Utility of PAS and β-catenin staining in histological categorisation and prediction of prognosis of hepatoblastomas

Aims

The aim of this study was to assess the usefulness of PAS, β-catenin and Ki-67 in categorising hepatoblastomas (HBs) and their significance in predicting prognosis. In addition, we have also documented the various chemotherapy induced histologic changes in HBs.

Method

In this retrospective observational study of 29 cases of hepatoblastomas, 27 cases were considered for statistical analysis, excluding two cases of diagnostic core biopsies. All clinicopathological parameters and follow up data were collected. All HB cases including the mixed epithelial and mesenchymal HBs were classified into two groups: fetal predominant and embryonal predominant type according to the predominant epithelial component. PAS, β-catenin and Ki-67 staining were done and their correlation with histologic subtypes was assessed. Kaplan–Meier survival analysis was performed in relation to histology, PAS, β-catenin and Ki-67 staining characteristics.

Result

Diffuse nuclear staining of β-catenin was significantly more common in embryonal predominant type (p = 0.036), whereas strong PAS positivity was significantly associated with fetal predominant type (p = 0.002). But no significant correlation was seen between histologic type and Ki-67 staining (p = 0.42). Survival analysis showed cases with diffuse PAS positivity, focal nuclear β-catenin staining and low Ki-67 LI had better survival.

Conclusion

PAS is a simple stain and can be utilised in histological categorisation of HB and also predicting its outcome. Nuclear β-catenin staining which is significantly common in embryonal elements in HB predicts shorter survival.

     

Which method of breastfeeding supplementation is best? The beliefs and practices of paediatricians and nurses

The aims of the present study were to assess the practices of breastfeeding supplementation methods, and to explore the opinions and beliefs of health professionals with regard to breastfeeding supplementation methods and the nipple confusion phenomenon. The study was cross-sectional in design, and participants were recruited from five hospitals in Toronto, Ontario. All of the nurses and attending paediatricians in postpartum floors and level II nurseries were invited to participate in the study. A total of 87 nurses and 16 paediatricians completed the survey questionnaire. Bottle feeding was the most common breastfeeding supplementation method used in the nurseries followed by cup feeding. Only 15.0% of the level II nurses agreed that frequent bottle feeds lead to the nipple confusion phenomenon, compared with 44.4% of the postpartum nurses and 56.2% of the paediatricians. Findings demonstrated considerable variation in the practices and beliefs surrounding supplementation methods. A randomized controlled trial comparing the safety, efficiency and subsequent breastfeeding rates of different breastfeeding supplementation methods is warranted.     

Possible association of Down syndrome and exstrophy–epispadias complex: report of two new cases and review of the literature

In the past, several midline defects have been associated with Down syndrome (DS) on a regular basis, e.g. heart defects, cleft lip and palate, neural tube defects, omphalocele and anal atresia. The exstrophy–epispadias complex (EEC) represents a rare midline defect, rarely described in association with DS. Here, we report on the co-occurrence of DS and EEC in two, so far, unreported cases and present a review of the literature. We suggest that EEC represents a rare but inherent part in the spectrum of DS-associated midline defects.     

Nonparental care and infant health: do number of hours and number of concurrent arrangements matter?

Objectives

Previous research found that centre-based childcare is related to more illnesses early in life. The goal of this longitudinal study is to determine whether infant health in the first year of life is also related to the amount of time spent in non-parental care and the number of concurrent non-parental care arrangements.

Methods

Information on infant health and non-parental care was obtained through monthly maternal interviews across the first year of life. The occurences of respiratory, digestive, general, and skin illnesses and complaints were used as dependent variables, while the number of hours and the number of arrangements per week were used as predictors. Analyses were done separately in infants for whom centre-based childcare is included in their arrangements (n = 107), and for those which it is not (n = 61).

Results

Infants spending more hours in non-parental care had more respiratory and general illnesses. Infants who were cared for in more concurrent arrangements had fewer respiratory and general, but more skin illnesses. These results only applied to infants that included centre-based childcare in their arrangements. In the group of infants that did not attend centre-based childcare, health was not related to either the number of hours or the number of arrangements.

Conclusions

Number of hours and number of arrangements do matter in relation to infant health early in life, but only for infants who attend centre-based childcare. While more hours were related to more illnesses, more arrangements were related to both fewer and more illnesses, depending on the type of illnesses.     

