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At least 253 records · Page 14Linked to original sources
PubMed1985

The effect of intra-abdominal resection or fecal diversion on perianal disease in pediatric Crohn's disease.

To determine whether perianal Crohn's disease responds to resection of active intra-abdominal disease or to diversion, 273 cases of Crohn's disease in children (ages 4 to 18 years) were reviewed (all had been seen between 1970 and 1983). Of these, 104 patients underwent an intestinal operation, and 42 of the 104 (40%) had documented perianal disease at the time of their operation. Follow-up averaged 8 3/4 years from time of diagnosis. Three of the 42 died, at 1, 3, and 5 months postoperatively (mortality, 7%) and one was unavailable for follow-up. In these 42 patients with active perianal disease, an intestinal resection with primary anastomosis was performed in 19; one died postoperatively and one was unavailable for follow-up. Of the remaining 17 patients, five (29%) improved and 12 (71%) had continued perianal disease, either without change or worse. At least one further intestinal resection was needed in five of the 17 and two have needed proctocolectomy. A defunctionalizing procedure was done as part of the initial operation in 11 of the 42 patients. Two showed improvement; however, both needed proctocolectomy for rectal disease, and none of the 11 with fecal diversion have had reestablishment of intestinal continuity. In 12 of the 42 patients proctocolectomy was the initial procedure, usually for severe rectosigmoid disease. Of 38 children followed, 20 had proctocolectomy, 11 initially and nine after lesser procedures; overall rate of proctocolectomy was 53%. Resection of active intra-abdominal disease is not necessarily followed by resolution of perianal lesions. Defunctionalizing the rectum with fecal diversion does not appear to alter perianal disease.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed2003

Cytokine tissue levels as markers of disease activity in pediatric Crohn disease.

The mucosal immune system is overactivated in Crohn disease (CD) and viral infections have been associated with clinical exacerbations. To investigate the potential association between mucosal inflammation and the cytokines involved in the early response to viruses, we analyzed colonic tissue levels of IL-2Ralpha, interferon-alpha, and IL-15 in CD. Patients undergoing diagnostic colonoscopy were classified into controls (n = 22) and three CD groups based on the histologic severity of inflammation and clinical activity: a) severely active CD (n = 3); b) mild to moderately active CD (n = 14); and c) quiescent CD (n = 23). Rectal biopsies (two per patient) were homogenized and cytokine levels determined by ELISA kits. Statistical analysis was performed by ANOVA with Tukey and Scheffé tests. IL-2Ralpha levels were increased in the active CD group compared with the quiescent CD group: a) 405 +/- 87, b) 159 +/- 31, and c) 33 +/- 15 pg/mg DNA (p < 0.001). The latter group was similar to controls (39 +/- 20 pg/mg DNA). Furthermore, a linear correlation (r = 0.98) between IL-2Ralpha and disease activity (Van Hees index) was observed. IL-15 levels were also higher in active compared with quiescent CD and controls: a) 0.69 +/- 0.23 and b) 0.72 +/- 0.31 versus c) 0.28 +/- 0.21 and 0.28 +/- 0.14 pg/mg DNA for controls (p < 0.05). Interferon-alpha levels were undetectable in all samples. Our data suggest that IL-2Ralpha tissue levels correlate with CD activity. IL-15 is also overproduced in inflamed CD tissue. The lack of a parallel elevation of interferon-alpha does not support a role for viral induction of IL-15 in inflamed CD samples.

Adolescent↗
PubMed1996

The incidence of pediatric rheumatic diseases: results from the Canadian Pediatric Rheumatology Association Disease Registry.

