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31.
Hyper-IgM syndrome with CHARGE association 总被引:1,自引:0,他引:1
Pilar Bahillo Teresa Cantero Pilar Solís Paz Aragón Samuel Gómez Rosario Cambronero 《Pediatric allergy and immunology》2003,14(6):487-489
A girl with coloboma of the iris, sensorineural deafness, growth delay, distinctive face, and cranial nerve dysfunction was diagnosed of CHARGE association in the first year of life. She presented with repeated otitis. At 3 yr of age, the patient suffered a septicemia ( Streptococcus pneumoniae , Corynebacterium sp.). The immunoglobulin G (IgG) and IgA serum levels were decreased, IgM increased and cellular immunity parameters were normal, supporting the diagnosis of hyper-IgM (HIM) syndrome. The sequence of CD40 ligand and cytidine deaminase genes were normal. From then on, she was receiving immunoglobulin intravenously with an excellent outcome . Here, we report the first case of CHARGE association and HIM syndrome in the same patient. Although the cause could not be identified, a non-random link is likely. 相似文献
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K. H. McAllister 《Psychopharmacology》1994,116(3):317-325
D-Cycloserine (DCS) has been reported to affect the central nervous system in man. To investigate whether the compound produces specific behavioural effects, DCS was administered to male mice in a resident-intruder situation and the behaviour of the interacting mice assessed using ethological analysis. Resident mice given DCS (32.0–320.0 mg/kg PO, 60 min before testing) showed dose-dependent increases in social investigation, smaller increases in sexual behaviour and decreased aggressiveness. Defensive and flight behaviour were not affected. Intruder mice showed slight increases in sexual behaviour that were not dose-dependent, and small increases in social investigation. The increases in social investigation induced by DCS (320.0 mg/kg) in resident mice were not reversible with R-HA 966 (32.0 mg/kg IP, 30 min before testing), a blocker of the strychnine-insensitive glycine modulatory site associated with theN-methyl-D-aspartate receptor, but were blocked by the GABA antagonist bicuculline (0.56 mg/kg IP, 5 min before testing). The small DCS-induced increase in sexual behaviour in residents was reversed by R-HA 966. Within the parameters of the resident-intruder situation, DCS exerts socio-sexual behaviour-enhancing effects which are dependent upon the role of the interactant, and which are mediated by an action upon multiple substrates. DCS may be regarded as another example of a sociotropic (approach-promoting) agent.Some of these results have been presented at the 1st International Congress on Hormones, Brain and Neuropsychopharmacology, Rodos, Greece, September 12–17, 1993 相似文献
34.
Respiratory infections (RI) are one of the major complaints in children and adolescents, and represent a demanding challenge for the pediatrician. It has been estimated that at least 6% of Italian children younger than 6 yr of age present recurrent respiratory infections (RRI). Children with RRI are not affected by severe alterations of the immune system. RRI represent essentially the consequence of an increased exposure to infectious agents during the first years of life, when immune functions are still largely immature. Several social and environmental factors, such as day-care attendance, family size, air pollution, parental smoking, and home dampness, represent important risk factors for airway diseases and may contribute in various degrees to determine the incidence of RRI. The main problem for the pediatrician is to discriminate normal children with high RI frequency related to an augmented exposure to environmental risk factors from children affected by other underlying pathological conditions (immunological or not), predisposing to infectious diseases. When RRI diagnosis has been formulated, removal of environmental risk factors (i.e. precocious day-care attendance, smoking in the household) must first be suggested. 相似文献
35.
本文报告胰岛细胞脑内移植治疗I型糖尿病共3例,分别经过3个月、4个月、6个月的临床观察,其临床症状明显改善或消失,空腹血糖由移植前平均12.38mmol/L下降至7.77mmol/L;3例患者普通胰岛素用量平均每日62.3~u,移植后第21~30天均完全停用胰岛素,其中1例已持续撤离胰岛素治疗达5个月。观察结果提示:胰岛移植物在患者的脑内成活,并具有良好的内分泌功能。 相似文献
36.
Summary. Originally discovered in 1994 by Folkman and coworkers, angiostatin was identified through its antitumor effects in mice and later shown to be a potent inhibitor of angiogenesis. An internal fragment of plasminogen, angiostatin consists of kringle domains that are known to be lysine-binding. The crystal structure of angiostatin was the first multikringle domain-containing structure to be published. This review will focus on what is known about the structure of angiostatin and its implications in function from the current literature. 相似文献
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Leann D. Ermel Paul B. Marshburn William H. Kutteh 《American journal of reproductive immunology (New York, N.Y. : 1989)》1995,33(1):14-20
PROBLEM: To determine if heparin may act directly with antiphospholipid antibodies (APA) to prevent recurrent pregnancy loss (RPL). METHOD: Patients were seen at the University of Texas Southwestern Medical Center. Twenty women with a history of RPL (≥3 miscarriages), positive APA, and an otherwise normal evaluation were treated with heparin in two daily subcutaneous dosages during a successful pregnancy. APA levels were obtained prior to conception and again at 6, 20, and 30 weeks. RESULTS: Heparin reduced APA binding to cardiolipin and phosphatidylserine in a dose-dependent fashion in ELISA. Heparin affinity chromatography absorbed over 80% of the IgG anticardiolipin antibody in serum from women with high levels of APA. Women treated with increasing dosages of heparin during pregnancy had inversely decreasing levels of IgG anticardiolipin antibody. CONCLUSION: Heparin may act by directly binding APA in vivo, thereby decreasing the adverse effects of APA in women with APA associated RPL. 相似文献
40.
Akira Fukushima 《Psychiatry and clinical neurosciences》1994,48(Z1):1-4
Abstract: Historical changes in forensic psychiatric evaluation on criminal responsibility and proceedings in psychopathological findings of amphetamine psychosis are reviewed at first. The classification of amphetamine related mental disorders are proposed in 6 types. Among them, the clinical characteristics and psychopathological features of “Anxiety-situational reaction type” (Fukushima) are described. According to some reasonable grounds, offenders diagnosed as anxiety-situational reaction type should be evaluated as diminished responsibility in place of irresponsibility. Finally, two cases of murder committed under the influence of amphetamine, are reported in detail. 相似文献