Migration of the dominant pacemaker region in the rat sinoatrial node

"Early onset sarcoidosis" is a chronic granulomatous disease occurring in children younger than 5 years of age, and characterized by a classic symptom triad consisting of skin, eye and joint lesions, with on rare occasion pulmonary involvement. The disorder often goes unrecognized because of its rarity and, since polyarthritis and uveitis are the predominant symptoms, most of these children are misdiagnosed as having juvenile chronic arthritis (JCA). A child with erythema nodosum at 7 months of age, later diagnosed as JCA and definitively recognized as "early onset sarcoidosis" is reported. This case shows that, whenever possible, a biopsy showing the typical picture of sarcoid granulomas is crucial to distinguish these clinical conditions.     

Wilson's disease: a review apropos of a clinical experience in 16 patients

Wilson's Disease is an inherited disorder of copper metabolism. We report 16 patients (6 males) with the disease; 6 had hepatic involvement exclusively, 4 had neurological involvement, 3 had a neurological and hepatic involvement and 3 were asymptomatic. The age onset was 9 years for hepatic and 17 years for neurologic involvement. The mean delay in diagnosis was 14 months. Chronic hepatitis, cirrhosis and fulminant hepatic failure were the clinical forms of liver disease. Patients with neurologic disorders had behavioral disturbances and extrapyramidal manifestations such as dystonia and parkinsonism. Patients had a good response to penicillamine, except 3 that died of liver complications, in whom the treatment was delayed or discontinued. We conclude that this metabolic disease must be suspected in pubertal children and in adults of less than 30 years old with liver disease of unknown origin or behavioral alterations associated to an extrapyramidal syndrome.     

Mode of presentation and multisystem involvement in Beh?et's disease: the influence of sex and age of disease onset

OBJECTIVE: To evaluate the correlation between the age at onset of Behcet's disease (BD) and sex distribution, mode of disease appearance, and number of organs involved during the disease. METHODS: BD was defined according to the International Study Group criteria. Data from medical files and from patient interviews were collected. Results were analyzed for children and adults, according to age at disease onset. A systemic involvement index was calculated as the sum of visceral organ systems involved. RESULTS: Fifty-nine patients with BD were studied, 26 male and 33 female. The mean age at disease onset was 8.4 +/- 4.5 years in children and 29.8 +/- 7.9 years in adults. The age of onset was significantly lower in male versus female patients. BD presented in children almost entirely as recurrent aphthous stomatitis, while in adults, less than one-third of patients presented first with oral ulcers. The mean age at disease onset of patients who presented first with oral ulcers was significantly lower than the age of patients presenting first with non-oral aphthosis. The mean systemic involvement index was higher in adult onset than in juvenile onset disease. A significant linear correlation was found between age of disease onset and total number of visceral organ systems involved. CONCLUSION: BD was observed to occur earlier in males than in females. The first manifestation of BD in children is almost exclusively in the form of oral ulcers, while older patients have a large proportion of non-oral aphthosis as their first disease manifestation. Disease onset at an older age is positively correlated with increased disease spectrum.     

Patterns of authority and affection in two generations.

Using data gleaned from a normative longitudinal study of more than 200 families by workers in the Institute of Child Welfare of the University of California at Berkeley, the research was devised to answer the question as to whether the perception of one's own parents influences parental attitudes towards one's own children. These relationships were studied through the media of 3 variables, viz., authority, affection, and involvement, and their intercorrelation. The "characteristic" or modal family pattern which emerges from the data is described and discussed. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

The etiology of idiopathic sudden sensorineural hearing loss. Experimental herpes simplex virus infection of the inner ear

