Intussusception in a tropical country: comparison among patient populations in Jakarta, Jogyakarta, and Amsterdam.

BACKGROUND: Intussusception is the most common cause of intestinal obstruction in young children, and high mortality rates remain a problem in developing countries. The purpose of this study was to describe and elucidate the differences in outcome between groups of children with intussusception in Indonesia, a developing country, and The Netherlands, a developed country. METHODS: In this retrospective review, 176 patients were studied in three types of hospitals. A comparison was made among children treated at a primary care rural hospital in Indonesia, at a secondary care urban hospital in Indonesia, and at a tertiary care urban hospital in The Netherlands. RESULTS: Children in the rural community hospital in Indonesia were more severely ill at arrival and had a significantly longer duration of symptoms, an increased incidence of nonviable bowel, and a mortality rate of 20%, in contrast to a mortality rate of 3% in the urban hospital in Indonesia and no deaths in the Dutch hospital. CONCLUSIONS: The mortality of children with intussusception in rural Indonesia is much higher than in urban Indonesia or in The Netherlands, probably because of delayed treatment, which results in more patients undergoing surgery in worse physical condition.     

Decreased homovanillic acid concentrations in cerebrospinal fluid in children without a known defect in dopamine metabolism.

Homovanillic acid (HVA) is a metabolite of dopamine, reflecting central dopamine metabolism, primarily situated in the striatum. Low HVA concentrations in the cerebrospinal fluid (CSF) may indicate metabolic deficiencies in the pathways of the biosynthesis or catabolism of dopamine. In this retrospective study, we investigated the clinical presentation of patients whose HVA concentration in the CSF had been determined routinely after spinal taps for a variety of clinical reasons. A decrease of HVA concentration in the CSF, due to a defect in the biosynthesis or reuptake of dopamine, is expected to cause extrapyramidal features. However, we found a remarkable variability in the clinical symptoms. Similarly, a decreased HVA concentration in the CSF failed to coincide with specific abnormalities at neuroimaging. In view of the diversity of the clinical presentation and in the absence of specific enzyme deficiencies, a decrease of HVA may be due to dysfunction of dopamine neurons, not resulting in specific extrapyramidal symptoms. Thus, with the exception of diseases associated with a specific enzyme deficiency in the metabolic pathways involving dopamine, a decrease of HVA concentration in the CSF is mainly a secondary or epiphenomenon in a variety of clinical conditions.     

Temporal disparity between reduction of cot death and reduction of prone sleeping prevalence

According to several reports sudden infant death rates have decreased significantly after public campaigns aimed at reducing the incidence of sleeping in a prone position. The Styrian population (1.2 million inhabitants), who have been studied from 1984, also showed a significant drop in the incidence of cot death during 1989 (from 2%o to 1%o). The year before, a campaign for the prevention of cot death had been launched. This included the recommendation to prevent infants from lying in a prone position during sleep. Part of the prevention programme consisted of a detailed questionnaire filled in and returned by the parents. These data, on 29 970 infants from 1989 to 1994, provided information on the frequency of prone sleeping in 37% of our total population and as a consequence on parental response to the campaign. Calculating the data per year led to the surprising result that the reduction by half (from 50% to 25%) in the prevalence of sleeping in a prone position did not occur in 1989, when the drop in the incidence of cot death occurred, but 3 years later, in 1992. The following years saw a further decrease of prone position to 7% but no appreciable change in the incidence of cot death. However, during those 11 years of study about 80% of the victims were consistently found dead lying in a prone position. Our results show a temporal disparity between the reduction of sudden infant death and the decrease of prone sleeping in a population. Although we do not deny sleeping in a prone position as a risk factor for cot death, there cannot be a simple relationship between sleeping habits in the population and incidence of cot death.     

