新生儿色析性荨麻疹一例

患儿,女,4天。第一胎第一产,孕39周顺产。生后3天发现双下肢起红色皮疹,次日即蔓延至躯干及四肢,无发热。生后曾肌注青霉素2天。父母非近亲婚配。其父幼年双下肢及腰部也有类似斑疹,后呈色素斑,青春期自愈。查体: T36.5℃,体重3.2kg,发育正常,反应好,躯干及上下肢满布大小不等玖瑰色风团样皮疹,多数融合成片,疹间可见正常皮肤,胸部散在2～3个黄豆大小结节样皮疹,无水疱及渗出,皮     

水痘并血小板减少性紫癜1例

患儿,女,6岁,因皮疹5天,发热3天,鼻衄伴皮肤紫癜1天入院.5天前患儿躯干部无明显诱因出现散在的充血性斑疹、斑丘疹、水疱疹,继之头面部四肢也出现且逐渐增多并伴瘙痒,皮疹主要分布于躯干及头面部.     

新生儿色素失禁症1例

男,26天。因皮疹26天、反复抽痉20多小时入院。第2胎第1产,足月顺产。患儿出生时左上臂处见有一蚕豆大小红色皮疹,高于皮面,后变水疱并增多。生后20天四肢见条索状分布水疱,部分结痂及色素沉着,渐波及面、躯干及颈部。于出生第25天突起四肢抽动,口角右歪伴吐白沫,持续1～2分钟自止并安睡,如此每半～1小时发作1次,至入院前达20余次。用葡萄糖酸钙、B_6无效。无发热、呕     

新生儿发热 贫血 皮疹 肝脾淋巴结肿大

<正> 1 病例资料 患儿,女,21d,反复皮疹21d,发热1d入院。患儿为足月剖宫产,生后6～8h即有皮疹,遍布全身,以躯干较多,并且逐渐增多,皮疹中心为水疱样丘疹,周围色红,逐渐出现脱屑、出血、渗出及溃疡。于外院曾诊断为“脓癌病”,先后应用青霉素、先锋霉     

小儿肺泡蛋白沉积症一例

患儿,男,45 d,因"全身皮肤发红1个月伴发热、咳嗽、气促1周"入院.患儿于生后1周左右出现颜面部粟粒样皮疹,迅速波及到躯干,在外院诊断为湿疹,给予外用药物治疗,约1周后皮疹消失,但出现全身皮肤发红,后在某皮肤病专科医院诊断为红皮病,给予多种口服及外用药物治疗,症状可短暂缓解.入院前1周患儿因受凉出现发热、咳嗽及气促,在外院按急性支气管肺炎住院治疗6 d,无好转转入我院.     

种痘样水疱病1例

患儿,女,8岁,因反复皮疹3^＋年加重4d于2005年7月入院,患儿自3^＋年前日晒后面部,四肢出现散在红斑,丘疹,水疱,水疱中央有脐窝,似水痘样（病初当地诊所曾诊断为水痘）,持续1周左右水疱破溃,糜烂,结痂,脱痂后留有痘疤样瘢痕,皮疹成批出现,发病为春夏加重,秋冬缓解,     

新生儿期渗出性多形红斑2例

渗出性多形红斑是具有特征性皮肤粘膜病变,伴有发热及周身症状的变态反应病。100多年前由Hebra及Barin描述,1922年Stevens和Johnson氏指出此病可累及周身多处粘膜,故又称Stevens—Johnson综合征。此病好发于儿童,男多于女,新生儿期极少见。现将近期所遇2例报告如下: 例1 男,25天,因发热、咳喘、全身起红疹5天入院,5天前患儿开始发热。咳嗽、双下肢有暗红色丘疹,后为水疱。3天来蔓延至躯干、上肢、面部、口腔粘膜及尿道口。皮疹融合片状紫癜。院外以过敏性紫     

