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Biomedical subjects

H L van Straaten

Publications and source records attributed to H L van Straaten.

14 recordsLinked to original sources

Efficacy of automated auditory brainstem response hearing screening in very preterm newborns.

OBJECTIVE: To investigate the efficacy of an automated auditory brainstem response (AABR) hearing screening method in very preterm newborns in the neonatal intensive care setting. STUDY DESIGN: In this prospective cohort study, 90 consecutive preterm newborns (<32 weeks' gestational age) had AABR hearing screening weekly from birth until a bilateral pass result was obtained. If the newborn had a unilateral pass result, AABR screening was repeated in the same week. Data were analyzed by using the Kaplan-Meier survival function technique, resulting in a cumulative pass rate curve for postmenstrual age. Cox's regression method was used to analyze the effect of co-variables, such as sex and growth restriction, on pass rates. RESULTS: Median gestational age was 29.5 weeks (range, 25.3-31.9 weeks), and median birth weight was 1115 g (range, 600-1960 g). Mean age was 6.2 days (SD 4.3) at first test, 15.7 (SD 8.1) at second test, and 21.4 (SD 8.6) at third test. Eighty percent (CI: 70.2%-89.8%) of the newborns passed at 30.3 weeks' postmenstrual age, 90% (CI: 83.6%-96.4%) passed at 31.2 weeks, and 100% passed at 34 weeks' postmenstrual age. The attainment of these pass rates correlated to postmenstrual age was not significantly influenced by sex, growth restriction, or gestational age at birth. Postnatal pass rates (in days) were strongly influenced by gestational age. CONCLUSION: AABR pass rates of >80% can be obtained from 30 weeks' postmenstrual age. Therefore AABR neonatal hearing screening can be used before discharge from a neonatal intensive care unit.

Brain Stem↗

[Neonatal hearing screening].

Approximately 1 to 2 per thousand live-born infants suffer from a serious perceptive hearing loss. Normal hearing from birth is essential for optimal human development (language and speech, social and emotional development, communicative skills and learning). The earlier the hearing loss is diagnosed the better the prognosis for the infant with a hearing impairment. Suitable methods are now available for neonatal hearing screening: automated measurement of auditory brain stem response and measurement of oto-acoustic emissions. Screening must be viewed as only the first step in a program of diagnosis, treatment and habilitation of these children. The ultimate goal of the implementation of neonatal hearing screening is: identification of bilateral hearing losses before the age of 3 months and start of therapy and counselling before the age of 6 months.

Age Factors↗

Evaluation of a strategy to limit blood donor exposure in high risk premature newborns based on clinical estimation of transfusion need.

OBJECTIVES: Reservation of dedicated series of pedipacks, consisting of 3 to 4 units of 70 ml filtered red cell concentrate in additive solution SAGM from 1 donor, may reduce donor exposure. In this prospective efficacy study the benefits, release and expiration of pedipacks (PP) assigned to preterm infants requiring neonatal intensive care are analyzed. METHODS: On the basis of clinical assessment of the need for multiple transfusions, 96 preterm neonates (gestational age < 32 wks and/or birth weight < 1500 g) were assigned to either the high risk group (HRG), who were to receive dedicated donor blood units, or the low risk group (LRG). Inclusion criteria for HRG were 1) estimated time of admission > 21 days and 2) expected need for multiple transfusions due to clinical cardiorespiratory instability, prolonged parental feeding or frequent blood sampling. To reduce wastage of donor blood, dedication of donor blood units was limited to 21 days. RESULTS: 50 series (192 PP) were assigned to 42 HRG infants. Two HRG infants received 3 series, 4 received 2 series and 36 received 1 series of PP. Mean transfusion rate was 3.1 PP in the HRG and 0.4 in the LRG. In the LRG 35 of 54 were not transfused, 19 received 1 to 2 PP. In both groups transfused newborns were exposed to 1.1 donors in average. In the HRG of 192 PP, 137 PP (71%) were used within 21 days, and another 30 (16%) before the expiration date < 35 days. Twenty five PP (13%) expired, mainly because of logistical problems in the introduction phase. CONCLUSION: Assignment of dedicated PP on the basis of clinical parameters at entry considerably reduces donor exposure in HRG. Wastage of dedicated blood transfusions was reduced by limitation of the dedicated period (21 days). In terms of efficacy, reservation and use of PP can be optimized by standardized administrative measures.

Anemia↗

"Shake hands"; diagnosing a floppy infant--myotonic dystrophy and the congenital subtype: a difficult perinatal diagnosis.