PURPOSE: To evaluate dimercaptosuccinic acid (DMSA) scintigraphy and urography in the detection of renal involvement in children with urinary tract infection (UTI) in order to identify patients with a high risk of developing renal damage. MATERIAL AND METHODS: A total of 157 children (median age 0.4 years, range 5 days to 5.8 years) with first-time symptomatic UTI were examined scintigraphy (with an assessment of renal area involvement) and urography at the time of UTI and 1 year later. All evaluations were made blindly. RESULTS: Of the total 314 kidneys, 80 (25%) were abnormal at initial scintigraphy. Of these 80 kidneys, 44 (55%) had normalized at follow-up. Of the 234 initially normal kidneys, 29 (12%) were abnormal at follow-up. One year after UTI, abnormalities were seen in 59 children at scintigraphy and in 18 children at urography. Renal area involvement was larger and split function abnormalities more common in kidneys that were abnormal at both scintigraphy and urography than in kidneys with only scintigraphic abnormalities. CONCLUSION: Quantitation of renal area involvement and split renal function at early scintigraphy would seem to be useful in identifying patients at risk of developing renal damage. Urography at 1 year after infection identified mainly those with the most severe scintigraphic abnormalities. The clinical importance of scintigraphic abnormalities that are not confirmed by urography is not known.     

Phylogenetic relationships of the Nassulida within the phylum Ciliophora inferred from the complete small subunit rRNA gene sequences of Furgasonia blochmanni, Obertrumia georgiana, and Pseudomicrothorax dubius

The objective of the study was to ascertain the occurrence and inter-relationships of locomotor symptoms, joint hypermobility and skin involvement in patients with the Marfan syndrome. A single clinical evaluation, using a standardized protocol, of randomly selected out-patients was made. Joint hypermobility was measured by two scales in wide clinical use (Beighton and Contompasis), skin hyperextensibility was assessed on the dorsum of the hand, and skin thickness and light transmissibility was measured at the same site with a modified Harpenden caliper. The setting was an out-patient medical genetics clinic at an urban teaching hospital in Baltimore, Maryland, USA. The subjects comprised 27 children and 48 adults who met strict diagnostic criteria for the Marfan syndrome. In patients less than 18 yr old, 70% had experienced at least one locomotor syndrome, and 40% had had multiple symptoms, of which arthralgia, myalgia and ligamentous injury were the most frequent. Symptoms were absent in children younger than 5 yr. Thereafter, the number of symptoms increased with age. Considerable joint hyperextensibility (> 3/9 of Beighton's criteria) was present in 85%. While one-third had received orthopaedic attention, there had been little if any rheumatological input. In adult patients, locomotor symptoms had occurred in 96%, with 88% having experienced more than one complaint. Spinal pain, arthralgia, ligament injury and fracture were the most common. Most (81%) of the adults had some (> 1/9), and 56% had considerable (> 2/9) evidence of joint hypermobility. Only 20% had received specialist attention for their locomotor symptoms. Skin changes are documented in the Marfan syndrome for the first time in this study.(ABSTRACT TRUNCATED AT 250 WORDS)     

Concentration-dependent killing of antibiotic-resistant pneumococci by the methoxyquinolone moxifloxacin

Acute lymphoblastic leukemia (ALL) is the most frequent cancer encountered in children. Little is known about the molecular pathology of childhood T cell ALL. Oncogenesis is a multistep process that involves alterations in proto-oncogenes and tumor suppressor genes. Recently, a mutator phenotype detectable by microsatellite instabilities was shown to be associated with predisposition to cancer. This new mechanism for human carcinogenesis is caused by defects in the DNA replication/repair system. To study the involvement of some of these mutational events in the development of T cell ALL, we have initiated a systematic search for losses of heterozygosity (LOH) and microsatellite instabilities in children affected with this disease. These patients were allelotyped by PCR using 56 microsatellite markers located near known or putative tumor suppressor genes. The microsatellite patterns were altered in more than 80% of the patients. LOH were detected in chromosomes 6p, 12p and 9p. Two third of the patients were deleted for chromosome 9p21, suggesting the involvement of a tumor suppressor gene, probably the p16 gene. The only patient refractory to chemotherapy was shown to be associated with a mutator phenotype. This is the first documented case of a childhood neoplasia associated with genomic instabilities. Our results suggest that defects in DNA replication/repair components are involved in the development of a subset of childhood T cell ALL.     

