Transient temporal lobe changes and a novel mutation in a patient with Menkes disease.
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Codon, Nonsense↗
Biomedical subjects
S Noma
Publications and source records attributed to S Noma.
[Occurrence of BOOP outside radiation field after radiation therapy for small cell lung cancer].
We report a case of bronchiolitis obliterans organizing pneumonia (BOOP) that occurred outside the radiation field after radiation therapy for small cell lung cancer. A 74-year-old woman received chemotherapy and a total of 60 Gy of radiation therapy to the right hilum and mediastinum for small cell carcinoma of the suprahilar area of the right lung. Radiation pneumonitis developed within the radiation port 3 months after the completion of radiation therapy. She complained of cough and was admitted 7 months after completion of the radiation therapy. Chest radiography and computed tomography demonstrated peripheral alveolar opacities outside the radiation field on the side contralateral to that receiving the radiation therapy. Bronchoalveolar lavage showed that the total cell count was increased, with a markedly increased percentage of lymphocytes. Transbronchial lung biopsy revealed a histologic pattern consistent with BOOP. Treatment with corticosteroids resulted in rapid improvement of the symptoms and complete resolution of the radiographic abnormalities of the left lung. Although some cases of BOOP following radiation therapy for breast cancer have been reported, none of BOOP after radiation therapy for lung cancer have appeared in the literature.
Aged↗
Zonisamide - induced urinary lithiasis in patients with intractable epilepsy.
We report here three patients with intractable epilepsy who developed urinary lithiasis during zonisamide (ZNS) treatment. Abdominal pain due to left-sided hydronephrosis was the initial symptom in the first patient, and it was resolved after the excretion of a stone. The second patient, who had no specific symptoms, was found to have a thick sludge of calcium phosphate in the bladder when he suffered from aspiration pneumonia and dehydration. The third patient, who had a history of recurrent urinary obstruction, was also found to have a thick sludge of calcium oxalate in the bladder. The urinalysis of the three patients revealed alkaline urine and hypercalciuria. Although their urinary lithiasis was resolved by discontinuation of ZNS and supportive therapy, routine examination of urine parameters such as pH and sediments, and daily urine-output checks are thought to be necessary during treatment with ZNS, especially for patients who are bedridden for a long time and receive multiple antiepileptic drugs.
Adolescent↗
Acute disseminated encephalomyelitis associated with poliomyelitis vaccine.
A 6-year-old female patient with acute disseminated encephalomyelitis associated with poliomyelitis vaccine virus is reported. She had a history of high fever, headache, and gait disturbance. Neurologic examination confirmed spastic triparesis, urinary incontinence, diminution of tactile sensation, and vision deterioration. Hemography, serum laboratory findings, and urinalysis were normal. The cerebrospinal fluid was clear, with normal pressure, 9 leukocytes/mm(3), and 27 mg/dL protein, but the myelin basic protein was elevated to 10.7 ng/mL. T(2)-weighted magnetic resonance imaging disclosed multifocal high-intensity lesions of the spinal cord. The serum polio virus type 2 antibody titer was raised in the acute phase, and polio vaccine virus type 2 was detected in viral cultures of the cerebrospinal fluid and pharynx swab and had undergone an A-G neurovirulence mutation at nucleotide 481. Finally, she had human leukocyte antigen (HLA)-Cw3 and HLA-DR2, to which multiple sclerosis is related in Japan. Thus the cause of ADEM may have been related to her HLA type.
Child↗
[Occurrence of BOOP outside radiation field after tangential radiation therapy for breast carcinoma].
We report three cases of bronchiolitis obliterans organizing pneumonia (BOOP) that occurred outside the radiation field after radiation therapy using tangential fields for breast carcinoma. All patients complained of a cough between 14 and 20 weeks after completion of radiation therapy. Fever also developed in two of the three. Chest radiography and computed tomography demonstrated peripheral alveolar opacities outside the radiation field on the same side as the radiation therapy. Laboratory data showed an increased level of C-reactive protein and an increased erythrocyte sedimentation rate. Bronchoalveolar lavage showed an elevated total cell count with a very high percentage of lymphocytes. Transbronchial lung biopsy revealed a histologic pattern consistent with BOOP. Treatment with corticosteroids resulted in rapid clinical improvement and complete resolution of the radiographic abnormalities. This pulmonary disorder appears to be induced by radiation, especially when a tangential field is employed for breast carcinoma, though the etiology has not been fully investigated. It is important to be aware of this type of pulmonary complication in patients given radiotherapy for breast carcinoma.
Breast Neoplasms↗
[Myositis and rhabdomyolysis with influenza infection].
