Showing papers in "American Journal of Perinatology Reports in 2015"
TL;DR: It is concluded that physicians must be aware that supplementation with L. rhamnosus GG can cause sepsis in high-risk patients on rare occasions and that probiotic supplementation safety in neonates is under discussion.
Abstract: Many term and preterm infants are commonly supplemented with probiotics to prevent adverse effects of antibiotic administration and necrotizing enterocolitis and they are believed to be safe. However, the supplementation with Lactobacillus rhamnosus GG has been associated with the development of sepsis with a cause–effect relationship in six newborns and children. In this study, we report two further cases and discuss the emerging issue of probiotic supplementation safety in neonates. We conclude that physicians must be aware that supplementation with L. rhamnosus GG can cause sepsis in high-risk patients on rare occasions.
74 citations
TL;DR: Low-level evidence supports the role of TCE, including in the event of persistent bleeding following initial embolization, for the treatment of acquired uterine AVMs, and the variety of embolic agents and laterality of approach delineate the importance of refining procedural protocols.
Abstract: Objective An acquired uterine arteriovenous malformation (AVM) is a rare cause of vaginal bleeding and, although hysterectomy is the definitive therapy, transcatheter embolization (TCE) provides an alternative treatment option. This systematic review presents the indications, technique, and outcomes for transcatheter treatment of the acquired uterine AVMs. Study Design Literature databases were searched from 2003 to 2013 for eligible clinical studies, including the patient characteristics, procedural indication, results, complications, as well as descriptions on laterality and embolic agents utilized. Results A total of 40 studies were included comprising of 54 patients (average age of 33.4 years). TCE had a primary success rate with symptomatic control of 61% (31 patients) and secondary success rate of 91% after repeated embolization. When combined with medical therapy, symptom resolution was noted in 48 (85%) patients without more invasive surgical procedures. Conclusion Low-level evidence supports the role of TCE, including in the event of persistent bleeding following initial embolization, for the treatment of acquired uterine AVMs. The variety of embolic agents and laterality of approach delineate the importance of refining procedural protocols in the treatment of the acquired uterine AVM. Condensation A review on the management of patients with acquired uterine AVMs.
70 citations
TL;DR: Laser ablation and embolization of chorioangiomas via minimally invasive approach may prevent or reverse fetal hydrops due to high cardiac states, however, further studies are needed to refine the appropriate selection criteria that will justify the risk of this invasive in utero therapy for chorioangsiomas.
Abstract: Objective To review techniques and outcomes of different prenatal treatments for large placental chorioangiomas. Study Design Presentation of a case of laparoscopic-assisted laser coagulation and a systematic review of the literature for articles related to intervention for placental chorioangioma. Results A total of 37 cases of definitive (n = 23) and supportive therapy (n = 14) were evaluated, including one case treated in our center. Approximately 35% of the patients had a spontaneous preterm delivery in definitive treatment group versus 36% in the supportive group. The infant survival rates were 65 and 71% in the two groups, respectively. We further compared the two types of laser ablation (fetoscopic [n = 10] and interstitial [n = 4]). Approximately 30% of the patients in the fetoscopic and 25% in interstitial group, had a spontaneous preterm delivery. Survival rates were 60 and 100% in fetoscopic and interstitial groups, respectively. Conclusion Laser ablation and embolization of chorioangiomas via minimally invasive approach may prevent or reverse fetal hydrops due to high cardiac states. However, further studies are needed to refine the appropriate selection criteria that will justify the risk of this invasive in utero therapy for chorioangiomas.
21 citations
TL;DR: An insight is given into the syndrome and the possible pathological mechanisms, the diagnostic approach, complications, and therapeutic interventions of neonatal thrombocytopenia are summarized and the involvement of other cell lines, apart from platelets in the newborns are identified.
