Oral ferrous salts are standard treatment for children with iron deficiency anemia (IDA). The objective of our study was to monitor oral iron therapy in children, aged 3 months–12 years, with IDA. We ...prospectively collected clinical and hematological data of children with IDA, from 15 AIEOP (Associazione Italiana di Ematologia ed. Oncologia Pediatrica) centers. Response was measured by the increase of Hb from baseline. Of the 107 analyzed patients, 18 received ferrous gluconate/sulfate 2 mg/kg (ferrous 2), 7 ferrous gluconate/sulfate 4 mg/kg (ferrous 4), 7 ferric iron salts 2 mg/kg (ferric), 62 bis-glycinate iron 0.45 mg/kg (glycinate), and 13 liposomal iron 0.7–1.4 mg/kg (liposomal). Increase in reticulocytes was evident at 3 days, while Hb increase appeared at 2 weeks. Gain of Hb at 2 and 8 weeks revealed a higher median increase in both ferrous 2 and ferrous 4 groups. Gastro-intestinal side effects were reported in 16% (ferrous 2), 14% (ferrous 4), 6% (glycinate), and 0 (ferric and liposomal) patients. The reticulocyte counts significantly increased after 3 days from the start of oral iron supplementation. Bis-glycinate iron formulation had a good efficacy/safety profile and offers an acceptable alternative to ferrous iron preparations.
Abstract
SMARCB1 inactivation is a well-established trigger event in atypical teratoid/rhabdoid tumor. Recently, a role for SMARCB1 inactivation has emerged as a mechanism of clonal evolution in ...other tumor types, including rare brain tumors. We describe an unusual malignant intra-axial SMARCB1-deficient spindle cell desmoplastic neoplasm, occurring in a 6-year-old child with meningioangiomatosis and a long history of seizures. Striking features of the tumor were a storiform pattern and strong CD34 expression. Undifferentiated round cell areas with isolated rhabdoid cells showing high mitotic index and focal necrosis with INI1 expression loss were present. The meningioangiomatosis component showed few chromosomal imbalances, including chromosomal 22 monosomy (where SMARCB1 maps) and gain at 6q14.3. In addition to these abnormalities, the spindle cell desmoplastic neoplasm and its dedifferentiated SMARCB1-deficient component shared several other aberrations, including homozygous deletion at 9p21.3, losses at 1p, 3p, 3q, 10p, and 13q, gains and losses at 5p and 11p. In line with INI1 loss, the dedifferentiated component showed remarkably decreased levels of SMARCB1 transcript. The residual SMARCB1 allele was wildtype. Our findings suggest progression from the meningioangiomatosis to the malignant desmoplastic neoplasm through the occurrence of complex chromosomal abnormalities, and point to functional silencing of SMARCB1 in the dedifferentiation component.
Having cancer in childhood is a risk factor for separation anxiety symptoms, with negative effects on the disease and psychological wellbeing. The Covid-19-pandemic-related concerns could have a ...negative effect. The present study explores the interplay between separation anxiety symptoms and COVID-19-related worries in pediatric cancer patients and their mothers, compared to a group of healthy children and their mothers, during the pandemic. Thirty-three subjects with cancer, aged 7-15 years, and their mothers were compared to a control sample of 36 healthy children and caregivers. They were administered a pandemic-related psychological experience survey and standardized questionnaires assessing psychological wellbeing, anxiety, and separation anxiety symptoms. Children with cancer reported significantly higher prosocial behaviors, fear of being alone, and fear of abandonment. Their mothers had worse psychological wellbeing, higher COVID-19 concerns, anxiety, and separation anxiety symptoms. The multiple linear regression model showed that an increase in children's separation anxiety symptoms was associated with younger age, more recent diagnosis, more mother-child time, lower mothers' worry for children's contagion, and higher mothers' and children's anxiety. COVID-19-related worries of clinical children's mothers seem to be protective for children's psychological wellbeing. Early psychosocial support interventions for mothers are essential in health services for cancer.
The laparoscopic splenectomy in pediatric patients is performed worldwide but often the disproportion between size of patients and size of organs requires an extra laparotomic access for spleen ...removal. The aim of the present study was to evaluate the safety and effectiveness of the Alexis
®
system to retrieve the spleen without additional laparotomic access. The charts of all patients who underwent splenectomy at our center during the last 5 years were retrieved. In all the cases the Alexis
®
system was placed in the umbilicus, thru which a 10 mm camera was inserted. Three additional 5 mm standard trocars were inserted. Seven patients, affected by spherocytosis (3), epidermoid cyst (2), idiopathic thrombocytopenic purpura (2) and thalassemia (1), underwent laparoscopic splenectomy at a median age of 10 years (range: 8–17). Median patients’ weight was 32.5 kg (range: 25–71) and spleen size 15 cm (11–18). In all the cases, upon removal of the camera, the retrieval bag was inserted thru the umbilicus under direct view, the spleen retrieved, morcellated, and removed. No conversion nor enlargement of one of the ports nor an extra laparotomic access were required. The patients were discharged on the fifth post-operative day and the cosmetic results were excellent. Removal of the spleen can be safely performed without any additional laparotomy thru the Alexis
®
system placed in the umbilicus. This system is effective also in case of major patient/organ size disproportion and the final cosmetic aspect is excellent.
