Retinoblastoma in Kazakhstan: Incidence Trends and Survival Analysis (2015–2024)

Delays in Cervical Cancer Treatment Initiation for Patients Living With or Without HIV in Botswana: An Observational Cohort Analysis (2015-2019)

Background. This article presents the results of an analysis of the activities of the anesthesiology and resuscitation department at the Scientific Center for Pediatrics and Pediatric Surgery over the period 2020–2022. The study focused on key performance indicators of the resuscitation and intensive care units, including the number of patients treated, mortality rates, and the number of patients transferred to specialized departments. Purpose of the study. To analyze the activities of the intensive care unit departments to assess the efficiency of the resuscitation and anesthesiology unit and identify key aspects that require optimization to improve the quality of medical care. Materials and methods. The annual reporting data of the anesthesiology and resuscitation block of the Scientific Center for Pediatrics and Pediatric Surgery for the period 2020–2022 were used for the analysis. Results. As part of the study, data from four specialized departments of anesthesiology and resuscitation block at the Scientific Center for Pediatrics and Pediatric Surgery were analyzed. Among them, the oncology and hematology department ranked highest in terms of the number of patients treated, the number of children transferred from resuscitation and intensive care units, and the mortality rate. The analysis revealed that the highest number of fatal cases was recorded among newborns, with the primary cause being multiple congenital malformations. Conclusions. The Pediatric Center needs to focus its efforts on creating conditions for sustainable staffing with anesthesiologists and intensivists, as well as on regular professional development, with a particular emphasis on specialization in the field of oncology and hematology.

OEIS Syndrome: Omphalocele, Exstrophy of the Cloaca, Imperforate Anus, and Spinal Defects

The global objective of this paper is to review from the “Fellow” perspective, the current status of pediatric minimal access surgery (MAS) in terms of teaching feasibility, safety and impact on standard practice paradigms. In the pediatric general surgery field, surgeons are dealing with a wide range of pathology that includes thoracic, abdominal, urological and gynecological procedures. The learning curve is slow because of a relatively small volume of patients. However, gradually but steadily, a significant proportion of the procedures traditionally performed, with major open exposures at present, are preferentially performed by minimal access. Currently, minimal access surgery training is incorporated into adult general surgery residency/fellowship programs and teaching techniques of pediatric MAS are available only as seldom international workshops. Pediatric surgery fellowship programs with incorporated guidelines for MAS training are just recently feasible in select centers, mostly as “self” established programs. In many other pediatric surgery centers, teaching the “glamour” of MAS is quite dependent on a program director’s vision. Integration of MAS training into the secondary residency/fellowship curriculum of pediatric surgeons is the inevitable goal. MAS- minded education and research through adequate training will pay dividends and “manufacture” competent, contemporary trainees. National Pediatric Surgery Associations should be responsible for setting criteria that consider the MAS for accreditation with maintaining the international standards of these teaching programs.

Rheumatoid arthritis (RA) may be biologically reversible if treated in the first several months, yet it is unknown whether patients are diagnosed that early. We investigated the lag time between symptom onset and diagnosis of RA in a population with excellent access to rheumatology care. Using review of medical records, we evaluated all patients newly diagnosed as having RA from 1987 through 1990, at a health maintenance organization in central Massachusetts. Total lag time from symptom onset to first definite diagnosis was divided into medical encounter lag time (from symptom onset to first medical encounter) and diagnosis lag time (from first medical encounter to diagnosis). The median total lag time was 36 weeks (range 4 weeks to > 10 years). The median medical encounter lag time was 4 weeks (not all patients included in the analysis). The median diagnosis lag time was 18 weeks. Diagnosis lag time was shorter for patients with progressive disease and positive rheumatoid factor on the initial test. Of 25 patients with symmetric arthritis and positive rheumatoid factor, only 5 (20%) were diagnosed within 2 months, and 10 (40%) were diagnosed more than 6 months after symptom onset. RA diagnosis is usually delayed for several months after symptoms begin, in large part because of delay in diagnosis by the physician. Thus, the goal of initiating treatment extremely early may be unrealistic for most patients.

