Herald of the National Children's Medical Center

Early extubation and reduced icu stay as markers of effective infusion support in children after congenital heart defect repair

Wed, 06 Aug 2025 00:00:00 +0500

Aim. To assess the impact of different initial infusion strategies, including the use of balanced Ringer’s solution, on the early postoperative course in children after surgical correction of congenital heart defects.

Materials and methods. This prospective cohort study included 70 pediatric patients who underwent radical correction of tetralogy of Fallot. The patients were divided into two groups: the control group (0.9% NaCl) and the study group (balanced Ringer’s solution). Acid-base status, electrolyte levels, hemodynamic parameters, echocardiographic findings, as well as the time to extubation and duration of stay in the intensive care unit (ICU) were analyzed.

Results. The study group demonstrated significantly higher pH values, less pronounced hyperchloremic acidosis, stable plasma osmolality, improved cardiac output (higher ejection fraction and lower end-diastolic volume). Mean arterial pressure after 24 hours was higher, and central venous pressure remained more stable. Time to extubation and ICU stay were reduced by 31.3% and 26.6%, respectively, in the study group.

Conclusion. Optimization of infusion therapy using balanced crystalloid solutions improves myocardial function and accelerates recovery in pediatric patients after cardiac surgery, as evidenced by earlier extubation and shorter ICU stay.

Comparative Effectiveness of Crystalloid and Blood-Based del Nido Cardioplegia in Children with Septal Congenital Heart Defects

Wed, 06 Aug 2025 00:00:00 +0500

Aim. To assess the frequency of myocardial ischemic injury in young children with septal congenital heart defects (CHDs) undergoing open-heart surgery, depending on the method of cardioplegic protection.

Materials and methods. A prospective study included 95 patients aged 1 to 3 years who underwent surgery under cardiopulmonary bypass at the National Children’s Medical Center. Patients were divided into two groups: the control group (n = 42) received crystalloid pharmacohypothermic cardioplegia (CC), and the study group (n = 53) received modified blood-based del Nido cardioplegia.

Results. Troponin I levels at 6 and 24 hours postoperatively, ejection fraction, mechanical ventilation duration, and ICU stay were the main outcome measures. Elevated troponin I 10 times the upper limit of normal was observed in 31.3% of the del Nido group and 62.5% of the CC group (p < 0.001). Ejection fraction decreased by 30.7% in the CC group and by 18% in the del Nido group at 24 hours post-surgery.

Conclusion. Blood-based del Nido cardioplegia provides superior myocardial protection and reduces the incidence of myocardial injury in the early postoperative period in children with CHDs.

Author’s Modification of Tip-M in Distal Forms of Hypospadias in Children: Scientific Justification and Technical Description of the Innovative Method

Dostonjon S.Nurmamotov , Furkat A.Otamuradov — Wed, 06 Aug 2025 00:00:00 +0500

Aim. To develop and validate TIP-M modification for distal hypospadias repair.

Materials and methods. Prospective cohort study of 56 patients (mean age 6.4±3.8 years) with distal hypospadias treated using TIP-M technique. The modification involves limiting urethral plate incision depth to 2mm with defect replacement using vascularized flap from inner prepuce.

Results. Early complications occurred in 26.8% patients, late complications in 7.1%. Excellent overall results achieved in 73.2% cases. Normal voiding restored in 100% patients with excellent cosmetic outcomes in 94.6%. Reoperation rate was 5.4%. Mean urethral plate width increase was 35.2±8.7%.

Conclusion. TIP-M modification demonstrates superior outcomes compared to classical techniques, providing universal applicability regardless of anatomical conditions. The technique can be recommended as first-choice method for distal hypospadias repair.

Transcatheter ventricular septal defect closure in infants under 10 kg using only arterial access: a single center experience

Alisher Yusubov , Shukhratjon Salakhitdinov — Mon, 30 Jun 2025 00:00:00 +0500

Aim. Transcatheter closure of ventricular septal defect (VSD) in infants under 10 kg is technically challenging due to small patient size, vascular access limitations, and increased risk of complications. Exclusive arterial access may offer procedural advantages by simplifying the approach. This study evaluates the safety, efficacy, and mid-term outcomes of transcatheter VSD closure using only arterial access in infants under 10 kg.

