ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1988.tb02489.x

出版商:Blackwell Publishing Ltd    

年代:1988

数据来源: WILEY

摘要:

AbstractHistopathology and histochemistry were studied in biopsied muscles from eight patients with the congenital form of myotonic dystrophy (congenital MyD) and one patient with the adult form (adult MyD). In the muscle pathology of the four patients aged between 5 and 11 years with congenital MyD, there was no immaturity of the fibers and the histological alterations were minimal. The pathological findings of the adult patient with congenital MyD resembled those of adult MyD. The immature condition of the musculature observed during the early infantile period, therefore, may once improve with motor development during childhood and, after that, the muscle fibers may degenerate in a similar manner to that seen in adult MyD.Two patients with marked talipes equinovarus displayed grouped atrophy. Small angular fibers and pyknotic nuclear clumps were observed in five patients. These findings suggest that some neurogenic factor might be involved in the muscular changes in this disorder.

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1988.tb02490.x

出版商:Blackwell Publishing Ltd    

年代:1988

数据来源: WILEY

摘要:

AbstractWe experienced a small outbreak of acute gastroenteritis in December 1985 in hospitalized children. Two different serotypes of rotavirus and two serotypes of adenovirus were found by molecular epidemiological and serological studies. Four patients were considered to have two serotypes of rotavirus. The results showed that a single strain of a virus was not always the cause of nosocomial infection.

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1988.tb02491.x

出版商:Blackwell Publishing Ltd    

年代:1988

数据来源: WILEY

摘要:

AbstractBetween 1980 and 1986, we observed lower respiratory‐tract infection caused by respiratory syncytial virus (RSV) in 19 infants and young children with various forms of congenital heart disease. Of these, seven patients who presented with a severe illness were treated with intramuscular injections of 0.5 to 1 times 106IU per day of human leukocyte interferon (IFN) for three to five days. Intrinsic IFN activity developed before treatment in three subjects. Viral isolation studies showed active RSV infection prior to treatment, and no infectious RSV was recovered one to four days after treatment. Although there were no obvious changes in the clinical course after IFN therapy, no deaths related to RSV infection occurred. Analysis of the different types of congenital heart disease showed that ventricular septal defect and/or patent ductus arter‐iosus with pulmonary hypertension were associated with the most severe RSV illness. The therapeutic value of IFN may improve in this group of subjects if it is given without de

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1988.tb02492.x

出版商:Blackwell Publishing Ltd    

年代:1988

数据来源: WILEY

摘要:

AbstractTo investigate the initial focus of streptococcal invasion, aerosol‐induced infection in rabbits that resembled natural infection was examined by transmission electron microscopy (TEM). Group A type 12 streptococci were adjusted to a concentration of 108‐109colony forming units (CFU)/ml in saline. The rabbits were exposed to the aerosolized suspension for about two hours and sacrificed at two hours to 14 days after the exposure. Pharyngeal mucous membranes, palatine tonsils and tracheas were observed by TEM.Bacterial attachment was recognized on the tonsillar surface epithelium and abnormalities such as vacuolization of cytoplasm and an increased number of plasma cells were observed in the tonsillar medulla. In addition, specialized epithelial cells included micropinocytotic vesicles with spherical substances similar to those of the bacteria.The micropores of tonsillar crypts are considered to be the pathway of infiltrating cells and initial focus of foreign antigens at present. These findings suggested that tonsils have an important function as the initial focus of invasion of streptoco

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1988.tb02493.x

出版商:Blackwell Publishing Ltd    

年代:1988

数据来源: WILEY

摘要:

AbstractThe initial focus of streptococcal invasion and histological changes of rabbit tonsils infected by aerosolinduced streptococci was investigated by scanning electron microscopic studies of the free and fractured surface of the tonsils.Group A type 12 streptococci were suspended in saline at a concentration of 108to 109colony forming units per ml. Rabbits were exposed to the aerosolized suspension for about 120 minutes and sacrificed within 48 hours after the exposure.The tonsilar epithelium was organized in two different types. The micro‐pores of the crypts were divided into three types. Some interesting findings were observed in the micropores covered by cobblestone‐like cells with micro‐villi or cillia (the third type). That is, the micropore had a trace left by something that had passed. Also, the infiltrating cells appeared numerously after the exposure and continuously from the parenchyma of the tonsils.These data suggested that the third type micropores play an important role in the initial focus of invasion and in the defense or immunological pr

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1988.tb02494.x

出版商:Blackwell Publishing Ltd    

年代:1988

数据来源: WILEY

摘要:

