Transitional care models in adolescent kidney transplant recipients-a systematic review.

BACKGROUND: Adolescence is a time of significant change for patients, guardians and clinicians. The paediatrician must ensure patients develop the necessary skills and knowledge required to transition and to function as an independent entity, with autonomy over their own care. The transfer from paediatric to adult care carries an increased risk of graft-related complications attributable to a multitude of reasons, particularly non-adherence to immunosuppressive medicines and poor attendance at scheduled appointments. This systematic review was conducted to ascertain the transitional care models available to clinicians caring for kidney transplant recipients and to compare the approach in each respective case. METHODS: A systematic review was performed, in a methodology outlined by the PRISMA guidelines. OVID MEDLINE and EMBASE databases were searched for studies that outlined valid, replicable models pertaining to transitional care of paediatric kidney transplant recipients between 1946 and Quarter 3 of 2021. The reference lists of selected articles were also perused for further eligible studies and experts in the field were consulted for further eligible articles. Two investigators assessed all studies for eligibility and independently performed data extraction. Any discrepancies were settled by consensus. RESULTS: A total of 1121 abstracts were identified, which was reduced to 1029 upon removal of duplicates. A total of 51 articles were deemed appropriate for full-text review and critical appraisal. A total of 12 articles that described models for transition pertaining to kidney transplant patients were included in qualitative synthesis. Every paper utilized a different transition model. All but one model included a physician and nurse at minimum in the transition process. The involvement of adult nephrologists, medical social work, psychology and psychiatry was variable. The mean age for the initiation of transition was 13.4 years (range: 10-17.5 years). The mean age at transfer to adult services was 18.3 years (range: 16-20.5 years). CONCLUSIONS: Despite the well-established need for good transitional care for paediatric solid-organ transplant recipients, models tailored specifically for kidney transplant recipients are lacking. Further research and validation studies are required to ascertain the best method of providing effective transitional care to these patients. Transitional care should become a standardized process for adolescents and young adults with kidney transplants.

Neurological involvement in children with hemolytic uremic syndrome.

Our objective was to establish the rate of neurological involvement in Shiga toxin-producing Escherichia coli-hemolytic uremic syndrome (STEC-HUS) and describe the clinical presentation, management and outcome. A retrospective chart review of children aged ≤ 16 years with STEC-HUS in Children's Health Ireland from 2005 to 2018 was conducted. Laboratory confirmation of STEC infection was required for inclusion. Neurological involvement was defined as encephalopathy, focal neurological deficit, and/or seizure activity. Data on clinical presentation, management, and outcome were collected. We identified 240 children with HUS; 202 had confirmed STEC infection. Neurological involvement occurred in 22 (11%). The most common presentation was seizures (73%). In the neurological group, 19 (86%) were treated with plasma exchange and/or eculizumab. Of the 21 surviving children with neurological involvement, 19 (91%) achieved a complete neurological recovery. A higher proportion of children in the neurological group had renal sequelae (27% vs. 12%, P = .031). One patient died from multi-organ failure.Conclusion: We have identified the rate of neurological involvement in a large cohort of children with STEC-HUS as 11%. Neurological involvement in STEC-HUS is associated with good long-term outcome (complete neurological recovery in 91%) and a low case-fatality rate (4.5%) in our cohort. What is Known: • HUS is associated with neurological involvement in up to 30% of cases. • Neurological involvement has been reported as predictor of poor outcome, with associated increased morbidity and mortality. What is New: • The incidence of neurological involvement in STEC-HUS is 11%. • Neurological involvement is associated with predominantly good long-term outcome (90%) and a reduced case-fatality rate (4.5%) compared to older reports.

Long-term outcome of transplant ureterostomy in children: A National Review.

BACKGROUND: CAKUT are the most common cause of end-stage renal failure in children (Pediatr Nephrol. 24, 2009, 1719). Many children with CAKUT have poor urinary drainage which can compromise post-transplant outcome. Identifying safe ways to manage anatomical abnormalities and provide effective urinary drainage is key to transplant success. Much debate exists regarding optimum urinary diversion techniques. The definitive formation of a continent urinary diversion is always preferable but may not always be possible. We explore the role of ureterostomy formation at transplantation in a complex pediatric group. METHODS: We report six pediatric patients who had ureterostomy formation at the time of transplantation at the National Paediatric Transplant Centre in Dublin, Ireland. We compared renal function and burden of urinary tract infection to a group with alternative urinary diversion procedures and a group with normal bladders over a 5-year period. RESULTS: There was no demonstrable difference in estimated glomerular filtration rate between the groups at 5-year follow-up. The overall burden of UTI was low and similar in frequency between the three groups. CONCLUSIONS: Ureterostomy formation is a safe and effective option for temporary urinary diversion in children with complex abdominal anatomy facilitating transplantation; it is, however, important to consider the implications and risk of ureterostomy for definitive surgery after transplantation.

