Causas del hidrops fetal: experiencia en un hospital obstétrico del Occidente de México / Etiology of fetal hydrops: experience in an obstetric hospital in western Mexico

Resumen OBJETIVO: Identificar las causas de hidrops fetal no inmunitario en un hospital obstétrico de referencia del Occidente de México. MATERIALES Y MÉTODOS: Estudio de serie de casos, con un muestreo no probabilístico por conveniencia, llevado a cabo de octubre de 2014 a septiembre de 2015 al que se incluyeron pacientes (entre las 15 y 38 semanas de embarazo), mayores de edad (en casos de menores de edad se solicitó consentimiento informado a los padres o tutores), con diagnóstico de hidrops fetal por ultrasonido obstétrico. Para el análisis estadístico se generó una base de datos en Excel y se aplicó estadística descriptiva. RESULTADOS: Se reunieron 33 embarazadas en quienes el hidrops fetal no inmunitario fue el más frecuente (n = 31) y la causa idiopática más común (n = 10) seguida por errores innatos del metabolismo, alteraciones cromosómicas y cardiacas (n = 6 de cada una). Posteriormente, las causas hematológicas (n = 4), linfáticas y sindrómicas (n = 3 de cada una), y las infecciosas y tumorales (n = 1 de cada una). En este estudio los errores innatos del metabolismo (específicamente síndrome Sly) tuvieron una frecuencia superior a la referida en la bibliografía. CONCLUSIONES: Los errores innatos del metabolismo, las anomalías cromosómicas y cardiacas fueron la segunda causa más frecuente de hidrops fetal no inmunitario. Se sugiere tener en cuenta las causas metabólicas en el enfoque diagnóstico del hidrops fetal, sobre todo para el establecimiento del tratamiento temprano.

Abstract OBJECTIVE: To identify the causes of nonimmune fetal hydrops fetalis in an obstetric referral hospital in Western Mexico. MATERIALS AND METHODS: Case series study, with non-probabilistic sampling by convenience, carried out from October 2014 to September 2015 which included patients (between 15 and 38 weeks of pregnancy), of legal age (in cases of minors, informed consent was requested from parents or guardians), with a diagnosis of fetal hydrops fetalis by obstetric ultrasound. For statistical analysis, an Excel database was generated and descriptive statistics were applied. RESULTS: Thirty-three pregnant women were included, in whom non-immune fetal hydrops fetalis was the most frequent (94%) and idiopathic was the most common cause (n = 10), followed by inborn errors of metabolism, chromosomal and cardiac alterations (n = 6 each). This was followed by hematologic (n = 4), lymphatic and syndromic causes (n = 3 each), and infectious and tumor causes (n = 1 each). In this study, inborn errors of metabolism (specifically Sly syndrome) had a higher frequency than that reported in the literature. CONCLUSIONS: Inborn errors of metabolism, chromosomal and cardiac abnormalities were the second most frequent cause of nonimmune fetal hydrops. It is suggested that metabolic causes be taken into account in the diagnostic approach to fetal hydrops, especially for the establishment of early treatment.

Meta-analysis of Non-immune Related Adverse Event s Caused by ICIs Alone or Combined with Routine Che- motherapy in the Treatment of Non-small Cell Lung Cancer / 中国药房

