies are based on observable costs, CAHs will receive no support for their intangible costs (eg, workflow disruption). A variety of policies may be used to address these problems of financial risk, uncertain returns in a rural setting, and limited resources. © 2011 National Rural Health Association.

Late diagnosis of influenza in adult patients during a seasonal outbreak.

PubMed

Choi, Seong-Ho; Chung, Jin-Won; Kim, Tark; Park, Ki-Ho; Lee, Mi Suk; Kwak, Yee Gyung

2018-03-01

Due to advances in diagnostic techniques, clinicians are more frequently performing influenza diagnostic tests and referring to their test results ahead of the administration of neuraminidase inhibitors (NAIs). To investigate the clinical significance of the time from symptom onset to laboratory diagnosis, we reviewed the clinical characteristics of adult patients with influenza who had an early laboratory diagnosis (ED) or a late laboratory diagnosis (LD) at one of four tertiary care centers during a seasonal outbreak of influenza. Clinical data were collected from 1,405 adults during the 2013 to 2014 influenza season. A patient was regarded as receiving an ED or LD if he/she received an influenza diagnostic test at 0 to 1 or 4 to 7 days after symptom onset, respectively. Early NAI therapy and late NAI therapy were defined as the administration of NAI ≤ 2 or > 2 days after symptom onset, respectively. Nearly half of the patients (47.0%) received an ED (n = 661), whereas 13.5% (n = 190) received a LD. Patients with a LD had initial symptoms of cough, sputum production, and dyspnea and experienced pneumonia, antibiotic therapy, hospitalization, and admission to the intensive care unit more often than those with an ED. NAI therapy and early NAI therapy were less frequent in patients with a LD than those with an ED. Of the analyzed baseline characteristics, age ≥ 50 years, influenza B infection, and diagnosis using a polymerase chain reaction test were significantly associated with a LD. LD was associated with inappropriate antiviral therapy and complicated presenting features in adult patients with seasonal influenza. ED of influenza should be emphasized, especially for older adults.

Congenital adrenal hyperplasia: problems with developmental anomalies of the external genitalia and sex assignment.

PubMed

Al-Maghribi, Hussein

2007-09-01

A retrospective study was performed on all patients with congenital adrenal hyperplasia (CAH) who were followed up at the King Hussein Medical Center (KHMC), Amman, Jordan, during the period from January 1996 to June 2006. The aim was to evaluate the clinical features, special problems, and corrective interventions for these patients. The records of 73 children (39 were genetic females and 34 were genetic males) with CAH were reviewed in the study. The age of the patients at last follow-up was between five months and 18 years. Diagnostic criteria for CAH were typical clinical features of the illness (salt loss, dehydration, virilization, macrogenitosomia, ambiguous genitalia, and accelerated growth) and typical hormonal abnormalities (decreased serum cortisol and elevated serum 17-hydroxyprogesterone). There were 62 patients with classical presentation; among them, salt-wasting (SW) form was seen in 41 patients (66%). There were 5 patients with the nonclassic form, while 6 others had cryptic presentation. Seven patients (9%) had hypertension, mostly due to salt-retaining CAH. Among the 39 females with CAH, 27 had developed mental anomalies of the external genitalia; 20 of them underwent surgical interventions of their external genitalia. Fourteen genetically female patients were wrongly diagnosed as 'male sex' at birth due to severe virilization. Seven of them were reassigned 'female sex' socially, legally, and surgically; the parents of one of them (a four-year-old girl) wanted the surgical intervention postponed for two to three years. Hysterectomy and gonadectomy were carried out for 6 of the other 7 patients who chose to keep the male gender. Our study indicates that newborns with developmental anomalies of the external genitalia should be diagnosed as early as possible so that medical, psychological, and social complications are minimized. A neonatal screening program for such a disorder can identify infants at risk for the development of life

Clinical and histopathological results of the adult patients with unilateral cryptorchidism

PubMed Central

Ateş, Ferhat; Soydan, Hasan; Okçelik, Sezgin; Çırakoğlu, Abdullah; Yılmaz, İsmail; Malkoç, Ercan; Karademir, Kenan

2016-01-01

Objective To evaluate the clinical and histopathological results of adult unilateral cryptorchidism patients. Material and methods Data from adult unilateral cryptorchidism patients that underwent orchiectomy in our clinic between between January 2004 and March 2013 were retrospectively evaluated. Patients were divided into three groups as intra-abdominal, inguinal canal and superficial inguinal region according to the location of the undescended testes. Patients were also grouped according to their testicular volume (<4 cc, 4.1–12 cc, and >12 cc). Histopathology results of orchiectomy specimens were classified as follows: 1. Sertoli cells only, testicular atrophy and vanished testis (anorchia) 2. Hypospermatogenesis, and 3. Maturation arrest. Patients were grouped as normospermia, azoospermia and oligo/astheno/teratospermia groups according to semen analysis results. Correlations between testicular localization, testicular size, semen analysis and pathology results were evaluated. Incidental tumor detection rates were also calculated. Results Two hundred and forty-four adult unilateral cryptorchidism patients underwent orchiectomy in our clinic. There was no a significant relationship between location of the testis and testicular pathology results (p=0.707). Most common semen analysis results was normospermia in patients with high testicular volume group however azoospermia and oligoasthenospermia observed commonly in patients with low testicular volume group. There was a significant relationship between testicular volume and semen analysis results (p=0.023). No significant relationship was observed between semen analysis and pathological results (p=0.929). After an evaluation of all factors with possible effects on the semen analysis results, only testicular volume (p=0.036) was found to have a significant impact. Only one case (0.4%) was incidentally diagnosed seminoma after a review of 233 patients with available histopathological results on record

A Correlational Study of Spiritual Well-being and Depression in the Adult Cancer Patient.

PubMed

Stutzman, Hannah; Abraham, Sam

Depression in adult cancer patients has been widely studied, along with spiritual effects of traumatic events and even spiritual growth after a diagnosis of cancer. There has been limited research determining a direct correlation between spiritual well-being and depression in adult cancer patients. The purpose of this research study was to examine the relationship between spiritual well-being and depression in adult cancer patients. This was a descriptive correlational study using 59 patients older than 18 years from an outpatient cancer center. The researchers hypothesized that patients with a low spiritual well-being score would be more likely to have a high depressive symptom score, thus providing support for a correlation between cancer patient's spiritual well-being and risk of depression. Implications of this study lead to evidence for better screening processes for cancer patients regarding spiritual well-being.

Infective Dermatitis in an Adult Patient With HTLV-1

PubMed Central

Riveros, Rosalba; Medina, Raquel; Morel, Maida

2015-01-01

Abstract: Infective dermatitis is a chronic exudative eczematous eruption presenting in human T-lymphotropic virus type 1 (HTLV-1)–infected people. It presents with relapsing erythematous, scaly, and crusted lesions affecting simultaneously the scalp, external ear, retroauricular area, eyelid, paranasal skin, neck axilla, and groin. Superimposed Staphylococcus and Streptococcus infection are common. It mainly affects children and exceptionally adults, and there are only a few published cases. The authors present the first reported case in Paraguay of an adult patient who had symptoms of human T-lymphotropic virus type 1–associated progressive tropical spastic paraparesis, and 6 years after the onset of the neurological symptoms, the patient developed infective dermatitis lesions on the skin, with frequent exacerbations since then. PMID:26588341

The Islamic Perspectives of Gender-Related Issues in the Management of Patients With Disorders of Sex Development.

PubMed

Zainuddin, Ani Amelia; Mahdy, Zaleha Abdullah

2017-02-01

In Islam, the person with somatic sex ambiguity due to a disorder of sex development (DSD), such as 46,XX congenital adrenal hyperplasia or 46,XY androgen insensitivity, is recognized as khunsa. Two types of khunsa are distinguished: wadhih (discernible) and musykil (intractable). A recent fatwa (religious edict) in Malaysia decreed that it is permissible for male-assigned patients from these two groups to have gender reassignment surgery to female following diagnosis; however, the religious authority has yet to rule on the reassignment from female to male, if requested. The different schools of law in Islam agree on some aspects of gender-related issues like the position of khunsa in prayer congregations, but differ in their opinions on others such as property inheritance and bathing rituals. For purposes of illustration, this article includes three case reports on Muslim patients with DSD in Malaysia, focusing on issues of gender assignment: (1) a patient with 46,XX CAH, assigned as female, requesting reassignment to male; (2) a patient with 46,XX CAH, assigned female, and gender dysphoric, but undecided on the gender to be; and (3) a patient with 46,XY complete gonadal dysgenesis, raised female due to her phenotype at birth, diagnosed late, at age 18 years, and content to remain female. Gender-related issues from the perspective of Islamic jurisprudence are highlighted and discussed. To ensure holistic care, health-service providers involved in the care of Muslim patients with DSDs need to be aware of the Islamic perspectives on gender-related issues and involve expert religious authorities.

Impact of Milrinone Administration in Adult Cardiac Surgery Patients: Updated Meta-Analysis.

PubMed

Ushio, Masahiro; Egi, Moritoki; Wakabayashi, Junji; Nishimura, Taichi; Miyatake, Yuji; Obata, Norihiko; Mizobuchi, Satoshi

2016-12-01

To determine the effects of milrinone on short-term mortality in cardiac surgery patients with focus on the presence or absence of heterogeneity of the effect. A systematic review and meta-analysis. Five hundred thirty-seven adult cardiac surgery patients from 12 RCTs. Milrinone administration. The authors conducted a systematic Medline and Pubmed search to assess the effect of milrinone on short-term mortality in adult cardiac surgery patients. Subanalysis was performed according to the timing for commencement of milrinone administration and the type of comparators. The primary outcome was any short-term mortality. Overall analysis showed no difference in mortality rates in patients who received milrinone and patients who received comparators (odds ratio = 1.25, 95% CI 0.45-3.51, p = 0.67). In subanalysis for the timing to commence milrinone administration and the type of comparators, odds ratio for mortality varied from 0.19 (placebo as control drug, start of administration after cardiopulmonary bypass) to 2.58 (levosimendan as control drug, start of administration after cardiopulmonary bypass). Among RCTs to assess the effect of milrinone administration in adult cardiac surgery patients, there are wide variations of the odds ratios of administration of milrinone for short-term mortality according to the comparators and the timing of administration. This fact may suggest that a simple pooling meta-analysis is not applicable for assessing the risk and benefit of milrinone administration in an adult cardiac surgery cohort. Copyright © 2016 Elsevier Inc. All rights reserved.

Metabolic Characteristics and Risks Associated with Stone Recurrence in Korean Young Adult Stone Patients.

PubMed

Kang, Ho Won; Seo, Sung Pil; Kim, Won Tae; Kim, Yong-June; Yun, Seok-Joong; Kim, Wun-Jae; Lee, Sang-Cheol

2017-08-01

The aim of this study was to assess the metabolic characteristics and risks of stone recurrence in young adult stone patients in Korea. The medical records of 1532 patients presenting with renal or ureteric stones at our stone clinic between 1994 and 2015 were retrospectively reviewed. Patients were grouped according to age (young adult, 18-29 years; intermediate onset, 30-59 years; old age, ≥60 years) at first presentation, and measurements of clinicometabolic characteristics and risks of stone recurrence were compared. Overall, excretion of urinary stone-forming substances was highest in the intermediate onset group, followed by the young adult and old age groups. Importantly, excretion of urinary citrate was lowest in the young adult group. Kaplan-Meier analyses identified a significant difference between the three age groups in terms of stone recurrence (log rank test, p < 0.001). Multivariate Cox regression analyses revealed that age at first stone presentation was an independent risk factor for stone recurrence. Urinary citrate excretion was an independent risk factor for stone recurrence in young adult stone patients. Younger age (18-29 years) at first stone presentation was a significant risk factor for stone recurrence, and urinary citrate excretion was an independent risk factor affecting recurrence in this group. Metabolic evaluation and potassium citrate therapy should be considered for young adult stone patients to prevent recurrence.

Brain morphological changes in adolescent and adult patients with anorexia nervosa.

PubMed

Seitz, J; Herpertz-Dahlmann, B; Konrad, K

2016-08-01

Gray matter (GM) and white matter (WM) volume loss occur in the brains of patients with acute anorexia nervosa (AN) and improve again upon weight restoration. Adolescence is an important time period for AN to begin. However, little is known about the differences between brain changes in adolescents vs adults. We used a meta-analysis and a qualitative review of all MRI studies regarding acute structural brain volume changes and their recovery in adolescents and adults with AN. 29 studies with 473 acute, 121 short-term weight-recovered and 255 long-term recovered patients with AN were included in the meta-analysis. In acute AN, GM and WM were reduced compared to healthy controls. Acute adolescent patients showed a significantly greater GM reduction than adults (-8.4 vs -3.1 %), the difference in WM (-4.0 vs -2.1 %) did not reach significance. Short-term weight-recovered patients showed a remaining GM deficit of 3.6 % and a non-significant WM reduction of 0.9 % with no age differences. Following 1.5-8 years of remission, GM and WM were no longer significantly reduced in adults (GM -0.4 %, WM -0.7 %); long-term studies for adolescents were scarce. The qualitative review showed that GM volume loss was correlated with cognitive deficits and three studies found GM regions, cerebellar deficits and WM to be predictive of outcome. GM and WM are strongly reduced in acute AN and even more pronounced in adolescence. Long-term recovery appears to be complete for adults while no conclusions can be drawn for adolescents, thus caution remains.

Patient-accident-fracture (PAF) classification of acute distal radius fractures in adults.

PubMed

Herzberg, G; Galissard, T; Burnier, M

2018-05-19

There is not enough evidence in the literature to support the use of any classification system for distal radius fractures (DRF) in adults. However, there is a need for identification of more homogeneous groups of patients with DRF so that the extent of preoperative workup and sophistication of treatment would best match the needs of the patient. The authors propose an innovative method to analyse and stratify acute DRF in adults. A one-page chart includes criteria related to the patient (P), the energy of the accident (A) and the pathology of the fracture (F). Analysis of the pathology includes not only the distal radius itself but also the associated ulnar and carpal lesions. Radiological suspicion of associated carpal of distal radio-ulnar joint ligamentous injuries is included in the analysis. The preliminary results of the use of this chart in 1610 consecutive adult patients (16-102 years) with unilateral acute DRF are presented. A total of six homogeneous groups of patients are described, and the relevance of this classification regarding the therapeutic options is discussed.

Gonadotropin-Releasing Hormone Agonist Combined With a Levonorgestrel-Releasing Intrauterine System or Letrozole for Fertility-Preserving Treatment of Endometrial Carcinoma and Complex Atypical Hyperplasia in Young Women.

PubMed

Zhou, Huimei; Cao, Dongyan; Yang, Jiaxin; Shen, Keng; Lang, Jinghe

2017-07-01

The aim of this study was to evaluate the efficacy and safety with gonadotropin-releasing hormone agonist (GnRHa) combined with a levonorgestrel-releasing intrauterine system or an aromatase inhibitor (letrozole) in young women with well-differentiated early endometrial carcinoma (EC) and complex atypical hyperplasia (CAH). We performed a retrospective analysis including the clinical characteristics of 29 patients younger than 45 years with early well-differentiated endometrioid adenocarcinoma of the uterus (EC) or CAH who were treated at the Department of Obstetrics and Gynecology, Peking Union Medical College Hospital, from January 2012 to April 2016. Eighteen patients were treated with the combination of intramuscular injections of GnRHa every 4 weeks with the levonorgestrel intrauterine hormonal system (Mirena® Bayer Health Care Pharmaceutical Inc, Wayne, NY) was inserted. Eleven patients were treated with the combination of intramuscular injections of GnRHa every 4 weeks with oral letrozole 2.5 mg daily. The patients underwent follow-up with endometrial sampling by hysteroscopy and curettage for endometrial response every 3 months. After a median follow-up of 18.7 months (range, 5.6-54.9 months), 15 women (88.2%) in the EC group and 12 women (100%) in the CAH group had complete response (CR) after GnRHa combination treatment. Among the women who achieved CR, 1 woman (8.3%) with CAH and 1 woman (5.9%) with EC had recurrence after CR, and they finally underwent a hysterectomy. Time to CR was similar in the 2 groups (4.5 ± 1.9 months in the CAH group vs 5.0 ± 2.9 months in the EC group). Ten women (34.5%) had CR after the first 3 months, 8 women (27.6%) had CR after 6 months, and 9 women (31.0%) had CR after 9 months. Both GnRHa with the levonorgestrel-releasing intrauterine system and GnRHa with letrozole are alternative treatments for women with CAH and EC who desire fertility preservation. A larger multicenter trial of the fertility-preserving treatment is

Monogenic autoinflammatory diseases: General concepts and presentation in adult patients.

PubMed

Hernández-Rodríguez, José; Ruiz-Ortiz, Estíbaliz; Yagüe, Jordi

2018-01-23

Monogenic autoinflammatory diseases (AIFD) are rare disorders characterized by an uncontrolled increase of the systemic inflammatory response, which is caused by mutations in genes involved in inflammatory pathways. Over the last few years, new genes and proteins responsible for new monogenic AIFD have been identified and a substantial improvement in their treatment has been achieved. Monogenic AIFD manifestations typically begin during childhood, but they can also occur in adults. Compared to pediatric patients, adults usually present with a less severe disease and fewer long-term complications. In addition, patients with adult-onset disease carry low-penetrance mutations more often than pathogenic variants. A late-onset of AIFD may be occasionally associated with the presence of somatic mutations. In this study, we review the most frequent monogenic AIFD, and others recently described, which may occur during adulthood. Copyright © 2017 Elsevier España, S.L.U. All rights reserved.

Fractures in pituitary adenoma patients from the Dutch National Registry of Growth Hormone Treatment in Adults.

PubMed

van Varsseveld, N C; van Bunderen, C C; Franken, A A M; Koppeschaar, H P F; van der Lely, A J; Drent, M L

2016-08-01

The effects of growth hormone (GH) replacement therapy on fracture risk in adult GH deficient (GHD) patients with different etiologies of pituitary GHD are not well known, due to limited data. The aim of this study was to investigate characteristics and fracture occurrence at start of (baseline) and during long-term GH replacement therapy in GHD adults previously treated for Cushing's disease (CD) or acromegaly, compared to patients with previous nonfunctioning pituitary adenoma (NFPA). From the Dutch National Registry of Growth Hormone Treatment in Adults, a nationwide surveillance study in severe GHD adults, all patients using ≥30 days of GH replacement therapy with previous NFPA (n = 783), CD (n = 180) and acromegaly (n = 65) were selected. Patient characteristics, fractures and potential influencing factors were investigated. At baseline, patients with previous CD were younger, more often female and had more often a history of osteopenia or osteoporosis, whereas patients with previous acromegaly had more often received cranial radiotherapy and a longer duration between treatment of their pituitary tumor and start of adult GH replacement therapy. During follow-up, a fracture occurred in 3.8 % (n = 39) of all patients. Compared to patients with previous NFPA, only patients with previous acromegaly had an increased fracture risk after 6 years of GH replacement therapy. During GH replacement therapy, an increased fracture risk was observed in severe GHD adult patients previously treated for acromegaly, but not in those previously treated for CD, compared to severe GHD adult patients using GH replacement therapy because of previous NFPA. Further studies are needed to confirm these findings and to elucidate potential underlying mechanisms.

Correlates of Neuropsychological Impairment in Older Adult Pain Clinic Patients

PubMed Central

Karp, Jordan F.; Reynolds, Charles F.; Butters, Meryl; Dew, Mary Amanda; Mazumdar, Sati; Begley, Amy E.; Lenze, Eric; Weiner, Debra K.

2010-01-01

Objective Persistent pain and cognitive impairment are common in older adults. Memory and mental flexibility are cognitive domains which may be vulnerable in the aging brain. We were interested in examining the effects of persistent pain and opioid use on cognition in community dwelling, non-demented older adults. Setting Older Adult Pain Management Program. Design 57 new patients (mean age 76.1) were recruited to describe 1) rates of persistent pain conditions and pain intensity, 2) cognition (memory and mental flexibility), 3) rates and severity of depression, and 4) sleep quality. All patients had non-malignant pain for at least 3 months. Pain intensity was measured with the McGill Pain Questionnaire. Diagnosis of depression was via the Patient Health Questionnaire and depression severity assessed with the Hamilton Rating Scale for Depression. Cognition was assessed with: 1) Mini Mental State Examination, 2) number-letter-switching and motor speed trail-making subtests, 3) Digit Symbol Subtest of the WAIS-R, and 4) free and paired recall of the WAIS-R. To determine which variables predicted poorer outcomes on mental flexibility tests, these variables were entered into a multiple regression. Results Pain severity was associated with impaired number-letter switching (r = −0.42, p = 0.002). Multiple regression showed pain severity was associated with impaired mental flexibility (parameter estimate = −0.29 (t = −2.00), p = 0.05). Patients taking opioids had worse memory (t = 2.17, df = 39, p = 0.04). Conclusions In community-dwelling older adults, pain severity is associated with impaired mental flexibility. In addition, opioids may increase memory problems. PMID:17014605

Characteristics of Avoidant/Restrictive Food Intake Disorder in a Cohort of Adult Patients.

PubMed

Nakai, Yoshikatsu; Nin, Kazuko; Noma, Shun'ichi; Teramukai, Satoshi; Wonderlich, Stephen A

2016-11-01

To assess and compare clinical symptoms and psychometric analysis of adult patients with avoidant/restrictive food intake disorder (ARFID) with those with anorexia nervosa (AN). We completed a retrospective review of adult patients with a feeding and eating disorder assessed between 1990 and 2005 that qualified for a diagnosis of ARFID. Patients with ARFID were compared with those with AN, with respect to the demographics, clinical symptoms and psychometric analysis. Using the criteria of the fifth edition of the Diagnostic and Statistical Manual, 95 (9.2%) of 1029 patients with a feeding and eating disorder met the criteria for ARFID. All patients with ARFID were women. The ARFID group had a significantly shorter duration of illness, lower rates of hospital admission history and less severe psychopathology than the AN group. Adult patients with ARFID in this study were clinically distinct from those with AN and somewhat different from paediatric patients with ARFID in previous studies. Copyright © 2016 John Wiley & Sons, Ltd and Eating Disorders Association. Copyright © 2016 John Wiley & Sons, Ltd and Eating Disorders Association.

Structure-based analysis of five novel disease-causing mutations in 21-hydroxylase-deficient patients.

PubMed

Minutolo, Carolina; Nadra, Alejandro D; Fernández, Cecilia; Taboas, Melisa; Buzzalino, Noemí; Casali, Bárbara; Belli, Susana; Charreau, Eduardo H; Alba, Liliana; Dain, Liliana

2011-01-11

Congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency is the most frequent inborn error of metabolism, and accounts for 90-95% of CAH cases. The affected enzyme, P450C21, is encoded by the CYP21A2 gene, located together with a 98% nucleotide sequence identity CYP21A1P pseudogene, on chromosome 6p21.3. Even though most patients carry CYP21A1P-derived mutations, an increasing number of novel and rare mutations in disease causing alleles were found in the last years. In the present work, we describe five CYP21A2 novel mutations, p.R132C, p.149C, p.M283V, p.E431K and a frameshift g.2511_2512delGG, in four non-classical and one salt wasting patients from Argentina. All novel point mutations are located in CYP21 protein residues that are conserved throughout mammalian species, and none of them were found in control individuals. The putative pathogenic mechanisms of the novel variants were analyzed in silico. A three-dimensional CYP21 structure was generated by homology modeling and the protein design algorithm FoldX was used to calculate changes in stability of CYP21A2 protein. Our analysis revealed changes in protein stability or in the surface charge of the mutant enzymes, which could be related to the clinical manifestation found in patients.

Structure-Based Analysis of Five Novel Disease-Causing Mutations in 21-Hydroxylase-Deficient Patients

PubMed Central

Fernández, Cecilia; Taboas, Melisa; Buzzalino, Noemí; Casali, Bárbara; Belli, Susana; Charreau, Eduardo H.; Alba, Liliana; Dain, Liliana

2011-01-01

Congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency is the most frequent inborn error of metabolism, and accounts for 90–95% of CAH cases. The affected enzyme, P450C21, is encoded by the CYP21A2 gene, located together with a 98% nucleotide sequence identity CYP21A1P pseudogene, on chromosome 6p21.3. Even though most patients carry CYP21A1P-derived mutations, an increasing number of novel and rare mutations in disease causing alleles were found in the last years. In the present work, we describe five CYP21A2 novel mutations, p.R132C, p.149C, p.M283V, p.E431K and a frameshift g.2511_2512delGG, in four non-classical and one salt wasting patients from Argentina. All novel point mutations are located in CYP21 protein residues that are conserved throughout mammalian species, and none of them were found in control individuals. The putative pathogenic mechanisms of the novel variants were analyzed in silico. A three-dimensional CYP21 structure was generated by homology modeling and the protein design algorithm FoldX was used to calculate changes in stability of CYP21A2 protein. Our analysis revealed changes in protein stability or in the surface charge of the mutant enzymes, which could be related to the clinical manifestation found in patients. PMID:21264314

Serial cardiac MRIs in adult Fontan patients detect progressive hepatic enlargement and congestion.

PubMed

Lewis, Matthew J; Hecht, Elizabeth; Ginns, Jonathan; Benton, Joshua; Prince, Martin; Rosenbaum, Marlon S

2017-03-01

The progression of hepatic disease in adult Fontan patients is not well understood. They reviewed the experience with serial cardiac MRIs (CMR) in adult Fontan patients to determine if hepatic anatomic markers of prolonged Fontan exposure were present and if clinical predictors of progressive hepatic congestion could be identified. A retrospective cohort study of all adult Fontan patients who had undergone at least two CMRs was performed. Hepatic dimensions, inferior vena cava (IVC) size, right hepatic vein (RHV) size and spleen diameter were determined from images acquired at the time of clinically guided CMR. Two radiologists with expertise in hepatic imaging graded congestion and liver size independently using post-gadolinium contrast sequences. Twenty-seven patients met inclusion criteria. Over a mean time of 5.1 years between CMRs, there was a significant increase in mean lateral-medial hepatic dimension (P = .005), mean RHV diameter (P = .004), and mean splenic diameter (P = .001). Serial post-gadolinium imaging was available in 25/27 (93%) patients of which 15/27 (55%) showed evidence of progressive hepatic congestion across serial studies. Progressive hepatic congestion was associated with single ventricle ejection fraction (SVEF) less than 50% (P = .008), and larger indexed end-diastolic (EDVI) and end-systolic volume (ESVI). RHV diameter was the only anatomic variable significantly correlated with time from Fontan completion (P = .004). Serial CMRs detected progressive liver and hepatic vein enlargement in our cohort of adult Fontan patients over a mean time of 5.2 years. Progressive hepatic congestion occurs in a significant number of adult Fontan patients and may be associated with ventricular enlargement and decreased ventricular function by CMR. © 2016 Wiley Periodicals, Inc.

Antithyroid Antibodies Are Implicated in Epileptogenesis of Adult Patients With Epilepsy.

PubMed

Tsai, Meng-Han; Fu, Ting-Ying; Chen, Nai-Ching; Shih, Fu-Yuan; Lu, Yan-Ting; Cheng, Mei-Yun; Chuang, Hung-Yi; Chuang, Yao-Chung

2015-07-01

Antithyroid antibodies (Abs) are associated with epilepsy in steroid-responsive encephalopathy, but have been rarely studied in unselected epilepsy patients. This study aimed to characterize the prevalence and associated factors of antithyroid Abs and other auto-Abs in adult patients with epilepsy.Epilepsy patients without autoimmune disorders were surveyed for antinuclear antibody (ANA), anti-β2 glycoprotein 1 antibody (aβ2GP1), anticardiolipin IgG Ab, antimicrosomal antibody (AMA), antithyroglobulin antibody (ATA), and thyroid function test.Of 319 patients, 75 (23.5%) were positive for at least 1 Ab. The most common Ab was anticardiolipin antibody (aCL) (30/319, 9.4%), followed by AMA (24/319, 7.5%), ANA (18/319, 5.6%), aβ2GP1 (18/319, 6.5%), and ATA (6/319, 3.25%). Antimicrosomal Abs were significantly more frequent in patients who were female, older at disease onset, older at the time of study, and had unknown seizure etiology. The presence of aCL was significantly associated with more frequent seizures. Most patients with antithyroid Ab were female and had focal seizures with unknown etiology.The association of different auto-Abs with different factors suggests that they may have different roles in adult patients with epilepsy. Recurrent seizures and certain antiepileptic medications may cause the production of aCL. The role of antithyroid Abs in adult focal epilepsy with unknown cause, especially in females, warrants further evaluation because of the potential implications on treatment.

Antithyroid Antibodies Are Implicated in Epileptogenesis of Adult Patients With Epilepsy

PubMed Central

Tsai, Meng-Han; Fu, Ting-Ying; Chen, Nai-Ching; Shih, Fu-Yuan; Lu, Yan-Ting; Cheng, Mei-Yun; Chuang, Hung-Yi; Chuang, Yao-Chung

2015-01-01

Abstract Antithyroid antibodies (Abs) are associated with epilepsy in steroid-responsive encephalopathy, but have been rarely studied in unselected epilepsy patients. This study aimed to characterize the prevalence and associated factors of antithyroid Abs and other auto-Abs in adult patients with epilepsy. Epilepsy patients without autoimmune disorders were surveyed for antinuclear antibody (ANA), anti-β2 glycoprotein 1 antibody (aβ2GP1), anticardiolipin IgG Ab, antimicrosomal antibody (AMA), antithyroglobulin antibody (ATA), and thyroid function test. Of 319 patients, 75 (23.5%) were positive for at least 1 Ab. The most common Ab was anticardiolipin antibody (aCL) (30/319, 9.4%), followed by AMA (24/319, 7.5%), ANA (18/319, 5.6%), aβ2GP1 (18/319, 6.5%), and ATA (6/319, 3.25%). Antimicrosomal Abs were significantly more frequent in patients who were female, older at disease onset, older at the time of study, and had unknown seizure etiology. The presence of aCL was significantly associated with more frequent seizures. Most patients with antithyroid Ab were female and had focal seizures with unknown etiology. The association of different auto-Abs with different factors suggests that they may have different roles in adult patients with epilepsy. Recurrent seizures and certain antiepileptic medications may cause the production of aCL. The role of antithyroid Abs in adult focal epilepsy with unknown cause, especially in females, warrants further evaluation because of the potential implications on treatment. PMID:26131823

Readability of online patient education materials on adult reconstruction Web sites.

PubMed

Polishchuk, Daniil L; Hashem, Jenifer; Sabharwal, Sanjeev

2012-05-01

Recommended readability of patient education materials is sixth-grade level or lower. Readability of 212 patient education materials pertaining to adult reconstruction topics available from the American Academy of Orthopaedic Surgeons, American Association of Hip and Knee Surgeons, and 3 other specialty and private practitioner Web sites was assessed using the Flesch-Kincaid grade formula. The mean Flesch-Kincaid score was 11.1 (range, 3-26.5). Only 5 (2%) articles had a readability level of sixth grade or lower. Readability of most of the articles for patient education on adult reconstruction Web sites evaluated may be too advanced for a substantial portion of patients. Further studies are needed to assess the optimal readability level of health information on the Internet. Copyright © 2012 Elsevier Inc. All rights reserved.

Molecular diagnostic experience of whole-exome sequencing in adult patients.

PubMed

Posey, Jennifer E; Rosenfeld, Jill A; James, Regis A; Bainbridge, Matthew; Niu, Zhiyv; Wang, Xia; Dhar, Shweta; Wiszniewski, Wojciech; Akdemir, Zeynep H C; Gambin, Tomasz; Xia, Fan; Person, Richard E; Walkiewicz, Magdalena; Shaw, Chad A; Sutton, V Reid; Beaudet, Arthur L; Muzny, Donna; Eng, Christine M; Yang, Yaping; Gibbs, Richard A; Lupski, James R; Boerwinkle, Eric; Plon, Sharon E

2016-07-01

Whole-exome sequencing (WES) is increasingly used as a diagnostic tool in medicine, but prior reports focus on predominantly pediatric cohorts with neurologic or developmental disorders. We describe the diagnostic yield and characteristics of WES in adults. We performed a retrospective analysis of consecutive WES reports for adults from a diagnostic laboratory. Phenotype composition was determined using Human Phenotype Ontology terms. Molecular diagnoses were reported for 17.5% (85/486) of adults, which is lower than that for a primarily pediatric population (25.2%; P = 0.0003); the diagnostic rate was higher (23.9%) for those 18-30 years of age compared to patients older than 30 years (10.4%; P = 0.0001). Dual Mendelian diagnoses contributed to 7% of diagnoses, revealing blended phenotypes. Diagnoses were more frequent among individuals with abnormalities of the nervous system, skeletal system, head/neck, and growth. Diagnostic rate was independent of family history information, and de novo mutations contributed to 61.4% of autosomal dominant diagnoses. Early WES experience in adults demonstrates molecular diagnoses in a substantial proportion of patients, informing clinical management, recurrence risk, and recommendations for relatives. A positive family history was not predictive, consistent with molecular diagnoses often revealed by de novo events, informing the Mendelian basis of genetic disease in adults.Genet Med 18 7, 678-685.

Sexual functioning of adult patients born with meningomyelocele. A pilot study.

PubMed

Vroege, J A; Zeijlemaker, B Y; Scheers, M M

1998-01-01

This pilot study was started to get an impression of the sexual activity and sexual difficulties of adult patients born with meningomyelocele in control at Utrecht University Hospital, and to find out whether these problems deserve more attention from the Spina Bifida Team. Seventeen patients (9 men, 8 women; aged 19-44) were asked to participate; 11 (8 men, 3 women) decided to take part in this study. All these patients were interviewed by their physician about their present sexual life; 6 of them also completed the 'Questionnaire for screening Sexual Dysfunctions' (QSD). With 1 exception, all patients expressed a desire for sexual contact. Five of them masturbated and 6 patients sometimes had sexual contact. Eight patients had problems in the field of sexuality, but only 3 were dissatisfied with their present sexual life. It is advantageous to invite adult patients born with meningomyelocele for an interview about their sexual life. When raising this subject, however, one has to act with caution: for quite a few patients (in this study mostly women) sexuality still is a delicate subject.

