IMMUNOHISTOCHEMISTRY OF MEDULLOEPITHELIOMA AND NEURAL TUBE

Immunohistochemistry profiles of medulloepithelioma (from two 2 1-year-old girls who had 2 cerebral medulloepitheliomas and a 35-week postconceptional female infant with congenital posterior fossa tumor) and neural tube are compared. Microscopically, the tumors contained a medulloepitheliomatous component, manifested as tubular epithelial structures lined by pseudostratified columnar epithelium delineated by well-defined basement membranes. In all cases, glial and neuronal differentiation were noted to differing extents. The medulloepitheliomatous components did not exhibit glial fibrillary acidic protein, neuron-specific enolase, or S-100 protein reactivity. Neurofilament, cytokeratin, and epithelial membrane antigen were focally present in one case. Extensive nestin immunopositivity was confined to the basal cell layer of the epithelium, leaving the luminal surface unreactive or slightly reactive. These cells also displayed a reactivity to vimentin and to microtubuleassociated protein type 5 similar to that of cells of the primitive neural tube. The similarity between the immunohistochemical profile of medulloepithelioma and that of neural tube epithelium suggests a possible reexpression of that component of the genome responsible for neural tube growth and differentiation in medulloepithelioma.     

PRACTICE OF PALLIATIVE SEDATION IN CHILDREN WITH BRAIN TUMORS AND SARCOMAS AT THE END OF LIFE

Despite progress in the treatment of pediatric cancer, approximately 25% of these children will die of the disease. The last period of life is characterized by profound physical and psychological suffering, both of the children and their loved ones. Adequate alleviation of this suffering becomes the priority in the management of these patients. The authors retrospectively evaluated the indications, incidence, and characteristics of palliative sedation (PS) in 19 children with brain tumors (BT) and 18 with sarcomas (S) at the end of life. Twelve of the 18 S patients received PS, as did 13 of the 19 BT patients. Indications for initiation of PS for those with BT were seizures and/or pain, for those with S were pain and/or respiratory insufficiency. It was concluded that PS may be the only efficacious and safe treatment for the alleviation of suffering in these children at the end of life, despite differing indications.     

CHILD CHARACTERISTICS AND THE EMPLOYMENT OF MOTHERS WITH YOUNG CHILDREN: A LONGITUDINAL STUDY

The present longitudinal study investigated the relative influence of child, maternal, and demographic characteristics on the labor force participation of 93 mothers with young children in the New York Longitudinal Study. Multiple regression analyses revealed that child characteristics such as temperamental difficulty and the presence of physical problems were as potent as demographic features in predicting the mother's labor market activities throughout the child's early years. Conclusions were that multiple aspects of the context, including child characteristics, must be addressed if a more comprehensive picture of the employment patterns of mothers is to be gained.     

CHARACTERIZATION AND PROPERTIES OF THE HUMAN AND BOVINE LACTOTRANSFERRINS EXTRACTED FROM THE FAECES OF NEWBORN INFANTS

ABSTRACT. Human lactotransferrin was characterized in the faecal extracts from breastfed babies. Quantitative determination of human copro-lactotransferrin from birth for a period of up to 3 weeks showed that the daily elimination decreased from 35 to 5 mg. The amount of copro-lactotransferrin corresponding to the endogenous secretion was calculated to be from 0.5 to 1mg per day. When a cow's milk diet supplemented by partially or completely iron-saturated human or bovine lactotransferrin was fed to the babies, the amounts of coprolactotransferrin excreted depended on the origin and on the iron saturation of the lactoransferrin. In particular, the amount of bovine copro-lactotransferrin in the faeces averaged 200 mg per day. The human and bovine copro-lactotransferrins were isolated by ion-exchange chromatography or by affinity chromatography and were still able to bind iron. The fingerprints of native human and bovine lactotransferrins hydrolysed in vitro by infant's gastric or duodenal secretions showed that both proteins were not extensively digested. This demonstrates that these lactotransferrins ingested by babies are not completely destroyed and keep their ability to bind iron, and thus may supplement the bacteriostatic effects of the endogenous lactotransferrin in the intestinal tract.     

