Giant placental chorioangioma followed by circulatory failure of the newborn and infantile hemangioma: Case report.

Placental chorioangioma (CA) is a benign placental tumor. No specific treatment is required for asymptomatic cases. We report a female infant born to a mother with giant placental CA. However fetal growth was normal and, fetal hydrops was not detected by ultrasound examination until delivery, she had hydrops, subgaleal hematoma, thrombocytopenia, hemolytic anemia, respiratory distress and circulatory failure after birth. She was successfully treated without any neurological sequelae. At 2 months of age, infantile hemangioma appeared in her lower lip. The present case suggested that giant placental CA might cause postnatal problems and be associated with the development of infantile hemangioma.

Inducing peripheral sympathetic nerve activity by therapeutic electrical stimulation.

PURPOSE: To examine whether the activity of peripheral sympathetic nerves in animals with spinal cord injury can be controlled using therapeutic electrical stimulation. METHODS: The spinal cords of 6 Wistar rats were severed at T12/T13 disk level and were given continuous therapeutic electrical stimulation. Microneurography was used to record sympathetic nerve activity at 24, 48, and 72 hours after severing the spinal cord. RESULTS: Integrated values of muscle sympathetic nerve activity after 72 hours of therapeutic electrical stimulation revealed significantly larger potentials on the stimulated side than the non-stimulated side. Skin sympathetic nerve activity showed no difference between the 2 sides. CONCLUSION: Therapeutic electrical stimulation was found to have a facilitatory effect on the muscle sympathetic nerve activity, whereas regulatory function was activated by the sympathetic nerves.

Sympathetic skin response evoked by respiratory stimulation as a measure of sympathetic function.

OBJECTIVES: To compare respiratory and electrical methods of evoking a sympathetic skin response (SSR). METHODS: SSRs evoked by both electrical and respiratory stimulation were recorded from the palms of 47 healthy volunteers. Expiration and inspiration were used as separate stimuli. The correlation coefficients between the amplitude and latency of the SSR from the palm electrodes and the various components of heart rate variability were calculated. RESULTS: Waveform patterns of the SSRs obtained from electrical stimulation showed varied responses to and habituation to this type of stimulation. On the other hand, no subjects showed a phase change in SSR waveform patterns between the first and last expiratory stimuli. The potentials recorded after expiratory stimulation had significantly greater amplitudes than those recorded after electrical stimuli. The low frequency component of heart rate variability induced by expiratory stimulation was significantly greater than that induced by electrical stimulation. The SSR may also correlate strongly with the change of respiratory rate since a more rapid pressure change occurs during expiratory movement than during inspiratory movements. CONCLUSIONS: The SSR evoked by expiratory stimulation is more reliable than either electrical stimulation or inspiratory stimulation for determining sympathetic function.

Lipid storage myopathy associated with recurrent Reye syndrome-like attacks, but with a normal carnitine level.

A 7-year-old girl developed recurrent episodes of hepatic and cerebral dysfunction which mimicked those in Reye syndrome (RS). Because of mild muscle weakness, she had repeated muscle biopsies which showed markedly increased amounts of lipid droplets, predominantly in type 1 fibers. Liver histological examination showed widespread hepatocellular steatosis. However, diffuse microvesicular fat, seen in RS, was not found in the cytoplasm. The concentrations of free- and acylcarnitine in serum and muscle were within normal ranges. Normal ketogenesis was induced by fasting. Based on the clinical, laboratory, and histopathological findings, our patient was initially thought to have systemic carnitine deficiency. However, the serum and muscle carnitine levels were within normal limits. Although the primary metabolic defect has yet to be elucidated, the present study indicates that lipid storage myopathy in the absence of carnitine deficiency can be complicated with RS-like episodes.

Sympathetic skin response in patients with spinal cord injury.

