Behavioral capabilities and mortality risk in adults with and without Down syndrome

A review of the diseases caused by Malassezia pachydermatis has led to the conclusion that the yeast is an opportunistic pathogen that depends on predisposing host factors and different immune suppressive mechanisms for clinical manifestation. Until recently, the role of M. pachydermatis in seborrhoeic dermatitis and otitis externa in dogs has been largely unrecognized. The clinical manifestation, aetiology, diagnosis and treatment of disease conditions in dogs caused by M. pachydermatis are reviewed. Human Malassezia furfur infections are briefly described.     

Treatment of functional decline in adults with Down syndrome using selective serotonin-reuptake inhibitor drugs

Alzheimer's disease (AD) is a common cause of functional decline in Down syndrome (DS) adults. Acquired cognitive deficits may be difficult to evaluate in the context of baseline impairments. Behavioral symptoms are also common and may represent the effects of depression, AD, or both. Therefore, the objective of this study was to report a clinical case series of selected adults with DS and behavioral change who responded to treatment with selective serotonin-reuptake inhibitor (SSRI) medication. Six patients, aged 23 to 63 years, 5 women and 1 man, with the clinical diagnosis of DS presented for diagnosis and treatment of functional decline in adult life. Noncognitive symptoms were prominent and included aggression, social withdrawal, and compulsive behaviors. Memory dysfunction was reported in varying degrees. Treatment with SSRI antidepressants was instituted for depressive, apathetic, and compulsive behaviors. Treated patients showed improvement in behaviors as reported by caregivers, and on objective measures, such as workplace productivity. Noncognitive symptoms are a cardinal feature of functional decline in adults with DS and may represent either depression or AD. In some patients, the symptoms respond well to SSRI agents with concomitant improvement in daily function. Treatment trials with SSRIs may, therefore, be warranted in such cases.     

Atypical background somatic mutant frequencies at the HPRT locus in children and adults with Down syndrome

Sixty-six human enterovirus serotypes have been described using antibody neutralization, with antigenic variants defined within several serotypes. Despite the availability of sequence data for numerous enteroviruses, the molecular basis of serotype is unknown. Previous studies by others have identified four major phylogenetic groups within the human enteroviruses, but there has been no complete database of homologous sequences for all human enterovirus serotypes. We have determined the homologous partial VP2 sequences for the 12 prototype strains for which VP2 sequence was unavailable and for eight well-characterized antigenic variants. Phylogenetic analysis of all prototype strains produced four major groups, consistent with published enterovirus phylogenies. Many antigenic variants, however, failed to cluster with their respective prototype strains, suggesting that this portion of VP2 may be inappropriate for consistent molecular inference of serotype and for detailed study of enterovirus evolution.     

Caring for the child with Down syndrome

This article reviews the etiology, treatment, and prognosis of Down syndrome. Effects of Down syndrome on growth and development, specific physiologic manifestations, and implications for school-based practice are discussed.     

Verb use by individuals with Down syndrome

Production of grammatical and lexical verbs in narratives from 29 individuals with Down syndrome and 29 typically developing control subjects matched on linguistic level (Brown's Stages 3, 4, and 5) was examined. We addressed recent theories proposing that verbs are central to syntactic development (Tomasello & Merriman, 1995). Consistent with predictions from the child talk model (Chapman et al., 1992), the individuals with Down syndrome produced fewer lexical or grammatical verbs per utterance compared to the control group but produced a greater diversity of lexical verbs. The findings suggest that the well-documented syntactic deficits evidenced by individuals with Down syndrome may reflect difficulty in accessing verbs when constructing utterances. This difficulty may stem from deficits in auditory short-term memory.     

Specialty endodontics: diagnostic challenges, Part 2

Endodontic diagnosis and treatment can range from simple to extremely complex. But no matter how simple a case may seem, a thorough examination by the treating endodontist is imperative before any treatment is done. Taking the time to perform an examination in a consistent and methodical approach can rescue the patient from unnecessary treatment, and the treating dentist from potential liability. The patient may be frustrated from repeating many of the same tests performed by the referring dentist, but this situation can be resolved with clear communication from the general dentist and the endodontist as to the benefits of the endodontist's exam. With proper communication, patients will recognize that their general dentist and endodontist are concerned that they receive the best of care.     

Thoracic spinal stenosis: diagnostic and treatment challenges

Thoracic stenosis may be defined by a narrowing of the anteroposterior (AP) diameter of the thoracic spinal canal to < 10 mm. Primary thoracic stenosis, documented when myelography is carried beyond the thoracolumbar junction into the upper thoracic canal, is most frequently associated with lumbar stenosis, whereas secondary stenosis, attributed to endocrinopathies and systemic diseases, more typically involves the entire spinal canal. Recognition of the presence of primary or secondary thoracic stenosis and the entire extent of attendant disease in the adjacent cervical or lumbar regions is essential to proper surgical management. Nine cases of primary and one instance of secondary thoracic spinal stenosis were reviewed. Seven of nine patients with primary thoracic stenosis had accompanying lumbar involvement, whereas one patient with secondary stenosis attributed to acromegaly had cervical, thoracic, and lumbar stenosis.     