A Future for Adult Educators in Patient Education

ERIC Educational Resources Information Center

Fleming, Jean E.

2014-01-01

Adult education in healthcare comes in several forms: degree and certificate programs aimed at preparing better academic and clinical educators; and community education programs aimed at wellness, rehabilitation, or learning to live with chronic diseases. Patient-centered healthcare, however, is part of something new: coordinated and transitional…

Influenza vaccination in adult patients with solid tumours treated with chemotherapy.

PubMed

Vollaard, Albert; Schreuder, Imke; Slok-Raijmakers, Lizzy; Opstelten, Wim; Rimmelzwaan, Guus; Gelderblom, Hans

2017-05-01

Patients with solid tumours receiving chemotherapy are at risk for influenza complications. Yearly influenza vaccination is recommended to patients treated with chemotherapy. However, adherence to vaccination is low, most likely due to lack of data on efficacy, optimal timing and safety of vaccination. There is scarce evidence for the effectiveness of the influenza vaccine in adult patients with solid tumours and chemotherapy on reduction of pneumonia, decreased mortality and fewer interruptions of oncological treatment. A review of 20 non-randomised serological studies in adult patients with different cancer types and chemotherapy provides insight in general trends of response to vaccination. Overall, the magnitude of the antibody response after influenza vaccination (i.e. seroconversion) can be lower than in healthy controls, but the majority of patients with solid tumours is able to mount a timely, protective immunological response (i.e. seroprotection) regardless of chemotherapy schedule, similar to healthy controls. Small sample sizes, patient heterogeneity and lack of comparable study designs limit more specific recommendations related to cancer type and optimal timing of vaccination. The inactivated influenza vaccine is safe to administer to immunosuppressed patients; side-effects are similar to those in healthy individuals. Although vaccination before start of chemotherapy is preferred to ensure optimal protection in adults with solid tumours, also vaccination during chemotherapy can reduce influenza-related complications considering the overall trends in serological response. Given the increased morbidity and mortality of influenza, influenza vaccination should be advocated as an inexpensive and safe preventive measure in patients with solid tumours receiving chemotherapy. Copyright © 2017 Elsevier Ltd. All rights reserved.

Fibromyalgia in 300 adult index patients with primary immunodeficiency.

PubMed

Barton, James C; Bertoli, Luigi F; Barton, Jackson C; Acton, Ronald T

2017-01-01

We sought to determine the prevalence and clinical and laboratory associations of fibromyalgia in adults with primary immunodeficiency (immunoglobulin (Ig) G subclass deficiency (IgGSD) and common variable immunodeficiency (CVID). We performed a retrospective analysis of these observations in 300 non-Hispanic white adult index patients with recurrent/severe respiratory tract infections and IgGSD or CVID: age; sex; IgGSD; fibromyalgia; chronic fatigue; autoimmune conditions (ACs); interstitial cystitis (IC); diabetes; body mass index; serum Ig isotypes; blood lymphocytes and subsets; and human leukocyte antigen (HLA)-A and -B types and haplotypes. We performed univariate comparisons, logistic multivariable regressions, and an analysis of covariance. Mean age was 49 ± 12 (standard deviation) y. There were 246 women (82.0%). IgGSD was diagnosed in 276 patients (92.0%). Fifty-six patients had fibromyalgia (18.7%; female:male 13:1). Other characteristics included: chronic fatigue, 63.0%; aggregate ACs, 35.3%; Sjögren's syndrome, 8.0%; IC, 3.0%; diabetes, 10.3%; and HLA-A*29, B*44 positivity, 9.7%. Prevalences of female sex; chronic fatigue; IC; and HLA-A*29, B*44 positivity were greater in patients with fibromyalgia. Logistic regression on fibromyalgia revealed three positive associations: chronic fatigue (p=0.0149; odds ratio 2.6 [95% confidence interval 1.2, 5.6]); Sjögren's syndrome (p=0.0004; 5.2 [2.1, 13.2]); and IC (p=0.0232; 5.7 [1.3, 25.7]). In an analysis of covariance, there were significant interactions of chronic fatigue, Sjögren's syndrome, and interstitial cystitis on fibromyalgia. Fibromyalgia is common in non-Hispanic white adult index patients with primary immunodeficiency, especially women. Chronic fatigue, Sjögren's syndrome, and IC are significantly associated with fibromyalgia after adjustment for other independent variables.

Liver function tests abnormality and clinical severity of dengue infection in adult patients.

PubMed

Kittitrakul, Chatporn; Silachamroon, Udomsak; Phumratanaprapin, Weerapong; Krudsood, Srivicha; Wilairatana, Polrat; Treeprasertsuk, Sombat

2015-01-01

The clinical manifestations of dengue infection in the adult are different from those in children, i.e. having less prevalence to bleeding, and more commonly, abnormal liver function tests. The primary objective is to describe the clinical manifestations of dengue infection in adult patients. The secondary objective is to compare the clinical manifestations of dengue infection between the groups of normal and abnormal liver function tests in adult patients. Retrospective study was done in adults (age 15 years) dengue patients admitted at the Hospital for Tropical Diseases from 2000-2002. Dengue infection diagnosed by WHO clinical criteria 1997 with serological tests confirmed by ELISA test or Rapid Immunochromatographic test. Liver function test was recorded by day of fever. There were 127 adult dengue patients with mean age 26.4 ± 11.5 years. Classifications of dengue infection by WHO criteria were DF 4.7%, DHF grade 126.0%, DHF grade 2 63.0% and DHF grade 3 6.3%. Mean duration of fever clearance time was 6.0 ± 1.9 days but the fever lasted longer in cases of high-level transaminases (> 10 folds). The common presenting symptoms and signs were myalgia (95.9%), nausea/vomiting (87.7%), positive tourniquet test (77.2%), abdominal pain (42.7%), hepatomegaly (34.6%), and bleeding (20.5%). The ratio of AST and ALTwas 1.8:1. Abnormal AST and ALT were found in 88.2% and 69.3% of the patients, respectively. Patients with nausea/vomiting, petechiae or duration of fever > 7 days more frequently had abnormal transaminases. Abnormal AST during the febrile stage was associated with bleeding. High-level AST and ALT occurred in 11.0% and 7.0%, respectively. Shock was associated with high-level ALT during the febrile stage. Adult dengue patients commonly showed abnormal liver function tests and accounted for at least two-thirds of them. High-level ALT during the febrile stage showed association with shock.

Total midgut volvulus in adults with intestinal malrotation. Report of eleven patients.

PubMed

Kotobi, H; Tan, V; Lefèvre, J; Duramé, F; Audry, G; Parc, Y

2017-06-01

Total small-intestinal volvulus with malrotation (TSIVM) classically presents in the neonatal period; it occurs much less frequently in the adult and is often misdiagnosed. Prognosis is directly related to the degree and duration of intestinal ischemia. Our goal is to describe our experience with TSIVM in the adult, to identify any specific findings and to discuss its management. Eleven patients who had undergone surgery for TSIVM at three centers between 1992 and 2012 were included. Surgery was performed as an emergency for five patients and surgery was elective for six. Mean follow-up was 63 months (range: 12-270). Six patients had had previous abdominal surgery. In nine cases, the diagnosis of TSIVM was made preoperatively, mainly by CT scan in eight cases. Seven patients had associated congenital failure of retroperitoneal fixation of the right colon and all of these underwent a Ladd procedure. The mortality rate was zero. Of the five patients who underwent emergency surgery, three required intestinal resections, one of whom developed a short bowel syndrome. The six patients who underwent surgery electively had no surgical complications. TSIVM is a very unusual finding in adult patients. The diagnosis can be made by CT scan with IV and oral contrast, but it often comes to light only at the time of surgery, even though the patients have often had recurrent episodes of abdominal symptomatology that dated back to childhood. The Ladd procedure, consisting of division of Ladd's bands, widening of the mesentery, and incidental appendectomy, remains the standard surgical repair. Digestive surgeons who care for adults should be familiar with this procedure, and it should be performed, as often as possible, with the assistance of a pediatric surgeon. Copyright © 2016. Published by Elsevier Masson SAS.

Perceptions and Attitudes of Patients About Adult Vaccination and Their Vaccination Status: Still a Long Way to Go?

PubMed

Ozisik, Lale; Calik Basaran, Nursel; Oz, S Gul; Sain Guven, Gulay; Durusu Tanriover, Mine

2017-06-29

BACKGROUND Immunization is one of the most effective public health measures to prevent disease, but vaccination rates in adult populations still remain below the targets. Patient and physician attitudes about vaccination are important for adult vaccination. In this study, we aimed to determine patient attitudes and perceptions about vaccination and the vaccination coverage rates of adult patients in a university hospital in Turkey. MATERIAL AND METHODS A survey was conducted between October 2014 and May 2015 at the Internal Medicine Outpatient Clinics of a university hospital. Adult patients were asked to fill out a questionnaire on their perceptions and attitudes about vaccination and their vaccination status. RESULTS We interviewed 512 patients ages 19-64 years. Eighty percent of the study population thought that adults should be vaccinated, while only 36.1% of the patients stated that vaccination was ever recommended to them in their adult life. Forty-eight percent of the patients stated that they were vaccinated at least once in their adulthood. The most commonly received vaccine was tetanus vaccine in general, while influenza vaccine was the leading vaccine among patients with chronic medical conditions. While 71.4% of the patients to whom vaccination was recommended received the vaccine, 34.9% of the patients received a vaccine without any recommendation. CONCLUSIONS Although the vaccine coverage rates among adults in this survey were low, the perceptions of patients about adult vaccination were mainly positive and of many of them positively reacted when their physician recommended a vaccine.

Improvement with Duloxetine in an Adult ADHD Patient

ERIC Educational Resources Information Center

Tourjman, Smadar Valerie; Bilodeau, Mathieu

2009-01-01

Introduction: Attention-deficit/hyperactivity disorder (ADHD) is a common and disabling disorder among adults and is treated with stimulant and non stimulant medication. Objective: To report the case of a patient with ADHD showing good clinical response to duloxetine, a selective serotonin and norepinephrine reuptake inhibitor (SSNRI). Case…

Ophthalmoscopic findings in adult patients with severe falciparum malaria.

PubMed

Mehta, Salil A; Ansari, Abdul Samad; Jiandani, Prakash

2008-01-01

To report the systemic and ocular findings of four Indian adult patients with severe Plasmodium falciparum infection. Case reports. The commonest findings seen were posterior pole hemorrhages that were seen in all patients. These included superficial and subretinal hemorrhages. Some of the eyes with subretinal hemorrhages were associated with a premacular hemorrhage. The findings of retinal whitening and vascular changes were seen in one patient. A spectrum of ocular lesions was noted, including pre-retinal, subretinal hemorrhages, retinal whitening, and retinal vascular anomalies.

Social Media and the Adolescent and Young Adult (AYA) patient with Cancer

PubMed Central

Perales, Miguel-Angel; Drake, Emily K; Pemmaraju, Naveen; Wood, William A

2016-01-01

Over 70,000 adolescent and young adults aged 15 to 39 years (AYA) are diagnosed with cancer each year in the US. The National Cancer Institute (NCI) has identified AYA cancer patients as a unique population. The most common cancers in this age group include tumors typically seen in pediatric patients such as acute lymphoblastic leukemia (ALL) and brain tumors, as well as cancers more typically seen in adult patients such as breast cancer and melanoma. In addition, some cancers have their highest incidence in AYA patients, such as Hodgkin Lymphoma, testicular cancer and bone tumors. AYA patients face additional unique issues due to their age, not just questions about treatment choices due to lack of data, but also questions about fertility, relationships, loss of autonomy, and interruptions in school/work with potentially significant financial complications. This age group also has very high rates of social media usage with up to 90% of adults aged 18 to 29 using social networking sites. In this review, we will describe the use of social media in AYAs with cancer and highlight some of the online resources for AYAs. PMID:26893061

Social Media and the Adolescent and Young Adult (AYA) Patient with Cancer.

PubMed

Perales, Miguel-Angel; Drake, Emily K; Pemmaraju, Naveen; Wood, William A

2016-12-01

Over 70,000 adolescent and young adults (AYA) aged 15 to 39 years are diagnosed with cancer each year in the US. The National Cancer Institute (NCI) has identified AYA cancer patients as a unique population. The most common cancers in this age group include tumors typically seen in pediatric patients such as acute lymphoblastic leukemia (ALL) and brain tumors, as well as cancers more typically seen in adult patients such as breast cancer and melanoma. In addition, some cancers have their highest incidence in AYA patients, such as Hodgkin Lymphoma, testicular cancer, and bone tumors. AYA patients face additional unique issues due to their age, not just questions about treatment choices due to lack of data but also questions about fertility, relationships, loss of autonomy, and interruptions in school/work with potentially significant financial complications. This age group also has very high rates of social media usage with up to 90 % of adults aged 18 to 29 using social networking sites. In this review, we will describe the use of social media in AYAs with cancer and highlight some of the online resources for AYAs.

Occupational Outcome in Adult ADHD: Impact of Symptom Profile, Comorbid Psychiatric Problems, and Treatment--A Cross-Sectional Study of 414 Clinically Diagnosed Adult ADHD Patients

ERIC Educational Resources Information Center

Halmoy, Anne; Fasmer, Ole Bernt; Gillberg, Christopher; Haavik, Jan

2009-01-01

Objective: To determine the effects of symptom profile, comorbid psychiatric problems, and treatment on occupational outcome in adult ADHD patients. Method: Adult ADHD patients (N = 414) responded to questionnaires rating past and present symptoms of ADHD, comorbid conditions, treatment history, and work status. Results: Of the patients, 24%…

A conservative approach for treating young adult patients with porcelain laminate veneers.

PubMed

Chen, Yen-Wei; Raigrodski, Ariel J

2008-01-01

Controversy persists regarding the treatment planning criteria for young adult patients in need of esthetic restorations. The trend of conservative treatment modalities continues to become widely acknowledged. One of the conservative treatment modalities is porcelain laminate veneers (PLVs). PLVs not only provide suitable esthetics but also reliable functional strength. This article presents two anterior esthetic cases to demonstrate a conservative treatment planning approach and its application as a nontraditional solution for young adult patients. It is recommended that a conservative approach be used wherever possible as an alternative to treatment options that may aggressively sacrifice tooth structure as well as the health of the supporting tissues. By using a conservative approach to treatment with porcelain veneers, long-lasting, esthetic, and functional results may be achieved. Sacrificing as little tooth structure as possible and conserving the supporting tissues will facilitate prospective treatments for young adult patients.

Transition Readiness in Adolescents and Emerging Adults with Diabetes: The Role of Patient-Provider Communication

PubMed Central

Hilliard, Marisa; Sweenie, Rachel; Riekert, Kristin

2013-01-01

Transition from pediatric to adult care represents a high risk period for adolescents and emerging adults with diabetes. Fundamental differences between pediatric and adult care delivery models may contribute to increased risk for poor health outcomes. This review provides a brief overview of models of care in pediatric and adult settings and focuses on patient-provider communication content and quality as potential points of intervention to improve transition-related outcomes. This review also highlights disparities in transition and communication for adolescents and emerging adults from racial/ethnic minority groups and discusses recent changes in health care legislation that have significant implications for the transition process. Intervention opportunities include programs to enhance developmentally-appropriate patient-provider interactions and increased attention to promoting transition readiness skills. Improving patient-provider communication may hasten the development of vital self-advocacy skills needed in adult health care systems and, thus, help establish a lasting pattern of positive diabetes self-care. PMID:24014075

The Patient Protection and Affordable Care Act dependent coverage expansion: Disparities in impact among young adult oncology patients.

PubMed

Alvarez, Elysia M; Keegan, Theresa H; Johnston, Emily E; Haile, Robert; Sanders, Lee; Wise, Paul H; Saynina, Olga; Chamberlain, Lisa J

2018-01-01

Private health insurance is associated with improved outcomes in patients with cancer. However, to the authors' knowledge, little is known regarding the impact of the Patient Protection and Affordable Care Act Dependent Coverage Expansion (ACA-DCE), which extended private insurance to young adults (to age 26 years) beginning in 2010, on the insurance status of young adults with cancer. The current study was a retrospective, population-based analysis of hospitalized young adult oncology patients (aged 22-30 years) in California during 2006 through 2014 (11,062 patients). Multivariable regression analyses examined factors associated with having private insurance. Results were presented as adjusted odds ratios and 95% confidence intervals. A difference-in-difference analysis examined the influence of the ACA-DCE on insurance coverage by race/ethnicity and federal poverty level. Multivariable regression demonstrated that patients of black and Hispanic race/ethnicity were less likely to have private insurance before and after the ACA-DCE, compared with white patients. Younger age (22-25 years) was associated with having private insurance after implementation of the ACA-DCE (odds ratio, 1.20; 95% confidence interval, 1.06-1.35). In the difference-in-difference analysis, private insurance increased among white patients aged 22 to 25 years who were living in medium-income (2006-2009: 64.6% vs 2011-2014: 69.1%; P = .003) and high-income (80.4% vs 82%; P = .043) zip codes and among Asians aged 22 to 25 years living in high-income zip codes (73.2 vs 85.7%; P = .022). Private insurance decreased for all Hispanic patients aged 22 to 25 years between the 2 time periods. The ACA-DCE provision increased insurance coverage, but not among all patients. Private insurance increased for white and Asian patients in higher income neighborhoods, potentially widening social disparities in private insurance coverage among young adults with cancer. Cancer 2018;124:110-7. © 2017 American

[Aetiology of candidiasis in paediatric patients: Comparative analysis with adult patients].

PubMed

Gil-Tomás, Jesús J; Colomina-Rodríguez, Javier

2016-01-01

Candida spp. represents a group of commensal yeasts that can act as pathogens and cause candidiasis in different anatomical locations. The aim of this study was to perform an epidemiological and comparative analysis between the isolates of Candida spp. in clinical specimens during a three year-period (2010-2012) from children (0-14 years) and adults (15-99 years) in the Valencian Community (RedMIVA). The microbiological surveillance network of Valencian Community was used as the information source. Candida was isolated in 52,436 patients (1,604 [3.1%] children and 50,832 [96.9%] adults). Candida albicans was significantly (p<0.05) the predominant species in both age groups, and in almost every type of clinical specimen. The distribution of other species varied depending on the sample type and age group. In blood specimens, Candida parapsilosis followed by C. albicans, Candida famata and Candida lusitaniae were the main species found in children, whereas C. albicans followed by C. parapsilosis, Candida glabrata and Candida tropicalis were the predominant species in adults. In sterile fluids, urine and lower respiratory tract samples, C. parapsilosis was the second most prevalent species in the children group, while C. glabrata and C. tropicalis were the main second species in adults. Copyright © 2015 Asociación Española de Micología. Published by Elsevier Espana. All rights reserved.

Theory of mind deficit in adult patients with congenital heart disease.

PubMed

Chiavarino, Claudia; Bianchino, Claudia; Brach-Prever, Silvia; Riggi, Chiara; Palumbo, Luigi; Bara, Bruno G; Bosco, Francesca M

2015-10-01

This article provides the first assessment of theory of mind, that is, the ability to reason about mental states, in adult patients with congenital heart disease. Patients with congenital heart disease and matched healthy controls were administered classical theory of mind tasks and a semi-structured interview which provides a multidimensional evaluation of theory of mind (Theory of Mind Assessment Scale). The patients with congenital heart disease performed worse than the controls on the Theory of Mind Assessment Scale, whereas they did as well as the control group on the classical theory-of-mind tasks. These findings provide the first evidence that adults with congenital heart disease may display specific impairments in theory of mind. © The Author(s) 2013.

Patient-specific FDG dosimetry for adult males, adult females, and very low birth weight infants

NASA Astrophysics Data System (ADS)

Niven, Erin

Fluorodeoxyglucose is the most commonly used radiopharmaceutical in Positron Emission Tomography, with applications in neurology, cardiology, and oncology. Despite its routine use worldwide, the radiation absorbed dose estimates from FDG have been based primarily on data obtained from two dogs studied in 1977 and 11 adults (most likely males) studied in 1982. In addition, the dose estimates calculated for FDG have been centered on the adult male, with little or no mention of variations in the dose estimates due to sex, age, height, weight, nationality, diet, or pathological condition. Through an extensive investigation into the Medical Internal Radiation Dose schema for calculating absorbed doses, I have developed a simple patient-specific equation; this equation incorporates the parameters necessary for alterations to the mathematical values of the human model to produce an estimate more representative of the individual under consideration. I have used this method to determine the range of absorbed doses to FDG from the collection of a large quantity of biological data obtained in adult males, adult females, and very low birth weight infants. Therefore, a more accurate quantification of the dose to humans from FDG has been completed. My results show that per unit administered activity, the absorbed dose from FDG is higher for infants compared to adults, and the dose for adult women is higher than for adult men. Given an injected activity of approximately 3.7 MBq kg-1, the doses for adult men, adult women, and full-term newborns would be on the order of 5.5, 7.1, and 2.8 mSv, respectively. These absorbed doses are comparable to the doses received from other nuclear medicine procedures.

Prone Positioning Improves Oxygenation in Adult Burn Patients with Severe Acute Respiratory Distress Syndrome

DTIC Science & Technology

2012-01-01

Prone positioning improves oxygenation in adult burn patients with severe acute respiratory distress syndrome Diane F. Hale, MD, Jeremy W. Cannon, MD...Kevin K. Chung, MD, San Antonio, Texas BACKGROUND: Prone positioning (PP) improves oxygenation and may provide a benefit in patients with acute... positioning improves oxygenation in adult burn patients with severe acute respiratory distress syndrome 5a. CONTRACT NUMBER 5b. GRANT NUMBER 5c. PROGRAM

Androgen excess in women: experience with over 1000 consecutive patients.

PubMed

Azziz, R; Sanchez, L A; Knochenhauer, E S; Moran, C; Lazenby, J; Stephens, K C; Taylor, K; Boots, L R

2004-02-01

The objective of the present study was to estimate the prevalence of the different pathological conditions causing clinically evident androgen excess and to document the degree of long-term success of suppressive and/or antiandrogen hormonal therapy in a large consecutive population of patients. All patients presenting for evaluation of symptoms potentially related to androgen excess between October 1987 and June 2002 were evaluated, and the data were maintained prospectively in a computerized database. For the assessment of therapeutic response, a retrospective review of the medical chart was performed, after the exclusion of those patients seeking fertility therapy only, or with inadequate follow-up or poor compliance. A total of 1281 consecutive patients were seen during the study period. Excluded from analysis were 408 patients in whom we were unable to evaluate hormonal status, determine ovulatory status, or find any evidence of androgen excess. In the remaining population of 873 patients, the unbiased prevalence of androgen-secreting neoplasms was 0.2%, 21-hydroxylase-deficient classic adrenal hyperplasia (CAH) was 0.6%, 21-hydroxylase-deficient nonclassic adrenal hyperplasia (NCAH) was 1.6%, hyperandrogenic insulin-resistant acanthosis nigricans (HAIRAN) syndrome was 3.1%, idiopathic hirsutism was 4.7%, and polycystic ovary syndrome (PCOS) was 82.0%. Fifty-nine (6.75%) patients had elevated androgen levels and hirsutism but normal ovulation. A total of 257 patients were included in the assessment of the response to hormonal therapy. The mean duration of follow-up was 33.5 months (range, 6-155). Hirsutism improved in 86%, menstrual dysfunction in 80%, acne in 81%, and hair loss in 33% of patients. The major side effects noted were irregular vaginal bleeding (16.1%), nausea (13.0%), and headaches (12.6%); only 36.6% of patients never complained of side effects. In this large study of consecutive patients presenting with clinically evident androgen excess

Phototherapy with Narrow-Band UVB in Adult Guttate Psoriasis: Results and Patient Assessment.

PubMed

Fernández-Guarino, Montserrat; Aboín-González, Sonsoles; Velázquez, Diana; Barchino, Lucia; Cano, Natividad; Lázaro, Pablo

2016-01-01

Acute guttate psoriasis (AGP) is a distinctive clinical entity with good response to treatment with narrow-band ultraviolet B (NB-UVB). To investigate the results of NB-UVB phototherapy in adult patients with adult guttate psoriasis. We carried out a prospective, open, and observational study. Patients over 18 years with more than 5% of body surface area affected were included. The PASI was assessed prior to and after treatment. The follow-up period was 18 months. After treatment, patients completed a simple questionnaire to assess their overall impression of the treatment. The 67 adult patients with AGP included in this study had an initial PASI of 8.55 (SD 5.03). Patients were treated with a mean of 19.9 sessions (SD 13.5) and mean doses of 14 mJ/cm2 (SD 10.5). Of the 67 patients, 52 achieved PASI90 with 96.15% of PASI reduction, and of these, 46 (88%) maintained PASI90 during the 18 months of follow-up. Patients were very satisfied with the treatment. AGP is a defined clinical entity with a variable course. Phototherapy with NB-UVB appears to be a very good option for treatment of AGP because of the good results obtained and patient satisfaction. © 2016 S. Karger AG, Basel.

Patient-provider relationship as mediator between adult attachment and self-management in primary care patients with multiple chronic conditions.

PubMed

Brenk-Franz, Katja; Strauß, Bernhard; Tiesler, Fabian; Fleischhauer, Christian; Schneider, Nico; Gensichen, Jochen

2017-06-01

The conceptual model of attachment theory has been applied to understand the predispositions of patients in medical care and the patient-provider relationship. In patients with chronic conditions insecure attachment was connected to poorer self-management. The patient-provider relationship is associated with a range of health related outcomes and self-management skills. We determined whether the quality of the patient-provider relationship mediates the link between adult attachment and self-management among primary care patients with multiple chronic diseases. 209 patients with a minimum of three chronic diseases (including type II diabetes, hypertension and at least one other chronic condition) between the ages of 50 and 85 from eight general practices were included in the APRICARE cohort study. Adult attachment was measured via self-report (ECR-RD), self-management skills by the FERUS and the patient-provider relationship by the PRA-D. The health status and chronicity were assessed by the GP. Multiple mediation analyses were used to examine whether aspects of the patient-provider relationship (communication, information, affectivity) are a mediators of associations between adult attachment and self-management. The analysis revealed that the quality of the patient-provider relationship mediated the effect of attachment on self-management in patients with multiple chronic conditions. Particularly the quality of communication and information over the course of treatment has a significant mediating influence. A personalized, attachment-related approach that promotes active patient-provider communication and gives information about the treatment to the patient may improve self-management skills in patients. Copyright © 2017 Elsevier Inc. All rights reserved.

Impairment in anthropometric parameters and body composition in females with classical 21-hydroxylase deficiency.

PubMed

Gonçalves, Ezequiel Moreira; de Lemos-Marini, Sofia Helena Valente; de Mello, Maricilda Palandi; Baptista, Maria Tereza Matias; D'Souza-Li, Lilia Freire Rodrigues; Baldin, Alexandre Duarte; Carvalho, Wellington Roberto Gomes; Farias, Edson Santos; Guerra-Junior, Gil

2009-06-01

The aim of this study was to evaluate the physical measurements and body composition of female patients with the classic form of congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency. Twenty-eight girls with CAH were classified according to both hormonal control (well or not well controlled) and the clinical form of the disease (simple virilizing or salt-wasting). In the control group, 112 healthy individuals were included, divided into two subgroups (male and female). Both patients and controls were subdivided by age into three groups according to pubertal stage: < or =10 years (prepubertal), 11-15 years (pubertal), and > or =15 years (postpubertal). Anthropometrical evaluations and bioelectrical impedance were used to obtain the physical measurement and body composition data. The patients with the simple virilizing form presented higher values for BMI, waist, arm fat area, and fat mass percentage. The not well controlled group presented shorter leg length. Values obtained for BMI as well as for arm fat area, brachial circumference, waist, hip, bi-iliac diameter and fat mass percentage were significantly higher in the patients than in the controls, whereas leg length, hand size and the percentages of water and lean mass were lower. Alterations in body composition were observed in all age groups, mainly by increase of fat mass with age. After puberty, impairments in limb measurements (leg, hand and foot) were more evident. Patients with CAH presented differences in anthropometric parameters but mainly in body composition. Hence, more comprehensive and careful anthropometric evaluation during monitoring of patients with CAH is recommended.

Influence of hormonal control on LH pulsatility and secretion in women with classical congenital adrenal hyperplasia.

PubMed

Bachelot, Anne; Chakhtoura, Zeina; Plu-Bureau, Geneviève; Coudert, Mathieu; Coussieu, Christiane; Badachi, Yasmina; Dulon, Jérome; Charbit, Beny; Touraine, Philippe

2012-10-01

Women with classical congenital adrenal hyperplasia (CAH) exhibit reduced fertility due to several factors including anovulation. This has been attributed to a disturbed gonadotropic axis as in polycystic ovary syndrome (PCOS), but there is no precise evaluation. Our aim was to evaluate the gonadotropic axis and LH pulsatility patterns and to determine factor(s) that could account for the potential abnormality of LH pulsatility. Case/control study. Sixteen CAH women (11 with the salt-wasting form and five with the simple virilizing form), aged from 18 to 40 years, and 16 age-matched women, with regular menstrual cycles (28 ± 3 days), were included. LH pulse patterns over 6 h were determined in patients and controls. No differences were observed between patients and controls in terms of mean LH levels, LH pulse amplitude, or LH frequency. In CAH patients, LH pulsatility patterns were heterogeneous, leading us to perform a clustering analysis of LH data, resulting in a two-cluster partition. Patients in cluster 1 had similar LH pulsatility patterns to the controls. Patients in cluster 2 had: lower LH pulse amplitude and frequency and presented menstrual cycle disturbances more frequently; higher 17-OH progesterone, testosterone, progesterone, and androstenedione levels; and lower FSH levels. LH pulsatility may be normal in CAH women well controlled by hormonal treatment. Undertreatment is responsible for hypogonadotropic hypogonadism, with low LH pulse levels and frequency, but not PCOS. Suppression of progesterone and androgen concentrations during the follicular phase of the menstrual cycle should be a major objective in these patients.

Mental health care Monitor Older adults (MEMO): monitoring patient characteristics and outcome in Dutch mental health services for older adults.

PubMed

Veerbeek, Marjolein; Oude Voshaar, Richard; Depla, Marja; Pot, Anne Margriet

2013-06-01

Information on which older adults attend mental health care and whether they profit from the care they receive is important for policy-makers. To assess this information in daily practice, the "Mental health care Monitor Older adults" (MEMO) was developed in the Netherlands. The aim of this paper is to describe MEMO and the older adults who attend outpatient mental health care regarding their predisposing and enabling characteristics and need for care. In MEMO all patients referred to the division of old age psychiatry of the participating mental health care organisations are assessed at baseline and monitored at 4, 8 and 12-month follow-up. Primary outcomes are mental and social functioning, consumer satisfaction, and type of treatment provided (MEMO Basic). Over the years, MEMO Basic is repeated. In each cycle, additional information on specific patient groups is added (e.g. mood disorders). Data collection is supported by a web-based system for clinicians, including direct feedback to monitor patients throughout treatment. First results at baseline showed that the majority of patients that entered the division of old age psychiatry was female (69%), had low education (83%), lived alone (53%), was depressed (42%) and had a comorbid condition (82%). It seemed that older immigrants were not sufficiently reached. The current study is the first in the Netherlands to evaluate patient characteristics and outcome in mental health care provided for older adults in day-to-day practice. If MEMO works out successfully, the method should be extended to other target groups. Copyright © 2013 John Wiley & Sons, Ltd.

Psychotherapy Interventions for Managing Anxiety and Depressive Symptoms in Adult Brain Tumor Patients: A Scoping Review.

PubMed

Kangas, Maria

2015-01-01

Adult brain tumor (BT) patients and longer-term survivors are susceptible to experiencing emotional problems, including anxiety and/or depression disorders, which may further compromise their quality-of-life (QOL) and general well-being. The objective of this paper is to review psychological approaches for managing anxiety and depressive symptoms in adult BT patients. A review of psychological interventions comprising mixed samples of oncology patients, and which included BT patients is also evaluated. The review concludes with an overview of a recently developed transdiagnostic psychotherapy program, which was specifically designed to treat anxiety and/or depressive symptoms in adult BT patients. Electronic databases (PsycINFO, Medline, Embase, and Cochrane) were searched to identify published studies investigating psychological interventions for managing anxiety and depressive symptoms in adult BT patients. Only four randomized controlled trials (RCTs) were identified. Only one of the RCTs tested a psychosocial intervention, which was specifically developed for primary BT patients, and which was found to improve QOL including existential well-being as well as reducing depressive symptoms. A second study tested a combined cognitive rehabilitation and problem-solving intervention, although was not found to significantly improve mood or QOL. The remaining two studies tested multidisciplinary psychosocial interventions in heterogeneous samples of cancer patients (included BT patients) with advanced stage disease. Maintenance of QOL was found in both studies, although no secondary gains were found for improvements in mood. There is a notable paucity of psychological interventions for adult BT patients across the illness trajectory. Further research is required to strengthen the evidence base for psychological interventions in managing anxiety and depressive symptoms, and enhancing the QOL of distressed adults diagnosed with a BT.

Immunization of HIV-infected adult patients — French recommendations

PubMed Central

Frésard, Anne; Gagneux-Brunon, Amandine; Lucht, Frédéric; Botelho-Nevers, Elisabeth; Launay, Odile

2016-01-01

ABSTRACT Human immunodeficiency virus (HIV)-infected patients remain at increased risk of infection including vaccine-preventable diseases. Vaccines are therefore critical components in the protection of HIV-infected patients from an increasing number of preventable diseases. However, missed opportunities for vaccination among HIV-infected patients persist and vaccine coverage in this population could be improved. This article presents the French recommendations regarding immunization of HIV-infected adults in the light of the evidence-based literature on the benefits and the potential risks of vaccines among this vulnerable population. PMID:27409293

22q11.2 deletion syndrome lowers seizure threshold in adult patients without epilepsy.