PURPOSE: To assess the effectiveness of sympathetic skin response in evaluating peripheral sympathetic nerve activity of patients with spinal cord injury, and to report on the basic properties of sympathetic skin response. METHODS: Sympathetic skin response evoked by electrical stimulation was recorded from the palms and soles of healthy volunteers and patients with spinal cord injury. RESULTS: Sympathetic skin response was recorded in 17 healthy volunteers and 14 patients with spinal cord injury. Of the 4 waveforms, the shortest latency was obtained from the palm; the sympathetic skin response was 1.2 to 1.4 ms at all stimulated sites, 1.9 to 2.0 ms at the sole, with a difference of about 0.6 ms between the palm and the sole. None of the patients with spinal cord injury responded at either the upper or lower limbs. In patients with a thoracic cord injury, some responded at the upper limbs but none at the lower limbs; some responded at neither upper nor the lower limbs; and some responded at both upper and lower limbs. The conducting pathway of sympathetic skin response in the spinal cord for the upper limbs descends to the upper thoracic cord (T4-6), and the conducting pathway for the lower limbs departs from the spinal cord at the lower thoracic cord (T9-10). CONCLUSION: It appears that sympathetic skin response should be used for the evaluation and morbid investigation of the functional abnormalities of the sympathetic nervous system in patients with spinal cord lesions such as spinal cord injuries, cervical spondylosis, and spinal canal stenosis.

Diagnostic utility of waveform analysis of compound muscle action potentials for carpal tunnel syndrome.

PURPOSE: To determine the diagnostic utility of waveform analysis of compound muscle action potentials (CMAP) for carpal tunnel syndrome (CTS). METHODS: A total of 131 hands in 71 patients diagnosed with CTS (grouped according to severity) and 80 hands in 44 normal subjects were evaluated using nerve conduction test through the carpal tunnel combined with waveform analysis of CMAP. RESULTS: Compared to normal subjects, the sensory nerve conduction velocity and mean frequency of the CMAP waveform were significantly reduced in patients with CTS. Compared with distal motor latency and sensory nerve conduction velocity, the mean frequency of the CMAP decreased significantly with increasing clinical severity. CONCLUSION: This study suggests that waveform analysis of CMAP is of diagnostic value in CTS, and is also of value in objective evaluation of postoperative recovery of carpal median nerve dysfunction.

The relationship between nerve conduction study and clinical grading of carpal tunnel syndrome.

PURPOSE: To conduct a median nerve conduction study on patients with carpal tunnel syndrome and investigate the relationship between nerve conduction study parameters and clinical grading. METHODS: A nerve conduction study was performed on 60 upper limbs of 37 patients with idiopathic carpal tunnel syndrome, and the relationship between the clinical grade and various study parameters was assessed. RESULTS: The amplitude of the sensory nerve action potential and the motor nerve action potential differed according to clinical grading, but this pattern was not seen for sensory nerve conduction velocity, motor nerve conduction velocity, or motor nerve terminal latency and clinical grading. CONCLUSION: The amplitude of the sensory nerve action potential and motor nerve action potential reflect the functional state of axons, and are useful parameters for assessing clinical grading based on nerve conduction velocity.

[Acupuncture for urinary incontinence in patients with chronic spinal cord injury. A preliminary report].

PURPOSE: We investigated the possible use of acupuncture for the treatment of urinary incontinence caused by detrusor hyperflexia in patients with chronic spinal cord injury. METHOD: A total of 8 male chronic spinal cord injured patients with urinary incontinence were treated by acupuncture. Their ages ranged from 20 to 33 years (mean 27). The level of lesion was cervical in 4 and thoracic in 4. Detrusor hyperreflexia with uninhibited bladder contraction was confirmed by urodynamic studies in all of them. Acupuncture was performed using a disposable stainless needle (0.3 mm in diameter, 60 mm in length), which was inserted into bilateral BL-33 (Zhongliao) points and was rotated manually for 10 minutes. The treatment was conducted every week for 4 weeks. Urodynamic studies were repeated, immediately after the beginning of and a week after the completion of the treatment. Urinary symptoms were also checked before and after the treatment. RESULTS: No side effects were recognized throughout the treatment period. Among 8 patients, incontinence was controlled completely in 3 (38%) and partially in 3 (38%). The average maximum cystometric bladder capacity increased significantly, from 42.3 +/- 37.9 ml to 148.1 +/- 101.2 ml by the treatment (p < 0.05), while the average maximum bladder pressure was not changed. CONCLUSIONS: These data suggest that acupuncture could be a promising alternative for conventional therapies for urinary incontinence caused by detrusor hyperreflexia in patients with chronic spinal cord injuries.

