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Biomedical subjects

K Misugi

Publications and source records attributed to K Misugi.

At least 91 records · Page 5Linked to original sources
PubMed1984

Kawasaki disease complicated with mitral insufficiency. Autopsy findings with special reference to valvular lesion.

A case of mucocutaneous lymph node syndrome (Kawasaki disease, MCLS) complicated with mitral insufficiency is reported. This patient showed severe valvulitis, which was thought to be the main cause of mitral regurgitation. Two other patients with MCLS who did not present clinical signs of mitral insufficiency revealed the presence of mild valvulitis. The morphological alterations of cardiac valves were non-specific and mainly consisted of inflammatory infiltration, increment of fibrous connective tissue, and proliferation of small capillaries. In addition to the coronary aneurysms, the involvement of cardiac valves should receive attention as the sequelae of MCLS.

Aneurysm↗
PubMed1983

Hepatitis B surface antigen positive hepatocellular carcinoma in children. Report of a case and review of the literature.

This report describes a case of adult type hepatocellular carcinoma (HCC) of a ten-year-old boy. The liver showed no cirrhosis but hepatitis B surface antigen (HBs-antigen) was demonstrated in the non-neoplastic liver cells. Serological examination of his family revealed positive HBs-antigen of the patient's mother suggesting familial transmission to the patient in his early life. Histologic demonstration of HBs-antigen was performed on 36 adult HCC cases and 14 childhood cases including three HCC and eleven hepatoblastoma. HBs-antigen was demonstrated in 12 adult cases, one of which showed no cirrhosis. All childhood cases except for the one presented here showed no HBs-antigen. Review of the literature disclosed four other cases of HBs-antigen positive HCC in children. The present case is unique in that the non-neoplastic portion of the liver showed neither hepatitic nor cirrhotic manifestations. These HBs-antigen positive HCC in childhood may imply the significant relationship between HB virus and HCC not only in adult but also in childhood.

Carcinoma, Hepatocellular↗
PubMed1983

Congenital heart anomalies in the trisomy 18 syndrome, with reference to congenital polyvalvular disease.

Congenital polyvalvular disease (CPVD) is seen in trisomy 18 and other aneuploidy syndromes. However, its extent and nature have not been studied. Gross pathologic and histologic aspects of the heart were studied in 15 autopsied cases of trisomy 18. All had CPVD; other congenital defects included membranous ventricular septal defect (87%), patent ductus arteriosus (73%), and high takeoff of the right coronary ostium (80%). With a scoring system, histologic findings of the valves of all trisomy 18 cases were compared with those of 30 normal hearts of comparable age in order to determine the degree of morphologic abnormality. This included the presence of blood cysts, derangement of the spongiosa and fibrosa, vascular degeneration of the spongiosa, and defective elastic fibers. There were distinct differences between the changes seen in CPVD with trisomy 18 syndrome and those seen in the normal individuals. The most severe changes were present in the tricuspid and mitral valves with derangement of the spongiosa and fibrosa and defective elastic fibers. The valve tissue had a similar histologic appearance and structure to that of low birth weight infants (gestational age, 25 weeks). The valvular changes observed therefore are of fetal type and represent errors in tissue differentiation occurring as last as the third trimester.

Chromosomes, Human, 16-18↗
PubMed1983

A neuropathologic study of Werdnig-Hoffmann disease with special reference to the thalamus and posterior roots.

The brains of five cases of severe infantile form of Werdnig-Hoffmann (W-H) disease were studied to observe the pathologic changes of sensory neurons and the thalamus. The present study disclosed severe cell loss, chromatolytic degeneration, and empty cell beds of the spinal anterior horn and cranial motoneurons (V, VII, X, XII). Glial bundles were also noted in the anterior roots. In the sensory systems, glial bundles in the posterior roots (2/5), ghost cells in Clarke's column (2/5), and degeneration of the thalamus, mainly in the lateral formation (4/4) were noted. It was demonstrated that not only degeneration of lower motor neurons and glial bundles in the anterior roots, but also degeneration of sensory neurons and thalamus were present in W-H disease. These findings suggested the possibility that W-H disease is a multisystemic disease involving both the anterior and posterior root systems. No sensory involvement was found clinically. Characteristic wrist drop was observed in four cases, two of which also having motor nerve conduction velocity (MCV) delay. On the other hand, MCV of another case without wrist drop was normal. The possibility that wrist drop might be one of the clinical features of peripheral nerve dysfunction was discussed, but further pathologic evaluation of peripheral nerves is needed.