Sexual Risk Behaviors during Adolescence: Associations with Childhood and Adolescent BMI.

Research examining associations between unhealthy weight and sexual risk behaviors derives primarily from cross-sectional studies conducted at varying ages during adolescence. The present study examined longitudinal associations between adolescent sexual risk behaviors and Body Mass Index (BMI), the latter measured prospectively from childhood through adolescence. Data were drawn from an 8095 individuals between the ages of 14 and 19 at last assessment, including 1768 Hispanic (52% female), 2659 non-Hispanic Black (Black; 50% female), and 3668 non-Hispanic, non-Black (predominantly White; 49% female). Childhood BMI predicted increased sexual risk-taking during adolescence for Black and White youth, and increases in BMI predicted increased sexual risk-taking for White females and Black males. The findings highlight differential patterns of association between BMI and sexual risk behaviors for Hispanic, Black, and White females and males with implications for prevention discussed.

Interactive Effects of Maternal Alcohol Problems and Parental Separation on Timing of Daughter's First Drink.

BACKGROUND: Few studies examine risk to offspring who experience both parental alcohol problems and parental separation and still fewer consider gender of the affected parent. We examined interactive effects of maternal versus paternal alcohol problems and parental separation on timing of first alcoholic drink in daughters. METHODS: Data were drawn from a sample of 3,539 European (or other) ancestry (EA) and 611 African ancestry (AA) female twins born between 1975 and 1985, median age 15 at first assessment. Cox proportional hazards regression models were estimated predicting age at first full drink from parental history of alcohol problems (mother only, father only, or both parents), parental separation during childhood, and the interaction of parental alcohol problems and parental separation. Cox models were estimated without and with adjustment for correlated risk factors, separately for EA and AA twins. RESULTS: For both EA and AA twins, a significant interaction between parental separation and mother-only alcohol problems was observed, suggesting reduced risk of drinking associated with mother-only alcohol problems in separated versus intact families. CONCLUSIONS: Findings highlight parental separation as an important moderator of risk to children of mothers who have a history of problem drinking, with interactive effects observed consistently across racial group. To identify underlying processes, additional research is needed with more detailed characterization of separated families where mother only has a history of alcohol problems.

A De Novo Deletion in the Regulators of Complement Activation Cluster Producing a Hybrid Complement Factor H/Complement Factor H-Related 3 Gene in Atypical Hemolytic Uremic Syndrome.

The regulators of complement activation cluster at chromosome 1q32 contains the complement factor H (CFH) and five complement factor H-related (CFHR) genes. This area of the genome arose from several large genomic duplications, and these low-copy repeats can cause genome instability in this region. Genomic disorders affecting these genes have been described in atypical hemolytic uremic syndrome, arising commonly through nonallelic homologous recombination. We describe a novel CFH/CFHR3 hybrid gene secondary to a de novo 6.3-kb deletion that arose through microhomology-mediated end joining rather than nonallelic homologous recombination. We confirmed a transcript from this hybrid gene and showed a secreted protein product that lacks the recognition domain of factor H and exhibits impaired cell surface complement regulation. The fact that the formation of this hybrid gene arose as a de novo event suggests that this cluster is a dynamic area of the genome in which additional genomic disorders may arise.

Parental Separation and Offspring Alcohol Involvement: Findings from Offspring of Alcoholic and Drug Dependent Twin Fathers.