Abstract: Introduction Preeclampsia (preE) is pregnancy-induced hypertension affecting a significant proportion of pregnant women worldwide and can cause detrimental effects in the mother and newborn. Some of the effects in the newborn include neonatal thrombocytopenia. Pertaining specifically to neonatal thrombocytopenia, several questions remain unanswered. Discussion According to the current literature, neonatal thrombocytopenia due to maternal preE is highly prevalent in the general population and the incidence is reported to be around 30% worldwide. This review gives an insight into the syndrome and summarizes the possible pathological mechanisms, the diagnostic approach, complications, and therapeutic interventions of neonatal thrombocytopenia. It also identifies the involvement of other cell lines, apart from platelets in the newborns. Furthermore, we suggest a future prospective study to investigate the pathogenesis of preE and plan a study involving animal models to come up with a possible therapeutic intervention to prevent preE and its various consequences in neonates.
21 citations
TL;DR: Recognition of the congenital perineal groove at birth is important for the health care providers to deliver an appropriate parental counseling and appropriate follow-up.
Abstract: Perineal groove is a rare congenital malformation that is characterized by an exposed wet sulcus with nonkeratinized mucous membrane that extends from the posterior vaginal fourchette to the anterior ridge of the anal orifice. This condition is one of the uncommon anomalies of urogenital/anogenital region that is unknown to many clinicians. Although, this condition may be self-resolved before the age of 2 years, this nonepithelized mucous membrane can pose the risk of local irritation and infection, urinary tract infection, and the possibility of nonself-resolved condition that eventually needs surgical correction. Only a few reported cases (n = 23) were found in current medical literatures. This lesion could be misdiagnosed as contact dermatitis, trauma, or even sexual abuse. Therefore, recognition of the congenital perineal groove at birth is important for the health care providers to deliver an appropriate parental counseling and appropriate follow-up.
18 citations
TL;DR: Lactate levels during the first 4 days of life should be followed as a potential marker for suboptimal hemodynamic status in term asphyxiated newborns treated with hypothermia, for whom the maintenance of homeostasis during Hypothermia treatment is of utmost importance to alleviate brain injury.
Abstract: Objective To evaluate whether a secondary increase of serum lactate levels in asphyxiated newborns during hypothermia treatment may reflect suboptimal dynamics. Methods –Retrospective case series and review of the literature. We present the clinical course of four asphyxiated newborns treated with hypothermia who presented with hypotension requiring inotropic support, and who displayed a secondary increase of serum lactate levels during hypothermia treatment. Serial serum lactate levels are correlated with blood pressure and inotropic support within the first 96 hours of life. Results Lactate levels initially decreased in the four patients. However, each of them started to present lower blood pressure, and lactate levels started to increase again. Inotropic support was started to raise blood pressure. The introduction of an epinephrine drip consistently worsened the increase of lactate levels in these newborns, whereas dopamine and dobutamine enabled the clearance of lactate in addition to raising the blood pressure. Rewarming was associated with hemodynamics perturbations (a decrease of blood pressure and/or an increase of lactate levels) in the three newborns who survived. Conclusions Lactate levels during the first 4 days of life should be followed as a potential marker for suboptimal hemodynamic status in term asphyxiated newborns treated with hypothermia, for whom the maintenance of homeostasis during hypothermia treatment is of utmost importance to alleviate brain injury.
16 citations
TL;DR: The identification of the euploid embryo based on the NGS application was the first successful clinical application of NGS-based PGD in the case of the Robertsonian translocation carrier couple.
Abstract: Preimplantation genetic diagnosis (PGD) is well established method for treatment of genetic problems associated with infertility. Moreover, PGD with next-generation sequencing (NGS) provide new possibilities for diagnosis and new parameters for evaluation in, for example, aneuploidy screening. The aim of the study was to report the successful pregnancy outcome following PGD with NGS as the method for 24 chromosome aneuploidy screening in the case of Robertsonian translocation. Day 3 embryos screening for chromosomal aneuploidy was performed in two consecutive in vitro fertilization (IVF) cycles, first with fluorescent in situ hybridization (FISH), and then with NGS-based protocol. In each IVF attempt, three embryos were biopsied. Short duration of procedures enabled fresh embryo transfer without the need for vitrification. First IVF cycle with the embryo selected using PGD analysis with the FISH method ended with pregnancy loss in week 8. The second attempt with NGS-based aneuploidy screening led to exclusion of the following two embryos: one embryo with 22 monosomy and one with multiple aneuploidies. The transfer of the only euploid blastocyst resulted in the successful pregnancy outcome. The identification of the euploid embryo based on the NGS application was the first successful clinical application of NGS-based PGD in the case of the Robertsonian translocation carrier couple.
