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1.
AJNR Am J Neuroradiol ; 39(8): 1400-1405, 2018 08.
Article in English | MEDLINE | ID: mdl-29976832

ABSTRACT

BACKGROUND AND PURPOSE: Radiologists should manage the radiation dose for pediatric patients to maintain reasonable diagnostic confidence. We assessed the variation in estimated radiation dose indices for pediatric noncontrast head CT in the United States. MATERIALS AND METHODS: Radiation dose indices for single-phase noncontrast head CT examinations in patients 18 years of age and younger were retrospectively reviewed between July 2011 and June 2016 using the American College of Radiology CT Dose Index Registry. We used the reported volume CT dose index stratified by patient demographics and imaging facility characteristics. RESULTS: The registry included 295,296 single-phase pediatric noncontrast head CT studies from 1571 facilities (56% in male patients and 53% in children older than 10 years of age). The median volume CT dose index was 33 mGy (interquartile range = 22-47 mGy). The volume CT dose index increased as age increased. The volume CT dose index was lower in children's hospitals (median, 26 mGy) versus academic hospitals (median, 32 mGy) and community hospitals (median, 40 mGy). There was a lower volume CT dose index in level I and II trauma centers (median, 27 and 32 mGy, respectively) versus nontrauma centers (median, 40 mGy) and facilities in metropolitan locations (median, 30 mGy) versus those in suburban and rural locations (median, 41 mGy). CONCLUSIONS: Considerable variation in the radiation dose index for pediatric head CT exists. Median dose indices and practice variations at pediatric facilities were both lower compared with other practice settings. Decreasing dose variability through proper management of CT parameters in pediatric populations using benchmarks generated by data from registries can potentially decrease population exposure to ionizing radiation.


Subject(s)
Cone-Beam Computed Tomography/standards , Head/diagnostic imaging , Radiation Dosage , Adolescent , Child , Female , Humans , Infant , Male , Registries , Retrospective Studies , United States
3.
AJNR Am J Neuroradiol ; 35(4): 650-6, 2014 Apr.
Article in English | MEDLINE | ID: mdl-24157736

ABSTRACT

BACKGROUND AND PURPOSE: Neurointerventions in children have dramatically improved the clinical outlook for patients with previously intractable cerebrovascular conditions, such as vein of Galen malformations and complex arteriovenous fistulas. However, these complex and sometimes lengthy procedures are performed under fluoroscopic guidance and thus unavoidably expose vulnerable pediatric patients to the effects of ionizing radiation. Recent epidemiologic evidence from a national registry of children who underwent CT scans suggests a higher-than-expected incidence of secondary tumors. We sought to calculate the predicted risk of secondary tumors in a large cohort of pediatric neurointerventional patients. MATERIALS AND METHODS: We reviewed our cohort of pediatric neurointerventions, tabulated radiation dose delivered to the skin, and calculated the range of likely brain-absorbed doses by use of previously developed mathematical models. The predicted risk of secondary tumor development as a function of brain-absorbed dose in this cohort was then generated by use of the head CT registry findings. RESULTS: Maximal skin dose and brain-absorbed doses in our cohort were substantially lower than have been previously described. However, we found 1) a statistically significant correlation between radiation dose and age at procedure, as well as number and type of procedures, and 2) a substantial increase in lifetime predicted risk of tumor above baseline in the cohort of young children who undergo neurointerventions. CONCLUSIONS: Although neurointerventional procedures have dramatically improved the prognosis of children facing serious cerebrovascular conditions, the predicted risk of secondary tumors, particularly in the youngest patients and those undergoing multiple procedures, is sobering.


