Prenatal diagnosis of isovaleric acidemia by fast atom bombardment and tandem mass spectrometry.
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Amniotic Fluid↗
Biomedical subjects
Y Shigematsu
Publications and source records attributed to Y Shigematsu.
Effect of disopyramide on systolic and early diastolic time intervals in patients with hypertrophic cardiomyopathy.
The present study clarified the effect of disopyramide on left-ventricular function in patients with hypertrophic cardiomyopathy (5 obstructive type: HOCM, 21 non-obstructive type: HNCM). The systolic and early diastolic time intervals were assessed 3 hours after a single oral administration of 100-mg disopyramide. The following parameters were evaluated at rest and after administration of disopyramide: 1) left-ventricular ejection time index (LVETI), 2) pre-ejection period index (PEPI), 3) the interval from aortic component of the second heart sound to mitral valve opening (IIA-MVO), and 4) the interval from MVO to O point of apexcardiogram (MVO-O). LVETI in HNCM did not change after disopyramide but that in HOCM was significantly shortened (P less than .05). PEPI in both HOCM and HNCM was significantly prolonged after administration of disopyramide. IIA-MVO time in both HOCM and HNCM was not influenced by disopyramide. MVO-O time in both HOCM and HNCM was significantly shortened after disopyramide. These results suggest that 1) shortening of LVETI in HOCM after disopyramide seemed to be due to the decrease in pressure gradient, 2) PEPI prolongation after disopyramide reflected the decrease in myocardial contractility, and 3) shortening of MVO-O time after disopyramide indicated the improvement of left-ventricular filling. The authors conclude that disopyramide may be an important new therapeutic agent in the treatment of patients with hypertrophic cardiomyopathy.
Adult↗
Scintigraphic assessment of cardiac adrenergic innervation in patients with essential hypertension.
To assess the regional cardiac adrenergic innervation in patients with essential hypertension (EHT), simultaneous iodine-123 metaiodobenzylguanidine ([123I]MIBG) and thallium-201 (201Tl) myocardial imagings were performed in five patients with EHT, seven patients with hypertrophic cardiomyopathy (HCM), and seven normal subjects. Short axial images at rest were divided into five segments: anterior, septal, posterior, lateral, and apical segments. Percent regional uptake (%RU) of 201Tl except the septal segment in patients with EHT showed no significant difference. However, the %RU of [123I]MIBG at posterior, lateral, and apical segments was significantly lower than that at anterior and septal segments in EHT. This intraimage heterogeneity of [123I]MIBG was also observed in HCM. These results suggest that there is a difference in regional adrenergic innervation of the left ventricle with myocardial hypertrophy.
3-Iodobenzylguanidine↗
Evaluation of lumbar bone mineral density by dual energy x-ray absorptiometry.
Using dual-energy x-ray absorptiometry (DEXA), the lumbar spinal bone mineral density (BMD) in 49 Japanese children with or without metabolic bone disease (MBD) was determined. The following results were obtained: (a) The normal data for healthy Caucasians (J Clin Endocrinol Metab 1990; 70: 1330-1333) appear to be applicable to Japanese children; (b) BMD was normal in patients with congenital hydronephrosis with normal renal function; (c) One patient with congenital renal failure and one with Lowe syndrome had low BMD, but the MBD in the former improved markedly with peritoneal dialysis; (d) A reduced BMD was found in patients treated with long-term steroids, probably because of decreased turnover of bone; (e) A reduction in BMD was pronounced in preterm infants during the first few months of life. In conclusion, DEXA is a useful method of bone densitometry in MBD in children.
Absorptiometry, Photon↗
Mucopolysaccharidosis VI (Maroteaux-Lamy syndrome) with hearing impairment and pupillary membrane remnants.
A Japanese boy aged 13 months was referred to us because thickened ribs had been observed on a chest X-ray taken during a respiratory infection. mucopolysaccharidosis type VI (MPS VI) was diagnosed based on urinary glycosaminoglycan analysis and low activity of arylsulfatase B in peripheral leukocytes. He had mild pupillary membrane remnants, but no corneal opacities. The auditory brainstem response revealed moderate hearing impairment, which may have caused his subnormal DQ score of 85 at the age of 19 months. Although MPS VI is characterized by normal intellectual development with normal hearing in early infancy, it is important to examine for hearing loss, especially when an infant with this disease shows developmental delay.
