Febrile seizures]

The febrile seizures (FS) of the child are frequent. Their management is essentially based on the rigorous analysis of their characteristics, the clinical evaluation of the patient like on the collection of the family antecedents. In the case of "simple" FS, the forecast, always favourable, reduce the indications of assessment or treatment, including under possible repetitions. The possibility of an infection of the central nervous system must however always be isolated. In the case of "complicated" FS, the more important risk of later epilepsy makes often necessary of complementary explorations, as well as a treatment. Genetic predispositions were highlighted. In the case of a favourable forecast, contrast always large between the benignity of the FS and the parental dramatic experience. It must be taken into account in the evaluation and the choice of the therapeutic attitude.     

Lacerations involving glass. The role of routine roentgenograms.

OBJECTIVE--To determine, when evaluating a laceration caused by glass, whether seeing that the bottom of the wound is free of glass eliminates the possibility that glass is present in the wound. RESEARCH DESIGN--Prospective patient series. SETTING--Two pediatric emergency departments. PARTICIPANTS--226 children with lacerations due to glass occurring in a period of 21 months. SELECTION PROCEDURES--Consecutive sample. INTERVENTIONS--Before obtaining a roentgenogram, the triage nurse or the managing physician visually inspected each wound and recorded whether the bottom of the wound was seen, if glass was seen in the wound, and the length and depth of the wound. Further treatment of the wound was at the discretion of the managing physician. MEASUREMENTS/MAIN RESULTS--Glass was seen in the wounds of 10 children on initial inspection. Of the remaining 216 injuries, glass was contained in 12 (21.4%) of the 56 lacerations when the bottom of the wound was not visualized, and in 11 (6.9%) of the 160 lacerations when the bottom of the wound was visualized. There was a significant association between the depth of the wound and an inability to see the bottom of the wound, and deeper wounds were significantly more likely to contain glass. All but one of the lacerations containing glass had a depth of at least 0.5 cm. CONCLUSIONS--In the population studied, seeing that the bottom of the laceration was free of glass reduced, but did not eliminate, the possibility that glass was present in the wound. In addition, superficial lacerations (less than 0.5 cm) rarely contained undetected glass fragments. We recommended that further investigation concerning the mechanism of injury, the depth of the wound, and the type of glass involved is needed before physicians abandon routine roentgenography for lacerations due to glass.     

Glycosylation of therapeutic proteins in different production systems

Glycosylation plays an important role in a number of therapeutic proteins, including monoclonal antibodies. The enzymatic activity of a therapeutic protein is mainly determined by the protein structure, whereas the pharmacokinetics, pharmacodistribution, solubility, stability, enhancement of effector function and receptor binding are all influenced by the carbohydrate moiety. Hyperglycosylated proteins show increased serum half-life, are less sensitive to proteolysis and more heat-stable compared with the non-glycosylated forms. Molecular engineering of the TNK-tissue plasminogen activator molecule results in a more complex type of glycosylation and increases the half-life of the protein, which allows a single bolus injection at a lower dose for the treatment of acute myocardial infarction. Antibody-dependent cell cytotoxicity (ADCC) is determined partially by the specific N-glycosylation of the Fc domain of the monoclonal antibody. Specific glycoforms of monoclonal antibodies, which interact solely with the FcgammaRIIIa receptor of natural killer cells, result in superior ADCC compared with heterogeneous glycoforms that interact with different Fc receptors. This demonstrates that glycoengineering for directed glycosylation of therapeutic proteins can improve the therapeutic effect. While the amino acid sequence of the therapeutic protein is determined by the nucleotide sequence of the inserted gene, glycosylation depends on the glycosylating enzymes in the endoplasmatic reticulum and the Golgi apparatus of the eukaryotic host cell. In addition, the glycosylation of the therapeutic protein is affected by the culture medium used, the efficiency of protein expression and the physiological status of the host cell. CONCLUSION: For a given protein, changes in the type of host cell, composition of the culture media and fermentation conditions during process development will most likely result in changes in the site occupation and heterogeneity of glycosylation. This, of course, can influence the therapeutic profile. Therefore, the early selection of the host cell and selection of upstream parameters are key in the process development of a product.     

