PEDIATRICS recent issues

Sleep Problems in 2- to 5-Year-Olds With Autism Spectrum Disorder and Other Developmental Delays

BACKGROUND:

Sleep problems can impact daytime behavior, quality of life, and overall health. We compared sleep habits in young children with autism spectrum disorder (ASD) and other developmental delays and disorders and in children from the general population (POP).

METHODS:

We included 2- to 5-year-old children whose parent completed all items on the Children’s Sleep Habits Questionnaire (CSHQ) in a multisite case-control study: 522 children with ASD; 228 children with other developmental delays and disorders with autism spectrum disorder characteristics (DD w/ASD); 534 children with other developmental delays and disorders without autism spectrum disorder characteristics (DD w/o ASD); and 703 POP. Multivariable analysis of variance compared CSHQ mean total score (TS) and subscale scores between groups. Logistic regression analysis examined group differences by using TS cutoffs of 41 and 48. Analyses were adjusted for covariates.

RESULTS:

Mean CSHQ TS for children in each group: ASD (48.5); DD w/ASD (50.4); DD w/o ASD (44.4); and POP (43.3). Differences between children with ASD and both children with DD w/o ASD and POP were statistically significant. Using a TS cutoff of 48, the proportion of children with sleep problems was significantly higher in children in the ASD group versus DD w/o ASD and POP groups (adjusted odds ratios [95% confidence intervals]: 2.12 [1.57 to 2.87] and 2.37 [1.75 to 3.22], respectively).

CONCLUSIONS:

Sleep problems are more than twice as common in young children with ASD and DD w/ASD. Screening for sleep problems is important in young children to facilitate provision of appropriate interventions.

Vomiting and Dehydration in a 2-Year-Old

A 2-year-old girl with a past medical history of cutaneous mastocytosis and eczema presented with 1 day of yellow-green, nonbloody vomiting, bradycardia, and listlessness. She was evaluated by her pediatrician and sent to the emergency department because of concern for dehydration. In the emergency department, she improved with fluid rehydration but still had decreased energy and bradycardia. Her electrocardiogram revealed sinus bradycardia, and laboratory results did not reveal any electrolyte abnormalities. Glucose levels were normal. An abdominal radiograph revealed a moderate-to-large stool burden, and the results of a computed tomography scan of the head were normal. An abdominal ultrasound was obtained to evaluate for intussusception. The ultrasound revealed a blind-ending tubular structure in the right-lower quadrant with adjacent free fluid, which was concerning for appendicitis. The patient was admitted to the surgical service for further management and was taken to the operating room, where a definitive diagnosis was made.

Infant Regulation and Child Mental Health Concerns: A Longitudinal Study

OBJECTIVES:

To examine profiles of infant regulatory behaviors and associated family characteristics in a community sample of 12-month-old infants and mental health difficulties at 5 and 11 years of age.

METHODS:

Items relating to demographic characteristics, maternal distress, and infant regulation were completed by 1759 mothers when their infants were 8 to 12 months old. The Strengths and Difficulties Questionnaire was completed by mothers at child ages 5 (n = 1002) and 11 (n = 871) years.

RESULTS:

Analyses revealed 5 profiles ranging from the most settled infants (36.8%) to those with mainly sleep problems (25.4%), isolated mild-to-moderate tantrums (21.3%), complex regulatory difficulties (13.2%), and complex and severe regulatory difficulties (3.4%). Compared with those in the settled profile, children in the moderately unsettled profile were more likely to score in the clinical range for total difficulties at 11 years of age (odds ratio [OR] 2.85; 95% confidence interval [CI]: 1.28 to 6.36; P < .01), and children in the severely unsettled profile were more likely to score in the clinical range at 5 (OR 9.35; 95% CI: 2.49 to 35.11; P < .01) and 11 years of age (OR 10.37; 95% CI: 3.74 to 28.70; P < .01).

CONCLUSIONS:

Infants with multiple moderate-to-severe regulatory problems experience substantially heightened odds of clinically significant mental health concerns during childhood, and these symptoms appear to worsen over time. Clinicians must inquire about the extent, complexity, and severity of infant regulatory problems to identify those in the most urgent need of intervention and support.

