'Hypertrophic cardiomyopathy' in a girl with adult-type myotonic dystrophy.
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Biomedical subjects
Publications and source records attributed to T Nakada.
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In order to compare the daily voiding traits of elderly men with those of middle-aged men with lower urinary tract symptoms (LUTS), urinary frequency, bladder capacity at normal desire to void (CNDV), residual urine volume (RUV) and functional bladder capacity were evaluated at home during a 24 h period using a non-invasive method. The results showed that in both groups, urinary frequency was lowest from midnight to 06.00 h. The urinary frequency was the highest from 12.00 to 18.00 h. The CNDV in the elderly group was less than that of the middle-aged group for 1 day. In particular, bladder capacity from 24.00 to 06.00 h showed the greatest difference (P < 0.05). The RUV in elderly men tended to accumulate more than those in middle-aged men. The findings suggest that the functional bladder capacity of elderly men was lower than that of middle-aged men in the home.
We report on a boy with long-arm deletion of chromosome 10 and compare this case to 10 previously reported patients. He had right cryptorchidism and absence of the left testis, but the size of his penis was normal. Cytogenetic analysis of the case showed the deficiency of 10q26.1-26.3 and the presence of 10qter. Four of 10 previously reported patients had an intersex phenotype, and all others had combinations of cryptorchidism, micropenis and hypospadias. These facts indicate that the terminal of chromosome 10q is strongly associated with abnormal male development.
We report a case of anorchia in conjunction with isolated idiopathic hypogonadotropic hypogonadism. Plasma levels of LH, FSH, TSH and testosterone were abnormally low. Administration of human chorionic gonadotropin caused no increase in plasma testosterone, and injection of luteinizing hormone-releasing hormone resulted in no change in plasma gonadotropins. We discuss the embryology and hormonal status of this patient.
The influence of immobilization stress on testicular germ cell apoptosis was investigated in rats. A transient increase in serum corticosterone and a transient decrease in serum testosterone were observed during each period of immobilization stress. Twenty-four hours after the last immobilization session, the testicular weight and serum concentrations of corticosterone and testosterone were the same between the immobilization stress and control groups. However, the percentages of apoptotic tubules and apoptotic cells in the stress group were significantly higher than those in controls (P < 0.001). These facts suggest that immobilization stress can enhance testicular germ cell apoptosis in rats.
Individuals of the wild silkworm, Bombyx mandarina, collected in South Korea (Taegu City) and Japan (Tsushima Islands and Fukuoka City) had the chromosome number of 2n = 54, while those collected in China (Hangzhou City) had the chromosome number of 2n = 56. Analysis by PCR (polymerase chain reaction) showed that the 66-bp-long retroposon-like insertion known in the arylphorin gene was present in the B. mandarina specimens with 2n = 54, but not in those with 2n = 56. Thus, dimorphism in the chromosome number coincided with the occurrence of the insertion. It is likely that the boundary dividing the two geographic B. mandarina populations lies somewhere in the northern part of the Korean Peninsula.
Most cases of drug eruption caused by nonionic contrast media (NICM) reported to date have been of the erythema multiforme type. Herein we report the first case of multiple fixed drug eruption (FDE) caused by iomeprol (Iomeron(R)). A 67-year-old woman developed multiple pea-sized erythematous papules on the trunk and extremities 4 days after receiving 100 ml of iomeprol for a computed tomography examination. Some of the papules coalesced, forming 7 large plaques on the limbs. Six months later, the patient was mistakenly administered iomeprol again. On the following morning, erythematous plaques admixed with vesicles recurred at the same sites as during the previous episode. In both episodes, the lesions cleared leaving pigmentation that faded with 6 weeks. Both patch testing and an intradermal test with iomeprol on lesional pigmented skin were positive. The present case indicates that NICM may cause multiple FDE and that repeated administration of the causative agent may increase the severity of the eruption.
OBJECTIVES: We examined the serum concentrations of total and free prostate-specific antigen (PSA) from patients with liver cirrhosis to clarify the influence of liver function on serum PSA levels. METHODS: Serum concentrations of total and free PSA, as well as the free/total PSA ratio were measured in 75 male patients with histologically confirmed liver cirrhosis. As a control group, 75 age-matched healthy blood donors were studied. RESULTS: The serum levels of total PSA in liver cirrhosis patients were significantly lower than those in controls (p = 0.0004). However, the serum free/total PSA ratios in liver cirrhosis patients were significantly higher than those of controls (p < 0.0001). CONCLUSIONS: Our results suggest the absence of an obvious clearance disturbance of serum PSA as a result of severe hepatic dysfunction and offer caution against the utility of standard cutoff values of serum PSA or free/total PSA ratios in the detection and staging of prostate cancer in men with severe liver dysfunction.
OBJECTIVE: To evaluate the influences of CO(2) insufflation on changes in blood gas analysis and end tidal CO(2) tension (PetCO(2)) during posterior retroperitoneoscopic adrenalectomy in the prone position. METHODS: Arterial blood gas analysis and measurements of PetCO(2) were carried out during CO(2) insufflation in 16 patients who underwent posterior retroperitoneoscopic adrenalectomy in the prone position (PRA group). The results were compared to 10 patients who underwent open posterior adrenalectomy (OPA group). Ventilation was artificially controlled during the study period in all cases. RESULTS: Arterial pH, PaCO(2), PetCO(2) and PaO(2) were not significantly different between the PRA and OPA groups. However, the PaCO(2)-PetCO(2) gradient in the PRA group was significantly higher than that in the OPA group (p < 0.01). CONCLUSION: Transperitoneal absorption of CO(2) occurs in patients undergoing retroperitoneoscopy in the prone position. The alveolo-arterial CO(2) gradient may be the only parameter which indicates the absorption of CO(2) during PRA.