Heterogeneity and polymorphism of the thymus hyperplasia syndrome in children in the first 3 years of life]

Sixteen years of observation over a large group of children with the syndrome of the enlarged thymus with the use of up-to-date methods of examining the CNS, immune, endocrine, neuroendocrine systems and the autopsy data allowed the heterogeneity and polymorphism of the given syndrome to be revealed. Both transitory and stable enlargement of the thymus may be observed. The syndrome of the stable-enlarged thymus is likely to be formed in the intrauterine period and postnatally as well. It may be associated and not associated with thymus-dependent immunodeficiency. The morphological investigation of the thymus may show an enlargement of the cortical area with enhanced T lymphocyte proliferation in it or formation of lymphoid follicles. In clinical practice, the syndrome of the stable-enlarged thymus should be differentiated with thymic tumor. The working classification of the syndrome and concept of the pathogenesis of the stable-enlarged thymus are offered.     

Transmission of genital mycoplasmas from mother to neonate in women with prolonged membrane rupture

Colonization of the neonate with genital mycoplasmas occurs during passage through a colonized birth canal or in utero via contamination of the amniotic fluid. To define further the route of transmission we obtained cultures from the maternal vagina, the amniotic fluid and the neonatal pharynx in 131 mother-baby pairs. Sixty-six percent (33 of 50) of the corresponding amniotic fluids were colonized when the vagina was colonized with Mycoplasma hominis. When the amniotic fluid contained M. hominis, 26% (9 of 34) of the neonates were colonized. Sixty percent (66 of 110) of the corresponding amniotic fluids were colonized when the vagina was colonized with Ureaplasma urealyticum. When the amniotic fluid contained U. urealyticum, 32% (22 of 69) of the neonates were colonized. No neonates were colonized with M. hominis without prior colonization of both the vagina and the amniotic fluid. We conclude that colonization of the amniotic fluid is an important intermediate step in colonization of the neonate with genital mycoplasmas.     

Use of the colposcope in childhood sexual abuse examinations

The addition of the colposcope to the armamentarium of the medical investigator of childhood sexual abuse has many advantages. The ability to accurately record anatomic findings has implications for the medical community, the judicial system, the accused, the victim, and the family. Through the use of the photographs produced by this instrument, examiners can inspect physical findings without the time constraints imposed by the short attention span of the young child. If necessary, colleagues can be consulted and interpretations discussed. The availability of these photographs to the courts has reduced the need to re-examine the child for another opinion. The colposcopic photograph has also proved to be an excellent teaching and research tool. As an aid to teaching, the photographs and slides produced by this instrument help the instructor demonstrate anatomic findings while allowing the student time to ask questions. As a research tool, this instrument has opened up a myriad of possibilities for medical examiners. It has facilitated the collection of clinical data, it has allowed the standardization of examination techniques, and with the help of computers it has made possible the sophisticated analysis of the information collected. The colposcope has limitations. Aside from its cost, it is a difficult instrument to use in the examination of the young child. The time required for an examination can increase substantially as the examiner attempts to maneuver the scope into a proper position. During this procedure, the maintenance of the child in a suitable state of relaxation, while avoiding further emotional trauma, can be a challenge. The reality that the photograph is two dimensional and represents only the findings at that moment will always be a limiting factor in its use as a means of assessing a child's anatomy. Even the multimethod approach employed to offset this problem may compound the situation by further increasing the length of the examination. Most of these and other dilemmas encountered in the use of the colposcope can be solved by additional experience with this instrument. Despite the improvements brought about by the introduction of the colposcope, more advanced technology may be needed to help solve some of the problems currently plaguing medical examiners. The use of video tape could provide a solution to the documentation of the changes that occur in the soft tissues as the child moves or becomes more or less relaxed. The potential of the computer appears unlimited, and its application to the problem of the interpretation of findings could make a significant contribution to the field.(ABSTRACT TRUNCATED AT 250 WORDS)     