IMMUNOHISTOCHEMICAL DISTRIBUTION OF VASCULAR ENDOTHELIAL GROW TH FACTOR IN THE HUMAN PLACENTA ASSOCIATED HYDROPS FETALIS WITH

The purpose of this study was to determine the distribution and the expression of vascular endothelial growth factor VEGF in human placentas obtained from 22 cases of nonimmunologic hydrops fetalis NIHF , ranging from 25 to 41 weeks of gestation, as compared with those in 75 normal placentas. The terminal villi were examined immunohistochemically using rabbit anti-human VEGF immunoglobulin G. The expression of VEGF in the terminal villi was assessed by the ratio of the VEGF-positive area to the capillary area. Among the 22 placentas, 18 placentas were hydropic and appeared to be histologically immature, and the other 4 placentas, whose fetuses eventually recovered before their deliveries, were nonhydropic. In both the HF and normal placentas, syncytiotrophoblasts expressed VEGF in the villi throughout gestation. VEGF was also expressed by stromal cells in the terminal villi of normal placentas in only the first trimester, whereas its expression continued until the term of pregnancy in 12 of 18 HF placentas. The ratio of the VEGF-positive area to the capillary area was significantly higher in the terminal villi of all hydropic HF placentas than in those of the normal placentas but was entirely the same in the four nonhydropic placentas as in the normal control placentas. These results suggest that the persistent expression of VEGF by stromal cells in the hydropic villi of NIHF, in addition to the VEGF expression by syncytioptrophoblasts, seems to be a reaction to poor villous vascular development and to participate in the regulatory mechanisms of vascular function including angiogenesis and permeability.     

STROMAL-PREDOMINANT MESENCHYMAL HAMARTOMA OF THE LIVER WITH ELEVATED SERUM ALPHA-FETOPROTEIN LEVEL

Mesenchymal hamartoma of the liver (MHL) is an uncommon, benign, tumor-like lesion and is usually diagnosed in the first 2 years of life. Its pathogenesis remains unclear. Treatment of choice is radical excision. The authors report a case of solid stromal predominant MHL in a 12-month-old male infant who also had an elevated serum α-fetoprotein level. He also had hypospadias, which might represent a spectrum of developmental anomalies. It usually presents as an asymptomatic mass, however, as in the reported case, it may cause several complications due to the compression of surrounding structures. He was successfully treated with total excision of the pedunculated large tumor without any complication.     

COLOBOMATA OF THE IRIS, CILIARY BODY AND CHOROID IN AN INFANT WITH OESOPHAGO-TRACHEAL FISTULA AND CONGENITAL HEART DEFECTS. AN UNKNOWN MALFORMATION COMPLEX

Abstract. Lillquist, K., Warburg, M., Ry Andersen, S. and Hägerstrand, I. (Paediatric Department, Odense University Hospital, The Copenhagen Eye Clinic for the Mentally Retarded, The Institute of Eye Pathology in Copenhagen and Pathological Institute, Odense University Hospital, Denmark). Colobomata of the iris, ciliary body and choroid in an infant with oesophago-tracheal fistula and congenital heart defects. An unknown malformation complex. Acta Paediatr Scand, 69:417, 1980.—A malformation pattern, not previously reported consisting of microphthalmia, uveal coloboma, oesophago-tracheal fistula and congenital heart defects is presented. The phaenotypical similarity and difference from previously reported cases are discussed.     

THE ELEVATED MARKERS OF HYPERCOAGULABILITY IN CHILDREN WITH HENOCH-SCHÖNLEIN PURPURA

Twenty-eight children with HSP and 79 healthy children were entered into study. Activities of protein C, free- protein S and antithrombin, activated protein C resistance, levels of fibrinogen, D-dimer, thrombin-antithrombin complex (TAT), prothrombin fragments 1+2 (PF_{1 + 2}) and von Willebrand factor antigen (vWAg) and its activity (RiCof), were investigated in acute and recovery phases of HSP and controls. Fibrinogen, D-dimer, TAT, PF_{1 + 2}, vWAg and RiCof levels in patients with HSP during the acute phase were significantly higher than those of recovery phase and of the controls. A significant correlation was detected between severity of disease and TAT, PF1+2, vWAg, D-dimer levels.     

