Minimally invasive therapy for the resolution of a subcapsular hepatic hematoma in a newborn. / Terapia mínimamente invasiva para el manejo de un hematoma subcapsular hepático en un recién nacido.

OBJECTIVE: To describe an innovative alternative to exploratory laparotomy in a newborn with a sub capsular hepatic hematoma secondary to umbilical vein catheterization. CLINICAL CASE: A preterm baby with a history of hyaline membrane disease, pulmonary hypertension, and large patent ductus arteriosus, requiring mechanical ventilation and the use of vasoactive drugs. Umbilical catheters were inserted and through an abdomen X-ray, we observed their proper position. The patient evolved with greater requirements of vasoactive drugs, abdominal wall pallor, and abdominal distention. Abdominal ultrasound showed a subcapsular hepatic hematoma, with no signs of active bleeding, so expectant management was decided. The patient required increased vasoactive drugs and presented a decrease in hematocrit. New ultrasound showed a larger subcapsular hematoma, abundant perihe patic fluid, and the intraparenchymal position of the umbilical catheter was confirmed. Endovascular embolization was performed through the umbilical catheter with Gelita®, achieving occlusion of the capsular path. Posterior ultrasound showed a reduction of the hematoma. CONCLUSIONS: The use of embolization through angiography is not commonly used in pediatric emergencies. It is a procedure with fewer comorbidities and complications than exploratory laparotomy, therefore it should be considered as first-line therapy in patients like the one presented above. The limitation for its routine performance is the lack of available angiography operating room and trained interventional radio logy team.

Excessive Prenatal Supplementation of Iodine and Fetal Goiter: Report of Two Cases Using Three-dimensional Ultrasound and Magnetic Resonance Imaging.

Fetal thyroid complications in pregnancy are uncommon, and are commonly related to the passage of substances through the placenta. The excessive iodine intake during the pregnancy is a well-known mechanism of fetal thyroid enlargement or goiter, and invasive procedures have been proposed for the treatment of fetal thyroid pathologies. In the present report, we demonstrate two cases from different centers of prenatal diagnosis of fetal thyroid enlargement and/or goiter in three fetuses (one pair of twins, wherein both fetuses were affected, and one singleton pregnancy). The anamnesis revealed the ingestion of iodine by the patients, prescribed from inadequate vitamin supplementation. In both cases, the cessation of iodine supplement intake resulted in a marked reduction of the volume of the fetal thyroid glands, demonstrating that conservative treatment may be an option in those cases. Also, clinicians must be aware that patients may be exposed to harmful dosages or substances during pregnancy.

As complicações fetais da tireoide na gravidez são incomuns e são comumente relacionadas à passagem de substâncias pela placenta. A ingestão excessiva de iodo durante a gravidez é um mecanismo bem conhecido de aumento da tireoide ou bócio fetal, e procedimentos invasivos foram propostos para o tratamento de patologias da tireoide fetal. No presente relato de caso, demonstramos dois casos de diferentes centros de diagnóstico pré-natal de aumento da tireoide fetal e/ou bócio em três fetos (um par de gêmeos, em que ambos os fetos foram afetados, e uma gravidez única). A anamnese revelou a ingestão de iodo pelos pacientes prescrita por suplementação inadequada de vitaminas. Nos dois casos, a interrupção da ingestão de suplemento de iodo resultou em uma redução acentuada do volume das glândulas tireoides fetais, demonstrando que o tratamento conservador pode ser uma opção nestes casos. Além disso, os médicos devem estar cientes de que as pacientes podem ser expostas a doses ou substâncias nocivas durante a gravidez.

Excessive prenatal supplementation of iodine and fetal goiter: report of two cases using three-dimensional ultrasound and magnetic resonance imaging / Suplementação pré-natal excessiva de iodo e bócio fetal: Relato de dois casos utilizando ultrassonografia tridimensional e ressonância magnética

Abstract Fetal thyroid complications in pregnancy are uncommon, and are commonly related to the passage of substances through the placenta. The excessive iodine intake during the pregnancy is a well-known mechanism of fetal thyroid enlargement or goiter, and invasive procedures have been proposed for the treatment of fetal thyroid pathologies. In the present report, we demonstrate two cases from different centers of prenatal diagnosis of fetal thyroid enlargement and/or goiter in three fetuses (one pair of twins, wherein both fetuses were affected, and one singleton pregnancy). The anamnesis revealed the ingestion of iodine by the patients, prescribed from inadequate vitamin supplementation. In both cases, the cessation of iodine supplement intake resulted in a marked reduction of the volume of the fetal thyroid glands, demonstrating that conservative treatmentmay be an option in those cases. Also, clinicians must be aware that patients may be exposed to harmful dosages or substances during pregnancy.