Subject(s)
Brain Neoplasms/epidemiology , Brain/diagnostic imaging , Cerebral Angiography/adverse effects , Fluoroscopy/adverse effects , Neoplasms, Radiation-Induced/epidemiology , Radiation Dosage , Adolescent , Age Distribution , Cerebral Angiography/methods , Cerebral Angiography/statistics & numerical data , Child , Child, Preschool , Fluoroscopy/methods , Fluoroscopy/statistics & numerical data , Humans , Infant , Infant, Newborn , Linear Models , Models, Theoretical , Registries/statistics & numerical data , Retrospective Studies , Risk Assessment/methods , Risk Factors , United Kingdom/epidemiology , Young Adult
5.
Diabetes Metab ; 39(5): 445-53, 2013 Oct.
Article in English | MEDLINE | ID: mdl-23886784

ABSTRACT

INTRODUCTION: Our objective was to assess the frequency of lipohypertrophy (LH) and its relationship to site rotation, needle reuse, glucose variability, hypoglycaemia and use of insulin. METHODS: The study included 430 outpatients injecting insulin who filled out a wide-ranging questionnaire regarding their injection technique. Then, a diabetes nurse examined their injection sites for the presence of LH. RESULTS: Nearly two-thirds (64.4%) of patients had LH. There was a strong relationship between the presence of LH and non-rotation of sites, with correct rotation technique having the strongest protective value against LH. Of the patients who correctly rotated sites, only 5% had LH while, of the patients with LH, 98% either did not rotate sites or rotated incorrectly. Also, 39.1% of patients with LH had unexplained hypoglycaemia and 49.1% had glycaemic variability compared with only 5.9% and 6.5%, respectively, in those without LH. LH was also related to needle reuse, with risk increasing significantly when needles were used > 5 times. Total daily insulin doses for patients with and without LH averaged 56 and 41 IU/day, respectively. This 15 IU difference equates to a total annual cost to the Spanish healthcare system of > €122 million. This was also the first study in which the use of ultrasound allowed the description of an "echo signature" for LH. CONCLUSION: Correct injection site rotation appears to be the critical factor in preventing LH, which is associated with reduced glucose variability, hypoglycaemia, insulin consumption and costs.


Subject(s)
Adipose Tissue/pathology , Diabetes Mellitus, Type 1/drug therapy , Diabetes Mellitus, Type 2/drug therapy , Hypoglycemic Agents/administration & dosage , Injections, Subcutaneous/adverse effects , Insulin/administration & dosage , Adipose Tissue/injuries , Blood Glucose/metabolism , Diabetes Mellitus, Type 1/complications , Diabetes Mellitus, Type 1/epidemiology , Diabetes Mellitus, Type 2/complications , Diabetes Mellitus, Type 2/epidemiology , Female , Glycated Hemoglobin/metabolism , Guidelines as Topic , Humans , Hypertrophy/epidemiology , Hypertrophy/etiology , Male , Middle Aged , Odds Ratio , Prevalence , Primary Health Care , Risk Factors , Spain/epidemiology , Young Adult
6.
Res Dev Disabil ; 34(9): 2967-85, 2013 Sep.
Article in English | MEDLINE | ID: mdl-23816632

ABSTRACT

This paper presents a comprehensive synthesis of six meta-analyses of early intensive behavioral interventions (EIBI) for young children with autism spectrum disorders published from 2009 to 2011. Analysis was conducted in three steps to account for different formats of treatment delivery and the extent to which parents took part in treatment. The three components of the synthesis were (a) descriptive analysis, (b) effect size analysis, and (c) mediator analysis via partial correlation and linear regressions. We completed the analysis by obtaining standardized mean difference effect sizes for 13 comparative studies ordered by comparison study type and 22 mean change effect sizes ordered by treatment delivery type. Results suggest that EIBI leads generally to positive medium-to-large effects for three available outcome measures: intellectual functioning, language skills and adaptive behaviors. Although favorable effects were apparent across comparative studies, analysis by type of delivery format revealed that EIBI programs that include parents in treatment provision are more effective. Mediator analyses suggest that treatment variables and child characteristics impact program effectiveness when accounting for the extent of parent inclusion. Clinical implications toward individualized treatment tailoring are discussed.