Hearing Disorders↗
Giant hydroureteronephrosis associated with primary obstructed megaureter.
The case is presented of a four year old boy with a large left abdominal mass. The patient was diagnosed as having a left giant hydroureteronephrosis due to a primary obstructed megaureter, based on the findings of ultrasonography, micturating cystourethrography, excretion urography, computed tomography and percutaneous antegrade pyeloureterography. Diuretic renography showed that the obstruction was partial. Since a static renal scintigram indicated that renal function on the affected side was moderately well preserved, ureteroplasty is planned.
Child, Preschool↗
Exercise-induced urinary excretion of leukotriene E4 in children with atopic asthma.
Urinary levels of leukotriene (LT) E4, a stable end-product of LTC4 and LTD4, were measured before and after exercise in 10 children with severe asthma and seven children with moderate asthma using HPLC and RIA to clarify the relationship of LT to the severity of asthma and to the degree of bronchospasm in exercise-induced asthma. The urinary LTE4 level significantly increased after exercise in the severe asthma group, but not in the moderate asthma group (14.3 +/- 14.5 to 24.3 +/- 20.6 versus 19.6 +/- 12.3 to 17.6 +/- 10.8 ng/mmol creatinine, p less than 0.05). The urinary LTE4 level increased in 10 patients (eight with severe asthma), and it decreased in seven patients (five with moderate asthma). A significant difference in the degree of bronchospasm after exercise (as shown by the maximal % fall in the peak expiratory flow rate), was seen when patients with increased urinary LTE4 excretion were compared with those with decreased excretion (60.4 +/- 17.3 versus 24.1 +/- 14.3%, p less than 0.01). Our findings suggest that exercise-induced asthma, or at least a subtype of exercise-induced asthma, may partly develop through the release of LTC4.
Asthma, Exercise-Induced↗
Pathophysiologic study on methylmalonic aciduria: decrease in liver high-energy phosphate after propionate loading in rats.
Methylmalonate or propionate was i.v. infused into B12-deprived and control rats. In the B12-deprived rats, the plasma and liver concentrations of B12 decreased to 8 and 13%, respectively, of those of the control rats. The propionate loading produced a disproportionate increase in liver propionate levels; the mean ratio of methylmalonate to propionate in the liver was approximately 1.0 after methylmalonate loading, whereas it was 0.1 to 0.2 after propionate loading. The liver propionate and methylmalonate levels in the B12-deprived rats were twice as high as those in the control rats. The mean ratio of beta-ATP to inorganic phosphate in the liver, measured with 31P-magnetic resonance spectroscopy, decreased from 0.60 to 0.48 in the B12-deprived rats and from 0.78 to 0.63 in the control rats after methylmalonate loading; the ratio decreased from 0.57 to 0.37 in the B12-deprived rats and from 0.76 to 0.56 in the controls after propionate loading. Statistical analysis showed that propionate loading caused a more marked decrease in ATP than did methylmalonate loading (F = 26.33, degree of freedom 1 and 15; p less than 0.001), while B12-deprivation caused a more marked decrease in ATP than did the control diet (F = 92.26, df 1 and 15; p less than 0.001). The concentrations of tricarboxylic acid cycle intermediates and related organic acids in the livers of the rats suggested that propionate inhibited NAD(+)-dependent enzymes in the cycle.(ABSTRACT TRUNCATED AT 250 WORDS)
Adenosine Triphosphate↗
Influence of age and severity of hypertension on blood pressure response to isometric handgrip exercise.