AbstractFabry's disease is an X‐linked recessive disorder of glycosphingolipid metabolism. Accumulation of neutral sphingolipid in vascular endothelium is known to cause various clinical manifestations, including cardiac problems. Female heterozygotes, however, may be asymptomatic or mildly affected. Cardiovascular manifestations in Fabry's disease were studied noninvasively in 10 male hemizygotes and 10 female heterozygotes from seven families. Mitral valve prolapse was found in both hemizygotes and heterozygotes. Measured echocardiographic variables were normalized by dividing them by normal values calculated from basal surface area (BSA) and age. Normalized thickness of the interventricular septum [IVS(n)] and left ventricular posterior wall thickness [LVPW(n)]increased significantly with age both in hemi‐ and heterozygotes. [IVS(n): Y=0.94+0.014X (age in years), r=0.68, LVPW(n): Y= 1.03+0.015X (age in years), r=0.64]. The incidence of ST‐T changes in the ECG was also observed in hemi‐ and heterozygotes. A short PR interval was recorded in one hemizygote and one heterozygote from the same family. Thus, heterozygotes appear not to be free from cardiac manifestations, as previously believed. It is possible that the cardiac changes in heterozygotes occur regardless of the amount of sphingolipid accum

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1988.tb02495.x

出版商:Blackwell Publishing Ltd    

年代:1988

数据来源: WILEY

摘要:

AbstractThe present study was designed to investigate the relation between endogenous dopamine and natriuresis during the early postnatal days in term infants. Urinary dopamine levels were measured by high‐performance liquid chromatography in the initial urines of 61 full‐term male infants and in serial collected urines at 3‐hour intervals during the first three days in five full‐term male. infants. Dopamine excretion rate (ng/mgCr or ng/hr) correlated significantly with sodium excretion rate in both the initial urines (mEq/mgCr) and serial collected urines (mEq/hr). In the initial urines, dopamine excretion rate (ng/mgCr) did not correlate with aldosterone excretion rate (ng/mgCr), which showed an inverse relation with sodium excretion rate (mEq/mgCr), but with cyclic AMP values (nmol/mgCr). In addition, dopamine excretion rate (ng/hr) significantly correlated with cyclic AMP excretion rate (nmol/hr) in one infant.Although the significance of the direct relation between urinary dopamine and sodium output needs to be further investigated, it is possible that endo‐genously produced dopamine may play a role in natriuresis, via the cyclic AMP producing system, during the early neonat

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1988.tb02496.x

出版商:Blackwell Publishing Ltd    

年代:1988

数据来源: WILEY

摘要:

AbstractEffects of the inhibition of maternal phenylalanine (Phe) or tyrosine (Tyr) hydroxylase on the postnatal maturation of catecholamine, thyroxine and amino acid metabolisms were examined in young rats. The intraperitoneal injections of 100 mg/kg p‐chlorophenylalanine three times during the second gestational week together with 0.2% Phe solution as drinking water produced marked reduction in birth weight, body weight gain, brain weight, cerebral Tyr hydroxylase activity, adrenal epinephrine, renal norepinephrine and free amino acids in plasma and brain as well as delayed maturation of thermoregulation in the offspring. In contrast, the administration of 100 mg/kg α‐methyl‐p‐tyrosine with 0.2% Tyr supplementation did not affect general growth, but only decreased cerebral Tyr hydroxylase and serum thyroxine level, while cerebral dopamine was increased. These results indicate that maternal Phe hydroxylase inhibition induces more severe growth failure including catecholamine and amino acid metabolism than Try hydroxylase inhibition in postnatal dev

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1988.tb02497.x

出版商:Blackwell Publishing Ltd    

年代:1988

数据来源: WILEY

摘要:

AbstractThe inhibitory action of long‐term low dose aspirin (1–2 mg/kg/day for over 10 months) on the cyclooxygenase pathway in platelets and vascular endothe‐lium was evaluated in 10 patients with Kawasaki disease. The results were compared with those obtained after taking aspirin at 5–10 mg/kg/day during the acute phase of the illness. Platelet aggregations induced by adenosinedi‐phosphate (ADP), epinephrine and collagen were inhibited by aspirin doses of 1–2 and 5–10 mg/kg/day, when compared with those of controls (p0.1). These values were significantly lower than those of the control group (22.88± 3.42 ng/ml PRP/105platelets) (p<0.05). No differences were found in platelet aggregation and TXB2productivity between the two aspirin doses. Levels of 6 keto‐prostaglandin F1α (6k‐PGF1α) in platelet‐poor plasma (PPP) did not differ significantly in these 3 sets of data. The results indicate that long‐term administration of low dose aspirin (1–2 mg/kg/day) inhibits platelet aggregation by inhibiting synthesis of thromboxane A2(TXA2), without interfering with prostacyclin production probably i

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1988.tb02498.x

出版商:Blackwell Publishing Ltd    

年代:1988

数据来源: WILEY