Classification and diagnostic criteria of variants of Hirschsprung’s disease

“Variants of Hirschsprung’s disease” are conditions that clinically resemble Hirschsprung’s disease (HD), despite the presence of ganglion cells in rectal suction biopsies. The diagnosis and management of these patients can be challenging. Specific histological, immunohistochemical and electron microscopic investigations are required to characterize this heterogeneous group of functional bowel disorders. Variants of HD include intestinal neuronal dysplasia, intestinal ganglioneuromatosis, isolated hypoganglionosis, immature ganglia, absence of the argyrophil plexus, internal anal sphincter achalasia and congenital smooth muscle cell disorders such as megacystis microcolon intestinal hypoperistalsis syndrome. This review article systematically classifies variants of HD based on current diagnostic criteria with an additional focus on pathogenesis, epidemiology, clinical presentation, management and outcome.     

Influence of short- and long-term inhalation of salbutamol on lung function and β2-andrenoceptors of mononuclear blood cells in asthmatic children

Clinical observations have shown that some asthma patients develop tachyphylaxis to -sympathomimetic drugs. As down-regulation of the number of -adrenoceptors in different human tissues after exposure to catecholamines and -adrenergic drugs is well known, we investigated whether a interrelation exists between -adrenoceptor down-regulation and clinically detectable -adrenergic subsensitivity during -sympathomimetic treatment. The following results were obtained: 1. ₂-Sympathomimetic inhalation treatment with salbutamol in therapeutic doses led to a significant downregulation of ₂-adrenoceptors and consecutive cyclic adenosine monophosphate response to isoprenaline. This effect was already detectable after short-term treatment of 3–7 days in 9 asthmatic children. 2. In the long-term study over 6 months, salbutamol inhalation in 12 asthmatic children led to a significant down-regulation of -adrenoceptor binding sites on mononuclear blood cells (MNC) from 1539±91 to 1115±99 after 14 days, remaining in this range thereafter. 3. The mean airway resistance (R_aw) of these 12 patients decreased significantly within 14 days from 8.1±0.8 to 5.7±0.5 cm H₂O/l/s to remain stable throughout the 6 months of salbutamol treatment. The differences in R_aw before and immediately after inhalation of 0.2 mg salbutamol (2 puffs) were unchanged during the study period. It is concluded, that long-term inhalative treatment with salbutamol over a period of 6 months does not result in refractoriness to -adrenergic drugs in the airways of asthmatic children, even though a significant down-regulation of ₂-receptors on peripheral MNC occurs.     

Association of colonic atresia and Hirschsprung’s disease in the newborn: report of a new case and review of the literature

Colonic atresia (CA) is an infrequent cause of lower gastrointestinal obstruction in the neonate. Coexistence with aganglionosis of the colon (Hirschsprung’s disease) has been reported but is generally not recognized in the neonatal period. We report another case and present a review of the literature. A boy with a lower gastrointestinal obstruction, caused by a CA type III, had creation of a proximal colostomy and a distal mucous fistula on the 1st day of life. In the preoperative work-up before restoring the continuity, rectal suction biopsies revealed the presence of Hirschsprung’s disease. When the boy was 6 months old, a distal colectomy and reanastomosis were done. Creation of a colostomy and reanastomosis in a second procedure is recommended for treating a type III CA unless distal aganglionosis has been ruled out.     

Short- and Mid-Term Outcomes of Total Correction of Taussig–Bing Anomaly

Double-outlet right ventricle (DORV)/Taussig–Bing (TB) anomaly is the second most common type of DORV. This study evaluates our experience and outcomes of total correction of DORV-TB anomaly at King Abdulaziz Cardiac Center. We conducted a retrospective study for all cases of TB anomaly repaired between June 2001 and April 2009. Patients were divided into two groups: Group A included patients repaired with arterial switch operation, and group (B) included patients repaired with Rastelli procedure. Thirteen patients with TB anomaly underwent total correction. There were 5 male (38%) and 8 female (62%) patients. Mean age and weight at surgery were 6.8 ± 6 weeks and 3.6 ± 0.7 kg, respectively. Of the 13 patients, 9 (69%) were in group A, and 4 (31%) were in group B. Aortic arch abnormalities were present in 9 patients (69%); abnormal coronary artery patterns were present in 7 patients (54%); side-by-side great arteries were present in 5 patients (38%); dextrotransposition of the great arteries was present in 7 patients (54%); and levo-malposition of the great arteries was present in 1 patient (8%). At postoperative follow-up, 4 patients (31%) had developed either left- or right-ventricular outflow tract (VOT) obstruction requiring surgical and/or catheter intervention. There was no early mortality, but there was 1 late mortality caused by left-ventricle dysfunction. DORV-TB is often associated with other congenital cardiac anomalies. In general, total repair is feasible in the majority of patients with satisfactory results and improved outcome. Residual lesion and development of VOT obstruction can occur, requiring close follow-up and intervention for residual lesion.     