Temporal rhythms in family life: Seasonal variation in the relation between parental work and family processes.

Families with school-age children were interviewed 3 times, at 6-mo intervals (winter-summer-winter), to test whether summer brings discontinuity in family processes. Longitudinal patterns of parent–child involvement, parental monitoring, and children's involvement in activities were examined for 125 families in 3 groups: DDD (consistently dual-earner), SSS (consistently single-earner), and DSD (dual-earner at Times 1 and 3, single-earner over the summer). In SSS and DSD families, mothers became more involved with their children over the summer, and the division of parenting became more traditional (with mothers more involved than fathers), whereas DDD families maintained a more egalitarian division of parenting. DSD fathers monitored their children less over the summer, whereas DDD fathers became more knowledgeable about their children's activities. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Metastatic brain involvement in Ewing family of tumors in children

OBJECTIVE: To evaluate the incidence and clinical characteristics of CNS involvement in Ewing family of tumors (EF) in children. METHODS: Chart reviews of children with EF treated in our center from 1972 to 1997. Clinical and imaging data regarding possible CNS involvement were collected. RESULTS: During this 25-year period, 80 children with EF were treated. Intracranial involvement was found in eight (10%) children: the brain was involved in seven children (8.8%) and a retro-orbital metastasis without parenchymal brain involvement was noted in one child. Metastases were localized intrahemispherically, or in the cerebellum or the basal ganglia. Intracranial spread was hematogenous in five children and by contiguous spread from the skull in three children. Intracranial involvement was diagnosed 1.3 to 11 years from initial presentation. Seizures and hemiparesis were the main neurologic complications. CONCLUSIONS: The rate of parenchymal brain involvement in our patients with EF was 8.8%. Spread was mainly hematogenous. Substantial morbidity was associated with CNS disease, which appeared in most patients late in the course of disease.     

Routine bilateral exploration for inguinal hernia in infancy and childhood

Whether to explore the contralateral side is a real question regarding the management of unilateral inguinal hernias in infants and children. The surgeon can easier make a decision if he knows the true incidence and prevalence of the bilateral involvement in different ages. During the years 1982 to 1991 the authors could find 138 contralateral hernias developed after unilateral herniorrhaphy in 2554 children (5.4%). Fifty-five percent of these children (76 patients) were younger than 1 year of age, 74% of them (102 patients) were younger than 3 years of age at the time of the initial repair. The interval between the operations was less than 1 year in 54% of the cases. To get more precise data, we explored 148 children younger than 3 years of age bilaterally during 1992. The result was positive in 104 cases (70.3%). The bilateral involvement was most frequent under the first 6 months of life (83.5%), then it dropped gradually. We found a patent processus vaginalis on the asymptomatic side in 74.3% of girls and in 61.1% of boys. The overall contralateral involvement of unilateral hernias was 64.8%, independently of which side the hernia had appeared on. The patent processus vaginalis can be regarded as a precursor of indirect hernias, so the contralateral exploration can prevent the development of a later hernia. The authors conclude that bilateral exploration is mainly justified during infancy, but in case of girls they suggested applying it until three years of age.     

A controlled evaluation of behavior modification with conduct problem children.

