ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1991.tb01533.x

出版商:Blackwell Publishing Ltd    

年代:1991

数据来源: WILEY

摘要:

The present study deals with the usefulness of1H‐NMR spectroscopy in the chemical diagnosis of propionic acidemia and methylmalonic acidemia. A Varian VXR‐500 spectrometer was used.xH‐NMR spectra of urine from a patient with propionic acidemia show peaks of 3‐hydroxy‐n‐valerate, methylcitrate, propionyl glycine, 3‐hydroxy‐n‐butyrate, lactate, 3‐hydroxypropionate, tiglylglycine and hippurate. The assignment was made possible by the use of 2D COSY. The spectra from a patient with methylmalonic acidemia show methylmalonate and glycine.1H‐NMR spectroscopy has the advantages of requiring a short time for analysis, relatively small amounts of urine and no p

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1991.tb01534.x

出版商:Blackwell Publishing Ltd    

年代:1991

数据来源: WILEY

摘要:

We have observed two patients who exhibited an exclusive increase of δ TCS1+subset of αδ T cells in the peripheral blood after bone marrow transplantation (BMT). In one case with severe combined immunodeficiency (SCID) who received haploidentical BMT from his father, αδ T cells appeared only after thymus transplantation. However, his T cell‐mediated immunity remained severely defective despite the generation of T cells of donor origin. In the other case with aplastic anemia, δ T CS1‐αδ T cells began to increase in the peripheral blood later. This indicates that the thymus is necessary for the generation of αδ T cells and that the δ TCS1+subset is dominant in the early stages of their ontogeny. δ TCS1+T cell lines were established from both patients, and allo‐reactivity was investigated. The cell line from the latter case reacted to recipient cells in a mixed lymphocyte reaction, but did not show cytotoxity to the allogeneic cells including recipient cells. The other cell line, from the former case, did not react to either donor or recipient cells. This indicates that an intact thymus is needed for αδ T cells to acquire allo‐reactivity. Both cell lines showed MHC non‐restricted cytotoxity against NK

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1991.tb01535.x

出版商:Blackwell Publishing Ltd    

年代:1991

数据来源: WILEY

摘要:

Along with the recent progress in controlling the diabetic state during pregnancy, the number of diabetic pregnancies in our country has been increasing. It is not appropriate to apply White's classification to Japanese pregnant women, because more than two‐thirds are NIDDM and it is not easy to clarify their duration of illness. Neonatal complications were analyzed in 160 infants of diabetic mothers (IDMs) who were admitted to Tokyo Women's Medical College over the past five years. All were inborn except four. Neonatal outcomes of IDDM and NIDDM were not statistically different. The overall incidence of neonatal complications in IDMs was 66%, and there were no neonatal deaths. Offspring whose mothers had been suffering from retinopathy were likely to be premature and light‐for‐date infants. A planned pregnancy in diabetic women is likely to decrease neonatal complications. The cooperative total care of diabetic mothers and their offspring by respective specialists, namely endocrinologists, obstetricians and neonatologists is essential for the better prognosis of infants of diabetic mo

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1991.tb01536.x

出版商:Blackwell Publishing Ltd    

年代:1991

数据来源: WILEY

摘要:

Lack of recovery of β‐hemolytic streptococci from the throat of 80 patients finally diagnosed as mucocutaneous lymph node syndrome was again confirmed, although α‐hemolytic streptococci were consistently isolated from all patients but one. The implications of these findings were discussed, particularly in terms of the possible role of Streptococcus pyogenes in the pathogenesis of this di

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1991.tb01537.x

出版商:Blackwell Publishing Ltd    

年代:1991

数据来源: WILEY

摘要:

We report two children of Greek origin with granulocytic sarcoma of the orbit and acute myelogenous leukemia. In the first case the orbital tumor was the initial manifestation of the disease, while the other occurred during hematological remission.

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1991.tb01538.x

出版商:Blackwell Publishing Ltd    

年代:1991

数据来源: WILEY

摘要:

Adrenocorticotropic hormone (ACTH) therapy is useful in the treatment of patients with West syndrome, and hypertrophic cardiomyopathy (HCM) in association with ACTH therapy has recently been reported. We describe the Doppler echocardiographic evaluation of an infant who had West syndrome and HCM. ACTH therapy produced increases in the interventricular septal thickness, the pressure gradient of the left ventricular outflow tract, and the heart rate. Doppler echocardiographic examination was found to be much more sensitive for monitoring the cardiac changes than either 2‐dimensional echocardiography or the heart rat

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1991.tb01539.x

出版商:Blackwell Publishing Ltd    

年代:1991

数据来源: WILEY

摘要:

In general, immunosuppressive agents such as corticosteroids are considered to be a contraindication for immunocompromised hosts with an active infection. Recently, however, a few cases have been reported where the obstructive lesions caused by the excessive inflammation in chronic granulomatous disease were successfully resolved with corticosteroids, resulting in a remission of the infection. As we have also experienced a case in which oral corticosteroid used in combination with antibiotics had a beneficial effect on an intractable respiratory tract infection which occurred in a patient with chronic granulomatous disease, we present the case report. Although it may be very difficult to determine the candidates, we consider that in selected cases a corticosteroid therapy with concomitant use of antibiotics will be a choice for the treatment of intractable infections in chronic granulomatous disease.

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1991.tb01540.x

出版商:Blackwell Publishing Ltd    

年代:1991

数据来源: WILEY

摘要:

Setleis bitemporal “forceps marks” syndrome is characterized by “forceps marks” and a peculiar facies. The syndrome has previously been reported mainly in the Puerto Rican population. We describe here a Japanese boy with the syndrome. In addition, the hypothesis is presented that the Setleis syndrome may result from an insufficient migration of neural crest cells into the frontonasal process and the first branchi

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1991.tb01541.x

出版商:Blackwell Publishing Ltd    

年代:1991

数据来源: WILEY

摘要:

A fatal case of leukemia complicated by mixed invasive aspergillosis and candidiasis is presented. Mixed fungal infections may occur in any immunocompromised patients. We should be careful to look for different morphologies of fungi in diagnostic examinations of lesions in these patients.

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1991.tb01542.x

出版商:Blackwell Publishing Ltd    

年代:1991

数据来源: WILEY