Materials and methods. Twenty infants (9 males [45%], 11 females [55%]) with a median age of 14.5 months (IQR 10–21.5) and median weight of 8.95 kg (IQR 8–9) underwent transcatheter VSD closure via arterial access alone. Median VSD size was 4.5 mm (IQR 3.5–4.95), and device diameter ranged from 7×5 to 8×6 mm (median 7×5). Median fluoroscopy dose was 19 mGr (IQR 13–29.5), and median procedure time was 50 minutes (IQR 40–60). The median follow-up duration was 15 months (IQR 12–22).

Results. Procedural success was 100%. Residual shunt at 1 year was observed in 15% of patients. Tricuspid regurgitation occurred in 30%, with no aortic regurgitation reported. No major complications or mortality were documented.

Conclusion. Transcatheter VSD closure in infants under 10 kg using only arterial access is a feasible and safe approach with favorable mid-term outcomes. This technique may reduce procedure time and contrast load, making it an attractive option for small infants.

Continuous three-stage method for the treatment of ventricular septal defects complicated by severe pulmonary hypertension

Mon, 30 Jun 2025 00:00:00 +0500

Aim. To evaluate the effectiveness of a three-stage medical-surgical method for treating ventricular septal defect (VSD) with severe pulmonary hypertension (PH) in children, aimed at improving hemodynamics and reducing mortality.

Materials and Methods. The study included 112 patients (aged 3 months to 3 years, mean age 14.4 ± 0.6 months) with VSD complicated by severe PH, from the Department of Cardiac Surgery of the National Children’s Medical Center (December 2022 – December 2023). Diagnosis was confirmed by clinical findings, chest radiography, and echocardiography. The three-stage medical-surgical method included: first stage — 4-week conservative therapy (furosemide 1–3 mg/kg, captopril 0.1–0.3 mg/kg, cefazolin 1.0 g, sildenafil 0.3–1 mg/kg); second stage — radical surgery in 61 patients (54.5%) (38 — classic VSD patch repair, 23 — optimized repair using a patch holder); third stage — postoperative therapy (furosemide, digoxin, dopamine/dobutamine, captopril, cefazolin). Surgeries were performed under cardiopulmonary bypass with hypothermia (32°C).

Results. Hemodynamic stabilization was achieved in 61 (54.5%) of 112 patients who subsequently underwent surgery: 38 classic repairs and 23 optimized repairs. The remaining 51 (45.5%) continued with repeated therapy. The optimized method reduced CPB time by 12.4% (12.5 minutes) and aortic cross-clamp time by 16.9% (10.2 minutes). No complications occurred; mortality was 3.3%.

Conclusion. The continuous three-stage medical-surgical method for treating VSD complicated by severe PH in children shows high effectiveness. It stabilizes preoperative hemodynamics, prevents progression of heart failure, and reduces mortality. The optimized VSD repair technique with a patch holder simplifies the procedure and shortens operative time. Hospital mortality was 3.3%, with deaths related to preoperative condition rather than surgical technique. No recurrences were recorded during three months of follow-up. Further studies are required to confirm long-term safety and efficacy, particularly in younger children.

Microbial Landscape and Antimicrobial Therapy of Sepsis/Septic Shock in Children and Neonates

Mon, 30 Jun 2025 00:00:00 +0500

Aim. To present an overview of current approaches to antimicrobial therapy for sepsis and septic shock in children and neonates, based on international guidelines and the microbiological characteristics of the pediatric population.

Materials and methods. A systematic review of scientific literature from the past 10–15 years was conducted using PubMed, eLibrary, Google Scholar, and ScienceDirect databases. The analysis focused on pediatric sepsis, antimicrobial therapy, and intensive care. Particular emphasis was placed on the Surviving Sepsis Campaign (2020–2021) guidelines and findings from major multicenter studies.

Results. The etiological profile of pediatric sepsis varies by age and underlying conditions. Gram- negative and gram-positive bacteria remain predominant pathogens, with an increasing prevalence of resistant strains. Early initiation of antibiotic therapy (within 1–3 hours) is associated with improved clinical outcomes. Empirical therapy should be broad-spectrum initially, followed by de-escalation guided by microbiological data. Special attention must be paid to the individualization of dosage and treatment duration.

Conclusion. Blood culture remains the primary diagnostic tool for sepsis, although the absence of bacteremia does not exclude the diagnosis when clinical and laboratory criteria are present. A single positive culture identifying pathogens such as Staphylococcus aureus (MRSA), Streptococcus pneumoniae, Klebsiella pneumoniae, Pseudomonas aeruginosa, Acinetobacter baumannii, or fungi is sufficient for confirmation. Timely initiation of antibiotic therapy—ideally within one hour of diagnosis—should be the goal for children with septic shock. For patients without overt signs of shock, antimicrobial therapy should be initiated as early as possible, with a diagnostic window of up to three hours. Decisions on continuation, narrowing, or discontinuation of therapy should be guided by clinical judgment and indirect evidence, taking into account the infection site, risk factors, and adequacy of clinical improvement.