15 citations
TL;DR: A prenatal ultrasound finding of a difference in AC to HC of 50 mm or more noted on antenatal ultrasound while uncommon is associated with shoulder dystocia.
Abstract: Objective This study aims to determine if shoulder dystocia is associated with a difference in the fetal abdominal (AC) to head circumference (HC) of 50 mm or more noted on antenatal ultrasound. Study Design A multicenter matched case–control study was performed comparing women who had shoulder dystocia to controls who did not. Women with vaginal births of live born nonanomalous singletons ≥ 36 weeks of gestation with an antenatal ultrasound within 4 weeks of delivery were included. Controls were matched for gestational age, route of delivery, and diabetes status. Results We identified 181 matched pairs. Only 5% of the fetuses had an AC to HC of ≥ 50 mm. The proportion of AC to HC difference of ≥ 50 mm was significantly higher in shoulder dystocia cases (8%) than controls (1%, p = 0.002). With multivariate regression, the three significant factors associated with shoulder dystocia were AC to HC ≥ 50 mm (odds ratio [OR], 7.3; confidence interval [CI], 1.6–33.3; p = 0.010), femur length (OR, 1.1; CI, 1.0–1.2; p = 0.002), and induced labor (OR, 1.8; CI, 1.1–3.1; p = 0.027). Conclusion A prenatal ultrasound finding of a difference in AC to HC of ≥ 50 mm while uncommon is associated with shoulder dystocia.
14 citations
TL;DR: PPROM and vaginal bleeding in the current pregnancy are relevant predictive factors in all women, as are maternal age, cervical length, and fFN in women without PPRom and nulliparity, history of preterm birth in women with PPROM.
Abstract: Objective The aim of this study was to assess which characteristics and results of vaginal examination are predictive for delivery within 7 days, in women with threatened preterm labor after initial treatment. Study Design A secondary analysis of a randomized controlled trial on maintenance nifedipine includes women who remained undelivered after threatened preterm labor for 48 hours. We developed one model for women with premature prelabor rupture of membranes (PPROM) and one without PPROM. The predictors were identified by backward selection. We assessed calibration and discrimination and used bootstrapping techniques to correct for potential overfitting. Results For women with PPROM (model 1), nulliparity, history of preterm birth, and vaginal bleeding were included in the multivariable analysis. For women without PPROM (model 2), maternal age, vaginal bleeding, cervical length, and fetal fibronectin (fFN) status were in the multivariable analysis. Discriminative capability was moderate to good (c-statistic 0.68; 95% confidence interval [CI] 0.60-0.77 for model 1 and 0.89; 95% CI, 0.84-0.93 for model 2). Conclusion PPROM and vaginal bleeding in the current pregnancy are relevant predictive factors in all women, as are maternal age, cervical length, and fFN in women without PPROM and nulliparity, history of preterm birth in women with PPROM.
13 citations
TL;DR: Iatrogenic preterm delivery may be indicated in early-severe recurrent ICP to prevent intrauterine fetal demise (IUFD), but more research is needed.
Abstract: Background Intrahepatic cholestasis of pregnancy (ICP) is a disorder of defective bile acid transport that results in systemic accumulation of bile acids and typically presents in the third trimester of pregnancy with intense pruritus. A positive linear correlation exists between total bile acid level and poor pregnancy outcome, and labor is typically induced at 37 weeks gestation to prevent intrauterine fetal demise (IUFD). Case Study We present the most severe reported case of recurrent ICP presenting early in the first trimester. The patient was delivered by repeat cesarean section at 31 6/7 weeks gestation resulting in a viable female infant. Conclusion Iatrogenic preterm delivery may be indicated in early-severe recurrent ICP to prevent IUFD, but more research is needed.