Subject(s)
Behavior Therapy/methods , Child Development Disorders, Pervasive/psychology , Child Development Disorders, Pervasive/therapy , Parent-Child Relations , Parents/psychology , Adult , Child , Child, Preschool , Early Intervention, Educational/methods , Humans , Infant
8.
Diabetes Metab ; 38 Suppl 1: S2-8, 2012 Jan.
Article in English | MEDLINE | ID: mdl-22305440

ABSTRACT

AIM: Injections and fingersticks administered to patients with diabetes in health care settings present a risk of blood exposure to the injector as well as other workers in potential contact with sharps. Such exposures could lead to transmission of bloodborne pathogens such as hepatitis and HIV. A recent EU Directive requires that where such risks have been identified, processes and devices must be put in place to reduce or eliminate the risk. The aim of this paper is to provide formal guidelines on the application of this Directive to diabetes care settings. These evidence-based recommendations were written and vetted by a large group of international safety experts. METHODS: A systematic literature search was conducted for all peer-reviewed studies and publications which bear on sharps safety in diabetes. Initially a group of experts reviewed this literature and drafted the recommendations. These were then presented for review, debate and revision to 57 experts from 14 countries at the WISE workshop in October, 2011. After the WISE meeting, the revised Recommendations were circulated electronically to attendees on three occasions, each time in a new iteration with revisions. RESULTS: Each recommendation was graded by the weight it should have in daily practice and by its degree of support in the medical literature. The topics covered include Risks of Sharps Injury and Muco-cutaneous Exposure, The EU Directive, Device Implications, Injection Technique Implications, Education and Training (Creating a "Safety Culture"), Value, Awareness and Responsibility. CONCLUSION: These safety recommendations provide practical guidance and fill an important gap in diabetes management. If followed, they should help ensure safe, effective and largely injury-free injections and fingersticks. They will serve as the roadmap for applying the new EU Directive to diabetes care.


Subject(s)
Diabetes Mellitus , HIV Infections/prevention & control , Hepatitis B/prevention & control , Hepatitis C/prevention & control , Infectious Disease Transmission, Patient-to-Professional , Needlestick Injuries/prevention & control , Evidence-Based Medicine , Guidelines as Topic , HIV Infections/transmission , Hepatitis B/transmission , Hepatitis C/transmission , Humans , Injections , Needlestick Injuries/complications , Safety Management
9.
Diabetes Metab ; 38 Suppl 1: S9-14, 2012 Jan.
Article in English | MEDLINE | ID: mdl-22305441

ABSTRACT

AIM: With the June 2010 publication of EU Council Directive 2010/32/EU scrutiny is now being focused on the safety and protection of diabetes nurses. METHODS: We used a questionnaire to study the frequency and risks of Needlestick Injuries (NSI) associated with diabetic injections in European hospitals. 634 nurses participated from 13 western European countries and Russia. RESULTS: When patients with diabetes who self-inject at home are hospitalized injections are given always by the staff in 31% of cases, by the patients themselves where possible in 33%, initially by staff, then the patient takes over in 12% and both staff and patient throughout the stay in 21%. 86% of nurses said their hospitals had a written policy on the prevention of NSI but, where it was available, only 56% were familiar with it. 67% of the nurses had not attended any training on the prevention of NSI and only 13% had attended one in the last year. 7.1% of nurses report recapping needles and 5.9% report storing unprotected needles temporarily on a tray, trolley or cart. 32% of nurses report suffering a NSI while giving a diabetic injection at some point in the past. 29.5% of NSI occurred while recapping a used needle. 57% of nurses unscrew pen needles using their own fingers. In 80% cases the source patient's identity was known and the sharp item was "contaminated" (known previous percutaneous exposure to patient) in almost half the cases (43%). NSIs were reported to the proper authorities in only 2/3 of cases. CONCLUSION: Our study shows that frequent NSI occur in European nurses treating people with diabetes in hospital settings. These injuries are a source of possible infection despite the small size of diabetes needles. The introduction of safety-engineered medical devices has been shown to reduce the risk of injury. A new European Directive that has now come into force specifically stipulates that wherever there is risk of sharps injury, the user and all healthcare workers must be protected by adequate safety precautions, including the use of "medical devices incorporating safety-engineered protection mechanisms".


