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Objective Fetal serum β2-microglobulin has been shown to predict postnatal renal outcome in cases of fetal obstructive uropathy. We assessed the value of serial measurements of fetal serum β2-microglobulin in the prediction of postnatal renal outcome. Methods We retrospectively studied renal outcome in 42 fetuses with bilateral or low urinary tract obstruction that had fetal blood sampling on at least two occasions to assay serum levels of β2-microglobulin. Amniotic fluid volume at the time of each sampling was recorded. We classified renal outcome as either favorable (when postnatal renal function was normal) or adverse (when postnatal chronic renal failure occurred or when renal dysplasia at autopsy was noted). A β2-microglobulin cut-off of 5 mg/L and amniotic fluid index of 5 cm were used to predict postnatal renal outcome. Results Renal outcome was adverse in 28 cases and favorable in 14. In 12 (28.6%) cases, fetal serum β2-microglobulin concentration differed between the first and last measurement. Prediction of postnatal renal outcome was correct in 11 of these cases based on the last β2-microglobulin measurement. The sensitivity of β2-microglobulin in predicting renal outcome was significantly higher (P = 0.005) when using the last rather than the first measurement (96.4% vs 64.3%), with similar specificity for both measurements (85.7% vs 78.6%, non-significant). The sensitivity of amniotic fluid volume was also significantly higher (P = 0.005) when using the last rather than the first measurement (75.0% vs 35.7%), with similar specificity for both measurements (64.3% vs 71.4%, non-significant). Conclusion Sequential measurement of serum β2-microglobulin, performed for adverse ultrasound findings, such as renal parenchymal abnormality or decreasing amniotic fluid volume, predicts postnatal renal outcome more accurately than does a single assay. This may be due to possible worsening of renal injury with increasing duration of urinary tract obstruction. Copyright © 2016 ISUOG. Published by John Wiley & Sons Ltd.

Bartter syndrome is a severe inherited tubulopathy responsible for renal salt wasting. It is caused by alterations in ion channels located in the thick ascending limb of Henle's loop. Five mutations in four genes – SLC12A1, KCNJ1, CLCNKB, and BSND – coding for transporters and one specific mutation (L125Q) in the CASR gene, which encodes the calcium‐sensing receptor, have been identified 1, 2. First described in 1962 3, two presentations are currently recognized. The classic form develops in childhood and is characterized by severe polyuria, dehydration, hypokalemic metabolic alkalosis, secondary hyper‐aldosteronism, and hypercalciuria sometimes leading to nephrocalcinosis. The second presentation is antenatal, more severe 4, 5 and is revealed by refractory polyhydramnios (caused by fetal polyuria) during the second trimester of pregnancy, without morphological anomalies. The major consequences are preterm birth, growth retardation, and severe dehydration at birth due to salt loss. In children with Bartter syndrome, urinalysis highlights increased sodium, potassium, chloride and calcium excretion, and as a consequence hyponatremia, hypochloremia, and hypokalemia 4, 6. However, until now, no studies have been published about fetal urinalysis in antenatal Bartter syndrome. A 28‐year‐old woman in a consanguineous marriage (first cousins), gravida 1, para 0, presented severe polyhydramnios at routine ultrasound examination at 22.6 weeks of amenorrhea. Megacystis was observed in a female fetus, without any other malformation. After genetic counseling, invasive procedures were offered to the parents and accepted: amniotic fluid sampling for karyotyping and biochemistry and fetal urine sampling for renal function evaluation. The karyotype was normal, 46,XX. A second amniocentesis was secondarily performed at 26.3 weeks for amniodrainage. Amniotic fluid alpha‐fetoprotein and total protein were decreased at both 22.6 and 26.3 weeks (Table 1), giving a Bartter index (total protein expressed in g/L x alpha‐fetoprotein in MoM) of 0.85 and 0.71, respectively 7. These values were strongly suggestive of Bartter syndrome. In fetal urine we assayed electrolytes, beta‐2 microglobulin, and total protein. Results are presented in Table 1 in comparison with two fetal urine controls (one with renal failure and one with normal renal function, both performed because of megacystis). Electrolytes were significantly increased in Bartter syndrome compared with controls (P < 0.001). A weekly check‐up was planned. The patient delivered a girl at 33.6 weeks of gestation. The baby presented clinical and laboratory signs of Bartter syndrome. Postnatal screening for Bartter syndrome mutations detected a homozygous mutation in the SLC12A1 gene. Table 1 Fetal urine and amniotic fluid electrolytes (mmol/L), β2‐microglobulin (β2 m), and total protein in Bartter syndrome and controls

Prenatal diagnosis of esophageal atresia (EA) may improve the outcome of affected neonates by allowing optimization of both prenatal and postnatal care. Prenatal sonographic detection is based on polyhydramnios and/or nonvisualization of the fetal stomach bubble, two signs with a large number of etiologies. We evaluated a biochemical approach to improving diagnostic efficiency. We compared amniotic fluid biochemical markers in 44 EA cases with 88 polyhydramnios and 88 nonpolyhydramnios controls. Both matched for GA with cases. Total proteins, alpha-fetoprotein (AFP), and digestive enzyme activities were assayed, including gamma-glutamyl transpeptidase (GGTP). We defined an EA index (AFP multiplied by GGTP). A significant difference (p < 0.0001) was observed for total protein, AFP, GGTP, and EA index between the EA group and each of the two control groups. No statistical difference was observed for any marker between the two most frequent EA subgroups (type I and type III) or between the two control groups. Using a cutoff of 3 for the EA index, 98% sensitivity and 100% specificity were observed for amniotic fluid prenatal diagnosis of EA, whatever the anatomical type. A large prospective series is required to confirm these results.