Myotonic dystrophy is a multi-organ disease inherited in a complicated way. Congenital myotonic dystrophy is a distinct entity with severe symptoms leading to a high rate of perinatal morbidity and mortality. The occurrence of congenital myotonic dystrophy often allows a subsequent diagnosis in the mother with important implications for her life, her further pregnancies and offspring. Genetic principles of anticipation and somatic mosaicism are involved and hamper the prenatal diagnostic possibilities. A family is presented in which maternal myotonic dystrophy and congenital myotonic dystrophy were diagnosed after the third pregnancy. The key features leading to the diagnosis were obstetric history, neonatal hypotonia and asphyxia, facial abnormalities in the mother together with the inability to bury eyelashes and delayed release of grip after shaking hands. The disorder is reviewed with respect to clinical symptoms, pathogenesis and genetics.

Adult↗

Automated auditory brainstem response in neonatal hearing screening.

Severe congenital hearing impairment is an important handicap affecting 0.1% of apparently healthy liveborn infants and 1-2% of graduates of neonatal intensive care units. The prognosis for intellectual, emotional, language and speech development in the hearing-impaired child is improved when the diagnosis is made early and intervention is begun before the age of 6 mo. Universal screening is preferable, since about 50% of infants with hearing loss are not discovered if neonatal hearing screening is restricted to high-risk groups. The automated auditory brainstem response (AABR) screener is a dedicated hearing screening device which provides information not only about the outer/middle ear and cochlea but also about the auditory pathway up to the brainstem. AABR has an agreement with conventional auditory brainstem response up to 98%. It uses a 35 dB near hearing level click. No operator interpretation is needed and it can be used on the ward and during oxygen therapy without disturbance from ambient noise. Reported referral rates in a hospital-based screening programme at the time of discharge vary, with an average of 4%. AABR has also been used in a home-based setting, with the same results. The time necessary for screening varies with the setting, but ranges from 4 to 15 min. Initial costs range from $15 to $25 per test, which is similar to neonatal screening for metabolic diseases. In addition to individual healthcare savings, early diagnosis may lead to savings on costs of intensive speech-language intervention and educational facilities.

Audiometry↗

The impact of surfactant replacement therapy on cerebral and systemic circulation and lung function.

UNLABELLED: The influence of surfactant administration on cerebral and systemic circulation and on lung function was evaluated in 12 premature mechanically ventilated infants (mean birth weight 1,560 +/- 770 g, mean gestational age 30.0 +/- 3.2 weeks) with respiratory distress syndrome (RDS) receiving surfactant replacement therapy. We measured mean cerebral blood flow velocity (MCBFV), heart rate (HR), mean arterial pressure (MAP), static compliance (Crs), resistance of respiratory system (Rrs), functional residual capacity (FRC) and fraction of inspired oxygen (FiO2). In addition to a very low compliance and a moderately elevated resistance of the respiratory system a significant drop in MAP, HR, MCBFV and FiO2 was noticed after surfactant administration. After 30 min HR, MAP and MCBFV values returned to baseline levels. We postulate that the drop in MCBFV, MAP, HR and FiO2 with a minor, though not significant improvement of the FRC can most likely be explained by a "relative" hypovolaemia in other organs and parts of the body due to expansion of the lung vascular bed. Compensation for the redistribution of circulatory volume occurred within several minutes. Blood pressure control and treatment of hypovolaemia is mandatory before surfactant is administered. CONCLUSION: In RDS patients there is a significant drop of MAP, HR, MCBFV and FiO2 after bolus surfactant administration.

Airway Resistance↗

Restrictive dermopathy and associated prenatal ultrasound findings: case report.

Restrictive dermopathy is a lethal autosomal recessive skin disease. Prenatal diagnosis has not yet been reported. We present a case of restrictive demography and describe the associated prenatal sonographic findings. A continuously open mouth was the most striking feature and this sonographic finding may be a marker of a skin disease.

Abnormalities, Multiple↗

Neonatal hearing screening.

UNLABELLED: Severe congenital hearing impairment is an important handicap affecting 0.1% of live-born infants and 1%-2% of graduates of Neonatal Intensive Care Units. The prognosis for intellectual, emotional, language and speech development in the hearing-impaired child is improved when the diagnosis is made early and intervention is begun before the age of 6 months. The usual age at diagnosis of hearing impairment is at least 18-30 months (or even later in cases of less severe hearing impairment) where there are no screening programmes. When screening is carried out using distraction methods at the age of approximately 9 months some hearing-impaired infants are missed and those discovered are at least 15-18 months before intervention begins. Neonatal screening could give hearing-impaired children the best chances for optimal care and development. Universal neonatal hearing screening is necessary, because, when neonatal hearing screening is restricted to high risk groups 30%-50% of infants with hearing loss are not discovered. The methods available for neonatal hearing screening are discussed in this paper. CONCLUSION: In our view automated measurement of auditory brainstem responses is the most valuable method for universal neonatal hearing screening.

Audiometry, Evoked Response↗

Evaluation of an automated auditory brainstem response infant hearing screening method in at risk neonates.