Development of multidrug resistance type I Cmdr1 expression in chicken embryonic gonads.

A primary role of P-glycoprotein (P-gp), encoded by the multidrug resistance type I gene, is to protect against naturally occurring xenotoxics. Recently, the preferential expression of chicken multidrug resistance type I (Cmdr1) was identified in the embryonic gonads during the early periods of development. Here we investigated the expression of Cmdr1 and P-gp in the gonads during embryogenesis, and compared to that in the ovarian follicles of domestic hens (Gallus gallus). As revealed by immunohistochemistry, P-gp was highly expressed in theca cells of mature follicles, whereas the expression was low in immature follicles. Immunohistochemical analysis showed that expression of Cmdr1-type P-gp was very low in embryonic gonads. Cmdr1 mRNA was undetectable in the gonads of 5-day embryos (E5) by RT-PCR, whereas Cmdr1 mRNA was significantly detectable in the developing gonads at E9 and E21. In the testicular tissues, germ cells were distributed along developing seminiferous cords as identified by a specific marker gene, whereas Cmdr1-type P-gp positive cells were observed evenly on testicular tissues. Collectively, it is concluded that Cmdr1 expression is initiated in the chicken ovary and testis after sexual differentiation, but expression of Cmdr1-type P-gp is very low through embryogenesis.

New diagnostic techniques for the detection of organic acidemias.

We describe an improved procedure for the extraction of organic acids which is equally suitable for urine, plasma and amniotic fluid and gives similar high recoveries for a wide variety of organic acids, without interferences from phosphate, sulfate, or urea. Internal standards are added, the oxoacids are converted to the pentafluorobenzyloximes; the sample is concentrated, acidified, chromatographed on a small column of silicic acid, and the effluent neutralized and dried. After forming the trimethylsilyl derivatives, gas chromatography is done on a 0.53 mm X 30 m low-polarity bonded-phase fused silica capillary column. For gas chromatographic-mass spectrometric quantification, the peak areas at the m/z of a characteristic fragment ion for each acid relative to the peak areas of ions of the internal standards are related to standard curves.

Thin-layer chromatographic screening procedure for some drugs in horse plasma.

A thin-layer chromatographic screening procedure for some basic, neutral and acidic drugs was developed using 3 ml of horse plasma. Chloroform-2-propanol (95:5, v/v) was used as the extraction solvent. The drugs were identified by a high-performance thin-layer chromatographic plate and spraying successively with some detection reagents. In this study, the extraction recovery rates and the detection limits were determined at the same time.

A study of the normal values and habituation phenomenon of sympathetic skin response.

Sympathetic skin response (SSR) has been developed recently as a method of capturing the autonomic nerve response as a parameter of the sweat gland function. In this study, our aim was to obtain the normal values with regard to both amplitude and latency of SSR from 50 healthy subjects and to find out the habituation mode, which is one of the most characteristic phenomena of SSR. The measurements were recorded from the hand and foot by rectangular waveform electric stimulation. The correlation coefficient regarding hand and foot leads, amplitude, and latency were studied at normal values. The result was that no significant difference could be observed between the left and right leads with regard to amplitude and latency recorded from the hand and foot leads. However, between hand and foot leads, a significant difference was observed for both latency and amplitude. As for the habituation, electric stimulations were also applied to 20 healthy subjects (age range, 21-62 yr) in the same manner as that used in taking the normal values. For the latency and amplitude of the response in association with the stimulation trials (Rn), the mean values of the hand as well as the foot were determined by averaging the values recorded from both hands and both feet of the 20 subjects. These values were designated as the latency and the amplitude of the hand and foot from R1 to R15. The changes observed in response to the transition in the number of stimulations were statistically evaluated as a gradual decrease in the amplitude. This phenomenon is thought to be a result of learning. On the basis of the 15 consecutive stimulations, a decrease in amplitude to the 7th and 8th stimulations and constancy in the results thereafter was observed, although minor differences in the results were apparent between the hand and foot leads.

