Miracles of science: Birth after uterus transplantation.

AIM: The concept of regaining childbearing ability via uterus transplantation (UTx) motivates many infertile women to pursue giving birth to their own children. This article provides insight into maternal and neonatal outcomes of the procedure globally and facilitates quality of care in related medical fields. METHODS: The authors searched ISI Web of Science, MEDLINE, non-PubMed-indexed journals, and common search engines to identify peer-review publications and unpublished sources in scientific reference databases. RESULTS: The feasibility of the procedure has been proven with 46 healthy children in 88 procedures so far. Success relies upon dedicated teamwork involving transplantation surgery, obstetrics and reproductive medicine, neonatology, pediatrics, psychology, and bioethics. However, challenges exist owing to donor, recipient, and fetus. Fetal growth in genetically foreign uterine allograft with altered feto-maternal interface and vascular anatomy, immunosuppressive exposure, lack of graft innervation leading to "unable-to-feel" uterine contractions and conception via assisted reproductive technology create notable risks during pregnancy. Significant portion of women are complicated by at least one or more obstetric problems. Preeclampsia, gestational hypertension and diabetes mellitus, elevated kidney indices, and preterm delivery are common complications. CONCLUSIONS: UTx has short- and long-term satisfying outcome. Advancements in the post-transplant management would undoubtedly lead this experimental procedure into mainstream clinical practice in the near future. However, both women and children of UTx need special consideration due to prematurity-related neonatal problems and the long-term effects of transplant pregnancy. Notable health risks for the recipient and fetus should be discussed with potential candidates for UTx.

Evaluation and management of neonatal onset hyperinsulinemic hypoglycemia: a single neonatal center experience.

OBJECTIVES: To evaluate the clinical characteristics and treatment options of neonates requiring prolonged hospitalization due to persistent hyperinsulinemic hypoglycemia (HH). METHODS: This retrospective cohort study included infants >34 weeks of gestation at birth who were born in our hospital between 2018 and 2021, diagnosed with HH, and required diazoxide within the first 28 days of life. The baseline clinical characteristics, age at the time of diagnosis and treatment options in diazoxide resistance cases were recorded. Genetic mutation analysis, if performed, was also included. RESULTS: A total of 32 infants diagnosed with neonatal HH were followed up. Among the cohort, 25 infants were classified as having transient form of HH and seven infants were classified as having congenital hyperinsulinemic hypoglycemia (CHI). Thirty-one percent of the infants had no risk factors. The median birth weight was significantly higher in the CHI group, whereas no differences were found in other baseline characteristics. Patients diagnosed with CHI required higher glucose infusion rate, higher doses, and longer duration of diazoxide treatment than those in the transient HH group. Eight patients were resistant to diazoxide, and six of them required treatment with octreotide and finally sirolimus. Sirolimus prevented the need of pancreatectomy in five of six patients without causing major side effects. Homozygous mutations in the ABCC8 gene were found in four patients with CHI. CONCLUSIONS: The risk of persistent neonatal hyperinsulinism should be considered in hypoglycemic neonates particularly located in regions with high rates of consanguinity. Our study demonstrated sirolimus as an effective treatment option in avoiding pancreatectomy in severe cases.

Specific Granule Deficiency Due To Novel Homozygote SMARCD2 Variant.

Background: Specific granule deficiency (SGD) is a rare immunodeficiency associated with CCAT/enhancer-binding protein epsilon (CEBPE) gene variants. It can cause severe recurrent infections and is lethal without successful stem cell transplantation. Few cases with SGD of both type 1 and type 2 have been described in the literature. In this study, we present the first report of a case with a novel homozygous c.511 C > T (p.Gln171Ter) mutation in the SMARCD2 gene of SGD type 2, which was successfully treated with bone marrow transplantation. Case: A male infant presented to our neonatal intensive care unit on the second day of life with an icteric appearance and mild hypotonia. He was evaluated for immunodeficiency as the cause of delayed cord separation and refractory neutropenia. At 6 weeks of age, SGD type 2 with a new variant was diagnosed and successfully treated by bone marrow transplantation. Conclusion: SGD is an immunodeficiency disease that is quite rare. However, we believe that SGD diagnosis and associated new variants can be detected more frequently with the widespread use of all whole-exome sequencing techniques.

Birth of a Healthy Baby 9 Years After a Surgically Successful Deceased Donor Uterus Transplant.