PubMed

Wither, Robert G; Borlot, Felippe; MacDonald, Alex; Butcher, Nancy J; Chow, Eva W C; Bassett, Anne S; Andrade, Danielle M

2017-06-01

Previous studies examining seizures in patients with 22q11.2 deletion syndrome (22q11.2DS) have focused primarily on children and adolescents. In this study we investigated the prevalence and characteristics of seizures and epilepsy in an adult 22q11.2DS population. The medical records of 202 adult patients with 22q11.2DS were retrospectively reviewed for documentation of seizures, electroencephalography (EEG) reports, and magnetic resonance imaging (MRI) findings. Epilepsy status was assigned in accordance with 2010 International League Against Epilepsy Classification. Of 202 patients, 32 (15.8%) had a documented history of seizure. Of these 32, 23 (71.8%) had acute symptomatic seizures, usually associated with hypocalcemia and/or antipsychotic or antidepressant use. Nine patients (9/32, 28%; 9/202, 4%) met diagnostic criteria for epilepsy. Two patients had genetic generalized epilepsy; two patients had focal seizures of unknown etiology; two had epilepsy due to malformations of cortical development; in two the epilepsy was due to acquired structural changes; and in one patient the epilepsy could not be further classified. Similarly to children, the prevalence of epilepsy and acute symptomatic seizures in adults with 22q11.2DS is higher than in the general population. Hypocalcemia continues to be a risk factor for adults, but differently from kids, the main cause of seizures in adults with 22q11.2DS is exposure to antipsychotics and antidepressants. Further prospective studies are warranted to investigate how 22q11.2 microdeletion leads to an overall decreased seizure threshold. Wiley Periodicals, Inc. © 2017 International League Against Epilepsy.

Acute encephalopathy with unilateral cortical-subcortical lesions in two unrelated kindreds treated with glucocorticoids prenatally for congenital adrenal hyperplasia due to 21-hydroxylase deficiency: established facts and novel insight.

PubMed

Grunt, Sebastian; Steinlin, Maja; Weisstanner, Christian; Schöning, Martin; Mullis, Primus E; Flück, Christa E

2013-01-01

Prenatal glucocorticoid (GC) treatment of the female fetus with 21-hydroxylase deficiency (21-OHD) may prevent genital virilization and androgen effects on the brain, but prenatal GC therapy is controversial because of possible adverse effects on fetal programming, the cardiovascular system and the brain. We report 2 patients with congenital adrenal hyperplasia (CAH) due to 21-OHD who were treated prenatally with dexamethasone, suffered from an acute encephalopathy and showed focal and multifocal cortical and subcortical diffusion restrictions in early MRI and signs of permanent alterations in the follow-up neuroimaging studies. Both patients recovered from the acute episode. Whereas the first patient recovered without neurological sequelae the second patient showed hemianopsia and spastic hemiplegia in the neurological follow-up examination. These are 2 children with CAH, both treated prenatally with high doses of dexamethasone to prevent virilization. The question arises whether prenatal high-dose GC treatment in patients with CAH might represent a risk factor for brain lesions in later life. Adverse effects/events should be reported systematically in patients undergoing prenatal GC treatment and long-term follow-up studies involving risk factors for cerebrovascular disease should be performed. Copyright © 2013 S. Karger AG, Basel.

Central nervous system involvement in adult patients with invasive infection caused by Streptococcus agalactiae.

PubMed

Oyanguren, B; Esteban, L; Guillán, M; de Felipe, A; Alonso Cánovas, A; Navas, E; Quereda, C; Corral, I

2015-04-01

Streptococcus agalactiae is frequently an asymptomatic coloniser and a cause of neonatal and puerperal sepsis. Infections in nonpregnant adults are uncommon. The frequency of neurological complications caused by invasive infection with this microorganism in adults remains unknown. Here, we study the frequency and characteristics of central nervous system (CNS) involvement in adults with invasive S. agalactiae infection. Review of all adults with invasive S. agalactiae infection between 2003 and 2011 in a tertiary hospital. S. agalactiae was isolated from blood, CSF or synovial fluid in 75 patients. Among them, 7 (9,3%) displayed neurological involvement: 5 men and 2 nonpregnant women, aged between 20 and 62 years. Diagnoses were spinal epidural abscess due to spondylodiscitis with spinal cord compression; acute bacterial meningitis; ischemic stroke as presentation of bacterial endocarditis (2 patients each); and meningoventriculitis after neurosurgery and ventricular shunting. One patient with endocarditis caused by S. agalactiae and S. aureus died in the acute phase, and another died 3 months later from metastatic cancer. The other patients recovered without sequelae. All patients had systemic predisposing factors for infection and 5 (71,4%) had experienced disruption of the mucocutaneous barrier as a possible origin of the infection. CNS involvement is not uncommon in adult patients with invasive infection caused by S. agalactiae. Isolating S. agalactiae, especially in cases of meningitis, should lead doctors to search for predisposing systemic disease and causes of mucocutaneous barrier disruption. Copyright © 2013 Sociedad Española de Neurología. Published by Elsevier Espana. All rights reserved.

Comparison of one-tier and two-tier newborn screening metrics for congenital adrenal hyperplasia.

PubMed

Sarafoglou, Kyriakie; Banks, Kathryn; Gaviglio, Amy; Hietala, Amy; McCann, Mark; Thomas, William

2012-11-01

Newborn screening (NBS) for the classic forms of congenital adrenal hyperplasia (CAH) is mandated in all states in the United States. Compared with other NBS disorders, the false-positive rate (FPR) of CAH screening remains high and has not been significantly improved by adjusting 17α-hydroxyprogesterone cutoff values for birth weight and/or gestational age. Minnesota was the first state to initiate, and only 1 of 4 states currently performing, second-tier steroid profiling for CAH. False-negative rates (FNRs) for CAH are not well known. This is a population-based study of all Minnesota infants (769,834) born 1999-2009, grouped by screening protocol (one-tier with repeat screen, January 1999 to May 2004; two-tier with second-tier steroid profiling, June 2004 to December 2009). FPR, FNR, and positive predictive value (PPV) were calculated per infant, rather than per sample, and compared between protocols. Overall, 15 false-negatives (4 salt-wasting, 11 simple-virilizing) and 45 true-positives were identified from 1999 to 2009. With two-tier screening, FNR was 32%, FPR increased to 0.065%, and PPV decreased to 8%, but these changes were not statistically significant. Second-tier steroid profiling obviated repeat screens of borderline results (355 per year average). In comparing the 2 screening protocols, the FPR of CAH NBS remains high, the PPV remains low, and false-negatives occur more frequently than has been reported. Physicians should be cautioned that a negative NBS does not necessarily rule out classic CAH; therefore, any patient for whom there is clinical concern for CAH should receive immediate diagnostic testing.

Newborn screening for congenital adrenal hyperplasia in Cuba: six years of experience.

PubMed

González, Ernesto Carlos; Carvajal, Frank; Frómeta, Amarilys; Arteaga, Ana Luisa; Castells, Elisa María; Espinosa, Tania; Coto, Remigio; Pérez, Pedro Lucio; Tejeda, Yileidis; Del Río, Lesley; Segura, Mary Triny; Almenares, Pedro; Robaina, René; Fernández, José Luis

2013-06-05

Since 2005, a newborn screening program for congenital adrenal hyperplasia (CAH) by measuring 17-alpha-hydroxyprogesterone (17OHP) in dried blood spots was introduced in Cuba. The hormone was measured by the 17OHP Neonatal UMELISA method, in samples collected on the 5th day as average. Confirmatory test was performed to those neonates with 17OHP values above 55 nmol/l. Some perinatal factors that can influence on 17OHP levels were studied. From January 2005 to December 2010, 621,303 newborns were screened and 39 CAH cases were detected. Coverage of the program reached 98%. The incidence of CAH in Cuba was 1:15,931, similar to that reported by other programs. A recall for suspected CAH was performed in 10,799 cases (1.74%). Therapy in classical CAH patients was started at the mean age of 22 days. 17OHP levels were significantly higher in newborns with lower birth-weight (BW) and/or gestational age (GA). In addition, 17OHP values were affected by the gender, twin status or mode of delivery. In Cuba, the nationwide newborn screening program has allowed the early detection of CAH. The use of an optimized cut-off level for BW or GA could lead to a reduction in the percentage of recalled babies. Copyright © 2013 Elsevier B.V. All rights reserved.

A rare CYP21A2 mutation in a congenital adrenal hyperplasia kindred displaying genotype-phenotype nonconcordance.

PubMed

Khattab, Ahmed; Yuen, Tony; Al-Malki, Sultan; Yau, Mabel; Kazmi, Diya; Sun, Li; Harbison, Madeleine; Haider, Shozeb; Zaidi, Mone; New, Maria I

2016-01-01

Congenital adrenal hyperplasia (CAH) owing to 21-hydroxylase deficiency is caused by the autosomal recessive inheritance of mutations in the gene CYP21A2. CYP21A2 mutations lead to variable impairment of the 21-hydroxylase enzyme, which, in turn, is associated with three clinical phenotypes, namely, salt wasting, simple virilizing, and nonclassical CAH. However, it is known that a given mutation can associate with different clinical phenotypes, resulting in a high rate of genotype-phenotype nonconcordance. We aimed to study the genotype-phenotype nonconcordance in a family with three siblings affected with nonclassical CAH. All had hormonal evidence of nonclassical CAH, but this phenotype could not be explained by the genotype obtained from commercial CYP21A2 genetic testing, which revealed heterozygosity for the maternal 30 kb deletion mutation. We performed Sanger sequencing of the entire CYP21A2 gene in this family to search for a rare mutation that was not covered by commercial testing and found in the three siblings a second, rare c.1097G>A (p.R366H) mutation in exon 8. Computational modeling confirmed that this was a mild mutation consistent with nonclassical CAH. We recommend that sequencing of entire genes for rare mutations should be carried out when genotype-phenotype nonconcordance is observed in patients with autosomal recessive monogenic disorders, including CAH. © 2015 New York Academy of Sciences.

Health conditions and motivations for marijuana use among young adult medical marijuana patients and non-patient marijuana users.

PubMed

Lankenau, Stephen E; Ataiants, Janna; Mohanty, Salini; Schrager, Sheree; Iverson, Ellen; Wong, Carolyn F

2018-02-01

While marijuana has been legal for medical purposes in California since 1996, little is known about the health histories of young adult medical marijuana patients who are a significant proportion of medical marijuana patients. We examined whether young adult medical marijuana patients reported health conditions and motivations for use that were consistent with medical use of marijuana in California. Young adults (N = 366) aged 18 to 26 years were sampled in Los Angeles in 2014-2015 and segmented into medical marijuana 'patients' (n = 210), marijuana users with a current recommendation, and non-patient users or 'non-patients' (n = 156), marijuana users who never had a medical marijuana recommendation. Differences between patients and non-patients regarding self-reported health histories and past/current motivations for marijuana use were expressed as unadjusted risk ratios. Compared with non-patients, patients were significantly more likely to report a range of lifetime health problems, such as psychological, physical pain and gastrointestinal. In the past 90 days, patients were significantly more likely to report motivations for marijuana use than non-patients concerning sleep, anxiety, physical pain and focusing. Psychological and pain problems were the most common health conditions reported to receive a medical marijuana recommendation. Patients were significantly less likely than non-patients to report any privacy concerns about obtaining a medical marijuana recommendation. Patients were significantly more likely to report a range of health conditions and motivations associated with medical use than non-patients. A great majority of patients reported obtaining a medical marijuana recommendation for health problems in accordance with the California law. [Lankenau SE, Ataiants J,Mohanty S, Schrager S, Iverson E, Wong CF.Health conditions and motivations for marijuana use among young adultmedical marijuana patients and non-patient marijuana users. Drug

Cystic fibrosis - Comparison between patients in paediatric and adult age.

PubMed

Santos, V; Cardoso, A V; Lopes, C; Azevedo, P; Gamboa, F; Amorim, A

Cystic fibrosis (CF) is the most common autosomal recessive disease in Caucasians. Although most cases are diagnosed in childhood, diagnosis in adults is apparently increasing. Evaluate the adult population with CF, comparing patients who were diagnosed before and after 18 years of age. Retrospective analysis of patients followed in three main medical centres in Portugal in 2012. Comparison of two groups: G1 - patients diagnosed at <18 years and G2 - patients diagnosed at ≥18 years. 89 adults were identified: 61.8% in G1, 38.2% in G2. Gender distribution was similar in both groups. Average age in G2 was higher (38.3±8.4 vs. 26.8±6.1 years, p<0.001). Respiratory symptoms most frequently led to CF diagnosis in all patients, mainly in adulthood. There was a greater percentage of patients homozygous for the mutation delF508 in G1 (43.6 vs. 8.8%, p=0.02). Respiratory and pancreatic function, and body mass index (BMI) showed a higher severity in G1 (G1 vs. G2: FEV1: 54.6±27.3 vs. 29.9±64.6%, p=0.177; pancreatic insufficiency 72.7 vs. 26.5%, p<0.001; BMI 20.2±3.4 vs. 22.2±4.8, p=0.018). Pseudomonas aeruginosa and methicillin-sensitive Staphylococcus aureus were the most frequently isolated microorganisms. Lung transplantation rate was higher in G2 (20.6 vs. 10.9%, p=0.231) while mortality rate was higher in G1 (0 vs. 3.6%, p=0.261). Hospital admission rate was higher in G1 as well as mortality rate. The results suggest that patients with CF diagnosed in childhood have characteristics that distinguish them from those diagnosed in adulthood, and these differences may have implications for diagnosis, prognosis and life expectancy. Copyright © 2016 Sociedade Portuguesa de Pneumologia. Published by Elsevier España, S.L.U. All rights reserved.

Dry mouth: a critical topic for older adult patients.

PubMed

Han, Phuu; Suarez-Durall, Piedad; Mulligan, Roseann

2015-01-01

Diminished salivary flow, or dry mouth impacts the oral health of many older adults, dentate and edentulous. As a result typical oral conditions can prove more challenging to both the patient's comfort and home care and the treatment selected by the clinician. This paper will review issues of dry mouth from a clinical and symptomatic perspective and will include the condition's causes, treatment and prevention. We performed a review of PubMed using the words: older adults, dry mouth, xerostomia, radiation-induced xerostomia, and salivary gland hypofunction. We selected 90 articles with a clinical application perspective. When it comes to treatment of dry mouth conditions, either objective or subjective, there are no easy answers as to the best course of action for a specific individual. While most of the cited studies have examined the most difficult cases of dry mouth (e.g., Sjögren's syndrome, and that seen during and post head and neck cancer treatments), there are many older adults who demonstrate dry mouth from the use of multiple medications. This paper presents a summary of the etiology, diagnosis, prevention, and pharmacological and non-pharmacological treatment of dry mouth (salivary hypofunction and xerostomia in older adults). It is important to understand the causes of dry mouth and to educate our patients. Starting a prevention program as early as possible considering the most practical, cost effective and efficient treatments with the best risk-benefit ratio will help to diminish dry mouth symptoms and sequelae. Copyright © 2014 Japan Prosthodontic Society. Published by Elsevier Ltd. All rights reserved.

Initial post marketing experience with lacosamide in adult patients with epilepsy.

PubMed

Harden, Cynthia L; Cohn, Aaron; Lowe, Merredith; Serrano, Enrique

2012-02-01

The outcomes of adult epilepsy patients prescribed lacosamide for additional seizure control. Responders were defined as having at least a 50% decrease in seizure frequency Sixty-seven patients were evaluated. Forty-six out of 67 patients (69%) were responders. Twelve of 14 patients not taking sodium channel-acting AEDs were responders (86%) and 34/53 patients taking sodium channel-acting AEDs were responders (64%) (difference not significant). Copyright © 2011 Elsevier B.V. All rights reserved.

Premonitory urges for tics in adult patients with Tourette syndrome.

PubMed

Crossley, Eleanor; Seri, Stefano; Stern, Jeremy S; Robertson, Mary M; Cavanna, Andrea E

2014-01-01

Patients with Tourette syndrome (TS) often report characteristic sensory experiences, also called premonitory urges (PUs), which precede tic expression and have high diagnostic relevance. This study investigated the usefulness of a scale developed and validated in children and adolescents-the Premonitory Urge for Tics Scale (PUTS, Woods et al., 2005 [13])-for the assessment of PUs in adult patients with TS. Standard statistical methods were applied to test the psychometric properties of the PUTS in 102 adult TS outpatients recruited from two specialist clinics in the United Kingdom. The PUTS showed good acceptability and endorsement rates, with evenly distributed scores and low floor and ceiling effects. Item-total correlations were moderate to strong; PUTS total scores were significantly correlated with quantitative measures of TS severity. The PUTS showed excellent internal consistency reliability (Cronbach's alpha=0.85) and Spearman's correlations demonstrated satisfactory convergent and discriminant validity. Although originally devised to assess urges to tic in young patients with TS, the PUTS demonstrated good psychometric properties in a large sample of adults recruited at specialist TS clinics. This instrument is therefore recommended for use across the life span as a valid and reliable self-report measure of sensory experiences accompanying tic expression. Copyright © 2013 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

Dysphagia among adult patients who underwent surgery for esophageal atresia at birth.

PubMed

Huynh Trudeau, Valérie; Maynard, Stéphanie; Terzic, Tatjana; Soucy, Geneviève; Bouin, Mickeal

2015-03-01

Clinical experiences of adults who underwent surgery for esophageal atresia at birth is limited. There is some evidence that suggests considerable long-term morbidity, partly because of dysphagia, which has been reported in up to 85% of adult patients who undergo surgery for esophageal atresia. The authors hypothesized that dysphagia in this population is caused by dysmotility and⁄or anatomical anomalies. To determine the motor and anatomical causes of dysphagia. A total of 41 adults, followed at the Esophageal Atresia Clinic at Hôpital Saint-Luc (Montreal, Quebec), were approached to particpate in the present prospective study. Evaluation was completed using upper endoscopy, manometry and barium swallow for the participants who consented. The medical charts of respondents were systematically reviewed from the neonatal period to 18 years of age to assess medical and surgical history. All 41 patients followed at the clinic consented and were included in the study. Dysphagia was present in 73% of patients. Esophagogastroduodenoscopy was performed in 32 patients: hiatal hernia was present in 62% (n=20); esophageal diverticulum in 13% (n=4); macroscopic Barrett esophagus in 31% (n=10); and esophagitis in 19% (n=6). Histological esophagitis was present in 20% and intestinal metaplasia in 10%. There were no cases of dysplagia or adenocarcinoma. Esophageal manometry was performed on 56% of the patients (n=23). Manometry revealed hypomotility in 100% of patients and included an insufficient number of peristaltic waves in 96%, nonpropagating peristalsis in 78% and low-wave amplitude in 95%. Complete aperistalsis was present in 78%. The lower esophageal sphincter was abnormal in 12 (52%) patients, with incomplete relaxation the most common anomaly. Of the 41 patients, 29 (71%) consented to a barium swallow, which was abnormal in 13 (45%). The anomalies found were short esophageal dilation in 28%, delay in esophageal emptying in 14%, diverticula in 14% and stenosis in 7

Low platelet-poor plasma levels of serotonin in adult autistic patients.

PubMed

Spivak, Baruch; Golubchik, Pavel; Mozes, Tamar; Vered, Yaffa; Nechmad, Allon; Weizman, Abraham; Strous, Rael D

2004-01-01

Hyperserotonemia has been reported in about a third of autistic patients. However, most studies have examined whole blood levels of serotonin (5-HT), the vast majority of which is found in platelets. The aim of this study was to determine 5-HT levels in platelet-poor plasma (PPP) in a group of adult patients with autism. Levels of PPP 5-HT were compared between 10 adult drug-free autistic patients and 12 healthy controls. The Ritvo-Freeman Real-Life Rating Scale and the Overt Aggression Scale (OAS) were administered to the autistic group as a measure of symptom severity. Significantly lower PPP 5-HT levels were observed in the autistic group as compared to the controls (p = 0.03). In addition, PPP 5-HT levels were inversely correlated with OAS scores among subjects with autism (r = -0.64, p < 0.05). PPP 5-HT ('free') levels appear to be low in autistic patients and may play a role in the pathophysiology and symptomatology of the disorder.

Management of Parenteral Nutrition in Hospitalized Adult Patients [Formula: see text].

PubMed

Mundi, Manpreet S; Nystrom, Erin M; Hurley, Daniel L; McMahon, M Molly

2017-05-01

Despite the high prevalence of malnutrition in adult hospitalized patients, surveys continue to report that many clinicians are undertrained in clinical nutrition, making targeted nutrition education for clinicians essential for best patient care. Clinical practice models also continue to evolve, with more disciplines prescribing parenteral nutrition (PN) or managing the cases of patients who are receiving it, further adding to the need for proficiency in general PN skills. This tutorial focuses on the daily management of adult hospitalized patients already receiving PN and reviews the following topics: (1) PN basics, including the determination of energy and volume requirements; (2) PN macronutrient content (protein, dextrose, and intravenous fat emulsion); (3) PN micronutrient content (electrolytes, minerals, vitamins, and trace elements); (4) alteration of PN for special situations, such as obesity, hyperglycemia, hypertriglyceridemia, refeeding, and hepatic/renal disease; (5) daily monitoring and adjustment of PN formula; and (6) PN-related complications (PN-associated liver disease and catheter-related complications).

Neuropsychiatric symptoms are common in immunocompetent adult patients with Toxoplasma gondii acute lymphadenitis.

PubMed

Wong, Weng Kit; Upton, Arlo; Thomas, Mark G

2013-05-01

Chronic toxoplasmosis has been shown to be strongly associated with a range of neuropsychiatric effects including schizophrenia and suicide. However there have not been any prospective, community-based studies of the neuropsychiatric effects of acute toxoplasmosis in adult immunocompetent patients. Adult patients with a positive serum IgM anti-Toxoplasma gondii test result, in the context of an acute illness with lymphadenopathy, were invited to complete a questionnaire seeking information relating to the nature, severity, and duration of symptoms in the months following the diagnosis of acute toxoplasmosis. Laboratory testing identified a total of 187 adults who had a positive serum IgM anti-T. gondii test result between 1 January and 30 November 2011. Consent to contact 108/187 (58%) patients was provided by their family doctor; 37 (34%) of these 108 patients completed and returned the questionnaire. Questionnaires from the 31/108 (29%) patients who reported swollen lymph nodes during their illness were included in the study. Fatigue (90%), headache (74%), difficulty concentrating (52%), and muscle aches (52%) were the most commonly reported symptoms. These symptoms commonly persisted for at least 4 weeks. Twenty-seven of 31 (87%) subjects reported a moderate or severe reduction in their overall physical and mental health during the first 2 months of illness. Acute toxoplasmosis in immunocompetent adults commonly causes moderately severe neuropsychiatric symptoms that might result from replication of the organism in the central nervous system with consequent effects on brain function. Patients should be advised that such symptoms are common and reassured that they usually resolve completely within a few months.

Pharmacokinetic Variability of Mycophenolic Acid in Pediatric and Adult Patients With Hematopoietic Stem Cell Transplantation.

PubMed

Zhang, Daping; Renbarger, Jamie L; Chow, Diana S-L

2016-11-01

The aim of this study was to evaluate the pharmacokinetic variations of mycophenolic acid (MPA), the active metabolite of mycophenolate mofetil (MMF), in both pediatric and adult patients following hematopoietic stem cell transplantation (HSCT). Twenty pediatric patients with a median age of 3 years (range 0.2-12 years) and 13 adult patients with a median age of 54 years (range 18-63 years) were enrolled. Blood samples were collected on days 0, 7, 14, 21, and 30 after allogeneic HSCT. Total and free (unbound) MPA as well as MPA 7-O-glucuronide (MPAG) were quantified using a validated LC-MS/MS assay. The plasma protein binding of MPA and MPAG did not change significantly in pediatric patients over the 1-month sampling period post-HSCT. However, it increased in adult patients from day 7 to day 30 post-HSCT, from 97.3 ± 0.8% to 98.3 ± 0.6% for MPA (P < .05), and 74.6 ± 9.4% to 82.9 ± 8.1% for MPAG (P < .05). The plasma protein binding of MPA was significantly higher in males compared to females in both pediatric (98.3 ± 1.1% vs 97.4 ± 1.1%) and adult (98.1 ± 0.7% vs 97.4 ± 1.2%) patients (P < .05). The MPAG/MPA ratios on a milligram-per-kilogram dose basis in adult patients were significantly higher than those in pediatric patients (4.3 ± 3.4 vs 2.4 ± 2.6; P < .05). Time-dependent plasma protein binding and age-related differences in MPA metabolism at least in part impact the reported large intra- and interindividual variability in MPA pharmacokinetics. These patient and pharmacologic factors, if incorporated into MMF regimen design and modification, may contribute to the rational dose selection of MMF in HSCT patients. © 2016, The American College of Clinical Pharmacology.

Cyclic Vomiting Syndrome in 41 adults: the illness, the patients, and problems of management

PubMed Central

Fleisher, David R; Gornowicz, Blake; Adams, Kathleen; Burch, Richard; Feldman, Edward J

2005-01-01

Background Cyclic Vomiting Syndrome (CVS) is a disorder characterized by recurrent, stereotypic episodes of incapacitating nausea, vomiting and other symptoms, separated by intervals of comparative wellness. This report describes the clinical features, co-morbidities and problems encountered in management of 41 adult patients who met the diagnostic criteria for CVS. Methods This is a retrospective study of adults with CVS seen between 1994 and 2003. Follow-up data were obtained by mailed questionnaires. Results Age of onset ranged from 2 to 49 years. The duration of CVS at the time of consultation ranged from less than 1 year to 49 years. CVS episodes were stereotypic in respect of their hours of onset, symptomatology and length. Ninety-three percent of patients had recognizable prodromes. Half of the patients experienced a constellation of symptoms consisting of CVS episodes, migraine diathesis, inter-episodic dyspeptic nausea and a history of panic attacks. Deterioration in the course of CVS is indicated by coalescence of episodes in time. The prognosis of CVS is favorable in the majority of patients. Conclusion CVS is a disabling disorder affecting adults as well as children. Because its occurrence in adults is little known, patients experience delayed or mis-diagnosis and ineffectual, sometimes inappropriately invasive management. PMID:16368014

Upper extremity open fractures in hospitalized road traffic accident patients: adult versus pediatric cases.

PubMed

Rubin, Guy; Peleg, Kobi; Givon, Adi; Rozen, Nimrod

2017-10-24

Fractures in pediatrics show epidemiological characteristics which are different from fractures in adults. The objective of this study was to examine the injury profiles of open upper extremity fractures (UEFs) in all modes of injury related to road traffic accidents (RTAs) in adult and pediatric hospitalized patients. Data on 103,465 RTA patients between 1997 and 2013 whose records were entered in a centralized country trauma database were reviewed. Data on open UEFs related to mode of injury (car, motorcycle, bicycle, and pedestrian) was compared between adult (18+ years) and pediatric (0-17 years) RTA patients. Of 103,465 RTA cases, 17,263 (16.7%) had UEFs. Of 73,087 adults, 13,237 (18.1%) included UEFs and of 30,378 pediatric cases, 4026 (13.2%) included UEFs (p < 0.0001). Of 17,263 cases with UEFs, we reviewed 22,132 fractures with 2, 743 (12.4%) open fractures. Adults had a greater risk for open fractures (2221, 13%) than the pediatric cases (522, 10.3%) (p < 0.0001). Overall, of a total of 22,132 UEFs, most of the fractures were in the radius (22.8%), humerus (20.3%), clavicle (17.5%), and ulna (15.4%). The adult pedestrian group had a significantly higher risk for open UEFs than the pediatric group (11 vs 8%, p = 0.0012). This study demonstrates the difference between adult and pediatric open fractures in hospitalized RTAs. We showed that adults had a greater risk for open UEFs compared to children, and the adult pedestrian group particularly had a significantly higher risk for open UEFs than the pediatric group.

Trends in the Body Mass Index of Pediatric and Adult Patients Undergoing Anterior Cruciate Ligament Reconstruction

PubMed Central

Burns, Ethan A.; Collins, Alexander D.; Jack, Robert A.; McCulloch, Patrick C.; Lintner, David M.; Harris, Joshua D.

2018-01-01

Background: The body mass index (BMI) in the United States (US) is rising and may be contributing to increased anterior cruciate ligament reconstruction (ACLR) rates. It is currently unknown whether the BMI is increasing in patients who undergo ACLR. Purpose: To determine whether (1) the BMI changed in pediatric and adult patients who previously underwent ACLR or revision ACLR over a 10-year eligibility period, (2) the BMI changed at a greater rate in pediatric or adult patients, and (3) the percentage of overweight and obese patients in the ACLR population was different than that of the general overweight population. Study Design: Case series; Level of evidence, 4. Methods: A retrospective investigation of patients who underwent ACLR by 6 surgeons from June 3, 2005, to June 3, 2015, was conducted. Patients were divided into pediatric (<18 years) and adult (≥18 years) categories. BMI at the time of surgery was defined as underweight (<18.5 kg/m2), normal (18.5-24.9 kg/m2), overweight (25.0-29.9 kg/m2), and obese (≥30.0 kg/m2). Patients with an indeterminate BMI were excluded. Comparisons of overweight and obese patients were made with general population trends determined by the Centers for Disease Control and Prevention (CDC) in a single US state. Pearson (R) and Spearman (R s) correlations were used to determine correlations, Student t tests were used for 2-variable comparisons, analyses of variance were used for multivariable comparisons, and analyses of covariance were used for comparing linear relationships. Results: There were 1305 patients (733 male, 572 female; 409 pediatric, 896 adult) included. Adults requiring surgical revision demonstrated a strong positive correlation with respect to BMI over time (R s = 0.906, P < .01). No other statistically significant trends in the BMI over time were found. The proportion of overweight pediatric patients undergoing ACLR was significantly greater than that of the general overweight pediatric population (P < .05

Analyses of demographical and injury characteristics of adult and pediatric patients injured in Syrian civil war.

PubMed

Er, Erhan; Çorbacıoğlu, Şeref Kerem; Güler, Sertaç; Aslan, Şahin; Seviner, Meltem; Aksel, Gökhan; Bekgöz, Burak

2017-01-01

Aimed to analyze demographical data and injury characteristics of patients who were injured in the Syrian Civil War (SCW) and to define differences in injury characteristics between adult and pediatric patients. Patients who were injured in the SCW and transferred to our emergency department were retrospectively analyzed in this study during the 15-month period between July 2013 and October 2014. During the study period, 1591 patients who were the victims of the SCW and admitted to our emergency department due to war injury enrolled in the study. Of these patients, 285 were children (18%). The median of the injury severity score was 16 (interquartile range [IQR]: 9-25) in all patients. The most frequent mechanism of injury was blunt trauma (899 cases, 55%), and the most frequently-injured region of the body was the head (676 cases, 42.5%). Head injury rates among the children's group were higher than those of the adult group (P < .001). In contrast, injury rates for the abdomen and extremities in the children's group were lower than those in the adult group (P < .001, P < .001). The majority of patients were adults, and the most frequent mechanism of injury was blunt trauma. Similarly, the children were substantially affected by war. Although the injury severity score values and mortality rates of the child and adult groups were similar, it was determined that the number of head injuries was higher, but the number of abdomen and extremity injuries was lower in the children's group than in the adult group. Copyright © 2016 Elsevier Inc. All rights reserved.

Cigarette Smoking, Desire to Quit, and Tobacco-Related Counseling Among Patients at Adult Health Centers

PubMed Central

Lebrun-Harris, Lydie A.; Fiore, Michael C.; Tomoyasu, Naomi; Ngo-Metzger, Quyen

2015-01-01

Objectives. We determined cigarette smoking prevalence, desire to quit, and tobacco-related counseling among a national sample of patients at health centers. Methods. Data came from the 2009 Health Center Patient Survey and the 2009 National Health Interview Survey. The analytic sample included 3949 adult patients at health centers and 27 731 US adults. Results. Thirty-one percent of health center patients were current smokers, compared with 21% of US adults in general. Among currently smoking health center patients, 83% desired to quit and 68% received tobacco counseling. In multivariable models, patients had higher adjusted odds of wanting to quit if they had indications of severe mental illness (adjusted odds ratio [AOR] = 3.26; 95% confidence interval [CI] = 1.19, 8.97) and lower odds if they had health insurance (AOR = 0.43; 95% CI = 0.22, 0.86). Patients had higher odds of receiving counseling if they had 2 or more chronic conditions (AOR = 2.05; 95% CI = 1.11, 3.78) and lower odds if they were Hispanic (AOR = 0.57; 95% CI = 0.34, 0.96). Conclusions. Cigarette smoking prevalence is substantially higher among patients at health centers than US adults in general. However, most smokers at health centers desire to quit. Continued efforts are warranted to reduce tobacco use in this vulnerable group. PMID:24625147

Cigarette Smoking, Desire to Quit, and Tobacco-Related Counseling Among Patients at Adult Health Centers.

PubMed

Lebrun-Harris, Lydie A; Fiore, Michael C; Tomoyasu, Naomi; Ngo-Metzger, Quyen

2015-01-01

Objectives. We determined cigarette smoking prevalence, desire to quit, and tobacco-related counseling among a national sample of patients at health centers. Methods. Data came from the 2009 Health Center Patient Survey and the 2009 National Health Interview Survey. The analytic sample included 3949 adult patients at health centers and 27 731 US adults. Results. Thirty-one percent of health center patients were current smokers, compared with 21% of US adults in general. Among currently smoking health center patients, 83% desired to quit and 68% received tobacco counseling. In multivariable models, patients had higher adjusted odds of wanting to quit if they had indications of severe mental illness (adjusted odds ratio [AOR] = 3.26; 95% confidence interval [CI] = 1.19, 8.97) and lower odds if they had health insurance (AOR = 0.43; 95% CI = 0.22, 0.86). Patients had higher odds of receiving counseling if they had 2 or more chronic conditions (AOR = 2.05; 95% CI = 1.11, 3.78) and lower odds if they were Hispanic (AOR = 0.57; 95% CI = 0.34, 0.96). Conclusions. Cigarette smoking prevalence is substantially higher among patients at health centers than US adults in general. However, most smokers at health centers desire to quit. Continued efforts are warranted to reduce tobacco use in this vulnerable group.

Movement Disorders in Adult Surviving Patients with Maple Syrup Urine Disease

PubMed Central

Carecchio, Miryam; Schneider, Susanne A.; Chan, Heidi; Lachmann, Robin; Lee, Philip J.; Murphy, Elaine; Bhatia, Kailash P.

