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Biomedical subjects

H Fahnenstich

Publications and source records attributed to H Fahnenstich.

At least 37 records · Page 2Linked to original sources

Measurements of sodium and potassium in newborns and prematures by ion-selective electrodes and flame photometry: influence of lipid and protein content of blood.

The alternative use of flame photometry and ion selective electrodes for electrolyte determinations in neonatology led to the question how the two methods agree. 104 routine blood samples were taken as matched pairs for a comparison of sodium and potassium determinations. Pre-analytical error was avoided by the mode of blood collection. The influence of the protein and lipid content of blood on the differences between the methods was determined by calculating its water content and making a post-correction of the flame values. Thus the differences for sodium could be reduced, whereas the differences for potassium slightly increased. As long as the imprecision of sodium determination is a meaningful methodological problem, the influence of the protein and lipid content of neonatal blood is not the main factor for differences between ISE and flame photometry.

Bilirubin↗

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.

Aerosols↗

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.

Blood Gas Monitoring, Transcutaneous↗

Animal experiments for the evaluation of laserspectroscopy in the fetus during labor.

Laserspectroscopy with near-infrared light is newly available for clinical application. The equipment consists of a near-infrared data collection unit (NIRDCU) and a personal computer. Emission of laser light at wavelengths of 775, 805, 845 and 904 nm is provided by the four laser diodes of the NIRDCU. By analyzing changes of optical density during laser radiation, information is obtained regarding the intracellular redox state and relative changes of blood volume. During measurement with the system on the fetal scalp during delivery we were able to get a first impression of the method's potential. While the application of the laser sensors is still cumbersome, signals providing information of the relative changes of hemoglobin concentration, relative changes of blood volume and cytochrome aa3 seems achievable. Laserspectrophotometric monitors may become safe, low cost and portable instruments for the non-invasive assessment of the biochemical and biophysical status in the fetus.

Animals↗

[Megacystis microcolon intestinal hypoperistalsis syndrome: A neuropathy?].

2 cases of megacystis microcolon intestinal hypoperistalsis are presented. A female newborn was capable of being fed completely enterally after three months. Laparotomy was not performed. A male newborn was subjected to laparotomy after 3 days and an ileal stoma was applied. The infant died after 6 months of complete parenteral feeding without any peristalsis having been initiated. Biopsies of the colon and small intestine of the patient showed normal HE staining findings. Histochemical examination revealed type B neuronal dysplasia with neuronal hypogenesis. The findings of 27 cases described in the literature are discussed with special reference to the histological findings of the intestinal wall.

Chronic Disease↗

[Maternal and fetal digoxin level in fetofetal transfusion syndrome (FFTS)].

BACKGROUND: Even though invasive intrauterine techniques for the treatment of TTTS such as punction of amniotic fluid and laser coagulation of placental vascular anastomoses are established methods in specialized centers, invasive methods are not always sufficiently successful. In conservative treatment of TTTS oral or intravenous maternal digoxin therapy in order to improve fetal cardiac insufficiency in combination with or after failure of invasive techniques is an useful method. PATIENTS AND METHODS: We investigated 12 TTTS pregnancies and 4 singleton pregnancies, which had been treated by maternal digoxin treatment for TTTS or arrhythmias, respectively. At birth, which was performed by means of caesarian section, venous cord blood samples of the newborns and venous maternal blood samples were collected, centrifugated and stored at minus 20 degrees C. Digoxin determinations were performed by radioimmunoassay. RESULTS: Fetal digoxin levels varied between 0.38 and 1.73 ng/ml, maternal levels ranged from 0.97 to 3.23 ng/ml. The fetomaternal digoxin gradient reached a mean of 0.56 (range 0.35 to 1.09). Donator and acceptor gradients were comparable and increased with birth weight or gestational week, respectively. CONCLUSIONS: In cases of pregnancies with TTTS a relatively high maternal digoxin level is necessary, especially during early gestational weeks, in order to reach therapeutical levels in the fetal circulation. Too low dosages might be responsible for unfavourable results in digoxin treatment of TTTS. Whether the maturation of placental villi during gestation could be the reason for increasing digoxin gradients requires further investigations.

Administration, Oral↗

[Prenatal diagnosis and management in VACTERL association].

