Therapy In Pediatric Patients Research Articles

AMINOSALICYLATE USE IN PEDIATRIC PATIENTS WITH CROHN’S DISEASE

Societal guidelines recommend against the use of 5-aminosalicylate (5-ASA) therapy for pediatric patients with Crohn’s Disease (pCD). Previous trials showed no benefit of 5-ASAs for inducing remission and similar one-year relapse rates when compared with placebo. Conversely, biologics are recommended as first-line therapy due to higher remission rates, corticosteroid-sparing effects, and potential to alter disease progression.

Efficacy of Intravitreal Anti Vascular Endothelial Growth Factor Injection Compared to Focal Therapy in Pediatric Patients with Coats Disease

Introduction: Coats disease is a retinal vascular disorder that may lead to progressive exudative retinal detachment. The standard regiment of treatment is focal therapy. However, its effectiveness may decrease when more than two quadrants of the retina exhibit vascular abnormalities. Thus, recent studies tried to use anti vascular endothelial growth factor (anti-VEGF) as therapy for the vascular problems. Aims: Comparing the efficacy between anti-VEGF injection and standard therapy regiment such as focal laser therapy. Methods: Literature searching was conducted through PubMed, ScienceDirect, Google Scholar, ProQuest, and SpringerLink. Search terms included "Coats’ Disease" and mesh terms of “Anti- VEGF". The efficacy is assessed based on Best-Corrected Visual Acuity (BCVA) and improvement in fundus manifestations. Results: All of the studies that conducted primary intravitreal anti-VEGF treatment were followed by necessary ablative treatment. These studies, such as by Yang Q and Zheng XX, et al. showed remarkable improvements in both visual acuity and anatomical outcomes. Laser therapy also gained satisfactory result in upgrading clinical stages, even though some complications including subretinal fibrosis, cataract, and vitreous hemorrhage were reported. A comparative study by Ray R, et al. noted that group undergoing anti-VEGF might require longer treatment sessions despite possible benefits. Conclusion: Anti-VEGF agents can be used as neoadjuvant in standard therapy. It did not reduce the time for full treatment, but the resolution of disease was seen in the most severe cases treated with combination therapy.

The Impact of COVID-19 Regulations on Adherence to Recombinant Human Growth Hormone Therapy: Evidence from Real-World Data

Worldwide regulations during COVID-19 positively and negatively impacted self-management in paediatric patients with chronic medical conditions. We investigated the impact of regulations on adherence to recombinant human growth hormone (r-hGH) therapy in paediatric patients with growth disorders, using real-world adherence data extracted March 2019–February 2020 (before COVID-19) and March 2020–February 2021 (during COVID-19) from the easypod™ connect ecosystem. Data from three measures of regulations were analysed: stringency index (SI), school closure and stay-at-home. The mean SI, and the proportion of days with required school closure or stay-at-home during COVID-19 were categorised as high versus medium/low based on the 75th percentile. Adherence was categorised as optimal (≥85%) versus suboptimal (<85%). Adherence data were available for 8915 patients before and 7606 patients during COVID-19. A high SI (mean ≥68) and a high proportion of required school closure (≥88%) resulted in an increase in the proportion of optimal adherence during COVID-19 versus pre-COVID-19 (p < 0.001). Stay-at-home requirements showed no statistically significant effect (p = 0.13). Stringent COVID-19 regulations resulted in improved adherence to r-hGH therapy in patients with growth disorders, supported by connected digital health technologies. Insights into patient behavior during this time are useful to understand potential influences and strategies to improve long-term adherence to r-hGH.

A Review of CAR-T Therapy in Pediatric and Young Adult B-Lineage Acute Leukemia: Clinical Perspectives in Singapore.