Myositis and rhabdomyolysis with influenza are rare, but sometimes serious complications. Patients with myositis more commonly have influenza B infection than influenza A. On the other hand, rhabdomyolysis are more frequently recognized in patients with influenza A infection than those with influenza B. Upper respiratory symptoms usually precede myositis, while rhabdomyolysis occurs simultaneously or shortly after the respiratory symptoms. Creatine kinase levels are elevated in myositis mildly and in rhabdomyolysis markedly. Influenza myositis improve spontaneously within 6 weeks, but influenza rhabdomyolysis sometimes induce renal failure with fatal outcome. Although the true incidence of myositis and rhabdomyolysis in the influenza infection remains unknown, careful medical care is necessary when patients have muscle pain and weakness.
Adolescent↗
[A case of Pryce type I intrapulmonary sequestration].
A twenty-year-old asymptomatic man hospitalized because of a vascular murmur and abnormal shadow in the left lower lung on X-ray film. An aortogram revealed an abnormal artery arising from the descending thoracic aorta and supplying the left basal segment, which had no other pulmonary arteries. Although lung ventilation scintigraphy demonstrated reduced ventilation to the left lower lobe, bronchogram showed an almost normal bronchial tree except that peripheral branches were slightly thin. A clinical diagnosis of Pryce type I intrapulmonary sequestration was made, and left lower lobectomy was performed successfully. We have analyzed 31 cases of Pryce type I intrapulmonary sequestration in Japan. A vascular murmur is often heard, and a chest X-ray usually shows either a mass shadow or increased vascular markings. In most of those cases, an abnormal artery arises from the descending thoracic aorta and it supplies the left basal segment. Because this type of sequestration causes hemoptysis and infections, surgical intervention is indicated.
Adult↗
[Successful removal of a left ventricular fibroma in a 10-year-old patient].
A 10-year-old girl with negative T wave in leads I, II, III, aVF, and V2-6 in the ECG was examined. The two-dimensional echocardiogram and the MRI revealed that she had a tumor in the free wall near the apex of the left ventricle. We removed the intramural fibroma (50 x 35 x 30 mm) of the left ventricle under cardiopulmonary bypass very carefully not to perforate the left ventricular cavity. After the removal, the defect was repaired by the sutures of the myocardial layers so that the volume inside the left ventricle could be kept and its shape could be maintained. She has been doing well without any trouble for 5 months after the operation.
Cardiac Surgical Procedures↗
Clinical role for a superantigen in Yersinia pseudotuberculosis infection.
Yersinia pseudotuberculosis is an enteric pathogen that causes a variety of clinical symptoms in the human. Recently, we reported the production of a superantigen (Y. pseudotuberculosis-derived mitogen, YPM) by this organism and characterized the gene structure of ypm. To further study the potential pathogenic role of YPM in Y. pseudotuberculosis infection, we assayed IgG anti-YPM antibodies and T cell antigen receptor-Vbeta expression of the T cells in peripheral blood and in mesenteric lymph node in patients acutely infected with Y. pseudotuberculosis. 20 out of 33 patients (61%) had an elevated antibody titer compared with healthy controls (P = 0.0001). Patients with systemic symptoms such as lymphadenopathy, transient renal dysfunction, and arthritis had significantly higher titers of anti-YPM than patients with gastrointestinal tract symptoms alone. T cells bearing the Vbeta3 gene segment were significantly increased (P = 0.009) among acute phase patients compared with healthy children. During the convalescence phase of the illness, there was a reduction in the abnormal level of Vbeta3 T cells. Moreover, in the mesenteric lymph node, an elevated level of Vbeta3 T cells compared with peripheral blood and a sequence diversity in the junctional region of the T cell antigen receptor beta-chain containing Vbeta3 element was observed in one patient. Together, these findings suggest that YPM was produced in vivo and played an important role in the pathogenesis of Y. pseudotuberculosis infection.
Adolescent↗
Autonomic nervous system activity in essential hypertension: a comparison between dippers and non-dippers.