Anaphylaxis to Carboxymethylcellulose: Add Food Additives to the List of Elicitors

A 14-year-old girl developed 4 episodes of anaphylaxis of unknown etiology, which required intramuscular adrenaline administration each time. She had eaten pizza and a cheeseburger immediately before the first 2 episodes, respectively, but had not eaten anything for several hours before the last 2 episodes. It turned out that she had eaten the same ice lolly 4 hours before the first 3 episodes and a Café au lait Swirkle (a half-frozen beverage) 4 hours before the last episode. We detected carboxymethylcellulose sodium as the only common ingredient in all anaphylactic episodes. Skin prick tests were positive for carboxymethylcellulose solution and carboxymethylcellulose-containing food products. We obtained a custom-made carboxymethylcellulose sodium–free ice lolly from the manufacturer and confirmed that it did not induce anaphylactic reactions by a challenge test. Carboxymethylcellulose, an anionic water-soluble polymer derived from native cellulose, is considered to be unabsorbable from the human gut and has been widely and increasingly used in pharmaceutical preparations, cosmetics, and food. This article is the first report of anaphylaxis caused by carboxymethylcellulose-containing foods, whereas anaphylaxis to carboxymethylcellulose has been rarely associated with carboxymethylcellulose-containing pharmaceuticals. Although the exact mechanisms underlying the induction of late-onset anaphylaxis by carboxymethylcellulose remain unclear, a small minority of cellulose-digesting microbial flora in the human colon and contamination of food products with carboxymethylcellulose of low molecular weight might be involved. The induction of recurrent anaphylaxis by various products should be a clue that prompts physicians to suspect food additives as a cause for anaphylaxis.

Reducing Invasive Care for Low-risk Febrile Infants Through Implementation of a Clinical Pathway

BACKGROUND AND OBJECTIVES:

Significant variation in management of febrile infants exists both nationally and within our institution. Risk stratification can be used to identify low-risk infants who can be managed as outpatients without lumbar puncture (LP) or antibiotics. Our objective was to reduce invasive interventions for febrile infants aged 29 to 60 days at low risk for serious bacterial infection (SBI) through implementation of a clinical pathway supported by quality improvement (QI).

METHODS:

The evidence-based clinical pathway was developed and implemented by a multidisciplinary team with continuous-process QI to sustain use. Low-risk infants who underwent LP, received antibiotics, and were admitted to the hospital were compared pre- and postpathway implementation with SBI in low-risk infants and appropriate care for high-risk infants as balancing measures.

RESULTS:

Of 350 included patients, 220 were pre- and 130 were postpathway implementation. With pathway implementation in July 2016, invasive interventions decreased significantly in low-risk infants, with LPs decreasing from 32% to 0%, antibiotic administration from 30% to 1%, and hospital admission from 17% to 2%. Postimplementation, there were 0 SBIs in low-risk infants versus 29.2% in high-risk infants. The percentage of high-risk patients receiving care per pathway remained unchanged. Improvement was sustained for 12 months through QI interventions, including order-set development and e-mail reminders.

CONCLUSIONS:

Implementation of a clinical pathway by using QI methods resulted in sustained reduction in invasive interventions for low-risk febrile infants without missed SBIs. Clinical pathways and QI can be key strategies in the delivery of evidence-based care for febrile infants.

The Potential Benefits of Research May Justify Certain Research Risks

US regulations allow institutional review boards to approve pediatric clinical trials only when the risks are minimal or (in some cases) a minor increase over minimal, or when the risks are justified by a potential for direct benefit to the participants. But how should an institutional review board determine if the risks of pediatric clinical trials are justified by a potential for participant benefit? In this Ethics Rounds article, we consider which potential benefits can justify which research risks with a focus on randomized clinical trials.

Predictive Validity of a 2-Question Alcohol Screen at 1-, 2-, and 3-Year Follow-up

BACKGROUND:

The National Institute on Alcohol Abuse and Alcoholism (NIAAA) 2-question screen is a valid adolescent alcohol screening tool. No studies have examined if this tool predicts future alcohol problems. We conducted a study at 16 pediatric emergency departments to determine the tool’s predictive validity for alcohol misuse and alcohol use disorders (AUDs).

METHODS:

Participants (N = 4834) completed a baseline assessment battery. A subsample of participants completed the battery at 1, 2, and 3 years follow up.