Approximately 10-15% of pediatric B-cell acute lymphoblastic leukemia (B-ALL) are high risk at diagnosis or relapsed/ refractory. Prior to the availability of chimeric antigen receptor T-cell (CAR-T) in Singapore and the region, the treatment options for these paediatric and young adults are conventional salvage chemotherapy or chemo-immunotherapy regimens as a bridge to allogeneic total body irradiation-based hematopoietic stem cell transplantation (allo-HSCT). This results in significant acute and long-term toxicities, with suboptimal survival outcomes. Finding a curative salvage therapy with fewer long-term toxicities would translate to improved quality-adjusted life years in these children and young adults. In this review, we focus on the burden of relapsed/refractory pediatric B-ALL, the limitations of current strategies, the emerging paradigms for the role of CAR-T in r/r B-ALL, our local perspectives on the health economics and future direction of CAR-T therapies in pediatric patients.

Safety of vagus nerve stimulation and responsive neurostimulation used in combination for multifocal and generalized onset epilepsy in pediatric patients.

The aim of this study was to assess the safety and efficacy of combined active responsive neurostimulation (RNS) and vagus nerve stimulation (VNS) therapies in pediatric patients with drug-resistant epilepsy. A single-center retrospective chart review was conducted on pediatric patients implanted with the RNS System with a concomitant active VNS System (VNS+RNS) between 2015 and 2021. Patients with at least 1 month of overlapping concomitant VNS and RNS treatment were included. Patients who had an RNS device implanted after 21 years of age, those who had responsive neurostimulators implanted after their VNS was inactivated, or those in whom the VNS battery died and was not replaced before RNS System implantation were excluded. Seven pediatric VNS+RNS patients were identified, and their courses of treatment were evaluated. All patients tolerated concurrent VNS and RNS treatment well, no device-device interactions were identified, and no major treatment-related adverse effects were noted. The median follow-up after RNS System implantation was 1.2 years. By electroclinical criteria, all 7 patients achieved 75%-99% reductions in the frequency of disabling seizures after RNS System implantation. By patient and caregiver report, 2 patients (28.6%) had 75%-99% reductions in the frequency of their disabling seizures, 2 patients (28.6%) achieved 50%-74% reductions, 2 patients achieved 1%-24% reduction in frequency of disabling seizures, and 1 patient (14.3%) experienced a 1%-24% increase in seizure frequency. The available VNS magnet swipe data identified 2 patients with 75%-99% reductions in seizure frequency as measured by magnet swipes, one with 25%-49% reductions and the other with 1%-24% increases in seizure frequency as measured by magnet swipes. This study demonstrated that RNS and VNS therapies can safely be used simultaneously in pediatric patients. RNS may potentially augment the therapeutic effects of VNS treatment. Patients in whom a response to VNS has been suboptimal should still be considered for RNS therapy.

Застосування кетогенної дієти в педіатрії: спектр можливостей

Purpose - to describe the possibilities of using a ketogenic diet (KD) in the pediatric population for therapeutic purposes in refractory seizure syndrome and congenital metabolic disorders. The historical aspects of the development of special medical nutrition to achieve stable ketosis in children for the treatment of seizures of various genesis are briefly highlighted; the development of the evidence base for the effectiveness of the KD in children is presented. A review of current data on the multifactorial mechanisms of neuroprotective effects of KD is carried out. In particular, individual ways of modulating fundamental biochemical pathways, presynaptic and postsynaptic changes in vesicular transport and release of excitotoxic and inhibitory substrates at synapses are considered. Four types of diets are described according to the distribution of proteins, fats, and carbohydrates: the classic, modified Atkins diet, medium-chain triglyceride diet, and low glycemic index diet. The indications and contraindications for the appointment of medical ketogenic dietary therapy are considered. The most appropriate age periods of childhood for the type of KD are outlined to achieve maximum effectiveness. The authors describe in detail the main objectives of the stages of preparation of the child and family for the start of dietary treatment, the principles of initiation of KD, laboratory and instrumental monitoring and discontinuation of ketogenic dietary therapy. Particular attention is paid to the issue of counseling a child before starting a KD, the main steps in calculating the proportion of macronutrients and the principles of daily nutrition. The possibilities of prescribing medical ketogenic dietary therapy outside of refractory seizures, in particular, in glucose transaminase 1 deficiency syndrome, pyruvate dehydrogenase deficiency, mitochondrial disorders, heart failure, and brain injury, are analyzed. Available special products for medical purposes (clinical nutrition) for the organization of medical ketogenic therapy in pediatric patients, especially in the first years of life, are presented. No conflict of interests was declared by the authors.