Resumo As complicações fetais da tireoide na gravidez são incomuns e são comumente relacionadas à passagem de substâncias pela placenta. A ingestão excessiva de iodo durante a gravidez é um mecanismo bem conhecido de aumento da tireoide ou bócio fetal, e procedimentos invasivos foram propostos para o tratamento de patologias da tireoide fetal. No presente relato de caso, demonstramos dois casos de diferentes centros de diagnóstico pré-natal de aumento da tireoide fetal e/ou bócio em três fetos (um par de gêmeos, em que ambos os fetos foram afetados, e uma gravidez única). A anamnese revelou a ingestão de iodo pelos pacientes prescrita por suplementação inadequada de vitaminas. Nos dois casos, a interrupção da ingestão de suplemento de iodo resultou em uma redução acentuada do volume das glândulas tireoides fetais, demonstrando que o tratamento conservador pode ser uma opção nestes casos. Além disso, os médicos devem estar cientes de que as pacientes podem ser expostas a doses ou substâncias nocivas durante a gravidez.

Growth restriction increases the risk of bronchopulmonary dysplasia, death, and sepsis in twins of 30 weeks or less of gestation. / Restricción de crecimiento aumenta el riesgo de displasia broncopulmonar, muerte y sepsis en gemelos de 30 o menos semanas de gestación.

INTRODUCTION: Multiple factors influence the risk of morbidity and mortality of premature infants with intrauterine growth restriction (IUGR). The comparison of twins with different intrauterine growth allows evaluating the effect of the restriction, excluding maternal factors and prenatal mana gement. Our objective was to assess the effect of IUGR on acute and chronic morbidity, and mortality of extreme preterm twins. PATIENTS AND METHOD: Twins weighing less than 1500 grams and gesta tion equal to or less than 30 weeks, of the Neocosur Network. Separate analyses were performed on concordant twin pairs, and on mild and severe discordant twins, evaluating the effect of IUGR on morbidity and mortality. A multivariate analysis was performed in order to establish the impact of this effect. RESULTS: 459 twin pairs, 227 concordant twins, 110 of mild discordance, and 122 of severe discordance. Among the concordant ones, there was only a difference in oxygen uptake at 36 weeks. In those of mild discordance, the smaller twin presented a lower frequency of hyaline membrane disease and required fewer doses of surfactant, but had a higher risk of bronchopulmonary dysplasia (BPD) or death. In severe discordant twins, the smaller one presented higher mortality, sepsis, use and permanence in mechanical ventilation, despite the lower frequency of hyaline membrane disease. In multiple regression analysis, the combined risk of BPD or death was higher in the smaller twin and of severe discordance. CONCLUSION: In discordant twins, the acute respiratory pathology was more frequent in the larger one, although the risk of BPD or death was higher in the one with IUGR.

Restricción de crecimiento aumenta el riesgo de displasia broncopulmonar, muerte y sepsis en gemelos de 30 o menos semanas de gestación / Growth restriction increases the risk of bronchopulmonary dysplasia, death, and sepsis in twins of 30 weeks or less of gestation

INTRODUCCIÓN: Múltiples factores influyen en el riesgo de morbimortalidad del prematuro con restricción del crecimiento intrauterino (RCIU). La comparación de gemelos con crecimiento intrauterino discordante permite evaluar su efecto, excluyendo factores maternos y manejo prenatal. Nuestro objetivo fue evaluar el efecto de la RCIU sobre la morbilidad aguda, crónica y mortalidad, en parejas de recién nacidos gemelares prematuros extremos. PACIENTES Y MÉTODO: Gemelos menores de 1500 g y 30 semanas de gestación, de la Red Neocosur. Se realizaron análisis separados de pares de gemelos concordantes, discordantes leves y severos, evaluando el efecto de la RCIU sobre morbi-mortalidad. Se realizó análisis multivariado para establecer magnitud del efecto. RESULTADOS: 459 pares de gemelos, 227 concordantes, 110 discordantes leves y 122 severos. Entre los concordantes solo hubo diferencia en uso de oxígeno a las 36 semanas. En discordantes leves, el menor tuvo menos enfermedad de membrana hialina y requirió menos dosis de surfactante, pero tuvo un mayor riesgo de Displasia broncopulmonar (DBP) o muerte. En discordantes severos, el menor presentó mayor mortalidad, sepsis, utilización y permanencia en ventilación mecánica, pese a menor frecuencia de enfermedad de membrana hialina. En regresión múltiple, el riesgo combinado de DBP o muerte fue mayor en gemelo menor y discordante severo. CONCLUSIÓN: En gemelos discordantes, la patología respiratoria aguda fue más frecuente en el gemelo mayor, aunque el riesgo de DBP o muerte fue mayor en el gemelo con RCIU.