Subject(s)
Diabetes Mellitus , HIV Infections/transmission , Hepatitis B/transmission , Hepatitis C/transmission , Infectious Disease Transmission, Patient-to-Professional/prevention & control , Needlestick Injuries/prevention & control , Occupational Injuries/prevention & control , Accidents, Occupational/prevention & control , Accidents, Occupational/statistics & numerical data , Europe/epidemiology , HIV Infections/prevention & control , Hepatitis B/prevention & control , Hepatitis C/prevention & control , Humans , Infectious Disease Transmission, Patient-to-Professional/statistics & numerical data , Needlestick Injuries/epidemiology , Nurses , Occupational Injuries/epidemiology , Russia/epidemiology , Surveys and Questionnaires
10.
Med Phys ; 39(6Part21): 3863-3864, 2012 Jun.
Article in English | MEDLINE | ID: mdl-28518262

ABSTRACT

As many as 6 - 8 million CT scans are performed annually in the United States on pediatric patients. The majority of these CT scans are not performed in pediatric hospitals that specialize in addressing the unique requirements of pediatric imaging. Instead, most of these scans occur in adult hospitals, where pediatric CT scanning is a small fraction of the total caseload. Both adult and pediatric hospitals need a simple method that allows the management of the CT radiation dose received by each patient based on the patient's physical size. This lecture suggests some simple tools and techniques that the qualified medical physicist can introduce to an individual practice in an effort to properly manage CT doses. This lecture begins by exploring the unique challenges presented by the pediatric patient in the management of their radiation dose during CT scanning. This is followed by an explanation of the basic science behind the development of the Size Specific Dose Estimate (SSDE) in CT, the strengths and weaknesses of the method, and some sample calculations. The presentation concludes by exploring the clinical application of SSDE in the day to day management of the radiation dose during CT scanning of not only small pediatric patients, but also patients who are larger than the average size adult. LEARNING OBJECTIVES: 1. Understand the basic challenges associated with CT imaging of children. 2. Understand the basic science used to develop SSDE, its strengths and its limitations 3. Understand the application of SSDE in the clinic in the daily management of pediatric CT doses.

11.
Pediatr Transplant ; 15(1): 58-64, 2011 Feb.
Article in English | MEDLINE | ID: mdl-20946191

ABSTRACT

MSUD is a complex metabolic disorder that has been associated with central nervous system damage, developmental delays, and neurocognitive deficits. Although liver transplantation provides a metabolic cure for MSUD, changes in cognitive and adaptive functioning following transplantation have not been investigated. In this report, we present data from 14 patients who completed cognitive and adaptive functioning testing pre- and one yr and/or three yr post-liver transplantation. Findings show either no significant change (n=8) or improvement (n=5) in IQ scores pre- to post-liver transplantation. Greater variability was observed in adaptive functioning scores, but the majority of patients evidenced no significant change (n=8) in adaptive scores. In general, findings indicate that liver transplantation minimizes the likelihood of additional central nervous system damage, providing an opportunity for possible stabilization or improvement in neurocognitive functioning.


Subject(s)
Liver Transplantation/methods , Maple Syrup Urine Disease/complications , Maple Syrup Urine Disease/therapy , Adaptation, Psychological , Adolescent , Child , Child, Preschool , Cognition , Cognition Disorders/complications , Cognition Disorders/etiology , Female , Humans , Intelligence Tests , Male , Neuropsychological Tests , Reproducibility of Results , Treatment Outcome
12.
Diabetes Metab ; 36 Suppl 2: S19-29, 2010 Sep.
Article in English | MEDLINE | ID: mdl-20933206

ABSTRACT

The first Injection Technique workshop brought together endocrinologists and injection experts from around the world in Strasbourg in 1997. From its work came groundbreaking recommendations which advanced best practices in areas such as the use of a skin fold when injecting. The second Injection Technique workshop, with an expanded format including nurses and diabetes educators, took place in Barcelona in 2000. The initial stimulus to use shorter injecting needles can be said to date from this meeting. The third Injection Technique workshop was held in Athens in September 2009 and involved 127 experts from across the globe. After a comprehensive review of all publications since 2000 as well as several unpublished studies, the attendees divided into smaller groups to debate and draft new injecting recommendations based on the new data and their collective experience. This paper summarizes all the formal presentations given at this practical consensus workshop.


