Biomedical subjects
Publications and source records attributed to K H Deeg.
Great sacral teratomas are rare congenital tumors. The frequency in liveborns is about 1:40,000. Well known complications of sacral teratomas are malignant degeneration, infection and, in case of intraabdominal expansion, compression of the urogenital tract. We report a case of unusual fulminant perinatal bleeding out of a great sacral teratoma ruptured during birth.
Pathologic flow profiles in renal arteries can be found in cardiovascular and renovascular disease. Obstructions of the left hart (aortic stenosis, hypoplastic left heart syndrome, coarctation of the aorta) cause diminished flow with low peak systolic velocities. In renal arteries of infants with leakage of the aortic "Windkessel" (persistent ductus arteriosus, truncus arteriosus communis etc.) a low diastolic amplitude can be found. Significant left-to-right shunts can lead to a diminished or even retrograde diastolic flow. In renal artery stenosis a high velocity jet with spectral broadening can be found in the region of the stenosis. Distal to the stenosis low blood flow velocities can be shown. In renal vein thrombosis the obstruction of the peripheral vessels cause a missing or even negative diastolic flow in the renal arteries, whereas venous blood flow is diminished or even missing. Kidney diseases with swelling of the organ (acute rejection of a transplanted kidney) can cause diminished, missing or even retrograde diastolic flow in the renal arteries dependent on the severity of the edema. These alterations can be used for the early diagnosis of acute rejection which offers the opportunity for early and efficient therapeutic management.
Doppler sonography allows measurement of renal blood flow velocities non invasively. Combination of pulsed Doppler with gray scale imaging (Duplex-scan) yields precise measurement of the flow in defined vessels. By means of the Duplex technique not only the flow parameters in the renal artery and vein can be measured, but also in the segmental, interlobar and interlobular arteries and veins. Colour coded Doppler sonography allows simultaneous display of the flow within the vessels and the gray scale image. With the help of colour coded Doppler sonography the sample volume of the pulsed Doppler device can be placed precisely within the vessels, which makes possible the determination of absolute flow velocities. Besides the flow velocities other different indices have been defined in the literature. All indices result from comparison of systolic and diastolic flow amplitudes in different ways. The flow parameters in the renal vessels are dependent on the site of the flow measurement and the age of the children. When pathologic flow parameters are analyzed the vessel and the age of the child have to be mentioned always. The interpretation of pathologic flow profiles becomes easier by knowledge of normal values for the flow velocities and the different indices.
From 1968 to 1988, 47 children and adolescents, their ages ranged from a few days to 21 years, were treated for tumors of the ovary in the hospital for children and adolescents and the surgical department of the University of Erlangen-Nürnberg. In 33 cases ovarian cysts were found, 14 patients suffered from a neoplasm of the ovary. 8 of the neoplasms were benign cystic and solid teratomata, 4 were of epithelial origin and 2 identified as juvenile granulosa cell tumors. There was a follow up in all patients with tumors ranging up to 19 years.
Colour flow imaging was used for the visualization of the great intracranial arteries in 100 healthy infants. All children were investigated by computersonography with a 5 MHz linear transducer. Simultaneous imaging of brain tissue, displayed in gray scale and flow, displayed in colour was possible. Flow towards the transducer was displayed in red, flow away from the transducer in blud. The following arteries could be shown in sagittal and coronal sections: anterior cerebral artery, both internal carotid arteries, the basilar artery, both vertebral arteries and the posterior cerebral arteries. The middle cerebral artery could only be shown in axial sections through the temporal bone. With the help of colour flow imaging the exact course of the great intracranial arteries can be shown. Precise placement of the sample volume as well as angle correction is possible when absolute flow velocities are measured by pulsed dopplersonography. Congenital vascular abnormalities can be visualised within the image.
121 healthy premature born infants and full term newborns (corrected gestational age 29 to 45 weeks, weight at investigation 1070 to 3750 g) were investigated by pulsed Doppler sonography with a 5 MHz transducer. In all infants pulsed Doppler recordings were obtained from the internal carotid arteries (ICA), the basilar artery (BA) and both anterior cerebral arteries (ACA). From the flow profile the maximal systolic velocity (Vs), the endsystolic velocity (Ves) and the enddiastolic velocity (Ved), the time average velocity (TAV) and the time average maximal velocity (TAMX) as well as the resistance-index (RI) and the pulsatility-index (PI) were measured. For all parameters the relationship to the gestational age was analysed and normal values were established. There was a linear increase of all flow velocities with increasing gestational age. Vs in the ICA was about 20% higher than in the ACA and BA whereas Ves and Ved were not significantly different in the three arteries. The TAV in the ICA was 9% higher than in the ACA and 15% higher than in the BA. The TAMX in the ICA was 10% higher than in the ACA and 14% higher than in the BA. In contrast to the increase of the flow velocities neither the RI nor the PI showed a significant age dependency. For the RI in the ICA 0.77 +/- 0.08, in the ACA 0.73 +/- 0.08 and in the BA 0.72 +/- 0.09 were measured. The PI in the ICA was 3.0 +/- 0.08, in the ACA 2.7 +/- 0.09 and in the BA 2.7 +/- 0.7.(ABSTRACT TRUNCATED AT 250 WORDS)
In two newborns with severe coarctation of the aorta and interrupted aortic arch, subclavian-steal was shown by angiocardiography. In both children pulsed doppler recordings were obtained in the cerebral arteries: normal forward flow during systole and diastole could be shown in the anterior cerebral arteries, both internal carotid arteries, the basilar artery and the right vertebral artery. In the left vertebral artery in both infants a negative flow indicating backflow from the brain could be shown. Pulsed doppler sonography of the flow in the vertebral arteries is a non invasive method for diagnosis of subclavian steal in infants with coarctation of the aorta and interrupted aortic arch.
