Imaging findings in hepatic Langerhans’ cell histiocytosis

We describe ultrasonographic and computed tomographic features of hepatic, lesions in two cases of disseminated Langerhans cell histiocytosis affecting children. In the first case, hyperechoic band like periportal lesions were observed at ultrasonography, which on computed tomography was found to be hypodense admixed with fatty attenuation (HU≡23 to-57) at places. In addition, the caudate lobe was very prominent. In the second case, the hepatic parenchyma showed predominantly hyperechoic diffusely heterogeneous echogenicity. There were features of cirrhosis of liver with portal hypertension in the form of atrophy of right lobe with hypertrophy of left lobe of liver with lobulated outline, prominent main portal vein and splenoportal axis, splenomegaly and gastroesophageal varices. Both the patients were put on chemotherapy as per schedule (Protocol: DAL HX—83) and are on follow up.     

Chest CT findings in pediatric Wegener’s granulomatosis

Background Although pulmonary involvement occurs in the majority of children and adolescents with Wegener’s granulomatosis (WG), relatively little has been published regarding the CT imaging manifestations in this group of patients. Objective To determine the frequency and types of chest CT abnormalities in active pediatric WG (pWG). Materials and methods The study was a retrospective examination of 29 chest CT examinations performed at diagnosis (n=14) and during disease flares (n=15) in 18 children. Results The most common abnormalities were nodules (seen in 90% of examinations), ground-glass opacification (52%), and air-space opacification (45%). Of examinations with nodules, 73% demonstrated nodules >5 mm in diameter and 69% demonstrated more than five nodules; 17% had cavitary lesions. The only abnormality with a significant difference in prevalence between diagnosis and disease flares was air-space opacification, present in 71% and 20%, respectively (P < 0.01). Conclusions In accordance with the findings of published adult studies and at variance with those of prior pediatric studies, our findings indicate that chest CT abnormalities in active pWG are frequent, most commonly comprising nodules and ground-glass opacification, which may be difficult to detect on plain radiography. We therefore advocate the routine use of chest CT for all affected patients, both at the time of presentation and during disease flares.     

A yellowish subconjunctival lesion in an infant—congenital orbital fat herniation

A very rare case of a congenital orbital fat herniation is demonstrated. Clinically, the child presented a progressive, small yellowish and mobile mass on the temporal orbital wall of her right eye. Magnetic resonance imaging showed a fat isointensive structure in T1-weighing without contrast enhancement. The possible aetiology of the herniation could be a congenital weakness of the Tenon's capsule or the conjunctival fornix.     

Management of Hirschsprung’s disease: a comparison of Soave’s and Duhamel’s pull-through methods

The aim of this study was to compare the safety and benefits of Soaves and Duhamels pull-through procedures for the management of Hirschsprungs disease (HD). The patients consisted of 33 boys (85%) and six girls (15%), a ratio of 5.5:1. Their ages ranged from 1 day to 8 years. Rectal biopsy was performed to confirm the diagnosis of HD. Twenty-five patients (64%) underwent Soaves pull-through, and 13 patients (33%) underwent Duhamels pull-through. Twenty children (80%) out of the 25 undergoing Soaves pull-through recovered uneventfully, compared with 11 out of the 13 (84%) undergoing Duhamels pull-through. The complications following Soaves procedure included strictures in two patients (8%), enterocolitis in another two (8%), and anastomotic leakage in one (4%). Additional operations were required in two patients (8%). The complications following Duhamels procedure included stricture in one patient (7.6%) and enterocolitis in another (7.6%). An additional operation was required in one patient (7.6%). The rate of constipation was 16% after the Soaves pull-through compared with 15% after the Duhamels pull-through. There was no significant difference between the two procedures in postoperative surgical morbidity or in long-term risk of enterocolitis. In the light of present findings, both procedures appear comparable in terms of efficiency and associated complications.     

Complete innervation profile of whole bowel resected at pull-through for Hirschsprung’s disease. Unexpected findings

We used Acetylcholinesterase (AchE) staining and neural cell adhesion molecule (NCAM) immunoreactivity to examine such resected lengths to determine the complete innervation profile of resected bowel in Hirschsprung’s disease (HD). Resected specimens of colon obtained at pull-through surgery from 15 patients with HD [short type (S-type: n=5), recto-sigmoid type (RS-type: n=5), long type (L-type: n=5)] were sectioned at 1.5 cm intervals and stained conventionally with AchE histochemistry and NCAM immunohistochemistry. The number of positive nerve fibers (PNFs) in the lamina propria and smooth muscle layers was assessed on a scale of 0 to 3 where 0 meant no PNF and 3 meant many PNFs. The three types of HD had different AchE and NCAM innervation profiles, especially the L-type. There were also different AchE and NCAM innervation patterns seen within the same aganglionic or transitional segments of bowel depending on the site of sampling. The mean proportion of transitional segment in resected specimens from RS-type HD was significantly larger than that of S-type HD (P<0.001) although the proportion of aganglionic segments from S- and RS-type HD were almost the same. Our results suggest that the etiology of L-type HD may be different from the etiology of S- and RS-type HD because of different innervation profiles. Because segments of the excised bowel would appear to have different innervation patterns depending on whether the specimen is sampled proximally or distally, it is important to record the exact site of sampling to allow valid comparisons between types of HD to be made.     