An index for the evaluation of growth in children

Conclusion Proper assessment of a child's growth as time passes is essential as a guide for treatment of disease or disability. The best possible progress for the child who has genetic or acquired handicaps is promoted in various ways, but principally by helping the parents to understand the particular needs of their child at each stage and to protect it from unfavourable environmental influences. A knowledge of growth and development of the child is of basic importance to the physician who would attempt to care for children and to advise parents as to normality of their progress. The index developed here is a step towards this goal. It helps the physician to assess the growth of the child from the clinical point of view and the parents to evaluate the development of their child. It is hoped that the index will increase the knowledge of both the physician and the parents in understanding the growth pattern of children. From the Department of Preventive and Social Medicine. G.R. Medical College, Gwalior.     

Adaptation of a single injection clearance method to physiological and pathophysiological facts. A review of data obtained in infancy and childhood.

A retrospective analysis of 395 51Chromiumethylenediaminotetraacetate single injection clearances performed in infants and children is presented. In 61% of infants and 30% of the children the clearance values were calculated on the basis of a plasma disappearance half time of the reference substance, which was longer than the standard study, i.e. on the basis of extrapolated data. Plasma creatinine and urea levels were found to be appropriate indicators for predicting the plasma disappearance half time of the marker substance. 14 additional patients were studied prospectively with a duration of the study predicted by means of the plasma creatinine and urea levels. In these patients, separate determinations of the clearances using either the data obtained during the standard time of procedure only, or the data of the entire study, clearly demonstrated that the clearances obtained by means of the standard procedure overestimated glomerular filtration rate. The analysis of the data in infants show that the plasma urea level is a reasonably good indicator for predicting the time schedule of the study whereas plasma creatinine should not be used. Additionally the retrospective data indicate that a prolongation of the study should be recommended in all infants. This study demonstrates the necessity and offers means of adapting the time schedule of isotope single injection clearances to physiological and pathophysiological facts.     

In-vitro studies on the kinetics of bone-marrow erythropoesis during the first trimester of life (Trimenonreduction)]

This study tries to give further insight into the mechanism and location of the physiological reduction of the bone marrow erythropoiesis during the first trimester (Trimenonreduction). Methods utilized included red blood values, bone marrow morphology, 3H-Thymidine Autoradiography, Feulgen-cytophotometry and 3H-, 14C-Thymidine double labelling techniques of bone marrow erythroblasts of healthy children of different age groups. Besides already known techniques we used especially a modification of the double labelling techniques, developed in our laboratories. We draw the following conclusions from our results: 1. Newborns have a higher rate of bone marrow erythropoiesis in comparison with normal controls of other ages. The reduction of the bone marrow erythropoiesis starts already in the first 2 days of life. 2. The reduction of the bone marrow erythropoiesis in the investigated infants in the second week of life was about to one fifth of values which proved to be normal for healthy older children. 3. This reduction is caused partially by prolongation of proliferation and maturation phases of erythroblasts, partially by a decrease of new erythroblast-formation from the stem cell pool. Medium values of DNA-synthesistime of infants with the highest reduction is double compared with values of healthy controls in vitro. 4. The decrease of cell proliferation and maturation during the reduction of the bone-marrow erythropoiesis includes all precursors and all phases of the cell cycle equally. In the first few days of life however it seems that the decrease of DNA synthesizing erythroblasts surpasses the reduction of maturing cells. 5. An ineffectiveness of erythropoiesis could not be found responsible for the reduction. 6. The reduction in erythropoiesis is seen in those steps in which other autors found stimulations by erythropoietin. Therefore this study supports the thesis, that the trimenonreduction is caused by a lack of erythropoietin stimulation. 7. The sequence of the trimenon reduction in humans is different from results found in animals.     