OBJECTIVE：To systematically evaluate the occurren ce of non-immune related adverse events （AEs）caused by immune checkpoint inhibitors （ICIs）alone or combined with routine chemotherapy in the treatment of non-small cell lung cancer （NSCLC），and to provide evidence-based reference for clinical medication. METHODS ：Retrieved from PubMed ，Cochrane Library，Embase，CNKI，CBM，VIP and Wanfang database during the inception to Oct. 2020，randomized controlled trials （RCT） about ICIs alone or combined with routine chemotherapy （trial group ）versus routine chemotherapy or placebo combined with routine chemotherapy （control group ） were collected. After literature screening and data extraction ，the quality of included literatures were evaluated with bias risk evaluation tool recommended by Cochrane systematic evaluator manual 5.1.0. Meta-analysis was performed by using Rev Man 5.3 software and Stata 15.0 software. Sensitivity analysis was conducted with Stata 15.0 software. Inverted funnel plot and Egger ’s test were used to analyze publication bias. RESULTS ：A total of 20 RCTs were included ， involving 12 283 patients. Results of Meta-analysis showed that the incidence of all grades and s evere AEs ，anemia，neutropenia， vomiting and alopecia as well as the incidence of thrombocytopenia，nausea and peripheral neuropathy in all grades of trial group were all significantly lower than control com group（P＜0.05）. There was no statistical significance in the incidence of termination of treatment ， death， severe thrombocytopenia， severe nausea and severe peripheral neuropathy or all grades and severe diarrhea between 2 groups（P＞0.05）. Subgroup analysis showed that the incidence of all grade and total severe AEs ，the incidence of anemia ，neutropenia，thrombocytopenia，clinically relevant symptoms （except for severe diarrhea），termination of treatment and death of patients receiving ICIs alone in trial group were significantly lower than control group（P＜0.05）. The incidence of ermination of treatment and death ，the incidence of nausea ，vomiting，diarrhea and alopecia in all grade ，severe diarrhea of patients receiving ICIs and chemotherapy in trial group were all significantly higher than control group （P＜0.05）. Sensitivity analysis supported the above results. Analyze publication bias results showed that the possibility of publication bias in this study was small. CONCLUSIONS ：For NSCLC patients ，the safety of ICIs is better than that of routine chemotherapy or placebo combined with routine chemotherapy in the treatment-related AEs ，hematologic toxicity and clinically relevant symptoms ；however，the risks of treatment discontinuation ，AEs-induced deaths ，and all-grade nausea ，vomiting， diarrhea，alopecia and severe diarrhea will be increased in the ICIs combined with routine chemotherapy.

Non-immune hydrops fetalis: a case series

Hydrops fetalis is a clinical condition characterized by pathological fluid accumulation in soft tissues and serous cavities of the fetus like peritoneal cavity, pleural cavity, pericardial space, and body wall edema. Hydrops fetalis is broadly classified into Immune Hydrops Fetalis (IHF) and Non-Immune Hydrops Fetalis (NIHF). Incidence of immune hydrops fetalis due to Erythroblastosis fetalis secondary to Rh Iso-immunisation has drastically reduced due to widespread use of anti-D immunoglobulin. In the last few decades, the majority of cases are identified as non-immune hydrops. It is important to determine the cause of the hydrops fetalis in order to administer optimal management of the neonate at birth. Despite recent advances the mortality of non-immune hydrops is still high. Authors report here six cases of non-immune hydrops fetalis encountered at our tertiary care hospital over last three years.

A study of clinicopathological features and outcomes of crescentic glomerulonephritis

Background: Crescentic Glomerulonephritis (CrGN) is characterized by rapidly progressive renal failure. Most of the literatures have defined >50% crescents in biopsy as CrGN. Only very few studies have included the presence of <50% crescents as CrGN.Methods: To assess the clinico-pathological features and outcome of CrGN with >10% crescents on renal biopsy and comparing them splitting our diagnosis into Immune Complex Mediated CrGN (ICCGN) and non-immune complex mediated CrGN (NICCGN) groups.Results: ICCGN was the commonest group. When compared to ICCGN group, NICCGN patients were older, anuric, had more glomerular necrosis and severe IFTA in biopsy at presentation, more became dialysis dependent at index visit discharge. When patients with >50% crescents in both the groups were compared similar results were seen except that infective complications and proliferative lesions were more in ICCGN. When patients with <50% crescents in both the groups were compared similar findings were seen except that no difference was seen in clinical features and dialysis dependency between them.Conclusions: Oliguria at presentation, Hb <9 g/dl, index visit eGFR<15 ml/min, crescents >60%, moderate to severe IFTA are the independent risk factors for dialysis dependency at index visit discharge.