12 citations
TL;DR: The course of POTS in pregnancy is variable, and not directly linked to increase perinatal morbidity or mortality, and women can safely undergo regional anesthesia, and vaginal delivery with close monitoring of hemodynamic changes.
Abstract: Purpose Postural orthostatic tachycardia syndrome (POTS) is a form of orthostatic intolerance characterized by an increased heart rate upon transition from supine to standing, and head-up tilt without orthostatic hypotension. Its etiology is multifactorial, and no clear cause has been identified. Common symptoms include light-headedness, blurred vision, weakness, cognitive difficulties, and fatigue and are often accompanied by palpitations, shortness of breath, syncope, or gastrointestinal symptoms. Management includes volume expansion, physical counter maneuvers, and pharmacological agents such as fludrocortisone, midodrine, propranolol, and pyridostigmine. The course of POTS in pregnancy is variable and POTS has not been directly implicated in any adverse outcomes for the mother or fetus. Methods Two cases of POTS in pregnancy are presented, along with a review of the literature for reports of POTS in pregnancy. Results Along with our 2 cases, 10 other case reports were identified and included. Conclusion The course of POTS in pregnancy is variable, and not directly linked to increase perinatal morbidity or mortality. Women can safely undergo regional anesthesia, and vaginal delivery with close monitoring of hemodynamic changes.
TL;DR: The pilot study provides feasibility data for a multicenter randomized clinical trial to determine if third trimester USE, compared with RPNC, improves the detection of SGA and composite neonatal morbidity.
Abstract: Objective The purpose of this multicenter pilot study was to determine the feasibility of randomizing uncomplicated pregnancies (UPs) to have third trimester ultrasonographic exams (USE) versus routine prenatal care (RPNC) to improve the detection of small for gestational age (SGA; birth weight Material and Methods At three referral centers, 50 UPs were randomized after gestational diabetes was ruled out. Women needed to screen, consenting, and loss to follow-up was ascertained, as was the detection rate of SGA in the two groups. Results During the study period at the three centers, there were 7,680 births, of which 64% were uncomplicated. Of the 234 women approached for randomization, 36% declined. We recruited 149 women and had follow-up delivery data on 97%. The antenatal detection rate of SGA in the intervention group was 67% (95% confidence intervals 31–91%) and 9% (0.5–43%) in control. Conclusion The pilot study provides feasibility data for a multicenter randomized clinical trial to determine if third trimester USE, compared with RPNC, improves the detection of SGA and composite neonatal morbidity.
TL;DR: There is a difference between fasting and nonfasting total serum bile acids after a 100-g glucose load in healthy pregnant women.
Abstract: Objective Intrahepatic cholestasis of pregnancy (ICP) is a rare liver disorder, usually manifesting in the third trimester and associated with increased perinatal morbidity and mortality. The hallmark laboratory abnormality in ICP is elevated fasting serum bile acids; however, there are limited data on whether a nonfasting state affects a pregnant woman's total bile acids. This study assesses fasting and nonfasting bile acid levels in 10 healthy pregnant women after a standardized glucose load to provide insight into the effects of a glucose load on bile acid profiles. Study Design Pilot prospective cohort analysis of serum bile acids in pregnant women. A total of 10 healthy pregnant women from 28 to 32 weeks' gestation were recruited for the study before undergoing a glucose tolerance test. Total serum bile acids were collected for each subject in the overnight fasting state, and 1 and 3 hours after the 100-g glucose load. Results There was a statistically significant difference between fasting versus 3-hour values. There was no statistically significant difference between fasting versus 1-hour and 1-hour versus 3-hour values. Conclusion There is a difference between fasting and nonfasting total serum bile acids after a 100-g glucose load in healthy pregnant women.
TL;DR: The authors present the first description of a successful medical case using TESA-ICSI annexin V sperm sorting, in a couple where the male presented high sperm DNA fragmentation.