Evaluated a behavioral intervention program for conduct problem children with both behavioral and academic difficulties in elementary school. 16 Ss were each assigned to either a treatment or a "no-contact" control group. A standardized 20-hr treatment program involving the child, parents, and the teacher was provided by clinical psychologists. Both observational recordings and teacher ratings of social and academic behavior demonstrated that significantly greater behavioral improvement had occurred for treated than for control children. However, at a 9-mo follow-up, the control group had improved sufficiently that these differences were no longer significant. Although no differences existed between treated and untreated children in achievement test performance (California Achievement Test) or grades at termination, follow-up revealed that the treated Ss had significantly better achievement scores and grades 9 mo after termination. Ratings of therapists by teachers and parents were uniformly positive, and there were no dropouts during the 3-4 mo course of treatment. (21 ref) (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Vernal keratoconjunctivitis in the child. Clinical and complementary study apropos of 22 cases

PURPOSE: Vernal kératoconjunctivitis was studied in a population of 22 children aged 3 to 14 years and followed up in an allergy and ophthalmology outpatient clinic. The role of allergy and the severity of inflammation where assessed by a systematic exploration, which combined a detailed allergy evaluation and blood and lacrimal sampling. MATERIALS AND METHODS: Allergy criteria chosen and recorded in 9 cases are: an increase of total IgE over the higher limit for the age, a positive skin prick test to one allergen, a positive serum specific IgE dosage (> 0.35 IU/mL) of specific IgE. Conjunctival allergy was present in 6 of the 9 children with a positive allergenic provocation test, or with a high local production of total IgE and a lacrimal/serum eosinophilic cationic protein ratio greater than one. RESULTS: Criteria used for supporting the IgE mediated hypersensitivity diagnosis are discussed: they have to be very strict to eliminate false positive results. Allergen involvement can only be evidenced by a specific provocation test. When evidenced as described, limbic or palpebral conjunctivitis had the same frequency. Lacrimal ICAM 1 levels seemed to be higher (p < 0.05) in the severe limbal forms (24.7 +/- 3 pg/mL) than in the palpebral ones (8.1 +/- 6.5 pg/mL). Interpretation of biological parameters evidencing conjunctival inflammation is more difficult. CONCLUSION: Allergic involvement in child vernal keratoconjunctivitis can only be assessed through a detailed evaluation, leading to a specialised ophthalmic and allergic management. A specific treatment can then be established, based on allergen eviction and possibly on specific immunotherapy (5 cases). H1 antihistamin treatments are dedicated only to children with a positive allergic evaluation.     

Sarcoidosis in children. Epidemiology in Danes, clinical features, diagnosis, treatment and prognosis

This paper reviews current knowledge of childhood sarcoidosis with regard to the epidemiology in Danes, clinical presentation, diagnostic procedures, treatment and prognosis. Sarcoidosis is a granulomatous disease of unknown aetiology, with multiorgan involvement. The diagnosis is confirmed by the demonstration of epitheloid cell granulomas in tissue biopsy specimens. During the period 1980-92, three cases of childhood sarcoidosis were recorded in Copenhagen County, which has a total population of 610,000. The approximate incidence of clinically recognized sarcoidosis in Danish children younger than 15 y of age was 0.22-0.27/100,000 children per year, corresponding to approximately three new cases in Denmark each year. The true incidence is unknown, since the disease is often asymptomatic and resolves without a clinical diagnosis being made. In children younger than 5 y of age, the disease is characterized by involvement of skin, eyes and joints, whereas in older children involvement of lungs, lymph nodes and eyes predominate. The mainstay of treatment consists of oral corticosteroids. The risk/benefit ratio of using long-term corticosteroids needs to be evaluated in each individual patient. Some patients may benefit from additional therapy with methotrexate. The long-term prognosis is not well established, but it seems to be poorer in children younger than 5 y. Older children appear to have as favourable a prognosis as young adults.     