A predictive model of response to metoprolol in children and adolescents with postural tachycardia syndrome

BackgroundThe present work was designed to explore whether electrocardiogram (ECG) index-based models could predict the effectiveness of metoprolol therapy in pediatric patients with postural tachycardia syndrome (POTS).MethodsThis study consisted of a training set and an external validation set. Children and adolescents with POTS who were given metoprolol treatment were enrolled, and after follow-up, they were grouped into non-responders and responders depending on the efficacy of metoprolol. The difference in pre-treatment baseline ECG indicators was analyzed between the two groups in the training set. Binary logistic regression analysis was further conducted on the association between significantly different baseline variables and therapeutic efficacy. Nomogram models were established to predict therapeutic response to metoprolol. The receiver-operating characteristic curve (ROC), calibration, and internal validation were used to evaluate the prediction model. The predictive ability of the model was validated in the external validation set.ResultsOf the 95 enrolled patients, 65 responded to metoprolol treatment, and 30 failed to respond. In the responders, the maximum value of the P wave after correction (Pcmax), P wave dispersion (Pd), Pd after correction (Pcd), QT interval dispersion (QTd), QTd after correction (QTcd), maximum T-peak-to-T-end interval (Tpemax), and T-peak-to-T-end interval dispersion (Tped) were prolonged (all P < 0.01), and the P wave amplitude was increased (P < 0.05) compared with those of the non-responders. In contrast, the minimum value of the P wave duration after correction (Pcmin), the minimum value of the QT interval after correction (QTcmin), and the minimum T-peak-to-T-end interval (Tpemin) in the responders were shorter (P < 0.01, < 0.01 and < 0.01, respectively) than those in the non-responders. The above indicators were screened based on the clinical significance and multicollinearity analysis to construct a binary logistic regression. As a result, pre-treatment Pcmax, QTcmin, and Tped were identified as significantly associated factors that could be combined to provide an accurate prediction of the therapeutic response to metoprolol among the study subjects, yielding good discrimination [area under curve (AUC) = 0.970, 95% confidence interval (CI) 0.942–0.998] with a predictive sensitivity of 93.8%, specificity of 90.0%, good calibration, and corrected C-index of 0.961. In addition, the calibration curve and standard curve had a good fit. The accuracy of internal validation with bootstrap repeated sampling was 0.902. In contrast, the kappa value was 0.769, indicating satisfactory agreement between the predictive model and the results from the actual observations. In the external validation set, the AUC for the prediction model was 0.895, and the sensitivity and specificity were 90.9% and 95.0%, respectively.ConclusionsA high-precision predictive model was successfully developed and externally validated. It had an excellent predictive value of the therapeutic effect of metoprolol on POTS among children and adolescents.

Proton beam therapy in pediatric patients with central nervous system tumors: method features, clinical data analysis

