6533b85efe1ef96bd12bfef0
RESEARCH PRODUCT
Liver pathology in transient neonatal hyperammonemia.
Arthur ZimmermannClaude BachmannJ GehlerI HöggerA MielfriedH PetersE Mönchsubject
MalePathologymedicine.medical_specialtyHistologyMitochondria LiverBiologyMitochondrionOrganic aciduriaUltrastructural PathologyPathology and Forensic MedicineAmmoniaInternal medicinemedicineHumansMolecular BiologyStaining and LabelingMetabolic disorderHepatobiliary diseaseInfant NewbornHyperammonemiaCell BiologyGeneral Medicinemedicine.diseaseMicroscopy Electronmedicine.anatomical_structureEndocrinologyLiverUrea cycleHepatocyteAnatomyMetabolism Inborn Errorsdescription
Ultrastructural investigations have been performed on two cases of transient neonatal hyperammonaemia (TNH). This newly recognized metabolic disorder is chiefly characterized by severe hyperammonaemia in the postnatal period, a comatous state, absence of abnormal organic aciduria, normal activity of urea cycle enzymes and, usually, complete recovery. The aetiology is presently unknown. Electron microscopy uncovered rather congruent alterations of hepatocyte structure, with a wide spectrum of mitochondrial lesions, an increase of autophagous bodies with organelle remnants, and changes in the excretory apparatus. Thus, in contrast to some of the hereditary disorders of the urea cycle, no specific structural changes could be found in TNH. This finding is in line with the observation of normal activities of main urea enzymes in these cases, and indicates that a different biochemical system may be pathogenetically involved in TNH.
| year | journal | country | edition | language |
|---|---|---|---|---|
| 1983-01-01 | Virchows Archiv. A, Pathological anatomy and histopathology |