Female↗
PubMed1982

Cloverleaf skull syndrome. An autopsy case and review of literature.

An autopsy case of cloverleaf skull deformity associated with hydrocephalus, systemic skeletal malformation including facial dysostosis, fused elbow, syndactyly of the toes, odd digits and striking anomaly of tracheal cartilage is presented. Extra-skeletal abnormalities included covered anus, dermal sinus and the absence of corpus callosum and the septum pellucidum. Several basal skull deformity appeared to be a primary morphologic alteration associated with premature closure of the specific sutures, which terminally resulted in life-threatening hydrocephalus. Histological investigation showed the abnormalities of endochondral ossification in the cartilage at the epi- and meta-physis of the fused elbows. In addition, electron microscopical study revealed unusual fat droplet-containing chondrocytes even in the resting and multiplicative phase. Association of the tracheal anomaly with this syndrome was disclosed in this case. Generalized bone and cartilage abnormalities not only in the skeletal system but also in the internal organs strongly suggest that this disorder involves generalized osteocartilagenous system. The present case makes a total of 14 cases of the cloverleaf skull syndrome reported in the Japanese literatures to date. Major clinical and pathologic findings of these cases were summerized.

Abnormalities, Multiple↗
PubMed1980

Carcinoma of the prostate in childhood and adolescence: report of a case and review of the literature.

This paper reports a case of carcinoma of the prostate in an 11-year-old boy. The clinical findings were characterized by a mass in the prostatic region, extensive osteoblastic bone metastasis, and normal serum acid phosphatase. Autopsy demonstrated an undifferentiated tumor, which probably originated from the outer gland of the prostate. Metastases to the bones, liver, lungs, and the lymph nodes were present. Light and electron microscopic studies revealed undifferentiated neoplastic cell, which is in contrast to the usual adenocarcinoma in older individuals. Histochemical examination failed to demonstrate acid phosphatase activity within the tumor cells. The authors considered that this tumor probably originated from immature basal cells of the prostatic gland. Review of the literature disclosed 15 cases of carcinoma of the prostate in individuals under 21 years of age. These cases were also characterized by an undifferentiated appearance of tumor cells and normal serum acid phosphatase level.

Acid Phosphatase↗
PubMed1979

Lymphoma of the brain associated with polyposis of the colon--report of a case and review of Turcot's syndrome.

Pathological findings of a 15-year-old girl with polyposis of the colon who has subsequently developed primary lymphoma of the brain are reported. The authors consider this case to be a rare example of Turcot's syndrome although the histological typing of the brain tumor is not classical and familial background of the polyposis has not been demonstrated. Literatures on 14 previously reported cases of Turcot's syndrome are reviewed and clinical and pathological findings are summarized. The present case is the second report of Turcot's syndrome associated with lymphoma of the brain and is unique in regard to the age as cerebral lymphoma is extremely rare in the second decade.

Adolescent↗
PubMed1976

Hyperalaninemia hyperpyruvicemia and lactic acidosis due to pyruvate carboxylase deficiency of the liver; treatment with thiamine and lipoic acid.

A 16-month-old female infant with severe mental and motor retardation, clinically diagnosed as Leigh's encephalomyelopathy, forms the basis of this study. This infant was found to have lactic acidosis, low cerebrospinal fluid glucose, hyperalaninemia, and increased levels of urine lactate, pyruvate and alanine. These laboratory studies suggested an inborn error in gluconeogenesis. Further investigation revealed a low level of hepatic pyruvate carboxylase activity. The patient's elder sister who also had mental and motor deterioration was then also found to have an elevated blood lactate. These two siblings clinically and biochemically showed improvement with treatment consisting of thiamine and lipoic acid.

Acidosis↗
PubMed1975

Hypophosphatasia-study on two autopsy cases.

The autopsy findings of two cases of infantile hypophosphatasia are described and compared with those of 16 previously reported cases. Histochemical and biochemical tissue analysis for alkaline phosphatase showed a marked decrease in activity in liver, kidney, and bones. However, intestinal alkaline phosphatase possessed normal or slightly elevated activity. Nephrocalcinosis is a frequent complication and its development depends on hypercalcemia and length of survival of the patient. Electron microscopic findings are illustrated, and a mechanism for the development of nephrocalcinosis is proposed. For the first time, marked elevations of parathyroid hormone was detected. This finding, coupled with the extreme difficulty in locating the parathyroid glands in cases of hypophosphatasia, is enigmatic. Areas for furture investigation are suggested.

Alkaline Phosphatase↗