Subject(s)
Diabetes Mellitus/drug therapy , Hypoglycemic Agents/administration & dosage , Injections, Subcutaneous , Insulin/administration & dosage , Needles , Subcutaneous Fat, Abdominal , Blood Glucose/metabolism , Diabetes Mellitus/blood , Diabetes Mellitus/epidemiology , Diabetes Mellitus, Type 1/drug therapy , Drug Storage , Equipment Design , Europe/epidemiology , Evidence-Based Medicine , Expert Testimony , Female , Humans , Hypertrophy/etiology , Hypertrophy/prevention & control , Injections, Subcutaneous/adverse effects , Injections, Subcutaneous/instrumentation , Injections, Subcutaneous/methods , Injections, Subcutaneous/psychology , Insulin/analogs & derivatives , Insulin Glargine , Insulin Infusion Systems/trends , Insulin, Long-Acting , Male , Needlestick Injuries/etiology , Needlestick Injuries/prevention & control , Patient Education as Topic , Practice Guidelines as Topic , Pregnancy , Pregnancy Complications/prevention & control , Subcutaneous Fat, Abdominal/injuries , Subcutaneous Fat, Abdominal/pathology , Syringes , United Kingdom , United States
13.
Diabetes Metab ; 36 Suppl 2: S3-18, 2010 Sep.
Article in English | MEDLINE | ID: mdl-20933208

ABSTRACT

AIM: Injections administered by patients are one of the mainstays of diabetes management. Proper injection technique is vital to avoiding intramuscular injections, ensuring appropriate delivery to the subcutaneous tissues and avoiding common complications such as lipohypertrophy. Yet few formal guidelines have been published summarizing all that is known about best practice. We propose new injection guidelines which are thoroughly evidence-based, written and vetted by a large group of international injection experts. METHODS: A systematic literature study was conducted for all peer-reviewed studies and publications which bear on injections in diabetes. An international group of experts met regularly over a two-year period to review this literature and draft the recommendations. These were then presented for review and revision to 127 experts from 27 countries at the TITAN workshop in September, 2009. RESULTS: Of 292 articles reviewed, 157 were found to meet the criteria of relevance to the recommendations. Each recommendation was graded by the weight it should have in daily practice and by its degree of support in the medical literature. The topics covered include The Role of the Professional, Psychological Challenges, Education, Site Care, Storage, Suspension and Priming, Injecting Process, Proper Use of Pens and Syringes, Insulin analogues, Human and Pre-mixed Insulins, GLP-1 analogs, Needle Length, Skin Folds, Lipohypertrophy, Rotation, Bleeding and Bruising, Pregnancy, Safety and Disposal. CONCLUSION: These injecting recommendations provide practical guidance and fill an important gap in diabetes management. If followed, they should help ensure comfortable, effective and largely complication-free injections.


Subject(s)
Diabetes Mellitus/drug therapy , Hypoglycemic Agents/administration & dosage , Injections, Subcutaneous , Insulin/administration & dosage , Adolescent , Adult , Blood Glucose/metabolism , Child , Consensus Development Conferences as Topic , Diabetes Mellitus/blood , Diabetes Mellitus, Type 1/drug therapy , Drug Storage , Evidence-Based Medicine , Expert Testimony , Female , Humans , Hypertrophy/etiology , Hypertrophy/prevention & control , Injections, Subcutaneous/adverse effects , Injections, Subcutaneous/instrumentation , Injections, Subcutaneous/methods , Injections, Subcutaneous/psychology , Insulin/analogs & derivatives , Needles , Needlestick Injuries/etiology , Needlestick Injuries/prevention & control , Patient Education as Topic , Pregnancy , Pregnancy Complications/prevention & control , Subcutaneous Fat, Abdominal/injuries , Subcutaneous Fat, Abdominal/pathology , Syringes
14.
Acta Anaesthesiol Scand ; 52(6): 798-804, 2008 Jul.
Article in English | MEDLINE | ID: mdl-18477072

