Adverse childhood experiences in children and adolescents with sickle cell disease: A retrospective cohort study.

BACKGROUND: Adverse childhood experiences (ACEs) are linked to poor health outcomes; however, the relationship between ACEs and health outcomes among children and adolescents with sickle cell disease (SCD) has limited documentation in the published literature. PROCEDURE: This retrospective cohort study involved 45 children and 30 adolescents. Participants were screened using the Center for Youth Wellness ACE Questionnaire. Parents completed the questionnaire for children. Adolescents provided self-report. ACEs were treated as continuous and categorical scales: 0-1 verus ≥2 original ACEs (individual and/or familial level); 0-1 versus ≥2 additional ACEs (community level); and 0-3 versus ≥4 expanded ACEs (original + additional). Pain and acute chest syndrome events were compared using Wilcoxon rank-sum tests, and correlated with cumulative ACE scores using Spearman's correlation. Multivariable models were fitted to examine the association between ACEs and pain/acute chest syndrome. RESULTS: The cumulative number of original ACEs positively correlated with acute chest syndrome events (rho = .53, p = .003) and pain (rho = .40, p = .028) among adolescents. Adolescents with ≥2 versus 0-1 original ACEs had a higher number of acute chest syndrome events (4.9 ± 2.6 vs. 1.6 ± 2.2, p = .002); however, this association was confounded by asthma. Acute chest syndrome events and hospitalizations for pain did not differ among child ACE groups. Emergency department (ED) pain visits were higher among children with ≥4 versus 0-3 expanded ACEs (1.6 ± 2.8 vs. 3.3 ± 3.2, p = .042), even after controlling for SCD genotype, asthma, disease-modifying treatment, and follow-up years (p = .027). CONCLUSION: ACEs are linked to increased morbidity among children and adolescents with SCD. Prospective studies are needed to further understand this relationship and test ACE-protective remedies.

Effect of Sickle Cell Anemia Therapies on the Natural History of Growth and Puberty Patterns.

As pediatric patients with sickle cell anemia (SCA) have impaired growth and puberty patterns, we studied the effect of disease-modifying therapies on growth and puberty patterns for patients with SCA receiving hydroxyurea (HU), transfusions, or no therapy. We performed a retrospective study of children with SCA in whom anthropometric measurements and therapy type were recorded. Penalized smoothing splines were fitted to estimate growth curves and growth velocity, and linear mixed models were used to examine differences across treatment groups. Across group analyses were divided into early childhood (4.0 to 7.9 y) and peripubertal (8.0 to 12.0 y). We analyzed growth data on 157 SCA patients. From 8.0 to 12.0 years, girls on transfusion therapy were significantly taller than girls on HU (range, 5.7 to 7.2 cm; P-value range 0.002 to 0.01). From 10.0 to 12.0 years, boys on transfusion therapy were significantly taller than boys on HU (range, 4.1 to 9.4 cm; P-value range <0.0001 to 0.04). In addition, boys on transfusion therapy had an earlier peak height velocity as compared with boys on either HU or no therapy. In conclusion, children receiving transfusions tended to be taller than children on HU or no therapy. Children on HU did not demonstrate superior growth pattern when compared with children on no therapy in the peripubertal years.

Bortezomib Based Chemotherapy for Light Chain Deposition Disease.

Light chain deposition disease (LCDD) is a rare systemic disorder in which monoclonal light chains are abnormally secreted due to clonal proliferation of plasma cells and get deposited in various organs; the kidneys being the common one to be affected leading to renal failure. Advocated therapeutic options include chemotherapy with alkylating agents and steroids, High-Dose Melphalan (HDM) with Autologous Stem Cell Transplantation. Recently, Bortezomib has proven to be a novel therapeutic option in these patients when combined with dexamethasone. Here, we report a patient who presented with acute renal failure, was diagnosed to have LCDD and treated with bortezomib and dexamethasone.

Carotid Stump Syndrome: An Uncommon Cause of Recurrent Ipsilateral Strokes.

Carotid stump syndrome is a rare cause of recurrent cerebrovascular accidents. Carotid stump is the patent proximal remnant below the completely occluded internal carotid artery (ICA). Cerebral and retinal ischemic symptoms seen after complete occlusion of ipsilateral ICA is known as carotid stump syndrome. Known for causing recurrent ipsilateral cerebrovascular accidents, it is a potentially treatable entity. The therapeutic goal is medical management with a statin, dual anti-platelet therapy along with surgical intervention either with an endovascular repair or carotid endarterectomy. Herein, we present a case of carotid stump syndrome managed medically.