以罕见皮损为主要表现的小儿非霍奇金淋巴瘤一例

患儿男 ,8岁。主因反复发热皮疹 4年 ,发现脾大半年入院。患儿于 4年前无明显诱因颜面部反复起皮疹 ,为红色丘疹 ,不痛不痒 ,压之褪色。用抗生素、地塞米松治疗后可消退 ,不留痕迹。以后皮疹渐扩展至四肢和躯干 ,主要分布在面部及前臂伸侧暴露部位 ,与季节及日晒无关。 1年前又出皮疹 ,初为红色丘疹 ,后转为脓疱样 ,渐转为结疤性色素沉着 ,消退后遗留凹陷性疤痕。每次出现皮疹均伴发热。半年前于当地发现脾大。外院曾行皮肤活检未能确诊。既往个人、家族史无特殊。体检 :一般情况好 ,颜面及耳后散在结疤性皮疹 ,躯干见成簇红色丘疹。面部、…     

疑难病例分析——反复出现湿疹样出血性皮疹1年伴高热10天

病史简介 男,2岁。1岁时在无明显诱因的情况下出现皮疹,表现为红色丘疹,伴渗出、脱屑和糜烂。皮疹分布于躯干和头皮。1年来,皮疹有时缓解,有时加重,但总的趋势呈进行性加重,偶有瘀斑出现,伴睡眠不佳和口腔粘膜溃疡。病程中无多饮多尿现象。多家医院均诊断为湿疹,治疗效果不佳。10天前突发高热而住某医院,因高热不退,按湿疹继发感染治疗无     

皮肤疾病

910303 婴儿坏疽性皮炎1例/黄剑清…∥中华皮肤科杂志.-1990,23(4).-284患儿男,9个月,四肢、躯干、面部起黄豆大水疱,伴发热,3日后转为脓疱、结痂,脱落后形成深在性溃疡,且逐渐扩大;有蚕豆大,部份溃疡深达脂膜层,基底红,表面有黄     

Allergic factors associated with the development of asthma and the influence of cetirizine in a double-blind, randomised, placebo-controlled trial: First results of ETA®

There is a common progression known as the allergic march from atopic dermatitis to allergic asthma. Cetirizine has several antiallergic properties that suggest a potential effect on the development of airway inflammation and asthma in infants with atopic dermatitis. Methods. Over a two year period, 817 infants aged one to two years who suffered from atopic dermatitis and with a history of atopic disease in a parent or sibling were included in the ETAC^® (Early Treatment of the Atopic Child) trial, a multi-country, double-blind, randomised, placebo-controlled trial. The infants were treated for 18 months with either cetirizine (0.25mg/ kg b.i.d.) or placebo. The number of infants who developed asthma was compared between the two groups. Clinical and biological assessments including analysis of total and specific IgE antibodies were performed. Results. In the placebo group, the relative risk (RR) for developing asthma was elevated in patients with a raised level of total IgE (≥ 30 kU/I) or specific IgE (≥ 0.35 kUA/I) for grass pollen, house dust mite or cat dander (RR between 1.4 and 1.7). Compared to placebo, cetirizine significantly reduced the incidence of asthma for patients sensitised to grass pollen (RR = 0.5) or to house dust mite (RR = 0.6). However, in the population that included all infants with normal and elevated total or specific IgE (intention-to-treat - ITT), there was no difference between the numbers of infants developing asthma while receiving cetirizine or placebo. The adverse events profile was similar in the two treatment groups. Discussion. Raised total IgE level and raised specific IgE levels to grass pollen, house dust mite or cat dander were predictive of subsequent asthma. Cetirizine halved the number of patients developing asthma in the subgroups sensitised to grass pollen or house dust mite (i.e. 20% of the study population). In view of the proven safety of the drug, we propose this treatment as a primary pharmacological intervention strategy to prevent the development of asthma in specifically sensitised infants with atopic dermatitis.     