Our Experience with Pelvic Osteotomy in Bladder Exstrophy

Talat G.Aliev , Saidanvar T.Agzamhadjayev , Zafar B.Abdulayev — Mon, 30 Jun 2025 00:00:00 +0500

Aim. To analyze the effectiveness of pelvic osteotomy in bladder exstrophy.

Materials and Methods. The study analyzed data from 30 patients with bladder exstrophy who received treatment in the Department of Pediatric Urology at the National Children’s Medical Center between October 2022 and June 2024. The age of patients ranged from 3 months to 10 years. Among them, 21 were boys (69%) and 9 were girls (31%), with a gender ratio of 2:1. Out of these 30 patients, 25 underwent surgical correction with Salter pelvic osteotomy using different fixation methods. In 13 patients, osteotomy and pelvic fixation were performed with pins and plaster cast, while in 12 patients, osteotomy and external fixation were applied. One patient underwent posterior osteotomy, and 4 patients underwent modern surgical correction of bladder exstrophy without pelvic osteotomy.

Results. Briefly summarize the main findings of the article.

Conclusion. Pelvic osteotomy in bladder exstrophy, performed during primary closure, provides several advantages: approximation of the symphysis with reduced tension during abdominal wall closure without the need for fascial flaps; placement of the posterior vesico-urethral complex deep within the pelvic ring, which increases the bladder’s outlet resistance; and approximation of the major pelvic floor muscles to the midline, where they can support the bladder neck and ultimately contribute to urinary continence.

Surgical Treatment of Congenital Lung Malformations in Children

Khashim Kh.Sultanov , Ali M.Auyezov — Mon, 30 Jun 2025 00:00:00 +0500

Aim. To improve the outcomes of surgical treatment by developing new diagnostic approaches and introducing minimally invasive organ-preserving interventions in children with congenital lung malformations (CLM).

Materials and Methods. The study is based on the results of examination and treatment of 146 pediatric patients with CLM, aged from birth to 18 years, who underwent inpatient treatment between 2004 and 2024 at the clinics of TashPMI and the National Children’s Medical Center (NCMC). Clinical and instrumental methods were applied (X-ray, MSCT, bronchoscopy, echocardiography, angiography), bronchography to determine the volume of surgery, intraoperative visual assessment, and surgical treatment including resections and bronchial sealing with biopolymer.

Results. Surgical treatment of CLM in children (VATS lobectomy and bronchial sealing) yielded positive outcomes in 92.7% of patients. VATS lobectomy and sealing provided better functional results and fewer complications, with preserved ventilatory capacity.

Conclusion. Comprehensive diagnostics is essential for evaluating CLM. VATS lobectomy and bronchial sealing, as organ-preserving methods, are effective, reduce surgical trauma, hospitalization time, and the risk of disability.

Vitamin D Deficiency and Its Clinical Correlates in Children with Dermo-Respiratory Syndrome: A Prospective Study

Dilbar Kattayeva , Dilafruz Abdullayeva — Mon, 30 Jun 2025 00:00:00 +0500

Aim. Dermo-respiratory syndrome (DRS) in children is a multifactorial condition frequently associ- ated with allergic manifestations and recurrent respiratory diseases. Vitamin D deficiency has been proposed as a contributing factor to immune dysregulation in this patient population, yet its role remains insufficiently defined in the pediatric setting.

Materials and methods. This prospective study included 151 children aged 3–15 years diagnosed with DRS and 50 age-matched healthy controls. Anthropometric measurements, body mass index (BMI), serum 25(OH)D levels, and relevant biochemical and hematological markers were evaluated. Subgroups were analyzed based on vitamin D status, severity of clinical manifestations, and frequency of exacerbations. Statistical analysis included ANOVA and X² tests to assess differences between groups.

Results. Vitamin D deficiency (<20 ng/mL) was detected in 72.2% of children with DRS, compared to 18% in controls (p<0.001). Children with severe deficiency demonstrated higher BMI values (p<0.05), increased frequency of respiratory exacerbations (mean 5.1 ± 1.3 per year), and more pronounced dermatological symptoms. Correlation analysis revealed a significant inverse relationship between serum 25(OH)D levels and both exacerbation frequency (r = –0.56) and BMI (r = –0.43). Implementation of vitamin D supplementation over a 6-month follow-up resulted in improved clinical scores and reduced exacerbation frequency by 38%.