The present study investigated the ability of human choriodecidua to induce myometrial cell apoptosis through the secretion of tumor necrosis factor alpha (TNF). The secretion of TNF was evaluated in the culture supernatants of amnion and choriodecidua explants that were exposed to the bacterial endotoxin lipopolysaccharide (LPS) to mimic inflammation. The choriodecidua explants produced more TNF than the amnion explants in response to LPS stimulation, despite the fact that the choriodecidua had lower levels of TLR4 expression. Moreover, conditioned medium obtained from LPS-treated choriodecidua explants, but not that from amnion explants, decreased the number of viable cultured myometrial cells and induced cell apoptosis by inducing the overexpression of the proapoptotic protein BAX and by decreasing the expression of the anti-apoptotic protein BCL2. Neutralization of TNF in the choriodecidua-conditioned medium reversed this effect. Exogenous TNF mimicked LPS-treated choriodecidua-conditioned medium in that it induced myometrial cell apoptosis, reduced BCL2 expression, and increased BAX expression. Using neutralizing antibodies against both subtypes of TNF receptors, we found that only TNFRSF1A participates in TNF-induced myometrial cell apoptosis. Our in vitro model of LPS-induced inflammation of human fetal membrane explants suggests a mechanism by which TNF secreted by choriodecidua governs human myometrial cell apoptosis at the end of pregnancy. These data support the hypothesis that TNF participates in the complex network of signaling processes associated with uterine involution.

cc ep te d A rti cl e Non-visualization of fetal gallbladder: fetal assessment based on two case reports Francoise MULLER, Pierre BERNARD, Laurent J. SALOMON, Sophie DREUX, Bichr ALLAF, Isabelle CZERKIEWICZ, Yves VILLE 1. Department of Biochemistry-Hormonology, Robert Debre Hospital AP-HP, Paris, France 2. University Paris Ile de France Ouest, Versailles Saint-Quentin, France 3. Department of Obstetrics and Maternal Fetal Medicine, Saint Luc Academic Hospital, Brussels, Belgium. 4. Department of Obstetrics and Maternal Fetal Medicine, Necker-Enfants Malades Hospital, AP-HP, Paris, France 5. University Paris Descartes and Sorbonne Paris-Cite, Paris, France Corresponding author: Francoise Muller, Biochimie-Hormonologie, Hopital Robert Debre 48 Boulevard Serurier, 75019 Paris, France Tel: (33) 1 40 03 53 82, Fax: (33) 1 40 03 53 80 e-mail: francoise.muller@rdb.aphp.fr

Objectives To determine whether the prognostic value of fetal serum β-2-microglobulin is altered and whether the occurrence of fetal urinary ascites prevents kidney damage in cases of fetal obstructive uropathy with urinary ascites. Methods This was a retrospective study of cases of fetal bilateral obstructive uropathy that occurred between 2006 and 2010, for which both fetal serum and ascites samples were sent to our laboratory for analysis. β-2-microglobulin was assayed in both fetal serum and the corresponding ascites. Renal outcome was analyzed. Histological features of the kidney in cases of termination of pregnancy and renal function of liveborn infants were recorded. Results Fourteen cases with analysis of fetal serum and fetal ascites in a context of urinary obstruction were included. Renal outcome was unfavorable in eight cases (57%) and favorable in six (43%). When fetal serum β-2-microglobulin was

Objectives To determine whether there is an association between the fetal ultrasound finding of hyperechoic colon and the gestational age at which it presents and cystinuria. Methods A prospective national survey was performed in France including all observations of isolated fetal hyperechoic colon detected at routine second- and third-trimester ultrasound over a 2-year period. Collected images were reviewed by experts. Colon was defined as being hyperechoic when its echogenicity was at least equal to that of the iliac bone. It was diagnosed when large tubular echogenic portions of the colon, without a focal mass and without posterior acoustic shadows, were observed at the periphery of the abdomen. Urinary amino acid analysis was performed after birth in the cases identified to test for cystinuria. Results Nineteen fetuses with ultrasound findings of hyperechoic colon were included, and the mothers of 16 of these agreed to participate in the study. In eight of nine cases of hyperechoic colon observed before 36 weeks' gestation cystinuria was confirmed at birth. In the seven remaining cases, observed after 36 weeks, none was found to have cystinuria and all had normal images at previous routine ultrasound scans at 22 and 33 weeks. When present, no difference in the sonographic appearance of hyperechoic colon was noted between the two groups. In the cystinuria-affected cases, the length of the hyperechoic mass appeared to increase with gestational age. Conclusions In our experience, the presence of a hyperechoic colon at routine ultrasound scan before 36 weeks' gestation should prompt screening for cystinuria at birth, while later observation (> 36 weeks) of this finding does not appear to be related to any disease. Copyright © 2011 ISUOG. Published by John Wiley & Sons, Ltd.

Methods Fifty-five pregnant women with renal disease were compared with 110 patients matched for maternal age, maternal weight, smoking status, and gestational age at a 1: 2 ratio. Maternal renal function was assayed at the time of the combined screening and renal insufficiency was defined by serum creatinine > 90 μmol/L and renal clearance < 80 mL/min. We defined 3 groups based on renal function: kidney disease and normal function (group 1), kidney disease and renal insufficiency (group 2) and a control group (group 3). The values of nuchal translucency, PAPP-A and hCGβ as well as false-positive rates for Down syndrome screening were studied and compared between groups.