Structure of interstitial nephritis in children]

The authors provide the data on 24 cases of interstitial nephritis in children treated at the Nephrological Center of the city of Kuibyshev in 1986-1990. Describe the etiological structure of interstitial nephritis using the classification developed by N. A. Korovina and coworkers (1982), randomization according to the age, sex, and the disease course. Demonstrate the predominance of the postviral and toxicoallergic disease patterns in children of the early, preschool and junior school age. Estimate the importance of the types of urine proteinograms in the diagnosis of interstitial nephritis.     

成纤维生长因子在大鼠后肢骨骼发育早期不同阶段表达的变化及意义

目的通过大鼠肢体发育早期血清学成纤维生长因子（fibroblast growth factors,FGFs）的表达以及后肢标本中碱性成纤维生长因子（base fibroblast growth factor,bFGF）的表达情况,观察其在大鼠后肢肢体骨骼发育早期过程中,组织学上随时问变化的规律。方法将5只清洁级孕鼠的新生鼠分组为A、B、C、D、E五组,分别为10、6、6、8、6、6只。用ELISA法测定各组血清中FGF的浓度,并对其后肢标本进行bFGF的免疫组化染色,从而观察bFGF在关节软骨、骨骺、干骺端及骨膜中的表达情况。结果在新生小鼠的后肢标本免疫组化染色中,bFGF在软骨、骨膜和关节面均有表达,且随出生后时问的变化存在一定的规律：①在关节软骨中bFGF在关节面软骨的表达要强于中心部位的软骨,这种表达随出生后天数增加呈逐渐减弱的趋势。@bFGF在骨骺部增殖层和肥大细胞层以及骨膜内早期表达较强,随时间变化呈逐渐减弱趋势,在出生后10d以后bFGF表达已不明显。③在干骺端成骨细胞内bF-GF的表达不同于它在其他组织内的表达,一直呈现为较高表达,并不随生后时间的延长而呈现减弱趋势。结论在大鼠后肢发育早期,bFGF在后肢肢体各组织中存在良好的表达,提示它在肢体发育过程中与软骨增殖、关节发育与关节内软骨基质的形成和发育有密切的关系。     

Mucopolysaccharidosis 6-A (Maroteaux-Lamy disease): comparison of clinical and pathologico-anatomic findings in a 27-year-old patient

The autopsy of the 27-year-old patient suffering from a severe form of mucopolysaccharidosis type VI-A (Maroteaux-Lamy) described in the article by Rampini et al. revealed the compressive cervical myelopathy (C1) to be due to a severe, slit-like deformity of the vertebral canal which was due to a massive thickening of the ligaments, dislocation of the posterior arch of the atlas and broadening of the dura. The massive, generalized disorder of the enchondral ossification involving mainly tubular bones and spine, led to short stature, arthrosis of large joints, and to an angular thoraco-lumbar kyphoscoliosis. Our patient had a massive V-shaped stenosis of the upper third of the trachea. The atrio-ventricular valves of the heart were thickened, and there was fibrosis of the endocardium. The cardiac conduction system displayed fibrosis and foam cell transformation of the fibroblasts in the bundle of His, a lesion not previously reported. Light and electron microscopic investigation showed a massive increase of mucopolysaccharides mainly in the matrix of the fibrous tissue and of the cartilage. Furthermore, the fibroblasts and chondrocytes as well as the hepatocytes and the ganglion cells of the central nervous system contained increased amounts of glycosaminoglycans.     