ABSTRACT

BACKGROUND: Peripheral intravenous catheters are among the most widely used medical devices in the world. European patients are increasingly aware of the risk of health care associated infections and the role catheters play in their facilitation. AIMS: We intend to show that European health care providers are increasingly aware of the occupational risks of bloodborne infections such as HIV and hepatitis which can be transmitted by the needles from catheters and that the political will is building to take action to ensure safer devices are provided. METHODS: We review the wide variety of peripheral intravenous catheters which are specially engineered to reduce these risks. RESULTS: Available safety devices include spring-loaded retractable needles, guards that shield the dangerous tips and closed, needle-free access valves for intravenous sets. CONCLUSIONS: It is no longer necessary for patients and professionals to take risks to health and life when solutions which minimize these risks are at hand.


Subject(s)
Catheterization, Peripheral/instrumentation , Needlestick Injuries/prevention & control , Catheterization, Peripheral/standards , Equipment Design , Equipment Safety/standards , Europe , Humans
15.
Chirurg ; 79(8): 745-52, 2008 Aug.
Article in German | MEDLINE | ID: mdl-18437326

ABSTRACT

OBJECTIVES: Ruptured abdominal aortic aneurysms (rAAA) still represent a life-threatening vascular disease, with high mortality despite improved diagnostic tools and perioperative patient management. The aim of this study was to reveal predictors of perioperative mortality and survival after open (conventional) rAAA repair. PATIENTS AND METHODS: We analyzed data from our department containing 67 patient histories and clinical notes which were collected between January 1984 and December 2004. The study patients underwent emergent surgery for rAAA. In these cases we defined 72 preoperative, 47 intraoperative, and 39 postoperative variables for further analysis. RESULTS: Our results indicate that the worst survival prognosis could be defined in patients with rAAA and aneurysmatic inclusion of the iliac arteries with concomitant prolonged shock who received an aorto-iliac bypass. For these patients we calculated a cumulative 30-day survival rate of 59.7% and 1-year survival of 43.3%. An influence of age and comorbidity on the mortality rate could not be proven. Furthermore the conclusion cannot be drawn that postoperative course was influenced by intra- vs retroperitoneal rupture localization. CONCLUSION: This study provides evidence that neither old patient age nor comorbidities influence the mortality of patients suffering from rAAA, for whom time-consuming case selection according to previous morbidities should therefore be omitted. Instead we recommend conventional surgical repair as soon as possible to maximize the chances of survival.


Subject(s)
Aortic Aneurysm, Abdominal/surgery , Aortic Rupture/surgery , Intraoperative Complications/mortality , Postoperative Complications/mortality , Aged , Aged, 80 and over , Aortic Aneurysm, Abdominal/diagnosis , Aortic Aneurysm, Abdominal/mortality , Aortic Rupture/diagnosis , Blood Vessel Prosthesis Implantation , Female , Germany , Health Status Indicators , Hospital Mortality , Humans , Male , Middle Aged , Risk Factors , Survival Rate
16.
J Insect Physiol ; 53(7): 649-55, 2007 Jul.
Article in English | MEDLINE | ID: mdl-17543984

