Impaired lung function following e-cigarette or vaping product use associated lung injury in the first cohort of hospitalized adolescents.

BACKGROUND: Beginning June 2019, Children's Wisconsin was the first hospital to identify a cohort of adolescent patients hospitalized with symptoms likely associated with e-cigarette use. Our report adds to the growing literature describing the radiographic, gross and cytopathologic bronchoscopic findings, and short-term lung function outcomes in this cohort of adolescents with e-cigarette or vaping product use associated lung injury (EVALI). METHODS: We present 15 adolescents hospitalized from June to September, 2019 with confirmed EVALI. We abstracted data from inpatient hospitalization and first outpatient pulmonary clinic visit. RESULTS: There were 15 patients (11 male, 12 White) with a mean age of 17.1 years. All patients presented with subacute pulmonary, gastrointestinal and constitutional complaints. Diagnostic workup was guided by the Centers for Disease Control criteria for confirmed EVALI case surveillance. Flexible bronchoscopy was performed in 13/15 patients with 10/13 demonstrating gross pathologic abnormalities. Seven of 15 patients required intensive care and 2 met criteria for pediatric Acute Respiratory Distress Syndrome. Patients had dramatic improvement with systemic glucocorticoid therapy and 14/15 were discharged on room air. Eleven patients were seen as outpatients. Despite 11/11 patients reporting resolved or improved symptoms, 7/11 had abnormalities on pulmonary function testing. We initiated inhaled corticosteroids for 5/11 patients and 4/11 patients remained on their corticosteroid wean. CONCLUSIONS AND RELEVANCE: We report short-term outcomes of the first cohort of adolescent patients hospitalized with EVALI. An association is observed between clinical improvement and treatment with systemic corticosteroids. However, residual airway reactivity or diffusion abnormalities persisted when patients were re-evaluated in the short-term period (mean 4.5 weeks).

Oral health of cystic fibrosis patients at a north american center: A pilot study

Background: The objective of this study was to describe the oral health status of Cystic Fibrosis (CF) children in a US facility. Material and Methods: Twenty CF children ages 6-18 were recruited from Children's Hospital of Wisconsin Pulmonary Clinic. Parents completed a health questionnaire. Clinical examinations checked dental caries using the dmft/DMFT index, dental hygiene using the Simplified Greene-Vermillion Index (DI-S), gingival inflammation using the Community Periodontal Index of Treatment Needs, and enamel defects using the modified Developmental Defects of Enamel Index. Results: The majority (90%) brush twice a day, 65% consume sugary snacks, and 70% visit the dentist every 6 months. Clinically, they presented DMFT 0.25 and dmft 0.90, fair oral hygiene with DI-S 1.02, 75% had mild gingivitis and 50% had enamel defects. The more antibiotics they took, significantly more frequent (p = 0.007) and more severe (p = 0.017) enamel defects were noted. Similar trend was found between the number of surgeries and the presence of enamel defects (p=0.076) and dental caries (p = 0.028). Conclusions: Within the limitations of this study, CF patients were found to be at oral health risk due to the high prevalence of dental enamel defects. Oral health for CF children should be part of the multidisciplinary care

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Follow-up of phase I trial of adalimumab and rosiglitazone in FSGS: III. Report of the FONT study group.

BACKGROUND: Patients with resistant primary focal segmental glomerulosclerosis (FSGS) are at high risk of progression to chronic kidney disease stage V. Antifibrotic agents may slow or halt this process. We present outcomes of follow-up after a Phase I trial of adalimumab and rosiglitazone, antifibrotic drugs tested in the Novel Therapies in Resistant FSGS (FONT) study. METHODS: 21 patients--12 males and 9 females, age 16.0 +/- 7.5 yr, and estimated GFR (GFRe) 121 +/- 56 mL/min/1.73 m2--received adalimumab (n = 10), 24 mg/m2 every 14 days or rosiglitazone (n = 11), 3 mg/m2 per day for 16 weeks. The change in GFRe per month prior to entry and after completion of the Phase I trial was compared. RESULTS: 19 patients completed the 16-week FONT treatment phase. The observation period pre-FONT was 18.3 +/- 10.2 months and 16.1 +/- 5.7 months after the study. A similar percentage of patients, 71% and 56%, in the rosiglitazone and adalimumab cohorts, respectively, had stabilization in GFRe, defined as a reduced negative slope of the line plotting GFRe versus time without requiring renal replacement therapy after completion of the FONT treatment period (P = 0.63). CONCLUSION: Nearly 50% of patients with resistant FSGS who receive novel antifibrotic agents may have a legacy effect with delayed deterioration in kidney function after completion of therapy. Based on this proof-of-concept preliminary study, we recommend long-term follow-up of patients enrolled in clinical trials to ascertain a more comprehensive assessment of the efficacy of experimental treatments.