Conclusion. Vitamin D deficiency is highly prevalent among children with dermo-respiratory syn- drome and correlates with disease severity and comorbid obesity. Routine screening and correction of vitamin D status should be considered as part of the comprehensive management strategy for pediatric DRS.

Ultrasound assessment of hemodynamic parameters for early detection and prediction of life-threatening conditions

Mon, 30 Jun 2025 00:00:00 +0500

Aim. To systematize current data on the potential of ultrasound assessment of hemodynamic parameters in preterm infants, with a focus on the use of cerebral Doppler and targeted neonatal echocardiography (TNE) for the early detection of critical conditions.

Materials and methods. A review was conducted of publications in peer-reviewed journals from 2018 to 2025, including systematic reviews, clinical guidelines, and original studies addressing the assessment of cerebral blood flow (parameters RI, PI, PSV) and functional echocardiography for the diagnosis of cardiovascular instability. The analysis included data on the application of innovative technologies such as NeoDoppler and protocols for dynamic ultrasound monitoring in neonatal intensive care units.

Results. The summarized evidence confirms the high prognostic value of cerebral Doppler in detecting the risk of ischemic and hemorrhagic brain injuries, as well as the effectiveness of TNE for assessing cardiac output, pulmonary hypertension, and patent ductus arteriosus. Integrating these methods enables the creation of a comprehensive model for hemodynamic monitoring in preterm infants.

Conclusion. Ultrasound assessment of hemodynamic parameters, including cerebral Doppler and targeted echocardiography, is a promising tool for the early prediction and prevention of life-threatening conditions in preterm infants. Further clinical studies are needed to standardize normal parameter values, develop unified protocols, and integrate innovative technologies into routine neonatal intensive care practice.

Assessment of the Adequacy of Volume Load in Craniotomies for Brain Lesions in Children

Mon, 30 Jun 2025 00:00:00 +0500

Craniotomy for primary brain lesions in children is considered one of the specific challenges of anesthesia. One of the main goals of anesthesia is to optimize intracranial pressure and maintain cerebral perfusion pressure. This ensures adequate oxygen delivery to brain tissue and, at the same time, prevents secondary brain injury. In neurosurgery, optimizing intraoperative infusion therapy has a significant effect on early awakening, hemodynamic stability, and postoperative recovery. Studies on the use of pulse pressure variation (PPV) in neurosurgical patients remain insufficient, and its impact on infusion therapy, hemodynamic stability, and tissue perfusion under central venous pressure monitoring has not been fully evaluated. Neurosurgical operations for brain tumor removal may carry a risk of hypovolemia for various reasons, including inadequate control of fluid infusion, physiologically compensated arterial hypertension, and treatment with osmotic diuretics. Formula-based fluid management is considered ineffective in neuro-oncological surgeries. Individualized “goal-directed infusion therapy” has been proven to improve postoperative outcomes (Gan T.J., Sopitt A., Maruf M., El-Moalem H., Robertson K.M., Moretti E. Anesthesiology. 2022;02(4):820–26).

These will be removed by our Editorial Office. Аssessment of the effectiveness of hodgkin’s lymphoma treatment in children using 18F-FDG PET-CT: А clinical case

Mon, 30 Jun 2025 00:00:00 +0500

Aim. To evaluate the effectiveness of Hodgkin’s lymphoma treatment in children by conducting a PET/CT scan with 18F-fluorodeoxyglucose (18F-FDG) after chemotherapy courses, based on a clinical case.

Materials and methods. The study utilized a clinical case analysis to evaluate the effectiveness of Hodgkin’s lymphoma treatment in a child using positron emission tomography combined with computed tomography (PET/CT) with 18F-fluorodeoxyglucose (18F-FDG). The approach consisted of an initial PET/CT scan to determine the disease stage and extent of involvement before treatment, followed by courses of chemotherapy and a subsequent PET/CT scan to assess the therapeutic response. The Deauville scale was used to standardize the interpretation of the results and to assess the metabolic activity of the tumor.

Results. The initial PET/CT scan on October 16, 2024, revealed extensive involvement of Hodgkin’s lymphoma, affecting the supraclavicular, mediastinal, and abdominal lymph nodes, as well as both lungs; this condition was rated as 5 points on the Deauville scale, indicating high metabolic activity. After courses of chemotherapy, a follow-up PET/CT scan on December 18, 2024, documented a complete metabolic response, as the pathological isotope uptake in the previously affected areas was no longer observed, and the result was rated as 1 point on the Deauville scale.