ABSTRACT

Protein-rich diets are known to promote ovarian and egg development in workers of the honeybee, Apis mellifera, even in the presence of a queen. Since the main source of protein for honeybees is pollen, its quality and digestibility might be important dietary factors determining reproductive capacity. We have compared the effect of two types of pollen-sunflower, Helianthus annuus, and aloe, Aloe greatheadii var davyana-on ovarian development in A. mellifera scutellata workers. Under queenright conditions in the field, worker bees exhibited greater ovarian development when feeding on aloe pollen than on sunflower pollen. In their midgut, we observed higher extraction efficiency for aloe (80%) than for sunflower (69%) pollen. This may be attributed to the morphology and size of the two kinds of pollen grains and explains, together with the high protein content of aloe pollen (32% dry mass in bee-collected pollen) compared to sunflower pollen (15%), why aloe pollen promoted higher ovarian development. However, in the laboratory workers sustained on aloe pollen had significantly less-developed ovaries and higher mortality than those fed sunflower pollen. These detrimental effects may be due to an unbalanced protein:carbohydrate ratio. We discuss the effects of unbalanced diets on the physiology and ecology of honeybee reproduction.


Subject(s)
Animal Feed , Bees/growth & development , Ovary/growth & development , Pollen/chemistry , Animals , Dietary Proteins , Female , Microscopy, Electron, Scanning , Pollen/ultrastructure , Social Behavior
17.
Acta Anaesthesiol Belg ; 58(1): 19-25, 2007.
Article in English | MEDLINE | ID: mdl-17486920

ABSTRACT

Up to eighty percent of all patients admitted to hospital worldwide will receive a peripheral IV and this procedure is now considered indispensable to human health. However, despite its global use, the choice of catheter is not always governed by clear and universal guidelines. After reviewing the few best-practice recommendations which exist, we propose a patient--and therapy--driven matrix for deciding on the gauge and length of peripheral catheter for the individual patient. This matrix takes patient age, clinical stability, current state of veins, therapy duration and the nature of the medication to be delivered into consideration. Use of such a matrix will not deliver a formulaic answer but will orient choices along logical, evidence-based lines. This approach will be an advance on the all-too-common reliance on habit and tradition in the choice of peripheral IV catheter.


Subject(s)
Catheterization, Peripheral/instrumentation , Practice Guidelines as Topic , Anti-Bacterial Agents/administration & dosage , Antineoplastic Agents/administration & dosage , Blood Transfusion/instrumentation , Blood Transfusion/standards , Catheterization, Peripheral/adverse effects , Catheterization, Peripheral/standards , Evidence-Based Medicine , Humans , Infusions, Intravenous/instrumentation , Parenteral Nutrition/instrumentation , Parenteral Nutrition/standards , Phlebitis/etiology , Resuscitation/instrumentation , Resuscitation/standards
19.
Am J Transplant ; 6(3): 557-64, 2006 Mar.
Article in English | MEDLINE | ID: mdl-16468966

ABSTRACT

An 8.5-year-old girl with classical maple syrup urine disease (MSUD) required liver transplantation for hypervitaminosis A and was effectively cured of MSUD over an 8-year clinical follow-up period. We developed a collaborative multidisciplinary effort to evaluate the effects of elective liver transplantation in 10 additional children (age range 1.9-20.5 years) with classical MSUD. Patients were transplanted with whole cadaveric livers under a protocol designed to optimize safe pre- and post-transplant management of MSUD. All patients are alive and well with normal allograft function after 106 months of follow-up in the index patient and a median follow-up period of 14 months (range 4-18 months) in the 10 remaining patients. Leucine, isoleucine and valine levels stabilized within 6 hours post-transplant and remained so on an unrestricted protein intake in all patients. Metabolic cure was documented as a sustained increase in weight-adjusted leucine tolerance, normalization of plasma concentration relationships among branched-chain and other essential and nonessential amino acids, and metabolic and clinical stability during protein loading and intercurrent illnesses. Costs and risks associated with surgery and immune suppression were similar to other pediatric liver transplant populations.


Subject(s)
Elective Surgical Procedures/methods , Liver Transplantation , Maple Syrup Urine Disease/surgery , Adolescent , Adult , Child , Child, Preschool , Female , Follow-Up Studies , Humans , Infant , Leucine/blood , Maple Syrup Urine Disease/blood , Time Factors , Treatment Outcome
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