OBJECTIVE: To describe surgical procedures, previous failed pregnancies, methods for overcoming pregnancy failure and, most importantly, birth of a healthy infant, in a uterus transplantation from a deceased donor. BACKGROUND: Majority of uterus transplants have involved live donors, but several advantages make deceased donor transplantation a practicable option, principally by eliminating surgical risks to the live donor. METHODS: Uterus transplantation from a deceased donor was performed in September 2011 in Turkey. After 5 miscarriages, perfusion computed tomography revealed an obstructed blood-outflow. To overcome this blood flow obstruction, a saphenous vein graft was anastomosed between utero-ovarian and left ovarian vein with laparotomy. Follow-up computed tomography confirmed resolution of venous congestion and a decrease in uterine volume. RESULTS: Following vascular augmentation surgery, fetal cardiac activity were observed 28 days after the first embryo transfer attempt. Preterm premature rupture of the membranes was diagnosed at 19 weeks' gestation. Cesarean section was planned at 28 weeks' gestation due to intrauterine growth restriction and suspected preeclampsia. A healthy 760 g male baby was delivered. The baby was discharged from the neonatal intensive care unit 79 days after delivery in good condition weighing 2475 g. CONCLUSIONS: Deceased donor uterus transplantation is a reasonable approach for treating uterine factor-related infertility. In case of recurrent miscarriages, regional vascular augmentation by arterial or venous supercharging may be required to overcome regional misperfused regions determined by imaging studies.

Comparison of the Efficacy Between Intravitreal Aflibercept and Laser Photocoagulation in the Treatment of Retinopathy of Prematurity.

PURPOSE: To compare the efficacy of intravitreal aflibercept and laser photocoagulation in the treatment of retinopathy of prematurity (ROP). METHODS: The files of patients who were diagnosed as having type 1 ROP or aggressive posterior ROP (APROP) and treated with laser photocoagulation and 1 mg/0.025 mL of intravitreal aflibercept were retrospectively analyzed. The patients' birth weight, gestational age, detection week of the disease, zone, stage, presence of plus disease and rubeosis, regression of ROP, re-treatments administered during the follow-up, and spherical equivalent values obtained at the corrected sixth month were recorded. RESULTS: The study included 27 eyes of 15 patients who underwent laser photocoagulation and 24 eyes of 12 patients who received intravitreal aflibercept. Retinal vascularization was in zone II in all eyes in the laser photocoagulation group and zone 1 in 22 eyes (91.7%) in the intravitreal aflibercept group (P < .05). In the laser photocoagulation group, 25 eyes (92.6%) had stage 3 ROP and 2 eyes (7.4%) had stage 2 ROP. In the intravitreal aflibercept group, 14 eyes (58.3%) had stage 3 ROP and 10 eyes (41.7%) had APROP (P < .05). Treatment was established at a postmenstrual age of 37.6 ± 2.5 weeks in the laser photocoagulation group and 34.2 ± 2.4 weeks in the intravitreal aflibercept group (P < .05). The regression rates after treatment were 92.6% and 100%, respectively (P > .05). In the intravitreal aflibercept group, laser photocoagulation was performed on 10 eyes (41.6%) during follow-up visits. Spherical equivalents were measured as +1.10 ± 2.30 and +1.50 ± 2.41 diopters, respectively (P < .05) at the corrected sixth month. CONCLUSIONS: Intravitreal aflibercept is an effective treatment for ROP. However, it requires more additional treatments than laser photocoagulation during the follow-up visits. [J Pediatr Ophthalmol Strabismus. 2020;57(1):54-60.].

Colonización e infección por un microorganismo poco frecuente en una unidad de cuidados intensivos neonatales: tres recién nacidos prematuros con Elizabethkingia meningoseptica / Colonization and infection with a rare microorganism in a neonatal intensive care unit: three preterm infants with Elizabethkingia meningoseptica

Elizabethkingia meningoseptica es un bacilo gramnegativo ampliamente distribuido en la naturaleza pero poco frecuente en humanos que se presenta en infecciones intrahospitalarias. Los avances en las instalaciones de cuidados intensivos neonatales y el uso de dispositivos médicos sofisticados fortalecen la capacidad infecciosa invasiva del microorganismo. Las manifestaciones clínicas suelen incluir bacteriemia primaria, meningitis, neumonía intrahospitalaria, bacteriemia relacionada con el uso de catéteres intravasculares e infecciones gastrointestinales y de las vías biliares. Es posible que, en entornos con recursos limitados, la falta de sistemas de diagnóstico mejorados sea una de las causas por las que no se notifican todas esas infecciones. Resulta bastante difícil distinguir entre colonización e infección, y el patrón de sensibilidad a los antibióticos es diferente. Por consiguiente, los médicos deben realizar el diagnóstico preciso para así evitar el tratamiento incorrecto. En este artículo, describimos tres casos de recién nacidos con diagnóstico de infección y colonización por E. meningoseptica con el objetivo de destacar la importancia del diagnóstico y el tratamiento oportunos de esta bacteria poco frecuente pero letal que ya está presente en las unidades de cuidados intensivos

