P070 Evaluation of the perception of pediatric rheumatology by pediatric residents: results of a national survey

Background Pediatric Rheumatology (PR) is a young specialty that has not yet gained momentum in Algeria. The aim of this study is the assessment of the perception of PR by pediatric residents in Algeria and the current practice of this young sub-specialty in our country. Methods Descriptive cross-sectional study evaluating the perception of PR by pediatric residents was created on Google forms and distributed via email and Facebook to be taken as a self-administered online survey. The survey is composed of three parts: socio-demographic characteristics of the participant, current practice of PR and perspectives of PR training. Results Seventy-seven residents responded to the online questionnaire, with an average age of 30.40 ± 3.13 years and 65% of participants being female. Participation included different regions of Algeria (Batna, Annaba, Setif, Constantine, Algiers and Oran) and different levels of training (from the first year to the fifth year of residency) Ninety-two percent (92%) of participants had managed PR consultations, with a frequency of once a week in 75.3% of cases and twice a week in 15.6% of cases. In 81% of cases, it was not the initial presentation. The patients were mainly referred by a general practitioner or a general pediatrician. The Management of these patients was provided by a general pediatrician in 69.4% of cases and only in 37.7% by a specialized pediatrician. In 71.4% of cases, there was no pediatric rheumatologist in the establishment where the resident was being trained. The level of knowledge in PR was admitted to be poor by 59.7% of the respondents. Seventy-five percent of the residents wanted to have rotations dedicated to this sub-specialty during their training. Seventy percent thought that PR is equally as important to know as the other subspecialties in pediatrics (pediatric endocrinology, pediatric pneumology ...). Interest in further PR training was expressed by 80.5% of participants, given the chance. Conclusion This survey reinforces the perception that PR training needs to be improved in Algeria, especially as the number of PR consultations is on the rise. The creation of specialized training in PR is becoming a necessity.

Severe hypercalcemia and a pelvic brown tumor in an adolescent with primary hyperparathyroidism: a case report

Background Primary hyperparathyroidism may present in a myriad of manners, varying from an incidental asymptomatic biochemical finding to gastrointestinal, psychiatric, renal, and bone manifestations. While hyperparathyroidism remains a rare diagnosis in the pediatric population, the initial approach to diagnosis and management of hypercalcemia in children is imperative for the general pediatrician. Herein, we describe an adolescent who presented with a lytic bone lesion and severe, symptomatic hypercalcemia due to primary hyperparathyroidism. Case presentation A 14-year-old male presented with vomiting, constipation, abdominal pain, and lethargy. He had an elevated total corrected calcium of 4.3 mmol/L. He was found to have a large pelvic lytic tumor consistent with a brown tumor due to primary hyperparathyroidism. He received pharmacologic therapy for stabilization of his hypercalcemia, including intravenous saline, intravenous bisphosphonates, and calcitonin. He subsequently received definitive therapy via parathyroidectomy and his post-operative course was complicated by hungry bone syndrome. Long-term follow-up has found full resolution of the lytic lesion and restored calcium homeostasis. Conclusions We present this case to highlight the possible presentations of hypercalcemia and hyperparathyroidism that are essential for a general pediatrician to recognize to ensure prompt diagnosis and management. Evaluation for hypercalcemia should be considered in patients with suggestive symptoms and physical exam findings. To our knowledge, this patient represents the first reported pediatric case of a pelvic brown tumor in an adolescent. While the multi-systemic complications of hyperparathyroidism may be quite severe, swift and appropriate management may mitigate these clinical outcomes.

Acute on Chronic Heart Failure Secondary to Left Ventricular Noncompaction

Left ventricular noncompaction (LVNC) is a rare cardiomyopathy characterized by hypertrabeculations and intertrabecular recesses most often seen in the left ventricle (LV). The patient may be asymptomatic or present with heart failure, arrhythmia, and sudden death. We discuss a previously healthy 7-year-old male who presented to the Emergency Department (ED) multiple times over a three-week period. His complaints evolved over the course of his illness, initially presenting with fatigue and suicidal ideation, followed by diffuse abdominal pain. Prior to his ICU admission, he had been discharged from the ED twice, due to well appearance and reassuring lab findings. He returned to the ED a final time with severe venous congestion and cardiogenic shock with acute hepatic injury. Echocardiogram revealed LV apical hypertrabeculation with a severe dilated cardiomyopathy and biventricular failure along with a large thrombus in the left ventricular cavity. Congestive heart failure and anticoagulation therapy was initiated, and the patient went on to biventricular assist device (BiVAD) placement and cardiac transplant. Although LVNC is rare, pediatric heart failure does present to the general pediatrician and has high morbidity and mortality. The presenting symptoms can be obscure and pose a challenge to pediatricians. This case report and review will assist in familiarizing the general pediatrician with pediatric heart failure presentation, treatment, and course.