2014-01-01

Maple syrup urine disease is a rare metabolic disorder caused by mutations in the branched-chain α-keto acid dehydrogenase complex gene. Patients generally present early in life with a toxic encephalopathy because of the accumulation of the branched-chain amino acids leucine, isoleucine, and valine and the corresponding ketoacids. Movement disorders in maple syrup urine disease have typically been described during decompensation episodes or at presentation in the context of a toxic encephalopathy, with complete resolution after appropriate dietary treatment. Movement disorders in patients surviving childhood are not well documented. We assessed 17 adult patients with maple syrup urine disease (mean age, 27.5 years) with a special focus on movement disorders. Twelve (70.6%) had a movement disorder on clinical examination, mainly tremor and dystonia or a combination of both. Parkinsonism and simple motor tics were also observed. Pyramidal signs were present in 11 patients (64.7%), and a spastic-dystonic gait was observed in 6 patients (35.2%). In summary, movement disorders are common in treated adult patients with maple syrup urine disease, and careful neurological examination is advisable to identify those who may benefit from specific therapy. PMID:21484869

Feminizing genitoplasties: Where are we now?

PubMed

Jesus, Lisieux Eyer

2018-04-26

Feminizing genitoplasties (FG) are controversial, because of possible adverse effects on sex life. Some have suggested limiting surgery to children presenting health problems related to their genital abnormality and patients who may give their informed consent. This paper analyzes research data about late results of FG, to substantiate the choice of whether to operate on children or to limit surgery to adults/adolescents. Review and synthesis of the literature about late results of FG. Eleven papers were found, involving different primary diseases and techniques (levels of evidence 3-4). There are no long-term data about corporeal sparing clitoroplasty, ASTRA procedures, and urogenital sinus mobilization. Surgery alters objective genital sensitivity, but most patients attest good subjective post-operative clitoral sensation. Complaints of poor clitoral sensation were related to reoperations, amputation, recession, atrophy, or neurovascular bundle injuries. CAH homozygous (null) genotypes show worse sensitivity and sex life, independent of surgery. Sexual function and avoidance are comparable between post-operated and virgin CAH patients. Problems with global auto-image were related to sexual difficulties. Introitus stenosis is frequent. Patients rarely reported distress concerning FG but vaginal self-dilatation is traumatic. Most patients operated on as young children evaluated timing of their surgery as correct. Biological, technical, and subjective factors influence females' attitudes towards sexual satisfaction. FG patients tend to be sexually insecure. Some sequelae described in adult women should be uncommon in contemporaneous cohorts, because of new techniques. Evidence about long-term sequelae of FG are of low quality and methodologically limited by unphysiological sensitivity measurement methods that do not correspond to subjective reports of the patients. Modern techniques have not been evaluated in the long term. The consequences of intentionally raising

Social, educational and vocational outcomes in patients with childhood-onset and young-adult-onset growth hormone deficiency.

PubMed

Mitra, M Tanya; Jönsson, Peter; Åkerblad, Ann-Charlotte; Clayton, Peter; Kołtowska-Häggström, Maria; Korbonits, Márta; Toogood, Andy; Gleeson, Helena

2017-04-01

Hypopituitarism diagnosed in childhood, adolescence and young adulthood has the potential to affect growth and somatic development. Less is known about the impact of such a diagnosis on other aspects of development. An analysis of the KIMS database (Pfizer International Metabolic Database) was performed to explore social, educational and vocational outcomes of adult patients diagnosed in childhood, adolescence and young adulthood compared with adult-onset controls. A total of 2952 adult patients diagnosed with hypothalamic pituitary conditions before the age of 25 were divided into two groups: childhood-onset [<16 years (CO)] (n = 1782) and young-adult-onset [16 to <25 years (YAO)] (n = 1170). A total of 1617 adult patients diagnosed with a nonfunctioning pituitary adenoma at the age of 25 or older formed the adult-onset control group (AO). KIMS Patient Life Situation Form which provided information on social, educational and vocational outcomes. Compared with the AO control group, CO and YAO patients were between 4·5 and 8·0 times more likely to live with their parents in adulthood; CO and YAO patients were also less likely to live in partnership and to have children. The impact on educational and vocational outcomes was less marked than on social outcomes with no significant differences compared with the AO control group. Educational and vocational outcomes showed the lowest level in male and female CO and YAO patients who had been previously diagnosed with a brain tumour. Social outcomes were more affected than educational and vocational outcomes. Although CO patients are more adversely affected, YAO patients were also failing to achieve social milestones. This has consequences for the delivery of endocrine care in both paediatric and adult services. © 2016 John Wiley & Sons Ltd.

Music Appreciation after Cochlear Implantation in Adult Patients: A Systematic Review.

PubMed

Riley, Phoebe E; Ruhl, Douglas S; Camacho, Macario; Tolisano, Anthony M

2018-06-01

Objective The cochlear implant (CI) improves quality of life for people who are severely and profoundly deafened, allowing implantees to perceive speech at levels similar to those of individuals with normal hearing. However, patients with CIs generally report a reduced appreciation of music after implantation. We aimed to systematically review the English-language literature for studies evaluating music enjoyment and perception among adult patients with CIs. Data Sources A systematic review of PubMed/MEDLINE, Scopus, Embase, and the Cochrane Library. Review Methods The PRISMA statement was utilized to identify English-language studies reporting music appreciation among adults with CIs. Two independent reviewers performed searches through May 2017. Included studies investigated parameters related to music enjoyment and music perception, including (1) pitch and timbre perception, (2) noise-canceling algorithms, and (3) the presence of dissonant chords, lyrics, or visual cues. Results A total of 508 articles were screened for relevance. Forty-one full-text articles were evaluated, and 18 met final inclusion criteria. Studies used heterogeneous methods of outcome measurement for identifying music appreciation. The outcome measures suggest that rhythm and lyrics are important components of enjoyment. Patients with CIs had difficulty with pitch and timbre perception. Conclusion The heterogeneous outcome measures identified in this systematic review suggest that rhythm and lyrics are important components of enjoyment, while patients with CIs had difficulty with pitch and timbre perception. Because there is no standardized reporting metric for music appreciation among adult patients with CIs, a standardized validated outcome-measuring tool is warranted.

Patient Portal Utilization Among Ethnically Diverse Low Income Older Adults: Observational Study

PubMed Central

Quandt, Sara A; Sandberg, Joanne C; Miller Jr, David P; Latulipe, Celine; Leng, Xiaoyan; Talton, Jenifer W; Melius, Kathryn P; Smith, Alden; Bertoni, Alain G

2017-01-01

Background Patient portals can improve patient communication with providers, provide patients with greater health information access, and help improve patient decision making, if they are used. Because research on factors facilitating and limiting patient portal utilization has not been conceptually based, no leverage points have been indicated for improving utilization. Objective The primary objective for this analysis was to use a conceptual framework to determine potentially modifiable factors affecting patient portal utilization by older adults (aged 55 years and older) who receive care at clinics that serve low income and ethnically diverse communities. The secondary objective was to delineate how patient portal utilization is associated with perceived usefulness and usability. Methods Patients from one urban and two rural clinics serving low income patients were recruited and completed interviewer-administered questionnaires on patient portal utilization. Results A total of 200 ethnically diverse patients completed questionnaires, of which 41 (20.5%) patients reported utilizing portals. Education, social support, and frequent Internet utilization improve the odds of patient portal utilization; receiving health care at a rural clinic decreases the odds of portal utilization. Conclusions Leverage points to address disparities in patient portal utilization include providing training for older adults in patient portal utilization, involving spouses or other care partners in this training, and making information technology access available at public places in rural and urban communities. PMID:29138129

Pharmacokinetics and pharmacodynamics of rocuronium in young adult and elderly patients undergoing elective surgery.

PubMed

Varrique, Renan M; Lauretti, Gabriela R; Matsumoto, Julia A; Lanchote, Vera L; de Moraes, Natalia V

2016-11-01

To evaluate the impact of advanced age on rocuronium kinetic disposition in ASA I-III patients undergoing elective surgeries. Young adult (20-50 years, n = 15) and elderly patients (65-85 years, n = 14) submitted to surgery under general anaesthesia were investigated. All patients were induced with individual intravenous doses of midazolam, rocuronium, fentanyl and propofol. Rocuronium-induced neuromuscular block was monitored by train of four stimulations of the adductor muscle of the thumb on the ulnar nerve. The pharmacokinetic parameters were calculated by non-compartmental analysis. The relationship between rocuronium plasma concentration and the neuromuscular blockade was described by a sigmoidal Emax model. Elderly patients presented decreased Cl (2.1 ml/kg per min vs 2.8 ml/kg per min; P = 0.0123); increased AUC/dose (507.8 μg min/ml (mg/kg) vs 392.2 μg min/ml/(mg/kg); P = 0.0168) and reduced volume of distribution (285.4 ml/kg vs 435.6 ml/kg, P = 0.0434) compared to young adults. The concentrations required to achieve 50% of maximum neuromuscular block (EC50) were similar for young adult (338.8 ng/ml) and elderly (462.7 ng/ml) patients (P > 0.05). Elderly patients showed increased AUC/D and reduced total Cl compared to young adult patients due to the age-related reduced renal function. Differences in the PK-PD properties of rocuronium in elderly population are due to changes in drug disposition rather than to alterations in the sensitivity to the drug. © 2016 Royal Pharmaceutical Society.

Reduced Capacity in a Dichotic Memory Test for Adult Patients with ADHD

ERIC Educational Resources Information Center

Dige, Niels; Maahr, Eija; Backenroth-Ohsako, Gunnel

2010-01-01

Objective: To evaluate whether a dichotic memory test would reveal deficits in short-term working-memory recall and long-term memory recall in a group of adult patients with ADHD. Methods: A dichotic memory test with ipsilateral backward speech distraction in an adult ADHD group (n = 69) and a control group (n = 66) is used to compare performance…

Extracorporeal Membrane Oxygenation Characteristics and Outcomes in Adult Patients With Down Syndrome.

PubMed

Duffy, Vicky; Gomez, Daniel; Rycus, Peter; Rivera, Brian; Santoro, Stephanie L; Backes, Carl H; Cua, Clifford L

2018-05-17

Patients with Down syndrome (DS) may have multiple medical issues that place them at risk for requiring extracorporeal membrane oxygenation. Use of extracorporeal membrane oxygenation in pediatric patients with Down syndrome has been described, but minimal data exist for extracorporeal membrane oxygenation use in adults with Down syndrome. The goal of this study was to describe the clinical characteristics and to determine if there were differences between adult extracorporeal membrane oxygenation patients with Down syndrome that were alive (aDS) versus those that died (dDS) prior to hospital discharge. Patients with Down syndrome that were 18 years and older registered in the Extracorporeal Life Support Organization registry from 1983 to 2016 were analyzed. Demographics and extracorporeal membrane oxygenation characteristics were recorded. A total of 21 adults with Down syndrome were identified. Incidence of extracorporeal membrane oxygenation in adults with Down syndrome was 0.88 per 1000 extracorporeal membrane oxygenation procedures. Hospital mortality was 57.1% (12/21). There were no significant differences between aDS versus dDS for age (24.9 ± 4.8 vs. 28.1 ± 10.2 years), weight (90.7 ± 13.0 vs. 79.1 ± 27.0 kg), gender (4 males vs. 8 males), initial pH (7.18 ± 0.19 vs. 7.27 ± 0.16), or initial pO2 (51.7 ± 13.9 vs. 45.4 ± 19.9), respectively. There were no significant differences between aDS versus dDS in duration of extracorporeal membrane oxygenation run (239 ± 159 h vs. 455 ± 570 h, respectively), ventilator or extracorporeal membrane oxygenation mode, and nitric oxide use. aDS had fewer incidences of mechanical and neurologic complications (41.7% vs. 0.0%, P < 0.05) versus dDS. There were no other significant differences in complication rates between the two groups. Use of extracorporeal membrane oxygenation in the adult population with Down syndrome is significantly less compared to the pediatric

Risk of seizure relapse after antiepileptic drug withdrawal in adult patients with focal epilepsy.

PubMed

He, Ru-Qian; Zeng, Qing-Yi; Zhu, Pan; Bao, Yi-Xin; Zheng, Rong-Yuan; Xu, Hui-Qin

2016-11-01

The objective of this study was to estimate the risk of a seizure relapse and the high-risk period of recurrence after antiepileptic drug (AED) withdrawal and to determine the predictive factors for a seizure relapse in adult patients with focal epilepsy who were seizure-free for more than 2years. Using the Wenzhou Epilepsy Follow-Up Registry Database, 200 adult patients with focal epilepsy were recruited, who were undergoing follow-up, met the inclusion criteria of this study, were seizure-free for more than 2years, began withdrawing between June 2003 and June 2014, and were followed up prospectively for at least 1year or until a seizure relapse. The risk of recurrence and the time to seizure relapse were analyzed by the Kaplan-Meier method, and the predictive factors were identified by the Cox proportional hazard regression model. A total of 99 patients had an unprovoked relapse during the follow-up period. The relapse rate was 49.5%, and each year, the recurrence probability of 12, 24, 36, 48, 60, 72, and 84months after AED withdrawal was 24.0%, 20.4%, 8.3%, 2.7%, 4.6%, 0.97%, and 0.98%, respectively. The two independent risk factors for recurrence after withdrawal in adult patients with focal epilepsy were a longer duration of active epilepsy and a shorter seizure-free period before withdrawal. The high-risk period of a seizure relapse in adult patients with focal epilepsy is the first 2years after withdrawal, and beyond 5years after withdrawal, seizures rarely relapse (relapse rate<1%). A seizure-free period for less than 4years before withdrawal is a predictive factor of risk for seizure recurrence after AED withdrawal in adult patients with focal epilepsy. Copyright © 2016 Elsevier Inc. All rights reserved.

The effectiveness of patient navigation programs for adult cancer patients undergoing treatment: a systematic review.

PubMed

Tho, Poh Chi; Ang, Emily

2016-02-01

Advancements in technology and medical treatment have made cancer care treatment more complex. With the current trend of sub-specialization in health care, cancer patients commonly receive care from multiple specialists and have wider treatment options. In view of this, there is a need to coordinate care and integrate information to enhance care and quality of outcomes for patients. Since the successful implementation of programs for increasing the survival rate of breast cancer patients at Harlem Hospital Center, New York, USA, patient navigation programs have been widely introduced in healthcare settings. Some literature has identified nurses as a primary candidate in assuming the role of a navigator. However, there is a need to further explore the effectiveness of patient navigation programs for their effectiveness in improving quality of life, and patient satisfaction and outcomes during the commencement of cancer treatment. The objective of this review was to synthesize the best available evidence on the effectiveness of patient navigation programs in adult cancer patients undergoing treatments such as radiotherapy and/or chemotherapy. This review considered studies that included adults aged 18 years and over, diagnosed with any type of cancer and undergoing treatment in an acute care hospital setting, including inpatient and outpatient/ambulatory care.This review considered studies that evaluated nurse-led patient navigation programs versus no patient navigation program or non-structured care coordination.A patient navigation program includes patient education, psychosocial support, and care coordination.This review considered randomized controlled trials and quasi-experimental studies.The review focused on the effects of patient navigator program clinical/patient outcomes. The review included studies on patient wellbeing and clinical outcomes, but excluded studies that had examined the impact of these programs on efficiency-related outcomes, such as length

Outdoor recreational fires: a review of 329 adult and pediatric patients.

PubMed

Neaman, Keith C; Do, Viet H; Olenzek, Emily K; Baca, Marissa; Ford, Ronald D; Wilcox, Richard M

2010-01-01

Outdoor recreational fires are a frequent occurrence during the summer months and can be associated with burns resulting in significant morbidity. Both pediatric and adult populations can be affected, and their mechanism of injury is often different. Understanding these mechanisms is important when designing prevention programs. It is the goal of this study to review our experience with outdoor recreational fires. All patients who presented to Spectrum Health Blodgett Regional Burn Unit for burns secondary to an outdoor recreational fire over an 8-year period were reviewed. Demographic data, mechanism of injury, body area involved, TBSA burned, treatments undertaken, and subsequent complications were recorded. Pediatric patients (aged 16 years and younger) were analyzed independently, and risk factors were determined. A total of 329 patients suffered burns secondary to outdoor recreational fires over the length of the study. More than 35% required inpatient treatment, with an average length of stay of 4.8 days. Hands were the most frequently affected body part, with the mean TBSA involved being 3.5%. Ninety-four patients (28.6%) required split-thickness skin grafting. The most common mechanism of injury in both adult and pediatric populations was falling into an ongoing fire. Wound infection was the most common complication. Alcohol intoxication was associated with a higher burn severity and complication rate. Pediatric patients represented 39.8% of the sample. Burns secondary to outdoor recreational fires are associated with significant morbidity. Adult prevention programs should target awareness with respect to alcohol consumption and campfires secondary to the morbidity associated with these injuries. Pediatric patients are particularly susceptible, and parents should remain diligent about campfire safety and be educated about the inherent dangers of both active and extinguished fires.

Impact of Pseudomonas aeruginosa Infection on Respiratory Muscle Function in Adult Cystic Fibrosis Patients.

PubMed

Magnet, Friederike Sophie; Callegari, Jens; Dieninghoff, Doris; Spielmanns, Marc; Storre, Jan Hendrik; Schmoor, Claudia; Windisch, Wolfram

2017-01-01

Pseudomonas aeruginosa infection impairs respiratory muscle function in adolescents with cystic fibrosis, but its impact on adult patients has not been characterised. To investigate respiratory muscle function in adult cystic fibrosis patients according to P. aeruginosa status (repetitive samples over 12 months). The pressure-time index of the respiratory muscles (PTImus), a measure of their efficiency, served as the primary outcome. In addition, respiratory load and maximal respiratory muscle strength were assessed. In 51 patients examined (65% female; median age 32 years, IQR 24-40), a median of 3.0 (IQR 2-4) different pathogens was found in each patient. The PTImus was 0.113 and 0.126 in Pseudomonas-positive (n = 33) and -negative (n = 18) patients, respectively (p = 0.53). Univariate analysis showed a lower PTImus in male than in female patients (p = 0.006). Respiratory muscle load and strength were otherwise comparable, with the exception of higher nasal sniff pressures in Pseudomonas-positive patients who were chronically infected (>50% of positive samples). Quality of Life (according to the Cystic Fibrosis Questionnaire-Revised) was higher if both respiratory load and the PTImus were low (high respiratory muscle efficiency). Chronic P. aeruginosa infection does not influence respiratory muscle efficiency in adult cystic fibrosis patients with otherwise multiple co-infections. In addition, patients with reduced respiratory muscle efficiency had worse Quality of Life. © 2016 S. Karger AG, Basel.

Dysphagia among adult patients who underwent surgery for esophageal atresia at birth

PubMed Central

Huynh-Trudeau, Valérie; Maynard, Stéphanie; Terzic, Tatjana; Soucy, Geneviève; Bouin, Mickael

2015-01-01

BACKGROUND: Clinical experiences of adults who underwent surgery for esophageal atresia at birth is limited. There is some evidence that suggests considerable long-term morbidity, partly because of dysphagia, which has been reported in up to 85% of adult patients who undergo surgery for esophageal atresia. The authors hypothesized that dysphagia in this population is caused by dysmotility and/or anatomical anomalies. OBJECTIVE: To determine the motor and anatomical causes of dysphagia. METHODS: A total of 41 adults, followed at the Esophageal Atresia Clinic at Hôpital Saint-Luc (Montreal, Quebec), were approached to particpate in the present prospective study. Evaluation was completed using upper endoscopy, manometry and barium swallow for the participants who consented. The medical charts of respondents were systematically reviewed from the neonatal period to 18 years of age to assess medical and surgical history. RESULTS: All 41 patients followed at the clinic consented and were included in the study. Dysphagia was present in 73% of patients. Esophagogastroduodenoscopy was performed in 32 patients: hiatal hernia was present in 62% (n=20); esophageal diverticulum in 13% (n=4); macroscopic Barrett esophagus in 31% (n=10); and esophagitis in 19% (n=6). Histological esophagitis was present in 20% and intestinal metaplasia in 10%. There were no cases of dysplagia or adenocarcinoma. Esophageal manometry was performed on 56% of the patients (n=23). Manometry revealed hypomotility in 100% of patients and included an insufficient number of peristaltic waves in 96%, non-propagating peristalsis in 78% and low-wave amplitude in 95%. Complete aperistalsis was present in 78%. The lower esophageal sphincter was abnormal in 12 (52%) patients, with incomplete relaxation the most common anomaly. Of the 41 patients, 29 (71%) consented to a barium swallow, which was abnormal in 13 (45%). The anomalies found were short esophageal dilation in 28%, delay in esophageal emptying in 14

Patient-oncologist alliance as protection against suicidal ideation in young adults with advanced cancer.

PubMed

Trevino, Kelly M; Abbott, Caroline H; Fisch, Michael J; Friedlander, Robert J; Duberstein, Paul R; Prigerson, Holly G

2014-08-01

Young adults with cancer are at an increased risk of suicidal ideation. To the authors' knowledge, the impact of the patient-oncologist alliance on suicidal ideation has not been examined to date. The current study examined the relationship between the patient-oncologist therapeutic alliance and suicidal ideation in young adults with advanced cancer. A total of 93 young adult patients (aged 20 years-40 years) with incurable, recurrent, or metastatic cancer were evaluated by trained interviewers. Suicidal ideation was assessed with the Yale Evaluation of Suicidality scale, dichotomized into a positive and negative score. Predictors included diagnoses of major depressive disorder and posttraumatic stress disorder, physical quality of life, social support, and use of mental health and supportive care services. The Human Connection Scale, dichotomized into a strong (upper third) and weak (lower two-thirds) therapeutic alliance, assessed the strength of the patients' perceived oncologist alliance. Approximately 22.6% of patients screened positive for suicidal ideation. Patients with a strong therapeutic alliance were found to be at reduced risk of suicidal ideation after controlling for confounding influences of cancer diagnosis, Karnofsky performance status, number of physical symptoms, physical quality of life, major depressive disorder, posttraumatic stress disorder, and social support. A strong therapeutic alliance was also associated with a reduced risk of suicidal ideation after controlling for mental health discussions with health care providers and use of mental health interventions. The patient-oncologist alliance was found to be a robust predictor of suicidal ideation and provided better protection against suicidal ideation than mental health interventions, including psychotropic medications. Oncologists may significantly influence patients' mental health and may benefit from training and guidance in building strong alliances with their young adult patients. �

Features and prevalence of patients with probable adult attention deficit hyperactivity disorder who request treatment for cocaine use disorders.

PubMed

Pérez de Los Cobos, José; Siñol, Núria; Puerta, Carmen; Cantillano, Vanessa; López Zurita, Cristina; Trujols, Joan

2011-01-30

To characterize those patients with probable adult attention deficit hyperactivity disorder (ADHD) who ask for treatment of cocaine use disorders; to estimate the prevalence of probable adult ADHD among these patients. This is a cross-sectional and multi-center study performed at outpatient resources of 12 addiction treatment centers in Spain. Participants were treatment-seeking primary cocaine abusers recruited consecutively at one center and through convenience sampling at the other centers. Assessments included semi-structured clinical interview focused on Diagnostic and Statistical Manual of Mental Disorders, fourth edition (DSM-IV) ADHD criteria adapted to adulthood, and the Wender-Utah Rating Scale (WURS) for screening childhood history of ADHD according to patients. Probable adult ADHD was diagnosed when patients met DSM-IV criteria of ADHD in adulthood and scored WURS>32. All participants were diagnosed with current cocaine dependence (n=190) or abuse (n=15). Patients with probable adult ADHD, compared with patients having no lifetime ADHD, were more frequently male, reported higher impulsivity, and began to use nicotine, alcohol, cannabis, or cocaine earlier. Before starting the current treatment, patients with probable adult ADHD also showed higher cocaine craving for the previous day, less frequent cocaine abstinence throughout the previous week, and higher use of cocaine and tobacco during the previous month. Impulsivity and male gender were the only independent risk factors of probable adult ADHD in a logistic regression analysis. The prevalence of probable adult ADHD was 20.5% in the sub-sample of patients consecutively recruited (n=78). A diagnosis of probable adult ADHD strongly distinguishes among treatment-seeking cocaine primary abusers regarding past and current key aspects of their addictive disorder; one-fifth of these patients present with probable adult ADHD. Copyright © 2009 Elsevier Ireland Ltd. All rights reserved.

Improving Pediatric Preparedness in Critical Access Hospital Emergency Departments: Impact of a Longitudinal In Situ Simulation Program.

PubMed

Katznelson, Jessica H; Wang, Jiangxia; Stevens, Martha W; Mills, William A

2018-01-01

Critical access hospitals (CAH) see few pediatric patients. Many of these hospitals do not have access to physicians with pediatric training. We sought to evaluate the impact of an in situ pediatric simulation program in the CAH emergency department setting on care team performance during resuscitation scenarios. Five CAHs conducted 6 high-fidelity pediatric simulations over a 12-month period. Team performance was evaluated using a validated 35-item checklist representing commonly expected resuscitation team interventions. Checklists were scored by assigning zero point for "yes" and 1 point for "no". A lower final score meant more items on the list had been completed. The Kruskal-Wallis rank test was used to assess for differences in average scores among institutions. A linear mixed effects model with a random institution intercept was used to examine trends in average scores over time. P < 0.05 was considered significant. The Kruskal-Wallis rank test showed no difference in average scores among institutions. (P = 0.90). Checklist scores showed a significant downward trend over time, with a scenario-to-scenario decrease of 0.022 (P < 0.01). One hundred percent of providers surveyed in the last month stated they would benefit from ongoing scenarios. Regularly scheduled pediatric simulations in the CAH emergency department setting improved team performance over time on expected resuscitation tasks. The program was accepted by providers. Implementation of simulation-based training programs can help address concerns regarding pediatric preparedness in the CAH setting. A future project will look at the impact of the program on patient care and safety.

Musical ear syndrome in adult cochlear implant patients.

PubMed

Low, W-K; Tham, C A; D'Souza, V-D; Teng, S-W

2013-09-01

Except for a single case report, musical ear syndrome in cochlear implantees has not been studied. We aimed to study the prevalence and nature of musical ear syndrome among adult cochlear implant patients, as well as the effect on their emotional well-being. STUDY DESIGN, PATIENTS AND INTERVENTION: A cross-sectional survey of patients aged 18 years and above who had received cochlear implants for profound hearing loss between 1997 and 2010. Of the 82 patients studied, 18 (22 per cent) were found to have experienced musical ear syndrome. Seven and 11 patients had musical ear syndrome prior to and after cochlear implantation, respectively. The character of musical ear syndrome symptoms was described as instrumental music (n = 2), singing (6) or both (10). Fourteen patients reported an adverse emotional effect, with three expressing ‘intolerance’. In this study, 22 per cent of cochlear implantees experienced musical ear syndrome. These symptoms affected patients’ emotional state, but most coped well. Musical ear syndrome can occur prior to and after cochlear implantation.

Meta-Analysis of Suicide-Related Behavior or Ideation in Child, Adolescent, and Adult Patients Treated with Atomoxetine

PubMed Central

Wietecha, Linda A.; Wang, Shufang; Buchanan, Andrew S.; Kelsey, Douglas K.

2014-01-01

Abstract Objective: This meta-analysis examined suicide-related events in the acute phases of double-blind, placebo-controlled atomoxetine trials in pediatric and adult patients with attention-deficit/hyperactivity disorder (ADHD). Methods: A total of 3883 pediatric and 3365 adult patients were included. Potential events were identified from the adverse events database using a text-string search. Mantel–Haenszel risk ratios (MHRR) were calculated for potential suicide-related events categorized according to United States Food and Drug Administration defined codes. Results: In this data set, no completed suicides were reported in the pediatric or adult populations. One pediatric (attempted suicide) (and no adult patient events) was categorized as suicidal behavior in the atomoxetine group. The frequency of combined suicidal behavior or ideation with atomoxetine treatment was 0.37% in pediatric patients (vs. 0.07% with placebo) and 0.11% in adults (vs. 0.12% with placebo) and the risk compared with placebo was not statistically significant (MHRR=1.57; p=0.42 and MHRR=0.96; p=0.96, respectively). In pediatric patients, suicidal ideation only was reported more frequently compared with placebo (MHRR=1.63; p=0.41). Conclusions: Overall in this data set, no completed suicides and 1 pediatric patient suicidal behavior event were reported in atomoxetine-treated pediatric and adult patients. Suicidal ideation was uncommon among atomoxetine-treated pediatric and adult patients, although it was reported more frequently in atomoxetine-treated pediatric patients compared with placebo; the reporting rate difference was not statistically significant. The MHRR of suicidal ideation was consistent with a previous meta-analysis of similar design. There was no evidence of increased risk for suicidal behavior in atomoxetine-treated pediatric or adult patients. Clinical trial registration information: http://www.clinicaltrials.gov. The data reported are from an analysis of 23 pediatric

Adult Co-morbidity Evaluation 27 scores of head and neck cancer patients using touch-screen technology: patient satisfaction and clinical verification.

PubMed

Brammer, C; Dawson, D; Joseph, M; Tipper, J; Jemmet, T; Liew, L; Spinou, C; Grew, N; Pigadas, N; Rehman, K

2017-05-01

This study aimed to assess head and neck cancer patient satisfaction with the use of a touch-screen computer patient-completed questionnaire for assessing Adult Co-morbidity Evaluation 27 co-morbidity scores prior to treatment, along with its clinical reliability. A total of 96 head and neck cancer patients were included in the audit. An accurate Adult Co-morbidity Evaluation 27 co-morbidity score was achieved via patient-completed questionnaire assessment for 97 per cent of participants. In all, 96 per cent of patients found the use of a touch-screen computer acceptable and would be willing to use one again, and 62 per cent would be willing to do so without help. Patients were more likely to be willing to use the computer again without help if they were aged 65 years or younger (χ2 test; p = 0.0054) or had a performance status of 0 or 1 (χ2 test; p = 0.00034). Use of a touch-screen computer is an acceptable approach for assessing Adult Co-morbidity Evaluation 27 scores at pre-treatment assessment in a multidisciplinary joint surgical-oncology clinic.

Endoscopic third ventriculostomy for treatment of adult hydrocephalus: long-term follow-up of 163 patients.

PubMed

Isaacs, Albert M; Bezchlibnyk, Yarema B; Yong, Heather; Koshy, Dilip; Urbaneja, Geberth; Hader, Walter J; Hamilton, Mark G

2016-09-01

OBJECTIVE The efficacy of endoscopic third ventriculostomy (ETV) for the treatment of pediatric hydrocephalus has been extensively reported in the literature. However, ETV-related long-term outcome data are lacking for the adult hydrocephalus population. The objective of the present study was to assess the role of ETV as a primary or secondary treatment for hydrocephalus in adults. METHODS The authors performed a retrospective chart review of all adult patients (age ≥ 18 years) with symptomatic hydrocephalus treated with ETV in Calgary, Canada, over a span of 20 years (1994-2014). Patients were dichotomized into a primary or secondary ETV cohort based on whether ETV was the initial treatment modality for the hydrocephalus or if other CSF diversion procedures had been previously attempted respectively. Primary outcomes were subjective patient-reported clinical improvement within 12 weeks of surgery and the need for any CSF diversion procedures after the initial ETV during the span of the study. Categorical and actuarial data analysis was done to compare the outcomes of the primary versus secondary ETV cohorts. RESULTS A total of 163 adult patients with symptomatic hydrocephalus treated with ETV were identified and followed over an average of 98.6 months (range 0.1-230.4 months). All patients presented with signs of intracranial hypertension or other neurological symptoms. The primary ETV group consisted of 112 patients, and the secondary ETV consisted of 51 patients who presented with failed ventriculoperitoneal (VP) shunts. After the initial ETV procedure, clinical improvement was reported more frequently by patients in the primary cohort (87%) relative to those in the secondary ETV cohort (65%, p = 0.001). Additionally, patients in the primary ETV group required fewer reoperations (p < 0.001), with cumulative ETV survival time favoring this primary ETV cohort over the course of the follow-up period (p < 0.001). Fifteen patients required repeat ETV, with all but

Movement disorders in adult surviving patients with maple syrup urine disease.

PubMed

Carecchio, Miryam; Schneider, Susanne A; Chan, Heidi; Lachmann, Robin; Lee, Philip J; Murphy, Elaine; Bhatia, Kailash P

2011-06-01

Maple syrup urine disease is a rare metabolic disorder caused by mutations in the branched-chain α-keto acid dehydrogenase complex gene. Patients generally present early in life with a toxic encephalopathy because of the accumulation of the branched-chain amino acids leucine, isoleucine, and valine and the corresponding ketoacids. Movement disorders in maple syrup urine disease have typically been described during decompensation episodes or at presentation in the context of a toxic encephalopathy, with complete resolution after appropriate dietary treatment. Movement disorders in patients surviving childhood are not well documented. We assessed 17 adult patients with maple syrup urine disease (mean age, 27.5 years) with a special focus on movement disorders. Twelve (70.6%) had a movement disorder on clinical examination, mainly tremor and dystonia or a combination of both. Parkinsonism and simple motor tics were also observed. Pyramidal signs were present in 11 patients (64.7%), and a spastic-dystonic gait was observed in 6 patients (35.2%). In summary, movement disorders are common in treated adult patients with maple syrup urine disease, and careful neurological examination is advisable to identify those who may benefit from specific therapy. © 2011 Movement Disorder Society. Copyright © 2011 Movement Disorder Society.

Physical activity during hospitalization: Activities and preferences of adults versus older adults.

PubMed

Meesters, Jorit; Conijn, D; Vermeulen, H M; Vliet Vlieland, Tpm

2018-04-16

Inactivity during hospitalization leads to a functional decline and an increased risk of complications. To date, studies focused on older adults. This study aims to compare the physical activities performed by older adult and adult hospitalized patients. Patients hospitalized for >3 days at a university hospital completed a questionnaire regarding their physical activities (% of days on which an activity was performed divided by the length of stay) and physical activity needs during hospitalization. Crude and adjusted comparisons of older adult (>60 years) and adult (≤60 years) patients were performed using parametric testing and regression analyses. Of 524 patients, 336 (64%) completed the questionnaire, including 166 (49%) older adult patients. On average, the patients were physically active on 35% or less of the days during their hospitalization. Linear regression analysis showed no significant associations between being an older adult and performing physical activities after adjusting for gender, length of stay, surgical intervention, and meeting physical activity recommendations prior to hospitalization. Most patients were well informed regarding physical activity during hospitalization; however, the older adult patients reported a need for information regarding physical activities after hospitalization more frequently (odds ratios, 2.47) after adjusting for educational level, gender, and physical therapy during hospitalization. Both older adult and adult patients are physically inactive during hospitalization, and older adult patients express a greater need for additional information regarding physical activity after hospitalization than adult patients. Therefore, personalized strategies that inform and motivate patients to resume physical activities during hospitalization are needed regardless of age.