From July 1994 to January 1999, three patients with penile squamous cell carcinoma with deep inguinal lymph node metastases without distant metastases were treated with multimodality treatment including intra-arterial infusion chemotherapy mainly with monthly courses of cisplatin, methotrexate and bleomycin. One patient achieved a complete response and 2 achieved a partial response. It appears to be reasonable to presume that intra-arterial chemotherapy has a beneficial effect in the management of penile cancer with fixed metastatic inguinal lymph nodes, but a larger number of patients and longer follow-up are required to confirm these results.
OBJECTIVES: This study was designed to compare urodynamic types of bladder outlet obstruction (BOO) and histological architecture of the prostate in patients with benign prostatic hyperplasia (BPH). PATIENTS AND METHODS: Twenty-four patients with symptomatic BPH, who underwent transurethral resection of the prostate (TURP), were included in the study. Before surgery, a pressure-flow (P-F) study was performed in all patients. According to the classification by Spanberg et al., they were divided into 5 normal type, 6 low-compliant, 6 compressive and 7 constrictive obstruction. The area density and total area of smooth muscle, fibrous tissue, glandular epithelium and glandular lumen in BPH specimens were estimated using morphometric analysis. RESULTS: In all groups, the stromal component occupied about three fourths of BPH specimens, and fibrous tissue was the most prominent element. However, neither area density nor total volume of each histological component showed a significant difference among the four groups. CONCLUSION: The type of BOO in patients with BPH cannot be explained by histological architecture alone.
The utility of three-dimensional anisotropy contrast (3DAC) magnetic resonance (MR) axonography, a method sensitive to neuronal fibers and their directionality, was investigated in the clinical setting using a 3-tesla MR imaging system based on a General Electric Signa platform. The study focused on healthy volunteers and patients with common structural central nervous system disorders, namely chronic infarction, brainstem cavernous hemangioma, supratentorial meningioma, and astrocytoma. Three orthogonal anisotropic diffusion-weighted images were first obtained. Three primary colors were each assigned to a diffusion-weighted image, respectively, and the images were subsequently combined into a single-color image in full-color spectrum (3DAC MR axonography image). Fiber-tract definition in the cerebral peduncle of the midbrain of healthy volunteers showed intersubject variation, with two general patterns recognized: dispersed (60% of cases) and compact (40% of cases). Pathological alterations in the fiber tracts were readily identified in cases involving wallerian degeneration of the pyramidal tract, as illustrated in the cases of chronic infarction. Displacement of major tracts, such as the medial lemniscus or corticospinal tract, as well as fiber directionality, was also easily recognized in cases of mass lesions. As an imaging method uniquely capable of providing information regarding axonal connectivity, 3DAC MR axonography appears to have promising potential for routine clinical application.
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The human indusium griseum (IG), the paired dorsal continuation of the hippocampus, was investigated with high-field (3.0T) MR imaging. The IG was clearly visible in 16 out of 20 healthy volunteers. The most common pattern was a single lateralized strip. The classical neuroanatomic pattern of paired symmetric strips along the midline was found in one case. The study clearly demonstrates that diminutive, hitherto overlooked structures such as the IG now can be readily investigated in vivo by non-invasive high-field MR imaging.
Magnetic resonance (MR) is a remarkably versatile technology applicable to various aspects of medical science. Currently, there are three categories of MR techniques available for probing human brain function in detail. The first category comprises the most widely utilized techniques which make use of the metabolic effects of brain activation, represented by BOLD functional magnetic resonance imaging (fMRI). The second category of techniques deals with apparent diffusion tensor in probing the cellular aspects of brain function and is represented by three dimensional anisotropy contrast (3DAC) axonograpy. The third category of techniques is the oldest and as yet the most underutilized. These techniques which include MR spectroscopy (MRS) and spectroscopic imaging (SI), are based on classical nuclear magnetic resonance (NMR) spectroscopy and are capable of providing biochemical information in vivo. Because of its inherently non-invasive nature, MR technologies are especially important for the field of pediatric neurology and developmental neuroscience.
There is mounting criticism regarding the validity of blood oxygenation level dependent contrast functional magnetic resonance imaging (BOLD-fMRI) studies. The main sources of error in BOLD-fMRI are pixel misalignment and physiological noise. One of the widely practiced tactics to ameliorate motion related fictitious activation is post-processing motion correction algorithms. Nevertheless, the validity of activation data following application of such algorithms has not been totally confirmed. In this article, the practical pitfalls of BOLD-fMRI and potential correction algorithms are concisely discussed.
Neuronal fibers, especially axonal fibers, have empirically been implicated in the generation of the observed anisotropism in apparent diffusion within the nervous system. Fast axonal flow is believed to play a main, if not sole, role in producing this observed anisotropy. Given that apparent diffusivity follows a Gaussian distribution, "averaged" diffusion length may be calculated as a function of diffusion coefficient (eigenvalue) and diffusion time. This method can be developed into a non-invasive imaging technique for obtaining an estimate of fast axonal flow in humans, in vivo. In this lecture, one of such technique, magnetic resonance axonography (MRX) is briefly described.