In terms of the VACTERL-Association we are dealing with a non-random association of malformations following a defect during mesodermal development of embryogenesis due to a variety of causes. We report on three cases with VACTERL-type malformations diagnosed by prenatal ultrasound presenting cardial defects, renal abnormalities, single umbilical arteries and esophageal stenosis. We present sonographical, clinical and autopsy findings and discuss the pathogenesis of VACTERL-Association as a defect of mesenchymal development in early embryogenesis.

Abnormalities, Multiple↗

[Near-infrared spectroscopy--a new method for non-invasive monitoring of cerebral hemodynamics].

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.

Blood Volume↗

[Organoid nevus syndrome (Schimmelpenning-Feuerstein-Mims syndrome): case report and literature].

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.

Dysplastic Nevus Syndrome↗

[7-week-old infant with a hydrolethalus syndrome: case report, differential diagnosis and literature review].

We report on an 7 weeks old male infant with hydrocephalus internus, dysplastic low-set ears, micrognathia, vitium cordis, hypogenitalism, and polyhydramnios in pregnancy. The variability of the Hydrolethalus syndrome will be demonstrated. Our patient shows, that this syndrome is not a lethal in all cases. We report on an 7 weeks old male infant with hydrocephalus internus, dysplastic low-set ears, micrognathia, vitium cordis, hypogenitalism, and polyhydramnios in pregnancy.

Abnormalities, Multiple↗

[Familial non-immunologic hydrops fetalis].

The recurrent non-immunological hydrops fetalis is classified as a separate entity. It is usual to differentiate between the chylous and the idiopathic form. The case presented indicates that the recurrent non-immunological hydrops fetalis should not be seen as an idiopathic but as a chylous form. Only in enterally fed children the initially amber coloured pleural effusion becomes opaque and the triglycerides are then elevated. This points to a fault in the development of lymph vessels.

Blood Proteins↗

[Nonimmunologic hydrops fetalis--a review of 31 cases].

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.

Cesarean Section↗

[X-chromosomal recessive hydrocephalus internus: a separate disease picture? 2 further case reports and review of the literature].

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.

Abnormalities, Multiple↗

[Erythropoietin as a biochemical parameter for fetal hypoxia].

The ability of parameters like umbilical arterial pH and Apgar-score to predict and/or to reflect fetal distress is limited. It is known that erythropoietin (EPO) increases due to hypoxic stimulation. 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 +/- 6.4; median 17.8; range 8.7-40.3 (mU/ml]) and in premature infants (n = 77) according to gestational age (median/range: < 30 weeks 11.0, 5.5-17.5; 30-32 weeks 18.1, 5.5-136; 33-34 weeks 17.7, 8.3-422.9; 35-37 weeks 17.3, 5.5-272 [mU/ml]). EPO concentrations significantly increased in the stressed group: in acute stress (n = 27): mean 153.4, range 6.5-641.7 [mU/ml], p < 0.003; and in chronic stress (n = 48): mean 102.6, range 12.4-544 [mU/ml], p < 0.002. 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. In part EPO also allows to grade stress in pregnancies, which are complicated by diseases like preeclampsia.

Asphyxia Neonatorum↗

Nonimmune hydrops fetalis with galactosialidosis: consequences for family planning.

At the 28th week of gestation a hydrops fetalis was first detected by ultrasound. At birth a generalized hydrops with Hurler-like craniofacial dysmorphism, hepatosplenomegaly and a moderate dystostosis multiplex was noted. High urinary excretion of oligosaccharides and a severe deficiency of neuraminidase and of beta-galactosidase in cultured skin fibroblasts could be found. Thus, a rare early infantile type of galactosialidosis was diagnosed. The patient died at the age of 3 months because of cardiac failure. The consanguineous but otherwise healthy parents received genetic counselling for further pregnancies and have been informed about the possibility of prenatal diagnosis. In view of this possibility, the parents decided to have more children. In the second pregnancy a severe combined enzyme deficiency had been detected and the pregnancy interrupted. In the third pregnancy prenatal diagnosis revealed normal fetal enzyme activities. It resulted in a healthy female child and in the fourth pregnancy reduced but still in the heterozygote level enzyme activities had been found, a healthy boy was born.

Family Planning Services↗

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.

Cholesterol↗