Jitteriness and tremors in the newborn period typically precipitate an extensive, invasive, and expensive search for the etiology. Vitamin D deficiency has not been historically included in the differential of tremors. We report a shivering, jittery newborn who was subjected to a battery of testing, with the only biochemical abnormality being vitamin D deficiency. A second case had chin tremors and vitamin D deficiency. Review of our patients suggests that shudders, shivers, jitteriness, or tremors may be the earliest sign of vitamin D deficiency in the newborn. Neonates who present with these signs should be investigated for vitamin D deficiency.
Lower respiratory infections (LRIs) are among the most common reasons for pediatric hospitalization and among the diagnoses with the highest number of readmissions. Characterizing LRI readmissions would help guide efforts to prevent them. We assessed variation in pediatric LRI readmission rates, risk factors for readmission, and readmission diagnoses.METHODS:
We analyzed 2008–2009 Medicaid Analytic eXtract data for patients <18 years of age in 26 states. We identified LRI hospitalizations based on a primary diagnosis of bronchiolitis, influenza, or community-acquired pneumonia or a secondary diagnosis of one of these LRIs plus a primary diagnosis of asthma, respiratory failure, or sepsis/bacteremia. Readmission rates were calculated as the proportion of hospitalizations followed by ≥1 unplanned readmission within 30 days. We used logistic regression with fixed effects for patient characteristics and a hospital random intercept to case-mix adjust rates and assess risk factors.RESULTS:
Of 150 590 LRI hospitalizations, 8233 (5.5%) were followed by ≥1 readmission. The median adjusted hospital readmission rate was 5.2% (interquartile range: 5.1%–5.4%), and rates varied across hospitals (P < .0001). Infants (patients <1 year of age), boys, and children with chronic conditions were more likely to be readmitted. The most common primary diagnoses on readmission were LRIs (48.2%), asthma (10.0%), fluid/electrolyte disorders (3.4%), respiratory failure (3.3%), and upper respiratory infections (2.7%).CONCLUSIONS:
LRI readmissions are common and vary across hospitals. Multiple risk factors are associated with readmission, indicating potential targets for strategies to reduce readmissions. Readmission diagnoses sometimes seem related to the original LRI.
Mercury (Hg) poisoning is considered a rare disease by the National Institutes of Health and the diagnosis can present great challenges to clinicians. Children who are exposed to Hg can present with a wide variety of symptoms, including acrodynia, tremor, excessive salivation, and psychiatric symptoms, including insomnia. However, endocrinologic manifestations from Hg exposure are less well known. This is a case report of a 12-year-old boy who presented with body rash, irritability, insomnia, and profuse sweating after returning from a summer camp. The child was initially managed in the outpatient setting, and the investigation was mainly targeted toward infectious etiology, including Rocky Mountain spotted fever and Lyme disease. He was eventually admitted to the hospital with altered mental status and was noted to have hyponatremia with serum sodium of 121 mEq/L. Thyroid studies also revealed elevated free thyroxine levels in the presence of normal triiodothyronine and thyrotropin. The patient developed hypertension and tachycardia, and was found to have elevated 24-hour vanillylmandelic acid and metanephrines. Finally, heavy metal measurements revealed a blood Hg level that was greater than the reference values of 0 to 9 ng/mL. Chelation treatment with 2,3-dimercaptopropane-1-sulfonate was subsequently initiated and over a period of 8 months his symptoms resolved and his thyroid function test returned to normal. This case highlights some of the challenges commonly encountered in identifying Hg exposure. More importantly, it illustrates that exposure to Hg should be considered in children who present with the symptoms and abnormal endocrinologic test results described in this report.
Since 2007, synthetic tetrahydrobiopterin (BH4) has been approved as a therapeutic option in BH4-responsive phenylketonuria (PKU) and since 2015 extended to infants younger than 4 years in Europe. The current definition of BH4 responsiveness relies on the observation of a 20% to 30% blood phenylalanine (Phe) decrease after BH4 administration, under nonstandardized conditions. By this definition, however, patients with the same genotype or even the same patients were alternatively reported as responsive or nonresponsive to the cofactor. These inconsistencies are troubling, as frustrating patient expectations and impairing cost-effectiveness of BH4-therapy. Here we tried a quantitative procedure through the comparison of the outcome of a simple Phe and a combined Phe plus BH4 loading in a series of infants with PKU, most of them harboring genotypes already reported as BH4 responsive. Under these ideal conditions, blood Phe clearance did not significantly differ after the 2 types of loading, and a 20% to 30% decrease of blood Phe occurred irrespective of BH4 administration in milder forms of PKU. Such early screening for BH4 responsiveness, based on a quantitative assay, is essential for warranting an evidence-based and cost-effective therapy in those patients with PKU eventually but definitely diagnosed as responsive to the cofactor.