Introduction. Central nervous system (CNS) tumors are the most common pediatric solid tumor, that need multimodality treatment approach including surgery, intensive chemotherapy (CT) and radiotherapy (RT). Due to toxicity, mostly late, RT has limited use in children, especially those under 3 years of age. The physical characteristics of proton beam therapy (PBT) minimize the risk and types of toxicity compared to photons, making it the preferred choice for pediatric use. Aim – to analyze the experience of PBT in patients under 18 years of age with various types of CNS malignancies, the assessment of risk factors for acute and late toxicity and its clinical manifestations. Materials and methods. A retrospective analysis of 63 courses of PBT performed in 58 patients with CNS malignancies for the period from 2018 to 2022 was carried out on Proton Center of the Sergey Beresin Medical Institute. The manifestations of local and systemic toxicity (predominantly acute) were analyzed, with an assessment of the influence of age, the volume of RT, the use of previous and concomitant CT on the severity of adverse events (AE). Results. In the study cohort, patients with embryonal CNS malignancies predominated (72.3 %). Median age was 5 years 2 months, median follow-up was 15 months. In 36 % of cases, a complete response was achieved by the start of PBT. Patients with craniospinal irradiation (CSI) prevailed (60 %). Among the manifestations of toxicity, AE grade I–II predominated. The most common AE were local reactions (dermatitis (84 %), focal alopecia (96 %)). Among systemic AE, hematological complications (84 %) were the most often occurred. A more pronounced toxicity profile was registered in the group with CSI, while concomitant CT, the patient’s age, previous CT did not significantly affect the severity of most of the assessed AE parameters (p &gt; 0.05). The analysis of late toxic effects was difficult due to the short follow-up period, the cumulative effect of other treatment modalities on the AE’s development. The reported complications included persistent focal alopecia (14 % of cases), endocrinopathies (7 %), neurological manifestations (6 %). One (2 %) child had a severe neurological deficit, secondary epilepsy with psychomotor regression, which can also be associated with the potentiating effect of previous CT and high-dose CT. Conclusion. The analysis of our data and literature review allows to conclude that PBT has relatively low toxicity profile, which is the determining factor for choosing this method of RT in pediatric patients. The main risk factor of AE’s was CSI. The acceptable acute toxicity of repeat PBT makes it possible to be used in pts with disease progression or relapse in case of absence of alternative therapeutic options.

Adherence to Continuous Positive Airway Pressure Therapy in Pediatric Patients with Obstructive Sleep Apnea: A Meta-Analysis.

Obstructive sleep apnea (OSA) is a public health problem that affects children. Although continuous positive airway pressure (CPAP) therapy is effective, the CPAP adherence rate in children is varied. This study aimed to evaluate the CPAP adherence rate and factors associated with CPAP adherence in children with OSA using a systematic review. The inclusion criteria were observational studies conducted in children with OSA and assessed adherence of CPAP using objective evaluation. The literature search was performed in four databases. Meta-analysis using fixed-effect model was conducted to combine results among included studies. In all, 34 studies that evaluated adherence rate and predictors of CPAP adherence in children with OSA were included, representing 21,737 patients with an average adherence rate of 46.56%. There were 11 calculations of factors predictive of CPAP adherence: age, sex, ethnicity, body mass index, obesity, income, sleep efficiency, the apnea-hypopnea index (AHI), severity of OSA, residual AHI, and lowest oxygen saturation level. Three different factors were linked to children with adherence and non-adherence to CPAP: age, body mass index, and AHI. The CPAP adherence rate in children with OSA was 46.56%. Young age, low body mass index, and high AHI were associated with acceptable CPAP adherence in children with OSA.

P534 Dual biologic or small molecules therapy in refractory paediatric inflammatory bowel disease (DOUBLE-PIBD): A multi-center study from the paediatric IBD Porto group of ESPGHAN

Abstract Background Current data on dual biologic or small molecules therapy in children are limited. The aim of the study was to evaluate the effectiveness and safety of dual therapy in paediatric patients with IBD. Methods This was a retrospective multicenter study from 14 centers affiliated with the IBD interest and Porto groups of ESPGHAN. We included children with IBD who were treated with combination of biologic agents or biologic and small molecule, with at least 3 months of follow-up under this therapy. Demographic, clinical, laboratory, endoscopic and imaging data were collected. Adverse events were recorded. All analyses were done in the intention-to-treat population. Children that discontinued therapy were considered treatment failures and were imputed for non-response, while missing data was imputed according to the last observation carried forward method. Results Sixty-two children [35 Crohn’s disease (CD), 27 ulcerative colitis (UC)], with a median age of 15.5 (IQR 13.1-16.8) years and disease duration of 3.8 (IQR 2.5-6.1) years were included. All children failed previous biologics and 47 (76%) failed at least two biologic agents. The dual therapy included anti-TNF agent and vedolizumab in 30 children (48%), anti-TNF and ustekinumab in 21 children (34%), vedolizumab and ustekinumab in 8 children (13%), and tofacitinib and other biologics in 3 children (5%). Clinical remission was observed in 21 (35%), 30 (50%) and 38 (63%) children at 3, 6 and 12 months, respectively. Of 27 children who were treated with corticosteroids at baseline, 20 (74.1%) were weaned within 3 months after the initiation of dual therapy. At 12 months of follow up, normalization of C-reactive protein and decrease in fecal calprotectin to &amp;lt; 250 mcg/g were achieved in 75% and 64%, respectively. Endoscopic and transmural healing were observed in 2/23 (9%) and 5/16 (31%) children, respectively. Male sex and diagnosis of UC were associated with higher likelihood of clinical remission (p=0.017 and p=0.020, respectively). Adverse events were reported in 29 (47%) children. While most adverse event were mild, 8 were regarded as serious and 6 (10%) led to discontinuation of dual therapy. The serious adverse events included infusion reaction to infliximab, fatigue and headache following vedolizumab infusion, severe skin eruptions (3 patients), cellulitis and skin abscess, elevated liver enzymes and deep vein thrombosis. Conclusion Dual biologic or small molecules therapy may be effective in children with otherwise refractory IBD. The risk of serious adverse events should be considered before recommending dual therapy.

