Prognostic factors and clinical features in liveborn neonates with hydrops fetalis.
ABSTRACT The purpose of this study was to delineate the etiology and the clinical features of liveborn neonates with hydrops fetalis, and to explore the prognostic factors for survival. Medical records of 28 liveborn neonates with hydrops fetalis between April 1995 and March 2005 were reviewed retrospectively. Demographic data, clinical manifestations, laboratory findings, and outcomes were analyzed. Most patients presented with pleural effusions (21 of 28) and ascites (22 of 28). The majority of patients had hydrops due to cardiovascular diseases (seven of 28), hematologic disorders (six of 28), lymphatic malformations (six of 28), and idiopathic origins (six of 28). The overall survival rate was 50% and was highest (83%) in infants with lymphatic malformations. By univariate analysis, risk factors for mortality are earlier ages at diagnosis and at birth, low Apgar scores, need for resuscitation in the delivery room, low serum albumin level, and severe acidemia. After using stepwise multiple logistic regression analysis, the most significant factors associated with fatality were younger gestational age at birth and lower serum albumin level. Hydrops fetalis remains a complex condition with a high mortality rate. Hydrops resulting from lymphatic malformations has a favorable outcome. Preterm birth at less than 34 weeks and serum albumin concentration lower than 2 g/dL are two poor prognostic factors for survival.
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ABSTRACT: Abstract Non-immune hydrops fetalis (NIHF) is a challenging entity as it represents the end stage of several different disorders. Renal and genitourinary causes of NIHF are rare and include congenital renal malformations, tumours and ureter-urethra disorders. Herein, two NIHF cases with different renal causes were presented. The first case that had antenatal NIHF was diagnosed Neonatal Bartter Syndrome. The second case of NIHF with antenatal large cyst in the surrenal gland area required surgery and ectopic renal cyst was diagnosed. To our best of knowledge, these are the first reports of NIHF associated with neonatal Bartter syndrome and ectopic renal cyst in neonates. Although it may be coincidental, these cases suggest that both neonatal Bartter syndrome and unilateral ectopic renal cyst may cause NIHF development in neonates by several different mechanisms. Therefore, these two rare entities should be suspected in cases of NIHF with similar findings.The journal of maternal-fetal & neonatal medicine: the official journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians 03/2013; · 1.36 Impact Factor
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ABSTRACT: BACKGROUND: Lysosomal storage disorders (LSD) are a rare cause of non immunological hydrops fetalis (NIHF) and congenital ascites. The reported incidence is about 1%. The incidence of idiopathic NIHF is estimated to be about 18%.Patients and methodsWe report four cases with transient hydrops fetalis resulting from LSD and performed a literature review on LSD with NIHF and congenital ascites in combination. RESULTS: At present, 12 different LSDs are described to be associated with NIHF or congenital ascites. Most patients had a family history of NIHF, where the preceding sibling had not been examined. A diagnostic approach to the fetus with NIHF due to suspected LSD either in utero or postnatal is suggested. Transient forms of NIHF and/or ascites in association with MPS IVA, MPS VII and NPC are described for the first time in this publication. CONCLUSIONS: LSD should be considered in transient hydrops. Enzymatic studies in chorionic villous sample or amniotic cultured cells, once the most common conditions associated with fetal ascites or hydrops have been ruled out, are important. This paper emphasizes the fact that LSD is significantly higher than the estimated 1% in previous studies, which is important for genetic counseling as there is a high risk of recurrence and the availability of enzyme replacement therapy for an increasing number of LSD.Orphanet Journal of Rare Diseases 11/2012; 7(1):86. · 4.32 Impact Factor
- Pediatrics & Neonatology 12/2013; · 0.93 Impact Factor