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Biomedical subjects

H Niederhoff

Publications and source records attributed to H Niederhoff.

At least 55 records · Page 3Linked to original sources

Severe hemorrhagic varicella with visceral involvement: virological and serological studies during treatment with cytosine arabinoside.

A boy 13 year-old suffered an extremely severe and prolonged attack of hemorrhagic chickenpox with visceral involvement, the diagnosis being confirmed by isolation of varicella-zoster-virus (VZV). There was no other compromising disease. All preceding vaccinations including two against smallpox had been uneventful. The severity of the attack could not be ascribed to any persistent cellular or humoral immunodeficiency. The patient developed a good antibody response. The course of serological reactions to VZV infection was studied extensively using the different techniques of complement fixation and immunofluorescence for IgG, IgM, and IgA antibodies. Therapy was conducted cautiously using cytosine arabinoside (Ara-C) between the 10th and 17th day of disease; the temperature fell and VZV multiplication ceased, strongly suggesting a beneficial influence on the patient, who recovered completely.

Adolescent↗

Increased serum urate in galactosemia patients after a galactose load: a possible role of nucleotide deficiency in galactosemic liver injury.

Five galactosemic and 5 normal children received an oral load of galactose under standardized conditions. The maximal blood galactose level after 1.5 hours was 12.6 +/- 2.0 (S.D.) mmol/l in individuals with a deficiency of uridylyl transferase (EC2.7.7.12) as compared to 5.8 +/- 1.2 (S.D.) mmol/l in the controls. The concentration of serum urate in galactosemics increased to 155% of the fasting level (P less than 0.005); no rise was detectable in the controls. The elimination of urate with the urine was augmented by the same amount in both groups. Our studies provide evidence for an increased catabolism of hepatic nucleotides. This may lead to a deficiency of nucleotides which is proposed as a cause of galactosemic liver injury.

Blood Glucose↗

[Myelomonocytic leukemia with Philadelphia-positive and Philadelphia-negative cell lines in early childhood (author's transl)].

The authors report on an infant with myeloproliferative syndrome of the myelomonocytic type. The findings fulfilled the criteria for juvenile chronic myeloid leukemia except that there was no increase of fetal hemoglobulin and no depression of erythrocyte carbonic anhydrase I. In one half of the bone marrow cells a Ph1-chromosome was found. During the course of the disease the Ph1-positive clone was successively replaced by cell lines with different chromosomal aberrations. The relationship between the cytogenetic mosaicism and the clinical and laboratory findings is discussed.

Adult↗

[Cholestatic jaundice following disseminated intravascular coagulation ("shock-liver") (author's transl)].

Recently we gained increasing evidence of the fact that cholostatic syndromes in young infants are frequently associated with preceeding shock and disseminated intravascular coagulation. This is illustrated by the case of a 4 weeks-old male infant with urosepticemia due to E. coli, which was successfully treated with additional streptokinase. Therefore, in the age-group of early infancy we have to pay attention to the liver as a potential shock-organ similar to the well known shock organs such as kidneys, lungs, skin and brain.

Anti-Bacterial Agents↗

[A new in vivo method for the detection of carriers of haemophilia (author's transl)].

We examined 14 carriers of haemophilia A with the "Haemorrhagometer". The cold tolerance test (at 17 degrees C) revealed that 9 out of 10 proven and 3 out of 4 probable carriers had a pathologically prolonged bleeding time. Accordingly blood loss was pathologically elevated in 9 out of 10 proven and 2 out of 4 probable carriers. At room temperature the difference between normal control persons and carriers of haemophilia A was not as pronounced. 5 of 8 proven and 3 of 4 probable carriers showed pathologically elevated bleeding intensity, only 1 of 8 proven and none of 4 probable carriers showed abnormally prolonged bleeding time. Haemorrhagometry at 17 degrees C (cold tolerance test) may be helpful to ascertain whether or not a woman is a carrier of haemophilia A.

Blood Coagulation Tests↗