Relevance: Hematopoietic stem cell transplantation (HSCT) is a method of providing highly specialized care to patients with various oncological and hematological diseases, primary immunodeficiencies, as well as other congenital and hereditary diseases affecting the hematopoietic and immune systems. In Kazakhstan, HSCT has been performed for pediatric patients since 2012 at the Scientific Center of Pediatrics and Pediatric Surgery (SCPPS, Almaty, Kazakhstan). The article presents the experience of conducting allogeneic HSCT in children with oncohematological pathology at SCPPS.The study aimed to analyze the results of allo-HSCT and the possible influence of factors such as gender, conditioning regimen, donor compatibility, and status of the underlying disease at the time of HSCT on the survival rates of patients after HSCT in order to improve the treatment results and quality of life of patients with high-risk oncohematological diseases.Methods: Retrospective analysis of observational data on 53 patients after HSCT, carried out at the Scientific Center of Pediatrics and Pediatric Surgery from 2012 to 2020. Patient survival was assessed according to Kaplan-Meier, and static processing was carried out using the SPSS Statistic program.Results: In our study, 39.6% of patients were diagnosed with acute lymphoblastic leukemia (ALL, n=21), 28.85% of patients (n=15) with acute myeloblastic leukemia (AML), for aplastic anemia alloHSCT was performed in 20.75% of cases (n=11), in 9.46% (n=5) alloHSCT was performed for myelodysplastic syndrome (MDS), of which three patients (60%) had juvenile myelomonocytic leukemia (JMML). According to the results of the study, when performing allo-HSCT, the overall survival rate of patients with ALL after from a matched related donor was 63.6%, while when performing HSCT in the earliest stages from the onset of the disease, survival rates were significantly higher (83.3%). The effectiveness of HSCT was also influenced by treatment before transplantation and the presence of a fully matched related donor. In aplastic anemia, the time from the start of therapy is a significant factor.Conclusion: HSCT is an important and necessary stage of therapy for oncological and hematological diseases of high-risk groups in the early stages and in case of relapses of diseases. When HSCT was performed in the earliest period from the onset of the disease, survival rates were significantly higher (83.3%) compared to those with HSCT performed during the 3rd remission. Also, it was shown that the success of HSCT depends on previous therapy. HSCT in children with aplastic anemia should be performed early from the onset of the disease with minimal hematological load to HSCT, which guarantees engraftment.

Diversity of Service Systems in Pediatric Surgery for Fetuses, Neonates, Infants, Children, and Adolescents in Europe

Comprehensive review of a large cohort of outpatient versus inpatient open renal and bladder surgery in children

Background:The clinical development of new drugs with radiation appears to be limited. We hypothesised that phase I clinical trials with radiation therapy (RT) are initiated too late into a new drug's lifetime, impeding the ability to complete RT–drug development programmes before patent expiration.Methods:We identified novel drug–radiation phase I combination trials performed between 1980 and 2012 within the PubMed and ClinicalTrials.gov databases. Data gathered for each drug included: date the initial phase I trial with/without RT was opened/published, date of the published positive phase III trials, and patent expiration dates. Lag time was defined as the interval between opening of the phase I trial without RT and the opening of the phase I with RT. Linear regression was used to model how the lag time has changed over time.Results:The median lag time was 6 years. The initial phase I trial with RT was typically published 2 years after the first published positive phase III trial and 11 years before patent expiration. Using a best-fit linear model, lag time decreased from 10 years for phase I trials published in 1990 to 5 years in 2005 (slope significantly non-zero, P<0.001).Conclusions:Clinical drug development with RT commences late in the life cycle of anti-cancer agents. Taking into account the additional time required for late-phase clinical trials, the delay in initiating clinical testing of drug–RT combinations discourages drug companies from further pursuing RT-based development. Encouragingly, lag time appears to be decreasing. Further reduction in lag time may accelerate RT-based drug development, potentially improving patient outcomes.

Outcome in cancer may be improved by early diagnosis and prompt treatment. The objectives of this study were to determine the prediagnostic intervals (lag time) in childhood cancer and the factors that influence them at the University College Hospital (UCH), Ibadan. The study was prospective and observational and involved children diagnosed with cancer from July 2012 to June 2014 at UCH, Ibadan, Nigeria. A history of the illness was obtained and physical examination performed on each patient. Information obtained and analyzed included sociodemographic data, cancer diagnosis and stage, time intervals between onset of symptoms and diagnosis, and the reasons for delayed diagnosis. A total of 91 children were studied, comprising 46 males and 45 females. Their ages ranged from 1 month to 15.0 years, with a median of 4.0 years. Median parent lag time was 2.0 weeks, median health system or physician lag time 8.0 weeks, and median overall lag time 15.5 weeks. Overall lag time had a negative correlation with age of child at diagnosis, a positive correlation with the number of health facilities visited before diagnosis, and was shorter in mothers younger than 40 years of age. Lag time was significantly different among the diagnostic tumor categories, with Burkitt lymphoma having short times and retinoblastoma with long times. Delayed diagnosis of childhood cancer is a significant problem in Ibadan. Education of parents and health workers on early presentation and accurate diagnosis are recommended.