The influence of age and severity of hypertension on the blood pressure response to isometric handgrip exercise (IHG) was studied in essential hypertensive patients (n = 122). The change in blood pressure during IHG in elderly patients with isolated systolic hypertension (ISH) (n = 12) was also studied. Left ventricular hypertrophy due to hypertension was used as an index of the severity of hypertension. The change in systolic blood pressure (SBP) during IHG was markedly greater in essential hypertensives than in normotensive subjects (n = 36). Among hypertensive patients, the change in SBP increased with increasing severity of hypertension. This change in SBP was not influenced by age. The change in SBP during IHG in patients with ISH was significantly smaller than that in essential hypertensive patients and was similar to that in normotensive subjects in the elderly. These results demonstrate that age does not affect the increased blood pressure response to IHG in essential hypertensive patients but the greater the severity of hypertension, the greater the increase in SBP during IHG. Elderly patients with ISH do not have an enhanced blood pressure response to IHG.
Adult↗
[Mycotic aneurysm of the sinus of Valsalva and complete atrioventricular block complicating infectious endocarditis with aortic regurgitation: a case report].
A patient with a mycotic aneurysm of the sinus of Valsalva and heart block secondary to infectious endocarditis was described. This 46-year-old man was admitted to our hospital on May 9, 1990, because of fever and progressive general malaise after extraction of a tooth. Physical examination on admission revealed blood pressure of 98/62 mmHg, pulse rate 96 per min, temperature 37.7 degrees C and respiration 35 per min. Auscultation of the heart revealed a grade 3/6 systolic murmur and a grade 2/6 diastolic murmur at the third left intercostal space. Chest radiograph showed mild cardiomegaly with moderate lung congestion. Electrocardiography revealed the first grade atrioventricular block. Echocardiography demonstrated vegetations on the aortic valve, and perforation of the non-coronary sinus of Valsalva. The prolapsed non-coronary sinus of Valsalva extended into the right atrium. Doppler echocardiography revealed a severe aortic regurgitant jet in the diastolic phase. We diagnosed the patient as having aortic regurgitation with a mycotic aneurysm of the non-coronary sinus of Valsalva due to infectious endocarditis. His condition remained severely ill despite intensive medical treatment. On May 14, 1990, aortic valve replacement and excision of the mycotic aneurysm were performed. The commissural portions of the aortic cusps were heavily thickened and calcified. The mycotic aneurysm was very fragile. During manipulating the mycotic aneurysm, the sinus accidentally perforated into the right atrium. The cardioaortic fistula was closed with a goretex patch. A demand pacemaker was implanted because of postoperative complete atrioventricular block.(ABSTRACT TRUNCATED AT 250 WORDS)
Aneurysm, Infected↗
Extraction of urinary leukotriene E4 by the combined use of octadecyl reversed-phase and NH2 normal-phase extraction columns.
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Adult↗
Leukotriene B4 production in children with steroid-responsive nephrotic syndrome.
Leukotriene B4 (LTB4) production in polymorphonuclear leucocytes (PMN) was examined in ten children with steroid-responsive nephrotic syndrome (SRNS) before, during, and after steroid administration. Comparison of LTB4 production was made in 14 children with non-inflammatory disease who were not receiving steroid therapy. No significant change was noted in PMN LTB4 biosynthesis in children with SRNS throughout any phase of the disease. Furthermore, there was no significant difference in LTB4 biosynthesis in PMN between SRNS patients before steroid therapy and patients with non-inflammatory disease. These findings suggest that inhibition of LTB4 production is not involved in the mechanism underlying steroid action in SRNS.
Adolescent↗
Acute infantile hemiplegia in a patient with propionic acidaemia.
A 10-month-old girl with mild developmental delay became hemiplegic after seizures. Cranial CT scan and magnetic resonance imaging (MRI) revealed no lesions related to vascular diseases, but brain atrophy on the right side was remarkable. Digital subtraction angiography showed slightly decreased visualization of peripheral branches of the right medial cerebral artery. Propionic acidaemia was diagnosed on the basis of high plasma levels of propionic acid and its metabolites and the elevated urinary excretion of these acids. With therapy, the levels of these acids fell, and her left hemiplegia disappeared 3 months later.
Amino Acid Metabolism, Inborn Errors↗
Diagnosis of iliopsoas pyomyositis by ultrasonography and CT.