Late results of the complex treatment of primary kidney neoplasm in children

88 children with primary renal tumours (85 with nephroblastoma and 3 with renal cell carcinoma) were treated between 1973 and 1990 in the paediatric urology department of Institute "Pirogov" and the paediatric haematological oncology department in Sofia. Combined therapy includes early surgery (nephrectomy + lymphadenectomy) and combination chemotherapy (Dactinomycin, Vincristine + Adriamycin, Endoxan). Stage II (with lymph node in involvement), III and IV disease was treated by radiotherapy. Chemotherapy and preoperative radiotherapy were administered in the case of very large tumours. In this series of 88 children, 64 (72.2%) are alive and 21 (23.8%) have died, while no information was available for 3 cases (3.5%). Survival was related to clinical stage, histological type and the child's age. One of the 2 cases with bilateral tumours survived for more than 2 years after the operation and combined therapy. One girl with liver and spleen metastases survived in remission for 11 years after the operation. Two children with pulmonary metastases, discovered one year after surgery for the primary tumour survived in remission for 4 and 14 years, respectively. The authors discuss the good results obtained with early combined therapy in this type of tumour.     

Pigmentary glaucoma? Relations to megalocornea

The student population at Edinburgh University was surveyed in 1974 to collect data on women's menstrual cycles and to examine sources of individual differences in reporting symptoms associated with the cycle. 2542 non-oral contraceptive (OC) users and 756 OC users provided data. Both sets of respondents were questioned about the occurrence, both premenstrually and during menstruation, of 9 symptoms. The "physical" symptoms (stomachache, backache, nausea, fainting) were more often reported during menstruation, whereas the so-called "emotional" symptoms (lethargy, irritability, depression, tension, headache) had a greater prevalence premenstrually. When the 9 menstrual symptoms were broken down by students' fields of concentration, arts students were found to report more "emotional" symptoms than those in the sciences and professions (medicine and law). Further analysis showed that only and 1st born children were slightly less likely to report symptoms than those whose nearest sibling is 7 or more years older or younger or later born children. A similar trend was found for the reporting of illness in an earlier analysis of data from this sample. When cycle length and reqularity were analyzed together, women with long and regular cycles were found to have the lowest proportion reporting symptoms during menstruating with the exception of tension, nausea, and fainting, and women with short and irregular cycles had a correspondingly high incidence of all symptoms. In addition, women with more regular cycles were more likely to report infrequent recall of dreams than those with irregular cycles. Overall, the women who are most likely to report menstrual symptoms, especially those of an "emotional" character, are more likely to report other illnesses with emotional connotations, recall their dreams more frequently, show a preference for the arts, and tend to have been brought up with older siblings. This cluster of personality attributes defines an "expressive" personaltiy in contrast to the more "controlled" style of women at the other extreme.     

Clinical and immunological profile of SLE: some unusual features

OBJECTIVE: To study the clinical and immunological profile of children with systemic lupus erythematosus (SLE). DESIGN: Retrospective hospital based study. SETTING: Tertiary level center of North India. SUBJECTS: Sixteen children in the age group 4-12 years. METHODS: Medical records of children with SLE were analyzed. Clinico pathological features were compared with 2 other series from India. RESULTS: Mean age of children at the time of diagnosis was 10 yr and 8 (50%) children were less than 10 yr of age. The female to male ratio was 7:1. Fever (56.2%), rash (87%) and arthritis (87%) were the common clinical manifestations, Renal involvement was noted in 56.2% of cases. Other clinical features included hemolytic anemia (31.2%), thrombocytopenia (18.6%) and Raynaud's phenomenon (12.5%). Cardiac involvement in the form of severe myocarditis and endocarditis occurred in one patient each. Pulmonary hypertension was the presenting feature in one child with right heart failure. One child had multiple sclerosis along with SLE--a rare combination. ANA positivity was seen in all children. Five children died; two had severe cardiac involvement. Three children had renal involvement and one died of pulmonary hypertension. Two-thirds of subjects with renal involvement improved after therapy according to NIH, Bethesda protocol. CONCLUSIONS: SLE must be considered in any child with multisystem disease, as the disease may have certain unusual presentations.     

