[Significance of CSF levels of tau protein and beta42-amyloid for diagnosis and differential diagnosis of dementia diseases in a specialized geriatric hospital]. / Stellenwert der Liquordiagnostik für die Diagnose und Differenzialdiagnose demenzieller Erkrankungen in einer geriatrischen Fachklinik.

Considering the incidence of dementia, the development of procedures that allow correct differential diagnosis is gaining increasing importance. The analysis of spinal fluid from 632 patients, who were admitted with suspected dementia, diagnosed dementia or dementia of unclear etiology, showed that there was high differential diagnostic power for tau protein, but not for beta-amyloid.

Neonatal screening for hearing disorders in infants at risk: incidence, risk factors, and follow-up.

OBJECTIVE: To determine the incidence and risk factors for hearing disorders in a selected group of neonates and the feasibility of selective hearing screening. SETTINGS: Multicenter prospective trial at five centers in Germany. METHODS: Enrollment criteria: in addition to previously defined risk factors by the Joint Committee on Infant Hearing (family history of hearing loss, in utero infections, craniofacial anomalies, birth weight <1500 g, critical hyperbilirubinemia, ototoxic medications, bacterial meningitis, postnatal asphyxia, mechanical ventilation >5 days, stigmata, or syndromes associated with hearing loss), the impact of maternal drug abuse, birth weight <10th percentile, persistent pulmonary hypertension, and intracranial hemorrhage more than or equal to grade III or periventricular leukomalacia on infant hearing were evaluated. The screening procedure was performed by automated auditory brainstem response (A-ABR; ALGO 1-plus; Natus Med Inc, San Carlos, CA). STATISTICS: univariate analyses of risk factors versus A-ABR results and a multivariate regression analysis were used; additionally, the total test time was recorded. RESULTS: Seven hundred seventy recordings from 777 infants enrolled consecutively constitute the basis of this analysis. Mean gestational age was 33.8 +/- 4.3 weeks, birth weight 2141 +/- 968 g; 431 infants being male and 339 female; 41 (5.3%) infants exhibited pathologic A-ABR results (16 bilateral and 25 unilateral). Meningitis or sepsis, craniofacial malformations, and familial hearing loss were independent significant risk factors. Median total test time was 25 minutes. Follow-up examinations in 31 infants revealed persistent hearing loss in 18 infants (13 infants sensorineural, 5 from mixed disorders), 7 requiring amplification. CONCLUSION: Hearing screening in high-risk neonates revealed a total of 5% of infants with pathologic A-ABR (bilateral 2%). Significant risk factors were familial hearing loss, bacterial infections, and craniofacial abnormalities. Other perinatal complications did not significantly influence screening results indicating improved perinatal handling in a neonatal population at risk for hearing disorders.

Erythropoietin gene expression in different areas of the developing human central nervous system.

UNLABELLED: Evidence from cell culture and animal experiments suggests a neuroprotective and neurotrophic function of erythropoietin (EPO). We have quantitated the distribution of EPO mRNA expression in the developing human central nervous system (CNS). PATIENTS AND METHODS: Up to seven biopsies from different areas of the CNS of four preterm fetuses (gestational age 23-37 weeks) were obtained at routine postmortem examinations. EPO mRNA was quantitated by competitive PCR in samples from the CNS, the kidneys, and the liver where the EPO gene is predominantly expressed at this gestational age. RESULTS: EPO mRNA was most abundant in one sample from the cerebellum (0.29 amol/microg total RNA [amol=10(-18)mol]) and two from the pituitary gland (0.23 amol/microg total RNA), but levels varied considerably. EPO mRNA in the cortex cerebri (median 0.12 amol/microg total RNA; n=4) dominated over the expression in the corpora amygdala (median 0.05 amol/microg total RNA; n=4), the hippocampus (median 0.03 amol/microg total RNA; n=4), or the basal ganglia (median 0.01 amol/microg total RNA; n=3). Only little EPO mRNA (<0.01 and 0.06 amol/microg total RNA) was found in the spinal cord. EPO mRNA levels in the cerebellum, pituitary gland, or the cerebral cortex were within the same range as in the liver (0.03-1.67 amol/microg total RNA; n=4), or the kidneys (0.06-0.79 amol/microg total RNA; n=4). CONCLUSION: We found the EPO gene expressed throughout the fetal human CNS. Our data provide the basis to discuss a function for EPO in the brain of humans as well.

Neurodevelopmental risks in twin-to-twin transfusion syndrome: preliminary findings.

