Eosinophilic gastrointestinal disorders and allergen immunotherapy: Lights and shadows

Allergic diseases, such as IgE-mediated food allergy, asthma, and allergic rhinitis, are relevant health problems worldwide and show an increasing prevalence. Therapies for food allergies are food avoidance and the prompt administration of intramuscular epinephrine in anaphylaxis occurring after accidental exposure. However, allergen immunotherapy (AIT) is being investigated as a new potential tool for treating severe food allergies. Effective oral immunotherapy (OIT) and epicutaneous immunotherapy (EPIT) induce desensitization and restore immune tolerance to the causal allergen. While immediate side effects are well known, the long-term effects of food AIT are still underestimated. In this regard, eosinophilic gastrointestinal disorders (EGIDs), mainly eosinophilic esophagitis, have been reported as putative complications of OIT for food allergy and sublingual immunotherapy (SLIT) for allergic asthma and rhinitis. Fortunately, these complications are usually reversible and the patient recovers after AIT discontinuation. This review summarizes current knowledge on the possible causative link between eosinophilic gastrointestinal disorders and AIT, highlighting recent evidence and controversies.     

肾病综合征患儿血浆一氧化氮及内皮素检测及其意义

为探讨一氧化氮(NO)及内皮素(ET)与肾病综合征之间的关系,采用比色法和放免法分别检测了活动期肾病综合征患儿59例、治疗后48例的血浆NO及ET水平,并设24例健康儿童作为对照。结果发现肾病综合征活动期血浆NO与ET水平显著高于缓解期,活动期与缓解期血浆NO与ET水平均显著高于对照组(P均<0.01),NO水平与ET水平呈显著正相关(r=0.565,P<0.05),显示NO与ET和肾病综合征的发生有关。     

Aktivierung einer bioelektrischen Epilepsie durch Hirntrauma

Zusammenfassung An Hand eines Krankheitsverlaufs mit Auftreten von Krampfmustern im Sinne kurzer Petit-Mal-Entladungen im EEG ohne klinische Manifestation im Anschluß nach einem schweren Schädelhirntrauma wird unter Berücksichtigung der EEG-VErläufe der Geschwister des Patienten dargelegt, daß zum Zustandekommen epileptischer Phänomene sowohl konstitutionell-erbliche Faktoren als auch organische Hirnschädigungen als Realisationsfaktoren mit Beeinflussung durch reifungsbiologische Determinanten zusammenwirken können und diese sich keineswegs gegenseitig ausschließen.

---

Three per second spike and wave discharges provoked by trauma

Summary The case history of a 5 year old boy who suffered severe head injuries which resulted in 13 days unconsciouness is given. 5 weeks after the accident had taken place, short paroxysms of three per second spike and wave discharges were observed in all leads over a period of about 8 weeks. No seizures occured while the patient received prophylactic anti-conculsive treatment. 3 years later the EEG showed general irregularities, especially in the temporal areas; focal slowing or spike and wave complexes were not observed. The EEGs of the two elder brothers showed the same abnormalities. Both of them had suffered one seizure of the grand mal type; one 3 days after a head injury received at the age of three, the other during a febrile infection at the age of one.These case histories show that there seems to be a hereditary factor which determines the individual pattern of the EEG as well as the tendency to develop spike and wave discharges or clinical seizures. Attacks of three per second spike and wave discharges are usually considered hereditary. In this particular case, however, the pattern only occurred during a short period in life and was provoked by an accident. Both, hereditary factors and brain injury, seem to be responsible for the occurrence of the spike and wave pattern, which furthermore seem to be age dependent.

     

Childhood CNS inflammatory demyelinating diseases

CNS inflammatory demyelinating diseases (CIDD) are rare. At first presentation children are diagnosed with acute disseminated encephalomyelitis (ADEM) or a clinically isolated syndrome (CIS) such as transverse myelitis (TM) or optic neuritis (ON). Many of these disorders are monophasic, however a small number of children relapse and are diagnosed with multiple sclerosis (MS) or neuromyelitis optica (NMO). Paediatric onset Multiple Sclerosis (POMS) is a chronic inflammatory neurodegenerative demyelinating disease of the CNS that is usually relapsing-remitting at onset. There has been significant recent interest and progress in these disorders. Encephalopathy (behavioural change or altered consciousness) distinguishes ADEM from other demyelinating conditions. A high index of suspicion for CIDD is required in children presenting with neurological deficits, encephalopathy or status epilepticus. Several UK and international studies are underway to further our understanding of these diseases. There has been significant development of new treatments for MS and NMO. A national service for these rare disorders will enable better management of these well deserving patients. In this review we describe current understanding of the epidemiology, pathogenesis, clinical features, outcome and management of childhood CIDD.     