INTRODUCTION: Multiple factors influence the risk of morbidity and mortality of premature infants with intrauterine growth restriction (IUGR). The comparison of twins with different intrauterine growth allows evaluating the effect of the restriction, excluding maternal factors and prenatal mana gement. Our objective was to assess the effect of IUGR on acute and chronic morbidity, and mortality of extreme preterm twins. PATIENTS AND METHOD: Twins weighing less than 1500 grams and gesta tion equal to or less than 30 weeks, of the Neocosur Network. Separate analyses were performed on concordant twin pairs, and on mild and severe discordant twins, evaluating the effect of IUGR on morbidity and mortality. A multivariate analysis was performed in order to establish the impact of this effect. RESULTS: 459 twin pairs, 227 concordant twins, 110 of mild discordance, and 122 of severe discordance. Among the concordant ones, there was only a difference in oxygen uptake at 36 weeks. In those of mild discordance, the smaller twin presented a lower frequency of hyaline membrane disease and required fewer doses of surfactant, but had a higher risk of bronchopulmonary dysplasia (BPD) or death. In severe discordant twins, the smaller one presented higher mortality, sepsis, use and permanence in mechanical ventilation, despite the lower frequency of hyaline membrane disease. In multiple regression analysis, the combined risk of BPD or death was higher in the smaller twin and of severe discordance. CONCLUSION: In discordant twins, the acute respiratory pathology was more frequent in the larger one, although the risk of BPD or death was higher in the one with IUGR.

Condylar Hyperplasia in a Monozygotic Twin Girl: An Argument About Etiology.

The diagnosis of unilateral condylar hyperplasia (UCH) requires a combined assessment consisting of clinical, radiological, and histopathological examination. The etiology of this condition is unknown. The purpose of this study is to report a rare case of UCH in a monozygotic twin. A 15-year-old girl was referred to our department complaining of facial asymmetry and malocclusion. Computed tomography and single-photon emission computed tomography imaging reveal enlargement of the left condyle and condylar neck, and an increased uptake that was diagnosed as active UCH. During the investigation of family and co-twin facial profile, no cases of UCH were identified. From the case reported in this study, the authors raise a hypothesis that can exist some environmental factor that is related in the development of condylar hyperplasia given the occurrence of this disease in one of twins.

Exploring the association between genetic and environmental factors and molar incisor hypomineralization: evidence from a twin study.

BACKGROUND: The etiology of molar-incisor hypomineralization (MIH) remains unknown. Studies indicate that it is multifactorial, and that genetic and environmental factors are involved. Research with twins provides important subsidy to investigate the Influence of genetics and environmental factors that act during pregnancy on the etiology of alterations. AIM: This cross-sectional study evaluated the agreement of molar incisor hypomineralization (MIH) between monozygotic and dizygotic twin pairs and the association with environmental factors. DESIGN: The sample consisted of 167 pairs of twins (8-15 years old), 94 monozygotic and 73 dizygotic. The parents answered a questionnaire on sociodemographic data and pre-, peri-, and postnatal health. A dental examination was performed by two calibrated examiners (Kappa ≥0.88) for MIH diagnosis, following the criteria proposed by the European Academy of Pediatric Dentistry in 2003. RESULTS: The prevalence of MIH was 29.3%. There was greater concordance of MIH between monozygotic twins for affected first molars and permanent incisors (P = 0.0012) and pairs of twins assessed (P = 0.0211). The presence of MIH was associated with family income between one and two wages (P = 0.009, prevalence ratio [PR] = 3.82, confidence interval [CI 95%] 1.40-10.44), above two wages (P = 0.007, PR = 4.60, 95% CI: 1.51-14.05), and gestational hemorrhage (P = 0.032, PR = 5.70, 95% CI: 1.16-28.14). CONCLUSIONS: The greater concordance in the diagnosis of MIH among monozygotic twins indicates a genetic influence, although environmental factors, such as family income and hemorrhage during pregnancy, are also associated with the occurrence of MIH.

Continuous Venovenous Hemodialysis Via Extracorporeal Membrane Oxygenation Pump for Treatment of Hyperammonemia Secondary to Propionic Acidemia in Monochorionic Diamniotic Twin Boys.