Unusual thoracic CT manifestations of osteosarcoma: review of 16 cases

Pulmonary metastases are common in osteosarcoma and the most common appearance is of multiple well-defined nodules in the lung parenchyma. However, a variety of atypical locations and presentations of osteosarcoma metastasis can occur in the thorax. We present a review of the thoracic CT findings in 16 patients with histopathologically confirmed osteosarcoma with unusual thoracic manifestations. The 16 patients were selected out of a total 136 patients who received a chest CT scan for osteosarcoma during a period of 3 years in a tertiary care hospital. Unusual imaging findings included a solitary large ossified lung mass, ossified mediastinal and hilar lymph nodes, an esophagomediastinal fistula, lymphangitic carcinomatosis, pulmonary artery tumor emboli, a solitary large pleural deposit along the major fissure, multiple pleural deposits, diffuse pleural calcification, pneumothorax, diaphragmatic deposits, an isolated chest wall deposit without lung involvement, and primary osteosarcoma of the rib. Our findings of lymphangitic carcinomatosis in a living patient as well as calcified mediastinal lymphadenopathy leading to esophageal fistula are unique in the literature, and there are only a few case reports of our other findings.     

Experimental design deck for clinical research: A new learning resource in aid of scientific thinking.

The Experimental Design Deck for Clinical Research is a simulation of a problem in experimental design in a card deck format. It is intended to stimulate the development of skills in scientific thinking by allowing the student, in a self-directed fashion, to design a clinical trial to test either a given hypothesis (specific deck) or a hypothesis that the player provides (general deck). In addition to its use as an educational tool, the Experimental Design Deck can be used as a guide to the application of scientific thinking in a variety of biomedical activities. It can easily be modified for application to problems in nonmedical research and evaluation.     

Thrombotic and hemorrhagic complications in children with the lupus anticoagulant

Endogenous circulating anticoagulants are unusual in children without a congenital factor deficiency. In particular, the lupus anticoagulant has only rarely been reported in children. Despite its functioning in vitro to prolong the partial thromboplastin time, patients more frequently have problems with thrombosis than bleeding, unless there is a coexistent prothrombin deficiency or thrombocytopenia. We report the cases of three children with the lupus anticoagulant. Two children had associated thromboses. One had a thrombosis of the iliofemoral system and the other had a partial Budd-Chiari syndrome, a thrombosis of the deep calf veins and ureteric obstruction. The third child had a concomitant prothrombin deficiency and bleeding after tooth extraction. Associated findings in these patients included a positive antinuclear antibody test in two, a positive anti-DNA antibody test in two, a false-positive VDRL test in two, and an antiphospholipid antibody test in two.     

Sacrococcygeal heart: a very rare differentiation in teratoma.

A mature teratoma was identified in a two-month-old girl who was operated for a sacrococcygeal mass. The cystic components of the mass were accidentally opened during surgery, and a solid, rudimentary organ resembling a heart emerged. It had a vascular pedicle and a pulsation like cardiac activity different from the infant's heart rate. The mass was totally excised together with the coccyx, and in histological examinations, it was diagnosed as a mature teratoma and a rudimentary heart. To the best of our knowledge, the case presented in this report is only the second case of a cardiac development in a teratoma in the literature. In the light of data obtained about this case and related literature, we consider that fetus-in-fetu and teratoma may not be irrelevant entities, and that they possibly have the same developmental malformation. We also suggest that such an intermediate case is a combination of fetus-in-fetu and teratoma.     

Unsupervised children in vehicles: a risk for pediatric trauma

In this study, a series of instances of children injured by a motor vehicle set in motion by an unsupervised child are reviewed. During a 24-month period, nine such children were identified through a multihospital and coroner's office monitoring system in a single urban county. Injuries ranged from multiple abrasions and contusions to serious leg and head injuries. Three children died. The typical circumstance involved a child releasing the brake or placing the vehicle in gear in a private driveway which resulted in the vehicle striking or rolling over the victim. In four of the nine cases, the child who set the vehicle in motion fell or jumped from the vehicle and then became the injured victim. The extent of these unusual motor vehicle-related injuries is unknown because they are unlikely to be reported in official police statistics. According to the study findings, there is a need to educate the public and health professionals about the risks associated with leaving a child unattended in a motor vehicle and the hazardous environment of the private driveway. Preventive measures would include not leaving a child unattended in a vehicle, locking unattended vehicles to prevent access, and redesigning of private driveways.     