Children’s Perceptions of Poverty

This article explores children’s perceptions of poverty and its causes; we want to know how children perceive poverty and what they think causes it. The study applied a qualitative approach, and the research data consisted of 30 semi-structured interviews with children aged 10–15. Respondents were not specifically recruited by any socioeconomic criteria and according to FAS children represent middle affluence group. The data was collected in the school located in one of the largest urban regions in Finland, in city in the outer urban area. The data were processed using content analysis and the major themes emerging from the data as a whole are the focus of this article. Children saw poverty as a relative absence of non-essential goods and items due to the scarcity of financial resources. It was not a question of poorer children lacking daily necessities, such as a home, clothes, equipment for leisure activities, or a mobile phone, but rather of poorer children having models that were outdated, or second-hand or broken goods. Poverty was seen as a phenomenon that almost by necessity shapes and influences people’s ways of thinking and actions. Poverty was also associated with humility, and poor children were considered to carry a social stigma. We condensed the children’s perceptions regarding the causes of poverty into six themes: individual blame, individual action, societal blame, societal situation, individual fate, and social fate. Some children emphasised the role of individual interpretations and independent choices; for others, poverty was ultimately explained by structural factors. Overall, however, the main accent was on structural explanations. On the other hand, these two sets of explanations also coexisted concurrently in children’s experiences, so that poverty was seen at once as both an individual and a societal phenomenon.     

Treatment of perianal Crohn’s disease

Perianal Crohn’s is a common manifestation of Crohn’s disease. Primary manifestations of perianal disease mirror common anorectal conditions, however treatment is less successful than in those patients without Crohn’s related perianal disease. A multimodal approach to therapy including medical and surgical modalities is often necessary. The goal of treatment is to manage symptoms while maintaining continence.     

Adenocarcinoma of the infant’s testis

Summary A case of adenocarcinoma of the testis in an infant aged 13 months has been described. From the Department of Pathology and Bacteriology, G.R. Medical College, Gwalior. Madhya Pradesh.     

Adenocarcinoma of the infant’s testis

Summary A case of adenocarcinoma of the infant’s testis has been reported. The signs, symptoms, treatment and histogenesis of the tumour have been discussed. The literature on the subject has been reviewed. From the Department of Surgery, King George’s Medical College, Lucknow.     

Children’s Discursive Constructions of the ‘Self’

The ways in which children construct and assign meaning to the ‘self’ could have an impact on their social and emotional well-being, including their coping skills, relationship formation, and behaviour. Furthermore, a child’s understanding of the ‘self’ could influence the way in which they make meaning out of their experiences and internalize these experiences as a means of understanding one’s abilities and self-worth. Conditions of poverty and oppression could negatively impact the development of the self-concept and a child’s overall well-being. Such conditions exist in South Africa, where the aftermath of apartheid’s system of structural racism continues in the form of social inequity, poverty, and violence. This study utilized a child participation framework to explore children’s discursive constructions of and meanings assigned to the ‘self’ within two urban communities of the Western Cape, South Africa. Eight focus group discussions were conducted amongst fifty-four children between the ages of nine to twelve. Thematic and discourse analysis were used to analyse the findings. The themes of childhood, social connectedness, and children’s spaces were identified as key influences on a child’s self-concept. Four underlying discourses emerged within the themes as central to the participant’s self-constructions. These included; (1) ‘forfeited childhood,’ (2) ‘vulnerability and helplessness,’ (3) ‘preserving the integrity of the self,’ and (4) ‘opportunities for escape.’     

Children’s Discourses of Natural Spaces: Considerations for children’s Subjective Well-Being

Based on the evidence provided in the literature, it is manifest that increased time spent in nature increases various aspects of children’s well-being. Using discourse analysis on focus group interviews with 28 children between the ages of 12 and 14 years old from three socio-economically diverse communities in the Western Cape of South Africa, the study aimed to explore how children construct and assign meaning to natural spaces. More specifically the study explored how children use specific discursive resources and repertoires to construct and assign meaning to their engagement with natural spaces, and the extent to which this influences their subjective well-being. Several pertinent discourses emerged from the participants’ accounts within four themes of: Safety and natural spaces, Appreciation for natural spaces, Degradation of Nature: Thinking environmentally, acting pro-environmentally, and Natural spaces and children’s subjective well-being. The study highlights the critical role that children’s engagement in natural spaces has on their subjective well-being, and how these benefits can be harnessed to better children’s overall quality of life.     