RESULTS:

Of the 2209 participants assigned to follow-up, 1611 (73%) completed a 1-year follow-up, 1591 (72%) completed a 2-year follow-up, and 1377 (62%) completed a 3-year follow-up. The differences in AUDs between baseline NIAAA screen nondrinkers and lower-risk drinkers were statistically significant at 1 year (P = .0002), 2 years (P <.0001), and 3 years (P = .0005), as were the differences between moderate- and highest-risk drinkers at 1 and 2 years (P < .0001 and P = .0088, respectively) but not at 3 years (P = .0758). The best combined score for sensitivity (86.2% at 1 year, 75.6% at 2 years, and 60.0% at 3 years) and specificity (78.1% at 1 year, 79.2% at 2 years, and 80.0% at 3 years) was achieved by using "lower risk" and higher as a cutoff for the prediction of a Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition diagnosis.

CONCLUSIONS:

The NIAAA 2-question screen can accurately characterize adolescent risk for future AUDs. Future studies are needed to determine optimaluse of the screen.

Childhood Assets and Cardiometabolic Health in Adolescence

BACKGROUND:

Research shows that the development of cardiometabolic disease can begin early in life with risk factors accumulating over time, but less is known about protective pathways to positive health. In this study, we use prospective data to test whether childhood assets predict a greater likelihood of being in optimal cardiometabolic health by age 17.

METHODS:

Data are from 3074 participants in the Avon Longitudinal Study of Parents and Children (mean age = 17.8). Four childhood assets were prospectively assessed via cognitive tests and parent report when children were between ages 8 and 10: strong executive functioning skills, prosocial behaviors, and low levels of internalizing and externalizing problems. Cardiometabolic health was assessed at ages 9 and 17 by using a composite dysregulation score derived from multiple biological parameters, including cholesterol, blood pressure, C-reactive protein, insulin resistance, and BMI. Associations between assets and optimal health at age 17 (ie, a dysregulation score of ≤1) were evaluated with Poisson regression models with robust error variances.

RESULTS:

After controlling for covariates (including sociodemographics, correlates of cardiometabolic health, and dysregulation scores at age 9), participants with multiple assets were 1.08 to 1.27 times more likely to be in optimal cardiometabolic health at age 17 compared with those with 0 or 1 asset. Each additional asset conferred a 6% greater likelihood of optimal health over time (relative risk = 1.06 [95% confidence interval: 1.01 to 1.11]).

CONCLUSIONS:

Childhood assets predicted cardiometabolic health with seemingly cumulative impacts. Identifying early assets may provide novel targets for prevention and elucidate pathways to positive adult health.

Food and Beverage Intake From 12 to 23 Months by WIC Status

BACKGROUND:

In 2009, the Special Supplemental Nutrition Program for Women, Infants, and Children (WIC) revised its food packages to align with updated nutrition science. Understanding how these revisions may impact current consumption patterns could be important.

METHODS:

Dietary data from the 2011–2014 NHANES were used to estimate the percentage of children who were aged 12 to 23 months consuming selected food and beverage categories on any given day by age and WIC status (children who were on WIC, those who were eligible for but not receiving WIC benefits, and those who were not eligible for WIC).

RESULTS:

Consumption of food and beverage categories differed by WIC status. On a given day, a lower percentage of children who were eligible for but did not receive WIC benefits consumed vegetables (excluding white potatoes; 42.3%) and grains (76.5%) compared with children who were participating in WIC (vegetables [excluding white potatoes]: 60.4%; grains: 85.5%) and those who were not eligible for WIC benefits (vegetables [excluding white potatoes]: 58.1%; grains: 87.2%; P < .05). A lower percentage of both children who were eligible for but not receiving WIC benefits and those who were participating in WIC consumed fruits (57.6% and 70.6%, respectively) and snacks (45.9% and 48.5%, respectively) than those who were not eligible for WIC (fruits: 86.4%; snacks: 69.1%; P < .05). A lower percentage of children who were receiving WIC consumed dairy than children who were not eligible for WIC (91.7% and 97.2%, respectively; P < .05). A higher percentage of those who were receiving WIC consumed 100% juice (70.6%) than children who were eligible for but not receiving WIC (51.6%) and children who were not eligible for WIC (50.8%; P < .05).

CONCLUSIONS:

Improving early WIC participation and retention could positively impact some diet-related disparities among young children who are eligible for WIC.