Hídrops fetal no inmunológico / Non-Immune Fetal Hydrops

El hídrops fetal se define como el acúmulo anormal de líquido en los tejidos blandos y cavidades serosas del feto (pleural, pericárdico y peritoneal). Se divide en dos grupos: hídrops fetal inmune e hídrops fetal no inmune. Se presenta el caso de gestante (9 semanas), calificada como riesgo genético incrementado por sus antecedentes obstétricos. Se procede según establece el Programa de Genética para la Detección Prenatal de Defectos Congénitos. Se resalta la importancia de la información de los resultados de las ecografías prenatales en el diagnóstico precoz de malformaciones congénitas y/o defectos estructurales del feto. Tras el seguimiento del caso y la realización de pruebas confirmativas se llegó al diagnóstico presuntivo de hídrops fetal no inmunológico, lo cual fue confirmado con posterioridad por anatomía patológica. Teniendo en cuenta que el pronóstico de esta entidad es generalmente desfavorable y con una tasa de mortalidad intrauterina muy alta, la pareja decidió la terminación del embarazo(AU)

serous cavities (pleural, pericardial, and peritoneal). It is divided into two groups: fetal immune hydrops and non-immune fetal hydrops. We report the case of a 9 weeks pregnant woman, classified to be at increased genetic risk by her obstetric history. We proceed as established by the Genetics Program for the Prenatal Detection of Birth Defects. The importance of the prenatal ultrasound information is relevant in the early diagnosis of congenital malformations and / or structural defects of the fetus. After the follow-up of the case and the performance of confirmatory tests, a presumptive nonimmunological fetal hydrops is diagnosed, which is subsequently confirmed by pathological anatomy. Taking into account that the prognosis of this entity is generally unfavorable and with very high intrauterine mortality rate, this couple decided to terminate the pregnancy(AU)

Ascitis fetal: a propósito de un caso y revisión de la literatura / Fetal ascites: Apropos of a case and literature review

Fetal ascites is defined as the presence of intraperitoneal fluid that may be part of a generalized or isolated hydrops. The mortality of non-immune ascites, both fetal and neonatal, is approximately 60%. We present a case of fetal ascites not associated with hydrops and we review the pathogenesis, clinical features, diagnostic approach and treatment of this fetal and neonatal condition.

La ascitis fetal se define como la presencia de líquido intraperitoneal que puede ser parte de un hidrops generalizado o aislado. La mortalidad de la ascitis no inmune, tanto fetal como neonatal, es aproximadamente 60%. Se presenta un caso de ascitis fetal no asociada a hidrops y se revisa la patogenia, clínica y el enfoque diagnóstico y tratamiento de esta condición fetal y neonatal.

Genetic analysis of a family with recurrent hydrops fetalis and dilated cardiomyopathy / 中华医学遗传学杂志

Objective@#To carry out genetic testing for a family with two pregnancies affected with hydrops fetalis and dilated cardiomyopathy (DCM) of the fetus.@*Methods@#DNA was extracted from fetal tissue as well as peripheral blood samples from the couple. Single nucleotide polymorphism array (SNP array) and next-generation sequencing (NGS) were carried out to screen potential mutation. Suspected mutation was validated with PCR and Sanger sequencing.@*Results@#The manifestation of fetal echocardiography was consistent with DCM. No obvious abnormality was found by SNP array analysis. A hemizygous c. 481G>A (p.G161R) mutation of the TAZ gene was detected in the male fetus by NGS and confirmed by Sanger sequencing. The mutation was inherited from his mother.@*Conclusion@#Barth syndrome due to the c. 481G>A mutation of the TAZ gene probably underlies the recurrent hydrops fetalis and fetal DCM in this family.