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??Objective??To analyze the clinical characteristics and outcomes of children with stage I testicular germ cell tumors??TGCT????and to discuss the treatment strategy for this disease after radical inguinal orchiectomy. Methods??Clinical data of 41 patients with stage I TGCT at Shanghai Children’s Medical Center??Shanghai Jiaotong University School of Medicine between June 2003 to December 2013 were retrospectively analyzed. Their clinical characteristics??therapy and outcomes were analyzed. Results????1??The median age at diagnosis was 18??3 to 43??months old. Among them??38 children??92.7%?? were younger than 3 years old.The pathological subtype included 35 cases of yolk sac??85.4%????4 immature teratoma??9.7%?? and 2 mixed germ cell tumors??4.9%??.??2??Serum AFP levels were elevated in 38 patients??92.7%??. AFP reduced to normal in 27 cases??71.1%??4 weeks after surgery and in 35 cases??92.1%?? after 2 courses of chemotherapy respectively??but 2 cases of them relapsed with AFP increasing again.??3??Forty out of 41 patients underwent surgery and 3??4 courses of chemotherapy with cyclophosvnamide??vincristine and dactinomycin D??VAC??. One patient received surgery alone. The median follow-up period was 64 months. One of the 32 patients who received adjuvant chemotherapy relapsed and then lost follow-up without treatment. The recurrence rate was 3.1% and the 5-year overall survival was 100%. No complication related to chemotherapy occurred during the follow-up. The only one patient treated with surgery alone relapsed at 5 months and achieved complete response after salvage treatment. Conclusion????1??The relationship between the decrease of serum AFP and the prognosis is uncertain and needs to be further studied.??2??Short course chemotherapy with mild toxic side effects can reduce the recurrence rate effectively?? so it can be used as a necessary treatment for patients with high risk of relapse??and it can also be used as a selective treatment for patients with low risk of recurrence.     

THE STATE OF HEALTH OF SWEDISH CHILDREN

Abstract. Sjölin, S. and Smedby, B. (Departments of Paediatrics and Social Medicine, University Hospital, Uppsala, Sweden). The state of health of Swedish children. An attempt is made briefly to describe the present health problems of Swedish children. The report is based on data available in official statistics or collected from special studies. It is concluded that the general state of health of Swedish children is good when assessed by commonly used criteria. The mortality pattern is dominated by perinatal and accidental deaths, but also neoplasms are of importance. There are indications that genetic and handicapping disorders, and psychosocial maladjustment will receive more attention in the future.     

ADOLESCENTS WITH CYSTIC FIBROSIS IN THE NETHERLANDS

ABSTRACT. In a survey-study of all living Dutch patients with cystic fibrosis (CF) born before 1971 data have been gathered on sex, age, age at diagnosis; height and weight; treatment and social development. The group consists of 102 schoolchildren (8–11 years), 179 adolescents (12–21 years) and 28 young adults (22–40 years). Males are in the majority, especially in the 12 to 17 age group (61 %). In 51% of all patients CF was diagnosed before the age of 2, and in 75% before the age of 5. Male CF patients are on average 6 cm (0.84 SD) shorter than normal and female patients 3 cm (0.48 SD). As far as weight for height is concerned, 54 % of the CF patients fall below the 10th percentile. Weight for height is slightly lower in females than in males. CF patients usually leave primary school at an older age than their healthy peers. In comparison to healthy adolescents of the same age, adolescents with CF attend full-time day-school for a longer time, more of them receive general education and fewer take vocational training. Adolescents and young adults with CF do not stay longer than normal in the protective environment of their parental home.     