肌酸激酶同工酶与血浆内皮素检测结合脑电图检查判断热性惊厥患儿脑损伤的程度及预后

目的探讨热性惊厥(FC)患儿血清脑型肌酸激酶同工酶(CK-BB)和血浆内皮素(ET)水平的变化及其两者结合脑电图(EEG)检查与脑损伤的关系。方法将2005-08—2006-12在潍坊医学院儿科就诊的52例FC患儿作为FC组,健康儿童28例作为正常对照组,分别进行血清CK-BB和血浆ET的测定及EEG检查,CK-BB采用比色法测定,ET采用放射免疫法测定。结果FC患儿血液中CK-BB和ET水平明显高于正常对照组(P均<0.01);单纯热惊厥(SFC)组CK-BB和ET的浓度与正常对照组比较差异无显著性(P>0.05);复杂热惊厥(CFC)组与正常对照组和SFC组比较差异均具有显著性(P均<0.01)。CFC组血清CK-BB与血浆ET呈正相关(r=0.652,P<0.01)。FC患儿惊厥发作后第1天EEG异常率为80.77%,其中SFC组78.12%,CFC组85.00%,差异无显著性(P>0.05);第14天为32.69%,SFC组仅18.75%,CFC组则55.00%,差异具有显著性(P<0.01)。结论CK-BB和ET水平与脑损伤的程度密切相关,CK-BB和ET含量越高脑损伤程度越严重。热性惊厥后及时检测CK-BB和ET,并结合脑电图检查对判断脑损伤程度及预后有重要价值。     

Tuberous sclerosis presenting as abdominal tumor

Tuberous sclerosis can present as an abdominal tumor due to angiomyolipoma even in a child without seizures, retardation or skin lesions. CT scanning is useful to differentiate this from polycystic kidneys.     

紫癜性肾炎患儿血浆内皮素—1、vWF、D—二聚体的变化及临床意义

对14例紫癜性肾炎(SHN)、17例不伴尿常规改变过敏性紫癜(SHP)和12例正常儿童的血浆内皮素-1(ET-1)、假性血友病因子(vWF)及D-二聚体(D-D)进行了检测。结果显示:SHN患儿的血浆ET-1(88.48±22.96)ng/L、vWF(1.59±0.38)U/ml及D-D(1.45±0.39)mg/L均显著高于对照组,分别为(43.73±17.89)ng/L、(0.99±0.30)U/ml,(0.28±0.23)mg/L.P均<0.01。在治疗后,SHN患儿的血浆ET-1、vWF和D-D水平均显著下降(P均<0.01);SHN患儿的血浆ET-1和D-D水平与血清肌酐呈正相关(分别为r-O.794,P<0.01;r=0.826,P<0.01)。表明肾血管内皮损伤所致ET-1过度生成、血管内凝血及继发性纤溶可能参与本病肾损伤过程。     

韧粘素在先天性心脏病合并肺高压患儿肺血管中的表达

为探讨韧粘素（ＴＮ）在先天性心脏病（先心病）合并肺高压发病机制中的作用及其其调节因素，采用免疫组化方法观察不同肺血清和／或不同肺动脉压力下，先心病患儿肺小动脉中ＴＮ的表达情况。图像分析结果表明，高肺血流伴肺高压时ＴＮ表达阳性面积比例明显高于高肺血流不伴肺高压和对照组（Ｐ均〈０．０１），ＴＮ表达与肺动脉压力升高显著相关（ｒ＝０．７５，Ｐ〈０．０１），与肺血流增多无关（ｒ＝－０．４８，Ｐ〉０．０５）。     

钙拮抗剂和钙调素拮抗剂对白血病多药耐药细胞内阿霉素浓度的影响及P-GP表达的关系

白血病多药耐药细胞K562/VCR细胞内Dox浓度明显低于对照组,而P-GP表达则明显高于对照组,提示P-GP的过度表达可能是肿瘤细胞产生MDR的机理之一。CaS及CaMAS可下调K562/VCR P-GP的表达,增加其胞内Dox浓度,表明CaS和CaMAS逆转肿瘤细胞MDR的机理也与降低细胞膜P-GP表达有关。     

特发性肾病综合征患儿血清游离甲状腺激素检测的临床意义

探索特发性肾病综合症（ＩＮＳ）患儿血清游离甲状腺激素（ＴＨ）、甲状腺激素结合球蛋白（ＴＢＧ）的变化情况及其变化的机理和临床意义。对１６例ＩＮＳ患儿及１５例正常儿童对照,用放免法测Ｔ３、Ｔ４、ＦＴ３、ＦＴ４,化学发光酶免疫分析法测ＴＳＨ、ＴＢＧ,放射配基单点饱和饱和结合分析法测糖皮质激素受体（ＧＣＲ）。结果：①ＩＮＳ患儿Ｔ３、Ｔ４、ＦＴ３、ＦＴ４均低于对照组,ＴＳＨ升高,差异非常显著。②ＴＢＧ降低,ＴＢＧ与ＴＨ呈正相关。③ＩＮＳ患儿ＧＣＲ升高,ＴＨ与ＧＣＲ未见相关性。提示对糖皮质激素（ＧＣ）治疗敏感的ＩＮＳ患儿,虽然其ＴＨ是降低的,但其ＧＣＲ是升高的。     