Age-Specific Patient Navigation Preferences Among Adolescents and Young Adults with Cancer.

PubMed

Pannier, Samantha T; Warner, Echo L; Fowler, Brynn; Fair, Douglas; Salmon, Sara K; Kirchhoff, Anne C

2017-11-23

Patient navigation is increasingly being directed at adolescent and young adult (AYA) patients. This study provides a novel description of differences in AYA cancer patients' preferences for navigation services by developmental age at diagnosis. Eligible patients were diagnosed with cancer between ages 15 and 39 and had completed at least 1 month of treatment. Between October 2015 and January 2016, patients completed semi-structured interviews about navigation preferences. Summary statistics of demographic and cancer characteristics were generated. Differences in patient navigation preferences were examined through qualitative analyses by developmental age at diagnosis. AYAs were interviewed (adolescents 15-18 years N = 8; emerging adults 19-25 years N = 8; young adults 26-39 years N = 23). On average, participants were 4.5 years from diagnosis. All age groups were interested in face-to-face connection with a navigator and using multiple communication platforms (phone, text, email) to follow-up. Three of the most frequently cited needs were insurance, finances, and information. AYAs differed in support, healthcare, and resource preferences by developmental age; only adolescents preferred educational support. While all groups preferred financial and family support, the specific type of assistance (medical versus living expenses, partner/spouse, child, or parental assistance) varied by age group. AYAs with cancer have different preferences for patient navigation by developmental age at diagnosis. AYAs are not a one-size-fits-all population, and navigation programs can better assist AYAs when services are targeted to appropriate developmental ages. Future research should examine fertility and navigation preferences by time since diagnosis. While some navigation needs to span the AYA age range, other needs are specific to developmental age.

Memory for the September 11, 2001, terrorist attacks one year later in patients with Alzheimer's disease, patients with mild cognitive impairment, and healthy older adults.

PubMed

Budson, Andrew E; Simons, Jon S; Waring, Jill D; Sullivan, Alison L; Hussoin, Trisha; Schacter, Daniel L

2007-10-01

Although there are many opportunities to study memory in patients with Alzheimer's disease (AD) in the laboratory, there are few opportunities to study memory for real world events in these patients. The September 11, 2001 terrorist attacks provided one such opportunity. Patients with AD, patients with mild cognitive impairment (MCI), and healthy older adults were given a telephone questionnaire in the initial weeks after the event, again three to four months later, and finally one year afterwards to evaluate their memory for the September 11, 2001 terrorist attacks. We were particularly interested in using the attacks as an opportunity to examine the decline of episodic memory in patients with AD, patients with MCI, and older adult controls over a period of months. We found that compared to healthy older adults, patients with AD and MCI showed impaired memory at the initial time point, more rapid forgetting from the initial to the three-month time point, and very similar changes in memory from the three-month to the one-year time point. We speculated that these findings were consistent with patients with AD and MCI showing initial impaired encoding and a more rapid rate of forgetting compared with healthy older adults, but that once the memories had been consolidated, their decay rate became similar to that of healthy older adults. Lastly, although memory distortions were common among all groups, they were greatest in the patients with AD.

Spontaneous gram-negative bacillary meningitis in adult patients: characteristics and outcome.

PubMed

Pomar, Virginia; Benito, Natividad; López-Contreras, Joaquin; Coll, Pere; Gurguí, Mercedes; Domingo, Pere

2013-09-30

Spontaneous meningitis caused by gram-negative bacilli in adult patients is uncommon and poorly characterized. Our objective is to describe and compare the characteristics and the outcome of adult patients with spontaneous gram-negative bacilli meningitis (GNBM) and spontaneous meningitis due to other pathogens. Prospective single hospital-based observational cohort study conducted between 1982 and 2006 in a university tertiary hospital in Barcelona (Spain). The Main Outcome Measure: In-hospital mortality. Gram-negative bacilli meningitis was diagnosed in 40 (7%) of 544 episodes of spontaneous acute bacterial meningitis. The most common pathogens were Escherichia coli and Pseudomonas species. On admission, characteristics associated with spontaneous gram-negative bacilli meningitis by multivariate modeling were advanced age, history of cancer, nosocomial acquisition of infection, urinary tract infection as distant focus of infection, absence of rash, hypotension, and a high cerebrospinal fluid white-cell count. Nine (23%) episodes were acquired in the hospital and they were most commonly caused by Pseudomonas. The in-hospital mortality rate was 53%. The mortality rate was higher among patients with Gram-negative bacillary meningitis than among those with other bacterial meningitis and their risk of death was twenty times higher than among patients infected with Neisseria meningitidis (odds ratio 20.47; 95% confidence interval 4.03-103.93; p<0.001). Gram-negative bacilli cause 9% of spontaneous bacterial meningitis of known etiology in adults. Characteristics associated with GNBM include advanced age, history of cancer, nosocomial acquisition, and urinary tract infection as distant focus of infection. The mortality rate is higher among patients with gram-negative bacillary meningitis than among those with other bacterial meningitides.

Symptoms and socio-economic impact of ependymoma on adult patients: results of the Adult Ependymoma Outcomes Project 2.

PubMed

Walbert, Tobias; Mendoza, Tito R; Vera-Bolaños, Elizabeth; Acquaye, Alvina; Gilbert, Mark R; Armstrong, Terri S

2015-01-01

Ependymoma is a rare central nervous system tumor of adults. Reports of patient symptoms, interference patterns and costs encountered by patients and families are limited. Adult ependymoma patients completed the online Ependymoma Outcomes Questionnaire II. The survey assesses disease and functional status as well as socio-economic factors. Descriptive statistics were used to report disease characteristics as well as economic and social impact. Independent samples t test was used to test if differences exist between high- and low-income groups in terms of symptom severity. Correlations were calculated between symptoms and cost estimates. 86 international patients participated (male = 50 %). The economic analysis focused on 78 respondents from the US. 48 % were employed and 55 % earned ≥$60,000. Tumors were located in the brain (44 %), spine (44 %) or both (12 %). Spine patients compared to brain patients reported significantly worse pain (4.4 versus 2.2, p < .003), numbness (5.3 versus 2.2, p < .001), fatigue (5.1 versus 3.6, p < .03), changes in bowel patterns (3.8 versus 1.4, p < .003) and weakness (4.2 versus 2.1, p < .006). Brain patients compared with spine patients had increased lack of appetite (.4 versus 2, p < .014). Patients with lower income (≤$59,999) had more problems concentrating (p < .024) and worse cognitive module severity scores (p < .024). Estimated average monthly out-of-pocket spending was $168 for medical co-pays and $59 for prescription medication. Patients with ependymoma are highly affected by their symptoms. Spinal patients report higher severity of symptoms. Patients in the lower income group report significantly higher severity of cognitive symptoms independent of disease site.

Management of acne vulgaris with hormonal therapies in adult female patients.

PubMed

Husein-ElAhmed, Husein

2015-01-01

Acne vulgaris is a very common condition affecting up of 93% of adolescents. Although rare, this disease may persist in adulthood. In adult women with acne (those older than 25 years old), this condition is particularly relevant because of the refractory to conventional therapies, which makes acne a challenge for dermatologists in this group of patients. In order to its potential risk for chronicity and the involvement of visible anatomical sites such as face and upper torso, acne has been associated with a wide spectrum of psychological and social dysfunction such as depression, anxiety, suicidal ideation, somatization, and social inhibition. In particular, adult women with acne have been shown to be adversely impacted by the effect of acne on their quality of life. For the last four decades, dermatologists have used hormonal therapies for the management of acne vulgaris in adult women, which are considered a rational choice given the severity and chronicity of this condition in this group of patients. The aim of this work is to review the hormonal drugs for management of acne. © 2015 Wiley Periodicals, Inc.

Outcomes of Allogeneic Hematopoietic Stem Cell Transplantation in Adult Patients with Myelodysplastic Syndrome Harboring Trisomy 8.

PubMed

Konuma, Takaaki; Miyazaki, Yasushi; Uchida, Naoyuki; Ohashi, Kazuteru; Kondo, Tadakazu; Nakamae, Hirohisa; Takahashi, Satoshi; Mori, Takehiko; Ozawa, Yukiyasu; Kato, Chiaki; Iwato, Koji; Fukuda, Takahiro; Ichinohe, Tatsuo; Atsuta, Yoshiko; Ishiyama, Ken

2017-01-01

Trisomy 8 (+8) is 1 of the most common cytogenetic abnormalities in adult patients with myelodysplastic syndrome (MDS). However, the outcome of allogeneic hematopoietic stem cell transplantation (HSCT) in adult patients with MDS harboring +8 remains unclear. To evaluate the outcome and prognostic factors in patients with MDS harboring +8 as the sole cytogenetic abnormality or in association with other abnormalities, we retrospectively analyzed the Japanese registration data of 381 adult patients with MDS harboring +8 treated with allogeneic HSCT between 1990 and 2013. With a median follow-up period of 53 months, the probability of overall survival and cumulative incidence of relapse at 4 years were 51% and 22%, respectively. In the multivariate analysis, age > 50 years, 2 or more additional cytogenetic abnormalities, and a high risk at the time of HSCT according to the FAB/WHO classification were significantly associated with a higher overall mortality. Nevertheless, no significant impact of the outcome was observed in patients with 1 cytogenetic abnormality in addition to +8. Although 221 patients (58%) had advanced MDS at the time of HSCT, allogeneic HSCT offered a curative option for adult patients with MDS harboring +8. Copyright © 2017 The American Society for Blood and Marrow Transplantation. Published by Elsevier Inc. All rights reserved.

Effect of recurrent severe hypoglycemia on cognitive performance in adult patients with diabetes: A meta-analysis.

PubMed

Chen, Yu-Xue; Liu, Zheng-Ren; Yu, Ying; Yao, En-Sheng; Liu, Xing-Hua; Liu, Lu

2017-10-01

The purpose of this study was to investigate the existence and extent of cognitive impairment in adult diabetes mellitus (DM) patients with episodes of recurrent severe hypoglycemia, by using meta-analysis to synthesize data across studies. PubMed, EMBASE and Cochrane library search engines were used to identify studies on cognitive performance in DM patients with recurrent severe hypoglycemia. Random-effects meta-analysis was performed on seven eligible studies using an inverse-variance method. Effect sizes, which are the standardized differences between the experimental group and the control group, were calculated. Of the 853 studies, 7 studies met the inclusion criteria. Compared with control subjects, the adult DM patients with episodes of recurrent severe hypoglycemia demonstrated a significantly lowered performance on memory in both types of DM patients, and poor performance of processing speed in type 2 DM patients. There was no significant difference between adult DM patients with and those without severe hypoglycemia in other cognitive domains such as general intelligence, executive function, processing speed and psychomotor efficiency. Our results seem to confirm the hypothesis that cognitive dysfunction is characterized by worse memory and processing speed in adult DM patients with a history of recurrent severe hypoglycemia, whereas general intelligence, executive function, and psychomotor efficiency are spared.

Risk factors for concurrent bacteremia in adult patients with dengue.

PubMed

Thein, Tun-Linn; Ng, Ee-Ling; Yeang, Ming S; Leo, Yee-Sin; Lye, David C

2017-06-01

Bacteremia in dengue may occur with common exposure to pathogens in association with severe organ impairment or severe dengue, which may result in death. Cohort studies identifying risk factors for concurrent bacteremia among patients with dengue are rare. We conducted a retrospective case-control study of adult patients with dengue who were admitted to the Department of Infectious Diseases at Tan Tock Seng Hospital, Singapore from 2004 to 2008. For each case of dengue with concurrent bacteremia (within the first 72 hours of admission), we selected four controls without bacteremia, who were matched on year of infection and dengue confirmation method. Conditional logistic regression was performed to identify risk factors for concurrent bacteremia. Among 9,553 patients with dengue, 29 (0.3%) had bacteremia. Eighteen of these patients (62.1%) had concurrent bacteremia. The predominant bacteria were Staphylococcus aureus, one of which was a methicillin-resistant strain. Dengue shock syndrome occurred more frequently and hospital stay was longer among cases than among controls. Three cases did not survive, whereas none of the controls died. In multivariate analysis, being critically ill at hospital presentation was independently associated with 15 times the likelihood of a patient with dengue having concurrent bacteremia. Concurrent bacteremia in adult patients with dengue is uncommon but presents atypically and results in more deaths and longer hospital stay. Given the associated mortality, collection of blood cultures and empiric antibiotic therapy may be considered in patients who are critically ill. Copyright © 2015. Published by Elsevier B.V.

Long-term survival of adult trauma patients.

PubMed

Davidson, Giana H; Hamlat, Christian A; Rivara, Frederick P; Koepsell, Thomas D; Jurkovich, Gregory J; Arbabi, Saman

2011-03-09

Inpatient trauma case fatality rates may provide an incomplete assessment for overall trauma care effectiveness. To date, there have been few large studies evaluating long-term mortality in trauma patients and identifying predictors that increase risk for death following hospital discharge. To determine the long-term mortality of patients following trauma admission and to evaluate survivorship in relationship with discharge disposition. Retrospective cohort study of 124,421 injured adult patients during January 1995 to December 2008 using the Washington State Trauma Registry linked to death certificate data. Kaplan-Meier and Cox proportional hazards models were used to evaluate long-term mortality following hospital admission for trauma. Of the 124,421 trauma patients, 7243 died before hospital discharge and 21,045 died following hospital discharge. Cumulative mortality at 3 years postinjury was 16% (95% confidence interval [CI], 15.8%-16.2%) compared with the expected population cumulative mortality of 5.9% (95% CI, 5.9%-5.9%). In-hospital mortality improved during the 14-year study period from 8% (n = 362) to 4.9% (n = 600), whereas long-term cumulative mortality increased from 4.7% (95% CI, 4.1%-5.4%) to 7.4% (95% CI, 6.8%-8.1%). After adjustments for confounders, patients who were older and those who were discharged to a skilled nursing facility had the highest risk of death. The adjusted hazard ratios (HRs) for death after discharge to a skilled nursing facility compared with that after discharge home were 1.41 (95% CI, 0.72-2.76) for patients aged 18 to 30 years, 1.92 (95% CI, 1.36-2.73) for patients aged 31 to 45 years, 2.02 (95% CI, 1.39-2.93) for patients aged 46 to 55 years, 1.93 (95% CI, 1.40-2.64) for patients aged 56 to 65 years, 1.49 (95% CI, 1.14-1.94) for patients aged 66 to 75 years, 1.54 (95% CI, 1.27-1.87) for patients aged 76 to 80 years, and 1.38 (95% CI, 1.09-1.74) for patients older than 80 years. Other significant predictors of mortality

Leading Change: A Case Study of the First Independent Critical-Access Hospital to Achieve Magnet® Designation.

PubMed

Nelson-Brantley, Heather V; Ford, Debra J; Miller, Karen L; Stegenga, Kristin A; Lee, Robert H; Bott, Marjorie J

2018-03-01

The aim of this study was to understand how nurses in a 25-bed critical-access hospital (CAH) led change to become the 1st to achieve Magnet®. Approximately 21% of the US population lives in rural areas served by CAHs. Rural nurse executives are particularly challenged with limited resources. Staff nurses, nurse managers, interprofessional care providers, the chief nursing officer, and board of directors (n = 27) were interviewed. Observations of hospital units and administrative meetings were done, and hospital reports were analyzed. Nine themes emerged to support a conceptual model of leading change. The CAH spent 3 years of its 6-year journey establishing organizational readiness. Nurses overcame complex challenges by balancing operational support and fostering relationships. The Magnet journey led to significantly improved nurse and patient outcomes. A new organizational culture centered on shared governance, evidence-based practice, and higher education emerged. The journey to Magnet leads to improved nurse, patient, and organization outcomes.

Pediatric Patient Blood Management Programs: Not Just Transfusing Little Adults.

PubMed

Goel, Ruchika; Cushing, Melissa M; Tobian, Aaron A R

2016-10-01

Red blood cell transfusions are a common life-saving intervention for neonates and children with anemia, but transfusion decisions, indications, and doses in neonates and children are different from those of adults. Patient blood management (PBM) programs are designed to assist clinicians with appropriately transfusing patients. Although PBM programs are well recognized and appreciated in the adult setting, they are quite far from standard of care in the pediatric patient population. Adult PBM standards cannot be uniformly applied to children, and there currently is significant variation in transfusion practices. Because transfusing unnecessarily can expose children to increased risk without benefit, it is important to design PBM programs to standardize transfusion decisions. This article assesses the key elements necessary for a successful pediatric PBM program, systematically explores various possible pediatric specific blood conservation strategies and the current available literature supporting them, and outlines the gaps in the evidence suggesting need for further/improved research. Pediatric PBM programs are critically important initiatives that not only involve a cooperative effort between pediatric surgery, anesthesia, perfusion, critical care, and transfusion medicine services but also need operational support from administration, clinical leadership, finance, and the hospital information technology personnel. These programs also expand the scope for high-quality collaborative research. A key component of pediatric PBM programs is monitoring pediatric blood utilization and assessing adherence to transfusion guidelines. Data suggest that restrictive transfusion strategies should be used for neonates and children similar to adults, but further research is needed to assess the best oxygenation requirements, hemoglobin threshold, and transfusion strategy for patients with active bleeding, hemodynamic instability, unstable cardiac disease, and cyanotic cardiac

Congenital Adrenal Hyperplasia: Unresolved Issues.

PubMed

Yau, Mabel; Khattab, Ahmed; Poppas, Dix; Ghizzoni, Lucia; New, Maria

2016-01-01

Congenital adrenal hyperplasia (CAH) describes a family of disorders that comes from enzymatic deficiencies in cortisol production, with 21-hydroxylase deficiency causing ∼90% of cases. Distinction is made between the severe classical form and milder nonclassical form of CAH. Molecular genetic analysis is used to confirm the hormonal diagnosis. A high rate of genotype-phenotype disconcordance has been found in 21-hydroxylase deficiency. The goal of treatment is to replace with synthetic glucocorticoids and mineralocorticoids and suppress adrenal androgen production. The treatment of patients affected with nonclassical CAH, particularly males, remains controversial. Variable synthetic glucocorticoids are used and new modes of glucocorticoid delivery are under investigation. To improve height, growth hormone and other adjuvant therapies are employed. Long-term outcomes of genital surgery using modern techniques in females affected with classical CAH continue to be investigated. Prenatal treatment with dexamethasone is available to avoid ambiguous genitalia in these females. Although studies have shown its safety to mother and fetus, prenatal treatment is still regarded as experimental. Currently, prenatal diagnosis of CAH can only be obtained through invasive methods. Recently, the detection of cell-free fetal DNA in maternal plasma has made it possible to make this diagnosis earlier and noninvasively. © 2016 S. Karger AG, Basel.

Patient-Oncologist Alliance, Psychosocial Well-Being, and Treatment Adherence Among Young Adults With Advanced Cancer

PubMed Central

Trevino, Kelly M.; Fasciano, Karen; Prigerson, Holly G.

2013-01-01

Purpose Patients who develop a strong alliance with their health care providers have been shown to have higher levels of psychosocial well-being and rates of treatment adherence. Young adults with cancer have lower levels of psychosocial well-being and treatment adherence relative to patients with cancer in other age groups. This study sought to evaluate the relationships between the patient-oncologist alliance, psychosocial well-being, and treatment adherence in young adults with advanced cancer. Patients and Methods Ninety-five young adults (age 20 to 40 years) with advanced cancer were administered measures of alliance, psychosocial well-being, willingness to adhere to treatment, and treatment adherence. Relationships between alliance and psychosocial well-being were examined bivariately. Multiple linear regression models examined the relationship between alliance and adherence, controlling for confounding influences (eg, psychosocial well-being). Results Alliance was significantly (P ≤ .01) and positively associated with greater perceived social support and less severe illness-related grief. After controlling for significant confounding influences (ie, metastases, appraised support, and grief), alliance remained significantly (P ≤ .01) associated with greater willingness to adhere to treatment and greater adherence to oral medication. Conclusion By developing a strong alliance, oncologists may enhance psychosocial well-being and increase treatment adherence in young adult patients with advanced cancer. PMID:23530105

Chymase Level Is a Predictive Biomarker of Dengue Hemorrhagic Fever in Pediatric and Adult Patients.

PubMed

Tissera, Hasitha; Rathore, Abhay P S; Leong, Wei Yee; Pike, Brian L; Warkentien, Tyler E; Farouk, Farouk S; Syenina, Ayesa; Eong Ooi, Eng; Gubler, Duane J; Wilder-Smith, Annelies; St John, Ashley L

2017-11-27

Most patients with dengue experience mild disease, dengue fever (DF), while few develop the life-threatening diseases dengue hemorrhagic fever (DHF) or dengue shock syndrome (DSS). No laboratory tests predict DHF or DSS. We evaluated whether the serum chymase level can predict DHF or DSS in adult and pediatric patients and the influence of preexisting conditions (PECs) on chymase levels. Serum chymase levels were measured in patients presenting with undifferentiated fever to hospitals in Colombo District, Sri Lanka. The value of serum the chymase concentration and clinical signs and symptoms as predictors of DHF and/or DSS was evaluated by multivariate analysis. We assessed the influence of age, PECs, and day after fever onset on the robustness of the chymase level as a biomarker for DHF and/or DSS. An elevated chymase level in acute phase blood samples was highly indicative of later diagnosis of DHF or DSS for pediatric and adult patients with dengue. No recorded PECs prevented an increase in the chymase level during DHF. However, certain PECs (obesity and cardiac or lung-associated diseases) resulted in a concomitant increase in chymase levels among adult patients with DHF. These results show that patients with acute dengue who present with high levels of serum chymase consistently are at greater risk of DHF. The chymase level is a robust prognostic biomarker of severe dengue for adult and pediatric patients. © The Author 2017. Published by Oxford University Press for the Infectious Diseases Society of America.

Prevalence of obesity among children and/or grandchildren of adult bariatric surgery patients.

PubMed

Bao, Jean J; Desai, Vikas; Christoffel, Katherine Kaufer; Smith-Ray, Patrick; Nagle, Alex P

2009-07-01

Clinical experience suggests that some adults who undergo bariatric surgery have children who are obese. Childhood obesity is associated with increased morbidity and mortality in later life. This study examined the prevalence of obesity among children and grandchildren (< or =12 years of age) of adult bariatric surgery patients. Patients in a prospective database of morbidly obese patients who underwent bariatric surgery between January 2004 and May 2007 were recruited by phone and in clinic. Patient demographics, body mass index (BMI) at surgery, and survey data were collected. The survey included questions regarding their child/grandchild's body habitus, weight, and height. Child obesity was defined as BMI percentile > or =95. Statistical significance was set at p < 0.05. One hundred twenty-two patients were enrolled in this study (77% women, mean BMI 49 kg/m(2)). One hundred thirty-four out of 233 children/grandchildren identified had complete data; 41% had a BMI percentile > or =95. Only 29% of these obese children were so identified by the adult respondents. Significantly more biological children/grandchildren were obese than nonbiological (p = 0.013), and significantly more biological children were obese than biological grandchildren (p = 0.027). This sample of bariatric surgery patients had a high proportion of obese preteen children/grandchildren. Obesity was most prevalent among biological children (vs. biological grandchildren and nonbiological children). Patients often did not recognize the degree of overweight in their children/grandchildren. Because families of bariatric surgery patients often include obese children, interventions aimed at all family members merit consideration.

[Genotypes of rhinoviruses in children and adults patients with acute respiratory tract infections].

PubMed

Demirkan, Eda; Kırdar, Sevin; Ceylan, Emel; Yenigün, Ayşe; Kurt Ömürlü, İmran

2017-10-01

Rhinovirus (RV) is one of the most frequent causative agent of acute respiratory tract infections in the world. The virus may cause a mild cold, as well as more serious clinical symptoms in patients with immune system deficiency or comorbidities. Rhinoviruses have been identified by molecular methods under three types: RV-A, RV-B and RV-C. In most of the cases, it was reported that RV-A and RV-C were related with lower respiratory tract infections and asthma exacerbations, while RV-B was rarely reported in lower respiratory tract infections. The main objective of this study was to investigate RV species by sequence analysis in nasopharyngeal samples in pediatric and adult patients who were admitted to hospital with acute respiratory tract infections and to establish the relationship between species and age, gender and clinical diagnosis of the patients. Secondly, it was planned to emphasize the efficiency of the sequence analysis method in the determination of RV species. One hundred twenty seven patients (children and adults) who were followed up with acute respiratory tract infections in our university hospital were evaluated between January 2014 and January 2016. Viral loads were determined by quantitative real-time PCR in RV positive patients detected by a commercial kit in nasopharyngeal swab specimens. Thirty-one samples whose viral loads could not be determined were excluded from the study. The remaining 96 samples (50 children and 46 adults) were retested by conventional PCR using the target of VP4/VP2 gene region. A total of 65 samples (32 adults and 33 children) with the bands (549 bp) corresponding to the VP4/VP2 gene regions after the conventional PCR were analyzed by DNA sequencing. A phylogenetic tree was constructed using the neighbour-joining method. After sequence analysis it was determined that 28 (43.07%) were RV-A, 7 (10.76%) were RV-B and 28 (43.07%) were RV-C; and moreover one of each enterovirus (EV) species EV-D68 (1.53%) and EV-C (1

[Maintenance Treatment With Antipsychotics for Adult Patients Diagnosed With Schizophrenia].

PubMed

Gómez-Restrepo, Carlos; Bohórquez Peñaranda, Adriana Patricia; de la Hoz Bradford, Ana María; Tamayo Martínez, Nathalie; García Valencia, Jenny; Jaramillo González, Luis Eduardo

2014-01-01

To determine the effectiveness and security of the antipsychotics available for the management of adult patients with schizophrenia in the maintenance phase. To develop recommendations of treatment for the maintenance phase of the disease. A clinical practice guideline was elaborated under the parameters of the Methodological Guide of the Ministerio de Salud y Protección Social to identify, synthesize and evaluate the evidence and make recommendations about the treatment and follow-up of adult patients with schizophrenia. The evidence of NICE guide 82 was adopted and updated. The evidence was presented to the Guideline Developing Group and recommendations, employing the GRADE system, were produced. 18 studies were included to evaluate the effectiveness and / or safety of different antipsychotic drugs first and second generation. Overall, antipsychotics (AP) showed superiority over placebo in relapse rate over 12 months (RR 0.59 95% CI 0.42, 0.82) and hospitalization rate over 24 months of follow-up (RR 0.38 95% 0.27, 0.55); its use is associated with increased risk of treatment dropout (RR 0.53 95% CI 0.46, 0.61) and adverse events such as weight gain, dystonia, extrapyramidal symptoms and sedation. There was no difference in the outcome of re hospitalizations, comparisons on quality of life, negative symptoms or weight gain between AP first and second generation. Continuous or standard dose regimens appear to be superior to intermittent or low doses in reducing the risk of abandonment of treatment regimes. Adult patients diagnosed with schizophrenia should receive maintenance treatment with antipsychotics. The medication of choice will depend on the management of the acute phase, the patient's tolerance to it and the presentation of adverse events. Copyright © 2014 Asociación Colombiana de Psiquiatría. Publicado por Elsevier España. All rights reserved.

Functional studies of novel CYP21A2 mutations detected in Norwegian patients with congenital adrenal hyperplasia

PubMed Central

Brønstad, Ingeborg; Breivik, Lars; Methlie, Paal; Wolff, Anette S B; Bratland, Eirik; Nermoen, Ingrid; Løvås, Kristian; Husebye, Eystein S

2014-01-01

In about 95% of cases, congenital adrenal hyperplasia (CAH) is caused by mutations in CYP21A2 gene encoding steroid 21-hydroxylase (21OH). Recently, we have reported four novel CYP21A2 variants in the Norwegian population of patients with CAH, of which p.L388R and p.E140K were associated with salt wasting (SW), p.P45L with simple virilising (SV) and p.V211M+p.V281L with SV to non-classical (NC) phenotypes. We aimed to characterise the novel variants functionally utilising a newly designed in vitro assay of 21OH enzyme activity and structural simulations and compare the results with clinical phenotypes. CYP21A2 mutations and variants were expressed in vitro. Enzyme activity was assayed by assessing the conversion of 17-hydroxyprogesterone to 11-deoxycortisol by liquid chromatography tandem mass spectroscopy. PyMOL 1.3 was used for structural simulations, and PolyPhen2 and PROVEAN for predicting the severity of the mutants. The CYP21A2 mutants, p.L388R and p.E140K, exhibited 1.1 and 11.3% of wt 21OH enzyme activity, respectively, in vitro. We could not detect any functional deficiency of the p.P45L variant in vitro; although prediction tools suggest p.P45L to be pathogenic. p.V211M displayed enzyme activity equivalent to the wt in vitro, which was supported by in silico analyses. We found good correlations between phenotype and the in vitro enzyme activities of the SW mutants, but not for the SV p.P45L variant. p.V211M might have a synergistic effect together with p.V281L, explaining a phenotype between SV and NC CAH. PMID:24671123

Transitioning issues in adolescent to young adult hemophilia patients with inhibitors: an approach for a growing population.

PubMed

Young, Guy

2010-09-01

The major adverse effect of factor replacement therapy in patients with hemophilia is the development of neutralizing antibodies termed inhibitors. This complication renders standard factor replacement therapy ineffective resulting in increased morbidity and mortality. Until recently, the population of adults with inhibitors was relatively small due to the death of many of the patients from HIV that they contracted from contaminated factor in the early 1980s. With the advent of factor products with reduced risks for deadly infections in the mid-1980s to early 1990s, a cohort of inhibitor patients is now beginning to enter adulthood thus raising the issues regarding the transition of these patients into adulthood. It is, therefore, expected that adult hematologists will be seeing more inhibitor patients and that pediatric hematologists will be faced with managing this transition process, which may not necessarily include transition to an adult facility or adult hematologist. This review will discuss the various issues ranging from choice of medical provider to a discussion of psychosocial and financial issues facing this specific patient population.

[Therapeutic Efficacy of VICP+L-ASP/TKI on Adult Patients with B-ALL].

PubMed

Shen, Ming-Fang; Wei, Ju-Ying; Yu, Wen-Juan; Wang, Jing-Han; Tong, Hong-Yan; Meng, Hai-Tao; Mai, Wen-Yuan; Qian, Wen-Bin; Jin, Jie

2016-12-01

To evaluate the therapeutic efficacy of VICP+L-ASP/TKI on adult patients with B-ALL and to explore the influence factors. Forty-one adult B-ALL patients treated with VICP+L-ASP/TKI from August 2008 to June 2014 were following-up. The complete remission(CR) rate, toxicity, overall survival(OS) and event free survival(EFS) after induction treatment were analyzed, the therapeutic outcome of patients between different risk stratification subgroups was compared, the influence of standardized consolidatory and maintaining treatment as well as allogeneic hematopoietic stem cell transplantation(allo-HSCT) on survival time was analyzed. The early death not occurred in 41 patients with B-ALL including 37 cases with CR; the CR rate of 1 course treatment was 90.2%. The follow-up time lasted to March 17, 2015, the median follow-up time was 25(9-79) months; the 1 year OS rate was 75.3%, the EFS rate was 58.3%. Analysis of risk factors showed that the initial WBC count over 30×10 9 /L, LDH over 250 U/L and minimal residual disease(MRD) over 10 -4 after treatment were poor prognostic factors. After remission, the standardized consolidatory treatment or allo-HSCT according to the "2012 China adult ALL diagnosis and treatment expert consensus" could improve long-term survival, 3 years OS rate was 73.8% and 61.5% respectively, 3 years EFS were 63.5% and 65.7% respectively. The main toxic and side effects were hematologic reactions, the hematologic adverse reaction of IV grade was observed in 97.6%(40/41) during induction treatment. Induction chemotherapy based on VICP+L-ASP/TKI and standardized consolidatory after remission according to the "2012 China adult acute lymphoblastic leukemia diagnosis and treatment expert consensus" can improve the therapeutic efficacy. The allo-HSCT should be actively performed for B-ALL paients with high risk(elevated initial WBC count and LDH level); at some time, the regularly monitoring MRD and adjusting therapeutic protocol according to monitoring

Health literacy and self-rated health in adults primary care patients.

PubMed

Marques, Suzana Raquel Lopes; Escarce, Andrezza Gonzalez; Lemos, Stela Maris Aguiar

2018-01-01

Purpose To verify the association between health literacy, social determinants and self-rated health in adult's primary health care patients. Methods this is an Observational cross-sectional study in which a total of 380 patients of the Unified Health System in the context of primary health care were interviewed. The sample was probabilistic, stratified by gender, age, and Basic Health Unit. Health literacy was evaluated by an instrument of analysis of the perception of adults about the understanding of health orientations and possible difficulties in this process (Health Literacy Scale). Descriptive and association analyses were performed (Pearson's chi-square test, p≤0.05). Results It was verified that the majority of the interviewees belongs to classes C1 and C2 and attended high school (complete or incomplete). Regarding self-rated health, to be considered healthy and with good health were the predominant perceptions. In the Health Literacy Scale, it was verified that most patients reported never presenting difficulties in the situations of this instrument, except understanding written orientations. It was observed the association with a statistical significance of the better perception of health literacy with higher educational level and economic classification, as well as with self-rated of good health. Conclusion There was a statistical association between health literacy, social determinants, and self-rated health in the analyzed adults. It is noteworthy the contribution of the Health Literacy Scale for emphasizing the perception of difficulties in everyday health situations. It is necessary to develop dialogic relationships that build more robust communication processes between professionals and healthcare patients to favor health literacy skills.

Voiding patterns of adult patients who underwent hypospadias repair in childhood.

