Cumulative risk factors and mental health of mothers of infants admitted to the neonatal intensive care unit.

Purpose: The present study examined predictive linkages between multiple risk factors and their contribution to the development of anxiety and depression in Puerto Rican mothers of infants admitted to the Neonatal Intensive Care Unit (NICU).Method: The scales used were: the Hamilton Anxiety Rating Scale, the Edinburgh Postpartum Depression Scale, the Hollingshead, and a Demographic Questionnaire was constructed to obtain information about mother and infant characteristics.Results: Both the cumulative psychosocial risk factor (B = 0.267, p = .011) and the cumulative neonatal risk factor (B = -0.220, p = .039) were significant predictors of mothers' anxiety.Discussion: It could be beneficial to create psychosocial interventions in the NICU to address parents' needs and promote emotional resilience. Also, training staff to provide an adequate explanation to mothers, regarding the infants' recovery process is of particular importance.

Congenital Zika Syndrome in Puerto Rico, Beyond Microcephaly, A Multiorgan Approach.

OBJECTIVE: Zika virus (ZIKV) infection was identified in Puerto Rico on December 2015, and the outbreak encouraged us to characterize clinical manifestations and laboratory findings of intrauterine exposed infants. METHODS: Retrospective medical record review of infants born to mothers with confirmed ZIKV infection during pregnancy was performed from January 2016-June 2017. We included patients admitted to UPH Neonatal Intensive Care Unit or referred for follow-up at UPH High Risk Clinics. The database was approved by the University of Puerto Rico, Medical Sciences Campus, IRB. RESULTS: 191 infants born to ZIKV positive mothers during pregnancy were identified. Normal head sonogram was found in 93% of the normo cephalic infants. Ocular findings were reported in 50% of the patients with microcephaly and 31% of the normo-cephalics. Fifteen newborns (7.8%) presented with microcephaly, of which 73% showed calcifications in head sonogram, and had severe anomalies on brain MRI. Auditory brainstem response test was performed on all newborns, 80% were within normal limits. CONCLUSION: Among the group of infants born to mothers with Zika positive test 4% had microcephaly. Of concern to us is the fact that 31% of normo cephalic infants had ocular manifestations and 7% of them had findings on head sonogram. While microcephaly is the severest form of Congenital Zika Syndrome, ocular manifestations might characterize the spectrum of disease. These findings reiterate the importance of detailed neonatal evaluations of exposed infants.

The Zika Virus Infection in Pregnancy: Review and Implications for Research and Care of Women and Infants in Affected Areas.

The world has encountered a new and serious epidemic which has disproportionately affected fetuses and infants. What makes the Zika virus (ZIKV) epidemic such a threat in our times, is that a whole generation can be affected by birth defects caused by a seemingly innocuous maternal infection, which in most cases go unnoticed and undiagnosed. Spreading to over 80 countries and affecting millions, it is associated with severe birth defects known as congenital Zika syndrome (CZS), which include fetal brain development abnormalities (microcephaly and brain calcifications), retinal abnormalities, and contractures and hypertonia of the extremities. Testing strategies are challenging because of the lack of symptoms and cross reactivity with other viral infections. Obstetrical complications include fetal loss and the need for an emergency cesarean delivery. The rate of CZS has been described as ranging from 5 to 6% among cohorts in the US, reaching 11% for 1st trimester exposure. Prolonged viremia during pregnancy has been documented in a few cases, reaching 89 days after the onset of symptoms in one case and 109 days after such onset in another. If the ZIKV can infect, multiply in, and persist in diverse placental cells, then movement across the placenta, the fetal brain, and the maternal peripheral blood is possible. There is a sense of urgency, and we need safe and effective vaccines and treatments, particularly for pregnant women. If we do not expand testing and develop methods for early diagnosis and treatment, thousands of infants will be exposed to a neurotropic virus that causes severe birth defects and that could also affect the lives of those who form the next generation.

Association of Zika Virus Exposure in Utero with Ocular Phenotypes in a Group of Newborns in Puerto Rico Exposure with ocular phenotypes in newborns in Puerto Rico.