Abstract: Introduction Sperm DNA integrity is a crucial paternal factor affecting fertilization and pregnancy rates, as well as embryo development. Case The present case report describes the successful pregnancy after testicular sperm aspiration (TESA) combined with intracytoplasmic sperm injection (ICSI) (TESA-ICSI) in a couple where the male presented high sperm DNA fragmentation. In order to sort damaged sperm presenting DNA fragmentation, magnetic activated cell sorting (MACS) with annexin V microbeads (MACS Miltenyi Biotec, Teterow, Germany) was used. Conclusion The authors present the first description of a successful medical case using TESA-ICSI annexin V sperm sorting. Additionally, a follow-up of the child at the age of 4 years old was done.
TL;DR: A fatal case ofSP-B deficiency caused by a homozygous C248X mutation in exon 7 of the SP-B gene is reported on, and thirty-four mutations have been published in the literature.
Abstract: Objective Surfactant protein B (SP-B) deficiency is a rare autosomal recessive disorder that is usually rapidly fatal. The c.397delCinsGAA mutation (121ins2) in exon 4 is found in more than two-thirds of patients. Design We report on a fatal case of SP-B deficiency caused by a homozygous C248X mutation in exon 7 of the SP-B gene. In addition, we provide an update of the current literature. The EMBASE, MEDLINE, and CINAHL databases were systematically searched to identify all papers published in the English and German literature on SP-B deficiency between 1989 and 2013. Results SP-B deficiency is characterized by progressive hypoxemic respiratory failure generally in full-term infants. They present with symptoms of respiratory distress and hypoxemia; chest X-ray resembles hyaline membrane disease. Prenatal diagnosis is possible from amniotic fluid or chorionic villi sampling. Conclusion Thirty-four mutations have been published in the literature. Treatment options are scarce. Gene therapy is hoped to be an option in the future.
TL;DR: A case of congenital and acquired CMV infection in twins is presented with a focus of dissimilarity in presentation, clinical course, and outcome.
Abstract: Background Cytomegalovirus (CMV) is one of the most common causes of serious viral intrauterine infections. It is universally distributed among the human population with an average incidence of 0.15 to 2%. Indeed, at least half of the women in the reproductive age have evidence of prior CMV infection. Epidemiology and Pathogenicity However, it is not a usual practice to screen asymptomatic pregnant woman or neonates for CMV. Even if a mother developed a primary CMV infection during pregnancy, up to 90% of the newborns with congenital CMV will be asymptomatic at the time of birth. Only 5 to 7% of the infected babies will be acutely symptomatic, and the typical clinical presentation includes intrauterine growth restriction, microcephaly, various cutaneous manifestations (including petechiae and purpura), hematological abnormalities (particularly resistant thrombocytopenia), hepatosplenomegaly, chorioretinitis, hepatitis, etc. In contrast, acquired CMV infection is extremely unlikely to cause any serious sequelae for the infant. Cases We present a case of congenital and acquired CMV infection in twins with a focus of dissimilarity in presentation, clinical course, and outcome.
TL;DR: Early diagnosis and management of myocardial infarction due to coronary artery dissection in the peripartum period is crucial and this condition should be suspected in young reproductive age women, even in the setting of minimal risk factors.
Abstract: Background Though rare, myocardial infarction secondary to coronary artery dissection is a life-threatening event. In reproductive age women, it commonly occurs during pregnancy or the postpartum period. Case We present a case of pregnancy-related acute myocardial infarction due to spontaneous coronary artery dissection in a 37-year-old woman who presented to the emergency room with shortness of breath and sudden onset of retrosternal chest pain 8 days after delivery of premature twins. Coronary artery catheterization showed 75 to 90% stenosis in the left main coronary artery (LMCA), extending into the proximal and mid left anterior descending (LAD) branch. The LMCA appearance in the heart catheterization was consistent with vasospasm, but it was not responsive to medical management. Subsequently, she underwent a second coronary artery catheterization and was found to have dissection requiring emergent coronary artery bypass graft × 3 in the LMCA, circumflex, and LAD that was followed by an uneventful recovery. Conclusion Early diagnosis and management of myocardial infarction due to coronary artery dissection in the peripartum period is crucial. This condition should be suspected in young reproductive age women, even in the setting of minimal risk factors. Angiography is required for diagnosis. Management should be individualized as it may include both invasive and noninvasive measures.
