Search PubMed⌕ Search

Biomedical subjects

N Shimozawa

Publications and source records attributed to N Shimozawa.

160 records · Page 9Linked to original sources
PubMed1986

The syndrome of the absence of a septum pellucidum with porencephaly.

Two cases of a complex brain malformation including the absence of a septum pellucidum and bilateral porencephaly were reported and compared with Aicardi's cases. The two cases have congenital hemiplegia or double hemiplegia and mental retardation, and one of them has optic atrophy. CT scans revealed the absence of a septum pellucidum and a specific position and shape of the clefts. The syndrome of the absence of a septum pellucidum and porencephaly may represent a specific type of true porencephaly, and the cause must occur before 16 weeks of gestation.

Adolescent↗
PubMedDate not supplied

A case of pseudo-Zellweger syndrome with a possible bifunctional enzyme deficiency but detectable enzyme protein. Comparison of two cases of Zellweger syndrome.

Three infants with peroxisomal disorders were investigated clinicobiochemically and neuroradiologically. Two had classical Zellweger syndrome, and cranial CT scans showed typical disproportionate enlargement of the occipital horns of the lateral ventricles (colpocephaly) with marked hypodensity of the white matter. In one female infant, although the clinical findings were similar to those in Zellweger syndrome, some findings, such as elevated transaminase levels, liver fibrosis, the absence of renal cortical cysts and colpocephaly, were negative or milder. Biochemical analyses revealed increased very long-chain fatty acids, dicarboxylic aciduria and impaired beta-oxidation of lignoceric acid. However, peroxisomes were abundantly present in hepatocytes and cultured fibroblasts, and all peroxisomal beta-oxidation enzyme proteins were detected on immunoblot analysis. A cell fusion study suggested that the enzyme responsible for this case of 'pseudo-Zellweger syndrome' is bifunctional.

Cerebral Ventriculography↗
PubMedDate not supplied

Effects of erucic acid therapy on Japanese patients with X-linked adrenoleukodystrophy.

Ten Japanese boys with childhood adrenoleukodystrophy (ALD), one adult patient with adrenomyeloneuropathy (AMN), and two presymptomatic ALD boys were treated with dietary erucic acid (C22:1) for more than 12 months; except in a case of childhood ALD patient who died 7 months after beginning erucic acid therapy. During erucic acid therapy, the serum levels of very long-chain fatty acid (VLCFA) (C24:0/C22:0) decreased within 1-2 months in all patients, and these levels in four of the patients decreased to the normal range. Neurological examination and MRI findings in all 10 of the childhood ALD patients showed progression of the disease while they were receiving the dietary therapy. However, the mean interval between the onset of awkward gait and a vegetative state in diet-treated patients was significantly longer than that in the untreated patients. One AMN patient showed slight improvement of spastic gait and lessened pain in the lower limbs due to spasticity. The two presymptomatic ALD boys remained intact on clinical examination and on MRI findings for 38 and 23 months, respectively, after starting the diet.

Adolescent↗
PubMedDate not supplied

Short latency somatosensory evoked potentials and 99mTc-HMPAO SPECT in a case of flunarizine-effective alternating hemiplegia in infancy.

Short latency somatosensory evoked potentials (SSEPs) and 99mTc-hexamethylpropylene amine oxime single photon emission computed tomography (99mTc-HMPAO SPECT) were examined in a patient with alternating hemiplegia in infancy (AHI) before and after flunarizine treatment. The low amplitude and elongation of the latency time at the C'4 level of the interictal SSEPs before treatment were ameliorated by flunarizine administration. The cerebral hypoperfusion in the right hemisphere observed on 99mTc-HMPAO SPECT before treatment improved, as seen by normal and symmetrical imaging, after flunarizine treatment. These observations suggest that AHI is caused by a functional disturbance in the cerebral area contralateral to the hemiplegia, and that SSEPs and 99mTc-HMPAO SPECT are useful for evaluating functional disturbance in the contralateral motoneurons and the effect of flunarizine treatment in AHI patients.