PubMed

Jaber, Jawdat; Kocherov, Stanislav; Chertin, Leonid; Farkas, Amicur; Chertin, Boris

2017-02-01

This study aimed at evaluating the voiding patterns of adult patients who underwent hypospadias repair in childhood. Following IRB approval 103 (22.7%) of 449 adult patients who underwent hypospadias repair between 1978 and 1993 responded to the following questionnaires: International Prostate Symptom Score (I-PSS) and Short Form 12 questionnaire (SF-12). Uroflowmetry (UF) was performed for all patients. The patients were divided into three groups according to the primary meatus localization. Group I had 63 patients (61.5%) treated for glanular hypospadias, group II had 19 patients (18.4%) treated for distal hypospadias, and group III comprised the remaining 21 patients (20.4%) treated for proximal hypospadias. The mean ± SD I-PSS score for all patients who responded to the questionnaire was 2.3 ± 2.4, and UF was 21.1 ± 4.3 mL/s. The patients from groups I and III had fewer urinary symptoms compared with those of the group II: 1.3 ± 1.5, 5.5 ± 2.4, and 1.6 ± 1.4, respectively (p < 0.0001). With regards to UF, the patients from the groups I and III did better compared with those from the group II: 22.1 ± 4.1 mL/s, 18.91 ± 4.2 mL/s, and 20.11 ± 3.42 mL/s, respectively (p = 0.021) (Figure). The UF was better in patients with normal vs. abnormal IPSS (p = 0.0064). The physical component summary was 49.8 ± 10.3, 51.1 ± 3.6, and 46.4 ± 0.3 in groups I, II, and III, respectively. The mental summary component was 42.64 ± 4.1, 42.2 ± 2.4, and 39.89 ± 2.9 in groups I, II, and III, respectively. Most of the adult patients who underwent hypospadias repair in childhood had normal or mild voiding disturbance, with no effects on their physical or mental status. Copyright © 2016 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.

The impact of patient autonomy on older adults with asthma.

PubMed

Karamched, Keerthi R; Hao, Wei; Song, Peter X; Carpenter, Laurie; Steinberg, Joel; Baptist, Alan P

2018-05-03

Understanding patient preferences and desire for involvement in making medical decisions is important when managing chronic conditions. Previous studies have utilized the Autonomy Preference Index (API) in younger asthmatic patients to evaluate these preferences. To identify factors associated with autonomy, and to determine if autonomy is related to asthma outcomes among older adults. 189 older adults (>55 yr) with persistent asthma were included. Preferences for autonomy were assessed using the API, with a higher score indicating higher desire for autonomy. Scores were separated into two domains of 'information seeking' and 'decision making' preferences. The separated scores were correlated with asthma outcomes and demographic variables. To control for confounding factors, a linear regression analysis was performed. Higher 'decision making' preference scores correlated with female gender (p=0.007), higher education level (p=0.01), and lower depression scores (p=0.04). Regarding outcomes, 'decision making' scores positively correlated with asthma quality of life questionnaire (AQLQ) scores (p=0.01). On linear regression analysis, the AQLQ score remained significantly associated with 'decision making' preference scores (p=0.03). There was no association with asthma control test scores, spirometry values, and healthcare utilization. 'Information seeking' preference scores correlated with education level (p=0.03), but there was no correlation with asthma outcomes. Older asthmatic adults with a greater desire for involvement in decision making have a higher asthma related quality of life. Future studies with the intention to increase patient autonomy may help establish a causal relationship. Copyright © 2018. Published by Elsevier Inc.

Clinical picture of meningitis in the adult patient and its relationship with age.

PubMed

Magazzini, Simone; Nazerian, Peiman; Vanni, Simone; Paladini, Barbara; Pepe, Giuseppe; Casanova, Barbara; Crugnola, Carolina; Grifoni, Stefano

2012-08-01

To analyze the clinical characteristics of acute meningitis and their relationship with age in adult patients presenting to the emergency department. We retrospectively investigated consecutive adult patients admitted with a diagnosis of bacterial or viral meningitis from 2002 to 2006. Data about patient's history, symptoms and signs at presentation, etiology and clinical course were collected. To investigate the relationship of clinical presentation with age, we divided patients in four age quartiles (<30 years, between 30 and 36 years, between 37 and 56 years, >56 years). Among the 202 patients considered in the study (mean age 42.8 ± 18.7 years, range 14-90), 162 (80.2%) patients had viral and 40 (19.8%) bacterial meningitis. Specific signs, such as neck stiffness or Kernig or Brudzinski signs, were more common in the first than in the fourth quartile (73.1 vs. 45.7% P = 0.041). Conversely, altered consciousness expressed as Glasgow Coma Scale (GCS) <15 was more frequent in the fourth (80.4%) than in the first (9.6%) quartile (P < 0.001). The linear regression analysis confirmed a significant decrease of GCS with the increasing of patient's age (r = -0.69, P < 0.001). At multivariate analysis, aging was associated with altered level of consciousness (OR 16.7, P < 0.001) independent of viral or bacterial etiology of the presence of comorbidities and of clinical severity (presence of severe sepsis or septic shock). Meningitis presentation largely differs with aging in adult patients. Level of consciousness is frequently altered in the older patients, when other specific signs become more rare, independent of etiology, comorbidities and clinical severity.

A monocentric experience of growth hormone replacement therapy in adult patients.

PubMed

Abdi, Lyès; Sahnoun-Fathallah, Mona; Morange, Isabelle; Albarel, Frédérique; Castinetti, Frédéric; Giorgi, Roch; Brue, Thierry

2014-07-01

To describe the results of growth hormone (GH) therapy in adult GH-deficient patients treated in a tertiary referral center, with a focus on quality of life and adherence. Retrospective study of patients followed over a total period of 11 years. Quality of life (QOL) was assessed by the QOL-Assessment of Growth Hormone Deficiency in Adults (QoL-AGHDA) score and adherence to treatment was measured by a specific questionnaire. Clinical, biological, body composition and bone mineralization parameters were also analyzed. Data from 81 patients were analyzed. After a median treatment duration of 7 years, 2/3 of patients reported improved QOL (mean decrease of AGHDA score of 3.0 points, P<0.001). A trend towards more frequent improvement was observed in middle-aged patients, women, childhood-onset GHD, and in patients with initially more impaired QOL. More than 60% of the patients reported continuing treatment without interruption. Seventy percent declared good adherence (≤2 missed injections/month). A majority reported enhanced well-being. Additionally, we observed a mean weight increase of 2 kg, while fat mass, waist/hip circumference ratio and lipids were unchanged. Bone mineral density was significantly increased at lumbar spine and femoral neck. Our study confirmed a sustained improvement in quality of life and showed that majority of patients were still on GH treatment after a median duration of 7 years. Copyright © 2014. Published by Elsevier Masson SAS.

Energy drink and other substance use among adolescent and young adult emergency department patients.

PubMed

Cotter, Bradford V; Jackson, Deidrya A E; Merchant, Roland C; Babu, Kavita M; Baird, Janette R; Nirenberg, Ted; Linakis, James G

2013-10-01

This study aimed to understand current patterns of energy drink use and compare the extent of usage of energy drinks and other commonly used and misused substances between adolescent (13-17-years-old) and young adult (18-25-years-old) emergency department (ED) patients. During a 6-week period between June and August 2010, all patients presenting to an adult or pediatric ED were asked to complete a computer-based, anonymous questionnaire regarding use of energy drinks and other substances. Wilcoxon rank-sum, 2-sample tests of binomial proportions, Pearson χ(2) testing, and regression models were used to compare energy drink and substance use by age groups. Past 30-day energy drink use was greater for young adults (57.9%) than adolescents (34.9%) (P < 0.03). Adolescents typically consumed a mean of 1.5 and young adults a mean of 2.6 energy drinks per day when using energy drinks and drank at most a mean of 2.4 and 2.6 drinks per day, respectively. Among adolescents, energy drink usage was more common than alcohol, "street" or illicit drugs, and tobacco usage, but less common than caffeine product usage. For young adults, energy drink usage was more common than "street" or illicit drugs, but less common than caffeine use, and similar to tobacco and alcohol usage. Young adult energy drink users were more likely than young adult non-energy drink users also to use tobacco and caffeine. Energy drink use is common among ED patients. Given the high prevalence of energy drink use observed, emergency physicians should consider the involvement of energy drinks in the presentations of young people.

Prehospital Care for the Adult and Pediatric Seizure Patient: Current Evidence-based Recommendations.

PubMed

Silverman, Eric C; Sporer, Karl A; Lemieux, Justin M; Brown, John F; Koenig, Kristi L; Gausche-Hill, Marianne; Rudnick, Eric M; Salvucci, Angelo A; Gilbert, Greg H

2017-04-01

We sought to develop evidence-based recommendations for the prehospital evaluation and treatment of adult and pediatric patients with a seizure and to compare these recommendations against the current protocol used by the 33 emergency medical services (EMS) agencies in California. We performed a review of the evidence in the prehospital treatment of patients with a seizure, and then compared the seizure protocols of each of the 33 EMS agencies for consistency with these recommendations. We analyzed the type and route of medication administered, number of additional rescue doses permitted, and requirements for glucose testing prior to medication. The treatment for eclampsia and seizures in pediatric patients were analyzed separately. Protocols across EMS Agencies in California varied widely. We identified multiple drugs, dosages, routes of administration, re-dosing instructions, and requirement for blood glucose testing prior to medication delivery. Blood glucose testing prior to benzodiazepine administration is required by 61% (20/33) of agencies for adult patients and 76% (25/33) for pediatric patients. All agencies have protocols for giving intramuscular benzodiazepines and 76% (25/33) have protocols for intranasal benzodiazepines. Intramuscular midazolam dosages ranged from 2 to 10 mg per single adult dose, 2 to 8 mg per single pediatric dose, and 0.1 to 0.2 mg/kg as a weight-based dose. Intranasal midazolam dosages ranged from 2 to 10 mg per single adult or pediatric dose, and 0.1 to 0.2 mg/kg as a weight-based dose. Intravenous/intrasosseous midazolam dosages ranged from 1 to 6 mg per single adult dose, 1 to 5 mg per single pediatric dose, and 0.05 to 0.1 mg/kg as a weight-based dose. Eclampsia is specifically addressed by 85% (28/33) of agencies. Forty-two percent (14/33) have a protocol for administering magnesium sulfate, with intravenous dosages ranging from 2 to 6 mg, and 58% (19/33) allow benzodiazepines to be administered. Protocols for a patient

Risk factor and etiology analysis of ischemic stroke in young adult patients.

PubMed

Renna, Rosaria; Pilato, Fabio; Profice, Paolo; Della Marca, Giacomo; Broccolini, Aldobrando; Morosetti, Roberta; Frisullo, Giovanni; Rossi, Elena; De Stefano, Valerio; Di Lazzaro, Vincenzo

2014-03-01

Approximately 10%-14% of ischemic strokes occur in young adults. To investigate risk factors and etiologies of strokes of young adults admitted to the "stroke unit" of Policlinico "Gemelli" of Rome from December 2005 to January 2013. In all, 150 consecutive patients younger than 50 years diagnosed with ischemic stroke were enrolled. Clinical evaluation consisted of a complete neurologic examination and the National Institutes of Health Stroke Scale. Diagnostic workup consisted of anamnesis, extensive laboratory, radiologic, and cardiologic examination. Stroke etiologies were classified according to the Trial of Org 10172 in Acute Stroke Treatment. Patients' mean age was 41 ± 8.0 years. The most common risk factors were dyslipidemia (52.7%), smoking (47.3%), hypertension (39.3%), and patent foramen ovale (PFO, 32.8%). Large-artery atherosclerosis was diagnosed as the cause of stroke in 17 patients (11.3%). Cardioembolism was presumed in 36 patients (24%), most of them presented a PFO at transesophageal echocardiography. Small-vessel occlusion was diagnosed in 12 patients (8%); all of them were hypertensive and most of them presented additional risk factors. Forty-one patients (27.3%) presented a stroke of other determined etiology and 44 (29.3%) presented a stroke of undetermined etiology. The 3-year survival was 96.8% and recurrent strokes occurred in only 3 cases. Traditional vascular risk factors are also very common in young adults with ischemic stroke, but such factors increase the susceptibility to stroke dependent to other causes as atherosclerosis and small-artery occlusion represent less than 20% of cases. Prognosis quoadvitam is good, being characterized by low mortality and recurrence rate. Copyright © 2014 National Stroke Association. Published by Elsevier Inc. All rights reserved.

Facilitating the transition of patients with special health care needs from pediatric to adult oral health care.

PubMed

Nowak, Arthur J; Casamassimo, Paul S; Slayton, Rebecca L

2010-11-01

Without guidelines or policies in dentistry for transitioning adolescents with special heath care needs from pediatric to adult oral health care, little is known about traditional support services. The authors surveyed pediatric dentists about their transition of adolescent patients with and without special health care needs (SHCNs) to adult care. In 2009, the authors e-mailed a pilot-tested survey modified from a survey used for U.S. pediatricians to 4,000 pediatric dentists. The survey included demographic questions and questions regarding services and barriers associated with the transition of patients to adult care. Responses were obtained from 1,686 (42.2 percent response) pediatric dentists who were mostly in group or solo private practices and were younger, in that most had completed their education in the preceding 15 years. More than one-half practiced in suburban settings, and most worked with both dental hygienists and dental assistants. Most assisted patients with SHCNs with their transitions to adult care, and the predominant barrier to transitioning to adult care was availability of general dentists and specialists who were willing to accept these new patients. Pediatric dentists' answers paralleled those of pediatricians for the most part in terms of services provided and barriers to transition. Most responding dentists helped adolescents with and without SHCNs make the transition into adult care, but the major barrier was the availability of general dentists and specialists. With an office protocol in place that includes trained staff members, transitioning patients (especially those with SHCNs) to adult care can be facilitated to provide the appropriate oral health and support services.

Contrasting outcomes of older versus middle-aged and younger adult chemical dependency patients in a managed care program.

PubMed

Satre, Derek D; Mertens, Jennifer; Areán, Patricia A; Weisner, Constance

2003-07-01

This study examined how well older chemical dependency patients succeed in treatment relative to middle-aged and younger patients in a mixed-age private HMO outpatient program. To predict successful outcome, we tested a model incorporating age group differences in individual, treatment and extratreatment factors. The sample included 89 patients aged 55 and over, 379 patients aged 40 to 54, and 736 patients aged 18-39 (N = 1,204). Baseline measures included DSM-IV substance misuse diagnoses, Addiction Severity Index (ASI), psychiatric symptom checklist, sources of suggestion to enter treatment, treatment history and motivation. Outcome measures were abstinence rates and ASI score 6 months posttreatment. At baseline, older adults showed higher levels of alcohol dependence, lower rates of drug dependence and lower psychiatric symptoms relative to younger individuals. Source of suggestions to enter treatment differed by age. Older and middle-aged patients were more likely to have an abstinence goal and to stay in treatment longer than younger adults. At 6 months posttreatment, 55% of older adults reported abstinence in the preceding 30 days, versus 59% of middle-aged adults and 50% of younger adults (p = .035). Lower rates of dependence and hostility, and greater abstinence motivation and length of stay in treatment--all of which were associated with greater age--positively affect prognosis of older adults in treatment.

Clinical value of antistreptolysin O levels in adult patients with tonsillitis: report I.

PubMed

Trushin, Vladimir; Englender, Moshe

2017-04-01

To assess the clinical value of antistreptolysin O (ASO) level in adult patients with acute tonsillitis of group A beta-hemolytic streptococcus (GABHS) etiology and its interaction with the Centor score and throat cultures data. ASO antibody titers and throat cultures were obtained from 260 adult patients with acute tonsillitis of GABHS etiology initially proven by the Centor score. The results were compared with the group of 100 adult patients with recurrent tonsillitis who underwent tonsillectomy and with the group of 100 healthy adults. Throat cultures revealed GABHS-positive results in 69 acute cases (26.5%) and in 24 recurrent cases (24%), i.e., with no significant differences between the groups (p = 0.845). There was no significant difference between cases with GABHS-positive and with GABHS-negative throat culture in ASO titers results (mean 250 and 280, respectively, p = 0.44) but these titers were significantly higher than established normative data (p < 0.01). For the group of recurrent tonsillitis cases, the mean ASO titer was 363 being significantly higher in comparison with acute cases (p = 0.015). The ASO antibody titers are significantly higher than normative ranges in cases of acute tonsillitis in adults. The detection of the elevated titers may lead to early antibiotherapy to tonsillitis. The Centor score is supported by the ASO data and less supported by throat cultures data. Further research should reveal if these titers might have predictive value for possible further recurrence or serve as an indicator for tonsillectomy in cases of recurrent tonsillitis.

Outcome differences in adolescent blunt severe polytrauma patients managed at pediatric versus adult trauma centers.

PubMed

Rogers, Amelia T; Gross, Brian W; Cook, Alan D; Rinehart, Cole D; Lynch, Caitlin A; Bradburn, Eric H; Heinle, Colin C; Jammula, Shreya; Rogers, Frederick B

2017-12-01

Previous research suggests adolescent trauma patients can be managed equally effectively at pediatric and adult trauma centers. We sought to determine whether this association would be upheld for adolescent severe polytrauma patients. We hypothesized that no difference in adjusted outcomes would be observed between pediatric trauma centers (PTCs) and adult trauma centers (ATCs) for this population. All severely injured adolescent (aged 12-17 years) polytrauma patients were extracted from the Pennsylvania Trauma Outcomes Study database from 2003 to 2015. Polytrauma was defined as an Abbreviated Injury Scale (AIS) score ≥3 for two or more AIS-defined body regions. Dead on arrival, transfer, and penetrating trauma patients were excluded from analysis. ATC were defined as adult-only centers, whereas standalone pediatric hospitals and adult centers with pediatric affiliation were considered PTC. Multilevel mixed-effects logistic regression models assessed the adjusted impact of center type on mortality and total complications while controlling for age, shock index, Injury Severity Score, Glasgow Coma Scale motor score, trauma center level, case volume, and injury year. A generalized linear mixed model characterized functional status at discharge (FSD) while controlling for the same variables. A total of 1,606 patients met inclusion criteria (PTC: 868 [54.1%]; ATC: 738 [45.9%]), 139 (8.66%) of which died in-hospital. No significant difference in mortality (adjusted odds ratio [AOR]: 1.10, 95% CI 0.54-2.24; p = 0.794; area under the receiver operating characteristic: 0.89) was observed between designations in adjusted analysis; however, FSD (AOR: 0.38, 95% CI 0.15-0.97; p = 0.043) was found to be lower and total complication trends higher (AOR: 1.78, 95% CI 0.98-3.32; p = 0.058) at PTC for adolescent polytrauma patients. Contrary to existing literature on adolescent trauma patients, our results suggest patients aged 12-17 presenting with polytrauma may experience

Successful cardiac transplantation outcomes in patients with adult congenital heart disease.

PubMed

Menachem, Jonathan N; Golbus, Jessica R; Molina, Maria; Mazurek, Jeremy A; Hornsby, Nicole; Atluri, Pavan; Fuller, Stephanie; Birati, Edo Y; Kim, Yuli Y; Goldberg, Lee R; Wald, Joyce W

2017-09-01

The purpose of our study is (1) to characterise patients with congenital heart disease undergoing heart transplantation by adult cardiac surgeons in a large academic medical centre and (2) to describe successful outcomes associated with our multidisciplinary approach to the evaluation and treatment of adults with congenital heart disease (ACHD) undergoing orthotopic heart transplantation (OHT). Heart failure is the leading cause of death in patients with ACHD leading to increasing referrals for OHT. The Penn Congenital Transplant Database comprises a cohort of patients with ACHD who underwent OHT between March 2010 and April 2016. We performed a retrospective cohort study of the 20 consecutive patients. Original cardiac diagnoses include single ventricle palliated with Fontan (n=8), dextro-transposition of the great arteries after atrial switch (n=4), tetralogy of Fallot (n=4), pulmonary atresia (n=1), Ebstein anomaly (n=1), unrepaired ventricular septal defect (n=1) and Noonan syndrome with coarctation of the aorta (n=1). Eight patients required pretransplant inotropes and two required pretransplant mechanical support. Nine patients underwent heart-liver transplant and three underwent heart-lung transplant. Three patients required postoperative mechanical circulatory support. Patients were followed for an average of 38 months as of April 2016, with 100% survival at 30 days and 1 year and 94% overall survival (19/20 patients). ACHD-OHT patients require highly specialised, complex and multidisciplinary healthcare. The success of our programme is attributed to using team-based, patient-centred care including our multidisciplinary staff and specialists across programmes and departments. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

Restoration of Oral Function for Adult Edentulous Patients with Ectodermal Dysplasia: A Prospective Preliminary Clinical Study.

PubMed

Wu, Yiqun; Wang, Xu Dong; Wang, Feng; Huang, Wei; Zhang, Zhiyong; Zhang, Zhiyuan; Kaigler, Darnell; Zou, Duohong

2015-10-01

Therapy with zygomatic implants (ZIs) or conventional implants (CIs) has proven to be an effective method to restore oral function for systemically healthy patients. However, it is still a major challenge to fully restore oral function to edentulous adult patients with ectodermal dysplasia (ED). The aim of this study was to determine an effective treatment protocol for restoring oral function using ZIs and CIs to edentulous adult ED patients. Ten edentulous adult ED patients were treated in this study. The treatment protocol involved the following: (1) bone augmentation in the region of the anterior teeth; (2) placement of two ZIs and four CIs in the maxilla, and four CIs in the mandible; (3) fabrication of dental prosthesis; and (4) psychological and oral education. Following treatment of these patients, implant success rates, biological complications, patient satisfaction, and psychological changes were recorded. Although there was evidence of bone graft resorption in the maxilla, bone augmentation of the mandible was successful in all patients. Nine CIs in the maxilla failed and were removed. All ZIs were successful, and the CIs success rates were 77.50% in the maxilla and 100% in the mandible, with a mean of 88.75%. The mean peri-implant bone resorption for the CIs ranged from 1.3 ± 0.4 mm to 1.8 ± 0.6 mm, and four cases exhibited gingival hyperplasia in the maxilla and mandible. One hundred percent of the patients were satisfied with the restoration of their oral function, and >50% of the patients exhibited enhanced self-confidence and self-esteem. This study demonstrates that oral function can be restored in edentulous adult ED patients using a comprehensive and systematic treatment protocol involving psychological and oral education, bone augmentation, implant placement, and denture fabrication. Despite these positive outcomes, bone augmentation remains challenging in the anterior region of the maxilla for edentulous adult ED patients. © 2015

Hormonal and echocardiographic abnormalities in adult patients with sickle-cell anemia in Bahrain

PubMed Central

Garadah, Taysir S; Jaradat, Ahmed A; Alalawi, Mohammed E; Hassan, Adla B

2016-01-01

Background Adrenal, thyroid, and parathyroid gland hormonal changes are recognized in children with homozygous (HbSS) sickle-cell anemia (SCA), but are not clear in adult patients with SCA. Aim To assess the metabolic and endocrine abnormalities in adult patients with SCA and evaluate left ventricular (LV) systolic and diastolic functions compared with patients with no SCA and further study the relationship between serum levels of cortisol, free thyroxine (T4), and testosterone with serum ferritin. Materials and methods The study was conducted on 82 patients with adult HbSS SCA compared with a sex- and age-matched control group. The serum levels of cortisol, parathyroid hormone (PTH), testosterone, thyroid-stimulating hormone (TSH), and free T4 were compared. Blood levels of hemoglobin, reticulocyte count, lactate dehydrogenase (LDH), calcium, alkaline phosphatase (ALP), vitamin D3, and ferritin were also compared. Pulsed Doppler echo was performed to evaluate the LV mass, wall thickness, and cavity dimensions with diastolic filling velocities of early (E) and atria (A) waves. Biometric data were analyzed as mean ± standard deviation between the two groups. Multiple regression analysis was performed between serum levels of ferritin as independent variable and testosterone, cortisol, and thyroid hormones. Results A total of 82 adult patients with HbSS SCA were enrolled who had a mean age of 21±5.7 years, with 51 males (62%). Patients with SCA compared with the control group had significantly lower hemoglobin, body mass index, cortisol, vitamin D3, testosterone, and T4. Furthermore, there were significantly high levels of reticulocyte count, PTH, TSH, ferritin, LDH, ALP, and uric acid. The incidence of subclinical hypothyroidism and adrenal insufficiency was 7% and 4.8%, respectively, with hypogonadism 9.8% and vitamin D3 deficiency 61%. There were inverse relationships between ferritin as independent variable and serum levels of testosterone, T4, and cortisol

Factors associated with malnutrition in adolescent and adult patients with cystic fibrosis.

PubMed

Barni, Gabriela Cristofoli; Forte, Gabriele Carra; Forgiarini, Luis Felipe; Abrahão, Claudine Lacerda de Oliveira; Dalcin, Paulo de Tarso Roth

2017-01-01

To determine the prevalence of malnutrition in patients attending an adult cystic fibrosis (CF) program and to investigate the associations of malnutrition with the clinical characteristics of those patients. This was a cross-sectional study involving patients with clinically stable CF patients (16 years of age or older). The patients underwent clinical assessment, nutritional assessments, pulmonary function tests, and pancreatic function assessment. They also completed a questionnaire regarding diet compliance. On the basis of their nutritional status, the patients were classified divided into three groups: adequate nutrition; at nutritional risk; and malnutrition. The study has included 73 patients (mean age, 25.6 ± 7.3 years), 40 of whom (54.8%) were female. The mean body mass index was 21.0 ± 3.0 kg/m2 and the mean FEV1 was 59.7 ± 30.6% of predicted. In this sample of patients, 32 (43.8%), 23 (31.5%), and 18 (24.7%) of the patients were allocated to the adequate nutrition, nutritional risk, and malnutrition groups, respectively. The logistic regression analysis identified three independent factors associated with the risk of malnutrition: Shwachman-Kulczycki score, percent predicted FEV1; and age. Malnutrition remains a common complication in adolescents and adults with CF, despite dietary advice. Malnutrition is associated with age, clinical severity, and lung function impairment.

[Types of Care for Adult Patients Diagnosed With Acute and Maintenance Phase Schizophrenia].

PubMed

Bohórquez Peñaranda, Adriana; Gómez Restrepo, Carlos; Oviedo Lugo, Gabriel Fernando; de la Hoz Bradford, Ana María; Castro Díaz, Sergio Mario; García Valencia, Jenny; Jaramillo González, Luis Eduardo; Ávila-Guerra, Mauricio

2014-01-01

To assist the clinician in making decisions about the types of care available for adults with schizophrenia. To determine which are the modalities of treatment associated with better outcomes in adults with schizophrenia. A clinical practice guideline was elaborated under the parameters of the Methodological Guide of the Ministerio de Salud y Protección Social to identify, synthesize and evaluate the evidence and make recommendations about the treatment and follow-up of adult patients with schizophrenia. The evidence of NICE guide 82 was adopted and updated. The evidence was presented to the Guideline Developing Group and recommendations, employing the GRADE system, were produced. Patients who were in Assertive community treatment had a lower risk of new hospitalizations. For the intensive case management, the results favored this intervention in the outcomes: medium term readmissions, social functioning and satisfaction with services. The crisis resolution teams was associated with better outcomes on outcomes of readmissions, social functioning and service satisfaction in comparison with standard care. The use of different modalities of care leads to the need of a comprehensive approach to patients to reduce the overall disability associated with the disease. Evidence shows overall benefit for most outcomes studied without encountering hazards for health of patients. This evaluation is recommended to use the professional ways of providing health services that are community-based and have a multidisciplinary group. It is not recommended the modality "day hospital" during the acute phase of schizophrenia in adults. Copyright © 2014 Asociación Colombiana de Psiquiatría. Publicado por Elsevier España. All rights reserved.

Autoantibody Profiling in a Cohort of Pediatric and Adult Patients With Autoimmune Hepatitis.

PubMed

Villalta, Danilo; Girolami, Elia; Alessio, Maria Grazia; Sorrentino, Maria Concetta; Tampoia, Marilina; Brusca, Ignazio; Daves, Massimo; Porcelli, Brunetta; Barberio, Giuseppina; Conte, Mariaelisabetta; Pantarotto, Lisa; Bizzaro, Nicola

2016-01-01

Autoimmune hepatitis (AIH) is a rare condition characterized by the presence of autoantibodies distinctive of type 1 AIH (AIH-1) and type 2 AIH (AIH-2). The aim of this study was to evaluate the autoantibody profile in a cohort of pediatric and adult AIH patients, using both indirect immunofluorescence (IIF) and a new multiplexed line-blot assay. Sera from 63 pediatric and 53 adult AIH patients were tested for antinuclear (ANA), antismooth muscle (SMA), anti-liver kidney microsome 1 (anti-LKM1), anti-liver cytosol 1 (anti-LC1) autoantibodies using IIF methods; for anti-LKM1, anti-LC1, and soluble liver antigen/liver-pancreas (anti-SLA/LP) autoantibodies using the line-blot; for anti-F-actin autoantibodies using IIF both on VSM47 cell-line and on rat intestinal epithelial cells. AIH-1 was the most common type of AIH in the adult cohort (73.6%), while AIH-2 was the most common AIH in the pediatric cohort (61.9%). Both in adult and pediatric AIH-2 anti-LKM1 were the prevalent autoantibodies. In pediatric AIH-2 anti-LC1 autoantibodies were more frequent than in adult AIH-2 (59 vs. 28.6%), and in 35.9% of cases they were present alone. In 17 patients anti-LC1 autoantibodies were detected only with the line-blot assay. The levels of anti-LKM1 and of anti-LC1 were not different between adult and pediatric AIH, and the overall agreement between the results obtained with the two IIF methods for F-actin detection was 98.8% (CI 95%: 94.4-99.7%). The line-blot assay showed a higher sensitivity than IIF for anti-LC1 detection. Anti-LKM1 and anti-LC1 autoantibody levels are not different in adults and children. An almost perfect agreement between the two IIF methods for anti-F-actin detection has been observed. © 2014 Wiley Periodicals, Inc.

Severe malnutrition evaluated by patient-generated subjective global assessment results in poor outcome among adult patients with acute leukemia

PubMed Central

Li, Ji; Wang, Chang; Liu, Xiaoliang; Liu, Qiuju; Lin, Hai; Liu, Chunshui; Jin, Fengyan; Yang, Yan; Bai, Ou; Tan, Yehui; Gao, Sujun; Li, Wei

2018-01-01

Abstract To evaluate nutritional status in adult patients with acute leukemia (AL) using patient-generated subjective global assessment (PG-SGA) and to investigate the influence of nutritional status on prognosis. We observationally investigated 68 adult patients with newly diagnosed AL who received PG-SGA at the First Hospital of Jilin University between May 2013 and July 2015. Clinical features, chemotherapy regimens, biochemical indexes, body composition, complete remission (CR) rate, minimal residual disease (MRD), survival time, and side-effects of chemotherapy were compared between patients with and without severe malnutrition. Mean PG-SGA scores of the total patients were 6.1 ± 4.0, and 19 of 68 (27.9%) patients had severe malnutrition (PG-SGA score ≥9). Patients with acute myeloid leukemia (AML) had higher scores than those with acute lymphocytic leukemia (ALL; P = .011) and high-risk patients had higher scores regardless of whether they had AML or ALL (AML, P = .012; ALL, P = .043). Univariate analysis showed that severe malnutrition was correlated with age (P = .041), transferrin (P = .042), Karnofsky Performance Status score (P = .006), and C-reactive protein (CRP) (P = .018). Multivariate analysis demonstrated that severe malnutrition was associated with CRP (hazard ratio [HR] = 1.020, 95% confidence interval [CI]: 1.002–1.039, P = .026). No difference was found in CR rate (P = .831) between patients with and without malnutrition, but those who were severely malnourished had higher MRD (P = .048 in AML patients, P = .036 in ALL patients) and more gastrointestinal side-effects (P = .014). Severe malnutrition was also associated with inferior overall survival (HR = 0.243, 95% CI: 0.063–0.945, P = .041) but not with event-free survival (HR = 0.808, 95% CI: 0.338–1.934, P = .663). Severe malnutrition defined by PG-SGA in adult patients with de novo AL may result in poor outcome

Dental infections increase the likelihood of hospital admissions among adult patients with sickle cell disease

PubMed Central

Laurence, B.; Haywood, C; Lanzkron, S.

2014-01-01

The objective To determine if dental infections increase the likelihood of hospital admission among adult patients with sickle cell disease (SCD). Basic Research Design Cross-sectional analysis of data from the Nationwide Emergency Department Sample (NEDS) pooled for the years 2006 through 2008. Prevalence ratios (PR) for the effects of interest were estimated using Poisson regression with robust estimates of the variance. Participants Adults, aged 18 and over, diagnosed with SCD using ICD-9-CM codes excluding participants discharged with a code for sickle cell trait. Main outcome measure Emergency department (ED) visit disposition, dichotomised to represent whether or not the ED visit ended in admission versus being treated and released. Results Among patients having a sickle cell crisis, those with dental infections were 72% more likely to be admitted compared to those not having dental infections (PR=1.72, 95%CI 1.58-1.87). No association was observed among adult SCD patients not having a sickle crisis event. Based on preliminary data from this analysis, prevention of dental infection among patients with SCD could result in an estimated cost saving of $2.5 million dollars per year. Conclusions Having a dental infection complicated by a sickle cell crisis significantly increases the likelihood of hospital admission among adult SCD patients presenting to the ED. PMID:24151791

Outcomes of cardiac pacing in adult patients after a Fontan operation.

