[Prenatal diagnosis of polycystic kidneys and Mullerian duplication. A case report]. / Diagnóstico prenatal de riñones poliquísticos y duplicación mulleriana. Reporte de un caso.

Polycystic kidney disease is a common genetic cause of chronic kidney disease, characterized by the formation of multiple cysts in the kidneys and other organs, occurs in 1 in 20,000 live births. 30 to 50% of affected newborns die shortly after birth because of respiratory and renal insufficiency. This study reports the case of a newborn with polycystic kidney disease diagnosed by obstetric ultrasound at 26 weeks of gestation and kidneys anhidramnios due to increased volume and appearance "in sponge." Neonato a primigravida 19 years of age. At 26 weeks you will be detected in a routine obstetric evaluation that measures were in line for somatométricas fetal gestational age, and anhidramnios increase in renal mass and bilateral thorax narrow. The pregnancy ended in a cesarean section at 37 weeks with a newborn of 3140 g, women who died within minutes after birth. We requested an autopsy because of the need for genetic counseling. The findings were: enlarged kidneys with microcystic dilatation of collecting duct and in the renal cortex and medulla, liver fibrosis and Müllerian duplication with double uterine cavity. It is a rare association between polycystic kidney disease and Müllerian duplication, liver fibrosis confirmed by autopsy and has not been documented previously.

[Perinatal mortality in multiple pregnancies]. / Mortalidad perinatal en embarazos múltiples.

BACKGROUND: The incidence of multiple pregnancies has increased over the last decade and hence the hospital stay and perinatal morbidity and mortality. OBJECTIVE: to know the perinatal mortality rate of multiple pregnancies treated in a regional hospital. MATERIAL AND METHODS: Retrospective study conducted from the hospital records of deaths that occurred from January 2002 to December 2007 at the Hospital Regional de Especialidades No. 23 IMSS, Monterrey, Nuevo Leon, Mexico. RESULTS: during the study period (six years) were 122,822 births and 95 were multiple pregnancies (0.77/1000 births): 81 (85%) of triplets, 11 (11%) of quadruplets, two of quintillizos and one sextillizos with a total of 303 newborns. 26% (25/95) of multiple pregnancies had one or more deaths. The perinatal mortality rate was 165/1000 births (50/303) compared with that of pregnancies with one fetus in the same period 14.5/1000 births (p<0.0001). 20.9% (17/81) of triplet pregnancies and 54.5% (6/11) of the four had one or more deaths (p<0.02), with a perinatal mortality rate of 131/1000 births (32/243) and 295/1000 births (13/44) (p<0.006), respectively. Only 2/10 (20%) of newborns in two pregnancies quintillizos died and 3/6 (50%) of a pregnancy sextillizos. There was no difference in the proportion of sexes, but in the average weight of newborns: the weight of the triplets was alive vs dead vs 1659 g 713 g (p<0.0001) and the quadruplets from 750 g vs 1341 g (p<0.0007), respectively. CONCLUSIONS: 26% of multiple pregnancies had one or more deaths and 15% of the neonates died (165/1000 births). Multiple pregnancies were complicated with one or more deaths at 20% of triplets, 54% of quadruplets and 100% of quintillizos. The perinatal mortality rate of pregnancies with multiple fetuses 3 and 4 was No. 131/1000 and No. 295/1000, respectively.

[Normal parameters of ear acoustic emissions in neonates]. / Parámetros de normalidad de las otoemisiones acústicas en neonatos.

INTRODUCTION: The ear acoustic emissions (EAE) are produced from pure low intensity tones coming from the cochlea in response to a stimulus. Hypocusia in the neonate may be detected by universal hearing screening programs using EAE that have a sensitivity of 91% and specificity of 85%. OBJECTIVE: To define the normal limits we measured the EAE in a group of healthy newborns and calculated the media and standard deviation (SD). PATIENTS AND METHODS: We enrolled 280 healthy neonates, previous external ear evaluation for permeability and we measured the EAE at 7 to 30 days of life at 2000, 2500, 3187, 4000, 5062 y 6375 Hz, using a 55-65 dB stimulus for each frequency band. RESULTS: In each frequency band evaluated we calculated the media: 10.76, 9.16, 9.46, 7.89, 4.83, 2 dB SPL, and minus two SD: 0.54, -0.33, 0.06, -2.04, -4.37, -7.43 dB SPL, that we considered the lower limit of normality for both ears in each frequency band tested. There were no significant differences between measurements in both ears (p>0.05). CONCLUSIONS: The EAE measurement is an easy, and low cost test that can be used to make the universal hearing screening accessible. These results support the normal levels of EAE and the Corti organ function in healthy newborns.

[Hearing screening and diagnosis of hearing loss: high risk versus low risk neonates]. / Tamizaje y confirmación diagnóstica de hipoacusia. Neonatos de alto riesgo versus población abierta.

INTRODUCTION: congenital hearing loss is a serious health problem affecting 1 to 3 out of 1000 neonates, and is the most common cause of neurosensorial defect; hearing screening helps to identifying early permanent childhood hearing loss (HL). OBJECTIVE: to establish the percentage of hearing loss in newborns with and without risk factors through hearing screening. MATERIAL AND METHODS: by using a cross-sectional design, a group of neonates with risk factors and a group without risk factors were included in a two-stage hearing screening program. The otoacoustic emissions (OAEs) procedure was done in the first stage and the automated auditory brain stem response (AABR) procedure in the second stage. The latter was performed only in children with abnormal results in the OAE procedure. RESULTS: 518 newborns were included in the hearing screening procedures; 220 neonates with risk factors and 298 without risk factors. 35 had + OAE test and the ABBR procedure helped to confirm that 30 neonates had hearing loss, 26 had bilateral HL; 10 had asymmetric HL; 4 had unilateral HL and 5 were false positives. The AABR procedure ascertained 17 HL cases with a threshold > 40 dB (86 per 100,000), 11 cases (55 per 100,000) in the risk group and 6 (30 per 100,000) in the non-risk group (p < 0.05). The main risk factors were prematurity, craniofacial anomalies, mechanical ventilation, prescription of amikacin and cytomegalovirus infection. CONCLUSIONS: the two-stage hearing screening program ascertained that 5.7 % had HL. AABR (threshold > 40 dB) confirmed 17 (86 per 100,000) neonates with HL, which was more frequent in the risk group (5 %) than in the non-risk group (2 %; p < 0.05).