Evoked Potentials, Somatosensory↗
PubMedDate not supplied

Prenatal diagnosis of peroxisomal disorders. Biochemical and immunocytochemical studies on peroxisomes in human amniocytes.

Prenatal diagnoses of peroxisomal disorders, including peroxisome-deficient Zellweger syndrome, isolated deficiency of peroxisomal beta-oxidation enzyme and rhizomelic type chondrodysplasia punctata were investigated by means of the lignoceric acid oxidation assay, indirect immunofluorescence staining and pulse-chase experiments, using cultured amniocytes. Assessment of peroxisomal beta-oxidation activity by means of [1-14C]lignoceric acid oxidation is essential for the diagnosis of a single enzyme deficiency of peroxisomal beta-oxidation with detectable enzyme protein. For the diagnosis of Zellweger syndrome, the absence of peroxisomes was readily determined by immunofluorescence staining of only a few amniocytes. Evidence for abnormal processing of 3-ketoacyl-CoA thiolase leads to the diagnosis of rhizomelic chondrodysplasia punctata. All the fetuses were considered to be normal and the neonates were normal. Use of these methods requires only a small number of amniocytes and will facilitate the prenatal diagnosis of peroxisomal disorders.

Acetyl-CoA C-Acyltransferase↗
PubMedDate not supplied

Ataxia telangiectasia associated with B-cell lymphoma: the effect of a half-dose of the drugs administered according to the acute lymphoblastic leukemia standard risk protocol.

Ataxia telangiectasia (A-T) is a rare autosomal recessive disorder characterized by cerebellar ataxia, oculocutaneous telangiectasia, and variable degrees of humoral and cellular immunodeficiency. Affected individuals are known to exhibit a high incidence of lymphoma and leukemia. Because of increased chemosensitivity, the treatment of A-T patients with malignancies requires extremely careful planning and caution with respect to the use of chemotherapy. The authors report on a 12-year-old boy with A-T who developed B-cell lymphoma. He received a half-dose of the drugs administered according to the acute lymphoblastic leukemia (ALL) protocol issued by our children's cancer study group (9104 Standard Risk Protocol, Tokai Pediatric Oncology Study Group). As a result, he continues to be in complete remission and free of treatment complications 32 months after the diagnosis of B-cell lymphoma.

Antineoplastic Combined Chemotherapy Protocols↗
PubMedDate not supplied

A case of Zollinger-Ellison syndrome produced by gastrinoma in the duodenum accompanied with multiple endocrine neoplasia type 1.

A case of Zollinger-Ellison syndrome produced by gastrinoma in the duodenum accompanied by multiple endocrine neoplasia type-1 (MEN-1) is reported. A 46 year-old female underwent distal gastrectomy due to gastric ulcer 5 years ago. As ulceration of the residual stomach recurred, further examination was performed. Hyperprolactinemia, hypergastrinemia, primary hyperparathyroidism, pancreatic tumor, and duodenal carcinoid were evident, and the diagnoses of Zollinger-Ellison syndrome and MEN-1 were established. The origin of the gastrin secretion was suspected to be from the pancreatic tumor, so sampling of the portal blood was performed. As lesion on the gastrinoma in the pancreas could not be identified, total parathyroidectomy was performed for primary hyperparathyroidism. The level of the gastrin secretion, however, remained high. Partial resection of the duodenum for the duodenal carcinoid and a distal pancreatectomy were carried out concurrently. Immunohistochemical study of the anti-gastrin antibody revealed duodenal tumor cells. Initially, the gastrinoma was thought to be in the pancreas, however, the lesion accompanied with MEN-1 and the Zollinger-Ellison syndrome had occurred in the duodenum.