Pediatric medical professionals have an increasing desire for quality improvement (QI) methods that produce sustainable changes in health care delivery. Previous reports have described QI in single settings or single coordinating entities that work with multiple sites. The objectives of this project are (1) to improve care for children with asthma across multiple practice settings and (2) to develop state-level expertise to support QI projects across entities in multiple states.METHODS:
Using a multiwave approach, the Chapter Quality Network of the American Academy of Pediatrics implemented statewide learning collaboratives in several states. For each cycle, a national leadership team coached multiple American Academy of Pediatrics chapter leadership teams, which, in turn, coached individual pediatric practices through 2 nested learning collaboratives. State chapters received data and reporting tools and a curriculum fostering QI learning and support change at the practice level. Practices implemented an asthma assessment tool and registry, analyzed work flows, and implemented self-management tools in plan-do-study-act cycles. Sixteen process and outcome measures, including optimal asthma care, were collected and analyzed by using run charts on a monthly dashboard. Chapter leaders provided feedback on sustainable QI change through surveys and interviews.RESULTS:
Optimal asthma care improved from 42% to 81% across the 4 waves. The percentage of patients rated by physicians as well controlled rose from 59% to 74%.CONCLUSIONS:
Asthma care can be improved by supporting practice change through statewide QI learning collaboratives.
Congenital diarrheal disorders are a group of rare enteropathies that often present with life-threatening diarrhea in the first weeks of life. Enteric anendocrinosis, characterized by a lack of intestinal enteroendocrine cells due to recessively inherited mutations in the Neurogenin-3 (NEUROG3) gene, has been described as a cause of congenital malabsorptive diarrhea. Diabetes mellitus also is typically associated with NEUROG3 mutations, be it early onset or a later presentation. Here we report a case of a 16-year-old male patient with severe malabsorptive diarrhea from birth, who was parenteral nutrition dependent and who developed diabetes mellitus at 11 years old. To the best of our knowledge, only 9 cases of recessively inherited NEUROG3 mutations have been reported in the literature to date. Our patient presents with several remarkable differences compared with previously published cases. This report can contribute by deepening our knowledge on new aspects of such an extremely rare disease.
To implement data-driven vital sign parameters to reduce bedside monitor alarm burden.METHODS:
Single-center, quality-improvement initiative with historical controls assessing the impact of age-based, inpatient-derived heart rate (HR) and respiratory rate (RR) parameters on a 20-bed acute care ward that serves primarily pediatric cardiology patients. The primary outcome was the number of alarms per monitored bed day (MBD) with the aim to decrease the alarms per MBD. Balancing measures included the frequency of missed rapid response team activations, acute respiratory code events, and cardiorespiratory arrest events in the unit with the new vital sign parameters.RESULTS:
The median number of all cardiorespiratory monitor alarms per MBD decreased by 21% from 52 (baseline period) to 41 (postintervention period) (P < .001). This included a 17% decrease in the median HR alarms (9–7.5 per MBD) and a 53% drop in RR alarms (16.8–8.0 per MBD). There were 57 rapid response team activations, 8 acute respiratory code events, and no cardiorespiratory arrest events after the implementation of the new parameters. An evaluation of HRs and RRs recorded at the time of the event revealed that all patients with HRs and/or RRs out of range per former default parameters would also be out of range with the new parameters.CONCLUSIONS:
Implementation of data-driven HR and iteratively derived RR parameters safely decreased the total alarm frequency by 21% in a pediatric acute care unit.
Phlebotomy excess contributes to anemia in PICU patients and increases the likelihood of red blood cell transfusion, which is associated with risk of adverse outcomes. Excessive phlebotomy reduction (EPR) strategies may reduce the need for transfusion, but have not been evaluated in a PICU population. We hypothesized that EPR strategies, facilitated by implementation science methods, would decrease excess blood drawn and reduce transfusion frequency.METHODS:
Quantitative and qualitative methods were used. Patient and blood draw data were collected with survey and focus group data to evaluate knowledge and attitudes before and after EPR intervention. The Consolidated Framework for Implementation Research was used to interpret qualitative data. Multivariate regression was employed to adjust for potential confounders for blood overdraw volume and transfusion incidence.RESULTS:
Populations were similar pre- and postintervention. EPR strategies decreased blood overdraw volumes 62% from 5.5 mL (interquartile range 1–23) preintervention to 2.1 mL (interquartile range 0–7.9 mL) postintervention (P < .001). Fewer patients received red blood cell transfusions postintervention (32.1% preintervention versus 20.7% postintervention, P = .04). Regression analyses showed that EPR strategies reduced blood overdraw volume (P < .001) and lowered transfusion frequency (P = .05). Postintervention surveys reflected a high degree of satisfaction (93%) with EPR strategies, and 97% agreed EPR was a priority postintervention.CONCLUSIONS:
Implementation science methods aided in the selection of EPR strategies and enhanced acceptance which, in this cohort, reduced excessive overdraw volumes and transfusion frequency. Larger trials are needed to determine if this approach can be applied in broader PICU populations.