Duration of Initial Empirical Antibiotic Therapy and Outcomes in Very Low Birth Weight Infants

BACKGROUND:

Overuse of antibiotics can facilitate antibiotic resistance and is associated with adverse neonatal outcomes. We studied the association between duration of antibiotic therapy and short-term outcomes of very low birth weight (VLBW) (<1500 g) infants without culture-proven sepsis.

METHODS:

We included VLBW infants admitted to NICUs in the Canadian Neonatal Network between 2010–2016 who were exposed to antibiotics but did not have culture-proven sepsis in the first week. Antibiotic exposure was calculated as the number of days an infant received antibiotics in the first week of life. Composite primary outcome was defined as mortality or any major morbidity (severe neurologic injury, retinopathy of prematurity, necrotizing enterocolitis, chronic lung disease, or hospital-acquired infection).

RESULTS:

Of the 14 207 included infants, 21% (n = 2950), 38% (n = 5401), and 41% (n = 5856) received 0, 1 to 3, and 4 to 7 days of antibiotics, respectively. Antibiotic exposure for 4 to 7 days was associated with higher odds of the composite outcome (adjusted odds ratio 1.24; 95% confidence interval [CI] 1.09–1.41). Each additional day of antibiotic use was associated with 4.7% (95% CI 2.6%–6.8%) increased odds of composite outcome and 7.3% (95% CI 3.3%–11.4%) increased odds in VLBW infants at low risk of early-onset sepsis (born via cesarean delivery, without labor and without chorioamnionitis).

CONCLUSIONS:

Prolonged empirical antibiotic exposure within the first week after birth in VLBW infants is associated with increased odds of the composite outcome. This practice is a potential target for antimicrobial stewardship.

A Parenting and Life Skills Intervention for Teen Mothers: A Randomized Controlled Trial

BACKGROUND:

Teen mothers often present with depression, social complexity, and inadequate parenting skills. Many have rapid repeat pregnancy, which increases risk for poor outcomes. We conducted a randomized controlled trial of a parenting and life skills intervention for teen mothers aimed at impacting parenting and reproductive outcomes.

METHODS:

Teen mothers were recruited from a teen-tot clinic with integrated medical care and social services. Participants were randomly assigned 1:1 to receive (1) teen-tot services plus 5 interactive parenting and life skills modules adapted from the Nurturing and Ansell-Casey Life Skills curricula, delivered by a nurse and social worker over the infant’s first 15 months or (2) teen-tot services alone. A computerized questionnaire was self-administered at intake, 12, 24, and 36 months. Outcomes included maternal self-esteem, parenting attitudes associated with child maltreatment risk, maternal depression, life skills, and repeat pregnancy over a 36-month follow-up. We used generalized linear mixed modeling and logistic regression to examine intervention effects.

RESULTS:

Of 152 invited, 140 (92%) participated (intervention = 72; control = 68). At 36 months, maternal self-esteem was higher in the intervention group compared with controls (P = .011), with higher scores on preparedness for mothering role (P = .011), acceptance of infant (P = .008), and expected relationship with infant (P = .029). Repeat pregnancy by 36 months was significantly lower for intervention versus control participants.

CONCLUSIONS:

A brief parenting and/or life skills intervention paired with medical care for teens and their children has positive effects on maternal self-esteem and repeat pregnancy over 36 months.

Monoclonal Antibody Treatment of RSV Bronchiolitis in Young Infants: A Randomized Trial

BACKGROUND:

Monoclonal antibody to respiratory syncytial virus (RSV; palivizumab) is recommend for prophylaxis of high-risk infants during bronchiolitis seasons but not for RSV bronchiolitis treatment. Our aim was to determine if palivizumab would be helpful in young infants with acute RSV bronchiolitis.

METHODS:

Eligible infants ≤3 months old presenting to the pediatric emergency service with RSV-positive bronchiolitis requiring inpatient admission underwent double-blind random assignment to single-dose intravenous palivizumab (15 mg/kg) or placebo. The primary efficacy outcome was the need for inpatient readmission in the 3 weeks after discharge. Secondary outcomes were time to readiness for hospital discharge, need for PICU on the initial admission, and need for revisit not requiring readmission for the same illness during 3-week follow-up.

RESULTS:

A total of 420 infants (median age 49 days) diagnosed with RSV bronchiolitis were randomly assigned; 417 received treatment, and 413 completed follow-up. Readmission during follow-up was needed for 23 (11%) patients on palivizumab and 19 (9.3%) patients in the placebo group (difference 1.8%; 95% confidence interval –4.4% to 7.7%; P = .51). Geometric mean time to readiness for discharge was 29.5 hours for the palivizumab group and 30.2 hours for the placebo group (ratio 0.98; 95% confidence interval 0.81 to 1.20). No safety issues were reported.