新生儿缺氧缺血性脑病与NO、NOS、PRL水平的相关性研究

采用放免法和比色法测定68例新生儿缺氧缺血性脑病(HIE)及30例正常新生儿血浆一氧化氮(N0)、一氧化氮合酶(NOS)及血清催乳素(PRL)活性水平,以探讨NO、NOS及PRL在HIE中的发病机理、病情进展及预后中的作用。结果;HIE急性期NO、NOS、PRL水平较正常新生儿明显升高(P<0.05),中度和重度HIE的3项水平升高更显著(P均<0.01);HIE惊厥组急性期血清PRL水平显著高于非惊厥组(P<0.01),且与惊厥发作持续时间呈正相关(r=0.485,P<0.01)。提示:NO、NOS、PRL水平高低与HIE患儿脑损伤严重程度有关,可作为判断病情、预后及惊厥是否发作的指标。     

Prolactin response to exercise,metoclopramide and other provocative agents in children

Metoclopramide (MCP), a derivative of procainamide was compared with exercise, arginine, insulin and thyrotropin releasing hormone (TRH) as a prolactin (PRL) releaser in children. The peak response of plasma PRL after oral administration of MCP was greater than that after strenuous exercise and after i.v. administration of pharmacodynamic agents. Normal PRL and TSH responses were observed after TRH administration in all subjects. Variable PRL responses were seen after exercise and after i.v. administration of arginine and insulin, despite significant growth hormone (GH) release following the administration of these agents. MCP produced no increase in plasma TSH. Metoclopramide may be useful for dynamic testing of PRL release in children. It can be taken orally and is free of side-effects.     

Improved growth with growth hormone therapy in a child with glycogen storage disease Ib

In type Ib glycogen storage disease (GSD) growth is typically affected and short stature is a common feature. Reported use and effect of growth hormone (GH) therapy in children with GSD is limited. We report on the use of substitutive GH treatment in a poorly growing adolescent female with GSD type Ib. The patient's growth velocity increased from a baseline level of 2.5 cm/y to an average growth velocity during GH therapy of 8.7 cm/y. During GH therapy this patient did not demonstrate metabolic decompensation but increased levels of cholesterol and triglycerides were seen (A-1).

Conclusion: Patients with GSD may experience an improvement in growth response with GH treatment. Prior to GH therapy, treatment of hyperlipidemia associated with GSD should allow the therapy to be safely tolerated.     

Cell mediated immunity in childhood malaria

Children with malarial infection, due to P.Vivax and P.falcifarum, were tested for cell mediated immunity (CMI) by lymphocyte proliferate response to mitogens PHA (phytohaemagglutinin)and PWM (poke weed mitogen) and antigen PPD (purified protein derivative). This was done during the period of parasitemia and after treatment, and compared to 19 normal matched controls. There was no significant difference between the patients and the control group with regard to PHA (patients 57.4 ±50.5; controls 61.3 ±54.9); PWM (patients 27.4 ±19.9, controls 29.9 ±24.5); PPD (patients 2.2 ±1.2, controls 1.9 ±1.4). There was also no significant difference in the lymphocyte responses during the period of parasitemia and after treatment. Hence, there does not seem to be any depression of CMI as shown by lymphocyte proliferative responses during childhood malaria.     

新生儿缺氧缺血性脑病血NSE与ET变化及其与高压氧治疗相关性的研究

目的 探讨肿瘤坏死因子-α(TNF-α)和降钙素基因相关肽(CGRP)在新生儿缺氧缺血性脑病(HIE)中的变化及临床意义。方法 采用放射免疫分析法对38例HIE患儿和18例健康足月新生儿血浆TNF-α与CGRP水平进行了同期动态观察。结果 HIE患儿急性期TNF-α,CGRP水平分别为(1.12±0.42) ng/ml,(88.92±23.16) ng/ml;恢复期分别为(0.61±0.18) ng/ml,(68.39±19.32) ng/ml;对照组分别为(0.54±0.15) ng/ml,(66.2±14.54) ng/ml。急性期血浆TNF-α和CGRP水平较恢复期显著增高(P<0.01),并明显高于同期对照组水平(P<0.01),恢复期与正常对照组无显著差异,急性期不同程度HIE与对照组TNF-α,CGRP水平比较,重度HIE组TNF-α,CGRP分别为(1.28±0.41) ng/ml,(118.12±30.25) ng/ml;中度HIE组分别为(0.95±0.3) ng/ml,(86.49±24.36) ng/ml,轻度HIE组分别为(0.63±0.19) ng/ml,(68.31±18.38) ng/ml,重度组明显高于对照组、轻度组及中度组,中度组高于对照组和轻度组,轻度组与对照组无显著性差异。急性期患儿血浆TNF-α与CGRP呈直线正相关关系(r=0.513,P<0.05)。结论 TNF-α和CGRP参与了新生儿HIE的发病过程。急性期TNF-α的增高可能是促发HIE脑损伤的一个重要因素,而CGRP增高在HIE中对脑损伤可能具有一定的保护作用。