PubMed

Egbe, Alexander C; Huntley, Geoffery D; Connolly, Heidi M; Ammash, Naser M; Deshmukh, Abhishek J; Khan, Arooj R; Said, Sameh M; Akintoye, Emmanuel; Warnes, Carole A; Kapa, Suraj

2017-12-01

Cardiac pacing can be challenging after a Fontan operation, and limited data exist regarding pacing in adult Fontan patients. The objectives of our study were to determine risk factors for pacing and occurrence of device-related complications (DRCs) and pacemaker reinterventions. We performed a retrospective review of Fontan patients from 1994 through 2014. We defined DRCs as lead failure, lead recall, cardiac perforation, lead thrombus/vegetation, or device-related infection, and cardiovascular adverse events (CAEs) as venous thrombosis, stroke, death, or heart transplant. Pacemaker reintervention was defined as lead failure or recall. Of 439 patients, 166 (38%) had pacemakers implanted (79 during childhood; 87, adulthood); 114 patients (69%) received epicardial leads initially, and 52 (31%), endocardial leads. Pacing was initially atrial in 52 patients (31%); ventricular, 30 (18%); or dual chamber, 84 (51%). There were 37 reinterventions (1.9% per year) and 48 DRCs (2.4% per year). Pacemaker implantation during childhood was a risk factor for DRCs (hazard ratio, 2.01 [CI, 1.22-5.63]; P = .03). There were 70 CAEs (venous thrombosis, 5; stroke, 11; transplant, 8; and death, 46), yielding a rate of 3.5% per year. DRCs, CAEs, and reintervention rates were comparable for patients with epicardial or endocardial leads. More than one-third of adult Fontan patients referred to Mayo Clinic had pacemaker implantation. Epicardial leads were associated with high rate of pacemaker reinterventions but similar DRC rates in comparison to endocardial leads. Copyright © 2017 Elsevier Inc. All rights reserved.

Reduction of L-phenylalanine in protein hydrolysates using L-phenylalanine ammonia-lyase from Rhodosporidium toruloides.

PubMed

Castañeda, María Teresita; Adachi, Osao; Hours, Roque Alberto

2015-10-01

L-Phenylalanine ammonia-lyase (PAL, EC 4.3.1.25) from Rhodosporidium toruloides was utilized to remove L-phenylalanine (L-Phe) from different commercial protein hydrolysates. A casein acid hydrolysate (CAH, L-Phe ~2.28 %) was employed as a model substrate. t-Cinnamic acid resulting from deamination of L-Phe was extracted, analyzed at λ = 290 nm, and used for PAL activity determination. Optimum reaction conditions, optimized using successive Doehlert design, were 35 mg mL(-1) of CAH and 800 mU mL(-1) of PAL, while temperature and pH were 42 °C and 8.7, respectively. Reaction kinetics of PAL with CAH was determined under optimized conditions. Then, removal of L-Phe from CAH was tested. Results showed that more than 92 % of initial L-Phe was eliminated. Similar results were obtained with other protein hydrolysates. These findings demonstrate that PAL is a useful biocatalyst for L-Phe removal from protein hydrolysates, which can be evaluated as potential ingredients in foodstuffs for PKU patients.

A comparison study of pelvic fractures and associated abdominal injuries between pediatric and adult blunt trauma patients.

PubMed

Swaid, Forat; Peleg, Kobi; Alfici, Ricardo; Olsha, Oded; Givon, Adi; Kessel, Boris

2017-03-01

Pelvic fractures are a marker of severe injury, mandating a thorough investigation for the presence of associated injuries. Anatomical and physiological differences between adults and children may lead to a different impact of pelvic fractures on these populations. The purpose of this study is to compare pelvic fractures between pediatric and adult blunt trauma victims, mainly regarding their severity and associated intraabdominal injuries. A retrospective study involving blunt trauma patients suffering pelvic fractures, according to the records of the Israeli National Trauma Registry. Patients included children, aged 0-14years, and adults between 15 and 64years. The presence and severity of associated injuries were assessed. Overall, 7621 patients aged 0-64years were identified with pelvic fractures following blunt trauma. The incidence of pelvic fractures in children was (0.8%), as compared to 4.3% in adults, p <0.0001. The most common mechanism of injury was motor vehicle accident (MVA) in adults, and pedestrian hit by car (PHBC) in children. About a quarter of the patients in both groups had an ISS >25. Adults sustained significantly more moderate to severe pelvic fractures (AIS≥3) than children (26.7% vs. 17.4%, p<0.0001). The overall mortality rate was similar among the two groups (5.4% in adults, 5.2% in children, p=0.7554). The only associated injury with statistically significant difference in incidence among the two groups was rectal injury (1.2% among children, 0.2% among adults, p<0.0001). Among adult patients, there was a clear correlation between the severity of pelvic fractures and the severity of concomitant splenic and hepatic injuries (p=0.026, p=0.0004, respectively). Among children, a similar correlation was not demonstrated. Adults involved in blunt trauma are more likely to sustain pelvic fractures, and these are generally more severe fractures, as compared to children suffering from blunt trauma. Nonetheless, mortality rates were found

Falling and fall risk in adult patients with severe haemophilia.

PubMed

Rehm, Hanna; Schmolders, Jan; Koob, Sebastian; Bornemann, Rahel; Goldmann, Georg; Oldenburg, Johannes; Pennekamp, Peter; Strauss, Andreas C

2017-05-10

The objective of this study was to define fall rates and to identify possible fall risk factors in adult patients with severe haemophilia. 147 patients with severe haemophilia A and B were evaluated using a standardized test battery consisting of demographic, medical and clinical variables and fall evaluation. 41 (27.9 %) patients reported a fall in the past 12 months, 22 (53.7 %) of them more than once. Young age, subjective gait insecurity and a higher number of artificial joints seem to be risk factors for falling. Falls seem to be a common phenomenon in patients with severe haemophilia. Fall risk screening and fall prevention should be implemented into daily practice.

Body height and weight of patients with childhood onset and adult onset thyrotoxicosis.

PubMed

Takamatsu, J; Kobe, N; Ito, M; Ohsawa, N

1999-03-01

The present study has compared body height and weight of thyrotoxic female patients of childhood onset and adult onset. The body height of 141 out of 143 (99%) adult-onset thyrotoxic patients was within the range of mean +/- 2SD for the age-matched general Japanese female population. On the other hand, in 42 patients with childhood-onset thyrotoxicosis, 6 (14%) had their height being greater than the mean + 2SD of general population, and 34 (81%) were taller than the mean value. In 86 patients with siblings, 42 (49%) were at least 2 cm taller than their sisters, and 26 (30%) were more than 2 cm shorter than their sisters. The body weight of 27 out of 42 (68%) patients younger than 20 years was not decreased but was even greater than the mean value for the age-matched general population. The results indicate that excessive thyroid hormone in vivo enhances body height in humans. The increased body weight in some young patients suggests that enhanced dietary intake due to increased appetite in hyperthyroidism has overcome the energy loss with increased metabolism.

Deletions of fetal and adult muscle cDNA in Duchenne and Becker muscular dystrophy patients.

PubMed Central

Cross, G S; Speer, A; Rosenthal, A; Forrest, S M; Smith, T J; Edwards, Y; Flint, T; Hill, D; Davies, K E

1987-01-01

We have isolated a cDNA molecule from a human adult muscle cDNA library which is deleted in several Duchenne muscular dystrophy patients. Patient deletions have been used to map the exons across the Xp21 region of the short arm of the X chromosome. We demonstrate that a very mildly affected 61 year old patient is deleted for at least nine exons of the adult cDNA. We find no evidence for differential exon usage between adult and fetal muscle in this region of the gene. There must therefore be less essential domains of the protein structure which can be removed without complete loss of function. The sequence of 2.0 kb of the adult cDNA shows no homology to any previously described protein listed in the data banks although sequence comparison at the amino acid level suggests that the protein has a structure not dissimilar to rod structures of cytoskeletal proteins such as lamin and myosin. There are single nucleotide differences in the DNA sequence between the adult and fetal cDNAs which result in amino acid changes but none that would be predicted to change the structure of the protein dramatically. Images Fig. 1. Fig. 2. Fig. 3. Fig. 4. Fig. 5. Fig. 7. PMID:3428261

Final adult height in long-term growth hormone-treated achondroplasia patients.

PubMed

Harada, Daisuke; Namba, Noriyuki; Hanioka, Yuki; Ueyama, Kaoru; Sakamoto, Natsuko; Nakano, Yukako; Izui, Masafumi; Nagamatsu, Yuiko; Kashiwagi, Hiroko; Yamamuro, Miho; Ishiura, Yoshihito; Ogitani, Ayako; Seino, Yoshiki

2017-07-01

The objective of this study was to evaluate the gain in final height of achondroplasia (ACH) patients with long-term growth hormone (GH) treatment. We analyzed medical data of 22 adult patients (8 males and 14 females) treated with GH at a dose of 0.05 mg/kg/day. Optionally, tibial lengthening (TL) was performed with the Ilizalov method in 15 patients and TL as well as femoral lengthening (FL) in 6 patients. Concomitant gonadal suppression therapy with buserelin acetate was applied in 13 patients. The mean treatment periods with GH were 10.7 ± 4.0 and 9.3 ± 2.5 years for males and females, respectively. GH treatment augmented the final height +0.60 ± 0.52 SD (+3.5 cm) and +0.51 ± 1.29 SD (+2.8 cm) in males and females compared to non-treated ACH patients, respectively. Final height of ACH patients that underwent GH and TL increased +1.72 ± 0.72 SD (+10.0 cm) and +1.95 ± 1.34 SD (+9.8 cm) in males and females, respectively. GH, TL, and FL increased their final height +2.97 SD (+17.2 cm) and +3.41 ± 1.63 SD (+17.3 cm) in males and females, respectively. Gonadal suppression therapy had no impact on final height. Long-term GH treatment contributes to 2.6 and 2.1% of final adult height in male and female ACH patients, respectively.

The Need to Consider Longer-term Outcomes of Care: Racial/Ethnic Disparities Among Adult and Older Adult Emergency General Surgery Patients at 30, 90, and 180 Days.

PubMed

Zogg, Cheryl K; Olufajo, Olubode A; Jiang, Wei; Bystricky, Anna; Scott, John W; Shafi, Shahid; Havens, Joaquim M; Salim, Ali; Schoenfeld, Andrew J; Haider, Adil H

2017-07-01

Following calls from the National Institutes of Health and American College of Surgeons for "urgently needed" research, the objectives of the present study were to (1) ascertain whether differences in 30/90/180-day mortality, major morbidity, and unplanned readmissions exist among adult (18-64 yr) and older adult (≥65 yr) emergency general surgery (EGS) patients; (2) vary by diagnostic category; and (3) are explained by variations in insurance, income, teaching status, hospital EGS volume, and a hospital's proportion of minority patients. Racial/ethnic disparities have been described in in-hospital and 30-day settings. How longer-term outcomes compare-a critical consideration for the lived experience of patients-has, however, only been limitedly considered. Survival analysis of 2007 to 2011 California State Inpatient Database using Cox proportional hazards models. A total of 737,092 adults and 552,845 older adults were included. In both cohorts, significant differences in 30/90/180-day mortality, major morbidity, and unplanned readmissions were found, pointing to persistently worse outcomes between non-Hispanic Black and White patients [180-d readmission hazard ratio (95% confidence interval):1.04 (1.03-1.06)] and paradoxically better outcomes among Hispanic adults [0.85 (0.84-0.86)] that were not encountered among Hispanic older adults [1.06 (1.04-1.07)]. Stratified results demonstrated robust morbidity and readmission trends between non-Hispanic Black and White patients for the majority of diagnostic categories, whereas variations in insurance/income/teaching status/EGS volume/proportion of minority patients all significantly altered the effect-combined accounting for up to 80% of risk-adjusted differences between racial/ethnic groups. Racial/ethnic disparities exist in longer-term outcomes of EGS patients and are, in part, determined by differences in factors associated with emergency care. Efforts such as these are needed to understand the interplay of

Benefits of a transfer clinic in adolescent and young adult kidney transplant patients.

PubMed

McQuillan, Rory F; Toulany, Alene; Kaufman, Miriam; Schiff, Jeffrey R

2015-01-01

Adolescent and young adult kidney transplant recipients have worse graft outcomes than older and younger age groups. Difficulties in the process of transition, defined as the purposeful, planned movement of adolescents with chronic health conditions from child to adult-centered health care systems, may contribute to this. Improving the process of transition may improve adherence post-transfer to adult care services. The purpose of this study is to investigate whether a kidney transplant transfer clinic for adolescent and young adult kidney transplant recipients transitioning from pediatric to adult care improves adherence post-transfer. We developed a joint kidney transplant transfer clinic between a pediatric kidney transplant program, adult kidney transplant program, and adolescent medicine at two academic health centers. The transfer clinic facilitated communication between the adult and pediatric transplant teams, a face-to-face meeting of the patient with the adult team, and a meeting with the adolescent medicine physician. We compared the outcomes of 16 kidney transplant recipients transferred before the clinic was established with 16 patients who attended the clinic. The primary outcome was a composite measure of non-adherence. Non-adherence was defined as either self-reported medication non-adherence or displaying two of the following three characteristics: non-attendance at clinic, non-attendance for blood work appointments, or undetectable calcineurin inhibitor levels within 1 year post-transfer. The two groups were similar at baseline, with non-adherence identified in 43.75 % of patients. Non-adherent behavior in the year post-transfer, which included missing clinic visits, missing regular blood tests, and undetectable calcineurin inhibitor levels, was significantly lower in the cohort which attended the transfer clinic (18.8 versus 62.5 %, p = 0.03). The median change in estimated glomerular filtration rate (eGFR) in the year following transfer

Post-traumatic stress disorder symptoms in family caregivers of adult patients with acute leukemia from a dyadic perspective.

PubMed

Jia, Mutian; Li, Jie; Chen, Chunyan; Cao, Fenglin

2015-12-01

Acute leukemia is a fatal disease in adults that not only affects the patients who suffer from it but also their family caregivers. No studies have investigated post-traumatic stress disorder symptoms (PTSS) in family caregivers of adult patients with acute leukemia using a matched sample. The current study examined PTSS in adult patients with acute leukemia and their family caregivers and investigated the factors associated with caregivers' PTSS. A total of 163 patient-caregiver dyads completed questionnaires assessing their PTSS, psychological resilience, and perceived social support. Hierarchical linear regression was used to explore the related factors of caregivers' PTSS. More caregivers than patients met caseness criteria for PTSS (36.8% vs. 18.4%, p < 0.001). Among caregivers, being more closely related to the patients (e.g., spouses and parents), having patients with higher PTSS and having lower psychological resilience were independently associated with more severe PTSS. Caregivers of acute leukemia patients had significantly more severe PTSS than did their patients. This study is the first to investigate PTSS among family caregivers of adult patients with acute leukemia and its related factors in a matched sample. More attention should be paid to the caregivers of patients with acute leukemia to minimize their PTSS and thus improve mental health of caregivers and reduce potential negative consequences for the patients themselves. Copyright © 2015 John Wiley & Sons, Ltd.

Intradetrusor injections of botulinum toxin A in adult patients with spinal dysraphism.

PubMed

Peyronnet, Benoit; Even, Alexia; Capon, Grégoire; de Seze, Marianne; Hascoet, Juliette; Biardeau, Xavier; Baron, Maximilien; Perrouin-Verbe, Marie-Aimée; Boutin, Jean-Michel; Saussine, Christian; Phé, Véronique; Lenormand, Loic; Chartier-Kastler, Emmanuel; Cornu, Jean-Nicolas; Karsenty, Gilles; Manunta, Andrea; Schurch, Brigitte; Denys, Pierre; Amarenco, Gérard; Game, Xavier

2018-05-07

The aim of the present study was to report the outcomes of botulinum toxin A (BTX-A) intradetrusor injections in adult patients with spina bifida. All patients with spinal dysraphism who had undergone intradetrusor injections of BTX-A from 2002 to 2016 in 14 centers were included retrospectively. The primary endpoint was the global success of injections, defined subjectively as the combination of urgency, urinary incontinence and detrusor overactivity/low bladder compliance resolution. Univariate and multivariate analysis were performed to seek for predictors of global success. 125 patients were included with a global success rate of the first injection was 62.3% with resolution of urinary incontinence in 73.5% of patients. All urodynamic parameters improved significantly at 6-8 weeks compared to baseline including maximum detrusor pressure (-12 cmH2O; p<0.001), maximum cystometric capacity (+86.6 ml ; p<0.001) and compliance (+8.9 ml/cmH2O ; p=0.002). Out of 561 intradetrusor BTX-A injections, 20 complications were recorded (3.6%) with three muscular weaknesses. Global success rate of the first injection was significantly lower in case of poor compliance (34.4% vs. 86.9%; OR=0.08; p<0.001). In multivariate analysis, poor compliance was associated with lower global success rate (OR=0.13; p<0.001) and female gender (OR=3.53; p=0.01) and age (OR=39.9; p<0.001) were predictors of global success. Intradetrusor BTX-A injections were effective in adult spina bifida patients exhibiting detrusor overactivity. In contrast, the effectiveness was much lower in adult spina bifida patients with poor bladder compliance. The other predictors of global success were female gender and older age. Copyright © 2018 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.

Loss of olfactory function and nutritional status in vital older adults and geriatric patients.

PubMed

Toussaint, Nicole; de Roon, Margot; van Campen, Jos P C M; Kremer, Stefanie; Boesveldt, Sanne

2015-03-01

The aim of this cross-sectional study was to assess the association of olfactory function and nutritional status in vital older adults and geriatric patients. Three hundred forty-five vital (mean age 67.1 years) and 138 geriatric older adults (mean age 80.9 years) were included. Nutritional status was assessed using the mini nutritional assessment-short form. The Sniffin' Sticks was used to measure olfactory function. Eleven percentage of the vital older adults were at risk of malnutrition, whereas 60% of the geriatric participants were malnourished or at risk. Only 2% of the vital older adults were anosmic, compared with 46% of the geriatric participants. Linear regression demonstrated a significant association (P = 0.015) between olfactory function and nutritional status in the geriatric subjects. However, this association became insignificant after adjustment for confounders. Both crude and adjusted analysis in the vital older adults did not show a significant association. The results indicate that, in both groups of elderly, there is no direct relation between olfactory function and nutritional status. We suggest that a decline in olfactory function may still be considered as one of the risk-factors for malnutrition in geriatric patients-once co-occurring with other mental and/or physical problems that are more likely to occur in those patients experience. © The Author 2015. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

Parental substance abuse, reports of chronic pain and coping in adult patients with sickle cell disease.

PubMed Central

Edwards, Christopher; Whitfield, Keith; Sudhakar, Shiv; Pearce, Michele; Byrd, Goldie; Wood, Mary; Feliu, Miriam; Leach-Beale, Brittani; DeCastro, Laura; Whitworth, Elaine; Abrams, Mary; Jonassaint, Jude; Harrison, M. Ojinga; Mathis, Markece; Scott, Lydia; Johnson, Stephanie; Durant, Lauren; Holmes, Anita; Presnell, Katherine; Bennett, Gary; Shelby, Rebecca; Robinson, Elwood

2006-01-01

There is increasing interest from a social learning perspective in understanding the role of parental factors on adult health behaviors and health outcomes. Our review revealed no studies, to date, that have evaluated the effects of parental substance abuse on reports of chronic pain and coping in adult patients with sickle cell disease (SCD). We explored the effects of parental substance (alcohol or drug) abuse on reports of the sensory, affective and summary indices of pain in 67 adult patients, mean age 38.9 (13.5), with SCD. We also explored the effects of parental substance abuse on psychopathology associated with pain and active coping. Twenty-four percent of patients reported that their parent(s) abused substances. Patients whose parent(s) were characterized as substance abusers reported greater sensory (p=0.02), affective (p=0.01) and summary (VAS; p=0.02) indices of pain as compared to their counterparts, whose parent(s) were not characterized as substance abusers. Patients did not differ in average age, education or the propensity to respond in a socially acceptable manner. There was a significant trend towards patients who characterized their parents as abusers scoring higher than their counterparts on active coping. We propose a Social Learning Theory to explain the current findings and suggest a need for additional prospective research to simultaneously explore biological (genetic) and social factors that influence the interpretation, experience and reporting of chronic pain in adult patients with chronic disease. PMID:16573309

Application of the WHOQOL-100 for the assessment of quality of life of adult patients with inherited metabolic diseases.

PubMed

Cazzorla, Chiara; Del Rizzo, Monica; Burgard, Peter; Zanco, Chiara; Bordugo, Andrea; Burlina, Alberto B; Burlina, Alessandro P

2012-05-01

As advances in neonatal and pediatric care for patients affected by inherited metabolic diseases (IMD) improve their outcome and allow for better survival rates, there is a growing interest in the quality of life (QoL) of patients reaching adulthood. In order to address this subject we designed a study to evaluate the QoL of a group of adult IMD patients who are receiving various treatments, in a comprehensive manner. A mixed-method study was conducted to assess the QoL in adult IMD patients. The multidimensional World Health Organization Quality of Life questionnaire (WHOQOL-100) was applied for quantitative evaluations, and an additional semi-standardized interview, was conducted for qualitative measurement of patients' perceptions of the impact of illness on their daily life, and the perceived adherence to their treatment recommendations. A total of 82 patients affected by IMD were enrolled. The inherited metabolic disorders included principally amino acids disorders, urea cycle defects, organic acidurias, carbohydrates disorders, and lysosomal disorders. The WHOQOL-100 and the semi-standardized interview were administered in a clinical setting to adult patients with IMD. The mean for the whole group indicates that adult patients with IMD can have a normal value of General QoL. Despite this value, the results of each domain show lower scores in the domains of perception of independence and quality of social relationships. We made a further analysis to compare the patients with dietary treatment with the patients with pharmacological treatment, and we observed a statistically significant difference in General QoL, in the Physical, Independence, Spiritual domains and in the facet of Medication. These results suggest that Global QoL measures might not be sufficient to assess the QoL for adult patients with IMD. Furthermore, the implementation of a qualitative semi-standardized interview, especially suitable for adult patients, added important features on illness

Successful treatment of heart failure in an adult patient with Prader-Willi syndrome.

PubMed

Kawano, Hiroaki; Ikeda, Tooru; Shimazaki, Koichi; Arakawa, Shuji; Matsumoto, Yuji; Hayano, Motonobu; Maemura, Koji

2013-01-01

Prader-Willi Syndrome (PWS) is a rare genetic disorder characterized by physical, psychological and physiological abnormalities. Obesity and related cardiovascular diseases are a common problem in adult patients with PWS. This report describes a case of adult PWS with heart failure associated with marked obesity and sleep-disordered breathing that was successfully treated with oxygen therapy, adaptive servoventilation, medications, diet therapy and rehabilitation.

Clinical evaluation for sublingual immunotherapy with Dermatophagoides farinae drops in adult patients with allergic asthma.

PubMed

Zhong, C; Yang, W; Li, Y; Zou, L; Deng, Z; Liu, M; Huang, X

2018-05-01

The efficacy and safety of sublingual immunotherapy (SLIT) in house dust mite-induced allergic asthma (AA) have yet to be firmly established, especially in adult patients. Our objective is to evaluate the efficacy of SLIT with Dermatophagoides farinae drops in adult patients with AA. One hundred and thirty-four adult patient data with house dust mite (HDM)-induced AA who had been treated for 2 years were collected. These patient data that we collected were divided into the SLIT group (n = 85) and control group (n = 49). All patients were treated with low to moderate dose of inhaled glucocorticoid and long-acting β2 agonists. Patients in the SLIT group were further treated with D. farinae drops. Clinical scores including the total asthma symptom score (TASS), total asthma medicine score (TAMS), asthma control test (ACT), and peak flow percentage (PEF%) were assessed before treatment and at yearly visits. The presence of adverse events (AEs) were recorded once a month. Before treatment, the PEF% in the SLIT group was significantly lower than that in the control group (p < 0.05). After 2 years, both treatments were effective in the clinical scores when compared with baseline values (all p < 0.001). Meanwhile, the SLIT group showed significantly lower TASS and TAMS (all p < 0.001) and higher ACT (p < 0.001) and PEF% (p < 0.05) when compared with the control group. No severe systemic AEs were reported. SLIT with D. farinae drops plus pharmacotherapy is more effective than routine drug treatment in adult patients with AA.

Self-efficacy as a predictor of patient-reported outcomes in adults with congenital heart disease.

PubMed

Thomet, Corina; Moons, Philip; Schwerzmann, Markus; Apers, Silke; Luyckx, Koen; Oechslin, Erwin N; Kovacs, Adrienne H

2018-04-01

Self-efficacy is a known predictor of patient-reported outcomes in individuals with acquired diseases. With an overall objective of better understanding patient-reported outcomes in adults with congenital heart disease, this study aimed to: (i) assess self-efficacy in adults with congenital heart disease, (ii) explore potential demographic and medical correlates of self-efficacy and (iii) determine whether self-efficacy explains additional variance in patient-reported outcomes above and beyond known predictors. As part of a large cross-sectional international multi-site study (APPROACH-IS), we enrolled 454 adults (median age 32 years, range: 18-81) with congenital heart disease in two tertiary care centres in Canada and Switzerland. Self-efficacy was measured using the General Self-Efficacy (GSE) scale, which produces a total score ranging from 10 to 40. Variance in the following patient-reported outcomes was assessed: perceived health status, psychological functioning, health behaviours and quality of life. Hierarchical multivariable linear regression analysis was performed. Patients' mean GSE score was 30.1 ± 3.3 (range: 10-40). Lower GSE was associated with female sex ( p = 0.025), not having a job ( p = 0.001) and poorer functional class ( p = 0.048). GSE positively predicted health status and quality of life, and negatively predicted symptoms of anxiety and depression, with an additional explained variance up to 13.6%. No associations between self-efficacy and health behaviours were found. GSE adds considerably to our understanding of patient-reported outcomes in adults with congenital heart disease. Given that self-efficacy is a modifiable psychosocial factor, it may be an important focus for interventions targeting congenital heart disease patients' well-being.

Sub-dissociative-dose intranasal ketamine for moderate to severe pain in adult emergency department patients.

PubMed

Yeaman, Fiona; Meek, Robert; Egerton-Warburton, Diana; Rosengarten, Pamela; Graudins, Andis

2014-06-01

There are currently no studies assessing effectiveness of sub-dissociative intranasal (IN) ketamine as the initial analgesic for adult patients in the ED. The study aims to examine the effectiveness of sub-dissociative IN ketamine as a primary analgesic agent for adult patients in the ED. This is a prospective, observational study of adult ED patients presenting with severe pain (≥6 on 11-point scale at triage). IN ketamine dose was 0.7 mg/kg, with secondary dose of 0.5 mg/kg at 15 min if pain did not improve. After 6 months, initial dose was increased to 1.0 mg/kg with the same optional secondary dose. The primary outcomes are change in VAS rating at 30 min; percentage of patients reporting clinically significant reduction in VAS (≥20 mm) at 30 min; dose resulting in clinically significant pain reduction. Of the 72 patients available for analysis, median age was 34.5 years and 64% were men. Median initial VAS rating was 76 mm (interquartile range [IQR]: 65-82). Median total dose of IN ketamine for all patients was 0.98 mg/kg (IQR: 0.75-1.15, range: 0.59-1.57). Median reduction in VAS rating at 30 min was 24 mm (IQR: 2-45). Forty (56%, 95% CI: 44.0-66.7) reported VAS reduction ≥20 mm, these patients having had a total median ketamine dose of 0.94 mg/kg (IQR: 0.72-1.04). IN ketamine, at a dose of about 1 mg/kg, was an effective analgesic agent in 56% of study patients. The place of IN ketamine in analgesic guidelines for adults requires further investigation. © 2014 Australasian College for Emergency Medicine and Australasian Society for Emergency Medicine.

Social independence of adult congenital heart disease patients in Japan.

PubMed

Ochiai, Ryota; Ikeda, Yukitaka; Kato, Hitoshi; Shiraishi, Isao

2017-06-01

As treatment outcomes for congenital heart disease (CHD) have improved, the social independence of adult CHD patients has become a key goal. The aims of this study were therefore to (i) determine the relationship between social independence and psychological profile, and (ii) identify patient anxieties, difficulties, and demands related to life in society. A total of 143 patients aged ≥15 years with physical disability certificates were selected using a questionnaire distributed by a patients' association. Each participant was asked about employment status, income, and receipt of disability pension as a social independence index, and about financial and psychological distress as a psychological status index. Furthermore, each participant was asked to freely describe his or her difficulties, anxieties, and needs pertaining to life in society. The subjects were 15-73 years old. Seventy-one (50%) were female, and 94 (66%) had a grade 1 physical disability certificate. Fifty-nine subjects (41%) were employed, 37 (26%) were unemployed, and 45 (31%) were students. Of those employed, 34 subjects (58%) reported annual individual income ≤2 million yen. Frequent hospital visits, low total household income, low individual annual income, work dissatisfaction, and receipt of a disability pension were associated with poorer psychological profile. In an open description section, subjects expressed desires for better pension systems, support for medical fees, and employment support. Because financial issues can adversely affect the psychological profiles of adult CHD patients, enhancement of social welfare and employment support may improve their social independence. © 2017 Japan Pediatric Society.

Towards a new conceptualization of depression in older adult cancer patients: a review of the literature

PubMed Central

Saracino, Rebecca M.; Rosenfeld, Barry; Nelson, Christian J.

2016-01-01

Objectives Identifying depression in older adults with cancer presents a set of unique challenges, as it combines the confounding influences of cancer and its treatment with the developmental changes associated with aging. This paper reviews the phenomenology of depression in older adults, and individuals diagnosed with cancer. Method PsychInfo, PubMed, Web of Science, and Google Scholar databases were searched for English-language studies addressing the phenomenology, symptoms, or assessment of depression in older adults and those with cancer. Results The Diagnostic and Statistical Manual for Mental Disorders (DSM) criteria that appear to be relevant to both older adults and cancer patients are anhedonia, concentration difficulties, sleep disturbances, psychomotor retardation/agitation, and loss of energy. Possible alternative criteria that may be important considerations included constructs such as loss of purpose, loneliness, and irritability in older adults. Among cancer patients, tearfulness, social withdrawal, and not participating in treatment despite ability to do so were identified as potentially important symptoms. Conclusions Current DSM criteria may not adequately assess depression in older cancer patients and alternative criteria may be important to inform the understanding and identification of depression in this population. Enhancing diagnostic accuracy of depression is important as both the over-diagnosis and under-diagnosis is accompanied with significant costs. Thus, continued research exploring the phenomenology and identifying effective indicators of depression in older cancer patients is needed. PMID:26312455

Clinical presentation of metabolic alkalosis in an adult patient with cystic fibrosis.

PubMed

Sweetser, Lisel J; Douglas, James A; Riha, Renata L; Bell, Scott C

2005-03-01

In subtropical and tropical climates, dehydration is common in cystic fibrosis patients with respiratory exacerbations. This may lead to a clinical presentation of metabolic alkalosis with associated hyponatraemia and hypochloraemia. An adult cystic fibrosis patient who presented with a severe respiratory exacerbation accompanied by metabolic alkalosis is presented and the effects of volume correction are reported.

Physician communication behaviors from the perspective of adult HIV patients in Kenya.

PubMed

Wachira, Juddy; Middlestadt, Susan; Reece, Michael; Peng, Chao-Ying Joanne; Braitstein, Paula

2014-04-01

We describe perceived physician communication behaviors and its association with adherence to care, among HIV patients in Kenya. This cross-sectional study was conducted between July and August 2011. The study was conducted in three adult HIV clinics within the Academic Model Providing Healthcare program in western Kenya. HIV adult patients. Patient's predisposition to attend clinic, missed appointment and missed combined antiretroviral therapy (cART) medication. A total of 400 patients were enrolled and participated in the study; the median age was 38 years (IQR = 33-44) and 56.5% were female. Patients perceived physicians engaged in a high number of communication behaviors (mean = 3.80, range 1-5). A higher perceived general health status (P = 0.01), shorter distance to the health facility (P = 0.03) and lesser time spent at the health facility (P = 0.02) were associated with a higher number of perceived physician communication behaviors. Physician-patient relationship factors were not associated with physician communication behaviors. In addition, a higher number of perceived physician communication behaviors was associated with a very high likelihood of patients' attending the next HIV clinic [adjusted odds ratio (AOR): 1.89, 95% confidence interval (CI): 1.49-2.40], a lower likelihood of patients' missing an appointment (AOR: 0.75, 95% CI: 0.61-0.92) and missing cART medication (AOR: 0.68, 95% CI: 0.52-0.87). Patients' perception of physician communication behaviors was found to be associated with their adherence to HIV care.

The process of death imminence awareness by family members of patients in adult critical care.

PubMed

Baumhover, Nancy C

2015-01-01

A focus on cost-effective quality end-of-life care remains a high priority in adult critical care given an aging population, high prevalence of death, and aggressive technologies used to extend or sustain life in this setting. A Glaserian grounded theory design was used to conduct this retrospective study to yield a substantive middle-range theory. The data source was semistructured interviews with 14 family members of decedents who died 6 to 60 months prior to the study. The purpose of this study was to generate a theory on how family members of patients in adult critical care come to realize that their loved one is dying. The Process of Death Imminence Awareness by Family Members of Patients in Adult Critical Care middle-range theory contained 6 phases: (1) patient's near-death awareness, (2) dying right in front of me, (3) turning points in the patient's condition, (4) no longer the person I once knew, (5) doing right by them, and (6) time to let go. Patient's near-death awareness preceded all other phases, if communicated by the decedent with their family. Then, family members iteratively moved through all the other key phases in the process until a time to let go became evident. This substantive middle-range theory will guide nursing education, practice, and research aimed at providing quality and cost-effective end-of-life care in adult critical care.

Association of leptin with cardiometabolic factors in schoolchildren and adolescents with congenital adrenal hyperplasia.

PubMed

Zurita-Cruz, Jessie Nallely; Villasís-Keever, Miguel Ángel; Damasio-Santana, Leticia; Manuel-Apolinar, Leticia; Ferrusca-Ceja, Rosalba; Nishimura-Meguro, Elisa; Rivera-Hernández, Aleida de J; Garrido-Magaña, Eulalia

2018-01-01

In congenital adrenal hyperplasia (CAH), obesity, hyperinsulinemia and leptin levels are increased. To identify the frequency of cardiometabolic risk factors (CRF) in children and adolescents with CAH and to explore the relationship with leptin levels. Cross-sectional study of 40 patients who underwent anthropometric measurements and had fasting glucose, insulin, triglycerides, 17-hidroxyprogesterone, leptin, HDL and LDL-cholesterol assessed. The patients were classified according to the number of CRFs, and leptin levels were analyzed with the Kruskal-Wallis test. Pearson's correlation was applied between leptin, body mass index (BMI) z-score and body fat percentage. Fifty percent of the patients had obesity and overweight, 59% had hypertriglyceridemia, 40%, hypoalphalipoproteinemia, 27.5%, high LDL-cholesterol and 22.5% insulin resistance. There was positive correlation between leptin and body fat percentage (r = 0.64), BMI z-score (r = 0.55) and the number of CRFs (r = 0.65). In the obesity-adjusted multivariate analysis, leptin levels were associated with the number of CRFs. CAH had a high frequency of CRFs and leptin appeared to be associated with a more adverse cardiometabolic profile in subjects with obesity and overweight. Copyright: © 2018 SecretarÍa de Salud.