BACKGROUND: We examined associations between parental separation during childhood and offspring alcohol involvement, adjusting for genetic and environmental risks specific to parental alcohol (AD) and cannabis/other illicit drug dependence (DD). METHODS: The sample consisted of 1,828 offspring of male twins from the Vietnam Era Twin (VET) Registry, who completed a telephone diagnostic interview. Cox proportional hazards regression analyses were conducted predicting onset of first use, transition from first use to first AD symptom, and transition from first use to AD diagnosis from paternal and avuncular AD and DD history, parental separation, and offspring and family background characteristics. Paternal/avuncular DD/AD was based on the DSM-III-R; offspring and maternal AD were based on DSM-IV criteria. RESULTS: Paternal DD/AD predicted increased offspring risk for all transitions, with genetic effects suggested on rate of transitioning to AD diagnosis. Parental separation was predictive of increased risk for early alcohol use, but a reduced rate of transition to both AD symptom onset and onset of AD. No interactions between separation and familial risk (indexed by paternal or avuncular DD/AD) were found. CONCLUSIONS: Findings highlight the contribution of both parental separation and paternal substance dependence in predicting timing of offspring alcohol initiation and problems across adolescence into early adulthood.

A novel hybrid CFH/CFHR3 gene generated by a microhomology-mediated deletion in familial atypical hemolytic uremic syndrome.

Genomic disorders affecting the genes encoding factor H (fH) and the 5 factor H related proteins have been described in association with atypical hemolytic uremic syndrome. These include deletions of CFHR3, CFHR1, and CFHR4 in association with fH autoantibodies and the formation of a hybrid CFH/CFHR1 gene. These occur through nonallelic homologous recombination secondary to the presence of large segmental duplications (macrohomology) in this region. Using multiplex ligation-dependent probe amplification to screen for such genomic disorders, we have identified a large atypical hemolytic uremic syndrome family where a deletion has occurred through microhomology-mediated end joining rather than nonallelic homologous recombination. In the 3 affected persons of this family, we have shown that the deletion results in formation of a CFH/CFHR3 gene. We have shown that the protein product of this is a 24 SCR protein that is secreted with normal fluid-phase activity but marked loss of complement regulation at cell surfaces despite increased heparin binding. In this study, we have therefore shown that microhomology in this area of chromosome 1 predisposes to disease associated genomic disorders and that the complement regulatory function of fH at the cell surface is critically dependent on the structural integrity of the whole molecule.

Human mate selection and addiction: a conceptual critique.

The authors review past work on modeling human mate selection, and suggest, using illustrations from existing literature on the impact of alcoholism on relationship formation and dissolution and reproduction, that the challenges of adequately characterizing human mate selection have not yet been overcome. Some paths forwards are suggested.

Genetic and environmental risk for major depression in African-American and European-American women.

It is unknown whether there are racial differences in the heritability of major depressive disorder (MDD) because most psychiatric genetic studies have been conducted in samples comprised largely of white non-Hispanics. To examine potential differences between African-American (AA) and European-American (EA) young adult women in (1) Diagnostic and Statistical Manual of Mental Disorders, 4th Edition (DSM-IV) MDD prevalence, symptomatology, and risk factors, and (2) genetic and/or environmental liability to MDD, we analyzed data from a large population-representative sample of twins ascertained from birth records (n = 550 AA and n = 3226 EA female twins) aged 18-28 years at the time of MDD assessment by semi-structured psychiatric interview. AA women were more likely to have MDD risk factors; however, there were no significant differences in lifetime MDD prevalence between AA and EA women after adjusting for covariates (odds ratio = 0.88, 95% confidence interval [CI]: 0.67-1.15). Most MDD risk factors identified among AA women were also associated with MDD at similar magnitudes among EA women. Although the MDD heritability point estimate was higher among AA women than EA women in a model with paths estimated separately by race (56%, 95% CI: 29-78% vs. 41%, 95% CI: 29-52%), the best fitting model was one in which additive genetic and non-shared environmental paths for AA and EA women were constrained to be equal (A = 43%, 33-53% and E = 57%, 47-67%). In spite of a marked elevation in the prevalence of environmental risk exposures related to MDD among AA women, there were no significant differences in lifetime prevalence or heritability of MDD between AA and EA young women.

Nested case-control study of the emergence of tigecycline resistance in multidrug-resistant Klebsiella pneumoniae.

We performed a nested case-control study (ratio of 1:4) on the emergence of tigecycline-resistant multidrug-resistant Klebsiella pneumoniae (TR-MDRKP) isolates among patients who initially presented with a tigecycline-susceptible MDRKP isolate. Out of 260 patients, 24 (9%) had a subsequent clinical culture positive for a TR-MDRKP isolate within the 90-day follow-up period. On logistic regression analyses, receipt of tigecycline (adjusted odds ratio [OR], 5.06; 95% confidence interval [CI], 1.80 to 14.23; P = 0.002) was the only independent predictor of subsequent isolation of a TR strain.