Síndrome de Ballantyne: segundo episodio en una embarazada. Reporte de caso y revisión de la bibliografía / Ballantyne syndrome: Second episode in a pregnant woman. Case report and literature review

Resumen ANTECEDENTES: El síndrome de Ballantyne es un cuadro poco frecuente asociado con hidrops fetal, en el que la madre refleja los síntomas fetales. Es decisivo diferenciarlo de la preeclampsia porque comparten signos de hipertensión y proteinuria. Su etiopatogenia se desconoce pero se han propuesto teorías asociadas con el desequilibrio entre factores angiogénicos y antiangiogénicos. CASO CLÍNICO: Paciente de 29 años, controlada en la consulta de Medicina Materno-Fetal debido al antecedente de síndrome de Ballantyne en el embarazo previo. En la ecografía de control a las 26 semanas se detectaron placentomegalia, ascitis fetal e incremento del líquido amniótico. Las ecografías posteriores demostraron polihidramnios e hidrops a las 28 semanas. Enseguida de la aparición del edema se estableció el diagnóstico de síndrome del espejo recidivante e hidrops no inmunitario. Se hospitalizó para drenaje del líquido. La amniorrexis se produjo a las 29 + 6 semanas. Una semana después se inició la dinámica uterina y el embarazo finalizó a las 31 semanas, después de la ruptura prematura de membranas. El neonato fue un varón de 3200 g, Apgar 2-6-8 al minuto, 5 y 10 minutos, respectivamente. Después del estudio postnatal se estableció el diagnóstico de perforación ileal múltiple. El recién nacido requirió 5 intervenciones quirúrgicas, con posoperatorio tórpido y se dio de alta a los 3 meses de vida. CONCLUSIONES: El síndrome del espejo es infradiagnosticado, a pesar de su potencial para complicar gravemente el embarazo asociado con hidrops. La recuperación de la madre suele ser favorable a los pocos días de posparto aunque la morbilidad y la mortalidad fetal son elevadas.

Abstract BACKGROUND: Ballantyne syndrome is characterized by the triad: fetal, placental and maternal edema. It is an uncommon condition associated with fetal hydrops, in which mother reflects fetal symptoms. It is essential to differentiate from preeclampsia, since there are common signs such as hypertension and proteinuria. Etiopathogenesis is unknown, although theories associated with an imbalance between angiogenic and antiangiogenic factors have been postulated. Treatment consists of ending the pregnancy or improving the fetal situation. CLINICAL CASE: We present the case of a 29-years pregnant woman controlled in the Maternal-Fetal Medicine Unit due to the history of Ballantyne Syndrome in the previous gestation. In the follow-up ultrasound performed at 26-weeks, placentomegaly, fetal ascites and increased amniotic fluid were detected. Subsequent ultrasounds showed polyhydramnios and fetal hydrops at 28-weeks. After maternal edema began, she was diagnosed with recurrent Mirror Syndrome and non-immune hydrops. Admission was indicated and amniodrainage was performed due to symptomatic polyhydramnios. Finally, premature rupture of membranes occurred at 29+6-weeks. She started uterine dynamic after one week, ending in a preterm delivery at 31-weeks after premature rupture of membranes. A 3200gr male was born with Apgar Scores 2-6-8 at 1, 5 and 10min respectively and, after postnatal study, he was diagnosed with multiple ileal perforation. Five surgical interventions were necessary, with a complicated postoperative period and could be discharged at 3 months of age. CONCLUSIONS: Mirror syndrome is an underdiagnosed pathology of unknown incidence that can seriously complicate gestation associated with fetal hydrops. Maternal recovery is favorable few days after delivery, but it leads to high fetal morbi-mortality.

Diagnostic Approach of Childhood Immune Thrombocytopenia / 임상소아혈액종양

Immune thrombocytopenia (ITP) is the most common cause of thrombocytopenia in children and can be defined as an autoimmune disorder of isolated thrombocytopenia without other causes of thrombocytopenia. This review will focus on the diagnostic approach of ITP, especially regarding the differential diagnosis. The practice of differential diagnosis has the goal of distinguishing primary ITP from secondary ITP and nonimmune thrombocytopenia requiring different treatments and showing different prognoses.