OBJECTIVE: After the outbreak in January 2016, researchers in Brazil reported the first cases of ophthalmic abnormalities in infants with microcephaly and presumed Zika virus (ZIKV) intrauterine infection. Screening for ocular lesions in all newborns exposed to ZIKV prenatally has been emphasized because of a chorioretinal macular scar found in a number normocephalic patient exposed to ZIKV. METHODS: A retrospective review of the medical records of infants born to mothers found to be ZIKV-positive during their pregnancies (January 2016-July 2017) was performed. We included all newborns and infants admitted to the NICU and/or receiving ambulatory care at the high-risk clinics at the University Pediatric Hospital, San Juan, Puerto Rico. The creation of this database was approved by the IRB of the University of Puerto Rico, Medical Sciences Campus. RESULTS: Records from 234 newborns born to Zika-positive mothers were identified. The ophthalmic evaluations of 95 patients were available. Sixty-three of them had normal findings (66%). Twelve of the 95 patients had microcephaly (12.6% of the cohort). Of the microcephalic group, half had normal ophthalmological findings. Of the normocephalic group (83/95), almost 31% had ocular findings: a small optic nerve in 3%, a double-ring sign in 10%, macular stippling in 8%, mottling in 4%, an oval optic nerve in 2%, a tilted nerve in 1%, and blunted foveal reflex in 1%. CONCLUSION: We report herein ZIKV associated ophthalmologic findings, similar to those published, in 34% of the infants with suspected/confirmed ZIKV fetal exposure. These data underline the importance of ocular examination in patients with suspected congenital Zika but without other findings on physical exam.

First Autopsy of a Newborn with Congenital Zika Syndrome in Puerto Rico.

We report on the first autopsy performed on a neonate with congenital Zika syndrome in Puerto Rico. A term male was born to a mother with confirmed Zika virus infection; he had a prenatal diagnosis of microcephaly and multiple cerebral calcifications, among other anomalies, and a normal male karyotype (determined by amniocentesis). He required neonatal resuscitation at birth and died at 2 days ofage. At autopsy, his head circumference was only 1.5 standard deviations below the mean, not fulfilling the criteria for microcephaly. He presented scissor legs, clenched and hyperflexed hands, and multiple contractures (arthrogryposis). The central nervous system findings were consistent with Zika encephalopathy: ventriculomegaly, lissencephaly, and severe encephalic degeneration with numerous dystrophic calcifications, among other findings. These anomalies were most likely secondary to congenital ZV infection. Although prenatally diagnosed with microcephaly, he did not fulfill the criteria after birth, which fact indicates the need for reassessment of the definition of microcephaly as it applies to patients exposed prenatally to the ZV.

Zika Virus Infection in Pregnancy: Maternal, Fetal, and Neonatal Considerations.

An infection with the Zika virus (ZIKV) is usually mild, with nonspecific symptoms and most often asymptomatic. However, because of its causal relationship with severe congenital malformations, the ZIKV epidemic became an imperative for mobilization, renewed strategies for vector control, and biomedical research. A congenital Zika syndrome (CZS) has been characterized with 5 distinctive features that focus on brain development abnormalities (including microcephaly and brain calcifications), retinal manifestations, and defects on extremities including congenital contractures and hypertonia. The CZS could be just "the tip of the iceberg", pending the documentation of a spectrum of disease that could manifest later in life, from mild dysfunction to severe disease. It will be a matter of time for neurodevelopmental abnormalities, learning disabilities, and other unknown but yet-to-be-described outcomes to be associated with intrauterine ZIKV infection. In addition, ZIKV infection during pregnancy has been associated with other adverse outcomes. Reports mostly include ZIKV-affected pregnancies, and it will be difficult to clearly establish causality without appropriate control groups. We are summarizing some of the known or reported consequences of such infection during pregnancy. Women of reproductive age and particularly pregnant women are the most vulnerable to the adverse consequences of the ZIKV epidemic. Vector control programs need to be expanded to curtail new infections. Research is needed to develop safe and effective treatments, a preventive or therapeutic vaccine, and specific and sensitive tests and to diagnose and identify correlates of long-term immunity. Vaccines and treatments should be safe to be used in pregnancy. To do nothing would allow thousands of pregnant women to expose their fetuses to an infection that causes birth defects and other problems. Prenatal diagnosis technology development is necessary to be able to predict or diagnose adverse fetal outcomes related to ZIKV. Moreover, these tests should be used in a manner similar to the testing/screening method for neural tube defects and common chromosomal anomalies during prenatal care.

Palivizumab compliance by infants in Puerto Rico during the 2009-2010 respiratory syncytial virus season.

Respiratory syncytial virus (RSV) is the leading viral pathogen responsible for bronchiolitis and pneumonia in infants. We assessed palivizumab prophylaxis compliance for infants in Puerto Rico. We retrospectively studied data from 868 infants (409 females, 459 males) during the 2009-2010 RSV season. The infants had a mean gestational age of 33 weeks (range 23-41) and a mean birth weight of 1,767 g (range 509-4,120). Only 74 % of the infants with indications received prophylaxis. The main reasons for noncompliance were non-approval by the medical insurance (53 %), parents' unavailability (31 %), and infant sickness (11 %). Infants with the government medical insurance were more likely to be denied prophylaxis and to receive fewer doses. Parents need to be educated on the importance of RSV prophylaxis. Physicians should be aware that many infants are not being dosed appropriately and that strategies need to be established to improve compliance.

Mortality and risk factors for invasive fungal infections after the implementation of a fluconazole prophylaxis protocol in very low birth weight infants.