We present a model based on an electric analogon that describes the haemodynamic situation of an isolated ventricular septal defect. The model shows a way to estimate the ratio of pulmonary to systemic vascular resistance by Doppler sonographic measurements of pulmonary to systemic flow ratio (Qp/Qs) and the ratio between the size of the defect and the cross-sectional area of the aorta (Av/Aa). The ratio of pulmonary to systemic vascular resistance is theoretically given by: Rp/Rs = Qs/Qp + log (Av/Aa) * (1-Qs/Qp). Systemic to pulmonary flow ratio can be measured by Doppler echocardiography as well as Av/Aa; therefore, Rp/Rs can be estimated noninvasively by the equation given above. The values estimated noninvasively by Doppler echocardiography showed a good correlation with the haemodynamic data obtained at cardiac catheterisation.
After an uneventful pregnancy and birth at home without vitamin K-prophylaxis, a 13-day-old breast-fed newborn showed an unexplained swelling of his right elbow and avoided movement of his right arm. One week later a swelling of his left thigh and hip with extensive hematoma led to the diagnosis of vitamin K-deficiency. The initial symptoms can be interpreted as an unusual primary manifestation of late hemorrhagic disease caused by vitamin K-deficiency.
In two families radial hemimelia is inherited as a dominant trait. The first proposita suffered from bilateral radial aplasia, the 2nd propositus exhibited (pseudo)phocomelia. In this case the diagnosis was Holt-Oram-syndrome. The affected mothers showed unilateral hypoplasia of the thumb only. Cases like these ones are likely to be overlooked or misinterpreted. The recurrence risk is 50%. Similar observations are quoted. The cause of "variable expressivity" is unknown.
The alobar form of holoprosencephaly is characterised by a huge spherical ventricle localized in the midline. The monoventricle communicates with a large occipital cyst, the "dorsal sac". Both thalami and plexus chorioidei are fused in the midline. The interhemisperic fissure and the falx cerebri are absent as well as the corpus callosum and the septum pellucidum.
Rhabdomyomas are benign cardiac tumors often occurring as multiple lesions. We describe an infant with multiple cardiac rhabdomyomas causing severe right-ventricular and left-ventricular outflow tract obstructions. The intracavitary parts of the rhabdomyomas were successfully resected when the patient was 5 weeks old. Echocardiography showed regression in size of the remaining intramural tumors. Since there is only a low growth potential in cardiac rhabdomyomas, surgical management is indicated only in patients with hemodynamic obstruction or significant arrhythmias. Surgery should be primarily directed to relieve hemodynamic obstruction. If complete resection is not possible without damage to vital structures, resection should be restricted to the intracavitary parts of the tumor.
An adapted cow's milk formula with or without supplemental taurine (480 mumol/l) was fed for 16 weeks to 20 low-birth-weight infants. In the 2nd and 16th weeks of life, respectively, the following parameters were determined: growth, sonography of heart and brain, ECG, EEG, neurological development and the taurine concentration of plasma and urine. None of the parameters investigated was influenced by taurine supplementation except the urinary taurine excretion. At least according to these data, the addition of taurine to whey-predominant infant formulae seems to be unnecessary for the development of heart and brain function in low-birth-weight infants.
In 121 prematurely born infants, newborn and older infants (age 1-30 days, weight at investigation 1070-3750 g) flow velocities in the anterior cerebral artery, the internal carotid artery and the basilar artery were measured. All investigations were performed by computerised pulsed Doppler sonography (Acuson 128), with a 5 MHz transducer. Maximal systolic, endsystolic, and end diastolic flow velocities were measured as well as the pulsatility index and the resistance index. The flow velocities showed a strong positive linear correlation with age. The pulsatility indices were independent of age. For all flow parameters normal values were estimated.