Memory encoded throughout our bodies: molecular and cellular basis of tissue regeneration

When a sheep loses its tail, it cannot regenerate it in the manner of lizards. On the other hand, it is possible to clone mammals from somatic cells, showing that a complete developmental program is intact in a wounded sheep's tail the same way it is in a lizard. Thus, there is a requirement for more than only the presence of the entire genetic code in somatic cells for regenerative abilities. Thoughts like this have motivated us to assemble more than just a factographic synopsis on tissue regeneration. As a model, we review skin wound healing in chronological order, and when possible, we use that overview as a framework to point out possible mechanisms of how damaged tissues can restore their original structure. This article postulates the existence of tissue structural memory as a complex distributed homeostatic mechanism. We support such an idea by referring to an extremely fragmented literature base, trying to synthesize a broad picture of important principles of how tissues and organs may store information about their own structure for the purposes of regeneration. Selected developmental, surgical, and tissue engineering aspects are presented and discussed in the light of recent findings in the field.When a sheep loses its tail, it cannot regenerate it in the manner of lizards. On the other hand, it is possible to clone mammals from somatic cells, showing that a complete developmental program is intact in a wounded sheep's tail the same way it is in a lizard. Thus, there is a requirement for more than only the presence of the entire genetic code in somatic cells for regenerative abilities. Thoughts like this have motivated us to assemble more than just a factographic synopsis on tissue regeneration. As a model, we review skin wound healing in chronological order, and when possible, we use that overview as a framework to point out possible mechanisms of how damaged tissues can restore their original structure. This article postulates the existence of tissue structural memory as a complex distributed homeostatic mechanism. We support such an idea by referring to an extremely fragmented literature base, trying to synthesize a broad picture of important principles of how tissues and organs may store information about their own structure for the purposes of regeneration. Selected developmental, surgical, and tissue engineering aspects are presented and discussed in the light of recent findings in the field.     

EpiPen epidemic: suggestions for rational prescribing in childhood food allergy

There has been a marked increase in community concerns of the risk of food induced anaphylaxis in children and a consequent increase in the provision of the self or carer injectable epinephrine (EpiPen) (CSL Ltd, Parkville, Victoria, Australia)). The Australian use of EpiPens in children under 10 years has increased by 300% over 5 years with a crude rate of EpiPen provision of 1 per 544 Australian children aged under 10 years. However, the risk of a fatal reaction to food, particularly in preschool children, is remote (in Australia, an estimated one fatality in 30 years in the under 5-year-old population and two deaths in 10 years in the entire child population). It is therefore important to provide a perspective on the risk of death from food induced anaphylactic to parents and carers in view of the anxiety generated on this issue. The indications for provision of an EpiPen to children are not well defined. Six risk factors, which can be considered in evaluating the risk of a life-threatening reaction (age over 5 years; a history of respiratory tract involvement with the initial or subsequent reactions; a history of asthma requiring preventer medication; peanut or tree nut sensitivity; reactions induced by traces or small amounts of allergen; a strongly positive skin prick test) are proposed. It is suggested that the greater the number that are positive, the lower the threshold for provision of an EpiPen. In addition, instruction in EpiPen administration and the provision of both a clear and simple anaphylaxis action plan and a rational perspective on the remote risk of death is just as important as the provision of the device itself.     

New pathogenetic aspects of gut dysmotility in aplastic and hypoplastic desmosis of early childhood

