ATS core curriculum 2023. Pediatric pulmonary medicine: Respiratory disorders in infants.

The American Thoracic Society Core Curriculum updates clinicians annually in pediatric pulmonary disease. This is a summary of the Pediatric Pulmonary Medicine Core Curriculum presented at the 2023 American Thoracic Society International Conference. The respiratory disorders of infancy discussed in this year's review include: the care of the patient with bronchopulmonary dysplasia in the neonatal intensive care unit, clinical phenotypes and comorbidities; diffuse lung disease; pulmonary hypertension; central and obstructive sleep apnea.

Extradural decompression versus duraplasty in Chiari malformation type I with syrinx: outcomes on scoliosis from the Park-Reeves Syringomyelia Research Consortium.

Objective: Scoliosis is common in patients with Chiari malformation type I (CM-I)-associated syringomyelia. While it is known that treatment with posterior fossa decompression (PFD) may reduce the progression of scoliosis, it is unknown if decompression with duraplasty is superior to extradural decompression.

Methods: A large multicenter retrospective and prospective registry of 1257 pediatric patients with CM-I (tonsils ≥ 5 mm below the foramen magnum) and syrinx (≥ 3 mm in axial width) was reviewed for patients with scoliosis who underwent PFD with or without duraplasty.

Genome-Wide Association Study Identifies Genetic Risk Factors for Spastic Cerebral Palsy.

Background: Although many clinical risk factors of spastic cerebral palsy (CP) have been identified, the genetic basis of spastic CP is largely unknown. Here, using whole-genome genetic information linked to a deidentified electronic health record (BioVU) with replication in the UK Biobank and FinnGen, we perform the first genome-wide association study (GWAS) for spastic CP.

Objective: To define the genetic basis of spastic CP.

Multi-omic analysis elucidates the genetic basis of hydrocephalus.

We conducted PrediXcan analysis of hydrocephalus risk in ten neurological tissues and whole blood. Decreased expression of MAEL in the brain was significantly associated (Bonferroni-adjusted p < 0.05) with hydrocephalus.

Dural augmentation approaches and complication rates after posterior fossa decompression for Chiari I malformation and syringomyelia: a Park-Reeves Syringomyelia Research Consortium study.

Objective: Posterior fossa decompression with duraplasty (PFDD) is commonly performed for Chiari I malformation (CM-I) with syringomyelia (SM). However, complication rates associated with various dural graft types are not well established. The objective of this study was to elucidate complication rates within 6 months of surgery among autograft and commonly used nonautologous grafts for pediatric patients who underwent PFDD for CM-I/SM.

Machine learning predicts risk of cerebrospinal fluid shunt failure in children: a study from the hydrocephalus clinical research network.

Purpose: While conventional statistical approaches have been used to identify risk factors for cerebrospinal fluid (CSF) shunt failure, these methods may not fully capture the complex contribution of clinical, radiologic, surgical, and shunt-specific variables influencing this outcome. Using prospectively collected data from the Hydrocephalus Clinical Research Network (HCRN) patient registry, we applied machine learning (ML) approaches to create a predictive model of CSF shunt failure.

Methods: Pediatric patients (age < 19 years) undergoing first-time CSF shunt placement at six HCRN centers were included.

Rare and de novo coding variants in chromodomain genes in Chiari I malformation.

Chiari I malformation (CM1), the displacement of the cerebellum through the foramen magnum into the spinal canal, is one of the most common pediatric neurological conditions. Individuals with CM1 can present with neurological symptoms, including severe headaches and sensory or motor deficits, often as a consequence of brainstem compression or syringomyelia (SM). We conducted whole-exome sequencing (WES) on 668 CM1 probands and 232 family members and performed gene-burden and de novo enrichment analyses.

Occipital-Cervical Fusion and Ventral Decompression in the Surgical Management of Chiari-1 Malformation and Syringomyelia: Analysis of Data From the Park-Reeves Syringomyelia Research Consortium.

Background: Occipital-cervical fusion (OCF) and ventral decompression (VD) may be used in the treatment of pediatric Chiari-1 malformation (CM-1) with syringomyelia (SM) as adjuncts to posterior fossa decompression (PFD) for complex craniovertebral junction pathology.

Objective: To examine factors influencing the use of OCF and OCF/VD in a multicenter cohort of pediatric CM-1 and SM subjects treated with PFD.

Methods: The Park-Reeves Syringomyelia Research Consortium registry was used to examine 637 subjects with cerebellar tonsillar ectopia ≥ 5 mm, syrinx diameter ≥ 3 mm, and at least 1 yr of follow-up after their index PFD.

Factors associated with syrinx size in pediatric patients treated for Chiari malformation type I and syringomyelia: a study from the Park-Reeves Syringomyelia Research Consortium.

Objective: Factors associated with syrinx size in pediatric patients undergoing posterior fossa decompression (PFD) or PFD with duraplasty (PFDD) for Chiari malformation type I (CM-I) with syringomyelia (SM; CM-I+SM) are not well established.

Methods: Using the Park-Reeves Syringomyelia Research Consortium registry, the authors analyzed variables associated with syrinx radiological outcomes in patients (< 20 years old at the time of surgery) with CM-I+SM undergoing PFD or PFDD. Syrinx resolution was defined as an anteroposterior (AP) diameter of ≤ 2 mm or ≤ 3 mm or a reduction in AP diameter of ≥ 50%.

Graft dural closure is associated with a reduction in CSF leak and hydrocephalus in pediatric patients undergoing posterior fossa brain tumor resection.

Objective: The authors aimed to evaluate clinical, radiological, and surgical factors associated with posterior fossa tumor resection (PFTR)-related outcomes, including postoperative complications related to dural augmentation (CSF leak and wound infection), persistent hydrocephalus ultimately requiring permanent CSF diversion after PFTR, and 90-day readmission rate.