Improved scheduling operations in diagnostic imaging.

This article describes the transformation of the scheduling function at Anne Arundel Diagnostic Imaging in Maryland, which once generated the most complaints at the facility. The vital function of scheduling was inconsistent in its approach where variation could result in errors. Errors might have required rescheduling, resulting in lost revenue for the unused appointment slot, whereas a successful scheduling operation depends on quickly answered calls. An analysis of call volume, talk times, abandonment rates, and wait times was conducted. Improvements included the installation of a new phone system and workflow changes resulting in increased prompt answering of calls, employee satisfaction, and physician referrals.

Therapeutic plasma exchange in paediatric nephrology in Ireland.

BACKGROUND: Therapeutic plasma exchange (TPE) is utilised in the management of a limited number of paediatric renal conditions. Despite its widespread acceptance and advancements in the practice of apheresis, there remains a paucity of data pertaining to paediatrics. We present a large retrospective review of our cohort of paediatric patients undergoing TPE for renal indications, outlining their outcomes and complications. METHODS: A retrospective chart review was conducted for all patients (under 16 years) undergoing TPE for renal conditions between January 2002 and June 2019 in Ireland. Demographic and clinical data were extracted, with patients anonymised and stratified according to their pathology. RESULTS: A total of 58 patients were identified. A total of 1137 exchanges were performed using heparin sodium anticoagulation. The median age was 35.5 months (IQR 18-110 months). The leading indication was neurological involvement in Shiga toxin-producing Escherichia coli haemolytic uraemic syndrome (STEC-HUS) (n = 29). Complications (minor or major) occurred in 65.5% (n = 38) of patients, with most experiencing minor complications 58.6% (n = 34). Asymptomatic hypocalcaemia was the most common complication in 43.1% (n = 25). CONCLUSIONS: Our experience of TPE, spanning 1137 exchanges, proved a safe, well-tolerated therapy. Most complications were minor, and with therapy conducted in specialised centres, there are very low levels of adverse events.

The interpretability of family history reports of alcoholism in general community samples: findings in a midwestern U.S. twin birth cohort.

BACKGROUND: Although there is a long tradition in alcoholism research of using family history ratings, the interpretability of family history reports of alcoholism from general community samples has yet to be established. METHODS: Telephone interview data obtained from a large cohort of female like-sex twins (N = 3,787, median age 22) and their biological parents (N = 2,928, assessed at twins' median age 15) were analyzed to determine agreement between parent self-report, parent ratings of coparent, and twin narrow (alcohol problems) and broad (problem or excessive drinking) ratings of each parent. RESULTS: In European ancestry (EA) families, high tetrachoric correlations were observed between twin and cotwin ratings of parental alcohol problems, between twin and parent ratings of coparent alcohol problems using symptom-based and single-item assessments, as well as moderately high correlations between twin and both mother and father self-reports. In African American (AA) families, inter-rater agreement was substantially lower than for EA families, with no cases where father ratings of maternal alcohol problems agreed with either twin ratings or mother self-report, and both cotwin agreement and mother-twin agreement were reduced. Differences between EA and AA families were not explained by differences in years of cohabitation with father or mother's education; however, underreporting of problems by AA parents may have contributed. CONCLUSIONS: Results support the use of family history ratings of parental alcoholism in general community surveys for EA families, but suggest that family history assessment in AA families requires improved methods.

Urinary incontinence in children: botulinum toxin is a safe and effective treatment option.

PURPOSE: This study's aim was to assess the use of intravesical injection of botulinum neurotoxin type A (BoNT-A) as a treatment of overactive bladder (OAB) in children. METHODS: A 6-year retrospective study of children who received BoNT-A for OAB was performed. Treatment outcome was classified as complete success (CS), partial success (PS) or treatment failure (TF). RESULTS: Of the 57 patients who received BoNT-A treatment for OAB, 35 were males. CS occurred in 74.2% of males and 54.5% of females. PS was achieved in 20% of males and 18.2% of females. TF occurred in 2.9% of males and 22.7% of females. Anticholinergics had previously been used and had been effective in 58.6% and 83.3% of males and females. Significant side effects to medications were experienced in 12 (41.4%) males and 4 (22.2%) females. Of these, BoNT-A achieved CS in seven (53.3%) males and two (50%) females and PS in three (25%) males and one (25%) female. BoNT-A was successful in seven (58.3%) males and two (66.7%) females where anticholinergics were ineffective. CONCLUSIONS: BoNT-A has a role in a carefully selected subgroup of children with overactive bladder symptoms including those with medication side effects and treatment compliance issues. It may have a role in patients who do not respond to conventional therapy.