Nonimmunocompromised patients with allergic bronchopulmonary aspergillosis: clinical analysis of 11 cases / 中华临床感染病杂志

Objective To analyze the clinical features of nonimmunocompromised patients with allergic bronchopulmonary aspergillosis (ABPA).Methods The clinical data of 11 nonimmunocompromised patients diagnosed as ABPA from June 2010 to December 2015 in Zhejiang Jinhua People's Hospital were retrospectively analyzed.SPSS 18.0 was used for analysis.Results Among 11 patients with ABPA, Five were males and 6 were females, with an average age of (49.3 ±11.0) years.All patients had cough, expectoration and wheezing;cough and tan sputum in 4 cases, bloody sputum in 3 cases, fever in 2 cases and chest pain in 2 cases.In auscultation dry rales were heard in all patients , and limited wet rales were heard in 3 cases.The peripheral blood leukocyte counts were elevated in 5 patients [11.7(10.3-13.5) × 109/L)] and the eosinophils counts were increased in 9 patients [1.79(0.09-7.63) ×109/L].The total IgE was elevated to 3640(1329-9430) IU/mL.Skin prick test was positive ( grade 3 to 5) in 10 cases, Aspergillus fumigatus specific IgE increased to 23.6(1.75-67.30) kU/L in 6 cases, Aspergillus fumigatus specific IgG raised to 83.3(51-126) mg/L in 5 cases.Chest CT showed patchy, punctate exudation in 8 cases, central bronchiectasis in 9 cases, bronchial mucosal plug formation in 4 cases, and atelectasis in 1 case.Mediastinal lymph nodes were found in 2 cases.All 11 patients were treated with glucocorticoid hormone, and 8 patients were also received itraconazole oral solution for treatment.After treatment, the clinical symptoms were improved rapidly.Conclusion Nonimmunocompromised patients with ABPA have no specific clinical manifestations , and often are misdiagnosed as asthma , which is worth the attention of clinicians.

Estudo da etiopatogenia da hidropisia fetal não-imune a partir de uma série de casos utilizando um protocolo de investigação ampliado / Study of etiopathogenesis of non-immune hydrops fetalis from a series of cases using an expanded investigation protocol

A hidropisia fetal não-imune (HFNI) é causada por um grupo heterogêneo de condições e atualmente corresponde à maior parte dos casos de hidropisia fetal. Em função da ampla diversidade etiopatogênica, a investigação dos casos de HFNI constitui um desafio diagnóstico. Esse estudo teve como objetivo a avaliação prospectiva e sistemática de uma série de casos de HFNI a partir de um protocolo de investigação ampliado, que incluiu a pesquisa de doenças metabólicas. O presente estudo também incluiu a revisão dos casos de HFNI registrados previamente pelo Programa de Genética Perinatal na maternidade da Unicamp. Durante aproximadamente dois anos (2010-2012), foram identificados 53 casos de HFNI. Nesse período, ocorreram 6.129 nascimentos na maternidade local, com registro de HFNI em 37 recém-nascidos, conferindo uma prevalência de 60 por 10.000 nascimentos, valor maior do que o observado no período anterior ao estudo (1987 a 2009). Para o restante da análise, quatro casos foram excluídos devido à impossibilidade de estudá-los adequadamente. A maioria dos hidrópicos nasceu pré-termo (43 - 73,5%). Houve registro de 23 nativivos (47%), 10 óbitos no período neonatal e 26 óbitos durante a gestação (53%), resultado em uma mortalidade geral (pré-natal e neonatal) de 73,4%. A hidropisia foi identificada no pré-natal na maioria dos casos (44 - 89,8%) e, apesar da condição ser comumente associada a mau prognóstico, em três pacientes (6,1%) houve resolução completa e espontânea da hidropisia durante a gestação. Os principais grupos diagnósticos encontrados foram: anomalias cromossômicas (17 casos - 34,7%), quadros sindrômicos (16,4% - oito casos), cardiopatias e infecções congênitas (8,2% - quatro casos cada). Os erros inatos do metabolismo (EIM) corresponderam a 6,1% da amostra (três casos de doenças de depósito lisossômico). Três casos (6,1%) foram classificados como idiopáticos.