Pre-hospital analgesia in pediatric trauma and critically ill patients: An analysis of a German air rescue service

BackgroundPain management in the pre-hospital setting remains a particular challenge for paramedics and emergency physicians, especially in children. This study evaluates the pre-hospital use and effect of analgesics in children with trauma or pain due to other reasons.MethodsThis study is a retrospective analysis of the database of a German air rescue service and was conducted over a period of 9 years (2012–2020) to assess pain in general and whether patients with trauma pain due to other reasons received treatment with analgesics. We included all patients in the registry under the age of 16 years. Patients with a Glasgow Coma Scale of 3 at hospital admission and incomplete records were excluded. The intensity of pain was determined by the emergency physician on scene at arrival and hospital admission in a ten-point rating scale (0 = no pain). Effective pain reduction was analyzed.ResultsOut of 227,458 cases, a total of 22,025 emergency cases involved pediatric patients aged 0–16 years. 20,405 cases were included in the study. 12,000 (58.8%) children had suffered a trauma, 8108 (39.7%) had pain due to other reasons and 297 (1.5%) had both. In total, 4,608 (38.4%) of the children with trauma were assessed having a numerical rating scale (NRS) > 4 at EMS arrival. These patients received mainly ketamine (34.5%) and the opioids fentanyl (38.7%) and piritramide (19.1%). The value on the NRS was significantly lower at admission to hospital (mean 1.9) compared with the EMS arrival (mean 6.9). In 4.9% the NRS at hospital admission was still > 4. 282 patients within the non-trauma group had a pre-hospital NRS of > 4. The pain therapy consisted of opioids (35.8%) and ketamine (2.8%). 28.4% patients in the non-trauma group received no pain medication. In 16.0% the NRS at hospital admission was still > 4.ConclusionsGerman emergency physicians achieved a sufficient pain therapy in pediatric patients with a NRS > 4 after trauma. In case of non-trauma, the pain management by the emergency physicians is restrained and less successful. The most common analgesic medications administered were ketamine and fentanyl, followed by piritramide.Trial registration: The study has been retrospectively registered at DRKS (DRKS00026222).