Resurgence of measles in Singapore: profile of hospital cases

OBJECTIVE: To ascertain the profile of cases of measles seen at a general hospital during a recent outbreak that occurred despite a measles vaccination program. METHODOLOGY: A retrospective study from January 1991 to March 1998. All patients with measles (ICD code 055. 9) seen at the emergency unit or as inpatients were included. RESULTS: There were 87 cases identified. The diagnosis was clinical in all and proven serologically in 71%. Eighty-five per cent of the cases occurred between January 1997 and March 1998. There was a bi-modal age distribution with peaks in the very young (相似文献   

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孤独症谱系障碍(autistic-spectrum disorders,ASDs)近年来患病率逐年攀升至1%左右,其症状往往伴随终生,成为严重威胁儿童健康和发展的神经发育性疾患;注意缺陷多动障碍(attention deficit hyperactivity disorder,ADHD)是儿童期最常见的精神障碍,国内报道患病率为4.13%～5.83%,其症状可延续至青少年期,甚至到成年期[1]。这两类精神障碍在成年期的临床表现、共患病、治疗策略和预后与儿童期有哪些不同呢?本文通过回顾相     

EXCITING NEW TREATMENT APPROACHES FOR PATHYPHYSIOLOGIC MECHANISMS OF SICKLE CELL DISEASE

During the past several decades, our understanding of the complex pathophysiology of vasoocclusion associated with sickle cell disease has improved greatly. Interaction of genes, hemoglobin molecules, red cell membrane and metabolic changes, cell-cell interactions and cell-plasma interactions, red cell adhesion to vascular endothelium, activation of coagulation, and vascular reactivity play a role in vaso occlusion. Penicillin prophylaxis of pneumococcal infections and appropriate use of blood transfusions and other supportive measures improved survival of sickle cell patients. Hydroxyurea made a major impact on sickle cell therapy when it was shown to decrease acute painful episodes, acute chest syndrome, and the need for blood transfusion in adults. Significant experience in the use of hydroxyurea has been accumulated in older children. The benefits and risks of hydroxyurea for younger children and long-term risks in all patients will be evaluated in future investigations. Other promising therapies include butyrate compounds, clotrimazole, magnesium supplementation, poloxamer 188, antiadhesion agents, anticoagulant approaches, and nitric oxide. Hemopoietic transplantation remains the only curative therapy. However, several transgenic mouse models are available for studies of gene therapy or other treatment approaches on biochemical, cellular, and pathologic effects of mutant genes.     

Infiltrations of Epstein-Barr virus-carrying cells in granular lymphocyte-proliferative disorders corresponding to chronic active Epstein-Barr virus infection

A 21-year-old man with granular lymphocyte-proliferative disorders (GLPD) associated with chronic active Epstein-Barr virus (EBV) infection is described. Chromosomal analyses revealed several clonal abnormalities and two of them were mainly repetitious. High copy numbers of monoclonal EBV genome were also detected in the proliferative large granular lymphocytes (LGLs), indicating the monoclonal expansion of EBV-infected LGLs. The patient had an indolent course for several years, and there was no evidence of infiltrations of his bone marrow until the end stage. At autopsy, microscopic studies revealed marked infiltrations of LGL in the liver and spleen, and the infiltrating cells were NK-cell immunophenotype. The infiltrated LGLs showed latency I.     

LUTEINIZING HORMONE RECEPTOR MUTATIONS IN DISORDERS OF SEXUAL DEVELOPMENT AND CANCER

Human male sexual development is regulated by chorionic gonadotropin (CG) and luteinizing hormone (LH). Aberrant sexual development caused by both activating and inactivating mutations of the human luteinizing hormone receptor (LHR) have been described. All known activating mutations of the LHR are missense mutations caused by single base substitution. The most common activating mutation is the replacement of Asp-578 by Gly due to the substitution of A by G at nucleotide position 1733. All activating mutations are present in exon 11 which encodes the transmembrane domain of the receptor. Constitutive activity of the LHR causes LH releasing hormone-independent precocious puberty in boys and the autosomal dominant disorder familial male-limited precocious puberty (FMPP). Both germline and somatic activating mutations of the LHR have been found in patients with testicular tumors. Activating mutations have no effect on females. The molecular genetics of the inactivating mutations of the LHR are more variable and include single base substitution, partial gene deletion, and insertion. These mutations are not localized and are present in both the extracellular and transmembrane domain of the receptor. Inactivation of the LHR gives rise to the autosomal recessive disorder Leydig cell hypoplasia (LCH) and male hypogonadism or male pseudohermaphroditism. Severity of the clinical phenotype in LCH patients correlates with the amount of residual activity of the mutated receptor. Females are less affected by inactivating mutation of the LHR. Symptoms caused by homozygous inactivating mutation of the LHR include polycystic ovaries and primary amenorrhea.     