In 2000, the US Congress authorized the National Institutes of Health to conduct a prospective national longitudinal study of environmental influences on children’s health and development from birth through 21 years. Several recruitment methodologies were piloted to determine the optimal strategy for a main National Children’s Study.METHODS:
After an initial pilot recruitment that used a household enumeration strategy performed poorly, the National Children’s Study Vanguard Study developed and evaluated the feasibility, acceptability, and cost of 4 alternate strategies to recruit a large prospective national probability sample of pregnant women and their newborn children. We compare household-based recruitment, provider-based recruitment, direct outreach, and provider-based sampling (PBS) strategies with respect to overall recruitment success, efficiency, cost, and fulfillment of scientific requirements.RESULTS:
Although all 5 strategies achieved similar enrollment rates (63%–81%) among eligible women, PBS achieved the highest recruitment success as measured by the ratio of observed-to-expected newborn enrollees per year of 0.99, exceeding those of the other strategies (range: 0.35–0.48). Because PBS could reach the enrollment target through sampling of high volume obstetric provider offices and birth hospitals, it achieved the lowest ratio of women screened to women enrolled and was also the least costly strategy. With the exception of direct outreach, all strategies enrolled a cohort of women whose demographics were similar to county natality data.CONCLUSIONS:
PBS demonstrated the optimal combination of recruitment success, efficiency, cost, and population representativeness and serves as a model for the assembly of future prospective probability-based birth cohorts.
Improving the diagnosis of serious bacterial infections (SBIs) in the children’s emergency department is a clinical priority. Early recognition reduces morbidity and mortality, and supporting clinicians in ruling out SBIs may limit unnecessary admissions and antibiotic use.METHODS:
A prospective, diagnostic accuracy study of clinical and biomarker variables in the diagnosis of SBIs (pneumonia or other SBI) in febrile children <16 years old. A diagnostic model was derived by using multinomial logistic regression and internally validated. External validation of a published model was undertaken, followed by model updating and extension by the inclusion of procalcitonin and resistin.RESULTS:
There were 1101 children studied, of whom 264 had an SBI. A diagnostic model discriminated well between pneumonia and no SBI (concordance statistic 0.84, 95% confidence interval 0.78–0.90) and between other SBIs and no SBI (0.77, 95% confidence interval 0.71–0.83) on internal validation. A published model discriminated well on external validation. Model updating yielded good calibration with good performance at both high-risk (positive likelihood ratios: 6.46 and 5.13 for pneumonia and other SBI, respectively) and low-risk (negative likelihood ratios: 0.16 and 0.13, respectively) thresholds. Extending the model with procalcitonin and resistin yielded improvements in discrimination.CONCLUSIONS:
Diagnostic models discriminated well between pneumonia, other SBIs, and no SBI in febrile children in the emergency department. Improvements in the classification of nonevents have the potential to reduce unnecessary hospital admissions and improve antibiotic prescribing. The benefits of this improved risk prediction should be further evaluated in robust impact studies.
To date, no studies have examined vocabulary outcomes of children meeting all 3 components of the Early Hearing Detection and Intervention (EHDI) guidelines (hearing screening by 1 month, diagnosis of hearing loss by 3 months, and intervention by 6 months of age). The primary purpose of the current study was to examine the impact of the current EHDI 1-3-6 policy on vocabulary outcomes across a wide geographic area. A secondary goal was to confirm the impact of other demographic variables previously reported to be related to language outcomes.METHODS:
This was a cross-sectional study of 448 children with bilateral hearing loss between 8 and 39 months of age (mean = 25.3 months, SD = 7.5 months). The children lived in 12 different states and were participating in the National Early Childhood Assessment Project.RESULTS:
The combination of 6 factors in a regression analysis accounted for 41% of the variance in vocabulary outcomes. Vocabulary quotients were significantly higher for children who met the EHDI guidelines, were younger, had no additional disabilities, had mild to moderate hearing loss, had parents who were deaf or hard of hearing, and had mothers with higher levels of education.CONCLUSIONS:
Vocabulary learning may be enhanced with system improvements that increase the number of children meeting the current early identification and intervention guidelines. In addition, intervention efforts need to focus on preventing widening delays with chronological age, assisting mothers with lower levels of education, and incorporating adults who are deaf/hard-of-hearing in the intervention process.