AMINOSALICYLATE USE IN PEDIATRIC PATIENTS WITH CROHN’S DISEASE

Abstract INTRODUCTION Societal guidelines recommend against the use of 5-aminosalicylate (5-ASA) therapy for pediatric patients with Crohn’s Disease (pCD). Previous trials showed no benefit of 5-ASAs for inducing remission and similar one-year relapse rates when compared with placebo. Conversely, biologics are recommended as first-line therapy due to higher remission rates, corticosteroid-sparing effects, and potential to alter disease progression. AIMS We aimed to describe the use of 5-ASAs in pCD and the associated patterns of biologic and corticosteroid use in a large multi-center patient cohort. METHODS We used data from the Biologic dISContinUation sTudy (BISCUIT), a multi-center study in which retrospective medical record abstraction from 7 US centers supplemented prospectively collected data from the ImproveCareNow (ICN) Network Registry. We included patients newly diagnosed with pCD (&amp;lt;18 years old) and compared patients who were prescribed a 5-ASA within 90 days of diagnosis to those who were not exposed to 5-ASAs. We used Student’s t-tests, chi-squared tests, and Wilcoxon rank sum to compare baseline characteristics, including demographics (age, gender, race, ethnicity, insurance status); disease characteristics (age at diagnosis, Paris classification, concomitant liver disease, extraintestinal manifestations); and disease activity measures (labs, disease activity indices, and extraintestinal manifestations). RESULTS Our study included 608 patients, 142 of whom (23.4%) were treated with 5-ASAs within 90 days after CD diagnosis. At baseline, younger patients (p&amp;lt;0.01), those with colonic disease (L2) (p=0.03), and those with Medicaid as their primary insurance (p=0.05) were more likely to receive 5-ASAs (Table 1). There was no difference in 5-ASA use by disease severity (p=0.52). Those on 5-ASA therapy had an 84-day longer delay in initiation of their first biologic medication, with 56.1% of patients starting a biologic at least 90 days after diagnosis (p&amp;lt;0.001). There were no differences in corticosteroid use (p=0.08) or dietary therapy (p=0.90) (Table 2). CONCLUSIONS We found 5-ASA use was common in pCD and was associated with a near 4-month delay in initiating biologic therapy. These results are a call-to-action to improve guideline dissemination and examine barriers such as insurance status in the evidence-based treatment of pCD. Our future work will evaluate differences in outcomes, biologic durability, and disease complication rates between these groups.

Prognostic value of 12-month response to therapy in pediatric patients with differentiated thyroid cancer

PurposeIn pediatric patients with differentiated thyroid cancer (DTC) we assessed the prognostic value of the 12-month response to therapy after initial treatment with surgery and radioactive iodine (RAI).MethodsWe retrospectively evaluated 94 pediatric patients with DTC, treated with surgery and RAI who were initially classified as low, intermediate or high risk of relapse of disease according to the American Thyroid Association (ATA) guidelines. Twelve months after RAI administration the response to therapy was assessed by serum thyroglobulin (Tg) measurement and neck ultrasound and patients were classified as having excellent response (ER) or no-ER.ResultsAt the 12 months evaluation, 62 (66%) patients had ER and 32 (34%) no-ER. During a mean follow-up time of 86 months (range 9–517), 19 events occurred (20% cumulative event rate). Events occurred more frequently in younger patients (p < 0.05), in those at ATA intermediate/high risk (p < 0.01) and with a pre-RAI therapy Tg level > 10 ng/mL (p < 0.001), and in those with no-ER (p < 0.001). At multivariate analysis, the evidence of no-ER was the only independent predictor of events.ConclusionIn pediatric patients with DTC, the response to therapy evaluated 12 months after initial treatment has an independent prognostic impact and is able to predict mid-term outcome. Patients with no-ER at 12 months after RAI therapy should be closely followed-up.

Continuous Renal Replacement Therapy in Pediatric Patients with Hepatitis A Virus associated Acute Liver Failure with Advanced Hepatic Encephalopathy- Effect on survival

Continuous Renal Replacement Therapy in Pediatric Patients with Hepatitis A Virus associated Acute Liver Failure with Advanced Hepatic Encephalopathy- Effect on survival

Voluntary deep inspiration breath-hold with volumetric modulated arc therapy in pediatric patients with mediastinal Hodgkin lymphoma.

Background: Deep inspiration breath-hold (DIBH) has been established to decrease normal tissue radiation dose in breast cancer. Methods: Forty-nine patients had two CT scans during DIBH or free breathing. Chest-wall position, setup verification and breath-hold monitoring were performed. Cone-beam CT and a surface image system were used for verification. Results: A total of 1617 breath-holds were analyzed in 401 fractions. The mean time bit was 6.01min. The mean breaths-holds per fraction was 4.96. The median for intra-breath hold variability was 3mm. No patient stopped treatment for intolerance. Clinical target volume margins were calculated as 0.36, 0.36and 0.32 for the three translational positions. The mean saved volume was 26.3%. Conclusion: Voluntary DIBHis feasible, tolerableand easy to apply for children with Hodgkin lymphoma involving the mediastinum.