Late-preterm twins with propionic acidemia developed severe hyperammonemic encephalopathy at 5 days of age. Continuous venovenous hemodialysis was performed successfully for both infants via extracorporeal membrane oxygenation pump, and both rapidly improved. They were taken off continuous venovenous hemodialysis and extracorporeal membrane oxygenation and discharged with dietary therapy. At 3 years of age, neurodevelopment showed globally delayed milestones.

Perinatal Outcome of Discordant Anomalous Twins: A Single-Center Experience in a Developing Country.

A dramatic increase in twin pregnancies has been observed in the past few decades, primarily related to assisted reproductive techniques (ART) and increased average maternal age during pregnancy. Multiple pregnancies, compared to singleton pregnancies, are associated with greater perinatal morbidity and mortality. The present study evaluated the perinatal outcomes of pregnancies with discordant anomalous twins in a tertiary maternity ward in a developing country. Data were retrospectively collected from the Instituto Fernandes Figueira/FIOCRUZ, Brazil between January 2002 and December 2014. We identified 74 twin pregnancies with discordant anomalous twins. Final data analysis was based on 40 pregnancies. Congenital defects were classified according to the International Classification of Diseases: ICD-10: the digestive system was responsible in 27 (34%) cases; the central nervous system was responsible in 18 (22%) cases; the urinary tract was responsible in 14 (17%) cases; and the circulatory system was responsible in 14 (17%) cases. A total of 19 deaths occurred during the study period, and delivery before 30.4 weeks was a significant prediction of fetal death (p = .01). The presence of hydrops in the affected fetus was related to a higher number of deaths in healthy fetuses and contributed to a worse prognosis. The presence of this condition was the cause of 12 (55.6%) deaths in healthy fetuses. A 10 times higher risk of death of a normal co-twin was observed in cases of death of the anomalous twin (p = .002, OR 10.55, 95% CI: 1.9-58.52).

Esculetin, a coumarin derivative, exerts in vitro and in vivo antiproliferative activity against hepatocellular carcinoma by initiating a mitochondrial-dependent apoptosis pathway

This study investigated the in vitro and in vivo antiproliferative activity of esculetin against hepatocellular carcinoma, and clarified its potential molecular mechanisms. Cell viability was determined by the MTT (tetrazolium) colorimetric assay. In vivo antitumor activity of esculetin was evaluated in a hepatocellular carcinoma mouse model. Seventy-five C57BL/6J mice were implanted with Hepa1-6 cells and randomized into five groups (n=15 each) given daily intraperitoneal injections of vehicle (physiological saline), esculetin (200, 400, or 700 mg·kg-1·day-1), or 5-Fu (200 mg·kg-1·day-1) for 15 days. Esculetin significantly decreased tumor growth in mice bearing Hepa1-6 cells. Tumor weight was decreased by 20.33, 40.37, and 55.42% with increasing doses of esculetin. Esculetin significantly inhibited proliferation of HCC cells in a concentration- and time-dependent manner and with an IC50 value of 2.24 mM. It blocked the cell cycle at S phase and induced apoptosis in SMMC-7721 cells with significant elevation of caspase-3 and caspase-9 activity, but did not affect caspase-8 activity. Moreover, esculetin treatment resulted in the collapse of mitochondrial membrane potential in vitro and in vivo accompanied by increased Bax expression and decreased Bcl-2 expression at both transcriptional and translational levels. Thus, esculetin exerted in vitro and in vivo antiproliferative activity in hepatocellular carcinoma, and its mechanisms involved initiation of a mitochondrial-mediated, caspase-dependent apoptosis pathway.

Congenital hypothyroidism caused by excess prenatal maternal iodine ingestion.

We report the cases of 3 infants with congenital hypothyroidism detected with the use of our newborn screening program, with evidence supporting excess maternal iodine ingestion (12.5 mg/d) as the etiology. Levels of whole blood iodine extracted from their newborn screening specimens were 10 times above mean control levels. Excess iodine ingestion from nutritional supplements is often unrecognized.

Outbreak of Candida parapsilosis in a neonatal intensive care unit: a health care workers source.

Nosocomial neonatal candidiasis is a major problem in infants, which require intensive therapy. The subjects of the present study were three preterm infants admitted to the neonatal intensive care unit of the General Hospital "Dr. Manuel Gea Gonzalez". The infants developed Candida parapsilosis infection on the mean age of 13.6 day of life. Prior to fungemia, infants had received assisted ventilation and hyperalimentation through central venous catheter. Sequence analysis of the internal transcribed spacer gene ruled out other Candida species and revealed that the eight isolates were C. parapsilosis. The isolates were examined based on their molecular relation by random amplified polymorphic DNA analysis. The profiles allowed the identification of two main genotypes of C. parapsilosis as the outbreak cause and as a result of the cross-infection with health care workers' hands. We conclude that C. parapsilosis commonly colonize through horizontal transmission due to the staff's noncompliance of hand hygiene procedures.

