The association between vitamin D deficiency and hospitalization outcomes in pediatric patients with sickle cell disease.

BACKGROUND: While there is a known association between low vitamin D levels and increased chronic pain in patients with Sickle Cell Disease (SCD), there are no reported studies evaluating the relationship of vitamin D levels and hospitalization outcomes in this population. The aim of this study was to assess this relationship with hospitalization outcomes defined as the number of emergency room (ER) visits, hospital admissions for pain crisis, and length of hospital stay. DESIGN: A retrospective chart review of all pediatric patients with SCD (1-21 years old) was performed from January 2015 to January 2016 in an urban-based hospital setting (n = 134). Those with at least one reported Vitamin D level who maintained follow up during the time studied were enrolled (n = 90). Patient hospitalizations rates were compared between vitamin D deficiency (<20 ng/ml) and sufficiency (>20 ng/ml). RESULTS: Patients with both SCD and vitamin D deficiency were more likely to have at least one Emergency Room visit (p < 0.01), at least one admission for pain crisis (p < 0.01), and a longer length of admission (p < 0.0001) when compared to patients with SCD and sufficient vitamin D levels. CONCLUSION: There is a significant association between vitamin D levels of <20 ng/ml and the number of ER visits, hospital admissions for pain crisis, and length of stay in patients with SCD. Further research is required to assess if correcting vitamin D levels may improve hospitalization outcomes in this population.

Acute Presentation of Tuberculosis Empyema in a Healthy Adolescent.

BACKGROUND Tuberculosis (TB) was the leading cause of infectious death worldwide until the COVID-19 pandemic, which reduced case reporting and disrupted TB diagnosis and services. While Mycobacterium tuberculosis remains a leading cause of morbidity and mortality globally, the disease burden within developed nations remains relatively rare. Although the many complications of TB are well known, no current data exists on those infected with TB who subsequently developed recurrent TB empyema, as it is such a rare complication, especially in pediatric and adolescent populations. CASE REPORT A previously healthy 15-year-old male patient presented with 5-day duration of cough, congestion, intermittent fever, and post-tussive emesis. Although born in the United States, 3 months before presentation, he returned from Senegal, where he had lived for 4 years. Imaging demonstrated consolidation with loculated effusion. Patient underwent video-assisted thoracoscopy and chest tube placement, draining 750 mL of purulent fluid testing positive for rare acid-fast bacilli. Rifampin, isoniazid, pyrazinamide, and ethambutol were administered, with discharge medication compliance ensured by daily videos surveillance through the Department of Health. Although compliant with medications, patient presented to the Emergency Department 2 months later with a multi-loculated fluid recollection and fistula formation requiring chest tube placement. After this discharge, patient experienced resolution of disease following completion of therapy. CONCLUSIONS TB complication should be considered as a differential diagnosis for pleural effusion in the appropriate clinical setting. Providers should not only consider the diagnosis but pursue appropriate testing and management early, particularly in those with risk factors, including travel to an endemic location.

Late Onset Diaphragmatic Hernia: A Forgotten Etiology of Recurrent Vomiting in the Adolescent Population.

Diaphragmatic hernia is a rare disorder in adolescents with oftentimes delayed diagnosis due to late-onset and non-specific clinical manifestations. In this report, we present a case of diaphragmatic hernia in an 18-year-old male, where initial diagnosis was complicated by confounding factors of type 1 diabetes mellitus and cannabinoid hyperemesis syndrome. This case highlights the importance of having a high index of suspicion for diaphragmatic hernia in patients with nonspecific gastrointestinal symptoms to ensure timely recognition and surgical intervention.

Drug Transaction Experiences in American High Schools 2001-2015.

BACKGROUND: Among adolescents, the pathway from being offered drugs to engaging in drug use has been established. The prevalence of drug transactions specifically in schools is less understood. The purpose of this study was to identify the prevalence of adolescents who have reported drug transaction experiences (being offered, sold, or given an illegal drug) on school property and subsequently identify behavioral correlates associated with these experiences. METHODS: This study analyzed YRBS data from 2001 to 2015 (N = 117,815). We used 95% confidence intervals to evaluate differences in prevalence across years and demographic subgroups. Odds ratios determined the likelihood of engaging in risk behaviors among those youth also experiencing drug transactions on school property. RESULTS: Every survey year, at least 1 in 5 youth reported having drug transaction experiences at school. Boys consistently reported significantly higher rates of drug transaction experiences in comparison to girls, with Hispanic boys representing the highest rates. Youth reporting these experiences with drug transactions were also more likely to carry a weapon to school and to be bullied at school. CONCLUSIONS: Implications for the reduction of drug transactions in high schools are discussed and recommendations for prevention efforts addressing youth engagement in multiple risk behaviors are identified.

Implementation of Electronic Medical Record Template Improves Screening for Complications in Children with Type 1 Diabetes Mellitus.

OBJECTIVE: Health professionals and patients should follow comprehensive screening guidelines to recognize early signs of long-term complications for insulin-dependent type 1 diabetes mellitus (T1DM). The aim of this study is to demonstrate that utilization of electronic medical record (EMR) templates for diabetes management improves adherence to International Society for Pediatric and Adolescent Diabetes (ISPAD) screening guidelines. METHODS: All patients with T1DM who were seen in the outpatient pediatric endocrine clinic (age 0-22 years old) at an urban community-based community hospital during the 2014 calendar year were enrolled in the study (n=49). A retrospective chart review was performed and audited against ISPAD guidelines. An EMR template and order set was then created based on ISPAD screening guidelines with the aim of improving compliance. The templates were implemented in 2015 (initial phase) and 2016 (maintenance phase) and these data were compared to baseline data. A chi-squared test was performed to analyze the differences between the data using SAS version 9.4 (SAS Institute, Inc). A p-value less than 0.05 was considered significant. RESULTS: Significant improvements (p< 0.05) in screening guideline adherence from baseline to maintenance phase data were found for annual retinopathy (0% to 45%) and neuropathic foot (0% to 64%) exams, screening for microalbuminuria (49% to 79%), celiac disease (6% to 81%), lipids (63% to 86%), and basic metabolic panel (69% to 88%). Of note, thyroid function testing was also increased, but was not statistically significant between the years. CONCLUSION: The utilization of EMR templates and order sets for T1DM are valuable tools to aid medical providers in adhering to ISPAD screening guideline.