The purpose of this study was to determine the neurodevelopmental risks in patients with twin-to-twin transfusion syndrome, a rare but serious complication of monochorionic twin gestations. From a total sample of 94 twins with twin-to-twin transfusion syndrome, admitted during 1989 and 1993, 49 patients survived and 40 patients were followed to a mean age of 24 months. Neurological status and psychomotor development (Denver and Griffiths Developmental Tests) were determined. Parameters of the neonatal period were evaluated for their potential prediction. Of the 40 tested patients 18 showed a normal psychomotor development. Thirteen patients exibited a specific delay in language development and/or showed minor neurological dysfunctions. Nine twins had severe psychomotor retardation in combination with cerebral palsy. Major neurological sequelae were found, more common in recipients than in donors (6/19 vs 3/21). Correspondingly, neonatal ultrasound showed more pathological results (especially periventricular leucomalacia) in recipients. Neither anaemia nor polycythaemia at birth can predict developmental outcome. Apart from a high prenatal mortality rate, both twins, donators as well as recipients, are highly at risk for brain damage of different aetiology, associated with abnormal neonatal cerebral ultrasound.

Aerosolised prostacyclin for pulmonary hypertension in neonates.

Aerosolised prostacyclin (PGI2) was administered to two neonates with pulmonary hypertension to dilate the pulmonary vessels selectively. The alveolar-arterial oxygen gradient fell from 73.2 (patient 1) and 71.8 kPa (patient 2) to 33.8 and 26.7 kPa respectively. Systemic blood pressure remained stable while pulmonary blood pressure declined in patient 1. When inhaled, PGI2 seems to improve oxygenation, mainly due to reduction of intrapulmonary shunting.

Serum lipids during parenteral nutrition with a 10% lipid emulsion with reduced phopholipid emulsifier content in premature infants.

Fourteen premature infants (range 26 + 0 to 32 + 3), all but two appropriate for gestational age with a mean body weight of 1196 g (range 860 to 2770 g) received a 10% lipid emulsion. This lipid emulsion contained half of the formerly used phospholipid emulsifier concentration reducing the phospholipid/triglyceride ratio to the ratio used for the 20% lipid emulsion (0.06 instead of 0.12). Lipid emulsion was given over a 10 day period commencing at the third day of life with 0.5 g/kg/24 h which was increased daily up to a dose of 2.0-2.5 g/kg/24 h which was reached in all patients at the seventh day of the observation period. During this time mean serum concentrations of cholesterol increased non-significantly from 76.1 mg/dl (SD 33.7) before lipid emulsion to 86.1 mg/dl (SD 36.4) on day seven of the observation period. 13 of the 14 patients (97%) showed no pathological increase of their serum triglyceride concentration during lipid infusion. Mean serum triglyceride concentration increased from 65.3 mg/dl (SD 32.0 mg/dl) before the start of lipid emulsion to 102.6 mg/dl (SD 76.5) on day four (p < 0.05) but with no further significant increase. Lipid emulsions with 10% triglyceride but lower phospholipid content are tolerated without pathological increase in triglyceride or cholesterol serum concentration in the vast majority of premature newborns.

Color Doppler echocardiographic evaluation of tricuspid regurgitation and systolic pulmonary artery pressure in the full-term and preterm newborn.

Color Doppler echocardiography of tricuspid valve regurgitation (TR) is a valid, noninvasive method of determining systolic pulmonary artery pressure (SPAP). In a prospective study the authors examined 56 healthy full-term newborns (group I), 36 healthy preterm newborns (group II), and 10 preterm newborns with severe respiratory distress syndrome requiring surfactant replacement therapy (group III). Doppler studies were repeated until the transtricuspid gradient was < 20 mm Hg. In 83.3% of children a reproducible spectral curve was recorded at least once. The authors estimated the transtricuspid gradient delta p (RV-RA) by using the modified Bernoulli equation. Within the first twenty-four hours delta p (RV-RA) was < 20 mm Hg in 72.7%, 50%, and 25% of children with measurable TR in groups I, II, and III, respectively, increasing to 91.1%, 78.6%, and 55.6% within forty-eight hours. There was no significant correlation between SPAP and gestational age, birth weight, mode of delivery, and ductal closing time. Continuous holosystolic envelope tracing of TR was recorded in 16.6%. In these patients delta p (RV-RA) was measured markedly higher (mean of 30.1 mm Hg) than in the others (mean 17.3 mm Hg). The authors conclude that there is a high prevalence of TR in neonates, which allows estimation of SPAP in > 80% of newborns without considerable impairment. Normalization of SPAP takes place within four days in most patients, but there is a delay in preterm infants with severe respiratory distress syndrome.

[Near-infrared spectroscopy--a new method for non-invasive monitoring of cerebral hemodynamics]. / Nahinfrarotspektroskopie--eine neue Methode zum nicht invasiven Monitoring zerebraler Haemodynamik.