The present study was undertaken to investigate the changes in autonomic nervous system activity in essential hypertension. Fourteen normotensive controls and 33 age-matched untreated hypertensive subjects, diagnosed by ambulatory blood pressure (ABP) measurement (24-h systolic ABP value over 140 mm Hg or 24-h diastolic ABP over 90 mm Hg, or both) were recruited. ABP and 24-h electrocardiogram were monitored simultaneously. Power spectral analysis of the R-R interval was performed by a fast Fourier transformation method and the powers of low frequency (LF; 0.04 to 0.15 Hz) and high frequency (HF; 0.15 to 0.4 Hz) components were obtained. Hypertensive subjects were divided into 'dippers', whose night-time systolic ABP fell by more than 10% of their daytime ABP, and 'non-dippers' in whom this phenomenon was absent. In hypertensive subjects, electrocardiogram monitoring and power spectral analysis were also performed for 5 min before and during 90 degrees tilt. There were no significant differences in the 24-h mean LF/HF power ratio, LF power or HF power between normotensive and hypertensive subjects. A significant negative correlation between the night-time systolic ABP level and the 24-h LF/HF power ratio was found (r= -0.36, P < 0.05) in the hypertensive subjects. A significant positive correlation was found between the 24-h LF/HF power ratio and the percentage nocturnal reduction of the daytime systolic ABP in hypertensive subjects (r = +0.40, P < 0.01). The 24-h LF/HF power ratio was significantly lower in non-dippers than in dippers (2.09 +/- 1.06 vs 3.24 +/- 0.97, P < 0.01). The mean daytime LF/HF power ratio was significantly lower in non-dippers than in dippers (2.50 +/- 1.43 vs 4.08 +/- 1.27, P < 0.01). The night-time LF/HF power ratio was not significantly different between the two groups. The LF/HF power ratio increased significantly in dippers (from 1.32 +/- 1.95 to 4.65 +/- 1.54, P < 0.001) during 90 degrees tilt, but there was no significant change in the LF/HF power ratio in non-dippers during tilt (from 1.13 +/- 0.28 to 1.36 +/- 0.78, NS). The 24-h LF/HF power ratio decreased according as the night-time systolic BP elevated in hypertensive subjects. During ambulatory monitoring, the non-dippers showed a significantly lower LF/HF power ratio than the dippers. The LF/HF power ratio increased significantly in dippers, but not in non-dippers during tilting. These results suggest that impaired cardiovascular reflexes might contribute to the decreased sympathovagal balance in non-dipper type hypertension.
Autonomic Nervous System↗
A Japanese boy with Young-Simpson syndrome.
In 1987 Young and Simpson reported a child with hypothyroidism, a congenital heart disease, severe mental retardation and striking facial dysmorphism, including microcephaly, blepharophimosis, bulbous nose, thin lip, low-set ears and micrognathia. This study presents an 8-month-old boy with virtually identical features to those in Young and Simpson's original case. The patient is a sporadic case and his parents are unrelated and phenotypically normal, hence the family data corresponds to any mode of inheritance. This is the first male case reported in the world and the first in Orientals.
Abnormalities, Multiple↗
Pulmonary involvement in polymyositis and dermatomyositis: sequential evaluation with CT.
OBJECTIVE: The characteristic findings of pulmonary involvement in polymyositis (PM) or dermatomyositis (DM) and the change in findings before and after treatment were evaluated with sequential high-resolution CT studies. MATERIALS AND METHODS: CT images of pulmonary involvement in 19 patients with PM or DM were reviewed. During a period of 2-61 months, 17 of these patients underwent sequential CT before and after treatment with corticosteroids, immunosuppressants, or both. RESULTS: Findings of the initial CT studies included pleural irregularities and prominent interlobular septa (n = 19), ground-glass attenuation (n = 19), patchy consolidation (n = 19), parenchymal bands (n = 15), irregular peribronchovascular thickening (n = 15), and subpleural lines (n = 7). Honeycombing was not detected on any CT images. These findings were more remarkable in the lower portion and the subpleural area of the lungs. In 16 of the 17 patients who underwent sequential CT conditions such as patchy consolidation, parenchymal bands, and irregular peribronchovascular thickening improved, becoming pleural irregularities and prominent interlobular septa, ground-glass attenuation, and subpleural lines on follow-up CT scans. CONCLUSION: Consolidation with patchy and subpleural distribution, parenchymal bands, and irregular peribronchovascular thickening were characteristic and reversible CT findings in patients with PM or DM.
Adult↗
[Body-related psychotherapy for anorexia nervosa--treatment at the psychosomatic hospital in Germany].
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Adult↗
Lack of potentiation with felbinac patch on the convulsive toxicity of enoxacin in rats.
We investigated the possible potentiation of the convulsive toxicity of enoxacin (ENX) by the concomitant topical application of a felbinac (FLB) patch in rats. A felbinac patch (Seltouch; 0.5%, 3 cm x 4 cm) was attached on the back of rats where their hair has been removed. ENX was infused from the left jugular vein at 8 h after the application of FLB patch under an unanesthetized and unstrained condition. Blood, CSF and brain samples were collected at the occurrence of convulsion, and ENX concentrations of each part were determined. As a result, no significant potentiation by FLB patch was found in the onset time of convulsion or in the ENX concentration of each part. Moreover, based on the assumption that there are no inter-species differences in ENX concentration in the brain at the occurrence of a convulsion (Cbr), the predicted plasma ENX concentration required to elicit convulsions in humans, which was estimated from the Cbr and Kp value of ENX in the brain of rats, was 20 times higher than the therapeutic plasma level.