Successful Rehabilitation of Traumatized Immature Teeth by Different Vital Pulp Therapies in Pediatric Patients

Conservative treatment of the traumatized immature vital permanent teeth is considered critical step for rehabilitating with continued normal development of root and apex closure without adverse sequelae. Hence, the present article describes the case series of successfully and effectively managing the fractured vital immature permanent teeth (Ellis Class-II and Ellis Class-III dental trauma) in pediatric patients by employing three different conservative Vital Pulp Therapy (VPT) approaches using suitable sealing/pulp-capping agents to promote apexogenesis, followed by functional and esthetic restoration. In all the three presented cases, Apexogenesis was achieved without any unfavorable outcome like pulpal or periapical sequelae. Furthermore, the current dental literature relevant to VPT procedures for apexogenesis in young permanent teeth has been thoroughly described in this article.

Aberrant myelomonocytic CD56 expression predicts response to cyclosporine therapy in pediatric patients with moderate aplastic anemia.

This study aimed to investigate the expression patterns and clinical significance of neural cell adhesion molecule-positive (CD56+) myelomonocytes in pediatric patients with moderate aplastic anemia (mAA). Fifty-six pediatric patients with mAA were enrolled in this study. The patients' clinical characteristics, laboratory data, and response to cyclosporine therapy were obtained. CD56 expression on bone marrow myelomonocytic cells was investigated using flow cytometry. The association between aberrant CD56 expression and cyclosporine response was evaluated by a multivariate analysis. CD56+ myelomonocytes were detected in 43% of the mAA cases. Aberrant CD56 expression was frequent on immature CD45dimCD16dim granulocytes and mature CD45brightCD14bright monocytes. Compared with patients with CD56- myelomonocytes (CD56- patients), patients with CD56+ myelomonocytes (CD56+ patients) were in moderate hematological condition and had a distinct bone marrow cellular composition profile, which included an increased proportion of myeloid cells and CD56bright natural killer cells and a reduced proportion of CD4+ T cells, CD8+ T cells, and B cells. The multivariate analysis determined that CD56+ myelomonocytes were a favorable factor for achieving response at 6 months after cyclosporine therapy. There was a trend towards a lower 3-year rate of evolution to severe aplastic anemia or relapse among the CD56+ patients (8%) than the CD56- patients (22%). CD56+ patients had an increased myeloid compartment and better prognosis compared with CD56- patients. The findings demonstrated the favorable role of CD56+ myelomonocytes in aplastic anemia progression.

The Prevalence and Indications of Intravenous Rehydration Therapy in Hospital Settings: A Systematic Review

(1) Objective: We performed a systematic review to explore the prevalence of intravenous (IV) rehydration therapy in hospital settings, and we assessed it by patient groups and populations. (2) Methods: A systematic review of major databases and grey literature was undertaken from inception to 28 March 2022. Studies reporting prevalence of IV rehydration therapy in a hospital setting were identified. The data were synthesised in a narrative approach. (3) Results: Overall, 29 papers met the inclusion criteria. The prevalence of IV rehydration therapy in paediatric patients ranged from 4.5% (hospitalised with diarrhoea and dehydration) to 100% (admitted to the emergency department with mild to moderate dehydration caused by viral gastroenteritis), and in adults this ranged from 1.5% (had single substance ingestion of modafinil) to 100% (hospitalised with hypercalcemia). The most common indication for IV rehydration therapy in paediatric patients was dehydration due to fluid loss from the gastrointestinal tract. Other causes included malnutrition, neuromuscular disease, bronchiolitis, and influenza. In adults, indications for IV rehydration therapy were much more diverse: fever, diarrhoea, drug intoxication, hypercalcemia, cancer, and postural tachycardia syndrome; (4) Conclusions: This systematic review showed that IV rehydration therapy in paediatric patients is often used to treat dehydration and diarrhoea, while in adults it has a broader spectrum of use. While IV rehydration therapy is important in correcting fluid problems and electrolyte status, the maintenance fluid prescribing practices vary considerably, and guidelines are scarce.