Ataxia-Telangiectasia

Five cases of ataxia-telangiectasia are reported. In 4 cases, there is no parental consanguinity. In the other case the parents are unknown. The maternal grandfather of one of the patients hadtelangiectasiae in the scalp, face and ears, but no ataxia and the γ_A-globulin content in the serum was normal. There was no known case of ataxia in any of the families. All of the patients had had bronchitis, but only 3 of them had been particularly susceptible to respiratory infections. The growth was retarded in all. None of them presented deformities of the feet or kyphoscoliosis. Only in one patient was the motor development retarded during the first year of life, in the remaining the disturbance of the psychomotor development appeared in the form of ataxia in the course of the second or third year of life when the patients started to walk. One patient developed telangiectasiae at 2-3 years of age, the others a t 5-7 years of age. Patches of poor pigmentation of the skin in the fa03 were present in 4 patients. Choreoathetotic movements and a tendency to turn the eyes upwards when focusing was observed in 4 cases. The sensibility was normal in all cases. The deep reflexes were normal in one case and impaired in four. In the latter normal reflexes had been demonstrated at an earlier age. Intelligence fell within the lower limits of normality in all patients. No definite progressive impairment was demonstrable. The EEG was normal in 2 cases. In one patient a moderate general cerebral dysrhythmia was found, most marked over the right temporal region but no epileptogenic activity. I n another a focus was registered in the left temporal region. One patient had not been submitted to EEG. Paper chromatography of the urine revealed a normal amino acid pattern in 4 cases, the 5th was not examined.     

儿童哮喘综合治疗管理与心理影响研究

对60例哮喘患儿采用WHO全球哮喘防治策略，使用持续倍氯米松加间断沙本胺醇吸入治疗、峰流速仪肺功能监测、检测及避免触发因素、社会教育管理、心理干预等综合疗法，跟踪观察1年。结果显示：总有效率为98.3%，住院率、急诊率，因病缺课率均下降，患儿和家长对治疗疾病信心增强，心理障碍改善。学习成绩进步，生活质量提高。     

先天性巨结肠Cajal间质细胞的研究

目的　本研究通过观察Cajal间质细胞 (interstitialcellofCajal,ICC)在先天性巨结肠患者狭窄段、移行段、扩张段中的分布情况 ,探讨ICC在先天性巨结肠发病中的作用。方法　收集我院1999～ 2 0 0 2年 2 6例先天性巨结肠患儿标本。短段型 2 4例 ,长段型 2例。于手术中分别选取扩张段 ,移行段及狭窄段肠壁的全层组织。采用SP法 (过氧化物酶标记的链霉卵白素法 )免疫组织化学技术 ,对 2 6例先天性巨结肠的狭窄段、移行段及扩张段标本分别进行c kit免疫组织化学反应 ,观察Ca jal间质细胞分布情况。 结果　发现ICC的密度从扩张段→移行段→狭窄段是逐渐减低的。ICC与肌间神经丛关系密切 ,在扩张段ICC分布在神经丛的周边部和内部 ,且数量相对较多 ,在狭窄段ICC偶见于神经丛的周边部 ,在神经丛内部未见该细胞。光学显微镜下比较同一例患者扩张段和狭窄段神经丛中Cajal间质细胞的数目不同 (t=2 3.0 4 ,P <0 .0 5 ) ,有统计学显著性差异。结论　ICC的分布异常与先天性巨结肠的发生有密切关系。我们推测胚胎基质的某种缺陷不仅损害了神经嵴细胞的移行 ,也影响ICC的分化和成熟。我们可以推论 ,与HD肠壁神经节缺失一样 ,ICC分布异常导致HD病变肠管慢波节律和兴奋传导异常 ,从而引起或加重HD的发病。     

A Study of the Body Image of Children with Chronic Renal Failure

This paper describes a study designed to evaluate the body image of children with chronic rental failure. The primary goal of the study was to gather baseline data on the effect of chronic renal failure on the developing body image of the child. The body image of sixteen subjects ranging for the ages of five to fifteen years was evaluated through the use of the Draw-A-Person Test, and the sub factor items of physical appearance of the Piers-Harris Self-Concept Index. The mental age and intellectual maturity of the subjects were determined by administering the Slossen Intelligence Test and Goddenought-Harris Test of Intellectual Maturity. Pertinent information such as chronological age, race, sex, and the length of the disease process was determine thought an interview and a review of medical records. Body image was not found to be significantly distorted. Mental age and intellectual maturity were found to be delayed. There were not significant differences in performance on any of the test of the children on dialysis versus the children not on dialysis. The development of both the body image and mental capacities seemed delayed. This indicates the need for further investigation of possible developmental delays in the child with chronic renal disease.     