A profile of respiratory symptoms in urban and rural South Australian school children

This report describes the cross-sectional analyses of data from the first year of a longitudinal study using questionnaire and respiratory function data over a 5 year period from a sample of rural South Australian school children. The cumulative or lifetime prevalences of respiratory symptoms were estimated in 825 rural and 1261 urban school children aged between 5 and 15 years in order to determine if the prevalence rates differed between rural and urban school children. The study found the overall cumulative prevalence of asthma and/or wheezy breathing (AWB) to be 24.1% in the rural school children compared to 27.6% in the urban school children. Most children developed AWB symptoms before the age of 7 years, with 20% reporting moderately severe symptoms and 10% having more than one attack per fortnight. The cumulative prevalence of bronchitis, loose/rattly cough (BLRC) differed significantly between the rural school children (34.1%) and urban school children (47.9%). The BLRC symptoms preceded the development of AWB in many cases. Urban school children also reported a higher prevalence of atopic conditions.     

Personality profiles and heart rate variability (vagal tone) in children with recurrent abdominal pain

The aim of the study was to explore psychological factors and autonomic activity in children with recurrent abdominal pain and to compare them with those in a control group of healthy children. The Personality Inventory for Children was used for assessment of developmental, emotional and psychosocial factors in 25 children with recurrent abdominal pain (age, 7-15 y). Parasympathetic and sympathetic functions in these children and in 23 healthy control subjects (age, 7-13 y) were also investigated, non-invasively using a computerized polygraph. Vagal tone (parasympathetic function) was indexed by calculation of respiratory sinus arrhythmia in beats/min. Skin conductance (sympathetic function) was recorded by the constant current method. On the Personality Inventory for Children, 16 patients had high scores on somatic concern. Several patients had scores in the clinical range for depression, withdrawal and anxiety, but the mean scores for these personality profile scales were well within the normal range of healthy children. Interestingly, there was a spike on the L (Lie)-scale for most of the patients and 15 patients had scores above or close to the clinical cut-off value. As compared with the scores in healthy children, vagal tone and sympathetic tone were normal. Conclusion: Many children with recurrent abdominal pain have scores in the clinical range for depression, withdrawal, anxiety and L-scale indicating coping problems, denial and a trend towards somatic concern that may contribute to the evolution of abdominal pain. Autonomic nerve activity was not disturbed in these children.     

Hauttemperaturen verschiedener Körperoberflächenareale des Rumpfes bei Säuglingen

Summary In two groups of infants (3–53 weeks old) skin temperatures were controlled in different areas of the trunk—i.e.: regions of sternum, lungs, heart, liver, spleen, kidneys—at different room-temperatures (group I: 21–25°C; group II: 29–32°C). Rectal temperatures of some probands in both groups also had been controlled simultaneously. A definite change in the reaction to heat was proofed in different periods of the first year of life. In higher environmental temperatures the skin temperature was almost constant at every controll-point of the skin, even in older infants. In lower environmental temperatures the skin temperatures lowered continuously with age till 7. to 9. moth. From 10. to 12. month the lowering of skin temperature discontinued. The rectal temperatures were relatively constant in all infants. Only in infants from 7. to 12. month, whose skin temperatures were controlled in lower as well as in higher environmental temperatures, a tendency to higher rectal temperatures was proofed in warmer environmental temperatures.The significance of these results is discussed.

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Untersuchungen mit Unterstützung durch die Deutsche Forschungsgemeinschaft.