OBJECTIVE: To determine the incidence of rheumatic diseases in children, and the frequency of musculoskeletal disorders seen by pediatric rheumatology specialists in Canada. METHODS: Applying standardized disease definitions and disease codes modified from ICD-9, members of the Canadian Pediatric Rheumatology Association from 13 centers in all 10 provinces of Canada registered all new patients seen between May 1, 1991 and April 30, 1993. Patient data included age, sex, ethnicity, date of birth, date of disease onset, date of diagnosis, and diagnostic codes (more than one diagnosis could be entered). To minimize the bias of right censoring, only data from patients with disease onset between May 1, 1991 and October 31, 1992 were used to estimate disease incidence. RESULTS: 3362 records totalling 3683 diagnoses (92 separate diagnoses) were registered. Median referral rate per year to a pediatric rheumatology center was 26 per 100,000 children at risk. The frequency of diseases seen was 23.3% for all forms of chronic arthritis, 6.5% for connective tissue diseases, and 6.1% for all forms of vasculitis. The minimum incidence rates per 100,000 children at risk per year calculated from the whole registry were: all forms of chronic arthritis 4.08 (95% CI: 3.62, 4.60), systemic lupus erythematosus 0.28 (0.18, 0.45), and dermatomyositis 0.15 (0.09, 0.29). Substantially higher figures were obtained if the figures were calculated excluding the 2 provinces (Alberta and Quebec) that had disproportionately low referral rates. CONCLUSION: Pediatric rheumatologists see children with a wide variety of diseases. It is important that pediatric rheumatology training reflects this and does not focus exclusively on the classical inflammatory arthropathies. The minimum incidence data show there are substantial numbers of children developing potentially lifelong chronic rheumatic diseases each year in Canada. These data should be helpful in planning the delivery of pediatric rheumatology services not only in Canada, but also in other developed countries.

Adolescent↗
PubMed1999

[Prion diseases in pediatrics].

Prion diseases are rare neurologic affections with a poor prognosis, occurring in both humans and animals. Creutzfeldt-Jakob disease (CJD) secondary to human extracted growth hormone treatment is the most frequent condition in pediatrics. In 1994, a new type of CJD (variant CJD) was described in young adults in the United Kingdom, only 10 years after the bovine spongiform encephalopathy epidemic, with recent works showing a direct relationship between the bovine epidemic and the human cases. An accumulation of a single protein called the prion protein (PrP) has been discovered in the brain in all of these cases, animal and human, leading to the hypothesis that a new infectious agent could proceed without any nuclear acid information; another hypothesis is that of a still unknown viral agent. The PRNP gene encoding for this PrP protein is well described: some mutations and a polymorphism in the 129th codon have been shown to be implicated in many cases of CJD. PrP is a ubiquitous protein, with yet unknown physiological function. There are still many questions to be answered: shall we expect new pediatric cases of variant CJD? Assuming that animal-human contamination is related to alimentation, are there other ways of contamination.

Adult↗
PubMed1995

[Analysis on the health status of residents from Diseases Surveillance Points in Gansu Province].

We have surveyed a population size of 6633315 from Diseases Surveillance Points (DSP) system in Gansu province for the last eleven years. The annual birth rate was 18.20% with an annual standard mortality rate 545.80/10(5). The annual standard mortality for male and female were 607.53/10(5) and 483.29/10(5) respectively. The major causes of death were Respiratory system diseases, Cardiovascular diseases, Neoplasms, Injuries, Digestive system diseases, Pediatric diseases, Infectious diseases in sequence. In eleven years, there seemed to be a rising trend in the mortalities of following diseases as: Cerebrovascular diseases, Ischemic heart diseases, Rheumatic fever and heart disease, Lung Cancer, Liver Cancer, Cancer of the Esophagus, Intestinal cancer, Cervical cancer, Injury, Congenital abnomalities, to different degrees. However, an obvious descending trend on the morbidity and mortality of infectious diseases was moticed. The average life expectancy was 71.05 years in DSP, with male 69.57 years, and female 72.72 years. Diseases with higher PYLL were Injuries, Neoplasms, Respiratory system diseases and the like. Data suggested not only the prevention andcontrol of infectious diseases, but also the surveillance of injuries and the prevention and control of chronic diseases should be strengthened.

Adolescent↗
PubMed1989

Nutritional support for pediatric patients with inflammatory bowel disease.

Pediatric patients with ulcerative colitis and Crohn's disease often suffer from malnutrition and growth failure. This is particularly true in pubertal children. Chronic insufficient nutrient intake is most often the cause of growth failure. Both parenteral nutrition and defined enteral formulas are available to rehabilitate patients with malnutrition and growth failure. Assessment of nutritional status and growth and the use of parenteral nutrition and defined enteral formulas to reverse malnutrition, growth failure, and inflammation in pediatric patients with inflammatory bowel disease are discussed.

Adolescent↗
PubMed2004

Pediatric neurotransmitter diseases.