Non-immune hydrops fetalis (NIHF) is caused by a hetereogenous group of conditions, currently accounting for the most cases of hydrops fetalis. Because of the wide etiopathogenic diversity, the investigation of NIHF cases constitutes a real diagnostic challenge. This study aimed to evaluate prospectively and systematically a series of NIHF cases from an expanded research protocol including the investigation of metabolic diseases. The present study also aimed to revise the NIHF cases previously recorded by Perinatal Genetics Program (PGP) in the maternity hospital of Unicamp. During approximately two years (2010-2012), 53 cases were identified. In this period, among 6,129 births that occurred in our hospital, NIHF was identified in 37 newborns, given a birth prevalence of 60 per 10,000, higher than that was observed in the previous period - 23:10,000 (1987-2009). For purpose of all other analysis, four of the 53 cases evaluated had to be excluded due to inability to assess them correctly. Most hydropic individuals were born preterm (43 - 73.5%). Twenty-three patients (47%) were live births, 10 of them died before hospital discharge; and 26 (53%) died in the prenatal period, given an overall mortality of 73.4%. The hydrops were identified in prenatal period in most cases (44 - 89.8%), and despite being commonly associated with poor prognosis, three cases (6.1%) had complete and spontaneous resolution of hydrops during pregnancy. The main diagnostic groups were chromosomal abnormalities (17 - 34.7%), syndromic (8 - 16.4%), isolated heart defects (4 - 8.2%), and congenital infections (4 - 8.2%). Inborn errors of metabolism (IEM) occurred in three cases (6.1%), all represented by lysosomal storage diseases. Three cases (6.1%) were classified as idiopathic.

Amniotic fluid amino acid levels in non-immune hydrops fetalis: a case-control study

In a prospective case-control study, we compared the amniotic fluid amino acid levels in non-immune hydrops fetalis (NIHF) and normal fetuses. Eighty fetuses underwent amniocentesis for different reasons at the prenatal diagnosis unit of the Department of Obstetrics and Gynecology, Faculty of Medicine, Dicle University. Forty of these fetuses were diagnosed with NIHF. The study included 40 women each in the NIHF (mean age: 27.69 ± 4.56 years) and control (27.52 ± 5.49 years) groups, who had abnormal double- or triple-screening test values with normal fetuses with gestational ages of 23.26 ± 1.98 and 23.68 ± 1.49 weeks at the time of sample collection, respectively. Amniotic fluid amino acid concentrations (intra-assay variation: 2.26-7.85 percent; interassay variation: 3.45-8.22 percent) were measured using EZ:faast kits (EZ:faast GC/FID free (physiological) amino acid kit; Phenomenex, USA) by gas chromatography. The standard for quantitation was a mixture of free amino acids from Phenomenex. The levels of 21 amino acids were measured. The mean phosphoserine and serine levels were significantly lower in the NIHF group, while the taurine, α-aminoadipic acid (aaa), glycine, cysteine, NH4, and arginine (Arg) levels were significantly higher compared to control. Significant risk variables for the NIHF group and odds coefficients were obtained using a binary logistic regression method. The respective odds ratios and 95 percent confidence intervals for the risk variables phosphoserine, taurine, aaa, Arg, and NH4 were 3.31 (1.84-5.97), 2.45 (1.56-3.86), 1.78 (1.18-2.68), 2.18 (1.56-3.04), and 2.41 (1.66-3.49), respectively. The significant difference between NIHF and control fetuses suggests that the amniotic fluid levels of some amino acids may be useful for the diagnosis of NIHF.