A patient with iliopsoas muscle pyomyositis was successfully treated with intravenous antibiotics alone. The diagnosis was made by ultrasonography and X-ray computed tomography after five days of spiking fevers. Ultrasonography and X-ray computed tomography revealed swelling of the muscle body but no abscess formation. We emphasize the usefulness of early diagnostic imaging to allow conservative treatment of iliopsoas muscle pyomyositis.
Child↗
Persistent elevation of cardiac enzymes in a patient with hypertrophic cardiomyopathy--with special reference to electrocardiographic, echocardiographic and 201-thallium myocardial scintigraphic findings.
A 17-year-old female patient with hypertrophic cardiomyopathy whose serum cardiac enzymes (creatine phosphokinase = CPK, lactic dehydrogenase = LDH) showed persistent elevation was presented. Percent of CPK-MB and LDH1 in 6-year-follow-up period ranged from 3.2% to 8.5%, and 58.0% to 63.2%, respectively. This finding strongly suggests the existence of a small amount of continuous myocardial necrosis for a long time. The influence of cardiac enzyme release on the heart was assessed by serial checks of electrocardiogram, echocardiogram and 201-thallium myocardial scintigram. In serial checks of electrocardiograms, markedly decreased R wave amplitude ranging between 31% and 47% was observed in II, III, aVF and V3-6. On echocardiograms, asymmetric septal hypertrophy and a narrow left ventricular cavity were observed in all echocardiograms through the follow-up period. But, in most recent apical two-dimensional echocardiograms, hourglass appearance of the left ventricle due to a distinct cavity-formation at the apex was observed. In 201-thallium single photon emission computed tomogram, hypoperfusion area markedly extended in anterior and lateral parts. These changes in electrocardiograms, echocardiograms and 201-thallium myocardial scintigrams seemed to reflect myocardial necrosis. Our case points to a mechanism for hypertrophic cardiomyopathy to change, over time, to dilated cardiomyopathy-like features.
Adolescent↗
Delayed auditory brainstem response in thiamin-deficient rats.
We recorded the auditory brainstem responses of rats fed a thiamin-deficient diet. The interpeak latencies between waves I and III, as well as those between waves I and IV, were significantly prolonged from day 24, while the latency of wave I was prolonged on day 26 of the thiamin-deficient diet. These delayed responses were corrected in 2 to 4 days after the initiation of daily intraperitoneal thiamin injections from day 32. The rats that were fed the thiamin-deficient diet, and then sacrificed on day 32, showed a decrease of total thiamin levels in the brain (26% of the level in control rat brains). Based on these results, we emphasize the value of the auditory brainstem response to detect thiamin deficiency.
Animals↗
[Dental management of the medically compromised patient. A study of 162 cases].
Dental management of 162 cases of medically compromised patients was reviewed. Over the past 3 years and 2 months, 130 patients with certain medical problems underwent 162 cases of dental treatment under local anesthesia. In the present study, research was done chiefly on intraoperative management of these patients. The following results were obtained: 1) In the population of 130 patients, those in their 7th decade were the most numerous. Among the subjects, essential hypertension was the most common underlying disease, and the majority of the patients had accompanying cardio-vascular diseases. 2) When the pre- and post-65-year-old patient groups were compared, the latter group had a higher frequency of multiple medical problems. 3) It is suggested that, to manage patients having hypertension or ischemic heart diseases as a complication, continuous blood pressure measurement and ECG monitoring are essential. 4) Among several local anesthetics, 3% prilocaine with 0.03 U/ml felypressin was used most frequently, especially for those with cardiovascular diseases. 5) In the management of hypertensive and ischemic heart patients, nitrous oxide inhalation sedation was effective. 6) For those who required vasodilation, administration of nifedipine or nitroglycerin was effective. 7) Although one case of syncope and another in which dental treatment procedure had to be suspended were found, no severe complications were encountered.
Aged↗
[Occlusion of basilar artery in a 7-year-old boy].
A case of occlusion of basilar artery in a boy aged 7 years was reported. Compared with CT, MR imaging was more valuable for the early diagnosis of this disease. SPECT with IMP was thought to be useful in evaluating cerebellar circulation. ABR revealed brain stem abnormalities that could not be detected by neuroradiological techniques.
Brain Stem↗