The pediatric neurotransmitter disorders represent a challenging group of rare neurometabolic disorders classified on the basis of alterations in neurotransmitter metabolic pathways. The disorders are currently classified into disturbances of monoamine and gamma-aminobutyric acid (GABA) metabolism, although disorders of other neurotransmitters, such as glutamate and melatonin, may well be recognized in future investigations. This review summarizes the clinical and laboratory features of selected pediatric neurotransmitter disorders that have been partially delineated. Of the monoamine group, these are Segawa disease or guanosine triphosphate-cyclohydrolase I deficiency, aromatic L-amino acid decarboxylase deficiency, and tyrosine hydroxylase deficiency. Of the GABA disorders, these are pyridoxine-dependent epilepsy, GABA transaminase deficiency, and succinic semialdehyde dehydrogenase deficiency. As proper collection, handling, and interpretation of cerebrospinal fluid is required for assessment of most of these disorders, we end by summarizing important considerations for obtaining cerebrospinal fluid samples.

Biogenic Monoamines↗
PubMed1989

Thyroid nodules in children: current profile of a changing disease.

Pediatric thyroid disease has changed since the decline of widespread cervical irradiation. Our analysis of 32 recently resected childhood thyroid nodules showed that the incidence of malignancy (22%) is decreasing, though the incidence of nodules is stable. Risk factors for development of thyroid disease include female sex, age around puberty, family history of thyroid disease, previous or coexisting thyroid disease, and history of a medical condition that may be steroid- or endocrine-related. Age, sex, location and size of nodule, duration of symptoms, and family or patient history of thyroid disease did not distinguish between malignant and benign disease preoperatively. Of the seven children with thyroid cancer, six (86%) had unrelated concurrent medical conditions, compared with 9/25 (36%) with benign disease and 2/11 (18%) with adenoma. Fine needle biopsies provided correct preoperative diagnosis in 6/7 cases. As pediatric thyroid malignancy becomes rarer and the high-risk nodule becomes better defined, criteria for surgical versus nonsurgical modes of diagnosis and treatment should become more distinct.

Adolescent↗
PubMed2003

The history of Pediatric Infectious Diseases.

The history of Pediatric Infectious Diseases closely parallels the history of Pediatrics at least until the last century, because historically infections comprised the major causes of childhood morbidity and mortality, as they still do in the developing world. This history reviews developments in the field through the centuries and is written so that it does not overlap the contribution to this series by Baker and Katz entitled 'Childhood Vaccine Development in the United States.' Remarkable descriptions of selected pediatric infections existed long before the invention of printing, and early pediatric texts included many chapters devoted to various infections. Coincident with the establishment of pediatric organizations in America in the late 19th and early 20th Centuries, major attention was focused on diphtheria, infant diarrheal illnesses, tuberculosis, streptococcal infections and their complications, and other pediatric infections, and substantial progress was made. The American Pediatric Society (1888), the American Academy of Pediatrics (1930), the Society for Pediatric Research (1931), and the American Board of Pediatrics (1933) all contributed to the evolution of the discipline of Pediatric Infectious Disease, and numerous leaders of these organizations had significant infectious diseases interests. The establishment of the Pediatric Infectious Diseases Society, the Pediatric Infectious Diseases sub-board, and an accreditation process for training programs, as well as sub-specialty textbooks and journal, further validated the development of this specialty, particularly in North America. The many remaining challenges related to infectious diseases in children (including HIV, emerging infections, antimicrobial resistance, opportunistic infections, and infections in the developing world) insure the future of the specialty. The genomic era of medicine and the tools of molecular biology will lead to new insights into pathogenesis, diagnosis, and treatment of infections. Pediatric Infectious Diseases physicians can celebrate the past triumphs of the discipline and future achievements, all contributing to improved health for children.

Child↗
PubMed2005

Evaluation of the pediatric crohn disease activity index: a prospective multicenter experience.