Paternal involvement with adolescents in intact families: the influence of fathers over the life course

We measure the quality and quantity of fathers' involvement with adolescent children in intact families over time using longitudinal data from The National Survey of Children. We examine differentials in fathers' involvement by children's and family characteristics and model the long-term effects of fathers' involvement on children's outcomes in the transition to adulthood. Fathers are more involved with sons than with daughters and they disengage from adolescents with increasing marital conflict. We find beneficial effects for children of father's involvement in three domains: educational and economic attainment, delinquent behavior, and psychological well-being. The course of affective relations throughout adolescence also has a beneficial effect on delinquent behavior and psychological well-being.     

Children of substance abusers: the life experiences of children of opiate addicts in methadone maintenance

We interviewed 70 substance abusers in methadone maintenance treatment regarding the life experiences of their 188 children. Sixty-four percent of the mothers reported using psychoactive drugs during their pregnancies. Eighty percent of parents reported an arrest during the time the child was growing up, 34% reported receiving treatment for an emotional disorder, and 14% were hospitalized. Four percent of the parents reported that their children had been placed in adoptive care, 9% in foster care, and 1% had a child in a group home. Forty-one percent of the parents reported that at least one of their children repeated a grade in school, 19% were involved in truancy, and 30% had been suspended from school. Twenty percent of parents reported their children's involvement with the law, and 17% reported drug or alcohol use. Problems with the law correlated highly with 1) being held back a grade, 2) truancy, 3) suspension from school, 4) expulsion from school, and 5) treatment for alcohol or drug abuse.     

From figurative to operative expectancy in the perceptual judgments of children.

Conducted 2 studies on "figurative expectancy," the tendency to make perceptual judgments on the basis of temporal patterns. In Exp I, 60 4-9 yr old children were shown 4 series of animal pictures in which the relative strengths of the temporal patterns were varied. Results show (a) a significant decrease with age in figurative expectancy and (b) significantly more figurative expectancy in the case of a strong temporal sequence linked with a weak critical figure than in the case of a weak temporal sequence linked with a strong critical figure. In Exp II, 66 4-yr-old children were shown a single series of pictures presented at different rates and with critical figures that were ambiguous or nonambiguous. Results show that there was more figurative expectancy the more rapid the rate of presentation. There was also an ambiguity effect showing more figurative expectancy judgments the greater the ambiguity of the critical figure. Results support Piaget's theory of perceptual development. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Cardiovascular changes in children with mucopolysaccharide storage diseases and related disorders--clinical and echocardiographic findings in 64 patients

Cardiovascular abnormalities were evaluated in 64 children aged between 1 year 9 months and 25 years with mucopolysaccharidoses (MPS) and related disorders. A heart murmur was heard in 18 patients, but in only 6 was it characteristic for specific valvular lesions. Echocardiography was performed in 63 children. In one girl cardiac lesions were diagnosed on autopsy. In 46 patients (72%), valvular lesions and/or different types of cardiomyopathy were detected. There were no characteristic changes for different types of MPS. In the majority of children in whom dermatan sulphate accumulated, cardiac involvement was the most frequent (88%) and severe. The most common lesion, regardless of MPS type, was thickening of the mitral valve (66%), with regurgitation or stenosis in 28 (44%). Aortic valve thickening was detected in 17 patients (27%), asymmetric septal hypertrophy or hypertrophic cardiomyopathy in 18, congestive cardiomyopathy in 1 and endocardial thickening in 13 patients. Cardiac involvement was less frequent in children with Sanfilippo disease. Two or more echocardiographic examinations were performed in 23 patients. In 19 of them (83%) cardiac changes were more severe during the second examination. One 7-year-old boy with Hunter disease underwent successful mitral valve replacement. CONCLUSIONS; Cardiac involvement is present in most patients with MPS although there are few clinical signs and symptoms. The most common and severe changes are in Hurler, Hunter, Maroteaux-Lamy and I-cell disease, rarely in Sanfilippo disease. Mitral valve deformation is most frequent in all patients. The cardiac lesions are progressive.