Hidropesía fetal no inmune: Informe de un caso / Nonimmune hydrops fetalis: case report

Introducción: La hidropesía fetal es una condición clínica que se caracteriza por la acumulación anormal de líquidos en los tejidos blandos y en alguna de las cavidades serosas del feto. Es importante establecer con antelación esta condición, ya que debe conocerse la causa más probable, para ayudar en la mejor reanimación al nacimiento, la cual en el caso de un neonato con hidropesía fetal es un reto para el neonatólogo. Presentación del caso: Se presenta el caso de una embarazada de grupo sanguíneo O Rh negativo, no isoinmunizada, que cursó con anemia y hipoalbuminemia graves, con eclampsia, de la cual mediante cesárea se obtiene un producto del sexo femenino, de 32 semanas de gestación, con hidropesía fetal no inmune. Conclusiones: Se hace una revisión del tema con una discusión del abordaje diagnóstico y terapéutico actual.

Background: Hydrops fetalis is a clinical condition characterized by an abnormal fluid accumulation in soft tissues and in some serous cavities of the fetus. It is important to know beforehand if this condition is present in order to establish the most probable origin and to be prepared to administer optimal reanimation management of the neonate at birth. The care given to a newborn with hydrops fetalis is always a challenge for the neonatologist. Case report. We present the case of a pregnant, non-isoimmunized patient with RhO negative blood type. The following conditions were associated with her pregnancy: severe anemia, hypoalbuminemia, and preeclampsia/eclampsia. Delivery was accomplished with Cesarean section where a female neonate of 32 weeks gestation was delivered. Non-immune hydrops fetalis was present. Conclusions. We present recommendations for optimal diagnosis and therapy.

Current opinion in management of hydrops fetalis / 대한산부인과학회지

Hydrops fetalis is a morbid condition caused by Rh alloimmunization or a wide variety of fetal, placental, and maternal diseases. Mortality is high and depends on the gestational age at the time of occurrence and underlying etiology. Recent advances in obstetric ultrasound, prenatal diagnostics have made it possible to differentiate various etiologies involved. It is also possible to treat some of these fetuses prenatally. However, the majority of cases diagnosed remain untreatable. Early diagnosis of untreatable cases allows parents to make informed choices about subsequent management. Etiologies, maternal complications, diagnostic approach, treatment plans and prognosis are covered in this review.

A Case of Non-immune Hydrops Fetalis due to Congenital Syphilis

Congenital syphilis is a rare cause of non-immune hydrops fetalis. We cared for a neonate with hydrops fetalis who was delivered by emergency Cesarean section due to prolonged fetal bradycardia and ascites at 34 weeks of gestation. He had anemia, purpura, and hepatosplenomegaly, and the serologic tests revealed congenital syphilis (high titers of serum VDRL and TPHA, and a positive serum FTA-ABS IgM). He survived after aspiration of ascitic fluid, ventilator care, and intravenous penicillin therapy. We report a case of non-immune hydrops fetalis due to congenital syphilis with a brief review of literature.

Heart Poblems of Newborns with Nonimmune Hydrops Fetalis

PURPOSE: To understand the pathophysiology of nonimmune hydrops fetalis, retrospective study was achieved. We evaluated cardiac anomalies and heart functions of newborns with nonimmune hydrops fetalis admitted to our neonatal intensive care unit. METHODS: A retrospective study was conducted on the newborn diagnosed as nonimmune hydrops fetalis (NIHF) between January 1995 and December 2005. To analyze cardiac structures and heart functions of the study population, echocardiographic data were used that carried out within 2 days after birth. RESULTS: During the study period, 29 newborns (18 males and 11 females; mean birth weight 2,877 g; mean gestational age 34.4 weeks) were identified as NIHF. There were 15 cases of structural cardiac anomaly, 5 cases of cardiomegaly, 2 cases of arrhythmia and one case of pericardial effusion. Among those patent ductus arteriosus were observed in 12 cases and there were 10 cases of patent foramen ovale (PFO) or atrial septal defect. Ebstein's anomaly with PFO and atrioventricular septal defect was one case respectively. Most cases appeared hypoalbuminemia and anemia. Difference of heart functions between neonatal survival group and neonatal death group had no statistical significance. CONCLUSION: Because there was no significant difference in cardiac function between neonatal survival group and neonatal death group, simple decline of the heart function is not sufficient for the explanation of pathophysiologic mechanisms. Nevertheless, NIHF remains a challenging entity to improve perinatal outcome.