Elizabethkingia meningoseptica is a widespread gram-negative bacillus in the environment, but a arely reported human pathogen presenting mostly as nosocomial infections. Advances in neonatal intensive care facilities and usage of sophisticated medical devices strengthen the invasive infectious potential of the microorganism. Clinical manifestations usually include primary bacteremia, meningitis, nosocomial pneumoniae, intravascular catheter-related bacteremia and gastrointestinal and biliary tract infections. Lack of improved diagnostic systems in resource constrained settings, might be a cause of underreporting of such infections. Discrimination between colonization and infection is quite difficult, and it has an unusual antibiotic susceptibility pattern. Therefore clinicians should pay special attention to accurate diagnosis in order to prevent mistreatment. Here we report three newborn cases with the diagnosis of E. meningoseptica infection and colopnization, with the aim of drawing attention to the diagnosis and management of this rare but lethal bacteria that is already present in the intensive care unit environment

Prevalencia de microorganismos patógenos respiratorios durante dos temporadas consecutivas de virus sincicial respiratorio en un centro de atención de alta complejidad / Prevalence of respiratory pathogens during two consecutive respiratory syncytial virus seasons at a tertiary medical care center

Objetivo. Determinar el perfil etiológico de la infección de vías respiratorias bajas (IVRB) en los neonatos durante la temporada de virus sincicial respiratorio (VSR) y definir las características de esta y otras infecciones. Métodos. Estudio retrospectivo con neonatos hospitalizados por IVRB durante dos temporadas de VSR, con reacción múltiple en cadena de la polimerasa en tiempo real. Se revisaron los datos relevantes y se compararon las características de la infección por VSR con las de otros microorganismos. Resultados. Entre 224 pacientes, 160 (71 %) fueron positivos para, al menos, un agente causal potencial. Entre ellos, el 65 % tenía VSR y el 15 %, más de un agente (coinfección). En el grupo con VSR, hubo más dificultad respiratoria (p < 0,01), radiografías de tórax anormales (p < 0,01), requerimiento de atención en terapia intensiva (p < 0,01) y más días de oxigenoterapia (p < 0,01). No obstante, hubo menos casos de fiebre al ingreso y menos días de antibioticoterapia (en ambos, p < 0,01), y la duración de la hospitalización no fue más prolongada. Los pacientes con coinfección requirieron atención en terapia intensiva en mayor medida que los demás (25 % contra 6,5 %, p < 0,01). Conclusión. Este estudio demostró que el VSR fue más frecuente en los neonatos hospitalizados por IVRB durante la temporada y condujo a una evolución más grave que otros microorganismos patógenos detectados. Posiblemente la gravedad de la infección por VSR se viera aumentada por la presencia de una coinfección y radiografía de tórax anormal.

Aim: To determine the etiological profiles of lower respiratory tract infection (LRI) in neonates during respiratory syncytial virus(RSV) season, and to define the clinical features of RSV-related infection and others. Methods: The retrospective study included newborn infants who were hospitalized for LRI during the two consecutive RSV seasons, and then tested for possible etiological agent by multiplex real-time polymerase chain reaction. All relevant data were reviewed, and the clinical characteristics of RSV-related infection were compared to those of others. Results: Of 224 patients, 160 (71 %) were positive for at least one potentially causative agent. Of them, 65 % had RSV, and 15 % had more than on ecausative agent (co-infection). The RSV group had more the findings of respiratory distress (p< 0.01), abnormal chest radiography (p< 0.01), need for intensive care (p< 0.01), and duration of oxygen requirement (p< 0.01) but less fever on admission and duration of antibiotic use (for both, p< 0.01), and no longer hospital stay. Need of intensive care nursery was more common in patients with co-infection than others (25 % vs. 6.5 %, p< 0.01). Conclusion: This study highlighted that RSV was the most frequent agent in neonates hospitalized for LRI during the season, with a more severe clinical course than other detected pathogens. The disease severity of RSV infection may have seemed to be increased by the presence of coinfection and abnormal chest radiography.