Knowledge, Perceptions and Practices about Oral Rehydration Therapy in Under Five Children Suffering from Diarrhea in Mirpur, AJK

Background: Dehydration associated with diarrheal diseases is a leading cause of mortality. Most of these deaths can be prevented by preventing or timely treating dehydration and using oral rehydration therapy is one of the options. Aim: To evaluate the knowledge and practices associated with the preparation and use of oral rehydration therapy in pediatric patients suffering from diarrhea. Study design: Descriptive cross-sectional study, Place &amp; duration of study: Mohi-ud-Din Teaching Hospital, Mirpur, AJK, from January to August 2021, Methods: Mothers and care-givers of children were selected. A structured questionnaire was used to assess the knowledge and practices of the participants. The information gathered included epidemiological and socio-demographic data as well as about the knowledge, attitude and practices regarding prevention of diarrhea, usage, preparation and mode of administration of oral rehydration therapy among study respondents. Result: Mean age of care givers was 31.53±5.973 years. Mean income of a household was 28233±10986 PKR while median income was 30000 PKR. As per the employment status, 78.33% were housewives and 21.67% of women were working. Regarding the educational status, 15.67% were illiterate while 84.33% were literate. About 46.66% of the participants knew about the various ways to prevent diarrhea. Regarding the knowledge and assessment of practices of care givers about home-based remedies and ORT, majority of the participants, 78.33%, knew about ORT, 75% have used ORT and 69.33% knew about how to prepare ORT. Furthermore, 64.66% prepared ORT using standard method and 65% knew that ORT replaced lost water in the body. Conclusion: There was a high level of information and awareness among caregivers about the oral rehydration therapy, its correct preparation, usage and method of administration leading to its appropriate usage in diarrhea patients. But still there is a need to increase the awareness among people by mass awareness campaigns to better inform the masses and to maximize the acceptability, usage and benefits of oral rehydration therapy. Keywords: Dehydration, diarrhea, rehydration, therapy, oral

Diagnosis, prophylaxis and treatment of oral complications in children with acute lymphoblastic leukemia. A narrative review

Acute lymphoblastic leukemia (ALL) is a malignant condition defined by the proliferation of immature hematopoietic bone marrow cells, which replace the normal bone marrow elements. Furthermore, immature cells will accumulate in various areas of the body. Chemotherapeutic drugs are cytotoxic and have unwanted effects on normal cells. This toxicity is acute, or it may be chronic. Approximately 60% of survivors of ALL will suffer from one or more health problems or adverse side effects of this treatment. Oral complications are a frequent consequence of oncology therapy in pediatric patients. Xerostomia, carious lesions, gingivitis, tumors of the oral cavity, bone deformities of the jaw, temporomandibular joint disorders, trismus, and infections due to abundant microbial flora are common in these children. The risk of dental anomalies (dental or root agenesis, root malformations, enamel defects, dental hypoplasia) is higher in those who started treatment before permanent teeth erupt. All children are at risk of developing oral lesions due to poor hygiene, but children who have survived cancer are at even higher risk. The survival rate of children diagnosed with ALL has significantly increased thanks to remarkable advances in chemotherapy. Therefore, it is important that these patients are constantly monitored by the dentist to treat complications arising from the treatments in a timely manner. In children diagnosed with ALL, oral health is of great importance as the oral environment changes as a side effect of hematological disorders and their treatments. Treatment of carious lesions, periodontal problems and lesions of the oral mucosa must be done before starting chemotherapy by cleaning the oral cavity to eliminate any possible risk of infection. The participation of the dentist is important in improving the preventive protocols and oral examination of the pathological processes that occur in children with ALL. Interdisciplinary collaboration between physicians is essential during the treatment of pediatric cancer patients.