Nutritional status plays a crucial role in the mortality rates of the pediatric oncology patients. However, there is a lack of systematic approaches for nutritional assessment in this population. ...This study aims to assess the current practice for nutritional assessment and care of pediatric cancer patients in Italy. A 25-items web-based, nation-wide questionnaire was circulated as of January 9, 2023 among physicians within the AIEOP network, composed of 49 national centers, out of which 21 routinely perform HCT. This survey examined the practices of 21 Italian pediatric oncology centers, revealing significant heterogeneity in nutritional practices. Only half of the centers routinely assessed all patients, utilizing different clinical and biochemical parameters. The use of neutropenic diets remained prevalent after chemotherapy or stem cell transplantation.
Conclusion
: This study underscores the pressing need for unified recommendations to improve nutritional care and potentially enhance outcomes for pediatric cancer patients.
What is Known:
• The assessment and support of nutrition are gaining interest in the overall care of children with cancer.
• The assessment and management of nutritional needs in pediatric cancer patients, including those undergoing hematopoietic cell transplantation, currently lack a systematic approach.
What is New:
• There is considerable variability in the nutritional assessment and support among Italian centers treating pediatric patients with cancer.
• To enhance nutritional assessment and support for pediatric cancer patients, it is essential to establish shared national and international guidelines.
Immune thrombocytopenia (ITP) is an acquired immune-mediated bleeding disorder characterized by isolated thrombocytopenia. Its estimated yearly incidence in the pediatric population is ...1.9-6.4/100,000. ITP in children is usually a self-limiting and benign disorder. The clinical management of children with ITP often remains controversial, as robust randomized trials on the management of this disorder are lacking. Treatments vary widely in clinical practice and existing guidelines from hematology societies on clinical management offer indications based largely on expert opinion rather than strong evidence.
The Coagulative Disorder Working Group of the Italian Association of Pediatric Hematology and Oncology (AIEOP) developed this document to collect shared expert opinions on the management of newly diagnosed ITP, updating previous guidelines and providing recommendations to pediatricians. Each statement has been given a score expressing the strength of evidence, appropriateness and agreement among participants.
Clear-cut definitions of the clinical phases of the disease and clinical response are stated. Recommendations are given regarding the classification of bleeding symptoms, evaluation of bleeding risk, diagnosis, and prognostic factors. Specific recommendations for treatment include indications for first-line (intravenous immunoglobulins, steroids) and second-line (combined therapy, thrombopoietin receptor agonists, immunosuppressive drugs, rituximab) therapeutic agents, as well as hemorrhagic emergency and supportive treatment, including emergency splenectomy. The optimal follow-up schedule, the relation between ITP and vaccines and health-related quality-of-life issues are also discussed.
The panel achieved broad consensus on issues related to how to treat children with newly diagnosed ITP, providing a comprehensive review of all relevant clinical aspects.
Despite the continuous improvement of cancer treatment protocols, altered testicular function and infertility frequently represent major adverse effects of oncologic treatments. Thus, strong efforts ...are needed to avoid or at least to reduce these complications that are particularly relevant in young men without offspring. Furthermore in the last years, concerns have been raised about the possible mutagenic effect of chemotherapy on sperm. Alkylating agents are frequently and successfully used in the treatment of paediatric tumors despite their well-known gonadotoxic effect. While gonadal toxicity of cyclophosphamide has been well demonstrated, little and conflicting data are reported about the effects on testicular function of ifosfamide. The aim of this study was to compare long-term effects of ifosfamide versus cyclophosphamide based therapies, on testicular function, fertility and sperm aneuploidies in a group of 33 young males survivors of childhood cancer. Patients who had received cyclophosphamide showed a severe gonadal failure characterized by reduced testicular size, very low sperm count and some degree of Leydig cell impairment. On the contrary, in subjects who had received ifosfamide all parameters of testicular function including sperm aneuploidies were in the normal range, despite of different dose, protocol of infusion and pubertal stage at treatment. In conclusion, our results confirm data of literature reporting the high gonadal toxicity of cyclophosphamide and suggest that ifosfamide treatment seems to be safer for testicular function and fertility.
Exercise stimulates immune responses, but the appropriate "doses" for such achievements are unsettled. Conversely, in metabolic tissues, exercise improves the heat shock (HS) response, a universal ...cytoprotective response to proteostasis challenges that are centred on the expression of the 70-kDa family of intracellular heat shock proteins (iHSP70), which are anti-inflammatory. Concurrently, exercise triggers the export of HSP70 towards the extracellular milieu (eHSP70), where they work as pro-inflammatory cytokines. As the HS response is severely compromised in chronic degenerative diseases of inflammatory nature, we wondered whether acute exercise bouts of different intensities could alter the HS response of lymphocytes from secondary lymphoid organs and whether this would be related to immunoinflammatory responses. Adult male Wistar rats swam for 20 min at low, moderate, high or strenuous intensities as per an overload in tail base. Controls remained at rest under the same conditions. Afterwards, mesenteric lymph node lymphocytes were assessed for the potency of the HS response (42 °C for 2 h), NF-KB binding activity, mitogen-stimulated proliferation and cytokine production. Exercise stimulated cell proliferation in an "inverted-U" fashion peaking at moderate load, which was paralleled by suppression of NF-KB activation and nuclear location, and followed by enhanced HS response in relation to non-exercised animals. Comparative levels of eHSP70 to iHSP70 (H-index) matched IL-2/IL-10 ratios. We conclude that exercise, in a workload-dependent way, stimulates immunoinflammatory performance of lymphocytes of tissues far from the circulation and this is associated with H-index of stress response, which is useful to assess training status and immunosurveillance balance.
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