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Biomedical subjects

Y Uesaka

Publications and source records attributed to Y Uesaka.

48 records · Page 3Linked to original sources

Transitory type of sphingomyelinase deficient Niemann-Pick disease: clinical and morphological studies and follow-up of two sisters.

The transitory type is a rare and ill-defined variant of sphingomyelinase deficient NPD. Here reported are full clinical and morphological studies on two sisters with the transitory type. Our cases have the following features in common: 1) prominent visceral involvement from early infancy, such as marked hepatosplenomegaly, pulmonary infiltration, and numerous characteristic foam cells in the bone marrow aspirates; 2) very low sphingomyelinase activities in cultured skin fibroblasts; 3) atypical cherry-red spots in the retina; despite 4) absence of any neurological symptoms even at the age of 5 years and 6 months, and 3 years and 9 months, respectively. Furthermore, a few lines of evidence indicating nervous system involvement are also disclosed, that is, 5) vacuolated macrophages in the cerebrospinal fluid and ultrastructurally typical inclusion bodies in axons and Schwann cells in the rectal biopsy specimens, which were firstly revealed in this type of NPD. It is stressed that every case with apparent type B NPD should be searched for the presence or absence of neuronal involvement by not merely fundoscopy but other available means as we employed, which would greatly contribute to elucidate the clinico-pathogenetical knowledge not to mention the current classification of NPD.

Bone Marrow↗

Lysozyme secreting tumor: a case of gastric cancer associated with myelofibrosis due to disseminated bone marrow metastasis.

One patient with gastric carcinoma and secondary myelofibrosis due to disseminated bone marrow metastasis had markedly elevated lysozyme (LZM) levels in serum and urine with the intense presence of LZM within tumor tissues. It is considered to be a case of gastric carcinoma with LZM secreting functional capacities. To date, there were many reported cases to verify the LZM positive cells by LZM staining in the tissue of gastric carcinoma and to demonstrate the elevated serum levels of LZM in malignant tumor bearing patients, whereas no papers to disclose the elevated levels of LZM in serum and urine originated from the productions and secretions of gastric carcinoma cells. So, this report might be the first reported case of LZM secreting tumor verified by LZM staining of carcinoma cells except for haematological malignancies such as acute monocytic leukemia or myelomonocytic leukemia.

Adenocarcinoma↗

Production of cholera-like enterotoxin by Aeromonas hydrophila.

A total of 249 strains of mesophilic Aeromonas including 179 A. hydrophila and 70 A. caviae were tested for production of cholera-like enterotoxin by reversed passive latex agglutination (RPLA) assay. A cholera-like enterotoxin neutralized with cholera antitoxin was demonstrated in the culture filtrates from eight (4.5%) of the 179 A. hydrophila strains, while none of A. caviae strains revealed the enterotoxin production in the test. Production of the cholera-like enterotoxin in the eight strains of A. hydrophila was also confirmed by enzyme-linked immunosorbent assay (ELISA).

Aeromonas↗

Renal involvement patterns of amyloid nephropathy: a comparison with diabetic nephropathy.

Thirty kidney specimens obtained from 26 patients with intrarenal amyloid deposition were evaluated. The deposited amyloid belonged to AL protein in 15 cases and AA protein in 11. The intrarenal distribution was examined. Intraglomerular nodular pattern showed a wider extraglomerular distribution of amyloid with a florid accumulation along the vasa recta in the outer medulla, whereas intraglomerular diffuse pattern had amyloid deposition confined mainly to a narrow range surrounding the glomeruli. However, no essential difference was appreciated between AL and AA proteins in the intrarenal distribution, although the former more often disclosed the diffuse pattern. There were many morphological expressions resembling diabetic nephropathy; in addition to the diffuse and nodular pictures, capsular drop-like deposits along Bowman's capsule, simultaneous deposition in the afferent and efferent arterioles, glomerular microaneurysm of early stage, and accumulation along the tubular basement membrane were observed in amyloid nephropathy. Thus, the spicule formation in renal amyloidosis and the diffuse thickening of glomerular and tubular basement membranes in the diabetic emerged as the respective specific lesion. The implicated pathogenesis of diabetic nephropathy was also discussed.

Adult↗

Group I pepsinogen for early detection of gastric cancer recurrence after total gastrectomy.