UNLABELLED: Fungal infections are a common cause of late-onset sepsis in very low birth weight infants and can impact mortality and morbidity. The purpose of this study is to determine the impact of systemic fluconazole prophylaxis in the prevalence of fungemia. METHODS: Subjects included 402 very low birth weight premature infants with birth weights between 500 and 1,250 grams admitted to the University Pediatric Hospital NICU over a six year period. The period before and after prophylaxis was retrospectively compared RESULTS: There was a marginal decrease in fungemia in the prophylaxis group (2.6% vs. 6.7%, p = 0.0525). Factors associated to fungemia were chorioamnionitis (p = 0.0240), H2-receptor inhibitors use (p = 0.0109), mechanical ventilati (p = 0.0049), prolonged antibiotic t py (p = 0.0015), and parenteral nutriti (p = 0.0048). Infants with fungemia had longer lengths of stay (p = 0.0143) and lower survival (p = 0.002). CONCLUSION: Prophylaxis with fluconazole decreases mortality and morbidity in an environment with a high incidence of late onset fungal infections. Early identification and prevention of risk factors must be reinforced.

Improvement in knowledge of nursing staff after an educational intervention about control of newborn temperature.

UNLABELLED: Newborn infants are at risk for hypothermia. Providing an adequate thermal environment is fundamental OBJECTIVE: To assess nurses knowledge about temperature control in newborns before and after an educational intervention and its effect in the prevalence of hypothermia. METHODS: Registered nurses working in a NICU received an educational intervention. They answered a pretest and posttest. Prevalence of hypothermia was recorded. RESULTS: Subjects included 55 nurses (89% females, 11% males). The subjects showed improvement in knowledge (81% correct answers before. 88% after, p < 0.01) Prevalence of hypothermia was similar before and after the intervention. CONCLUSION: An educational intervention in small group format was successful in improving nurse knowledge about temperature control in newborns. There was no effect in the prevalence of hypothermia. The intervention was aimed to registered nurses but neonatal hypothermia prevention requires interactions by all caregivers, thus educational interventions should be aimed to all personnel involved in neonatal care.

[Role of the speech pathologist: language in the neonatal intensive care unit]. / El rol del patologo del habla: lenguaje dentro de la Unidad de Cuidado Intensivo Neonatal.

In 2004, the American Speech and Language Association (ASHA) established the primary roles and responsibilities of the speech and language pathologists (SLP) in the Neonatal Intensive Care Unit (NICU) breaking down the skills and knowledge needed to become a member of the neonatal team. It establishes as main parameters the evaluation and intervention in communication, feeding and swallowing: parents and caregivers counseling and education: as well as the collaboration and education of the neonatal team. In the NICU, the SLP works in the preparation to initiate the nutritive sucking process and describes maturation levels for nutritive sucking with bottle and breastfeeding The SLP establishes an intervention plan for the development of safe feeding and swallowing skills as well as for the training of parents and caregivers during the process. Research should be promoted to support these interventions and establish the standards for the field.

MTHFR polymorphisms in Puerto Rican children with isolated congenital heart disease and their mothers.

Congenital heart defects (CHD) are among the most common birth defects. There is evidence suggesting that polymorphisms in folate metabolism could alter susceptibility to CHD. The MTHFR 677TT genotype has been associated with the development of structural congenital heart malformations. The objective of this study was to identify common polymorphisms in the MTHFR gene in children with isolated CHD and their mothers. The DNA analysis for the C677T and A1298C mutations was performed. The study group included 27 mothers, 27 children with CHD, and 220 controls. The prevalence of the TT polymorphism was higher in mothers (22%) than in controls (10%). Compound heterozygosity for both polymorphisms was 3.7 times more common in children with CHD than in the newborn controls. Mothers of children with CHD were more likely to be compound heterozygotes. The higher prevalence of C677T polymorphisms in mothers of children with CHD and of compound heterozygosity for both polymorphisms suggests the possible role of folic acid in the prevention of CHD. Due to the relation of this enzyme to folate metabolism, current folate recommendations for women in childbearing age in Puerto Rico to reduce neural tube defects may need to be extended to the prevention of CHD.

The use of folic acid for the prevention of birth defects in Puerto Rico.

