The role of intravenous immunoglobulins in pediatric diseases.
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Many nations now export military health as a proactive arm of the nation's contribution to the maintenance of international peace in trouble regions of the world; and all nations are called upon from time to time in emergency and disaster situations to help out in their regions of interest. Children and young teenagers constitute some 50% of war-stricken populations. This paper explores this increasingly important role of military medicine from the point of view of a practicing pediatrician and career doctor-soldier. Many international operational deployments undertaken in the last 5 years have required the insertion of pediatric clinical and preventive health resources. Deployments to Rwanda, the countries of the former Yugoslavia, Somalia, Bougainville (in Papua New Guinea), Irian Jaya (in Indonesia), and the Aitape tsunami disaster response (the Sepik region of Papua New Guinea) have all necessitated major pediatric interventions. In some operational deployments, in excess of one-third of patient and clinical contacts have involved the care of children, including clinical treatments ranging from life-saving resuscitation to the care of children with both tropical and subtropical illnesses. They have also involved mass immunization campaigns (e.g., in Rwanda) to prevent measles and meningococcal septicemia. In developing countries, at any time approximately 1 to 4 teenage and adult women is pregnant; and of these, 1 in 15 is suffering a miscarriage during any 2-week period. The implications of this audit are that service members must be multi-skilled not only in the traditional aspects of military medicine and nursing but also in (a) the developmental aspects of childhood; (b) the prevention of infectious childhood diseases by immunization and other means; (c) the recognition and management of diseases of childhood; and (d) the management of the normal neonate and infant, especially those orphaned in refugee disaster and other emergency situations. Doctor-soldiers hold special credentials to be advocates for the protection of children caught up in armed conflict and its aftermath. In this context, advocacy to ban anti-personnel land mines is topical, because unfortunately the more than 2,000 deaths and injuries each month involve children and their families, often injured long after the cessation of hostilities. Pediatric issues are now the business of all who serve in the health disciplines in the broader profession of arms.
In this ethnographic study, the authors examined the experiences and perspectives of children hospitalized because of SARS (severe acute respiratory syndrome), their parents, and pediatric health care providers. The sample included 5 children, 10 parents, and 8 health care providers who were directly affected by SARS during the time of the outbreaks and extreme infection control procedures. The data analyses illuminated a range of perceived experiences for this triadic sample. Issues related to social isolation due to infection control precautions were predominant. Themes included emotional upheaval, communication challenges, and changes in parental and professional roles. These findings reveal the cogent effects of SARS on family-centered care. The notion of providing family-centered care within an environment plagued by an infectious outbreak suggests an omniously difficult task. Efforts must be made to optimize family-centered care despite obstacles. The authors suggest effective clinical approaches in the event of future outbreaks.
New pharmaceutical technologies had created either enthusiastic hope for major improvements in the medical treatment or major doubts on the use of research data and wrongdoing in clinical trials. Clinical investigators have to consider methodological and scientific principles, to stick to ethical rules, national law and international guidelines. With the invention of General Clinical Research Centres the necessary infrastructure in hospitals with well-trained personnel and the availability of laboratory, clinical and supporting service has been created in the US. Taking GCRC as a model to improve the clinical research in pediatrics, a formal collaboration between the Department of Pediatrics and the Institute for Biometry and Medical Informatics was established at the Otto-von-Guericke-University Magdeburg in 2001. Both nation-wide quality assurance programmes in Pediatric Endocrinology and clinical trials according to the principles of good clinical practice ('GCP') were planned and currently performed. Thus with the resources of the Medical faculty and additional project bound finances the necessary infrastructure has been developed.
The field of genetics will continue to grow because of advances in molecular biology. These advances will lead to further understanding of genetic conditions, which will enhance the urologist's knowledge of the urologic manifestations of these conditions. This will enable improved parental counseling and patient care.
