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Biomedical subjects

H Niederhoff

Publications and source records attributed to H Niederhoff.

At least 19 recordsLinked to original sources
PubMed1990

Origin of the left coronary artery from the right pulmonary artery and ventricular septal defect in a child of a mother with raised plasma phenylalanine concentrations throughout pregnancy.

A child with anomalous origin of the left coronary artery from the right pulmonary artery, ventricular septal defect, fetal growth retardation, and facial abnormalities was born to a woman in whom plasma phenylalanine concentrations had been raised throughout pregnancy. The cardiac abnormalities were diagnosed by angiography when the child was eight months old. The anomalous coronary artery was imaged in a subsequent echocardiogram. Development retardation was caused by maternal phenylketonuria, which may also have been responsible for the development of the ventricular septal defect and the coronary anomaly. If dietary treatment of the mother had been started before pregnancy damage to the child might have been prevented.

Abnormalities, Multiple↗
PubMed1987

[Embryofetopathy caused by postnatally detected maternal phenylketonuria].

A case of a now 10-month-old female infant is reported, who presented at birth with microcephalus, growth retardation, dystrophia, facial dysplasia and cardiac defect. Etiologically a classical phenylketonuria of the mother with very high levels of serum phenylalanine (51 and 41 mg/dl, respectively), which was not known until then, was diagnosed already after her confinement. The mother, aged 26, originates from Roumania. She had never been treated by any phenylalanine-limited diet. Psychological testing revealed a severely reduced intelligence (IQ = 63). The child, having normal levels of serum phenylalanine, presented with mild statomotor retardation at the age of ten months. Even in countries with a general neonatal screening program, a hitherto undiagnosed maternal phenylketonuria has to be considered within the differential diagnosis of a dystrophic microcephalic newborn, beside more common causes like the fetal alcohol syndrome.

Adult↗
PubMed1985

[Need for the determination of chloramphenicol levels in the treatment of bacterial-purulent meningitis with chloramphenicol succinate in infants and small children].

17 cases of purulent meningitis in 15 children, aged 1 day to 5 years (median 8 months) were treated with continuous i.v. infusion of chloramphenicol succinate. Free chloramphenicol in serum and cerebrospinal fluid (C.F.) was assayed by high performance liquid chromatography (HPLC). CF chloramphenicol levels averaged 45 +/- 14% of the serum level. Out of 16 patients only five received the usually recommended dosage. In three others because of initially or progressively high serum levels the dose had to be diminished. In eight others because of subtherapeutic levels the dose had to be raised. The highest dose (390 mg/kg body weight/d) was required in a 2 month old boy. He was shown to have a clearance rate for free chloramphenicol considerably higher than has been reported so far. Maturation of the metabolism could be observed in a small-for-date newborn who acquired a grey baby syndrome during the treatment of his first meningitis. Several weeks later he required exactly the recommended dose to reach therapeutic chloramphenicol levels. As a consequence of these observations we strongly recommend meticulous drug monitoring of chloramphenicol in order to meet the large biological variations seen particularly in neonates and young infants in their capacity to reach and maintain therapeutic serum levels.

Bacterial Infections↗
PubMed1985

[Physiology of hemostasis in newborn infants. I].

Ever since neonatal hemostasis has been studied systematically, a great number of single data und laboratory parameters have been collected which all differ more or less distinctly from results gained in adults. This has been leading repeatedly to the conclusion that the hemostasis in newborns is still immature or somehow insufficient. At least in healthy and term infants this does not apply; each pre- and postnatal stage of development has its own optimally functioning hemostasis changing with age due to respective physiological peculiarities which nevertheless may gain considerable pathogenetic significance for preterm or sick newborns. A good example are for instance the vascular structures of the periventricular germinal matrix in prematures born during the critical period around the 24th and 34th week of gestation. This assumption being in agreement with an important principle of developmental physiology is proven in this paper particularly for platelets and plasmatic coagulation.

Blood Coagulation↗
PubMed1985

[Pathology of hemostasis in newborn infants. II].

Already in newborns almost all congenital and acquired disorders of hemostasis can be encountered. Especially gestational age and developmental peculiarities of hemostasis influence the incidence of the different causes for hemorrhages. Due to laboratory progress the well-known question of vitamin K deficiency and related bleeding has again become a point of interest and can be answered more clearly now than some years ago. Other significant disturbances of neonatal hemostasis are disseminated intravascular coagulation, intracranial hemorrhages, and the thrombocytopenias of the newborn. Disseminated intravascular coagulation is a pathogenetically important and frequent complication of numerous diseases in term as well as particularly in preterm infants. Ultrasonography gave new information about frequency and prognosis of intracranial hemorrhages in affected newborns. Finally, qualitative and quantitative disorders of the platelets present always a true challenge for the neonatologist in terms of differential diagnosis and differential therapy.

Blood Coagulation Disorders↗
PubMed1984

Seven years of experience with selective screening for organic acidurias.

Between 1975 and 1981 nearly 9000 patients with suspected inherited metabolic diseases were investigated by a selective screening procedure including, apart from simple tests for ketone bodies, sugars and SH-containing compounds, high voltage electrophoresis of amino acids as well as gas liquid chromatography and gas liquid chromatography-mass spectrometry of the organic acids. Fifty-two cases with 18 different inborn errors of metabolism were detected. The effectivity index was calculated to be 0.6% or 1 case in about 170 requests. From the presented and from already existing data in the literature the overall incidences for all organic acidurias together and for propionic acidemia separately were appraised to be 1:10000 and 1:50000, respectively. About half of the patients diagnosed by this screening may benefit from the diagnosis.