Non-invasive techniques allow monitoring in prematures and newborns with the maxime of "minimal handling". Cerebral changes in oxi- and deoxihemoglobin and in total hemoglobin, which reflects the intracranial blood volume, can be monitored continuously by near infrared spectroscopy (NIRS) for the first time. As derived parameter the cerebral blood flow can be determined. With reservations the method allows to get a view of the cellular level by recorded changes of the cytochromeoxidase. This article gives a survey of the physics, the technique and shows the clinical application of the method.

Erythropoietin concentrations and erythropoiesis in newborns suffering from renal agenesis and congenital kidney diseases.

UNLABELLED: We studied serum concentrations of erythropoietin (EPO) in the cord blood of 31 newborns. In patients with renal agenesis (n = 6), the EPO levels were 68.2 (23-177) mU/ml (median, range). These values are clearly above EPO levels in the reference groups (median/range: < 30 weeks 11.0 (5.5-17.5) mU/ml; 30-32 weeks 18.1 (5.5-136) mU/ml; 33-34 weeks 17.7 (8.3-423) mU/ml; 35-37 weeks 17.3 (5.5-272) mU/ml; > or = 38 weeks 17.8 (8.7-40.3) mU/ml). Neonates with polycystic kidney diseases (n = 12, EPO 23.5 (9.7-491) mU/ml) and with severe bilateral hydronephrosis due to obstructive uropathy (n = 13, 18.6 (7.5-30.7) mU/ml) showed no difference to the reference groups. In all groups there were only slight differences in haemoglobin/haematocrit values. CONCLUSION: In spite of renal agenesis and severe congenital kidney diseases, erythropoiesis is sufficiently maintained during fetal life. The liver of congenitally kidney-damaged fetuses is sufficiently able to compensate the reduction in--or lack of--renal EPO production.

[X-chromosomal recessive hydrocephalus internus: a separate disease picture? 2 further case reports and review of the literature]. / X-chromosomaler rezessiver Hydrocephalus internus: Ein eigenständiges Krankheitsbild? 2 weitere Fallberichte und Literaturübersicht.

Two patients with X-chromosomal hydrocephalus internus (aqueduct stenosis, clasped thumbs, mental retardation and spasticity) habe been described. In both cases the family history revealed further affected relatives. The intra- and interfamilial variability of this rare X-chromosomal recessive disease will be demonstrated. In this context differentialdiagnoses like X-linked MASA syndrome and X-linked spastic paraplegia have been discussed on the basis of a variable expressivity of different mutations in the same gene.

[Intracranial hemodynamics in phenobarbital administration]. / Intrakranielle Hämodynamik unter Phenobarbitalmedikation.

The effect of phenobarbital application on cerebral hemodynamics was studied in premature lambs. Near infrared spectroscopy was used for noninvasive monitoring of oxyhemoglobin, deoxyhemoglobin, total hemoglobin and cytochrom a/a3. Total intracranial blood volume decreased shortly after giving the drug and remained then on a stable lowered niveau. This could be explained with shifting of blood in different brain regions or with a decreased total intracranial blood volume.

Longitudinal data for intrauterine levels of fetal IGF-I and IGF-II.

OBJECTIVE: An increasing body of evidence supports a major role for the insulin-like growth factors (IGFs) in the control of human fetal growth. Individual data at various times of pregnancy suggest that IGF-I and IGF-II levels remain stable up to the 33rd week of pregnancy. Thereafter, both increase to reach values 2-3 times higher at term. In order to provide an accurate reflection of fetal IGFs in utero, we sampled fetal blood from the umbilical cord by cordocentesis. METHODS: We measured IGF-I and IGF-II in 12 fetuses longitudinally for up to 5 times between the 21st week of gestation and delivery. RESULTS: All patients showed a progressive increase in IGF-I and IGF-II levels. Data determined during different time intervals (before 29th, 29th to 32nd, after 32nd week) were compared and the main increase was found after the 32nd week. The median for IGF-I before the 29th week was 33.5 ng/ml (range 19-40.5) and increased to 41 ng/ml (32-59) between the 29th to 32nd and further to 54.1 ng/ml (range 17-70) thereafter. During the same time interval, the median for IGF-II increased from 217 ng/ml (86-326) to 349 ng/ml (227-467). In 7 patients, cord blood after delivery was available. For IGF-II a further increase was consistently found after birth (from 282 ng/ml (175-511) to 393 ng/ml (297-513)), whereas only 2 fetuses showed an increase in IGF-I. CONCLUSION: We conclude that in human fetuses, IGF-I and IGF-II levels increase longitudinally throughout pregnancy. Therefore, they may become important markers of healthy fetal development.

Biochemical monitoring of fetal distress with serum-immunoreactive erythropoietin.