Autistic-like traits in adult patients with mood disorders and schizophrenia.

PubMed

Matsuo, Junko; Kamio, Yoko; Takahashi, Hidetoshi; Ota, Miho; Teraishi, Toshiya; Hori, Hiroaki; Nagashima, Anna; Takei, Reiko; Higuchi, Teruhiko; Motohashi, Nobutaka; Kunugi, Hiroshi

2015-01-01

Autism spectrum disorder often co-occurs with other psychiatric disorders. Although a high prevalence of autistic-like traits/symptoms has been identified in the pediatric psychiatric population of normal intelligence, there are no reports from adult psychiatric population. This study examined whether there is a greater prevalence of autistic-like traits/symptoms in patients with adult-onset psychiatric disorders such as major depressive disorder (MDD), bipolar disorder, or schizophrenia, and whether such an association is independent of symptom severity. The subjects were 290 adults of normal intelligence between 25 and 59 years of age (MDD, n=125; bipolar disorder, n=56; schizophrenia, n=44; healthy controls, n=65). Autistic-like traits/symptoms were measured using the Social Responsiveness Scale for Adults. Symptom severity was measured using the Positive and Negative Symptoms Scale, the Hamilton Depression Rating Scale, and/or the Young Mania Rating Scale. Almost half of the clinical subjects, except those with remitted MDD, exhibited autistic-like traits/symptoms at levels typical for sub-threshold or threshold autism spectrum disorder. Furthermore, the proportion of psychiatric patients that demonstrated high autistic-like traits/symptoms was significantly greater than that of healthy controls, and not different between that of remitted or unremitted subjects with bipolar disorder or schizophrenia. On the other hand, remitted subjects with MDD did not differ from healthy controls with regard to the prevalence or degree of high autistic-like traits/symptoms. A substantial proportion of adults with bipolar disorder and schizophrenia showed high autistic-like traits/symptoms independent of symptom severity, suggesting a shared pathophysiology among autism spectrum disorder and these psychiatric disorders. Conversely, autistic-like traits among subjects with MDD were associated with the depressive symptom severity. These findings suggest the importance of

Autistic-Like Traits in Adult Patients with Mood Disorders and Schizophrenia

PubMed Central

Matsuo, Junko; Kamio, Yoko; Takahashi, Hidetoshi; Ota, Miho; Teraishi, Toshiya; Hori, Hiroaki; Nagashima, Anna; Takei, Reiko; Higuchi, Teruhiko; Motohashi, Nobutaka; Kunugi, Hiroshi

2015-01-01

Autism spectrum disorder often co-occurs with other psychiatric disorders. Although a high prevalence of autistic-like traits/symptoms has been identified in the pediatric psychiatric population of normal intelligence, there are no reports from adult psychiatric population. This study examined whether there is a greater prevalence of autistic-like traits/symptoms in patients with adult-onset psychiatric disorders such as major depressive disorder (MDD), bipolar disorder, or schizophrenia, and whether such an association is independent of symptom severity. The subjects were 290 adults of normal intelligence between 25 and 59 years of age (MDD, n=125; bipolar disorder, n=56; schizophrenia, n=44; healthy controls, n=65). Autistic-like traits/symptoms were measured using the Social Responsiveness Scale for Adults. Symptom severity was measured using the Positive and Negative Symptoms Scale, the Hamilton Depression Rating Scale, and/or the Young Mania Rating Scale. Almost half of the clinical subjects, except those with remitted MDD, exhibited autistic-like traits/symptoms at levels typical for sub-threshold or threshold autism spectrum disorder. Furthermore, the proportion of psychiatric patients that demonstrated high autistic-like traits/symptoms was significantly greater than that of healthy controls, and not different between that of remitted or unremitted subjects with bipolar disorder or schizophrenia. On the other hand, remitted subjects with MDD did not differ from healthy controls with regard to the prevalence or degree of high autistic-like traits/symptoms. A substantial proportion of adults with bipolar disorder and schizophrenia showed high autistic-like traits/symptoms independent of symptom severity, suggesting a shared pathophysiology among autism spectrum disorder and these psychiatric disorders. Conversely, autistic-like traits among subjects with MDD were associated with the depressive symptom severity. These findings suggest the importance of

Consequences of orthodontic treatment in malocclusion patients: clinical and microbial effects in adults and children.

PubMed

Guo, Li; Feng, Ying; Guo, Hong-Gang; Liu, Bo-Wen; Zhang, Yang

2016-10-28

Malocclusion is a common disease of oral and maxillofacial region. The study was aimed to investigate levels changes of periodontal pathogens in malocclusion patients before, during and after orthodontic treatments, and to confirm the difference between adults and children. One hundred and eight malocclusion patients (46 adults and 62 children at the school-age) were randomly selected and received orthodontic treatment with fixed orthodontic appliances. Subgingival plaques were Porphyromonas gingivalis (P.gingivalis), Fusobacterium nucleatum (F. nucleatum), Prevotella intermedia (P. intermedia) and Tannerella forsythensis (T. forsythensis) collected from the observed regions before and after treatment. Clinical indexes, including plaque index (PLI), gingival index (GI), sulcus bleeding index (SBI), probing depth (PD) and attachment loss (AL) of observed teeth were examined. The detection rates of P.gingivalis, F. nucleatum, P. intermedia and T. forsythensis increased from baseline to the third month without significant difference, and then returned to pretreatment levels 12 month after applying fixed orthodontic appliances. Adults' percentage contents of P.gingivalis, F. nucleatum, P. intermedia and T. forsythensis were significantly higher than those of children at baseline and the first month, but not obvious at the third month. PLI and SBI were increased from baseline to the first and to the third month both in adults and children groups. Besides, PD were increased from baseline to first month, followed by a downward trend in the third month; however, all patients were failed to detect with AL. Periodontal and microbiological statuses of malocclusion patients may be influenced by fixed orthodontic appliances in both adults and children, more significant in children than in adults. Some microbiological indexes have synchronous trend with the clinical indexes. Long-term efficacy of fixed orthodontic appliances for malocclusion should be confirmed by future

[EMG activities of the head, neck and upper trunk muscles with mandibular movements in healthy adults and mandibular asymmetry patients].

PubMed

Jiang, Ting; Zhang, Zhenkang; Yang, Zhaohui; Yi, Biao; Feng, Hailan; Wang, Xing

2002-03-25

To study the activities of head, neck and upper trunk muscles during mandibular movements in healthy adults and mandibular asymmetry patients. Electromyographic integrogram was used to record and analyze the electromyographic activities of the anterior temporal (Ta), posterior temporal (Tp), sternocleidomastoid (SCM), and trapezius (TRAP) muscles in rest position and during mandibular movement among 10 normal adults and 10 mandibular asymmetry patients. All the four muscles showed constant electromyographic activities when the mandible was in the rest position. The activities of Ta, Tp, and SCM muscles increased with protrusion of mandible, mouth opening, tapping, maximum clenching, and chewing. The activities of Ta and Tp muscles of the patients were 1.7 times greater than that of the normal adults during mandibular movement without occlusion, and were weaker by 50% during mandibular movement with occlusion. The difference between electromyographic activities during mandibular movement and in rest position was less among patients than among normal adults. The TRAP muscle of the patients showed constant electromyographic activities with the activity volume nearly 1.8 times that of the normal adults. The difference between the muscle and its namesake at the opposite side was greater among the patients (21%) than among the normal adults (8%). All the four muscles participate in the maintenance of rest position of mandible and the realization of mandibular movements. The coordination of muscular activities among mandibular asymmetry patients is poorer than that among normal adults.

[Anterior rectal duplication in adult patient: a case report].

PubMed

Rodríguez-Cabrera, J; Villanueva-Sáenz, E; Bolaños-Badillo, L E

2009-01-01

To report a case of rectal duplication in the adult and make a literature review. The intestinal duplications are injuries of congenital origin that can exist from the base of the tongue to the anal verge, being the most frequent site at level of terminal ileum (22%) and at the rectal level in 5% To date approximately exist 80 reports in world-wide Literature generally in the pediatric population being little frequent in the adult age. Its presentation could be tubular or cystic. The recommended treatment is the surgical resection generally in block with coloanal anastomosis. A case review of rectal duplication in the adult and the conducted treatment. The case of a patient appears with diagnose of rectal duplication with tubular type,whose main symptom was constipation and fecal impactation. In the exploration was detect double rectal lumen (anterior and posterior) that it above initiates by of the anorectal ring with fibrous ulcer of fibrinoid aspect of 3 approx cm of length x 1 cm wide, at level of the septum that separates both rectal lumina. The rectal duplication is a rare pathology in the adult nevertheless is due to suspect before the existence of alterations in the mechanics of the defecation, rectal prolapse and rectal bleeding,the election treatment is a protectomy with colonic pouch in "J" and coloanal anastomosis.

A Continuous Flow Column Study of the Anaerobic Transformation of a CAH Mixture of Tetrachloroethene and Carbon Tetrachloride Using Formate as an Electron Donor

NASA Astrophysics Data System (ADS)

Semprini, L.; Azizian, M. F.; Kim, Y.

2011-12-01

Many groundwater sites are contaminated with mixtures of chlorinated aliphatic hydrocarbons (CAHs) that represent a challenge when biological remediation processes are being considered. This is especially challenging when high concentrations of CAHs are present.Trichloromethane (CF), for example, has been observed to inhibit and potentially exert toxicity on reductive dehalogenation of tetrachloroethene (PCE) and trichloroethene (TCE). Results will be presented from a continuous flow column study where the simultaneous transformation of PCE and carbon tetrachloride (CT) was achieved. The column was packed with a quartz sand and bioaugmented with the Evanite Culture (EV) that is capable of transforming PCE to ethene. The column was fed a synthetic groundwater that was amended with PCE to achieve an influent concentration near its solubility limit (0.10 mM) and formate (1.5 mM) that reacts to produce hydrogen as the ultimate electron donor. The column was operated for over 1600 days prior to the addition of CT. During this period PCE was transformed mainly to vinyl chloride (VC) and ethene (ETH) and minor amounts of cis-dichloroethene (cis-DCE) and TCE. The transformation extent achieved based on the column effluent concentrations ranged from about 50% ETH, 30% VC, and 20 cis-DCE up to 80% ETH and 20% VC. When the column was fed sulfate, it was completely transformed via sulfate reduction. Ferrous iron production from ferric iron reduction was observed early in the study. Acetate was also formed as a result of homoacetogenesis from hydrogen utilization. CT addition (0.015 mM) was started at 1600 days while PCE addition was continued. During the first 25 days of CT addition, CT concentrations gradually increased to 50% of the injection concentration and chloromethane (CM) and CF were observed as transformation products. CT concentrations then decreased with over 98% transformation achieved.CM was removed to below the detection limit and CF concentration decreases to

Potential Adult Medicaid Beneficiaries Under the Patient Protection and Affordable Care Act Compared With Current Adult Medicaid Beneficiaries

PubMed Central

Chang, Tammy; Davis, Matthew

2013-01-01

PURPOSE Under health care reform, states will have the opportunity to expand Medicaid to millions of uninsured US adults. Information regarding this population is vital to physicians as they prepare for more patients with coverage. Our objective was to describe demographic and health characteristics of potentially eligible Medicaid beneficiaries. METHODS We performed a cross-sectional study using data from the National Health and Nutrition Examination Survey (2007–2010) to identify and compare adult US citizens potentially eligible for Medicaid under provisions of the Patient Protection and Affordable Care Act (ACA) with current adult Medicaid beneficiaries. We compared demographic characteristics (age, sex, race/ethnicity, education) and health measures (self-reported health status; measured body mass index, hemoglobin A1c level, systolic and diastolic blood pressure, depression screen [9-item Patient Health Questionnaire], tobacco smoking, and alcohol use). RESULTS Analyses were based on an estimated 13.8 million current adult non-elderly Medicaid beneficiaries and 13.6 million nonelderly adults potentially eligible for Medicaid. Potentially eligible individuals are expected to be more likely male (49.2% potentially eligible vs 33.3% current beneficiaries; P <.001), to be more likely white and less likely black (58.8% white, 20.0% black vs 49.9% white, 25.2% black; P = .02), and to be statistically indistinguishable in terms of educational attainment. Overall, potentially eligible adults are expected to have better health status (34.8% “excellent” or “very good,” 40.4% “good”) than current beneficiaries (33.5% “excellent” or “very good,” 31.6% “good”; P <.001). The proportions obese (34.5% vs 42.9%; P = .008) and with depression (15.5% vs 22.3%; P = .003) among potentially eligible individuals are significantly lower than those for current beneficiaries, while there are no significant differences in the expected prevalence of diabetes or

Potential adult Medicaid beneficiaries under the Patient Protection and Affordable Care Act compared with current adult Medicaid beneficiaries.

PubMed

Chang, Tammy; Davis, Matthew

2013-01-01

Under health care reform, states will have the opportunity to expand Medicaid to millions of uninsured US adults. Information regarding this population is vital to physicians as they prepare for more patients with coverage. Our objective was to describe demographic and health characteristics of potentially eligible Medicaid beneficiaries. We performed a cross-sectional study using data from the National Health and Nutrition Examination Survey (2007-2010) to identify and compare adult US citizens potentially eligible for Medicaid under provisions of the Patient Protection and Affordable Care Act (ACA) with current adult Medicaid beneficiaries. We compared demographic characteristics (age, sex, race/ethnicity, education) and health measures (self-reported health status; measured body mass index, hemoglobin A1c level, systolic and diastolic blood pressure, depression screen [9-item Patient Health Questionnaire], tobacco smoking, and alcohol use). Analyses were based on an estimated 13.8 million current adult non-elderly Medicaid beneficiaries and 13.6 million nonelderly adults potentially eligible for Medicaid. Potentially eligible individuals are expected to be more likely male (49.2% potentially eligible vs 33.3% current beneficiaries; P <.001), to be more likely white and less likely black (58.8% white, 20.0% black vs 49.9% white, 25.2% black; P = .02), and to be statistically indistinguishable in terms of educational attainment. Overall, potentially eligible adults are expected to have better health status (34.8% "excellent" or "very good," 40.4% "good") than current beneficiaries (33.5% "excellent" or "very good," 31.6% "good"; P <.001). The proportions obese (34.5% vs 42.9%; P = .008) and with depression (15.5% vs 22.3%; P = .003) among potentially eligible individuals are significantly lower than those for current beneficiaries, while there are no significant differences in the expected prevalence of diabetes or hypertension. Current tobacco smoking (49.2% vs

Woman with virilizing congenital adrenal hyperplasia and Leydig cell tumor of the ovary.

PubMed

Fernández-García Salazar, Rosario; Muñoz-Darias, Carmen; Haro-Mora, Juan Jesús; Almaraz, M Cruz; Audí, Laura; Martínez-Tudela, Juana; Yahyaoui, Raquel; Esteva, Isabel

2014-08-01

We report the case of a 36-year-old woman with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency, and corticosteroid replacement therapy since birth. She manifested persistent virilization and high testosterone levels that were attributed to nonadherence to medical treatment. The patient was referred to our gender unit for genitoplastic surgery. We recommended the patient for left oophorectomy after detecting an ovarian mass. Pathologic findings confirmed an ovarian hilus cell tumor. Testosterone levels fell back to normal and masculinization disappeared but ACTH remained elevated. This case represents a very rare type of primary ovarian tumor that must be considered in persistent virilizing symptoms in women with CAH.

Clinical features of congenital adrenal insufficiency including growth patterns and significance of ACTH stimulation test.

PubMed

Koh, Ji Won; Kim, Gu Hwan; Yoo, Han Wook; Yu, Jeesuk

2013-11-01

Congenital adrenal insufficiency is caused by specific genetic mutations. Early suspicion and definite diagnosis are crucial because the disease can precipitate a life-threatening hypovolemic shock without prompt treatment. This study was designed to understand the clinical manifestations including growth patterns and to find the usefulness of ACTH stimulation test. Sixteen patients with confirmed genotyping were subdivided into three groups according to the genetic study results: congenital adrenal hyperplasia due to 21-hydroxylase deficiency (CAH, n=11), congenital lipoid adrenal hyperplasia (n=3) and X-linked adrenal hypoplasia congenita (n=2). Bone age advancement was prominent in patients with CAH especially after 60 months of chronologic age (n=6, 67%). They were diagnosed in older ages in group with bone age advancement (P<0.05). Comorbid conditions such as obesity, mental retardation, and central precocious puberty were also prominent in this group. In conclusion, this study showed the importance of understanding the clinical symptoms as well as genetic analysis for early diagnosis and management of congenital adrenal insufficiency. ACTH stimulation test played an important role to support the diagnosis and serum 17-hydroxyprogesterone levels were significantly elevated in all of the CAH patients. The test will be important for monitoring growth and puberty during follow up of patients with congenital adrenal insufficiency.

Orthodontic Treatment in Adult Patient with Reduced Periodontium: A Case Report.

PubMed

Shintcovsk, Ricardo Lima; Knop, Luegya Amorim Henriques; Pinto, Ary Santos; Gandini, Luiz Gonzaga; Martins, Lídia Parsekian

2015-01-01

Patients presenting reduced periodontium represent a major concern for orthodontists. The purpose of this article is to present the clinical case of an adult patient who presented sequel of periodontal disease (diastemas) compromising her dental aesthetics. She was subjected to an orthodontic treatment with the application of light forces distant from the teeth with reduced periodontium. A periodontal support therapy was successfully implemented. The final stage of the treatment indicated satisfactory occlusal and periodontal characteristics.

Managing Body Image Difficulties of Adult Cancer Patients: Lessons from Available Research

PubMed Central

Fingeret, Michelle Cororve; Teo, Irene; Epner, Daniel E.

2013-01-01

Background Body image is a critical psychosocial issue for cancer patients as they often undergo significant changes to appearance and functioning. In this review article, our primary purpose was to identify empirically-supported approaches to treat body image difficulties of adult cancer patients that can be incorporated into high-quality comprehensive cancer care. Methods We provided an overview of theoretical models of body image relevant to cancer patients, and presented findings from published literature on body image and cancer from 2003–2013. We integrated these data with information from the patient-doctor communication literature to delineate a practical approach for assessing and treating body image concerns of adult cancer patients. Results Body image difficulties were found across patients with diverse cancer sites, and were most prevalent in the immediate postoperative and treatment period. Age, body mass index, and specific cancer treatments have been identified as potential risk factors for body image disturbance in cancer patients. Current evidence supports the use of time-limited cognitive-behavioral therapy interventions for addressing these difficulties. Other intervention strategies also show promise but require further study. We identified potential indicators of body image difficulties to alert healthcare professionals when to refer patients for psychosocial care, and proposed a framework for approaching conversations about body image that can be used by the oncologic treatment team. Conclusions Body image issues affect a wide array of cancer patients. Providers can use available evidence combined with information from the healthcare communication literature to develop practical strategies for treating body image concerns of cancer patients. PMID:24895287

Managing body image difficulties of adult cancer patients: lessons from available research.

PubMed

Fingeret, Michelle Cororve; Teo, Irene; Epner, Daniel E

2014-03-01

Body image is a critical psychosocial issue for patients with cancer because they often undergo significant changes to appearance and functioning. The primary purpose of this review article was to identify empirically-supported approaches to treat body image difficulties of adult cancer patients that can be incorporated into high-quality comprehensive cancer care. An overview was provided of theoretical models of body image relevant to cancer patients, and findings were presented from published literature on body image and cancer from 2003 to 2013. These data were integrated with information from the patient-doctor communication literature to delineate a practical approach for assessing and treating body image concerns of adult cancer patients. Body image difficulties were found across patients with diverse cancer sites, and were most prevalent in the immediate postoperative and treatment period. Age, body mass index, and specific cancer treatments have been identified as potential risk factors for body image disturbance in cancer patients. Current evidence supports the use of time-limited cognitive-behavioral therapy interventions for addressing these difficulties. Other intervention strategies also show promise but require further study. Potential indicators of body image difficulties were identified to alert health care professionals when to refer patients for psychosocial care, and a framework was proposed for approaching conversations about body image that can be used by the oncologic treatment team. Body image issues affect a wide array of cancer patients. Providers can use available evidence combined with information from the health care communication literature to develop practical strategies for treating body image concerns of patients with cancer. © 2013 American Cancer Society.

[Lymphocytic Clonal Expansion in Adult Patients with Epstein-Barr Virus-Associated Lymphoproliferative Disease].

PubMed

Zhong, Feng-Luan; Zhang, Hong-Yu; Zhang, Qian; Feng, Jia; Zhang, Wen-Li; Xu, Lei; Xu, Hai-Chan; Wen, Juan-Juan; Meng, Qing-Xiang

2017-12-01

To explore the lymphocytic clonal expansion in adult patients with Epstein-Barr virus-associated lymphoproliferative diseases (EBV+LPD), and to investigate the experimental methods for EBV+LPD cells so as to provide a more objective measure for the diagnosis, classification and prognosis in the early stage of this disease. Peripheral blood samples from 5 patients with EBV+LPD, 4 patients with adult infectious mononucleosis(IM) as negative control and 3 patients with acute NK-cell leukemia(ANKL) as positive control were collected. Prior to immunochemotherapy, viral loads and clonality were analysed by flow cytometry (FCM), T cell receptor gene rearrangement (TCR) was detected by real-time polymerase chain reaction (RT-PCR), and diversity of EB virus terminal repeat (EBV-TR) was detected by Southern blot. FCM showed only 1 case with clonal TCRVβ in 5 patients with EBV+LPD, TCR clonal expansion could be detected both in patients with IM(4 of 4) and 4 patients with EBV+LPD(4 of 5), Out of patients with EBV+LPD, 1 patient displayed a monoclonal band and 2 patients showed oligoclonal bands when detecting EBV-TR by southen blot. Detecting the diversity of EBV-TR by Southern blot may be the most objective way to reflex clonal transformation of EBV+LPD, which is of great benefit to the diagnosis, classification and prognosis in the early stage of this disease.

Sleep-Related Rhythmic Movement Disorder and Obstructive Sleep Apnea in Five Adult Patients

PubMed Central

Chiaro, Giacomo; Maestri, Michelangelo; Riccardi, Silvia; Haba-Rubio, José; Miano, Silvia; Bassetti, Claudio L.; Heinzer, Raphaël C.; Manconi, Mauro

2017-01-01

Sleep-related rhythmic movements (SRRMs) are typical in infancy and childhood, where they usually occur at the wake-to-sleep transition. However, they have rarely been observed in adults, where they can be idiopathic or associated with other sleep disorders including sleep apnea. We report a case series of 5 adults with sleep-related rhythmic movement disorder, 4 of whom had a previous history of SRRMs in childhood. SRRMs mostly occurred in consolidated sleep, in association with pathological respiratory events, predominantly longer ones, especially during stage R sleep, and recovered in 1 patient with continuous positive airway pressure therapy. We hypothesize that sleep apneas may act as a trigger of rhythmic motor events through a respiratory-related arousal mechanism in genetically predisposed subjects. Citation: Chiaro G, Maestri M, Riccardi S, Haba-Rubio J, Miano S, Bassetti CL, Heinzer RC, Manconi M. Sleep-related rhythmic movement disorder and obstructive sleep apnea in five adult patients. J Clin Sleep Med. 2017;13(10):1213–1217. PMID:28859719

Childhood maltreatment, adult attachment and psychotic symptomatology: a study in patients, siblings and controls.

PubMed

van Dam, D S; Korver-Nieberg, N; Velthorst, E; Meijer, C J; de Haan, L

2014-11-01

The association between childhood maltreatment (ChM) and psychotic disorders is well established. However, there is an ongoing debate about which factors account for this relationship. One explanation is that the relationship between ChM and psychosis is mediated by adult attachment style. Therefore, in this study, we aimed to investigate whether adult attachment style mediates the relationship between ChM and positive and negative symptomatology. We investigated the relation between ChM and psychotic symptoms, taking into account levels of (insecure) attachment, in 131 patients with psychotic illness, 123 siblings and 72 controls. ChM was assessed with the Childhood Trauma Questionnaire (CTQ). Attachment dimensions of anxiety and avoidance were measured using the Psychosis Attachment Measure (PAM). In both patients and siblings, ChM predicted positive symptoms and this relationship was partly mediated by attachment style. This relationship was found to be stronger for siblings than for patients. ChM predicted negative symptoms in patients and siblings. In the patient sample, attachment style did not mediate the relationship between ChM and negative symptoms, whereas attachment style was found to be a mediator in the sibling sample. ChM was associated with positive and negative symptomatology in both patients and siblings. Particularly in siblings, the relationship between ChM and psychosis seems to be mediated by adult attachment style. Perhaps attachment style may play a more prominent role on a subclinical level.

Pharmacokinetics of temozolomide given three times a day in pediatric and adult patients.

PubMed

Riccardi, Anna; Mazzarella, Giorgio; Cefalo, Graziella; Garrè, Maria Luisa; Massimino, Maura; Barone, Carlo; Sandri, Alessandro; Ridola, Vita; Ruggiero, Antonio; Mastrangelo, Stefano; Lazzareschi, Ilaria; Caldarelli, Massimo; Maira, Giulio; Madon, Enrico; Riccardi, Riccardo

2003-12-01

To characterize and compare pharmacokinetic parameters in children and adults treated with temozolomide (TMZ) administered for 5 days in three doses daily, and to evaluate the possible relationship between AUC values and hematologic toxicity. TMZ pharmacokinetic parameters were characterized in pediatric and adult patients with primary central nervous system tumors treated with doses ranging from 120 to 200 mg/m2 per day, divided into three doses daily for 5 days. Plasma levels were measured over 8 h following oral administration in a fasting state. A total of 40 courses were studied in 22 children (mean age 10 years, range 3-16 years) and in 8 adults (mean age 30 years, range 19-54 years). In all patients, a linear relationship was found between systemic exposure (AUC) and increasing doses of TMZ. Time to peak concentration, elimination half-life, apparent clearance and volume of distribution were not related to TMZ dose. No differences were seen among TMZ C(max), t(1/2), V(d) or CL/F in children compared with adults. Intra- and interpatient variability of systemic exposure were limited in both children and adults. No statistically significant differences were found between the AUCs of children who experienced grade 4 hematologic toxicity and children who did not. No difference appears to exist between pharmacokinetic parameters in adults and children when TMZ is administered in three doses daily. Hematologic toxicity was not related to TMZ AUC. AUC measurement does not appear to be of any use in optimizing TMZ treatment.

Differences of Sagittal Lumbosacral Parameters between Patients with Lumbar Spondylolysis and Normal Adults

PubMed Central

Yin, Jin; Peng, Bao-Gan; Li, Yong-Chao; Zhang, Nai-Yang; Yang, Liang; Li, Duan-Ming

2016-01-01

Background: Recent studies have suggested an association between elevated pelvic incidence (PI) and the development of lumbar spondylolysis. However, there is still lack of investigation for Han Chinese people concerning the normal range of spinopelvic parameters and relationship between abnormal sagittal parameters and lumbar diseases. The objective of the study was to investigate sagittal lumbosacral parameters of adult lumbar spondylolysis patients in Han Chinese population. Methods: A total of 52 adult patients with symptomatic lumbar spondylolysis treated in the General Hospital of Armed Police Force (Beijing, China) were identified as the spondylolysis group. All the 52 patients were divided into two subgroups, Subgroup A: 36 patients with simple lumbar spondylolysis, and Subgroup B: 16 patients with lumbar spondylolysis accompanying with mild lumbar spondylolisthesis (slip percentage <30%). Altogether 207 healthy adults were chosen as the control group. All patients and the control group took lumbosacral lateral radiographs. Seven sagittal lumbosacral parameters, including PI, pelvic tilt (PT), sacral slope (SS), lumbar lordosis (LL), L5 incidence, L5 slope, and sacral table angle (STA), were measured in the lateral radiographs. All the parameters aforementioned were compared between the two subgroups and between the spondylolysis group and the control group with independent-sample t-test. Results: There were no statistically significant differences of all seven sagittal lumbosacral parameters between Subgroup A and Subgroup B. PI, PT, SS, and LL were higher (P < 0.05) in the spondylolysis group than those in the control group, but STA was lower (P < 0.001) in the spondylolysis group. Conclusions: Current study results suggest that increased PI and decreased STA may play important roles in the pathology of lumbar spondylolysis in Han Chinese population. PMID:27174324

Differences of Sagittal Lumbosacral Parameters between Patients with Lumbar Spondylolysis and Normal Adults.

PubMed

Yin, Jin; Peng, Bao-Gan; Li, Yong-Chao; Zhang, Nai-Yang; Yang, Liang; Li, Duan-Ming

2016-05-20

Recent studies have suggested an association between elevated pelvic incidence (PI) and the development of lumbar spondylolysis. However, there is still lack of investigation for Han Chinese people concerning the normal range of spinopelvic parameters and relationship between abnormal sagittal parameters and lumbar diseases. The objective of the study was to investigate sagittal lumbosacral parameters of adult lumbar spondylolysis patients in Han Chinese population. A total of 52 adult patients with symptomatic lumbar spondylolysis treated in the General Hospital of Armed Police Force (Beijing, China) were identified as the spondylolysis group. All the 52 patients were divided into two subgroups, Subgroup A: 36 patients with simple lumbar spondylolysis, and Subgroup B: 16 patients with lumbar spondylolysis accompanying with mild lumbar spondylolisthesis (slip percentage <30%). Altogether 207 healthy adults were chosen as the control group. All patients and the control group took lumbosacral lateral radiographs. Seven sagittal lumbosacral parameters, including PI, pelvic tilt (PT), sacral slope (SS), lumbar lordosis (LL), L5 incidence, L5 slope, and sacral table angle (STA), were measured in the lateral radiographs. All the parameters aforementioned were compared between the two subgroups and between the spondylolysis group and the control group with independent-sample t- test. There were no statistically significant differences of all seven sagittal lumbosacral parameters between Subgroup A and Subgroup B. PI, PT, SS, and LL were higher (P < 0.05) in the spondylolysis group than those in the control group, but STA was lower (P < 0.001) in the spondylolysis group. Current study results suggest that increased PI and decreased STA may play important roles in the pathology of lumbar spondylolysis in Han Chinese population.

Quality Measures for the Care of Adult Patients with Obstructive Sleep Apnea

PubMed Central

Aurora, R. Nisha; Collop, Nancy A.; Jacobowitz, Ofer; Thomas, Sherene M.; Quan, Stuart F.; Aronsky, Amy J.

2015-01-01

Obstructive sleep apnea (OSA) is a prevalent disorder associated with a multitude of adverse outcomes when left untreated. There is significant heterogeneity in the evaluation and management of OSA resulting in variation in cost and outcomes. Thus, the goal for developing these measures was to have a way to evaluate the outcomes and reliability of the processes involved with the standard care approaches used in the diagnosis and management of OSA. The OSA quality care measures presented here focus on both outcomes and processes. The AASM commissioned the Adult OSA Quality Measures Workgroup to develop quality care measures aimed at optimizing care for adult patients with OSA. These quality care measures developed by the Adult OSA Quality Measures Workgroup are an extension of the original Centers for Medicare & Medicaid Services (CMS) approved Physician Quality Reporting System (PQRS) measures group for OSA. The measures are based on the available scientific evidence, focus on public safety, and strive to improve quality of life and cardiovascular outcomes for individual OSA patients. The three outcomes that were selected were as follows: (1) improve disease detection and categorization; (2) improve quality of life; and (3) reduce cardiovascular risk. After selecting these relevant outcomes, a total of ten process measures were chosen that could be applied and assessed for the purpose of accomplishing these outcomes. In the future, the measures described in this document may be reported through the PQRS in addition to, or as a replacement for, the current OSA measures group. The overall objective for the development of these measures is that implementation of these quality measures will result in improved patient outcomes, reduce the public health burden of OSA, and provide a measurable standard for evaluating and managing OSA. Citation: Aurora RN, Collop NA, Jacobowitz O, Thomas SM, Quan SF, Aronsky AJ. Quality measures for the care of adult patients with

Co-occurring Attention Deficit Hyperactivity Disorder symptoms in adults affected by heroin dependence: Patients characteristics and treatment needs.

PubMed

Lugoboni, Fabio; Levin, Frances Rudnick; Pieri, Maria Chiara; Manfredini, Matteo; Zamboni, Lorenzo; Somaini, Lorenzo; Gerra, Gilberto; Gruppo InterSert Collaborazione Scientifica Gics

2017-04-01

Attention Deficit Hyperactivity Disorder (ADHD) is a risk for substance use disorders. The aim of this study was to investigate the association between adult ADHD symptoms, opioid use disorder, life dysfunction and co-occurring psychiatric symptoms. 1057 heroin dependent patients on opioid substitution treatment participated in the survey. All patients were screened for adult ADHD symptoms using the Adult ADHD Self-Report Scale (ASRS-v1.1). 19.4% of the patients screened positive for concurrent adult ADHD symptoms status and heroin dependence. Education level was lower among patients with ADHD symptoms, but not significant with respect to non-ADHD patients. Patients with greater ADHD symptoms severity were less likely to be employed. A positive association was observed between ADHD symptoms status and psychiatric symptoms. Patients with ADHD symptoms status were more likely to be smokers. Patients on methadone had a higher rate of ADHD symptoms status compared to buprenorphine. Those individuals prescribed psychoactive drugs were more likely to have ADHD symptoms. In conclusion, high rate of ADHD symptoms was found among heroin dependent patients, particularly those affected by the most severe form of addiction. These individuals had higher rates of unemployment, other co-morbid mental health conditions, heavy tobacco smoking. Additional psychopharmacological interventions targeting ADHD symptoms, other than opioid substitution, is a public health need. Copyright © 2017 Elsevier Ireland Ltd. All rights reserved.