Calling the anatomically posterior ventricle “left” we must nevertheless keep in mind its expansion steals both left and posterior space, jeopardizing the potential trajectory of the stylet. Even if the drain entrance into the chest is safe, the enlarged ventricle will be prone to meet the needle inside the cavity.

УДК 616.155.32-056.76-07-08

Гемофагоцитарний лімфогістіоцитоз (haemophagocytic lymphohistiocytosis, HLH) — рідкісне, потенційно смертельне захворювання, характеризується надмірною активацією імунної системи. При ньому порушується регуляція активації цитотоксичних Т-лімфоцитів, природних клітин-кілерів (natural killer, NK) і макрофагів, викликаючи ураження багатьох систем та органів. Сімейний гемофагоцитарний лімфогістіоцитоз (familial hemophagocytic lymphohistiocytosis, f-HLH) — форма HLH, спричинена спадковими мутаціями в генахрегуляторах цитотоксичної активності природних кілерів NK-клітин, цитотоксичних Т-лімфоцитів. Виявлення генетичної мутації в гені PRF1 підтверджує діагноз f-HLH-2, що визначально для заперечення вторинного HLH.

Aim: to review information resources and analysis of the own experience on this problem for the provision of modern knowledge in the pathogenesis of the pathology, the latest diagnostic and treatment technologies, with consideration of the need to adhere to a single strategy in the management of patients with BA.
Materials and Methods: The analysis of the data regarding the results of existing studies evaluating the clinical benefit and safety of diagnostic and treatment methods in Biliary atresia.
Conclusions: BA is the leading cause of neonatal cholestasis development. Early diagnostics of BA, based on the complex evaluation of clinical-laboratory, instrumental and morphological signs of the pathology, has a significant meaning. Surgical correction during the first 2 months of life – the Kasai procedure, as well as dynamic post-surgery follow-up significantly prolong the life of children and allow postponing liver transplantation. The highest patient survival both at the first stage of treatment - conduction of the Kasai procedure and the stage of liver transplantation may be achieved by joined work of surgeons and pediatricians, which allows considering the whole row of possible problems. 

УДК 616.345-002-07:616-003.829.1-056.7-008.89-053.2

Посттрансплантаційний лімфопроліферативний розлад (Posttransplant lymphoproliferative disorder, PTLD) — це лімфоїдна і/або плазмоцитарна проліферація, що виникає внаслідок імуносупресивної терапії (ІСТ), яку призначають пацієнтам для запобігання відторгнення трансплантованого органа, в умовах трансплантації солідних органів або алогенних гемопоетичних клітин. Тригером розвитку PTLD може бути активна реплікація Епштейна–Барра вірусу (Epstein–Barr virus, EBV), а також інші фактори ризику. Цей розлад становить спектр клінічних захворювань, від доброякісного захворювання, подібного до мононуклеозу, до фульмінантної лімфоми. Раннє розпізнавання PTLD важливе при трансплантації органів у пацієнтів, оскільки має тенденцію до швидкого прогресування. Знайомство з клінічними особливостями PTLD і підвищена пильність є важливими для встановлення діагнозу

INTRODUCTION AND OBJECTIVE:

Access to renal transplantation in children with severe chronic kidney disease can be endangered in dangerous sociopolitical environments. Despite such challenges, we established the very first adult and pediatric renal transplantation program in Ukraine in 2021 during an ongoing war and the COVID-19 pandemic, which caused significant delays and difficulties, including disrupted supply chains and shortages of critical medical supplies and equipment and availability and access to transplant resources and personnel. Here, we describe our experience with establishing and conducting a pediatric renal transplantation program during wartime and a pandemic in Ukraine.

METHODS:

We conducted a retrospective cohort study of 20 pediatric patients who underwent renal transplantation between January 2021 and September 2023 at two large-volume pediatric care centers in Lviv. Due to Ukrainian laws, donations could not be taken from soldiers and military personnel or civilians who suffered due to hostilities. We managed immunosuppressive medications and antibiotic prophylaxis or treatment post-transplant, and nearly all patients were on dialysis prior to transplantation.

RESULTS:

Our program constituted 23% (189/821) of all transplants performed in Ukraine in the last three years, and we have expanded our efforts to Western Ukraine. The majority of our patients did not undergo native nephrectomy, and most patients were on dialysis prior to transplantation. Average age at the time of transplant was 12.6 +4.5 (years), and average length of time on dialysis was 18 months. Overall, 30-day graft survival was 95%. Two patients experienced acute rejection that was successfully managed medically, while one had graft thrombosis requiring nephrectomy on the day of surgery.

CONCLUSIONS:

Despite the challenges of establishing a renal transplantation program during wartime and the impact of COVID-19, we have successfully started a pediatric renal transplantation program in Ukraine with a 95% 30-day graft survival rate. Our efforts have constituted 23% of all transplants performed in Ukraine in the last three years, and we have expanded our program to Western Ukraine. Our experience highlights the importance of access to necessary care in challenging environments and the need for continued support and collaboration in these settings.