Certain characteristics of the perinatal development of children whose parents were exposed to the action of uranium fission products]

Analysis was made of the character of the perinatal development of 238 children. In 65 of these, the parents were exposed to the action of uranium fission products in the basin of the river Techa in the Chelyabinsk region. The parents were radiated approximately 5 years before the children's birth. The mean equivalent dose for endocrine glands (including gonads) of the parents amounted to about 12 centiSievert U. As compared to the control group, the basic one manifested a higher incidence of placenta and umbilical cord pathology. In the group of the radiated persons' progeny, the number of small-weight children appeared greater; a larger physiological reduction of the body weight was recorded as were later times of funic residue falling off.     

Kirner's deformity of all fingers in a 5-year-old girl: soft-tissue enhancement with normal bones on contrast-enhanced MRI

Kirner's deformity is an uncommon, but characteristic volar-radial incurvature of the distal phalanx of the little finger. We report a 5-year-old girl with dystelephalangy of all fingers. Some members of the mother's family showed dystelephalangy of the little fingers; the father's family history was unaffected. Contrast-enhanced MRI showed enhancement of the soft tissues of the distal phalanges, but no bone deformities. This leads to the assumption that the radial and volar deviation of the distal phalanges is the result of a chronic inflammatory process or a vascularisation disorder of the soft tissue.     

Primary Noncompaction of the Ventricular Myocardium from the Morphogenetic Standpoint

This review compiles the current knowledge of normal and abnormal myocardial morphogenesis to facilitate an unambiguous diagnosis of primary myocardial noncompaction. During the early stages of development, the formation of trabeculae with the resulting increase in myocardial surface is a adaptation of the rapidly growing heart to improve nourishment by exchange diffusion from the cardiac lumen. Once the coronary vasculature has developed, the switch to cardiac nutrient supply through active circulation from the subepicardial space is paralleled by gradual compaction of the myocardial trabeculae. This results in a decrease of the inner, trabeculated myocardial layer with a parallel increase in thickness of the outer, compact myocardial layer. Similar to the direction of coronary arterial development, this process proceeds from the epicardium toward the endocardium and from the base of the heart to the apex. Based on developmental data, congenital myocardial noncompaction represents a failure of normal embryonic myocardial maturation. The time of arrest of this process will determine the extension of myocardial noncompaction within the ventricle. Whereas disturbances of myocardial microcirculation are frequent in these hearts, direct communications between the myocardial cavity and the coronary arteries (sinusoids) do not belong to this morphogenetic entity.     

Intracranial tumors in the 1st year of life

The introduction of the new diagnostic tools for neuroimaging has resulted in the early recognition of congenital brain tumors. In the present report we describe 39 children, in whom the diagnosis of an intracranial tumor has been obtained during the first 12 months of life. These patients represent 14.4% of a pediatric population of 271 children with brain tumors, observed in the same period of time (11 years) in the Neurosurgical Institute of the Catholic University of Rome. Most of the tumors were located within the supratentorial compartment. Midline tumors were common. Sixteen children underwent the total removal of the tumor. Four infants could not be operated either because their poor general condition or the extension of the tumor. In one case, treatment was refused by the parents. The remaining children underwent the partial removal of the tumor, which was followed by chemotherapy (malignant tumors) or by serial neuroradiological examination (benign tumors), with the aim of postponing radiotherapy at the end of 2 years of age at least. The total mortality (surgical deaths: 7 cases) and deaths during the follow-up period (11 cases) was 38.5%. Out of the 24 surviving children, 8 (20%) are normal: 7 (17%) exhibit only minimal neurosurgical deficits; 9 (22%) are seriously handicapped. One child has been lost for the follow-up observation.