CONCLUSIONS:

Intravenous palivizumab did not appear to help or harm young infants with acute RSV-positive bronchiolitis.

Nonmedical Prescription Opioid Use by Parents and Adolescents in the US

BACKGROUND:

To date, intergenerational patterns of nonmedical prescription opioid (NMPO) use have not been examined. We investigate the association between parental and adolescent NMPO use in the United States.

METHODS:

Data are from 35 000 parent-child dyads with an adolescent aged 12 to 17 years from the 2004–2012 nationally representative National Surveys on Drug Use and Health. Using multivariable logistic regression models, we estimated the association between self-reported parental and adolescent lifetime NMPO use, controlling for parental and adolescent use of other drugs, attitudes about drug use, parental and adolescent psychosocial risk factors, and sociodemographic characteristics.

RESULTS:

Controlling for other factors, parental NMPO use was associated with adolescent NMPO use (adjusted odds ratio [aOR] 1.30; 95% confidence interval [CI] 1.09–1.56). Mothers’ use had a stronger association with adolescent use than fathers’ use (aOR 1.62 [95% CI 1.28–2.056] versus aOR 0.98 [95% CI 0.74–1.24]). Associations between parental and adolescent NMPO use did not differ by adolescent sex or race and/or ethnicity. Parental lifetime smoking, low monitoring, and parent-adolescent conflict were uniquely associated with adolescent NMPO use (aOR 1.19–1.24) as were adolescent smoking, marijuana use, depression, delinquency, and perceived schoolmates’ drug use (aOR 1.25–1.71). Perceived risk of drug use and religiosity were associated with lower rates of adolescent NMPO use (aOR 0.77–0.93). Use among older adolescents was higher than among younger adolescents (aOR 1.27; 95% CI 1.21–1.34).

CONCLUSIONS:

Parent-based interventions targeted at adolescent NMPO use should address parental NMPO use and smoking and promote positive parenting.

Giant Congenital Melanocytic Nevus Treated With Trametinib

Giant congenital nevi are melanocytic proliferations of the skin that may be complicated by melanoma, neurocutaneous melanocytosis, pain, pruritus, and disfigurement. Current treatment options include surgical resection and medical management of associated symptoms. There is limited efficacy in these modalities. No effective pharmacologic treatments are available for the treatment of these lesions. We present the case of a 7-year-old girl with a giant congenital melanocytic nevus that had an AKAP9-BRAF fusion and was treated with trametinib, which resulted in rapid resolution of the patient’s lifelong, intractable pain and pruritus as well as dramatic improvement in the extent of her nevus.

Factors Associated With Unrestrained Young Passengers in Motor Vehicle Crashes

BACKGROUND AND OBJECTIVE:

Unrestrained child passengers are at significant risk of crash-related injury. Previous researchers using nationally representative crash data from 1992 to 1993 found an association between driver and passenger safety-belt use. Our objective in this study is to investigate factors associated with young, unrestrained passengers in fatal and nonfatal motor vehicle crashes using updated national crash data.

METHODS:

We analyzed 2011–2015 Fatality Analysis Reporting System and National Automotive Sampling System data and included vehicles with a young passenger (≤19 years old) in a crash. Driver and passenger characteristics were compared by using bivariate analyses separately for fatal and nonfatal crashes. Logistic regression analyses were performed on a combined data set to predict passenger restraint use.

RESULTS:

In unadjusted bivariate models, unrestrained drivers had a higher probability of having an unrestrained passenger across all passenger age groups for both fatal and nonfatal crashes. In multivariate logistic regression models that included both fatal and nonfatal crashes and were adjusted for several driver and passenger characteristics, unrestrained drivers had a higher risk of having an unrestrained young passenger across all age groups.

CONCLUSIONS:

In both fatal and nonfatal crashes, a driver being unrestrained is a strong predictor of the child passenger also being unrestrained. Policy and regulation to better ensure that drivers are properly restrained (eg, expanding primary seat-belt laws to all states) may serve as effective means for increasing rates of proper child-occupant–restraint use.