The peristaltic movement of the gut is a function of the alternating contraction and relaxation of circular and longitudinal muscles. This movement is induced by a tendon-like connective-tissue net (TCTN) in the circular and longitudinal muscles, which are both rooted in a connective-tissue plexus layer (CTPL). In children with a therapy-resistant aperistaltic or hypoperistaltic syndrome who had normally-developed enteric innervation, a lack of the TCTN in the muscularis propria was observed. Over the last 2 years, 241 children with chronic constipation were investigated histopathologically; 46 children surgically treated by partial resection of the gut or diagnostically investigated by whole-mount biopsy. Fifteen children had a hypoperistalsis syndrome and 3 had an aperistalsis syndrome. All specimens were native and cut in a cryostat. Enteric innervation was examined by acetylcholinesterase and dehydrogenase reactions. The TCTN in the muscularis propria was stained with picric acid/sirius red. In the children with an aperistalsis syndrome, a complete lack of the TCTN in circular and longitudinal muscles was observed. A CTPL was not developed. The children with a hypoperistalsis syndrome had no CTPL, but had a partly-developed TCTN in the circular and longitudinal muscles, which gradually faded in the direction of the plexus layer. Independent of a well-developed enteric nervous system, a lack of the TCTN in longitudinal and circular muscles and a missing CTPL (aplastic desmosis) abolishes the coordinated peristaltic movement of the gut. An isolated lack of the CTPL in the myenteric plexus (hypoplastic desmosis) results in a hypoperistalsis syndrome. An anomaly of the TCTN in the muscularis propria disturbs gut-muscle mechanics, and is another cause of gut dysmotility. Accepted: 14 April 2000     

533例肺炎儿童鼻咽抽吸物和支气管肺泡灌洗液的病原检出一致性分析

目的 探究肺炎儿童鼻咽抽吸物（nasopharyngeal aspiration，NPA）和支气管肺泡灌洗液（bronchoalveolar lavage fluid，BALF）的病原检出一致性。 方法 回顾性分析2017年2月至2020年3月间533例肺炎患儿的NPA与BALF检出病原结果，用配对卡方McNemar检验比较NPA与BALF之间病原检出差异，用Kappa检验对两种样本检出的病原进行一致性分析。 结果 NPA检出细菌的灵敏度为28%，特异度为74%，阳性预测值为14%，阴性预测值为91%，Kappa值为0.013，与BALF检测结果一致性较差；NPA检出病毒的灵敏度为52%，特异度为81%，阳性预测值为24%，阴性预测值为94%，Kappa值为0.213，与BALF检测结果一致性较差；NPA检测肺炎支原体的灵敏度为78%，特异度为71%，阳性预测值为49%，阴性预测值为90%，Kappa值为0.407，与BALF检测结果一致性中等。 结论 NPA与BALF检出细菌与病毒的一致性较差，临床医生根据NPA检出细菌或病毒来诊断下呼吸道感染时需谨慎；NPA与BALF检出肺炎支原体的一致性中等，临床医生根据NPA检出肺炎支原体来诊断下呼吸道感染可靠性较高，但仍需结合临床综合判断。 引用格式:     

Clinical predictors of acute bacterial diarrhea in young children

This prospective study assessed the value of presenting history, physical examination, and screening laboratory tests in predicting whether diarrhea in a young child is associated with a stool culture positive for a bacterial pathogen. Acutely ill children less than 4 years old were studied in a hospital outpatient setting. Two hundred patients were seen in a 9 1/2-month period, which encompassed the seasons of summer, fall, and winter. One hundred ninety-five patients had cultures completed and twenty-nine (15%) had a bacterial pathogen isolated. The best predictive variable for a stool culture positive for a bacterial pathogen was the presence of polymorphonuclear cells in the stool, with a sensitivity of 85%, a specificity of 88%, and positive and negative predictive values of 59% and 97%, respectively. A cluster of three historical variables--abrupt onset of diarrhea, greater than four stools per day, and no vomiting before the onset of diarrhea--was identified that delineated a subpopulation of patients with an increased probability of having a stool culture positive for a bacterial pathogen (27% v 4% if any of the three variables was absent). It is suggested that these findings can be combined in a stepwise manner using the historical cluster as an initial screening, followed by examination for stool polymorphonuclear cells in the high probability subgroup, to identify those patients with a very high probability of having a bacterial pathogen isolated in their stool.     

Burkitt's lymphoma and the role of Epstein-Barr virus

Burkitt's lymphoma is the most common childhood cancer in Africa. Most prevalent in areas endemic for malaria, the disease, a malignant growth of lymphoid tissue, usually presents itself as a large tumour of the jaw. When first characterized in the 1950s, the lymphoma was thought to spread by some infectious agent. Subsequent research indicates that the frequent involvement of an infectious agent is but one factor in a more complex aetiology. Today, Burkitt's lymphoma is considered an example of multistep carcinogenesis. Each step in the process results from a different agent. The agent in the first step is the Epstein-Barr virus, which infects B cells of the immune system causing a proliferation of these cells. The second step, malarial infection, furthers the proliferation of B cells providing a large population of cells available for a chromosomal translocation which represents the third step in the formation of the lymphoma. The chromosomal translocation places a cancer causing gene, c-myc, in close proximity to an active antibody-encoding its proliferation resulting in a cell capable of unlimited growth which serves as the nucleus of a B cell lymphoma.     

