Correlation between severity of clinical signs and transcranial magnetic motor evoked potentials in dogs with intervertebral disc herniation.

Transcranial magnetic motor evoked potentials (TMMEPs) can assess the functional integrity of the spinal cord descending motor pathways. In intervertebral disc herniation (IVDH), these pathways are compromised to varying degrees reflected by the severity of neurological deficits. The hypotheses of this study were as follows: (1) TMMEPs differ in dogs with IVDH and healthy control dogs; (2) TMMEPs reflect different severities of neurological signs; and (3) TMMEPs can document functional motor improvement and therefore monitor recovery of function. TMMEPs were recorded in 50 dogs with thoracolumbar IVDH. Clinical signs ranged from spinal hyperesthesia to non-ambulatory paraparesis in 19 dogs and paraplegia with/without deep pain sensation in 31 dogs. In these 31 paraplegic dogs, transcranial magnetic stimulation (TMS) was repeated during follow-up examinations. Ten healthy Beagle dogs served as controls. There was a significant increase in onset latency and decrease in peak-to-peak amplitude in the pelvic limb TMMEPs of dogs with spinal hyperesthesia to severe paraparesis compared to control dogs. Waveforms in dogs with IVDH were predominantly polyphasic in contrast to the biphasic waveforms of the control dogs. TMMEPs could not be generated in the pelvic limbs of paraplegic dogs. However, TMMEPs with markedly increased onset latencies and decreased peak-to-peak amplitudes reappeared in the pelvic limbs of dogs that were paraplegic before surgery and showed functional motor improvement during follow-up. The severity of neurological deficits was reflected by TMMEP findings, which could be used to document functional motor recovery in IVDH. TMS could therefore be used as an ancillary test to monitor response to therapy in dogs during rehabilitation.

Tetrasomy 9p mosaicism associated with a normal phenotype in two cases.

Tetrasomy 9p is a rare chromosomal syndrome and about 30% of known cases exhibit mosaicism. Approximately 50 of the reported cases with tetrasomy 9p mosaicism show a characteristic facial appearance, growth failure, and developmental delay. However, 3 patients with mosaicism for isochromosome 9p and a normal phenotype have also been reported. We report 2 additional cases of clinically normal young females with tetrasomy 9p mosaicism, one of whom also exhibited X chromosome aneuploidy mosaicism leading to an overall of 6 different cell lines. STR analysis performed on this complex mosaic case indicated that the extra isochromosome was of maternal origin while the X chromosome aneuploidy was of paternal origin, indicating a postzygotic event.

[Elimination of fat emulsions of various concentrations from the blood. Observational study in the intravenous administration of Lipovenös 10% and 20% in premature infants with very low birth weight]. / Elimination von Fettemulsionen unterschiedlicher Konzentration aus dem Serum. Beobachtungsstudie zur parenteralen Gabe von Lipovenös 10% und Lipovenös 20% bei Frühgeborenen mit sehr niedrigem Geburtsgewicht.

Within the scope of an observation study 40 premature low birth weight infants requiring parenteral nutrition received either 10% or 20% lipid emulsions (Lipovenös) for 7 days. The 10% lipid emulsion differs from the 20% lipid emulsion in the higher phospholipid/triglyceride-ratio (0.06 resp. 0.12). Lipid infusion was commenced at 0.5 g triglyceride/kg/24 hours and increased steadily to 2 g triglyceride/kg/24 hours. The aim of the study was to compare the effects of the two intravenously administered lipid emulsions on serum clearance. The serum concentrations of triglyceride and cholesterol did not change significantly during the infusion with 20% Lipovenös. Significant increases in the triglyceride and cholesterol content were observed only in the serum of the patients who were given the 10% Lipovenös. The reduced lipid serum clearance is attributable to the higher content of phospholipids in the 10% lipid emulsion. With regard to the risk of high cholesterol concentrations and an abnormal LPX serum accumulation, administration of 20% lipid emulsion is preferable to 10% lipid emulsion, also during the neonatal period.

[Therapy of hyperammonemia in carbamyl phosphate synthase deficiency with peritoneal dialysis and venovenous hemofiltration]. / Therapie der Hyperammonämie bei Carbamylphosphat-Synthetasemangel mittels Peritonealdialyse und venovenöser Hämofiltration.

Inborn errors of metabolism with hyperammonaemia cause emergency situations with unconsciousness, convulsion, hyperpnoea and hyperpyrexia. Therefore hyperammonaemia has to be treated immediately after diagnosis to avoid irreversible damage. Two newborns with carbamylphosphates synthetase deficiency are described. Both, continuous peritoneal dialysis and venovenous haemofiltration have proved to be effective methods to reduce serum ammonia concentration to values of less than 300 mu/l. Because of the severity of the enzyme defect in both cases, (carbamylphosphate synthetase was not detectable in liver tissue), treatment finally had to be stopped, and both patients died.

[Epidermolysis bullosa atrophicans gravis. Report of a therapeutic trial with dapsone]. / Epidermolysis bullosa atrophicans gravis. Bericht über einen Therapieversuch mit Dapson.

A case of junctional epidermolysis bullosa (Herlitz) is reported. The patient has been treated with high doses of Dapson. The result is encouraging.

3-Methylhistidine/creatinine ratio in urine from low-birth-weight infants. Statistical analysis.

3-Methylhistidine and creatinine concentrations were determined in 45 24-hour urine samples collected in 380 single voidings from 23 preterm infants (gestational age: 30-36 weeks, median: 33 weeks; birth weight: 1,613 +/- 219 g; age: 9-83 days postpartum) and from 7 infants small for gestational age (birth weight: 2,061 +/- 203 g; age: 2-30 days postpartum). Statistical analysis shows that diurnal variations of the ratio 3-methylhistidine/creatinine are negligible. The variability of this ratio is chiefly caused by differences in excretion on different collection dates and is probably due to differences in the metabolic state. Hence the determination of 3-methylhistidine/creatinine ratio in single voidings is sufficient even in low-birth-weight infants. In our collective the mean 3-methylhistidine/creatinine ratio for healthy, well-growing low-birth-weight infants (n = 21) was 19.6 +/- 2.3 mumol/mmol. Infants with stagnating or decreasing weight (n = 5) showed 3-methylhistidine/creatinine ratios clearly above that of the normal group.

3-Methylhistidine/creatinine ratio in urine from low-birth-weight infants. Clinical approach.

Single urine voidings were collected twice a week in the clinical course of 12 low-birth-weight infants (gestational age: 31.8 +/- 2.8 weeks; birth weight: 1,383 +/- 308 g) and analyzed for 3-methylhistidine and creatinine. The mean 3-methylhistidine/creatinine ratio for 6 healthy, well-fed, growing low-birth-weight infants was 20.2 +/- 1.9 mumol/mmol. In the clinical course of single individuals a rise of urinary 3-methylhistidine/creatinine ratio was observed in cases of acute infection and/or low energy supply (less than 100 kcal/kg/day) frequently coupled with insufficient weight gain. Mean 3-methylhistidine/creatinine ratios in infants with hyaline membrane syndrome under artificial respiration were generally higher than in the controls matched for energy supply.