Effects of exercise on plasma concentrations of caffeine and its metabolites in horses.

The effects of exercise on the metabolism of caffeine (CA) were studied 3h after administration of the drug to race horses which then underwent exercise sets (1000-m gallop). Analysis was made of pharmacokinetics of CA, changes in its plasma concentrations, its metabolites, i.e., theophylline (TP), theobromine (TB) and paraxanthine (PX), and the molar concentration ratios of CA to these metabolites. After exercise, AUC and t1/2 tended to decrease, and the concentration of CA decreased, while the concentrations of TP and TB significantly increased. The TP/CA ratio and TB/CA ratio significantly increased from 6 to 74h and from 25 to 50h after drug administration, respectively. This indicated promotion of metabolism of CA into TP and TB. The effects on PX were minimal.

Urinary excretion of 2-methylacetoacetate, 2-methyl-3-hydroxybutyrate and tiglylglycine after isoleucine loading in the diagnosis of 2-methylacetoacetyl-CoA thiolase deficiency.

The concentrations of 2-methylacetoacetate, 2-methyl-3-hydroxybutyrate and tiglylglycine were determined by gas chromatography-mass spectrometry in urine collected before and for 8 h after loading with 100 mg of isoleucine per kg of body weight. The sum of 2-methylacetoacetate and 2-butanone, a decarboxylation product, was determined as the 2-butanone dinitrophenylhydrazone derivative. Substantial increases in each compound were encountered in a patient with a documented defect of 2-methylacetoacetyl-CoA thiolase. Increased quantities of 2-methyl-3-hydroxybutyrate and tiglylglycine were also found in four children with clinical symptoms similar to those associated with 2-methylacetoacetyl-CoA thiolase deficiency but in whom the activity of the enzyme was found to be normal. The concentration of 2-methylacetoacetate plus 2-butanone in the urine increased after an isoleucine load only in the patient with 2-methylacetoacetyl-CoA thiolase deficiency.

Pharmacokinetics of caffeine and its metabolites in horses after intravenous, intramuscular or oral administration.

The pharmacokinetics of caffeine (CAF) and its metabolites, dimethylxanthines, were examined in horses administered 2.5 mg/kg of CAF intravenously (i.v.), intramusculary (i.m.), or orally (p.o.). The plasma samples were extracted by Extrelut and the concentrations of CAF and metabolites were determined by high performance liquid chromatography (HPLC) with a short column. The pharmacokinetics of CAF after bolus i.v. injection were described by the assumption of a two-compartment model, and those of CAF after i.m. or p.o. administration were done by the assumption of a one-compartment model. The biologic half lives of CAF were 15.5, 18.6, and 16.4 h after administering i.v., i.m. and p.o., respectively. The extent of the bioavailability of the p.o. administration was determined as 1.04 times the dose. The differences in pharmacokinetic parameters were not statistically significant among administration routes. A straight correlation existed between the logarithms of body weights of different species of animals and those of their biologic half lives of CAF. Therefore, the biologic half life of CAF in an animal might be predictable as a function of its body weight.

Methylcitrate in maternal urine during a pregnancy with a fetus affected with propionic acidaemia.

Patients homozygous for propionic acidaemia have high levels of methylcitric acid in urine while heterozygotes have normal levels. Methylcitric acid was determined by stable isotope dilution gas chromatography-mass spectrometry in maternal urine during a pregnancy with a fetus affected with propionic acidaemia. Although the levels increased linearly between 23 and 39 weeks of gestation, the levels were not significantly elevated above normal until after 30 weeks of gestation. Thus, determination of methylcitric acid in maternal urine is not of value for early prenatal diagnosis of propionic acidaemia.