The existence of progenitor (stem) cells in the human liver remains a matter of debate. In rodent models of hepatocarcinogenesis and injury, oval cells proliferate in the periportal regions of the portal tracts and are suggested to derive from a stem cell compartment, because they are capable of differentiating into hepatocytes or biliary epithelial cells. In this study, the rat oval cell marker, OV-6 has been used to investigate the hypothesis that there are stem cells present in fetal and pediatric human liver. The pattern of OV-6 expression was compared with the established adult biliary cell markers human epithelial antigen-125 (HEA-125) and cytokeratin-19 (CK-19). In normal pediatric liver (n = 7), bile ducts and ductules were immunostained with CK-19 and HEA-125, whereas OV-6 staining was consistently negative. In fetal tissue (n = 10), ductal plate cells, primitive bile ducts, and hepatoblasts were stained with CK-19 and HEA-125 although only some of the ductal plate cells and hepatoblasts were OV-6 positive. In biliary atresia (n = 6) and 1, anti-trypsin deficiency (1,AT) (n = 4), CK-19 and HEA-125 immunostained ductular proliferative cells that tended to form finely anastomosing ductules, whereas OV-6 staining was found more on discrete cells confined to portal tract margins. Additionally, in diseased liver, OV-6 was strongly positive in hepatocyte lobules with greatest intensity in the periseptal regions. This widespread hepatocyte OV-6 positivity suggests that the antibody may identify cells of a less differentiated phenotype (transitional hepatocytes) that have replaced the mature cells. Therefore, it is proposed that in human liver, OV-6 is recognizing cells with a progenitor stem cell-like phenotype with the capacity to differentiate into OV-6 positive ductular cells or lobular hepatocytes.
The stem cell factor (SCF)/c-kit ligand/receptor system has been implicated in stem (oval) cell activation following liver injury in the rat. The aim of this study was to determine the role of the SCF/c-kit system in pediatric human liver during acute and chronic liver injury. Tissue was obtained from hepatectomy specimens of patients undergoing liver transplantation for extrahepatic biliary atresia (EHBA) and fulminant hepatic failure (FHF). Specific expression of mRNA for c-kit and beta-actin was measured by ribonuclease protection and by immunohistochemistry to localize c-kit in tissue sections. Expression of c-kit was detected at relatively consistent levels in normal and cirrhotic (EHBA) livers. However, in FHF, c-kit mRNA levels were elevated in 3 of 6 specimens. Immunolocalization highlighted the presence of small numbers of c-kit-positive cells in the portal tracts of normal livers with increased numbers in cirrhotic livers. The highest c-kit staining, however, was observed in FHF, in which, in addition to the cells in the portal tracts, discrete c-kit-positive cells were also found integrated into bile ducts. Colocalization studies demonstrated some of the c-kit-positive cells to be of mast cell, leukocyte, and hematopoietic cell origin. However, there remained a subset that was also negative for these markers. The up-regulation of c-kit receptor expression in diseased livers suggests an involvement of this receptor/ligand system in hepatic repair mechanisms, and we speculate that c-kit-positive cells may represent a hepatic progenitor cell population. The origin and growth/differentiation potential of these c-kit-positive cells is under investigation.
Seasonal variations in the incidence of the most common acute diseases in childhood during the inter-epidemic year 1978 were investigated using a sample of 7,068 walk-in patients attending. Aurora Hospital, Helsinki. At least seven out of the ten most common diseases were of infectious origin. Generally, boys and the younger groups were affected most often. The clearest seasonal difference in both sexes was observed in patients with gastroenteritis and otitis media. Among boys, there was also a marked fluctuation in the incidence of asthmatic bronchitis and asthma, pneumonia, and laryngitis, whereas, among girls, marked variations were observed only in urinary tract infections and acute upper respiratory infections. Children between 1 and 3 years of age appeared to be most prone to the common cold syndrome all the year round.
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BACKGROUND: Schimke immuno-osseous dysplasia (SIOD) is a rare autosomal recessive multisystemic disorder caused by mutations of the SMARCAL 1 gene (SWI/SNF-related, matrix-associated, actin-dependent regulator of chromatin, subfamily a-like 1). CLINICAL FEATURES: Main clinical features are: disproportional growth deficiency due to spondyloepiphyseal dysplasia, nephrotic syndrome with focal and segmental glomerulosclerosis, and defective cellular immunity. Patients with severe SIOD have life-limiting complications like cerebral ischemia due to vaso-occlusive processes. Only a few patients reached adulthood. CASE REPORTS: The clinical course of four adult SIOD patients is presented. CONCLUSION: Even patients with severe SIOD can reach adulthood. Therefore, doctors working in the field of internal medicine and family doctors should be familiar with the clinical picture of SIOD.