Methods: Pediatric patients (0-17 years old) undergoing PFTR between 2000 and 2016 at Monroe Carell Jr. Children's Hospital of Vanderbilt University were retrospectively reviewed.

Pediatric thoracolumbar spine surgery and return to athletics: a systematic review.

Objective: Spinal conditions and injuries in the pediatric population can necessitate surgical treatment. For many pediatric patients, a return to athletic activity after spinal surgery is a significant postoperative focus. However, there is a lack of standardized guidelines to determine criteria for safe return to play (RTP).

Outcomes following a clinical algorithm allowing for delayed hysterectomy in the management of severe placenta accreta spectrum.

Background: The incidence of placenta accreta spectrum is rising. Management is most commonly with cesarean hysterectomy. These deliveries often are complicated by massive hemorrhage, urinary tract injury, and admission to the intensive care unit.

Clinical and biological predictors of response to standardised paediatric colitis therapy (PROTECT): a multicentre inception cohort study.

Background: Lack of evidence-based outcomes data leads to uncertainty in developing treatment regimens in children who are newly diagnosed with ulcerative colitis. We hypothesised that pretreatment clinical, transcriptomic, and microbial factors predict disease course.

Methods: In this inception cohort study, we recruited paediatric patients aged 4-17 years with newly diagnosed ulcerative colitis from 29 centres in the USA and Canada.

Using an artificial neural network to predict traumatic brain injury.

In BriefPediatric traumatic brain injury (TBI) is common, but not all injuries require hospitalization. A computational tool for ruling-in patients who will have clinically relevant TBI (CRTBI) would be valuable, providing an evidence-based mechanism for safe discharge. Here, using data from 12,902 patients from the Pediatric Emergency Care Applied Research Network (PECARN) TBI data set, the authors utilize artificial intelligence to predict CRTBI using radiologist-interpreted CT information with > 99% sensitivity and an AUC of 0.

Deep brain stimulation in pediatric dystonia: a systematic review.

While deep brain stimulation (DBS) treatment is relatively rare in children, it may have a role in dystonia to reduce motor symptoms and disability. Pediatric DBS studies are sparse and limited by small sample size, and thus, outcomes are poorly understood. Thus, we performed a systematic review of the literature including studies of DBS for pediatric (age < 21) dystonia.

Factors associated with early outcomes following standardised therapy in children with ulcerative colitis (PROTECT): a multicentre inception cohort study.

Background: Previous retrospective studies of paediatric ulcerative colitis have had limited ability to describe disease progression and identify predictors of treatment response. In this study, we aimed to identify characteristics associated with outcomes following standardised therapy after initial diagnosis.

Methods: The PROTECT multicentre inception cohort study was based at 29 centres in the USA and Canada and included paediatric patients aged 4-17 years who were newly diagnosed with ulcerative colitis.

X-ray vs. CT in identifying significant C-spine injuries in the pediatric population.

Purpose: Evaluation of cervical spine injury (CSI) in children requires rapid, yet accurate assessment of damage. Given concerns of radiation exposure, expert consensus advises that computed tomography (CT) should be used sparingly. However, CT can provide superior image resolution and detection of pathology.

Use of health care services by inner-city infants in an early discharge program.

Objective: To assess the use of health care services by inner-city infants enrolled in an early discharge program who received care in tertiary care children's hospital primary care clinic.

Design: Retrospective cohort study.

Setting: Large, metropolitan university hospital and a children's hospital.

The value of Doppler resistive index and peak systolic velocity in the evaluation of porcine renal obstruction.

Purpose: We evaluated the use of Doppler resistive indexes and systolic velocities as diagnostic tools in a partially obstructed animal model.

Materials And Methods: We studied 12 normal pigs by Doppler sonography, which served as control units. In 9 subjects partial ureteral obstruction was then created surgically, and 3 continued as controls.

The natural history of nephrocalcinosis in premature infants treated with loop diuretics.

Purpose: We investigated the natural history of nephrocalcinosis in premature infants treated with furosemide and attempted to identify factors to predict infants most at risk.

Materials And Methods: We evaluated 13 preterm infants in this longitudinal pilot study. During hospitalization and while receiving a loop diuretic nephrocalcinosis developed in each patient.

The use of laparoscopy in the management of the nonpalpable testis.

Fifty-eight children with a nonpalpable testis have been evaluated at our institution between 1988 and 1994. Laparoscopy was performed in these children to effect a treatment plan. The children range in age from 10 months to 14 years.

Autologous buccal mucosal graft for urethral reconstruction.

Objectives: The search for an adequate tissue for reconstruction of the urethra in those patients with a paucity of local skin continues. Over the past 18 months, the use of buccal mucosa as a substitution for urethra was evaluated.

Methods: Six patients who had complex hypospadias had buccal mucosa grafts for urethral reconstruction.

Intrapelvic pressure monitoring in the partially obstructed porcine kidney.

Objectives: The diagnosis of urinary obstruction in newborns and infants remains difficult because the diagnostic studies available at present are fraught with many problems. It is our premise that precise measurement of renal pelvic pressures under physiologic conditions in patients with urinary tract dilation will allow diagnosis of obstruction and prediction of further renal damage. This study evaluates an intrapelvic pressure monitoring system.

The frequency and frequency dysuria syndromes of childhood: hypercalciuria as a possible etiology.

Objectives: The syndromes of frequency and frequency dysuria of childhood are believed to have multifactorial etiologies. Hypercalciuria has been reported to be associated with the frequency dysuria syndrome and a direct cause and effect relationship has been postulated due to a positive response to treatment with hydrochlorothiazide. This study was designed to compare the urinary calciums of children with the frequency syndrome and the frequency dysuria syndrome.