A case of chorioangioma of placenta complicated by fetal anemia and fetal hydrops / 대한산부인과학회지

Benign chorioangioma of the placenta is the most common primary tumor of the placenta similar to hamartoma. Most small-sized tumors do not make any clinical problem, but uncommon large tumors (>5 cm in diameter) may produce both maternal and fetal complications, such as polyhydramnios, preterm labor, fetal hydrops, microangiopathic hemolytic anemia, disseminated intravascular coagulation, intrauterine growth restriction, preeclampsia and placental abruption. In this respect, the diagnosis and management of chorioangioma and its complication should be done appropriately. We report a case of chorioangioma presenting with polyhydramnios, preterm labor, fetal anemia and fetal hydrops, diagnosed by antenatal ultrasonography and postnatal placental histologic examination, and live born baby with the brief review of the literature related to this type of tumor.

A Case of Non-immune Hydrops Fetalis due to Intraperitoneal Hemangioma

Hydrops describes the infant who has generalized edema due to accumulation of excess fluid. In severe case, massive edema with ascites and pleural and pericardial effusions are commonly combined. The main etiology of hydrops fetalis has been changed from immune type which is caused by fetomaternal blood group incompatibility to nonimmune type. Although cardiovascular diseases are the most common (23% to 38%) causes for nonimmune hydrops fetalis, fetal tumors still compromise 5% to 7% of the diseases. We report a case of nonimmune hydrops fetalis due to intraperitoneal hemangioma. The newborn infant was managed surgically and had excellent outcome.

Nonimmune hydrops fetalis: clinical characteristics and prognostic factors of neonatal survival / 대한산부인과학회잡지

OBJECTIVE: We undertook this study to find out clinical characteristics and prognostic factors of neonatal survival in nonimmune hydrops fetalis (NIHF). METHODS: From Oct. 1988 to Feb. 2001, 54 cases of nonimmune hydrops fetalis diagnosed at Seoul National University Hospital (SNUH) were included in our study. The incidence and perinatal mortality were investigated. The diagnostic work-up for associated conditions (or etiology) included detailed ultrasonography, karyotyping, fetal echocardiography, infection work-up (TORCH, parvovirus), and autopsy (if fetus was dead). Among 54 cases, 20 cases of liveborns were divided into two groups. Group I survived beyond neonatal period (survived>28 days) and group II did not (expired3 (OR=21, CI 1.77, 248.1; p3 (p<0.01). CONCLUSION: Over 3 of 1-min and 5-min AS were meaningful factors for neonatal survival in NIHF.

A Case of Nonimmune Hydrops Fetalis with neuroblastoma / 대한산부인과학회잡지

Hydrops fetalis is diagnosed when abnormal fluid collections are manifest in two or more fetal compartmnets including abdominal ascite, pleural effusion, percardial effusion, skin edema, polyhydroamniosis and placental edema. Although fetal hydrops was historically most commonly associated with Rh blood group isoimmunization, the availability of Rh immunoglobulin has increased the proportion of fetuses affected due to nonimmune etiologies. Neuroblastoma is a malignant tumor which originates in the autonomous nervous system. Congenital neuroblastoma is the most common solid malignant tumor of the neonatal period, incidence ranges 1:10,000 of all live births, retroperitoneal space being the most frequent localization. We have experienced a case of nonimmune hydrops fetalis with neuroblastoma at 32 weeks of gestation in 39 year old woman and reported that with brief review of related literatures.