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1.
Nephrotic syndrome in a mother and her infant: relationship with cytomegalovirus infection 总被引:1,自引:0,他引:1
Marisa Giani Alberto Edefonti Beatrice Damiani Giuseppina Marra Daniela Colombo Giovanni Banfi Emilio Rivolta Erich H. Strøm Michael Mihatsch 《Pediatric nephrology (Berlin, Germany)》1996,10(1):73-75
This case report describes infantile nephrotic syndrome (NS) in a baby girl with a clinically severe cytomegalovirus (CMV) infection. Culture of the baby's urine was positive for CMV and IgM anti-CMV antibodies were detected. After an unsuccessful course of corticosteroids, gancyclovir treatment was started and a remission of cutaneous, pulmonary, and renal symptoms was achieved. As the mother also developed NS at the end of pregnancy, a common etiology could be postulated, although there were no signs of recent CMV infection in the mother, only anti-CMV IgG. The relationship between CMV infection and glomerular disease is still unclear: NS may represent another manifestation of CMV disease. 相似文献
2.
Various neocortical areas from four females aged 16–24 years with Rett syndrome (RS) were investigated and compared with
brains of therapy-resistant partial epilepsy (TRPE) patients (18–25 years), infantile autism (IA), and control brains (24
and 58 years). The cytoarchitecture of area 10 (frontal), area 21 (temporal), area 4 (primary motor cortex), and area 17 (primary
visual cortex) was studied by the combined Klüver-Barrera (luxol fast blue and cresyl violet) standard procedure. Autofluorescence
of lipofuscin, immunofluorescence of synaptic vesicle proteins [synaptophysin (p38)] and lectin-stained (Wisteria floribunda agglutinin) perineuronal nets (PNs) were studied in the cortices using dual-channel confocal laser scanning microscopy. The
brains of RS females show various types of morphological/cytoarchitectonical abnormalities of single pyramidal neurons in
layers II–III, and V–VII of different cortical areas. The abnormalities include mild losses of pyramidal neurons, more pronounced
in layers II and III than in layers V and VII, and more evident in frontal and temporal areas than in the visual cortex. Microdysgenesis,
including abnormalities due to neuronal migration disorders, was not found in RS, in contrast to the observations in TRPE
patients, strongly indicating that RS is not a neuronal migration disorder. Lipofuscin distribution was normal but amounts
were lower in RS cases than in control and TRPE brains. PNs were less expressed in cortices of the IA case, but were clearly
overexpressed in the motor cortex of RS. Quantitative analysis of p38 showed a decrease in the area occupied by p38 immunoreactivity
by 20–40% in RS compared with controls. It is concluded that RS could best be explained by a postnatal synaptogenic developmental
deficiency; the basic defect, however, is still completely unknown.
Received: 26 February 1996 / Revised, accepted: 11 July 1996 相似文献
3.
Tracy A. Glauser 《Epilepsia》2004,45(S5):23-26
Summary: As patients with catastrophic epilepsies move from childhood to adulthood, evolving and innovative therapeutic regimens are often required. However, the goal of providing the best quality of life while minimizing both seizures and side effects remains the same. Clinicians can develop appropriate care plans by being aware of patients' changing needs. Clinical symptoms of the catastrophic epilepsies may change over time; by understanding the natural history of a patient's condition, clinicians can help ease the transition from childhood to adulthood. Additionally, as children with catastrophic epilepsies become adults, medical issues (e.g., medication side effects, tolerance, and dependence) and nonmedical issues (e.g., guardian/caretaker issue, group home applications, and respite care options) must be considered when developing strategies for patient care. Regular assessment of patients, the development of emergency plans, and maintenance of consistency in the delivery of care are also important issues to consider. Finally, a multidisciplinary care plan that incorporates resources from health-care practitioners, social service professionals, and community agencies can be valuable in optimizing treatment for patients with catastrophic epilepsies. 相似文献
4.
M. F. Gagnadoux J. L. Bacri M. Broyer R. Habib 《Pediatric nephrology (Berlin, Germany)》1989,3(1):50-55
Over a 15-year period we observed seven children (four girls, three boys) who presented within the first months of life with severe renal failure and acidosis, associated with hypertension in five patients and polyuria in four. In addition, one patient had a severe cholestatic liver disease. In two families, a similarly affected sibling had died previously. Four patients were referred with the clinical diagnosis of polycystic kidney disease because of moderate enlargement of kidneys, but renal imaging (intravenous pyelography and ultrasonography) did not confirm this diagnosis. A renal biopsy, performed in all patients, showed similar features characterized by a diffuse chronic tubulo-interstitial nephritis (TIN) and particularly by the presence of microcystic dilatation of proximal tubules and Bowman's space. Liver pathology was normal in two patients, including one with hepatomegaly. However, in the patient with cholestasis there was inflammatory portal fibrosis with mild duct proliferation. Progression of the renal disease was extremely rapid and all patients reached end-stage renal failure (ESRF) before the age of 2 years (11–22 months). Two children had successful renal transplants. Although this chronic TIN shares some features with nephronophthisis, we suggest that it represents a distinct entity both on clinical and morphological grounds. The specific clinical features of this disease are its early onset and rapid progression to ESRF. Pathologically, it differs from nephronophthisis by the absence of medullary cysts and thickened tubular basement membranes and by the presence of cortical microcysts. 相似文献
5.