Alcoholic marriage: later start, sooner end.

BACKGROUND: Although associations between drinking behavior and marital status are well documented, timing of marital transitions as a function of alcohol use or disorder has received limited empirical attention. METHODS: We examine the relationship between lifetime history of alcohol dependence (AD) and timing and survival of first marriages in a sample of 3,575 female and 1,845 male adult Australian twins born mostly between 1940 and 1964. Survival analyses were conducted using Cox proportional hazards regression models. RESULTS: Results indicate moderate delays in marriage associated with AD for both women and men. Among ever-married respondents, AD was strongly predictive of early separation, with similar effects observed for women and men. Heritable sources of covariation were also documented. For women, genetic influences shared between early-onset AD and marital timing were found. Genetic influences shared between AD and marital survival were observed for women without regard to onset and for men with later-onset AD. CONCLUSIONS: Results confirm the importance of AD as a predictor of both timing and survival of first marriages, with genetic influences contributing to observed associations.

Living and coping with Parkinson's disease: perceptions of informal carers.

A review of the literature highlights the important role informal carers play in the provision of palliative care in the community. In order to explore the caring experience of relatives with Parkinson's Disease (PD), interviews were conducted with 26 informal family caregivers. Interviews were taped, transcribed and subjected to content analysis. All caregivers were spouses, the majority female (n = 17) and all were responsible for providing physical, social and emotional care in the home. Although they viewed care giving as their role and duty, the results highlight the widespread burden of providing care on the emotional and physical health of the caregivers. The financial implications for providing care were outlined, with many reporting difficulty in accessing benefits. From the point of diagnosis, which had a huge emotional impact on relatives and carers, carers did not feel health professionals integrated them within the caring journey. Since diagnosis, carers commented on the lack of continued and coordinated care plans for relatives, resulting in symptoms being mismanaged and care opportunities for relatives and carers missed. Stereotypes of the meaning and timing of palliative care were common with many viewing it as being synonymous with cancer and not applicable to a person with PD. As the well-being of the informal carer directly influences the care of the person with PD, support interventions are required to relieve their burden, maximize outcomes and ensure targeting of services.

Palliative care in Parkinson's disease.

Parkinson's disease is a progressive, neurodegenerative condition with no known cure. People with the condition can have complex palliative care needs similar to those of cancer patients. This article describes the four stages of Parkinson's disease, and illustrates how nurses can apply the principles of palliative care to support and care at each stage.

Timing of mortality in mothers with recurrent convictions for driving under the influence of alcohol and their children, from childbirth to child age 17.

BACKGROUND: We tested variation in the timing of child and maternal mortality associated with severe maternal AUD, as represented by recurrent arrests for driving under the influence of alcohol (rDUI). METHODS: rDUI mothers (N = 1614) and Controls with no alcohol-related driving offenses (N = 109,928) who gave birth in Missouri from 2000 to 2004 were identified using vital records. Propensity score matching adjusted for birth record measures including delayed prenatal care, smoking during pregnancy, relationship with reproductive partner [married/unmarried, paternity acknowledged/unacknowledged], partner DUI status from driving records, and for socioeconomic characteristics of maternal residential census tract at birth derived from census data. Survival analysis was used to test months from childbirth to child or maternal death as a function of lifetime rDUI status. RESULTS: Maternal rDUIs were associated with a consistently elevated probability of child mortality from birth through child age 17 after propensity score-adjustment (Hazard Ratio [HR] = 1.70, 95 % CI = 1.17-2.47). Maternal mortality was not elevated, relative to Controls, until child age 6-11 (HR = 1.58, 95 % CI = 1.05-2.35) and increased again from child age 12-17 (HR = 4.12, 95 % CI = 3.04-5.86). CONCLUSIONS: Severe maternal AUD, as characterized by rDUI, increases the risk for child mortality over that of Controls through age 17. Delays in rDUI maternal mortality until child age 6 may indicate a period when maternal referral for intervention to reduce harm to child and mother is likely to be especially effective.