Recombinant factor VIIa (rFVIIa) is increasingly used outside the labeled indications for treatment of life-threatening bleeding episodes after failure of the respective standard therapy. An interdisciplinary group of experts summarizes the state of knowledge of the use of rFVIIa in gastroenterology and hepatology, thrombocytopenia and -pathia, coagulation factor deficiencies, von Willebrand's disease, periinterventional bleeding without specific bleeding diathesis, drug-induced bleeding, disseminated intravascular coagulation, and neonatology. The most commonly used dose is 90 microg/kg body weight rFVIIa as bolus, if necessary followed by additional injections at intervals of 2-3 h. In factor VII deficiency lower dosages of 15-30 microg/kg body weight of rFVIIa are given every 4-6 h, whereas higher doses of 150-200 microg/kg body weight are used in neonates.
PHENOMENON PAIN: While pain is one of the main reasons for an unscheduled visit to the paediatrician, pain due to painful procedures is of major importance in scheduled visits. Actual pain therapy is illustrated in the treatment of burns. Incomplete analgesia may have an unfavourable impact on morbidity and mortality. The pain score does not correlate with the extent of the burned area, and is regularly underestimated. General anaesthesia or analgo-sedation are warranted during the care of the burned patient. INSUFFICIENT ANALGESIA: Consequence of insufficient analgesia during primary care is an increased need of analgesics, and an increased pain treatment failure rate during subsequent procedures. Pain is interfering with anxiety, sleep disturbances and post-traumatic psychologic alterations. All those symptoms must be treated adequately. ACUTE ILLNESS AND INJECTIONS: This article covers pain from otitis media, pharyngitis, Guillain-Barré syndrome, purpura fulminans, Toxic Epidermal Nekrolysis, as well as the usage of local anaesthesia during injections, not to forget the application of non-pharmacologic methods for pain therapy and prophylaxis.
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Since 1990, management options available for children with paroxysmal supraventricular tachycardia (PSVT) have included radiofrequency catheter ablation (RCA). To determine the efficacy and safety of the procedure and to maintain a database for long-term follow-up, the Pediatric Electrophysiology Society began a Pediatric RCA Registry on January 1, 1991, to which 46 centers have submitted data from 4,135 total children and adolescents (patient age 0.1 to 20.9 years) who underwent 4,651 RCAs (through September 15, 1996). Of the 88% with a structurally normal heart, PSVT mechanisms (n = 4,030) included 3,110 accessory pathways and 920 atrioventricular node reentry tachycardia (AVNRT) during 3,653 procedures for 3,277 patients. During the 7 years of the Registry, analysis of indications for the procedure has shown a gradual shift. During the first year of the Registry for this PSVT group, "medically refractory tachycardia" was listed as the indication for 44% and "patient choice" was listed as 33%, compared with 29% and 58%, respectively, for the years 1995 to 1996 (p <0.005). Registry results were: 90% immediate success for accessory pathways (95% for left lateral; 87% for septal; 86% for right free wall) and 96% for AVNRT; mean fluoroscopy time 47.6 +/- 40 SD minutes; procedure time 257 +/- 157 SD minutes; major complication rate at the time of the procedure 3.2%. Procedure-related deaths included 1 immediate and 3 at 2, 12 and 68 weeks after the procedure (2 were infants). Follow-up revealed 77% and 71% freedom from recurrence at 3 years for accessory pathways AVNRT, respectively, and rare (<1%) detection of additional complications. RCA has evolved into a standard management option for PSVT in children with a structurally normal heart. RCA for children and adolescents should be recommended after consideration of the procedural risk/benefit compared with that of other management options, the natural history, and individual tolerance/symptoms related to PSVT.
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