Conclusion. The presented clinical case demonstrates the high informational value and clinical significance of PET/CT with 18F-FDG in assessing the effectiveness of Hodgkin’s lymphoma treatment in children. This technique is the "gold standard"for evaluating therapeutic response in this disease and allows for the timely and accurate determination of a complete metabolic response. This, in turn, makes it possible to reduce the toxic burden on a growing organism by avoiding unnecessary radiotherapy and modifying treatment regimens, which ultimately improves the patient’s quality of life and chances of recovery. Therefore, the widespread implementation of PET/CT in pediatric oncology should be a priority for modern diagnostic standards.

Antibioticresistant infection and hydrocephalus: successful resolution of complications and consequences of traumatic brain injury (Case study)

Elbek Sh.Halikulov , Odilkhon A.Usmanhanov , Khusniddin B.Mamatqobilov — Mon, 30 Jun 2025 00:00:00 +0500

The brain is especially protected by the cranial vault, meninges, and blood-brain barrier. Surgical aggression increases the risk of cerebrospinal fluid leakage, which facilitates the penetration of viral and bacterial infections with subsequent meningoencephalitis. We have presented an example of the management of antibiotic-resistant meningoencephalitis and subsequent hydrocephalus by surgical correction. The experience gained, in our opinion, will complement the arsenal for combating this category of disease.

Congenital and Acquired Microcephaly: Etiopathogenesis, Clinical Course, Diagnosis and Recommendations Based on Clinical Case Observations

Umida T.Omonova , Mirzaolim F.Kholmatov — Mon, 30 Jun 2025 00:00:00 +0500

Aim. The aim of this study was to describe the etiopathogenesis, clinical course, and diagnostic features of congenital and acquired microcephaly through the presentation of clinical case observations.

Materials and methods. Two pediatric patients with microcephaly were analyzed. Clinical evaluation included history, anthropometry, neurological assessment, and instrumental investigations such as MRI, EEG, biochemical tests, and genetic analysis by next-generation sequencing (NGS).

Results. The first case revealed a homozygous pathogenic mutation in the ASPM gene, confirming primary microcephaly type 5. The second case demonstrated a PAH gene mutation associated with phenylketonuria, resulting in secondary microcephaly. Both patients showed delayed psychomotor and speech development, symptomatic epilepsy, and neurological impairment.

Conclusion. Microcephaly represents a heterogeneous group of disorders with both congenital and acquired origins. Genetic testing plays a crucial role in early diagnosis and family counseling. Clinical case observations highlight the importance of molecular genetic studies in Uzbekistan to improve diagnostic accuracy, provide genetic counseling, and develop rehabilitation strategies for affected children.

Breakthrough CAR-T Therapy in Refractory B-Cell Lymphoma: MIOT International Chennai, India Case Study

Kishore Kumar , Sabirov R.Djakhongir — Mon, 30 Jun 2025 00:00:00 +0500

CAR-T cell therapy has emerged as a promising treatment for relapsed/refractory B-cell non-Hodgkin lymphoma (R/R B-NHL), especially in patients with limited therapeutic options. We report the case of a 60-year-old male with primary chemotherapy-refractory diffuse large B-cell lymphoma (DLBCL) and comorbidities including diabetes, hypertension, and stable coronary artery disease, treated with anti-CD19 CAR-T cell therapy (NEXCAR19) at MIOT International, Chennai. Following disease progression after seven cycles of R-CHOP chemotherapy and consolidation radiotherapy, lymphocyte apheresis was performed, yielding sufficient starting material for CAR-T cell production. The patient received fludarabine–cyclophosphamide conditioning followed by infusion of 100 mL of NEXCAR19 CAR-T cells, which was well tolerated without cytokine release syndrome or immune effector cell-associated neurotoxicity syndrome. PET-CT at day +29 demonstrated a marked metabolic response (Deauville score 4). Low-dose nivolumab maintenance therapy and IMRT- based consolidation radiotherapy to residual abdominal nodes were introduced to enhance remission durability and CAR-T sensitization. This case illustrates the feasibility, safety, and promising early efficacy of anti-CD19 CAR-T therapy in chemo-refractory DLBCL and highlights the potential benefit of integrating immunotherapy with targeted radiotherapy. Long-term follow-up will determine relapse risk and sustained response.