Triple A syndrome (Allgrove syndrome): improving outcomes with a multidisciplinary approach.

Allgrove syndrome or triple A (3A) syndrome is a multisystem disorder which classically involves the triad of esophageal achalasia, alacrima, and adrenal insufficiency due to adrenocorticotropin hormone insensitivity. It follows an autosomal recessive pattern of inheritance and is associated with mutations in the AAAS (achalasia-addisonianism-alacrima syndrome) gene. Since its first description in 1978, the knowledge on clinical and genetic characteristics has been expanding; however, the current literature is limited to case reports and case reviews. Early recognition of the syndrome is challenging, given the rarity of the condition and high phenotypic heterogeneity even among members of kin. The coordination of care for these patients requires a multidisciplinary team of specialists, including endocrinologists, neurologists, gastroenterologists, ophthalmologists, developmental specialists, dentists, geneticists, and surgeons. In this review, we aim to summarize the current recommendations for the diagnosis, management, and follow-up of patients with 3A syndrome.

Diagnosis of Tuberculosis in an Asymptomatic Child, Sibling, and Symptomatic Pregnant Mother in New York City by Tuberculin Skin Testing and the Importance of Screening High-Risk Urban Populations for Tuberculosis.

BACKGROUND In 2017, in New York City (NYC), 86% of the cases of tuberculosis (TB) occurred in patients who were born outside the United States (US). This case report illustrates the importance of the use of the tuberculin skin test (TST), and other tests for TB infection (TTBI), in screening high-risk groups, the challenges of diagnosing TB in young children, and highlights the importance of preventing a delay in the diagnosis of TB in family members. CASE REPORT Following a routine TST in an asymptomatic 10-year-old girl, a diagnosis of TB was made, which was confirmed on chest X-ray (CXR) and by the presence of acid-fast bacilli (AFB) in the sputum. Her family had emigrated from China to NYC ten years previously. All the family was screened using the TST, which was positive in her 2-year-old sister and her 37-year-old pregnant mother, and pulmonary TB was confirmed on CXR and by AFBs in the sputum. All three family members and the newborn baby were treated according to current guidelines, with a good clinical outcome. CONCLUSIONS This case report raises awareness about the lack of symptoms in childhood TB and the importance of screening high-risk patients in an urban immigrant population. In children under 5 years of age, a diagnosis of TB can indicate a sentinel event, suggesting a potential undiagnosed or untreated source case, which is usually an adult family member. This report highlights the challenges of diagnosing TB in children, who may be asymptomatic with negative laboratory findings.

Alacrima as a Harbinger of Adrenal Insufficiency in a Child with Allgrove (AAA) Syndrome.

BACKGROUND Allgrove syndrome, or triple "A" syndrome (3A syndrome), is a rare autosomal recessive syndrome with variable phenotype, and an estimated prevalence of 1 per 1,000,000 individuals. Patients usually display the triad of achalasia, alacrima, and adrenocorticotropin (ACTH) insensitive adrenal insufficiency, though the presentation is inconsistent. CASE REPORT Here, the authors report a case of Allgrove syndrome in a pediatric patient with delayed diagnosis in order to raise awareness of this potentially fatal disease as a differential diagnosis of alacrima. CONCLUSIONS The prevalence of Allgrove syndrome may be much higher as a result of underdiagnosis and missed diagnosis due to the variable presentation and sudden unexplained childhood death from adrenal crisis. The authors review the characteristic symptoms of Allgrove syndrome in relation to the case study in order to avoid missed or delayed diagnosis, potentially decreasing morbidity, and mortality in those affected by this disease.

Abrin poisoning in an 18-month-old child.

BACKGROUND: When discussing known poisons and their clinical consequences, few physicians are aware of the deadly poison abrin. The common symptoms of abrin toxicity include nausea, vomiting, abdominal pain, and diarrhea with or without bloody stool. However, with an estimated fatal human dose of less than 1 microgram/kg, death due to complications such as liver failure, renal failure, and cerebral edema are possible. CASE REPORT: An 18-month-old male presented to the emergency department with an abrupt onset of fever, vomiting, diarrhea, and dehydration. The parents had assumed the child was suffering from a severe gastroenteritis until they noticed 3 consecutive diapers containing colorful seeds that were identified by poison control as belonging to the Abrus precatorius plant. The child's gastrointestinal symptoms were consistent with reported cases of abrin poisoning, but the patient also had an isolated and significantly elevated alkaline phosphatase, with testing unable to reveal the responsible pathological process. CONCLUSIONS: To reduce morbidity and possible mortality, parents and pediatricians alike should be conscious of the danger the Abrus precatorius seed poses to the pediatric population. Children are at a greater risk of ingesting these seeds due to their colorful appearance, and the consequences could be fatal. Through this case report we hope to raise public awareness regarding this toxin. This includes the management of known cases, as well as the possibility of encountering an isolated elevated alkaline phosphatase level as a laboratory finding if ingestion occurs.