Group I pepsinogen (PG-I) staining was performed in the gastric carcinoma tissues of 75 patients by the peroxidase-antiperoxidase (PAP) method, 44 cases (59%) of which were positive for PG-I, suggesting that they were PG-I-producing gastric carcinomas. Type IV gastric carcinoma by the Borrmann classification and/or poorly-differentiated adenocarcinoma were positive for PG-I in high incidence. Serum and urinary levels of PG-I were determined by the Pepsinogen I Radioimmunoassay Kit in patients with gastric carcinoma who underwent total gastrectomy. The levels of PG-I declined remarkably or disappeared at 1 week after curative surgery. Changes of serum PG-I levels after total gastrectomy were observed in 9 patients with PG-I-producing gastric carcinomas, 7 of whom died of recurrence. The PG-I values became elevated with recurrence in 5 of them and the values increased with the passage of time. In contrast, no substantial changes in PG-I levels occurred in patients with no recurrence. These results suggest that PG-I is useful for the early detection of recurrent disease after total gastrectomy in patients with PG-I-producing gastric carcinomas.

Adenocarcinoma↗

[Acute compression neuropathy of the proximal sciatic nerve in a patient with facioscapulohumeral muscular dystrophy].

We reported a 58-year-old man with facioscapulohumeral muscular dystrophy who had acute compression neuropathy of the sciatic nerve after prolonged sitting position. MRI and percutaneous electric stimulation studies were usefull for the diagnosis. Gd-DTPA T1-weighted MRI images showed the enhanced right sciatic nerve at the level of gluteus maximus muscle. An evoked potential study by a percutaneous electric stimulation revealed evidence of a conduction block at the same level. After methylprednisolone pulse therapy, muscle weakness and sensory disturbance improved. Acute compression neuropathy of the sciatic nerve may be seen as a complication in the advanced stage of (FSH) muscular dystrophy.

Acute Disease↗

Pathophysiology of sensorimotor cortex in cortical myoclonus.

We have studied greatly enhanced cortical responses to somatosensory stimuli [giant somatosensory evoked potential (SEP) or magnetic field (SEF)] and cortical activities preceding the myoclonus (premyoclonus spike) in patients with cortical myoclonus, using magnetoencephalographic (MEG) techniques. A P1m component of giant SEF was estimated as a dipole positioned on the postcentral gyrus in all giant SEFs. This indicates that abnormally enhanced responses to sensory stimuli originate from the sensory cortex. In most of the patients with cortical reflex myoclonus, a dipole for the premyoclonus spike was also localized on the postcentral gyrus, which suggests that abnormal activation of the sensory cortex produces the spontaneous myoclonus. In one patient with galactosialidosis, two dipoles, one on the postcentral and the other on the precentral gyrus, could reasonably explain the premyoclonus spike. In the other patient with cortical reflex myoclonus, the premyoclonus spike associated with jerks of the lower limb muscle was estimated to be one dipole on the superior frontal gyrus just anterior to the paracentral lobule. This suggests that both spontaneous myoclonus and enhanced long loop reflex are generated by abnormal activation of the motor cortex in this patient. In a patient with epilepsia partialis continua who had no long loop reflexes, the premyoclonus spike was demonstrated to be positioned on the precentral gyrus, which indicates that abnormal activation of the motor cortex causes the spontaneous myoclonus in this patient. Our MEG studies demonstrated that abnormalities of the sensory or motor cortices contribute differently to the generation of myoclonus in cortical myoclonus, despite the fact that the sensory cortex is the main contributor in most of them.

Electric Stimulation↗

Primary malignant lymphoma of the duodenum: report of a case.

We present a case of long-term survival in primary malignant lymphoma of the duodenum treated with a curative surgical resection and post-operative chemotherapy. A 72 year-old man was admitted to our hospital complaining of epigastric discomfort. Endoscopy revealed an ulcerative tumor in the duodenal bulb. The tumor was diagnosed histologically as malignant lymphoma by endoscopic biopsy. A distal gastrectomy and duodenal bulb resection were performed with lymph nodes dissection. The tumor was histologically classified as B-cell phenotype, large cell, immunoblastic lymphoma of high grade, according to the Working Formulation. The patient received CHOP (cyclophosphamide, doxorubicin, vincristine and prednisone) chemotherapy after the operation, and he has no sign of recurrence during a follow-up period of 6 years.

Aged↗