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Biomedical subjects

H Gnehm

Publications and source records attributed to H Gnehm.

4 recordsLinked to original sources
PubMed1989

[Congenital methemoglobinemia with cytochrome-b5-reductase deficiency: 4th Swiss family].

In a female newborn presenting with pronounced cyanosis in the absence of cardiopulmonary disease, the cyanosis was due to methemoglobinemia of 33% at birth and 17% at 24 hours (upper limit 0.5%) which was found to be secondary to deficiency of red blood cell cytochrome b5 reductase (EC 1.6.2.2.). Only residual activity of this enzyme was measurable, thus indicating homozygosity. Both parents were found to be heterozygous for this inherited disease. Of the six sisters and brothers of the newborn's father, five were investigated and all found to be heterozygous for the defective allele. Measurement of cytochrome b5 reductase showed both soluble and membrane bound fractions to be affected equally in the red cells of the baby's heterozygous parents.

Adult↗
PubMed1984

[Neonatal Streptococcus group B sepsis: problems of early diagnosis, therapy and prevention].

38 cases of neonatal group B streptococcal (GBS) sepsis (31 with early onset and 7 with late onset) were observed during the years 1976-1982. Early onset disease showed the following clinical characteristics: 1. frequent lack of risk factors for infection in obstetric history, 2. very early onset of symptoms of respiratory distress, 3. rapid development of shock after only minor or missing clinical signs of infection, and 4. unspecific findings on chest radiography. Neutropenia or marked leukocyte left shift as well as the presence of gram-positive cocci in gastric or tracheal aspirate proved to be useful diagnostic clues. The latex agglutination test for the detection of GBS-antigen in urine yielded in our hands many false positive results and unspecific reactions. Given the big difficulties of early diagnosis of early onset GBS-sepsis, the relatively liberal use of antibiotics in newborns with respiratory distress is probably unavoidable. Hereby prior culturing of blood and early termination of antibiotic therapy with negative cultures (of blood, CSF, tracheal aspirate) seems essential to us. The clinical significance of newer therapeutic (granulocyte transfusions, exchange transfusions, immunoglobulins) and prophylactic (intra-partum antibiotics, vaccination) modalities is according to the current literature still not clear in many respects.

Antigens, Bacterial↗
PubMed1976

Quantification of bone mineralization using computed tomography.

Computed tomography was used to find a sensitive parameter for bone mineralization. A precision scanning instrument was constructed for determination of the mineral distribution in sections of the forearm. The quality of the reconstructed images allows separate quantification of compact and spongy bone even when gamma rays are used. Computer simulation and measured of models and macerated human bones showed that under clinical conditions it is possible to quantify spongy bone density within an accuracy of +/-2%.

Adolescent↗