TL;DR: A case of isolated distal anomalous origin of the right PA from the aorta that was diagnosed by fetal echocardiography at 25 weeks' of gestation is reported.
Abstract: The anomalous origin of a branch pulmonary artery from the aorta (AOPA) is characterized by the anomalous origin of one of the branch pulmonary arteries (PA) from the ascending aorta and a normal origin of the other PA from main PA. AOPA is an extremely rare cardiac malformation. Few studies have reported fetal anomalous origin of PA from aorta with other malformation. We report a case of isolated distal anomalous origin of the right PA from the aorta that was diagnosed by fetal echocardiography at 25 weeks' of gestation. Tracing the course of PA branches is important to make diagnosis.
TL;DR: A case of a neonate with H. parainfluenzae bacteremia following water birth is reported, and the child was successfully treated with both antibiotic and supportive care.
Abstract: Water birth has grown in popularity over the recent years. Although beneficial for mothers, there are concerns for the infants. There are previous reports of infection following water birth. The information regarding infection with Haemophilus parainfluenzae is limited. We report a case of a neonate with H. parainfluenzae bacteremia following water birth. The child was successfully treated with both antibiotic and supportive care. Previous reports of neonatal H. parainfluenzae infection are reviewed.
TL;DR: A full-term infant with interstitial changes resembling PIG and BPD who expired despite no convincing evidence of an anatomical maturational arrest or congenital heart disease is reported on.
Abstract: Pulmonary interstitial glycogenosis (PIG) is a rare interstitial lung disease in the newborns. We report on the clinical presentation and pathological findings of a full-term male infant with pulmonary hypertension requiring extracorporeal membrane oxygenation (ECMO). An open lung biopsy demonstrated interstitial changes resembling pulmonary interstitial glycogenosis as well as bronchopulmonary dysplasia (BPD), without convincing evidence of maturational arrest, infection, alveolar proteinosis, or alveolar capillary dysplasia. The boy was treated with glucocorticoids and, after a few days, was weaned from ECMO. A few hours later, the patient died due to acute severe pulmonary hypertension with acute right ventricular failure. The etiology and underlying pathogenic mechanisms of PIG are unknown. The clinical outcomes are quite varied. Deaths have been reported when PIG exists with abnormal lung development and pulmonary vascular growth and congenital heart disease. No mortality has been reported in PIG together with BPD in full-term infants. In this article, we reported on a full-term infant with interstitial changes resembling PIG and BPD who expired despite no convincing evidence of an anatomical maturational arrest or congenital heart disease.
TL;DR: No firm conclusions can be drawn regarding the optimal postexposure prophylaxis in LBW infants, and it is proposed that active and passive immunization at birth should be completed by three further active doses (0–1–2–12-month schedule) until further prospective studies are available.
Abstract: Objective Recommendations for immunoprophylaxis in low-birth-weight (LBW) infants born to hepatitis B surface antigen (HBsAg)-positive mothers vary. We successfully immunized an HBsAg-exposed infant (birth weight: 400 g) and performed a literature review on the outcome of postexposure immunoprophylaxis in HBsAg-exposed preterm and LBW infants. Methods By use of PubMed we identified articles relevant to the topic. Studies were included if the intended vaccine schedule was completed and follow-up data were reported. Results Antibody response was reported in 31 LBW infants (birth weight Conclusion Due to heterogeneity in immunization schedules, lack of information on transmission rates, and the small number of included subjects, no firm conclusions can be drawn regarding the optimal postexposure prophylaxis in LBW infants. We propose that active and passive immunization at birth should be completed by three further active doses (0–1–2–12 month schedule) until further prospective studies are available.
TL;DR: This case describes an infant with gastroschisis who developed refractory shock after an uncomplicated surgery for bowel atresia and was found to have adrenal insufficiency secondary to septo-optic dysplasia with panhypopituitarism.