The ability of parameters like umbilical arterial pH and Apgar score to predict and/or to reflect fetal distress are limited. It is known that erythropoietin (EPO) increases when partial pressure of oxygen is insufficient for metabolic demand. Therefore we studied the levels of EPO in the cord blood of stressed neonates (n = 75). In addition, reference values for EPO were established in a group of healthy term infants (n = 54) (mean +/- SD: 20.02 +/- [mU/ml]) and in premature infants (n = 77) according to gestational age. A significant increase in EPO concentrations was found in the stressed group: 153.4 +/- 418.8 [mU/ml], p < 0.003 (n = 27) in acute stress; and 102.6 +/- 127.1 [mU/ml], p < 0.002 (n = 48) in chronic stress. However parameters like hemoglobin, hematocrit, umbilical arterial pH and Apgar-score did not correlate with EPO values. A sensitivity of 59% and a specificity of 92% was calculated. We conclude that serum EPO concentrations are capable of detecting acute and chronic stress and could be useful as a screening method. In part EPO concentrations also allow us to grade stress in pregnancies that are complicated by diseases like preeclampsia.

Fentanyl-induced chest wall rigidity and laryngospasm in preterm and term infants.

OBJECTIVE: To assess the occurrence of muscle rigidity after fentanyl administration in premature and term neonates. DESIGN: Prospective case series, observational study. SETTING: A university hospital neonatal intensive care unit. PATIENTS: 8/89 preterm and term infants (25-40 wks gestational age) who received fentanyl for perioperative analgesia and sedation or intensive care procedures. INTERVENTIONS: Mechanical or bag mask ventilation and antagonization with naloxone. MEASUREMENTS AND MAIN RESULTS: We observed chest wall rigidity in 8 patients after low dosage of fentanyl (3-5 microg/kg body weight). All patients presented with respiratory distress, hypercapnia, and hypoxemia leading to bradycardia. In two patients, laryngospasm was noted and associated with muscle rigidity, thus making intubation impossible. Naloxone (20-40 microg/kg body weight) reversed the laryngospasm and muscle rigidity immediately, allowing restitution within 1 min. In our patient population, we found fentanyl-induced chest wall rigidity in 4% of neonates after fentanyl administration. CONCLUSION: Even low doses of fentanyl can lead to thoracic rigidity in neonates. Additionally, we observed laryngospasm in two patients and speculate that it might be a variant of muscle rigidity.

Good prognosis for psychomotor development in survivors with nonimmune hydrops fetalis.

We examined the psychomotor development of 33 of 61 surviving children, from a series of 107 consecutive live-born cases with nonimmune hydrops fetalis. The majority had a normal outcome. Three had a (simultaneous) serious underlying disease (e.g. fetal herpes infection) and had either severe psychomotor retardation or blindness. Two showed clumsiness and were considered to have minor neurological dysfunction. We conclude that survivors, especially those with transient benign intrauterine conditions, such as lymphatic aetiology have no additional risk to their psychomotor development.

Relative changes in oxyhemoglobin, deoxyhemoglobin and intracranial blood volume during surfactant replacement therapy in infants with respiratory distress syndrome.

Oxy-, deoxyhemoglobin and total blood volume were studied by near-infrared spectroscopy (NIRS) during surfactant replacement therapy. These parameters were compared with parameters watched during conventional noninvasive monitoring (pulseoximetry, transcutaneous pO2 and pCO2, heart rate). Seven premature infants (28 +/- 3 weeks of gestation, 940 g birth weight) were given surfactant intratracheally. Immediately after surfactant administration, oxyhemoglobin decreased, deoxyhemoglobin and total blood volume increased. The prior status was re-established after 60-220 s and then oxyhemoglobin increased to a very stable maximum. NIRS allows continuous bedside noninvasive monitoring of all parameters.

[Organoid nevus syndrome (Schimmelpenning-Feuerstein-Mims syndrome): case report and literature]. / Organoides Naevussyndrom (Schimmelpenning-Feuerstein-Mims-Syndrom): Kasuistik und Literatur.

The main symptom of the organoid nevus syndrome (Schimmelpenning-Feuerstein-Mims-Syndrom) is the nevus sebaceous, which is mostly linear and can be of variable expression. Malformations of the skeletal system and the eyes are usually associated, while malformations of the cardio-vascular system are less common. Neurological findings such as mental retardation and seizures are of clinical relevance. We describe a case and discuss this rare syndrome.

[Nonimmunologic hydrops fetalis--a review of 31 cases]. / Nicht-immunologischer Hydrops Fetalis--eine Ubersicht über 31 Fälle.

Non-immunologic hydrops fetalis-a review of 31 cases: 31 Patients with non-immunologic hydrops fetalis (NIHF) seen between 1984 and 1987 are described. 13 infants survived. The infants with major congenital malformations and connatal infections died. In 8 of the patients who died a cause for NIHF could not be identified. 10 of the survivors presented chylous ascites and/or chylothorax without major congenital anomalies. 2 infants had fetal tachyarrhythmia and 1 patient showed severe anemia due to fetomaternal hemorrhage.