Mucopolysaccharidosis Ⅶ:report of a case and renew of the literature

objective To investigate the clinical characteristics and diagnosis of mucopolysaccharidosis Ⅶ. Method The clinical and biochemical features of an infant with mucopolysaccharidosis Ⅶ confirmed by enzyme assay were analyzed.Result The 2 month-old male infant showed hydrops fetalis,mental retardation,coarse face,corneal clouding,hepatosplenomegaly,hernias,Alder-Reilly granules in the leucocytes and decreased platelet(32×109/L).The biochemical markers showed urinary glycosaminoglycans(GAG)(532.8 mg/L,controls＜70.0 mg/L).The ratio of GAG/creatinine was 161.3(controls:26.2±11.7).Serum chitotriosidase activity was 315.8 nmol/(ml·h) [control ＜53 mol/(ml·h)].Beta-glucuronidase activity was deficient in isolated leukocytes.Conclusion Severe form of mucopolysaecharidosis Ⅶ exhibited characteristics of hydrops fetalis.hepatosplenomegaly, coarse face,thromboeytopenia and Alder-Reilly granules in the leucocytes.The measurements of GAG in urinary and beta glucuronidase in leucocytes are critical to diagnosis and deferential diagnosis.     

Research status of intestinal flora and pathogenesis of epilepsy北大核心CSCD

癫痫是小儿神经系统的常见疾病之一,是神经元过度同步化放电造成的慢性脑功能障碍。反复的癫痫发作,往往导致儿童生理、智力等多方面损害,严重影响儿童的生长发育和生命健康。目前研究表明,癫痫患儿的肠道菌群与健康儿童的肠道菌群存在显著差异。肠道菌群通过免疫系统、神经内分泌-下丘脑-垂体-肾上腺轴、神经递质以及迷走神经等多种途径影响癫痫的发生发展。     

Overview of juvenile localized scleroderma and its management

Background Juvenile localized scleroderma(JLS)is a rare pediatric disease characterized by inflammation and skin thick ening.JLS is associated with deep tissue and extracutaneous involvement that often results in functional impairment and growth disturbances.This article provides an overview of the disease with a focus on active features and treatment.Data sources We searched databases including PubMed,Elsevier and MedLine and Wanfang,reviewing publications from 2013 to 2019.Selected earlier publications were also reviewed.Results Linear scleroderma is the most common JLS subtype.Several lines of evidence suggest that JLS is an autoimmune disease.Extracutaneous involvement is common and can present before the onset of skin disease.Multiple skin features are associated with disease activity,and activity can also manifest as arthritis,myositis,uveitis,seizures,and growth impairment.Systemic immunosuppressive treatment,commonly methotrexate with or without glucocorticoids,greatly improves outcome and is recommended for treating JLS patients with active disease and moderate or higher severity.Long term monitoring is needed because of the disease's chronicity and the high frequency of relapses off of treatment.Conclusions JLS is associated with a risk for disabling and disfiguring morbidity for the growing child.Identifying active disease is important for guiding treatment,but often difficult because of the paucity of markers and lack of a universal skin activity feature.More studies of JLS pathophysiology are needed to allow the identification of biomarkers and therapeutic targets.Comparative effectiveness treatment studies are also needed to work towards optimizing care and outcome.     

Biochemical monitoring of pregnancy and breast feeding in five patients with classical galactosaemia – and review of the literature

Pregnancy, delivery, and postpartal metabolic control was monitored biochemically in five patients (22–38 years of age) with clinically, enzymatically, and genotypically established classical galactosaemia and good dietary compliance. Three of the patients performed breast feeding of their newborns. Monitoring parameters were galactose-1-phosphate and galactitol concentrations in erythrocytes and urinary excretion of galactose, galactitol, galactonate, and lactose. During pregnancy, a small but steady increase of renal metabolite excretion rates was observed. After delivery, a moderate transient increase of metabolite concentrations with peak values within the first week post partum occurred, irrespective of breast feeding. Altogether, there was no evidence for clinically or subclinically significant changes of metabolic control during pregnancy, delivery, or lactation. In conclusion, a specific metabolic monitoring is apparently not required in pregnant galactosemic women, and breast feeding of the nongalactosemic offspring can be recommended.