INTRODUCTION: The occurrence of neural tube defects (NTDs) varies by race/ethnicity, and the highest rates are among women of Hispanic ethnicity. Women of reproductive age are advised to use folic acid to prevent NTDs and other birth defects. Since 1994, Puerto Rico has had a campaign to promote the use of folic acid, and since 1998, enriched grain products have been fortified with folic acid. After fortification, the incidence of NTDs in the island decreased. The objective of this study was to assess the use of folic acid by women of reproductive age in Puerto Rico and determine factors associated with its use. METHODS: A self-administered questionnaire was answered by 964 women around the island. RESULTS: Folic acid consumption was reported by 30% of the participants, 21% reported consuming it at least 4 times per week, and only 14% consumed it the day before the survey. Knowledge about the recommendation for women to consume folic acid was reported by 97% of the participants. The use of folic acid was lower among women of lower education and lower social class. Women with higher education were 8.3 times more likely to consume folic acid. CONCLUSION: The folic acid campaign has improved women's knowledge about the recommendation for folic acid supplementation. Nevertheless, its use is lower than is needed to continue reducing the incidence of birth defects in Puerto Rico. Education and social class continue to be barriers to eliminating the disparities in the preconception health of our population.

Pulmonary hypoplasia in Jarcho-Levin syndrome.

Jarcho-Levin syndrome, also known as spondylothoracic dysplasia and characterized by short trunk dwarfism, "crab-like" rib cage, with ribs and vertebral defects; it is not uncommon in Puerto Ricans. Many patients die in early infancy due to respiratory compromise associated to lung restriction and the reported cases emphasize mostly the skeletal malformations associated to the syndrome. We report the autopsy findings in a newborn with isolated Jarcho-Levin syndrome emphasizing pulmonary pathology. He was a pre-term male who died of respiratory failure at three hours old and, autopsy findings confirmed the clinical diagnosis. Internal examination showed hypoplastic lungs with normal lobation. The histological structure appeared normal and relatively mature; the diaphragm showed eventration and unilateral absence of musculature. This case shows the worst spectum of the Jarcho-Levin syndrome: pulmonary hypoplasia not compatible with extrauterine life. Since thoracic restriction is present during the fetal period, the degree of pulmonary hypoplasia probably defines survival beyond the neonatal period.

Long bone fractures in extreme low birth weight infants at birth: obstetrical considerations.

BACKGROUND: Cesarean section is a common delivery route for breech fetuses < 1000 gm to prevent trauma. However, abdominal and vaginal delivery maneuvers are similar. Cesarean section avoids the risk of head entrapment but long bone trauma can still occur. CASES: We identified three neonates with femoral fractures during a one year period. All mothers were in active labor. All were premature newborns less than 32 weeks gestation, in breech presentation and delivered by a low vertical cesarean section. Review of all cesarean sections done due to mal presentation (n = 26) during that time showed 11 classic and 15 lower segment vertical incisions (both vertical and transverse). CONCLUSIONS: The interest to reduce maternal morbidity may prompt physicians to perform a low segment vertical incision for delivery of a preterm breech. This decision may increase the chances of trauma by providing less area for the required obstetric maneuvers.

Erythrocyte volume, folate levels, and the presence of methylenetetrahydrofolate reductase polymorphism.

Homozygosity for a common polymorphism in the 5,10 methylenetetrahydrofolate reductase (MTHFR) gene (C677T) has been associated to an increased risk of neural tube defects as well as derangements in folate, homocysteine, and hematological parameters. This study analyzed the relationship between folate levels, the erythrocyte volume, and the presence of homozygosity for the C677T polymorphism in a group of 126 Puerto Rican healthy women of childbearing age. Blood samples were analyzed for erythrocyte mean corpuscular volume (MCV), mean erythrocyte hemoglobin content (MCH), folate, and RBC folate. Homozygosity for the C677T mutation was determined by PCR. Thirty-two percent (32%) of women used a folic acid supplement during the three months prior to sampling. Mean folate and RBC folate levels were within the normal range. Individuals homozygous for the MTHFR C677T polymorphism had no elevation of MCV (p = 0.70) or MCH (p = 0.68). Women in the lower quartile of folate levels did not show differences in their MCV or MCH. In this sample of Puerto Rican women, homozygosity for the C677T MTHFR polymorphism was not associated to elevations of MCV or MCH even in the presence of lower folate levels.

Presence of the 5,10-methylenetetrahydrofolate reductase C677T mutation in Puerto Rican patients with neural tube defects.

Folic acid supplementation can reduce the incidence of neural tube defects. The first reported genetic risk factor for neural tube defects is a C677T mutation in the 5,10-methylenetetrahydrofolate reductase gene, resulting in decreased activity of the enzyme. We examined the enzyme mutation role of methylenetetrahydrofolate reductase in the etiology of neural tube defects in our population. The study group consisted of 204 Puerto Rican individuals including 37 pregnant females with a prenatal diagnosis of neural tube defects in their fetuses, 31 newborns, 36 fathers, and 100 healthy adults. The prevalence of the C677T mutation was examined. Homozygosity for the alanine to valine substitution (TT) was observed in 9% of the controls and 19% of the mothers with children with neural tube defects. Our results indicate that the presence of the T allele at the methylenetetrahydrofolate reductase 677 position may increase the risk of giving birth to an infant with a neural tube defect.