摘要:

Immunologic parameters including quantitative and qualitative immunoglobulin studies, various T cell functions and neutrophil chemotaxis were evaluated in two patients with the Hyper-IgE syndrome. Both exhibited pruritic dermatitis in locations atypical for atopic dermatitis, marked elevations in serum IgE levels (to 40,000 IU/ml), recurrent staphylococcal abscesses, coarse facial features and variable chemotactic defects characteristic of this syndrome. Both patients responded favorably to courses of trimethoprim-sulfamethoxazole, particularly in helping control the cutaneous infections. We believe that this is a useful therapeutic alternative to anti-staphylococcal antibiotics and prophylactic treatment has permitted therapeutic response.Serum IgG, IgG subclasses, IgM, and IgA were normal for age. Serum IgD was markedly deficient in one patient. Functional IgM was normal with positive isohemagglutinin titers. IgG poliovirus titers were present in both patients; however, tetanus titers were not detectable in either patient, despite repeated immunizations.Despite normal E rosette numbers, subtle T cell abnormalities were noted with variable responses to bothin vivoSK-SD, candida, and mumps skin tests andin vitroPHA-, Con A- tetanus-induced lymphocyte proliferation. Lymphocyte production of macrophage inhibitory factor and interferon and responsiveness in a mixed lymphocyte culture were normal in both patients. Considerable Con-A-induced suppressor cell activity was present in one patient, but diminished in the other.In vivochemotaxis determined by a Rebuck skin window, revealed a markedly delayed PMN migration in both patients during a time when both patients were clinically free of furunculosis or dermatitis.In vitroneutrophil chemotaxis was variable: maximally impaired in one patient during severe pneumonia and normalizing with clinical resolution; the low chemotactic activity of the second patient did not consistently correspond to the clinical state.Both patients demonstrated high serum histamine levels of 75–100 ng/ml (normal7to 104M histamine, and comparable enhancement with the same concentrations of the H2antagonist, cimetidine (17–38%). Both histamine and cimetidine consistently depressed chemotaxis in one patient. The other patient had enhanced chemotaxis in the presence of histamine and an inconsistent response to cimetidine. Histamine and cimetidine may affect neutrophils directly via H2receptors, indirectly via H2feedback inhibition of basophil histamine release or by non-H2mechanisms.

ISSN:0031-3998    

出版商:OVID    

年代:1983

数据来源: OVID

摘要:

The concentration of urogastrone (URO) and lactose was measured in breast secretions from four women. URO levels were high in precolostrum and fell markedly around the time of parturition to relatively constant low levels in mature milk. When milk samples were fractionated on a Biogel P10 column, the major peak of immunoreactivity occurred at the point at which pure URO eluted.There was good correspondence between the fall in URO levels at birth and the increase in lactose levels, suggesting that the decrease in URO was a dilution effect as the osmotic influence of lactose increased.

ISSN:0031-3998    

出版商:OVID    

年代:1983

数据来源: OVID

摘要:

C6-C10-dicarboxylic and C6-C10-ω-1-hydroxy monocarboxylic acids were measured in postmitochondrial (10,000 g) fractions of rat liver after incubation with hexanoic, octanoic, and decanoic acids. In livers both from fed and starved rats, the proportion of decanoic acid converted to sebacic acid was high (approximately 25%) with only minor accumulation of the intermediate 10-hydroxy decanoic acid (1–2%). The conversion of octanoic and hexanoic acids to suberic and adipic acids, respectively, was low (i.e.,171 and 3.1 μmole/liter, respectively, whereas they are high for octanoic and hexanoic acids (8211 and 8822 μmole/liter, respectively). In two different humans, the corresponding Km values for dodecanoic, decanoic, octanoic, and hexanoic acids are 3.6–186, 522–247, 4861–3892, and 6825–10400 μmole/liter, respectively. Based on these results, it is argued that adipic and suberic acids found in urine from rats and humans with acyl-CoA dehydrogenation deficiencies are not biosynthesized by direct ω-oxidation of hexanoic and octanoic acids, but most probably by means of β-oxidation of sebacic and dodecanedioic acids, produced by direct ω-oxidation.The affinities of the ω-1-hydroxylation systems for the four monocarboxylic acids is similar in rats and humans. The apparent Km values for dodecanoic acid in rats and in the two human livers are low,i.e.,139 and 92–131 μmole/liter, respectively. The Km values for decanoic, octanoic, and hexanoic acids are all high in both rats and humans,i.e.,in the range from 638 μmole/liter (for decanoic in one of the human livers) to more than 20,000 μmole/liter (for hexanoic acid in both species). It is argued that 5-hydroxy hexanoic, 7-hydroxy octanoic, and 9-hydroxy decanoic acids found in some patients with acyl-CoA dehydrogenation deficiencies are produced by β-oxidation of 11-hydroxy dodecanoic acid, which may be produced directly by ω-1-hydroxylationof dodecanoic acid.

ISSN:0031-3998    

出版商:OVID    

年代:1983

数据来源: OVID

摘要:

We measured the serum-stimulated lipase activity, fatty acid content, and various biochemical parameters in the breast milk of a lactating mother suffering from familial lipoprotein lipase deficiency and of healthy control subjects. Serum-stimulated lipase activity was virtually undetectable in milk from our patient and the total fatty acid content was low. The fatty acid composition differed from normal showing a marked absolute and relative increase in the content of lauric (C12:0) and myristic (C14:0) fatty acids and considerably reduced levels of oleic (C18:1) and especially linoleic (C18:2) acids. Other fatty acid species showed lesser changes in concentration. Besides a reduced calcium concentration in the milk from our patient, other biochemical parameters were not significantly different from control subjects.

ISSN:0031-3998    

出版商:OVID    

年代:1983

数据来源: OVID

ISSN:0031-3998    

出版商:OVID    

年代:1983

数据来源: OVID