Validation of Administrative Codes for Palliative Care Consultation Among Critically Ill Children.

OBJECTIVES: To determine the validity of palliative care (PC) administrative codes (V66.7 and Z51.5) among critically ill pediatric patients. METHODS: In this single-center retrospective cohort study, we included all hospitalizations with a PICU admission between March 2016 and March 2018. Sensitivity, specificity, and positive and negative predictive values of the relevant codes were estimated by using a gold standard of a local PC registry. RESULTS: During the study period, 4670 hospitalizations were included. The median admission age was 5 years (interquartile range 1.5-12.9) with 55% having at least 1 complex chronic condition. The median length of PICU stay was 1.8 days (interquartile range 1-3.4) and mortality was low (1.3%). A total 182 (3.9%) hospitalizations had evidence of a PC consultation. Administrative codes for PC had a sensitivity of 11% (95% confidence interval [CI] 6.8%-16.5%) and a specificity of 99.8% (95% CI 99.6%-99.9%). The positive and negative predictive values were 66.7% (95% CI 47.2%-82.7%) and 96.5% (95% CI 95.9%-97.0%), respectively. CONCLUSIONS: Among critically ill children, PC administrative codes had high specificity but poor sensitivity. The potential for underascertainment of this resource should be considered in future research using administrative data.

Variation in Pediatric Palliative Care Allocation Among Critically Ill Children in the United States.

OBJECTIVES: The objectives are as follows: 1) estimate palliative care consult rates and trends among critically ill children and 2) characterize which children receive palliative care consults, including those meeting previously proposed ICU-specific palliative care screening criteria. DESIGN: Retrospective cohort. SETTING: Fifty-two United States children's hospitals participating in the Pediatric Health Information Systems database. PATIENTS: Hospitalized children with nonneonatal ICU admissions from 2007 to 2018. MEASUREMENTS AND MAIN RESULTS: The primary outcome was palliative care consultation, as identified by the palliative care International Classification of Disease code. Patient characteristics and outcomes were compared between those with and without palliative care. We used a mixed-effects multivariable model to estimate the independent association between the palliative care and patient characteristics accounting for institution and subject clustering. Hospitalizations were categorized into three mutually exclusive groups for comparative analyses: 1) meeting ICU-specific palliative care criteria, 2) presence of a complex chronic condition not in ICU-specific palliative care criteria, or 3) not meeting ICU-specific palliative care or complex chronic condition criteria. Rates and trends of palliative care consultation were estimated including variation among institutions and variation among subcategories of ICU-specific palliative care criteria. The study cohort included 740,890 subjects with 1,024,666 hospitalizations. About 1.36% of hospitalizations had a palliative care consultation. Palliative care consult was independently associated with older age, female sex, government insurance, inhospital mortality, and ICU-specific palliative care or complex chronic condition criteria. Among the hospitalizations, 30% met ICU-specific palliative care criteria, 40% complex chronic condition criteria, and 30% neither. ICU-specific palliative care patients received more mechanical ventilation and cardiopulmonary resuscitation, had longer hospital and ICU lengths of stay, and had higher inhospital mortality (p < 0.001). Palliative care utilization increased over the study period with considerable variation between the institutions especially in the ICU-specific palliative care cohort and its subgroups. CONCLUSIONS: Palliative care consultation for critically ill children in the United States is low. Palliative care utilization is increasing but considerable variation exists across institutions, suggesting inequity in palliative care allocation among this vulnerable population. Future studies should evaluate factors influencing allocation of palliative care among critically ill children in the United States and the drivers of differences between the institutional practices.

Increased homozygosity in the first Hispanic patient with plantar lipomatosis, unusual facies, and developmental delay (Pierpont syndrome): a case report.

BACKGROUND: Pierpont syndrome was first described in 1998 with key characteristics including developmental delay, dysmorphic facial features, fat pads on hands and feet, and feeding difficulties. To date the mechanism of inheritance is unknown. Nine out of ten previously described patients with Pierpont syndrome were boys. This is the first report of a case of a non-white patient with Pierpont syndrome and she is the second female patient to be described as having Pierpont syndrome. CASE PRESENTATION: Our patient is a 16-month-old Hispanic girl with extreme developmental delay, microcephaly, large ears, short and thick upper lip, broad philtrum, widely spaced teeth, constipation, dysphagia, fat pads on feet and hands, autistic behavior and seizure-like episodes. She had a normal karyotype (46,XX), and array testing showed greater than 8 % homozygosity with otherwise normal results. Genes within these areas of homozygosity may provide clues to an etiology and suggest autosomal recessive inheritance. This case report highlights the possibility of ethnic variations in this syndrome's presentation, which may have ramifications in uncovering the pathogenesis as well as expanding the phenotype. CONCLUSION: Pierpont syndrome should be considered in the evaluation of children with the described features, regardless of their gender and ethnicity.