BACKGROUND AND OBJECTIVES: Longitudinal assessment of disease activity is necessary for studies of therapeutic intervention in children with Crohn disease. The Pediatric Crohn Disease Activity Index (PCDAI) was developed a decade ago for such a purpose, but it function has only been examined in a small number of studies with a limited number of patients. The primary objectives of the present study were to develop cut scores reflecting disease activity as determined by physician global assessment (PGA) and to evaluate the responsiveness of the PCDAI to changes in patient condition after therapeutic interventions. METHODS: Data were derived from a prospective database of newly diagnosed children with inflammatory bowel disease established in 2002 at 18 pediatric gastroenterology centers in the United States and Canada. At diagnosis, at 30 days and 3 months after diagnosis, and quarterly thereafter, children (<16 years of age) with Crohn disease had disease assessment performed by PGA and PCDAI. Disease management was provided according to the dictates of the attending gastroenterologist and not by predetermined protocol. RESULTS: 181 patients had concomitant PGA and PCDAI performed at diagnosis, and 95 of these had similar assessment at short-term follow up. Mean +/- SD PCDAI scores for mild, moderate, and severe disease by PGA at diagnosis were 19.5 +/- 10.4, 32.2 +/- 12.7, and 47.8 +/- 14.9, respectively (P < 0.001 for all comparisons). Mean +/- SD PCDAI for inactive disease after treatment was 5.2 +/- 5.4. Receiver operating characteristic (ROC) curve analysis suggested that: 1) activity of moderate/severe disease was best reflected by a PCDAI of > or = 30 points, 2) clinical response (moderate/severe disease improving to mild/inactive) was best reflected by a decrease in PCDAI of > or = 12.5 points, and 3) a PCDAI < 10 best reflected inactive disease. CONCLUSIONS: PCDAI scores accurately reflect disease activity as assessed by physician global assessment. A PCDAI score of > or = 30 has acceptable sensitivity and specificity to indicate disease of moderate/severe activity. A PCDAI decrease of 12.5 points or greater following therapeutic intervention accurately reflects a clinically significant response. The PCDAI is an appropriate tool for intervention trials in Crohn disease in children.

Child↗
PubMed2001

Sleep disorders in children with neurologic diseases.

Pediatric neurologic diseases are often associated with different kinds of sleep disruption (mainly insomnia, less frequently hypersomnia or parasomnias). Due to the key-role of sleep for development, the effort to ameliorate sleep patterns in these children could have important prognostic benefits. Study of sleep architecture and organization in neurologic disorders could lead to a better comprehension of the pathogenesis and a better treatment of the disorders. This article focuses on the following specific neurologic diseases: nocturnal frontal lobe epilepsy and abnormal motor behaviors of epileptic origin, evaluating differential diagnosis with parasomnias; achondroplasia, confirming the crucial role of craniofacial deformity in determining sleep-disordered breathing; neuromuscular diseases, mainly Duchenne's muscular dystrophy and myotonic dystrophy; cerebral palsy, evaluating either the features of sleep architecture and the importance of the respiratory problems associated; headaches, confirming the strict relationships with sleep in terms of neurochemical and neurobehavioral substrates; and finally a review on the effectiveness of melatonin for sleep problems in children with neurologic syndromes and mental retardation, blindness, and epilepsy.

Anticonvulsants↗
PubMed2004

Five questions for assessing psychological problems in pediatric patients cured of neoplastic disease.

Pediatric patients who have been cured of cancer may have particular unexpressed needs and psychopathological consequences. The authors describe their experience with an apparently well-adjusted 20-year-old girl previously cured of rhabdomyosarcoma, who developed severe psychological distress with serious difficulty in controlling her emotions due to defense mechanisms and massive rationalization. The trauma of tumor and its treatment can be associated with psychopathological disorders that may significantly affect the future life of a person cured of cancer. Doctors must learn to notice telltale signs and take the time to investigate the patient's social life and identify any psychopathological disorders. Experience drawn from pediatric oncology might suggest an approach based on a physician-patient relationship that could provide the means for identifying patients' psychological needs and problems.

Adult↗
PubMed1996

Meningococcal disease prevention and control strategies for practice-based physicians. Committee on Infectious Diseases, American Academy of Pediatrics. Infectious Diseases and Immunization Committee, Canadian Paediatric Society.

Localized outbreaks of meningococcal disease in the United States and Canada continue to cause serious alarm within communities as a result of the fulminating pattern of the disease, high mortality rate, and high incidence among adolescents. The increasing number of outbeaks since 1991 has raised questions about the management and prevention of further cases during an outbreak. The purpose of this statement is to guide primary-care physicians in their role in infection control and prevention of both sporadic cases and outbreaks of invasive meningococcal disease. This statement provides information on the epidemiology of meningococcal disease, including definitions of sporadic, secondary, and coprimary cases, clusters of cases, and outbreaks. Data are presented on identification of cases, disease risk of contacts, and agents for chemoprophylaxis, and recommendations are given for: (1) risk assessment of contacts, (2) administration of chemoprophylaxis, (3) appropriate use of meningococcal vaccine, (4) appropriate use of the microbiology laboratory, (5) the necessity for timely and appropriate reporting of invasive meningococcal disease to local public health authorities, and (6) information on counseling and public education that may be helpful during an outbreak to minimize public anxiety. An additional section, "Information for Sharing," which uses a question-and-answer format and which may be helpful to parents and community and health care workers during an outbreak, is also provided.

Adolescent↗