[Twin pregnancy with complete mole and coexisting fetus after in vitro fertilization and embryo transfer complicated with placenta previa accreta. A case report]. / Embarazo gemelar con mola completa y feto coexistente posterior a fertilización in vitro y transferencia de embriones complicado con placenta previa acreta. Reporte de un caso.

The double twin pregnancy with complete hydatidiform mole and coexistent fetus is a rare event and perinatal treatment complex. Presents a significant case of this unusual partnership and describes their evolution. Patient of 33 years, secondary infertility factor-peritoneal tube and pregnancy achieved by in vitro fertilization and embryo transfer. An ultrasound early pregnancy reported twice, a sack was a complete mole, another bag was a fetus and placenta previa unchanged total. The case is carefully monitored and uterine inhibitors were administered at different stages of gestation. It settled the case by caesarean section at 37 weeks and obstetric hysterectomy for placenta previa percreta molar involution of the placenta and newborn health. The evolution of the mother and the child was appropriate.

Dizygotic twin pregnancy conceived with assisted reproductive technology associated with chromosomal anomaly, imprinting disorder, and monochorionic placentation.

Assisted reproductive technology (ART) has now become a cornerstone of treatment for involuntary infertility. Recent studies have raised concern regarding potential associations between ART and chromosomal aneuploidy, imprinting anomalies, and monochorionic placentation in dizygotic (DZ) twins. We report a case of DZ twins conceived by ART in which all three problems coexist.

Cluster of Candida parapsilosis primary bloodstream infection in a neonatal intensive care unit.

Candida parapsilosis is an increasingly important bloodstream pathogen in neonatal intensive care units (NICU). We investigated a cluster of bloodstream infections in a NICU to determine whether nosocomial transmission occurred. During a 3-day period, 3 premature infants hospitalized in the same unit presented with sepsis caused by C. parapsilosis. Electrophoretic karyotype of the organisms was performed by using pulsed field gel electrophoresis in a countour-clamped homogeneous electric field system. The isolate from 1 newborn could not be typed, and the isolates from the remaining 2 infants had identical patterns. All 3 cases are described. We conclude that nosocomial transmission of C. parapsilosis occurred and that neonates under intensive care may represent a risk group for this pathogen.

Malformaciones congénitas en gemelos / Congenital malformations in twins

Background: A higher prevalence of congenital malformations among twins than among single has been reported in the literature. Aim: To study the incidence of congenital malformations in twins in the maternity hospital of the University of Chile in the period 1983-1997. Material and methods: As part of the Latin American Collaborative Study of Congenital Malformations, every newborn in the maternity hospital was examined by a neonatologist and congenital malformations were recorded in a special file. Results: In the study period there were 48.663 deliveries of single pregnancies and 448 deliveries of twin pregnancies, giving birth to 886 twins borns alive and 17 stillbirths (1.9 percent mortality). Of these, there were 423 twins, 17 triplets, a sextuple delivery and two siamese. Seven point one percent of twins born alive and 6.5 percent of single newborns had a congenital malformation. The prevalence of malformations in twins and single stillbirths was 35.2 and 17.2 percent respectively (p <0.04). In four pairs and siamese newborns, malformations typical of monozygotic twins were present, such as an acardiac fetus with Trap sequence, a papyraceous fetus and two with hydroanencephaly. Twelve twins had malformations attributable to uterine compression. Conclusions: This study did not find a higher frequency of malformations comparing twins with single babies born alive, but in stillbirths there was a significant difference between single and twin stillbirths (7.1 percent versus 35.2 percent, respectively, p <0.05)

Neonatal hyperammonemia caused by a defect of carnitine-acylcarnitine translocase.

Carnitine-acylcarnitine translocase deficiency is a newly recognized inborn error of metabolism that involves transport of long-chain fatty acids into mitochondria, which in turn impairs mitochondrial beta-oxidation, and ketogenesis. We report a new familial example; the affected twins had neonatal distress, hyperammonemia, and transient intracardiac conduction defects. Clinical and biochemical analysis of both our patients and the two previously reported patients revealed that this inherited defect could be manifested during the neonatal period without any of the signs classically associated with fatty oxidation defects. In contrast, all four patients had sustained and "isolated" hyperammonemia, which could be misinterpreted as being caused by urea cycle defects. We conclude that carnitine-acylcarnitine translocase deficiency is a potential differential diagnosis in neonates with unexplained neonatal hyperammonemia. Cardiac and muscle involvement may represent further early pivotal symptoms.