Administration, Cutaneous↗
[Initial outcome of coronary intervention in elderly patients].
The effect of age on the early results of coronary intervention was examined retrospectively using the initial success rate of coronary angioplasty in 60 patients older than 70 years. Patients were selected from those who underwent coronary angioplasty at the Saiseikai Utsunomiya Hospital from January 1992 to December 1994. There were 267 patients with 350 lesions, 223 men and 44 women, aged from 31 to 79 years (mean age 61.4 +/- 9.8 years). The elderly group consisted of 60 patients (mean age 73.5 +/- 3.0 years) and the control group was 207 patients less than 70 years (mean age 57.9 +/- 8.2 years). Successful coronary dilatation was defined as > 20% reduction of stenosis with residual stenosis < 50%. Body mass index, presence of hypertension or diabetes mellitus and the ratio of smokers were not significantly different between the two groups. The elderly group included more women and patients with hyperlipidemia. Both groups had similar baseline extent of coronary artery disease, distribution of coronary artery stenosis type and left ventricular ejection fraction. The initial success rate for all patients was 93.4%. There was no significant difference in the initial success rate between the elderly and control groups (98.8% and 91.9%, respectively). There was no significant difference in major complication rate between the two groups (6.3% and 11.5%). Multivariate logistic regression analysis showed that only the type of coronary artery stenosis was significantly associated with initial success rate (p < 0.01). These results suggest that coronary intervention can be successfully performed with a low incidence of major complication in elderly patients.
Adult↗
Carmofur-induced leukoencephalopathy: MRI.
Carmofur, a derivative of 5-fluorouracil, has recently been noted to have an infrequent but serious association with leukoencephalopathy. To our knowledge, there has been no report of early MRI findings in this leukoencephalopathy. We describe a case in which diffuse high signal intensity of the entire cerebral white matter, including the corpus callosum, was seen on T2-weighted magnetic resonance images. Although similar findings can be seen in many other diseases, carmofur-induced leukoencephalopathy should be suspected in a patient treated with carmofur. It is important to know the clinical and MRI characteristics of this condition, for early diagnosis and better prognosis.
Antineoplastic Agents↗
Serial changes of cystic air spaces in fibrosing alveolitis: a CT-pathological study.
In patients with cryptogenic fibrosing alveolitis (CFA), subpleural large cysts are sometimes seen within honeycombing on computed tomography (CT). These cysts may be interpreted as bullae and the coexistence of emphysema with CFA might be assumed. We evaluated whether cystic spaces in honeycombing become larger in patients with CFA on serial CT scans. CT scans were obtained in 16 patients with CFA, who had undergone two CT examinations (6 to 43 months, mean 25.1 months apart), were reviewed focusing on the changes in size of the cystic spaces in honeycombing. The mechanism of enlargement of cystic spaces was investigated using microscopic serial sections of autopsied specimens. In 15 of 16 patients, the cystic spaces in honeycombing were larger on the second examination. Histologically, stenosis of bronchioles and slit-like structures between cysts and bronchioles were detected in addition to alveolar septal dissolution and bronchiolectasis. Both the bronchiolar stenosis and the slit-like gaps have the potential to work as check-valves and increase the volume of cystic spaces. Therefore, in the evaluation of CFA, large cysts should be interpreted with caution to avoid misinterpretation of the cysts as bullae associated with emphysema. From the results of this correlative study, we suggest that a check-valve effect is an important mechanism in the formation of large cysts in honeycomb lung.
Aged↗
Acute necrotising encephalopathy of childhood: a new syndrome presenting with multifocal, symmetric brain lesions.
The clinicopathological features of a previously unrecognised type of acute encephalopathy prevalent among Japanese children is described by reviewing the records of 13 consecutive patients treated and 28 previously reported cases. The hallmark of this encephalopathy, proposed to be a novel entity termed acute necrotising encephalopathy of childhood, is multiple, necrotic brain lesions showing a symmetric distribution. The encephalopathy was noted in previously healthy children after respiratory tract infections, with presenting symptoms of coma, convulsions, vomiting, hyperpyrexia, and hepatomegaly. Laboratory examinations disclosed liver dysfunction, uraemia, and hypoproteinaemia. The histological appearance of the liver was variable and non-specific. Cerebrospinal fluid contained an increased amount of protein. Computed tomography and MRI showed the presence of symmetrically distributed brain lesions of the thalamus, cerebral white matter, brainstem, and cerebellum. Necropsy examination confirmed extensive fresh necrosis of these regions with evidence of local breakdown of the blood-brain barrier. Based on the characteristic combination of clinical and pathological findings, acute necrotising encephalopathy of childhood can be distinguished from previously known encephalopathies, including Reye's syndrome.
Acute Disease↗