Acute acalculous cholecystitis during the course of primary Epstein-Barr virus infection: a new case and a review of the literature

OBJECTIVE: The aim of this study was to describe a case of acute acalculous cholecystitis occurring in the course of primary Epstein-Barr virus (EBV) infection. METHODS: The clinical features of the case were analyzed and compared to those of three other similar cases reported in the international literature. RESULTS: All cases occurred in European females with cholestatic hepatitis, presented with gallbladder wall thickening, and recovered uneventfully without the need for surgical intervention. CONCLUSIONS: Acute acalculous cholecystitis may occur during the course of acute EBV infection, especially in patients with cholestatic hepatitis. Clinicians should be aware of the possible involvement of the gallbladder during EBV infection to avoid unnecessary invasive procedures or the overuse of antibiotics

Exploring Psychological Needs and Burden of Care in Parents of Children with Hemato-Oncological Diseases

Caring for a child with an acute/life threatening disease exposes parents to multiple stressors and challenges, resulting in a physical and psychological burden. Parents experience many health-related issues and worries that often remain underestimated. The aims of the study were: (a) to explore the associations between needs/disease-related issues and burden in parents of children with leukemia or Hodgkin’s disease; (b) to estimate predictors of parents’ burden using a stepwise linear regression analysis. Children (N = 33) followed an active therapy protocol (48.5%), or they were off therapy (51.5%). Forty-four parents completed surveys on caregiver burden levels and needs to cope with the child’s illness. Parental factors impacting burden (personal resources, loss of control, depression) and child’s quality of life (QoL) were also assessed. Among the needs, information about the illness/resources were the most urgently expressed by parents, followed by reassurance against fears for the child’s development and future well-being. Parents reported severe (27.3%) and moderate (22.7%) burden, with a higher percentage of caregivers with severe burden in the off-therapy phase (18.2%) than in the active-therapy phase (9.1%). The child’s decreased physical QoL and parent’s loss of control predicted higher levels of burden. The implications for supportive interventions aimed at responding to parental needs and preventing caregiver burden are discussed

Vaccine-Induced Subacute Thyroiditis (De Quervain’s) after mRNA Vaccine against SARS-CoV-2: A Case Report and Systematic Review

De Quervain’s thyroiditis, sometimes referred to as subacute thyroiditis (SAT), is the most common granulomatous disease of the thyroid, typically found after a viral infection in middle-aged women. The mRNA encoding for the angiotensin-converting enzyme-2 (ACE-2) receptor is expressed in follicular thyroid cells, making them a potential target for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Besides infection, SARS-CoV-2 vaccines have also been implicated in SAT pathogenesis. We present a case of a woman developing SAT following vaccination with Comirnaty by Pfizer Inc. (New-York, USA). We performed a systematic review of similar cases available in the literature to provide a better understanding of the topic. We searched the databases PubMed and Embase and followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement. Patient records were then sorted according to the type of administered vaccine and a statistical analysis of the extracted data was performed. No statistically significant difference between mRNA vaccines and other vaccines in inducing SAT was found, nor was any found in terms of patient demographics, symptoms at presentation, initial, or follow-up blood tests. In our case report, we described the possible association between SARS-CoV-2 mRNA-based vaccine Comirnaty and SAT

Early response does not predict outcome in children and adolescents with chronic myeloid leukaemia treated with high-dose imatinib

We investigated the predictive value of the 3-month BCR-ABL1 transcript levels in terms of responses and outcome of 44 children and adolescents (<18 years at diagnosis) with chronic myeloid leukemia (CML) treated with high-dose imatinib (IM) (340 mg/m2/day). The transcript cutoff levels of 1% and 10% BCR-ABL1 IS were not predictive of either complete cytogenetic response at any time, overall molecular response (MR) and complete MR (CMR), and progression-free survival probabilities at 5 years. The 3-month transcript levels in children and adolescents with CML treated with high-dose IM do not appear to be informative for the prediction of outcomes

Long-term results of high-dose imatinib in children and adolescents with chronic myeloid leukaemia in chronic phase: The Italian experience

Imatinib mesylate (IM) is used for the management of childhood chronic myeloid leukaemia (CML). The most effective dosage of IM and its long-term efficacy in children are not well defined. The purpose of this multicentre study is to report on the long-term results of high-dose IM (340 mg/m2 /d) in CML patients in chronic phase (CP-CML) aged <18 years at diagnosis. A total of 47 CP-CML patients with a median age at diagnosis of 11 years 9 months were enrolled in nine Italian centres. Complete cytogenetic response was achieved in 91.5% of the evaluable patients at a median time of 6 months. BCR-ABL1 International Scale 64 0.1% (major molecular response; MMR) and 640.01% (molecular response; MR) at 12 months were 66.6% and 33%, respectively. During follow-up, MMR and MR were achieved in 78.6% and 61% of children, respectively. IM was safely discontinued in 3 long-term treated children with a durable MR. Twelve patients (eight cytogenetic/molecular responders) underwent stem cell transplantation. The progression-free survival probabilities at 96 months for responding patients who continued IM and for those transplanted were 60% and 50%, respectively. After a median follow-up of 52 months (range 3-146), all patients are alive. High-dose IM is a long-term effective therapy in children and adolescents with CP-CML