Delay in diagnosis in poststreptococcal glomerulonephritis.

OBJECTIVE: To determine the frequency and risk factors for diagnostic delays in children with poststreptococcal glomerulonephritis (PSGN). STUDY DESIGN: We reviewed the charts of 52 children with PSGN, and identified children with a delay in diagnosis of more than 24 hours. We determined risk factors for delay in diagnosis using univariate and multivariate logistic regression. RESULTS: 17 children (33%) with PSGN had a delay in diagnosis. Delay in diagnosis occurred in 14% of children with gross hematuria as a presenting complaint and in 54% of children without gross hematuria as a presenting complaint (3.8 increased relative risk, 95% CI = 1.4 to 10; P = .02). A delay in diagnosis was more common in children with a negative infection history (P = .04). In multiple logistic regression, only the absence of gross hematuria as a presenting complaint was associated with a delay in diagnosis (P = .01). All children with a delay in diagnosis had microscopic hematuria on their initial urinalysis. CONCLUSIONS: Delay in diagnosis is common in children with PSGN, especially if visible hematuria is not a presenting complaint. Physicians should consider the possibility of PSGN in children with symptoms that may be secondary to volume overload. A urinalysis is a helpful initial diagnostic test.

Obstacles to the prescribing of growth hormone in children with chronic kidney disease.

Despite its effectiveness, recombinant human growth hormone (rhGH) is under-utilized in short children with chronic kidney disease (CKD). We conducted a multicenter study to explore the obstacles preventing children with CKD from receiving rhGH. We investigated the use of rhGH in 307 children with CKD from seven pediatric nephrology centers. Among the 110 patients who fell below the 5th percentile, 56 (51%) had not received rhGH. The most common reasons given for these children not receiving rhGH were family refusal, secondary hyperparathyroidism, and non-compliance. However, no explanation was apparent for 25% of the short children with CKD. Boys were more likely than girls to receive rhGH (65% vs 31%; P = 0.002). Use of rhGH was similar in African Americans and non-Hispanic Whites. Children who had received rhGH achieved a 0.5 increase in height z-score in the first year after the initiation of rhGH therapy. Children who had not received rhGH achieved a 0.03 increase in height z-score during the first year after falling below the 5th percentile (P = 0.005 vs the children who had received rhGH). Waiting for insurance company approval led to a significant delay in the initiation of rhGH treatment in 18% of patients. The fact that more than 50% of short children with CKD did not receive rhGH is secondary to multiple factors, many of which may be amenable to intervention efforts.

Rickets in the Dairy State.

BACKGROUND: Nutritional vitamin D deficiency rickets occurs when children do not receive adequate vitamin D, which can be obtained from diet or manufactured in the skin when there is adequate sun exposure. A number of reports have described cases of vitamin D deficiency rickets in breastfed infants, but the public health significance of this problem in Wisconsin is unknown. OBJECTIVES: Our objectives were to identify cases of vitamin D deficiency rickets in Wisconsin infants and to determine the percentage of these infants participating in the Wisconsin Women, Infant and Children (WIC) program. METHODS: All cases of rickets due to nutritional vitamin D deficiency seen at Children's Hospital of Wisconsin or its associated outpatient clinics were identified by retrospective chart review. Data collected included date of birth, age at presentation, race, clinical presentation, diet history, history of vitamin supplementation, x-ray findings, and biochemical studies. The children with nutritional vitamin D deficiency rickets were cross-referenced with the Wisconsin WIC database. RESULTS: Fifty-one definite cases of nutritional vitamin D deficiency rickets were identified. Skeletal deformities, failure to thrive, fractures, seizures, incidental lab finding, tetany, and refusal to walk were the most common reasons for identifying rickets. All of the children were breastfed and did not receive vitamin supplementation. The infants had a mean age of 13.6 months and 46 (90%) were African American. Thirty-seven out of 51 children (73%) were enrolled in the Wisconsin WIC program. CONCLUSION: Vitamin D deficiency nutritional rickets is an important public health problem in Wisconsin. The Wisconsin WIC program may be an important site for intervention strategies.