In 150 girls from the newborn period up to the 18. year the size of the uterus was measured by sonography. In all children the length, width, depth and volume of the uterus as well as its shape and other signs of development were determined. In the newborn period and in the following first months of life an estrogen stimulated uterus with the shape of a drop could be shown. The size of the uterus decreased till the end of the first year. The length of the uterus in the neonatal period was 4.0 +/- 0.5 cm, the volume was 3.6 +/- 1.9 cm3. In the following rest phase up to the age of 8 years the length of the uterus was 2.8 +/- 0.4 cm and the volume was 1.2 +/- 0.4 cm3. The shape of the uterus in this age group was tubular. One to two years before secondary pubertal signs could be shown, sonography already demonstrated the beginning of pubertal uterus growth. In the postpubertal period the uterus had the typical pear shape with a corpus/cervix-relation of 2:1. In the postpubertal period the length was 6.8 +/- 1 cm with a volume of 33 +/- 22 cm3. The knowledge of the normal developmental dates of the uterus in children is essential for the diagnosis of malformations and tumours of the pelvis, but also for the diagnosis of distorted puberty such as pubertas praecox, pubertas tarda, amenorrhea and growth retardation.
In 121 healthy premature born infants, full-term newborns and older infants (gestational age: 29 to 45 weeks; weight at investigation: 1070 g to 3750 g), the flow velocities in the internal carotid arteries were measured by pulsed doppler sonography. All infants were investigated by computer sonography (Acuson 128) with a 5 MHz transducer. The following parameters were measured: The maximal systolic velocity, the end-systolic and end-diastolic velocity as well as the mean flow velocities, the pulsatility-index and the resistance-index. For all parameters the relationship to the gestational age and weight were analysed. All flow velocities increased linearly with increasing gestational age and weight. In contrast to the flow velocities neither the pulsatility-index nor the resistance-index changed with increasing gestational age and weight. As flow velocities in the internal carotid arteries increase with increasing gestational age and weight these parameters must be taken into consideration when pathologic flow velocities are analysed.
In 52 infants (weight: 3174 +/- 1165 g; gestational age: 41.3 +/- 6.5 weeks) with hydrocephalus pulsed doppler recordings were obtained in the anterior cerebral arteries. For comparison 52 healthy infants (weight: 3148 +/- 1118 g; gestational age: 40.6 +/- 5.7 weeks) were investigated. In all children the maximal systolic velocity, the end-systolic velocity, the end-diastolic velocity and the pulsatility-index were measured. In the healthy control group the maximal systolic velocity was 43 +/- 14 cm x s-1, the end-systolic velocity 20 +/- 8 cm x s-1, the end-diastolic velocity 11 +/- 5 cm x s-1 and the pulsatility index was 0.75 +/- 0.10. All 9 children with minimal ventricular dilation without progression showed normal flow profiles with normal flow velocities and pulsatility-index in the anterior cerebral arteries. 17 infants with moderate, slowly progressive ventricular enlargement showed significant increase of the maximal systolic velocity (60 +/- 27 cm x s-1) and the pulsatility-index PI (0.82 +/- 0.14). There was no difference in the end-systolic and end-diastolic velocities to the healthy control group. 26 children with marked and rapid progressive hydrocephalus showed significant decrease of the end-systolic and end-diastolic velocities and an increase in the pulsatility-index. The end-systolic velocity was 15 +/- 7 cm x s-1, the end-diastolic velocity was 4 +/- 7 cm x s-1 and the pulsatility-index measured 0.91 +/- 0.18. There was no difference in the maximal systolic velocity which measured 41 +/- 17 cm x s-1. All children with increased intracranial pressure showed a pathological flow profile with a decrease of diastolic forward flow. Absent or retrograde diastolic flow in rapid progressive hydrocephalus may lead to a decrease of brain perfusion resulting in hypoxemic ischemic brain lesions. After implantation of a ventriculo-atrial shunt an increase in the end-systolic and end-diastolic velocities and a decrease of the pulsatility-index could be shown. Shunt insufficiency can be shown early by a decrease in diastolic forward flow.
Intra- and periventricular haemorrhage (IVH/PVH) and, under certain conditions, the respiratory distress syndrome (RDS) seem to be typical sequelae of perinatal asphyxia in preterm born infants. Therefore, an association of IVH/PVH and RDS can be expected. We have retrospectively analyzed the data of 118 premature infants born between 1982 and 1986, weighing between 750 and 1499 g. 11 of these had experienced a severe IVH/PVH and a severe RDS at the same time, whereas 75 infants did not develop either of those. (2 of the 118 showed a severe IVH/PVH without evidence of severe RDS whereas 29 developed severe RDS without signs of serious IVH/PVH. 1 could not be evaluated due to missing data). This association of severe intracerebral haemorrhage and severe respiratory distress syndrome was statistically significant (p less than 0.005). The number of severe IVH/PVH has decreased during 1984-1986 in comparison to 1982/83 (4/76 vs. 9/42; p less than 0.05); the incidence of severe RDS has slightly declined. Comparing the perinatal conditions we found that the infants of the years 1984-1986 were more rarely delivered after an interval exceeding 24 h after premature rupture of the membranes (p less than 0.05), were more often delivered by caesarean section (p less than 0.005), and were nearly always primarily cared for by an experienced paediatrician (p less than 0.01). There were no significant differences between these two groups as far as dexamethasone-prophylaxis, mean birth weight, percentage of small-for-gestational-age infants and mean Apgar scores were concerned.(ABSTRACT TRUNCATED AT 250 WORDS)