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Biomedical subjects

H Niederhoff

Publications and source records attributed to H Niederhoff.

At least 37 records · Page 2Linked to original sources
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↗
PubMed1979

A case of biotin-responsive 3-methylcrotonylglycin- and 3-hydroxyisovaleric aciduria.

During selective screening for organic acidurias, a 10-week-old girl with muscular hypotonia and recurrent fits was shown to be excreting 3-methylcrotonylglycin and 3-hydroxyisovaleric acid. Besides these metabolites of leucine the presence of small but pathological amounts of propionic and methylcitric acids were demonstrable in her urine, pointing to a defect in the metabolism of biotin. On treatment with biotin (2 x 5 mg/day) the convulsions stopped at once, her clinical condition improved gradually, and the abnormal metabolites disappeared from the urine. Within 6 weeks the child was discharged in a good general condition without apparent signs of neurological damage.

Amino Acid Metabolism, Inborn Errors↗
PubMed1979

[Organization and performance of a selective screening programm for organic acidurias (author's transl)].

After definition and a short description of organic acidurias, the benefits of a selective screening for this group of diseases are demonstrated. In all children, being suspected to suffer from an inborn error of metabolism and giving rise to investigate the serum amino acids, the urinary organic acids should be analyzed additionally. A more detailled list of indications is given. Some specialized laboratories performed the necessary investigations routinely are announced.

Germany, West↗
PubMed1979

[Isovaleric acidemia combined with hypertrophic pylorstenosis (author's transl)].

A case of isovaleric acidemia combined with hypertrophic pylorostenosis is described. The diagnostic procedure, the biochemical findings as well as the performed therapy are discussed. Additionally the patient was successfully operated upon a pyloromyotomy. Thus the patient suffered form two conditions which are always to be considered in terms of differential diagnosis.

Amino Acid Metabolism, Inborn Errors↗
PubMed1978

Excretion of 2-methyl-3-oxovaleric acid in propionic acidemia.

A new case of propionic acidemia is presented, paying special attention to the early symptoms of this disease, such as increased drowsiness, muscular hypotonia, poor feeding, hypothermia, metabolic acidosis, ketonuria and vomiting. Investigation by gas chromatography (GC) and gas chromatography-mass spectrometry (GC-MS) revealed the excretion of fairly high amounts of 2-methyl-3-oxovaleric acid, a condensation product of two molecules of propionyl-CoA, as well as the known pathological metabolites such as propionic, 3-hydroxypropionic and methylcitric acids. Among the post mortem findings the histological studies of the liver were the most remarkable.

Acidosis↗
PubMed1977

[Intravascular coagulation in anorexia nervosa (author's transl)].

Hypercoagulation and intravascular coagulation developed a nearly 12-year-old girl with a one-year history of typical anorexia. Because of extreme cachexia she had been treated with numerous drugs elsewhere, among them ACTH an infusions of amino-acids. At the end of the second week of hospitalisation acute venous thrombosis of the right leg developed which was treated with heparin. Severe disseminated intravascular coagulopathy and thrombosis of the right leg were diagnosed on admission, the previously administered heparin was neutralised and streptokinase given for 60 hours, heparin was then given for several days, and the cachexia then treated. Both the local and general condition of the patient having been cured, the emotional state has also very much improved during the last year.

Anorexia Nervosa↗
PubMed1977

[Injury caused by glass splinters of the esophagus with smooth passage of the foreign body per vias naturales (author's transl)].

Case report on a 14 months old infant who swallowed a glas splinter while being fed from a jar with commercially prepared strained baby food (fruits). A perforating laceration in the upper posterior wall of the esophagus gave rise to a purulent inflammation of the paraesophageal tissue within 24 hours requiring incision and drainage. The suspected splinter was not found, neither by two esophagoscopies nor by the incision. Therefore the stools were collected for six days and carefully screened with a special technique. Thus a tiny curved glas splinter was detected fitting exactly into a corresponding defect of the upper inner rim of the used jar.

Esophagus↗