Background: With the Affordable Care Act (ACA), the challenge of expanding healthcare insurance to Americans without jeopardizing quality of care remains. This study was initiated to evaluate how the timing of MRI completion for shoulder pathology correlates with implementation of the ACA by comparing lag times between the initial visit and the date of MRI completion. In addition, the access to orthopaedic care after ACA implementation was evaluated by comparing shoulder MRI lag times among three cohorts based on insurer status. Methods: All shoulder and proximal upper extremity MRIs between 2009 and 2017 were reviewed, 5 yr before and 3 yr after ACA implementation. Patients were grouped into commercial, Medicare, and Medicaid cohorts. Average lag times for the pre- and post-ACA periods overall and among payor cohorts were calculated. Results: Included were 5900 MRIs, 1997 (33.8%) before and 3903 (66.2%) after ACA implementation. The difference in payor mix before and after ACA implementation was significantly different (P&lt;0.001). Median lag time increased from 23 days pre-ACA to 31 days post-ACA (P&lt;0.001). For commercial insurance, median lag time was 23 days pre-ACA compared with 28 days (P&lt;0.001) post-ACA. Median lag time pre- and post-ACA in the Medicaid cohort was 8 days compared with 30.5 days (P&lt;0.001), respectively. Pre- and post-ACA lag times in the Medicare cohort did not differ significantly (P=0.450). Conclusions: Lag times increased significantly after ACA implementation in patients with commercial and Medicaid insurance. This study provides valuable insight into unintended outcomes associated with the ACA legislation. Level of Evidence: Level III.

Efficacy and Safety of Recombinant Factor IX-FIAV and Bypassing Agents in Thrombin Generation Analyses in Hemophilia A Patient Plasma: The FIVITAS Study

Identifying a germline mutation in the multiple endocrine neoplasia type 1 (MEN1) gene in an index case has consequences for a whole family. Eligible family members should be offered genetic counseling and MEN1 mutation testing. Subsequently, clinical screening of mutation carriers according to the guidelines should be initiated. We assessed whether there is a lag time from MEN1 diagnosis of the index case to MEN1 diagnosis of family members. In addition, we determined whether this lag time was associated with an increased morbidity and mortality risk. A cohort study was performed using the Dutch MEN1 database, including >90% of the Dutch MEN1 population >16 years of age (n = 393). Fifty-eight MEN1 families were identified, of whom 57 were index cases and 247 were non-index cases (n = 304). The median lag time in MEN1 diagnosis of family members was 3.5 (range, 0-30) years. At the time of MEN1 diagnosis, 30 (12.1%) non-index cases had a duodenopancreatic neuroendocrine tumor, of whom 20% had metastases with a mean lag time of 10.9 years, in comparison with 7.1 years without metastases. Twenty-five (10.1%) non-index cases had a pituitary tumor, of whom 80% had a microadenoma and 20% had a macroadenoma, with mean lag times of 7.2 and 10.6 years, respectively. Ninety-five (38.4%) non-index cases had a primary hyperparathyroidism with a mean lag time of 9.5 years in comparison with seven patients without a primary hyperparathyroidism with a mean lag time of 3 years (P = .005). Ten non-index cases died because of a MEN1-related cause that developed during or before the lag time. There is a clinically relevant delay in MEN1 diagnosis in families because of a lag time between the diagnosis of an index case and the rest of the family. More emphasis should be placed on the conduct of proper counseling and genetic testing in all eligible family members.

What is the training in pediatric surgery like, how is pediatric surgery defined in other countries and what are the possibilities of quality control for training in pediatric surgery? The results of an inquiry together with information about training in pediatric surgery in 24 countries are summarised to show the different possibilities of organising training in pediatric surgery inside and outside Europe. The number of trainees in pediatric surgery (ranging from 0 to 339 trainees per country), the density of pediatric surgeons (ranging from 424 to 35 714 live births/year per pediatric surgeon) and the number of Centres of Pediatric Surgery (ranging from 4167 to 65,000 live births or 450,000 to 5,300,000 inhabitants per Centre) varies a lot in the different countries. Countries with a higher density of pediatric surgeons often also have a higher number of trainees irrespective of the birth rate and the number of inhabitants within the country, indicating a good infrastructure for pediatric surgery. In 87.5 % of the countries pediatric surgery is recognised as a specialty. The mean duration of training is about 6 years and 3 months, excluding the countries where it is necessary to become a fully accredited general surgeon before doing pediatric surgery. The mean duration of stay in general surgery is 2 years and 10 months. In one third of the countries it is obligatory to spend some time (3 to 12 months) in pediatrics. An elective or a compulsory period of time (1 - 6 months) in other specialties such as orthopedic surgery, plastic and reconstructive surgery, thoracic surgery and urology exists in 41.7 % of the nations. The most common subspecialties within pediatric surgery practised during the training by the resident are abdominal surgery, thoracic surgery, oncological surgery, head and neck surgery and urology. Possible means of control to guarantee a high quality of training include a defined time of stay in each subspecialty, the number of operations done by the trainee or the number of cases treated by the resident in the respective subspecialty and the distinction of different levels of surgery done by the trainee during his residence. In many cases the type of control is not specified.

POS0984 PREVALENCE OF AXIAL SPONDYLOARTHRITIS AMONG YOUNG PEOPLE CONSULTING BECAUSE OF CHRONIC LOW BACK PAIN IN A UNIVERSITY HOSPITAL IN ARGENTINA