Duodenal Neoplasms↗
PubMedDate not supplied

Effects of prostaglandin E1 administration during hepatectomy for cirrhotic hepatocellular carcinoma.

BACKGROUND/AIMS: Prostaglandin E1 has been used in hepatectomy based on a few limited clinical studies suggesting that PGE1 improves liver function. The aim of this study was to evaluate the effects of PGE1 administration during hepatectomy for cirrhotic hepatocellular carcinoma. METHODOLOGY: Forty-three patients undergoing hepatectomy for cirrhotic hepatocellular carcinoma were divided into 2 groups: hepatectomy with Prostaglandin E1 treatment (PG group; n = 19) and without Prostaglandin E1 treatment (control group; n = 24). Prostaglandin E1 (0.02-0.07 microgram/kg/min) was administered intravenously from beginning to end of surgery in the PG group. RESULTS: There were no significant differences between groups with respect to age, gender, preoperative liver and renal function, or intraoperative variables such as blood loss, weight of resected liver and total clamping time by the Pringle maneuver. No patient had severe postoperative complications. Initial postoperative maximum concentrations of serum total bilirubin, creatinine, and blood urea nitrogen in the PG group were significantly lower than those in the control group. CONCLUSIONS: Prostaglandin E1 administration during hepatectomy for cirrhotic heptocellular carcinoma resulted in improved renal and hepatic function.

Aged↗
PubMedDate not supplied

Suppression of IgA production by lymphocytes induced by diphenylhydantoin.

In this study, the mechanisms for suppression of immunoglobulin production by lymphocytes induced by diphenylhydantoin (DPH) were investigated using peripheral blood mononuclear cells from one patient 3 months after discontinuation of DPH, when IgA deficiency was improved. Immunoglobulin production in PBMCs was dose-dependently suppressed by DPH and the suppressive effect of DPH on IgA production tended to be greater than the effect on IgM or IgG production. Moreover, cell separation experiments indicated that DPH suppresses immunoglobulin production by PBMCs through a suppression of B cell, but not T cell, function. The finding that the suppressive effect of DPH on IgA production tended to be greater than the effect on IgM or IgG production might be related to the fact that the alpha-chain gene is located in the 3' end of the gene coding the constant regions of human heavy chains.

B-Lymphocytes↗
PubMedDate not supplied

Effects of the antiallergic drug azelastine hydrochloride on proliferative responses of lymphocytes to food antigens in patients with food-sensitive atopic dermatitis.

We previously reported that the proliferative responses of peripheral blood mononuclear cells (PBMCs) to ovalbumin (OA) or bovine serum albumin (BSA) in children with atopic dermatitis (AD) who are sensitive to hen's egg or cow's milk were significantly higher than those of healthy children and hen's egg- or cow's milk-sensitive children with immediate symptoms. Azelastine hydrochloride is an antiallergic drug used in the treatment of rhinitis and asthma. In this study, we have shown that the proliferative responses of PBMCs to OA are concentration-dependently inhibited by azelastine in patients with AD. Moreover, the inhibition resulted from the effects of azelastine on T-cells.

Animals↗
PubMedDate not supplied

Mixed hepatocellular carcinoma and cholangiocarcinoma treated by extended left hepatic lobectomy with resection of the right hepatic vein and preservation of the inferior right hepatic vein after hepatic arterial infusion chemotherapy.

We herein describe a patient with a giant mixed hepatocellular carcinoma and cholangiocarcinoma (MHC) surrounding the inferior vena cava (IVC). The patient was treated by extended left hepatic lobectomy with resection of the main right, left and middle hepatic veins and preservation of the inferior right hepatic vein (IRHV) after hepatic arterial infusion (HAI) chemotherapy. The patient died of distant metastases 4 years after initial HAI chemotherapy. As there is no hope of cure with HAI alone in the patients with MHC, this operative procedure even after HAI is recommended for patients with reduced liver function, tumor involving the RHV and surrounding the IVC.

Adult↗