过敏性紫癜患儿血钙、血磷变化临床观察及分析

为了探讨过敏性紫癜(ＨＳＰ)急性期血钙、血磷浓度的变化及意义,对６４例ＨＳＰ急性期患儿进行了血钙测定,同时测定其中３８例患儿的血磷。结果显示:(１)６４例患儿的血钙为:２．１０±０．２６ｍｍｏｌ/Ｌ,仅１例血钙在正常范围均值以上,６５．６%的患儿存在低钙血症,３８例患儿的血磷为:１．４７±０．３４ｍｍｏｌ/Ｌ,４２．１%存在低磷血症。(２)单纯型、关节型、腹型患儿血钙、血磷无显著性差异;关节型合并腹型患儿血钙显著低于单纯型、关节型和腹型患儿;紫癜性肾炎合并腹型和(或无)关节型患儿血钙显著低于其他患儿,血磷显著低于单纯型、关节型和腹型患儿。结论:ＨＳＰ急性期普遍存在血钙降低,近半数患儿存在低磷血症,且病情愈重,血钙、血磷愈低;检测血钙、血磷对评估病情轻重有参考价值。     

FINE-NEEDLE ASPIRATION OF SOLID AND PAPILLARY CYSTIC TUMOR OF THE PANCREAS

Solid and papillary cystic tumors of the pancreas are relatively rare tumors that occur in adolescent and young adult women. A case is presented in which preoperative diagnosis was made by fine-needle aspiration biopsy obtained under computed tomographic guidance. The cytologic smears were characterized by branching, papillary clusters of cells having a thin, well-defined, fibrovascular core and surfaced by multiple layers of cells. Sheets of cells were also present. The tumor cells had small, round to oval nuclei with finely stippled chromatin and an occasional small nucleolus. The nuclear membrane was delicate and nuclear infolding was frequent. The cytoplasm was scant and cell borders were indistinct. Mitotic figures were notably absent. The diagnosis was confirmed histologically following surgical resection. As this tumor has a favorable prognosis with complete surgical removal, fine-needle aspiration can play an important role in preoperative planning by distinguishing it from other pancreatic lesions with significantly different prognosis and treatment. Therefore, pediatric pathologists should be familiar with the cytologic manifestations of this tumor.     

GONADAL DYSGENESIS WITH LYMPHOCYTIC THYROIDITIS AND DELETION OF THE LONG ARM OF THE X CHROMOSOME

A 17-year-old girl with several stigmata of gonadal dysgenesis and a 45, X/46, XXq? mosaicism is presented. She had small hypoplastic ovaries and regular menstruations, and a lymphocytic thyroiditis. The relationship between gonadal dysgenesis and lymphocytic thyroiditis is discussed.     

PROGNOSIS FOR CHILDREN WITH SUPRATENTORIAL NEUROGLIAL TUMORS

Factor analysis of reliably identified histologic features in supratentorial glial tumors yielded five interpretable "factors": Spongy, Fibrillary, Proliferative, Jumbo, and Oligodendroglial. Quantitative scores can be calculated for each factor in a tumor to summarize its heterogeneity. The objective was to investigate whether factor scores are useful for prognostic purposes. The sample consisted of 703 children with supratentorial neuroglial tumors with factor scores for each of the five factors. Data were based on the presence or absence of 26 reliably identified histologic features, plus clinical and survival information. Multivariate proportional hazards models assessed each factor's contribution to survival for children who survived 1 month after operation ( n 609). Patient-specific clinical data were allowed in the models. Increased likelihood of survival is associated with greater tumor removal, later decade of surgery, and high Spongy and high Oligodendroglial factor scores. Decreased likelihood of survival is associated with high Proliferative factor scores and radiation and or chemotherapy treatment. Gender, age, location, and Jumbo and Fibrillary factor scores did not provide additional prognostic information. Three reliable histologic features, nondefining for any histologic factor, added prognostic information: Rosenthal fibers and glomeruli are associated with improved prognosis; pleomorphic nuclei are associated with worse prognosis. A high Oligodendroglial factor score is associated with a worse prognosis for some classes of astrocytoma but with a better prognosis for oligodendroglial tumors. A high Proliferative score is associated with a worse prognosis for anaplastic astrocytomas, ependymomas, and unclassifiable tumors. A high Spongy score is associated with a better prognosis for anaplastic astrocytomas but with a worse prognosis for pilocytic astrocytomas. For giant cell astrocytomas, gangliogliomas, and miscellaneous tumors, none of the factors is prognostic. Spongy, Oligodendroglial, and Proliferative factors provide important prognostic information for children with supratentorial neuroglial tumors.