High Dose Imatinib Is Effective In Children and Adolescents With Chronic Myelogenous Leukemia In Chronic Phase. The Italian Experience

Imatinib (IM) is an established first-line treatment for children with chronic myeloid leukemia (CML). However, the most effective dosage and duration of IM treatment are not well defined. This study was designed to evaluate the response to high-dose IM and long-term outcome in pediatric CML patients, previously untreated or resistant to IFN. Patients aged &lt;18 years with a diagnosis of CML in chronic phase (CP) were treated with IM at a dosage of 340 mg/m2/day. Cytogenetic analysis was performed on bone marrow (BM) cells before and during IM therapy, at planned intervals; quantitative RT-PCR was assessed on peripheral blood (PB) monthly and on BM every 3 months, according to the European LeukemiaNet recommendations for minimal residual disease quantification. Major molecular response (MMR) is defined as &lt;0.1% BCR-ABLIS, while complete MR (CMR) is considered as &lt;0.01% BCR-ABLIS. From March 2001 to February 2013, 45 CML patients in CP (18 females, 27 males; median age: 119/12 years) were recorded from 9 Italian pediatric centers. Eight patients had previously received IFN. IM was started in all patients, including 16 with an HLA-matched sibling. The dosage was modulated according to hematologic toxicity and/or appearance of WHO &gt;2 side- effects, mostly during the first 6 months of treatment (median administered dose: 309 mg/m2/day). Hematologic toxicity (medullary hypoplasia[n=1], neutropenia [n=11] and/or thrombocytopenia [n=6], anemia [n=1]) was observed in14/44 evaluable patients (32%); 13 patients (29.5%) experienced isolated or combined side effects: arthralgia/myalgia (n=10), nausea (n=1), vomiting (n=1), diarrhea (n=1), hepatitis (n=1), edema (n=1). After 3 months of IM treatment, 7/25 of tested patients (28%) obtained complete cytogenetic response (CCyR). Overall, 34/36 evaluable patients (94%) obtained a CCyR at a median time of 6 months. A molecular response (&lt;0.1% BCR-ABLIS) was achieved in 21/26 tested patients (81%) on PB and in 30/33 evaluable patients (91%) on BM. Seventeen of 26 patients (65%), including 2 with a HLA-matched sibling, obtained a CMR on PB cells and 19/33 (55%) on BM cells at a median time of 15 and 19 months, respectively. With the aim of reducing the risk of longitudinal growth impairment or to improve treatment compliance, 9 patients with sustained CMR and 2 adolescents with MMR lasting &gt;12 months received IM at the same daily dosage for 3 weeks a month (intermittent therapy). IM given without interruption was resumed in 3 of these 9 patients because of an increased BCR-ABL transcript. The growth rate showed a delay in height, which recovered over time, in 6 children who received IM prior to puberty. Overall, IM was stopped in 22/44 evaluable patients (50%) because of various reasons: stem cell transplant (SCT) in 8 patients (3 in CP, 1 in CCyR, 3 in MMR, 1 in CMR); hematologic (n=2) or extra-hematologic toxicity (n=2) (WHO grading &gt;3) during the first 6 months in 4; recurrent disease in 6 (3 increased BCR-ABL transcript, 2 cytogenetic relapse,1 blast crisis [BC]); no response in 1; CMR ( &lt;0.0032% BCR-ABL IS) lasting &gt;88 months in 2 and pancreatitis in 1 patient in CMR for 75 months. Twelve patients underwent a SCT after a median time of 8 months: 8 from an identical siblings (5 responders to IM [1 CMR, 3 MMR, 1 CCyR]), 3 MUD (2 in CCyR and 1 in MMR) and 1 cord blood in CCyR after chemotherapy for CB. Three patients, transplanted from an identical sibling, had disease recurrence after 24 (molecular relapse), 36 (cytogenetic relapse) and 83 (BC) months, respectively. At the last follow-up, all patients are alive (CMR= 25; MMR=14; CCyR=3; minor CyR=2; too early=1) at a median of 52 months (range: 3-146). Of 42 patients evaluable for treatment, 23 are receiving IM at a dosage of 340 mg/m2 (4 intermittent IM); 11 are in CMR without any treatment (8 after SCT; 3 at 32, 33 and 50 months after IM discontinuation); 4 are in treatment with dasatinib, 3 with nilotinib and 1 with IFN. In our experience, higher dose IM is an effective treatment for childhood CP CML, associated with sustained responses. Moreover, IM can be safely discontinued in pediatric CML patients with a deep CMR lasting more than 7 years. For patients candidates to a SCT, IM provides a safe bridging option to transplant, at no increased risk. Since patients may lose their response, a close and regular monitoring should be performed, mostly for those who stop IM, as SCT and new TK inhibitors may be successfully employed in patients failing IM