Acute Coronary Syndrome Presenting With Hiccups.

Acute coronary syndrome can present with atypical symptoms. Hiccups, generally considered benign and self-limiting, can be an indicator of myocardial ischemia if persistent. We present the case of a 62-year-old gentleman with a past medical history significant for hypertension, type II diabetes mellitus, and ischemic stroke who presented with persistent hiccups. Coronary angiogram revealed severe triple vessel disease and he underwent coronary artery bypass graft surgery, following which his hiccups resolved. There are very few cases that report the association of hiccups and myocardial ischemia. To our knowledge, this is the first reported case in which hiccups were a part of the primary symptoms associated with severe triple vessel coronary artery disease. This could be due to irritation of the phrenic nerve from the infarcted myocardium resulting in activation of the hiccup reflex arc. Our case highlights the association between these two common entities and stresses the importance of having a high index of suspicion, especially among high-risk and elderly patients.

Cardiac Tamponade Secondary to Influenza B Infection.

Every year, Influenza infection contributes to significant morbidity and mortality carrying a huge economic burden. Extra-pulmonary manifestations are increasingly being recognized. We present a 29-year-old woman with acute pericarditis and cardiac tamponade requiring emergent pericardiocentesis secondary to Influenza B infection. Although very rare in relation to Influenza B infection, the pericardial disease can occur during the acute infection or as a post-viral syndrome. Considering pericardial disease in patients with chest pain and any viral infection may facilitate timely diagnosis and prevent unnecessary life-threatening complications.

Challenges in Managing Pericardial Disease Related to Post Viral Syndrome After COVID-19 Infection.

Although primarily a respiratory illness, coronavirus disease 2019 (COVID-19) has been associated with cardiac involvement with reported cases of myocardial ischemia, arrhythmia, myocarditis, pericarditis, and pericardial effusion leading to cardiac tamponade. Most cases of pericardial disease in this setting have been during the acute infection. Here, we present a patient who developed pericarditis leading to cardiac tamponade after the resolution of the acute COVID-19 infection. Her course of illness was further complicated by poor response to initial medical therapy with non-steroidal anti-inflammatory drugs (NSAIDs) and colchicine which could possibly be related to early exposure to steroids. It is often difficult to establish an underlying etiology for acute pericarditis. Similarly, in our case, although there is no definitive test to prove the causal relationship, this effusion is highly suspicious of being secondary to post viral sequelae after COVID-19 infection when considering the clinical course. It is important to consider pericardial disease as a late complication of COVID-19 even after apparent resolution of the acute infection and be mindful of the therapeutic challenges that we might face while managing such patients.

Outcomes of Isolated Neutropenia Referred to Pediatric Hematology-Oncology Clinic.

BACKGROUND: Children with isolated neutropenia (absolute neutrophil count [ANC] <1500/µL) are frequently referred to pediatric hematology and oncology clinics for further diagnostic evaluation. Scant literature exists on interventions and outcomes for isolated neutropenia. We hypothesized that children will have resolution of their neutropenia without the need for intervention(s) by a pediatric hematologist and oncologist. METHODS: We performed a 5.5-year institutional review board-approved retrospective chart review of children referred to our pediatric hematology and oncology clinics for isolated neutropenia. Neutropenia was categorized as mild (ANC of 1001-1500/µL), moderate (ANC of 500-1000 µL), severe (ANC of 201-500/µL), or very severe (ANC of ≤200/µL). RESULTS: Among 155 children referred with isolated neutropenia, 45 (29%) had mild neutropenia, 65 (42%) had moderate neutropenia, 30 (19%) had severe neutropenia, and 15 (10%) had very severe neutropenia. Only 29 (19%) children changed to an ANC category lower than their initial referral category. At a median follow-up of 12 months, 101 children had resolution of neutropenia, 40 children had mild neutropenia, 10 children had moderate neutropenia, 3 children had severe neutropenia, and 1 patient had very severe neutropenia. A specific diagnosis was not identified in most (54%) children. The most common etiologies were viral suppression (16%), autoimmune neutropenia (14%), and drug-induced neutropenia (8%). Black children had a 3.5 higher odds of having persistent mild neutropenia. Six (4%) children received granulocyte colony-stimulating factor therapy. CONCLUSIONS: Most children referred for isolated neutropenia do not progress in severity and do not require subspecialty interventions or hospitalizations.