Abstract: Introduction Gastroschisis is considered to be an isolated abdominal wall defect that is infrequently associated with other anomalies. Case This case describes an infant with gastroschisis who developed refractory shock after an uncomplicated surgery for bowel atresia. He was found to have adrenal insufficiency secondary to septo-optic dysplasia with panhypopituitarism. Conclusion Gastroschisis and septo-optic dysplasia arise from vascular disruptions, therefore presence in the same infant can be more than just a coincidence. While this is not a common occurrence, our case illustrates the need for a high index of suspicion with an unusual clinical course.
TL;DR: A rare finding in fetal cardiac ultrasound in a 33-year-old woman who presented at 20 weeks' of gestation is reported, a rare form of congenital hypoplasia of the right ventricular myocardium.
Abstract: Uhl anomaly is a rare form of congenital hypoplasia of the right ventricular myocardium. Here, we report, a rare finding in fetal cardiac ultrasound in a 33-year-old woman who presented at 20 weeks' of gestation. A diagnosis of Uhl anomaly was made. An autopsy was performed at 23weeks gestation after obtaining permission for medicolegal termination of pregnancy. Histopathological examination confirmed the diagnosis. Diagnosing Uhl anomaly in fetal life is essential since mortality and survival mainly depend on the severity of right ventricle dysfunction related to, the either partial or complete absence of the myocardium. Hence, surviving cases need to be followed up carefully and counselled accordingly.
TL;DR: It is suggested that distinguishing underlying SLE from preeclampsia in the third trimester is particularly difficult, and helpful factors for diagnosis of suspected SLE in these cases were persistence of symptoms and new atypical symptoms revealed after delivery.
Abstract: Introduction New-onset systemic lupus erythematosus (SLE) during pregnancy is rare and difficult to diagnose, especially in cases that manifest as preeclampsia. We report a patient with new-onset SLE that manifested as preeclampsia during pregnancy and provide a review of the literature to identify factors for a rapid diagnosis. Case A 32-year-old primigravid Japanese woman was diagnosed with severe preeclampsia and underwent emergent cesarean section at 29 weeks of gestation. Her hypertension and renal disorder gradually improved after the operation, but her thrombocytopenia and anemia worsened. SLE was diagnosed on postoperative day 5 by a comprehensive autoimmune workup. She was discharged on postoperative day 34 with remission. Conclusion Our case and previous reports suggest that distinguishing underlying SLE from preeclampsia in the third trimester is particularly difficult. Helpful factors for diagnosis of suspected SLE in these cases were persistence of symptoms and new atypical symptoms for preeclampsia revealed after delivery (e.g., fever, renal disorder, and thrombocytopenia).
TL;DR: Four newborn infants with prominent Eustachian valve who were symptomatic after birth and required admission to the neonatal intensive care unit are presented.
Abstract: The Eustachian valve (EV) is an embryological remnant of the inferior vena cava that during fetal life helps divert oxygenated blood from the IVC toward the foramen ovale to escape the pulmonary circulation. This remnant usually regresses after birth and is considered a benign finding in the majority of cases. However, EV can lead to complications in the neonatal period or later in life. In this short case series, we present four newborn infants with prominent EV who were symptomatic after birth and required admission to the neonatal intensive care unit.
TL;DR: It is important to consider a diagnosis of milk protein allergy responsive to hypoallergenic formula in patients with SS who are refractory to conventional antireflux treatment.
Abstract: Objective To study the presentation and treatment in two infants who presented with refractory Sandifer syndrome (SS). Study Design We retrospectively reviewed the cases of two infants who presented to our outpatient clinic with SS who were refractory to conventional treatment. Results We report two patients with refractory SS who responded to treatment with hypoallergenic formula. Conclusion It is important to consider a diagnosis of milk protein allergy responsive to hypoallergenic formula in patients with SS who are refractory to conventional antireflux treatment.
TL;DR: Clinicians should encourage the surrogate to share her medical history with the embryo donor for appropriate counseling on pregnancy risks and carry a significant morbidity and mortality for the mother and fetus.