Fetus in fetu: a case report and literature review

Fetus in fetu is a rare condition in which a fetiform calcified mass often is present in the abdomen of its host, a newborn or an infant. We report on a case of a 19-month-old girl whose plain abdominal radiograph, ultrasonography, and computed tomography scan revealed a mass in which the contents favor a fetus in fetu rather than a teratoma. The noncalcified vertebral column invisible on the radiographs was identified by the pathologist; therefore, the nonvisualization of the vertebral axis on radiography or on computed tomography scan does not exclude the diagnosis of fetus in fetu.     

Troubles en mathématiques : une origine multiple ? L’exemple des syndromes de Turner et de l’X Fragile

Problems in mathematics are a frequent major complaint in neuropediatric departments, for which there are two explanatory theoretical models: the hypothesis of a genetic and modular origin (with a number sense deficit) and a multidetermined origin. The purpose of this paper is to review the mathematical difficulties described in Turner syndrome and Fragile X syndrome, because a specific mathematical disorder is usually reported in these populations, supporting the existence of a number sense. Analysis of the literature reveals highly variable cognitive phenotypes in these populations, especially regarding mathematical abilities. Performance heterogeneity might be related to different factors such as the abilities needed to perform the task, the variability of definitions, the different tests used in the studies and the heterogeneity of the syndromes themselves. A number sense deficit is usually described in these syndromes, but variable cognitive impairments are also observed. The idea of a modular functioning is then debated and we argue for the necessity of a global cognitive evaluation approach.     

Diurnal changes in plasma amino acids in maple syrup urine disease

Protein turnover is a cyclic process with a net loss of protein in the (catabolic) fasted state and a net gain in the (anabolic) fed state. In maple syrup urine disease (MSUD) the early block of degradation of the branched-chain amino acids (BCAA) brings about the opportunity for evaluation of the diurnal variation in net protein anabolism and catabolism by studying cyclic changes in the plasma concentrations of BCAA. The alterations in plasma BCAA in a 3-y-old boy with classical MSUD were studied in the fed and fasted state over a period of 19 months. For each amino acid a total of 34 data pairs was calculated. The plasma concentrations of the BCAA leucine, valine and isoleucine were constantly higher in the fasted than in the fed state. Plasma concentrations of alloisoleucine, being a non-protein amino acid, did not participate in cyclic changes. In contrast, the essential amino acid pair tyrosine and phenylalanine increased after meals. The fasting concentration of alanine increased after feeding, while glycine did not change significantly. Healthy subjects show a decrease in all amino acids in the fasted (mild catabolic) state and an increase in the fed state. These findings in MSUD suggest a net decrease in non-BCAA as result of a greater rate of amino acid oxidation rate than of protein breakdown and a net entry of BCAA into plasma in the fasted state due to the specific metabolic block. Such changes in amino acid plasma pools have to be taken into account during monitoring of treatment and especially when in vivo leucine oxidation is assessed.     

The victimization of children and youth: a comprehensive, national survey

This study examined a large spectrum of violence, crime, and victimization experiences in a nationally representative sample of children and youth ages 2 to 17 years. More than one half (530 per 1,000) of the children and youth had experienced a physical assault in the study year, more than 1 in 4 (273 per 1,000) a property offense, more than 1 in 8 (136 per 1,000) a form of child maltreatment, 1 in 12 (82 per 1,000) a sexual victimization, and more than 1 in 3 (357 per 1,000) had been a witness to violence or experienced another form of indirect victimization. Only a minority (29%) had no direct or indirect victimization. The mean number of victimizations for a child or youth with any victimization was 3.0, and a child or youth with one victimization had a 69% chance of experiencing another during a single year.