Early Use of Anti-influenza Medications in Hospitalized Children With Tracheostomy

BACKGROUND:

Early administration of anti-influenza medications is recommended for all children hospitalized with influenza. We investigated whether early use of anti-influenza medications is associated with improved outcomes in children with tracheostomy hospitalized with influenza.

METHODS:

We performed a multicenter retrospective cohort study through the Pediatric Health Information System database for patients aged 30 days to 19 years who were discharged between October 1, 2007, and September 30, 2015 with diagnostic codes for both influenza and tracheostomy. Our primary predictor was receipt of anti-influenza medications on hospital day 0 or 1. We used propensity score matching to adjust for confounding by indication. Primary outcomes were length of stay (LOS) and 30-day all-cause revisit rate (emergency department visit or hospital admission).

RESULTS:

Of 1436 discharges screened, 899 met inclusion criteria. The median admission age was 5 years (interquartile range: 2–10). The majority had multiple complex chronic conditions (median 3; interquartile range: 3–4) and technology dependence, such as gastrostomy tube (73.6%). After matching 772 unique admissions by propensity score, LOS was shorter for the cohort receiving early anti-influenza medications (6.4 vs 7.5 days; P = .01) without increase in revisit rate (27.5% vs 24.1%; P = .28). More than 80% in both cohorts received empirical antibiotics, and the duration of antibiotic therapy was similar (5.0 vs 5.6 days; P = .11).

CONCLUSIONS:

Early use of anti-influenza medications in children with tracheostomy hospitalized with influenza is associated with shorter LOS, but these children continue to receive antibiotics despite identification and treatment of their viral infections.

Advanced Ultrasound Techniques for Pediatric Imaging

Ultrasound has become a useful tool in the workup of pediatric patients because of the highly convenient, cost-effective, and safe nature of the examination. With rapid advancements in anatomic and functional ultrasound techniques over the recent years, the diagnostic and interventional utility of ultrasound has risen tremendously. Advanced ultrasound techniques constitute a suite of new technologies that employ microbubbles to provide contrast and enhance flow visualization, elastography to measure tissue stiffness, ultrafast Doppler to deliver high spatiotemporal resolution of flow, three- and four-dimensional technique to generate accurate spatiotemporal representation of anatomy, and high-frequency imaging to delineate anatomic structures at a resolution down to 30 μm. Application of these techniques can enhance the diagnosis of organ injury, viable tumor, and vascular pathologies at bedside. This has significant clinical implications in pediatric patients who are not easy candidates for lengthy MRI or radiation-requiring examination, and are also in need of a highly sensitive bedside technique for therapeutic guidance. To best use the currently available, advanced ultrasound techniques for pediatric patients, it is necessary to understand the diagnostic utility of each technique. In this review, we will educate the readers of emerging ultrasound techniques and their respective clinical applications.

Renal Replacement Therapy and Intermittent Catheterization Risk in Posterior Urethral Valves

OBJECTIVES:

Posterior urethral valves predispose children to renal replacement therapy (RRT) and bladder dysfunction. Researchers of single-institutional series were unable to refine risk stratification because of rarity of the disease. We aimed to identify clinical variables associated with the risk of RRT and clean intermittent catheterization (CIC) in a large multicenter cohort study.

METHODS:

Children with posterior urethral valves born between 1995 and 2005 who were treated before 90 days of life at 5 children’s hospitals were retrospectively reviewed. Outcomes included RRT and recommendation for CIC. Predictors and outcomes were assessed by using survival analysis.

RESULTS:

A total of 274 patients were managed for a median of 6.3 years, and 42 progressed to RRT. On survival analysis, 16% progressed to RRT by 10 years of age. RRT varied by the serum nadir creatinine level in the first year of life (SNC1) (log-rank P < .001). After stratifying by the SNC1, the estimated risk of progressing to RRT by 10 years of age was 0%, 2%, 27%, and 100% for an SNC1 of <0.4, an SNC1 of 0.4 to 0.69, an SNC1 of 0.7 to 0.99, and an SNC1 of ≥1.0 mg/dL, respectively. CIC was recommended in 60 patients, which translated on survival analysis to a risk of 26% by 10 years of age.

CONCLUSIONS:

Risk of RRT and CIC recommendation increased with age. The SNC1 strongly predicted need for RRT. These results allow for both improved family counseling and the potential for more appropriate screening and intervention strategies for those identified in higher-risk groups.

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