Anthony M. Norcia Russell D. Hamer Arthur Jampolsky Deborah Orel-Bixler 《Vision research》1995,35(23-24)
Monocular oscillatory-motion visual evoked potentials (VEPs) were measured in prospective and retrospective groups of infantile esotropia patients who had been aligned surgically at different ages. A nasalward-temporal response bias that is present prior to surgery was reduced below pre-surgery levels in the prospective group. Patients in the retrospective group who had been aligned before 2 yr of age showed lower levels of response asymmetry than those who were aligned after age 2. The data imply that binocular motion processing mechanisms in infantile esotropia patients are capable of some degree of recovery, and that this plasticity is restricted to a critical period of visual development. 相似文献
6.
婴幼儿血管瘤病理结构变化与临床演变过程的联系 总被引:1,自引:0,他引:1
目的探讨婴幼儿血管瘤患者临床演变过程和病理结构变化的内在联系。方法选取52例婴幼儿血管瘤标本,年龄2个月至11岁。采用HE染色观察各阶段血管瘤的病理特点,使用图像分析系统分析血管瘤组织中的细胞总数量、微血管总数量和总面积。结果按患儿出生后时间:1~6个月,血管瘤中细胞增殖速度快、排列紧密,微血管数量迅速增加;7~12个月,血管瘤中细胞数量迅速减少,微血管数量减少,但总面积继续扩大;1~3岁,大部分血管瘤组织呈微血管团样,微血管面积最大;3~5岁,微血管数量和总面积均迅速减少;5岁之后,大部分血管瘤消退完成,被纤维脂肪组织代替。结论婴幼儿血管瘤患者临床演变过程和病理结构变化有密切联系,病理结构变化主导临床演变过程。 相似文献
7.
1986—1987年对云南省路南县5岁以下儿童随机抽样1016例,每月调查急性腹泻病发病情况。结果表明年发病率为1.43±0.07次/年。6月至1岁组发病率最高。4、5、6月为发病高峰季节,病原学检查以产肠毒素埃希氏大肠杆菌为主占39.1%,其次为轮状病毒,占26%。两年定群研究得出小儿急性腹泻病发病危险因素为饮用溏水沟水,随地大小便,多子女家庭与体弱多病儿童及人畜共居等。因此,改善供水,培养卫生习惯,加强计划生育等措施是该县防治本病主导措施。 相似文献
8.
Summary: The electroencephalographic/video recordings of 955 spasms in children with cryptogenic and symptomatic West syndrome (WS) were reviewed to define the relation between a clinical manifestation of a spasm and its EEG pattern, and to examine whether these features reflect the etiology and prognosis of WS. The review confirmed the spasm to be a distinct type of seizure, with a unique clinical and EEG pattern unlike that of all other recognized seizures. Symmetric spasms were present in cryptogenic and symptomatic patients. In contrast, asymmetric spasms, or focal signs recognizable during a spasm, strongly indicated the existence of a cerebral lesion. In both etiological groups, the characteristic ictal EEG pattern of the spasms consisted of a positive-vertex slow wave. The other two patterns apparently correlated to a spasm, were fast activity, here called spindle-like, and decremental activity. The fast activity corresponded to a clinical stare, and the decremental activity, when present, represented a postictal event. Although it was independent from the etiology of the spasms, persisting hypsarrhythmia during a cluster of spasms appeared to be an EEG pattern that correlated with a favorable outcome. 相似文献
9.
Monosomy 9q and trisomy 16q in a case of congenital solitary infantile myofibromatosis 总被引:1,自引:0,他引:1
Sirvent N Perrin C Lacour JP Maire G Attias R Pedeutour F 《Virchows Archiv : an international journal of pathology》2004,445(5):537-540
Although infantile myofibromatosis (IM) is the most common fibrous proliferation of infancy, many aspects of this benign lesion have not been explored. IM histogenesis is still poorly understood, despite immunohistochemical staining and ultrastructural features that suggest a myofibroblastic origin. IM diagnosis is often made difficult by the predominance of small primitive spindle cells over myofibrobasts and the presence of intravascular growth. Genetic information is scarce, with only one karyotyped case. Here we describe a case of solitary IM discovered at birth in an otherwise healthy girl. The tumor was well circumscribed, arranged in nodules and made up of ovoid cells without atypia, in a myxoid background. Immunohistochemical evaluation indicated a myofibroblastic differentiation. The cytogenetic and fluorescence in situ hybridization analyses revealed an abnormal chromosome 9, derived from an unbalanced whole-arm translocation between chromosomes 9 and 16. On both chromosomes, the breakpoints were located in the pericentric heterochromatic region. This clonal abnormality has not been reported in other tumors and is different from the chromosome 6q deletion reported in the single previous reported IM karyotype. 相似文献
10.