Impact of cow’s milk allergy on enterocolitis associated with Hirschsprung’s disease

Purpose

To investigate the impact of cow’s milk allergy (CMA) on infants with Hirschsprung’s disease (HD).

Methods

Twenty-four patients, who developed gastrointestinal symptoms before the age of 60 days and underwent surgery for HD in the period between January 2003 and December 2012, were enrolled in this study. They were divided into two groups based on CMA-related findings: stimulation index of lymphocyte stimulation test >300 % and the presence of eosinophilic infiltration in the resected colon. Ten patients were determined specimen as not having CMA (Group A), because they did not satisfy any of the criteria. The remaining 14 were determined as having possible CMA (Group B), because they satisfied either or both findings. Patient background characteristics, pre- and postoperative clinical history, and laboratory data were compared between Groups A and B.

Results

Pre- and postoperative enterocolitis did not occur in Group A patients. Postoperative enterocolitis was more frequent in Group B than in Group A (p = 0.04). Other clinical and laboratory data did not show significant difference between the two groups.

Conclusion

CMA is a possible risk factor for postoperative enterocolitis in patients with HD.     

Crohn’s disease in a Meckel’s diverticulum: report of a case

Meckel’s diverticulum has previously been reported to be present in patients with Crohn’s disease. However, the finding is typically an incidental one, and involvement of the diverticulum in the disease process is uncommon. We report a case of an adolescent with known Crohn’s disease who presented with symptoms thought to be due to terminal ileitis. At the time of laparoscopy the inflammation was found to be due to involvement of the Meckel’s diverticulum with Crohn’s disease.     

A Systematic Review of Children’s Construction of the Self: Implications for Children’s Subjective Well-being

This research aimed to systematically review academic literature focused on how children construct and assign meaning to the ‘self.’ An article search and appraisal yielded 38 articles that met the inclusion criteria. Within these articles, six central thematic categories emerged as the key influences on children’s constructions of the self. These include multidimensionality, discursive practices, socio-environmental conditions, oppression & marginalisation, culture, and social support. The study highlights the multidimensional and fluid nature of the self and emphasises the self-concept to be mutually influenced and supported by an individual’s immediate social networks, socio-environmental resources, and internal processes. A child’s ability to meaningfully construct a sense of self was associated with higher levels of coping and resilience and improved well-being. The review provides considerations for increased interventions aimed at improving child well-being which must take into account the unique ways in which children construct and assign meaning to the self. Such interventions should be inclusive of the individual and contextual issues influencing a child’s self-constructions, including coping skills, structural challenges and proximal economic and social resources.     

Diagnosis and management of fulminant Wilson’s disease: a single center’s experience

Background

Medical therapy is rarely effective in patients with fulminant Wilson’s disease (FWD). Liver transplantation is limited by the lack of donor liver in most patients with FWD at the time of diagnosis. New Wilson’s index, model for end-stage liver disease (MELD) and Child-Pugh score are useful tools for decision-making of liver transplantation; however, none of them is an independent decisive tool. It is worthwhile to explore a more effective and practical therapeutic strategy and reevaluate the prediction systems for patients with FWD.

Methods

Nine patients with FWD associated with hemolytic crisis and fulminant hepatic failure (FHF) were investigated. The clinical presentation, prognostic score and medical therapies of the patients were analyzed.

Results

In 7 of the 9 patients with FWD who received the comprehensive therapy of corticosteroid, copper-chelating agent (dimercaptopropansulfonate sodium) and therapeutic plasma exchange (TPE), 6 patients recovered from FHF. The remaining one had been improved through the comprehensive therapy but died of septicemia 51 days later. Two patients with spontaneous bacterial peritonitis (SBP) died from liver failure in three or five hospital days without plasma exchange or chelating therapy. All of the 9 patients with FWD presented with acute hepatic failure, severe jaundice and mild to severe hemolytic anemia. No marked difference in the incidence of severe hemolytic anemia was detected between the survival and deceased groups. However, the incidence and the degree of hepatic encephalopathy (HE) in the non-survival group were higher than those in the survival group. Unlike the deceased group, the survival group had no complications induced by bacterial infection. Compared to new Wilson’s index, Child-Pugh score and MELD score, the variation of prothrombin activity (PTA) between the survival and deceased groups was more evident.

Conclusion

For patients with FWD, the episode of severe hepatic encephalopathy or/and spontaneous bacterial peritonitis indicates worse prognosis, and PTA is a recommendable predictor. An emergent liver transplantation should be considered for patients whose PTA is below 20%, or for those with severe HE or/and SBP. The comprehensive therapy of corticosteroid, copper-chelating agent and TPE is effective for patients without SBP and whose PTA is higher than 20%.