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P Hartlage

Publications and source records attributed to P Hartlage.

6 recordsLinked to original sources
PubMed1987

Peripheral neuronal changes in growth-retarded neonates: an ultrastructural study.

To study the effect of intrauterine growth retardation (IUGR) on fetal peripheral neuronal elements, we examined preputial skin tissues of ten growth-retarded neonates (mean gestational age 36 +/- 2.3 weeks) and six normal control neonates (mean gestational age 35 +/- 3.0 weeks) by transmission electron microscopy. The myelinated and unmyelinated nerve fibers of growth-retarded neonates contained significant ultrastructural alterations, consisting of 1) local aggregation of mitochondria, 2) loss of mitochondrial cristae, 3) the presence of large vacuoles within the axoplasm, and 4) myelinated fiber degeneration. The Schwann cells of these neonates exhibited accumulation of glycogen, disruption of cytoplasm, dilatation of rough endoplasmic reticulum, and extensive development of polyribosomes. None of the above changes were observed in the control infants, those appropriate for gestational age. We conclude that IUGR may cause significant ultrastructural changes in developing peripheral neuronal tissue.

Adult↗
PubMed1986

Mitochondrial acetoacetyl-CoA thiolase deficiency.

A patient with severe progressive neuropathy and growth retardation who showed a persistent ketosis despite normal blood glucose levels is described. A liver biopsy was analyzed for 3-oxoacyl-CoA thiolase activity. One of the mitochondrial 3-oxoacyl-CoA thiolases which in normal control liver could be activated by K+ was virtually absent in the patient's liver. An intensive search for 3-methylhydroxybutyric acid and 3-methylacetoacetic acid by gas chromatography/mass spectroscopy in the patient's urine failed to show the presence of these acids, demonstrating that the 3-methylacetoacetyl-CoA thiolase is functioning in this patient. It is therefore concluded that the persistent ketosis is due to a deficiency of the mitochondrial acetoacetyl-CoA specific thiolase.

Acetyl-CoA C-Acyltransferase↗
PubMedDate not supplied

Myelography of sacral agenesis.

In the past, neurologic deficits found in association with sacral agenesis were thought to be unamenable to surgical therapy. Recent experience and a careful review of autopsy and case reports form the literature have demonstrated that this assumption is unwarranted. Four cases of sacral agenesis are reported with description of the myelographic findings of each case. Surgical confirmation was obtained in three of these patients. Dural sac stenosis treated with duraplasty resulted in striking improvement in the neurologic status of two patients, while in the third, a 2-month-old infant, adhesive arachnoidal bands in the distal thecal sac were found at surgery and a taut and thickened filum terminale was transected. The fourth patient has a low-lying spinal cord and a posterior meningocele. The myelographic findings appear to be divisible into two categories. One group of patients may have high termination of the subarachnoid space with a dural sac stenosis and will benefit from duraplasty, while in the other, findings may include a widened or normal subarachnoid space and low-lying tethered spinal cord. It is emphasized that treatment of dural sac stenosis, tethered cord, and intrathecal or extrathecal masses that occur in some of these patients may afford significant improvement in their neurologic condition. These children deserve careful baseline neurologic evaluation and follow-up and a more aggressive approach toward adequate myelographic assessment.

Child↗