Carboxylic Acids↗
PubMed1983

Analgesics during pregnancy.

The thalidomide tragedy of the late 1950s clearly proved the need for caution, and questionable drug use should always be avoided. The teratogenic potential of a drug is related to dosage and time of administration. During blastogenesis, fetal death may occur; during embryogenesis, deformity may develop; and during the last trimester, functional anomalies or "covert embryopathy" may be seen. Finally, the benefit to risk ratio of every drug must be carefully weighed, and only those with proved safety to the feto-maternal unit should be prescribed. Aspirin may be administered to the pregnant woman as an anti-inflammatory agent but in the lowest therapeutic dosage. In the later stages of pregnancy, however, aspirin should be avoided since it may prolong labor, lead to greater blood loss during delivery, and increase the incidence of stillbirths. The pyrazolones, although not associated with teratogenic side effects, may lead to sometimes fatal agranulocytosis and, accordingly, are not recommended in pregnancy. Acetaminophen is the analgesic and antipyretic of choice during all phases of pregnancy.

Acetaminophen↗
PubMed1983

[Vitamin K deficiency hemorrhages in 4 exclusively breast-fed infants 4 to 6 weeks of age].

Haemorrhages were observed in four wholly breastfed infants beyond the neonatal period. These infants were observed within a period of 8 weeks and showed the following characteristics: 1. Onset of bleedings was unexpected and without prior indication. 2. They were of a serious nature and involved the CNS in two children. 3. In all cases infants between 4 and 6 weeks of life were affected. 4. All infants had been wholly breastfed. 5. All were male. 6. There was a prompt improvement after administration of vitamin K or after blood or blood derivatives. Although preliminary own investigations do not indicate general lowering of vitamin-K-dependent coagulation factors in wholly breastfed infants in the postneonatal period, these 4 cases observed within a short time confirm the necessity to consider vitamin K deficiency in haemorrhages in infants in the postneonatal period. Diagnostic steps have to be initiated immediately.

Blood Transfusion↗
PubMed1982

[How to diagnose intracranial haemorrhages in infants by two-dimensional ultrasound scanning (author's transl)].

Applying a rotatory sector-scanner, in 437 infants between the 29th gestational week and 18th month of life, a sonographic study was performed in order to look for an intracranial haemorrhage. The recording was performed real-time, using the anterior fontanelle as an acoustic window. In 42 infants we saw signs of an intracranial haemorrhage, which was confirmed 11 times anatomically and 11 times by CAT. Advantages and disadvantages of the method are discussed.

Cerebral Hemorrhage↗
PubMed1982

[Doppler-sonography of cerebral blood-flow in infants].

Doppler-sonography of the intracranial arteries in the sagittal section through the open anterior fontanelle may yield valuable information on the cerebral blood-flow in infants. Applying the directional method, the anterior-cerebral artery could be recorded in all probands; by using the pulsed technique at least one artery was identified in 95% of the cases. There was a significant difference in pulsation-index (P.I.) between the pulsed and the directional method. Both methods revealed a reduction of the P.I. in infants with brain edema, and an increase of the P.I. after intracranial haemorrhage had occurred. The P.I. was significantly increased in infants younger than 36 gestational weeks, compared with healthy term newborns older than 40 weeks. The clinical relevance for diagnostic purposes as well as for therapeutic monitoring is discussed.

Brain Edema↗
PubMed1980

[Cholestatic icterus and "shock liver" resulting from disseminated intravascular coagulation in newborns and babies (author's transl)].

The simultaneous occurrence of severe bacterial, especially urinary tract infections and cholostatic icterus in newborn and young infants, is a wellknown phenomenon. Since the pathogenetic principle is unknown, such types of icterus are described as "idiopathic", "septic" or "septic-toxic". However, in recent years an increasing number of pointers seems to indicate that cholostatic jaundice, being a polyaetiological syndrome, can be closely linked in respect of time and cause, with disseminated intra vascular coagulation. It would suggest itself to assume that pathogenetically speaking a severe infection (or some other triggering cause) may lead to shock which, in turn, produces an intravascular consumption reaction, resulting in severe disturbances of microcirculation in the liver and hence in disordered liver function which is clinically manifest in the form of a cholastatic icterus. Among the patients treated at the Unversity Paediatric Hospital at Freiburg, a total of 31 mostly male children--30 babies and one schoolchild--was seen in whom this causal chain is highly likely.

Bacterial Infections↗
PubMed1979

[Henoch-Schoenlein purpura (author's transl)].

Routine EEG investigations and observance of discrete neurological and psychological symptoms in 13 children in the acute phase of Henoch-Schoenlein purpura showed that involvement of the central nervous system in this disease is the rule rather than the exception. Capillary resistance was reduced in 51 out of 76 investigated children. On the other hand a reduction in factor XIII activity was much less commonly found (n =6). Immune complex determination in 28 children, together with antibody studies, showed that in 13 of them the Henoch-Schoenlein purpura was triggered off by an influenza-A-virus infection of the upper respiratory tract. Two thirds of the patients had markedly raised levels of total serum complement which fell weeks within several.

Adolescent↗