Abstract: Background The occurrence of hemolysis, elevated liver enzymes, low platelets (HELLP) syndrome before 20 weeks of gestation is rare. HELLP is a possible but rare syndrome in gestational surrogate pregnancies for surrogates with risk factors for development of preeclampsia. Case A 32-year-old patient with chronic hypertension and positive antinuclear antibody presented for prenatal care at 13 weeks and 1 day. She was a surrogate for the embryo of a 43-year-old couple. By 15 weeks she developed uncontrolled hypertension requiring hospitalization. She was expectantly managed until her condition deteriorated. At 16 weeks and 1 day she developed hemolysis, elevated liver enzymes, thrombocytopenia, and fetal demise. Conclusions HELLP syndrome is rare and carries a significant morbidity and mortality for the mother and fetus. Clinicians should encourage the surrogate to share her medical history with the embryo donor for appropriate counseling on pregnancy risks.
TL;DR: It is suggested that monitoring infants born to mothers with AFLP, for vitamin K deficiency bleeding will help reduce morbidity and mortality in these infants.
Abstract: Introduction Acute fatty liver of pregnancy (AFLP) is a rare but potentially fatal condition occurring in the third trimester or early postpartum period. It is characterized by microvesicular fatty infiltration of the liver. Clinically, the three most prominent derangements in women with AFLP are hepatic dysfunction, renal insufficiency, and impaired coagulation. AFLP is associated with an increased incidence of morbidity and mortality in neonates, though the exact cause for this remains unclear. Deficiency of vitamin K in patients with liver disease has been widely reported. Case Description We present a unique case of severe intracranial bleeding because of the early vitamin K deficiency in a neonate whose mother had AFLP along with accompanying renal insufficiency. Conclusion We suggest that monitoring infants born to mothers with AFLP, for vitamin K deficiency bleeding will help reduce morbidity and mortality in these infants.
TL;DR: Modern-staged repair of EEC guarantees socially acceptable urinary continence in up to 80% of cases; sexual function can be an issue in the long term, but overall quality of life can be good.
Abstract: Aim The aim of this report is to present a brief review of the current literature on the management of EEC. Case Report A term male neonate presented at birth with classic bladder exstrophy, a variant of the exstrophy-epispadias complex (EEC). The defect was covered with sterile silicon gauzes and waterproof dressing; at 72 hours of life, primary closure without osteotomy of bladder, pelvis, and abdominal wall was successfully performed. Discussion EEC incidence is approximately 2.15 per 1,00,000 live births; several urological, musculocutaneous, spinal, orthopedic, gastrointestinal, and gynecological anomalies may be associated to EEC. Initial medical management includes use of occlusive dressings to prevent air contact and dehydration of the open bladder template. Umbilical catheters should not be positioned. Surgical repair stages include initial closure of the bladder andabdominal wall with or without osteotomy, followed by epispadias repairat6to12months,andbladderneckrepairaround5yearsof life.Those who fail to attain continence eventually undergo bladder augmentation and placement of a catheterizable conduit. Conclusion Modern-staged repair of EEC guarantees socially acceptable urinary continence in up to 80% of cases; sexual function can be an issue in the long term, but overall quality of life can be good.
TL;DR: A 1-day-old infant presenting with an air-filled neck mass that enlarged with Valsalva and was not associated with respiratory distress and was found to have a third branchial cleft cyst with an internal opening into the pyriform sinus is reported.
Abstract: Branchial cleft cysts are common causes of congenital neck masses in the pediatric population. However, neonatal presentation of branchial cleft cysts is uncommon, but recognizable secondary to acute respiratory distress from airway compression or complications secondary to infection. We report a 1-day-old infant presenting with an air-filled neck mass that enlarged with Valsalva and was not associated with respiratory distress. The infant was found to have a third branchial cleft cyst with an internal opening into the pyriform sinus. The cyst was conservatively managed with endoscopic surgical decompression and cauterization of the tract and opening. We review the embryology of branchial cleft cysts and current management.