UNLABELLED: An automated auditory brainstem response (ABR) method-the ALGO-1 Plus- has been developed for hearing screening in healthy neonates. The aim of this study was to test the validity of this automated ABR screening method in at-risk neonates in a neonatal intensive care unit. Two hundred and fifty at-risk neonates were selected for screening according to the criteria of the American Joint Commerce on Infant Hearing. All 250 neonates were screened with the ALGO-1 Plus for bilateral hearing loss. When two consecutive screenings pointed to bilateral hearing loss ("refer") further audiological investigations were performed and where necessary therapeutic measures were taken. All children who "passed" the screening unilateral or bilateral enrolled in a nationwide behavioural screening programme at the age of 9 months as well as in a 6-monthly follow up programme documenting speech and language development. A total of 245 (98%) neonates passed the ALGO-1 screening, 230 (92%) at the first attempt and 15 (6%) at the second attempt. Five (2%) were referred with bilateral hearing loss. One of these died of congenital rubella shortly after screening and bilateral congenital hearing loss of > 35 dB was confirmed in the other 4. None of the infants who passed the screening were discovered to have moderate to severe bilateral hearing loss (> 40 dB) with behavioural screening (n = 183/233) or at follow up (n = 233/233). In this study, all at-risk neonates with bilateral congenital hearing loss were detected with ALGO-1 Plus screening. No false-negatives were discovered. CONCLUSION: The ALGO-1 Plus infant hearing screener can be used as a valid automated ABR-screener to detect hearing loss in at-risk neonates in a neonatal intensive care setting.

Evoked Potentials, Auditory, Brain Stem↗

Subependymal germinolytic cysts in Zellweger syndrome.

Ultrasonographic demonstration of periventricular cysts is usually associated with cystic periventricular leucomalacia due to necrosis of periventricular unmyelinated white matter. Cystic spaces with a similar appearance may also arise from prenatal lysis of fetal periventricular matrix tissue, rather than immature white matter. The resultant empty spaces are called "pseudocysts" or germinolytic cysts. Neuropathological studies have shown that germinolytic cysts may arise in Zellweger syndrome (generalized peroxisomal disorder). Here, we report their ultrasonographic demonstration in Zellweger syndrome, emphasizing the potential value of this sign in the diagnosis.

Brain Diseases↗

Chylothorax in the neonatal period.

Chylothorax is defined as an effusion of lymph in the pleural cavity. In the neonate both congenital and traumatic (iatrogenic) forms exist. Birth asphyxia and respiratory insufficiency are major symptoms of congenital chylothorax, requiring resuscitation and artificial ventilation. Antenatal diagnosis by ultrasound allows early therapeutic intervention such as ventilatory support and drainage of chylous fluid immediately after birth. Traumatic chylothorax is mainly seen after intrathoracic surgery. Treatment primarily consists of continuous or intermittent drainage of chyle with replacement of fluid-, electrolyte-, and protein losses and parenteral nutrition. Introduction of oral feeding is considered only after a substantial period without chyle production in the pleural cavity and consists of a medium-chain triglyceride containing formula. In a minority of cases surgical intervention is necessary.

Chylothorax↗

Comparison of the effect of midazolam or vecuronium on blood pressure and cerebral blood flow velocity in the premature newborn.

The effect of midazolam and vecuronium on mean arterial pressure (MAP) and mean cerebral blood flow velocity (MCBFV) was evaluated in premature infants (birthweight 550-2,560 g; gestational age 26-36 weeks) randomised to receive either 0.1 mg/kg midazolam (n = 7) or 0.05 mg/kg vecuronium (n = 8) intravenously. MAP, by means of an indwelling arterial catheter, and MCBFV, by means of non-invasive pulsed-Doppler of the middle cerebral artery, were measured every 5 min, starting at 10 min prior to until 1 h after drug administration. A transient 25-43% decrease in MCBFV (mean 0.06 m/s) dependent on a 8-23% decrease in blood pressure (mean 9 mm Hg) was noted in all patients within 15 min following administration of midazolam, which returned to baseline values within 1 h. In 2 out of 7 infants, a plasma expander was required. In contrast, vecuronium only decreased the MCBFV in 3 of 8 infants. Thus, a bolus of midazolam transiently decreased blood pressure and MCBFV, and should be used cautiously in sick preterm infants.

Blood Flow Velocity↗

[Chylothorax in the neonatal period].

The data of 11 infants with chylothorax in the neonatal period, seven with congenital chylothorax and 4 infants with iatrogenic chylothorax are reported. Chylothorax in the neonatal period may cause birth asphyxia and serious respiratory problems. Among the infants with congenital chylothorax diagnosis can be established before birth by ultrasound technique, followed by optimal resuscitation to prevent asphyxia. In most of the cases, conservative treatment with continuous drainage and total parenteral nutrition is sufficient.

Cardiac Surgical Procedures↗