Pulmonary tuberculosis: diagnostic delay in Ghanaian adults

Pica (pica = magpie) is an eating disorder that is manifested by a craving for oral ingestion of a given substance that is unusual in kind (nonfood items) or quantity (food items). Pica has been described as a world wide phenomenon, but there are more frequent occurrences of selected substances among selected groups--especially young children and black pregnant and nonpregnant women in the southern part of the USA. In Central Europe and Germany this syndrome has not been described in the moderne literature. For this reason, we report a case of pica for starch associated with severe iron deficiency anemia in Germany. Iron deficiency anemia and--less often-potassium and zinc deficiency are the main complications of an excessive starch or clay ingestion, followed by gastrointestinal obstructions due to gastroliths or impaction. Additionally, naphtalene poisoning (in pica for toilet air-freshener blocks), phosphorus poisoning (in matches pica), mercury poisoning (in paper pica), and lead poisoning (in dried paint pica) have been described.     

Down syndrome: 1. Medical aspects

Each organ of a patient with the Down's Syndrome (trisomy 21) shows the pathology. One notices the specific features already with an infant. The life expectation of these children has increased considerably and it depends upon the appearance or not of a heart defect. The ventricular septum defect is most frequent but a small number of these patients show a complex cardiopathy. The incidence of pulmonary hypertension is also high. The obstruction of gastroenteric tract can cause problems from the prenatal phase onwards. The main endocrinological difficulties are dysfunction of the thyroid gland and also infertility. Ocular disorders like refraction disorders occur frequently. Due to decreased conduction, there is a hearing loss. The cellular immunity is clearly reduced, hence, the susceptibility to infections like hepatitis B, increases. The major oral problems are apparently oversized tongue and a high sensitivity to gingivitis.     

Down syndrome associated malformations

This paper reports the associated malformations and the clinical findings that were observed in 417 cytogenetically confirmed Down Syndrome patients. Among them congenital heart defects have occurred more frequently [75; 17.98%] than osteoarticular malformations [23; 5.52]; eye anomalies [22; 5.27%]; and gastroenterological malformations [n 16; 3.84%]. With regard to prognosis and treatment appropriate counselling has been given to Down Syndrome patients and their families.     

Depression and Down syndrome

Emotional and behavioural disorders are frequent complications of mental retardation that often go unrecognised or untreated. We describe a 13-year old girl with Down's syndrome and depressive illness who responded well to paroxetin. The importance of organizing comprehensive health provision for children with mental retardation in a way that focuses both psychiatric and physical illness is emphasised.     

A tumor profile in Down syndrome

For most groups of biological control agents the relationship between laboratory (physiological) host range and the host range in the field (ecological host range) has not been explored empirically. The objective of our study was to investigate this relationship using the North America gypsy moth, Lymantria dispar, as a model nontarget host for microsporidia from native North American Lepidoptera. The gypsy moth, L. dispar, a native of Europe, has been established in North America for nearly 130 years and presumably exposed to many species of microsporidia from sympatric native Lepidoptera. Nevertheless, microsporidia have never been observed in North American populations of L. dispar. We conducted traditional laboratory feeding experiments using microsporidia from 20 lepidopteran host species and 1 coleopteran host species against L. dispar. Microsporidia from 18 native hosts infected L. dispar larvae. Although some of the infections were not typical of infections in the indigenous natural hosts, mature spores were produced in most of these infections. Horizontal transmission experiments, based on exposure of uninfected L. dispar larvae to infected L. dispar larvae, demonstrated that the microsporidia were far more host specific than the direct feeding experiments suggested. Of the three microsporidian biotypes that were horizontally transmitted between the nontarget L. dispar larvae, all were transmitted at very low levels. The results of our experiments provide additional evidence that the ecological host specificity of terrestrial microsporidia is much narrower than the physiological host specificity. Our studies establish the validity of using nonindigenous insect species with long-term data sets on natural enemies associated with them as a tool for testing hypotheses about host specificity.     

Nutrient intake and obesity in prepubescent children with Down syndrome

OBJECTIVE: The aim of this study was to measure nutrient intake and body composition in prepubescent children with Down syndrome to understand dietary barriers involved in the prevention and treatment of obesity. DESIGN: Dietary intake was determined from parent-reported 3-day diet records for children with Down syndrome and control subjects. Energy intake was compared with energy expenditure measured by the doubly labeled water method. Body composition was determined by deuterium dilution, bioelectrical impedance analysis, and skinfold thickness measurements. SUBJECTS/SETTING: Ten prepubescent children with Down syndrome and 10 control subjects were recruited from the hospital community. The study was conducted in the Clinical Research Center of the University of Chicago Medical Center. MAIN OUTCOME MEASURES: Nutrient intakes were compared with the Recommended Dietary Allowances (RDAs) to estimate risk for nutrient deficiency. Fat-free mass values determined by bioelectrical impedance analysis and measurement of skinfold thicknesses were compared with values determined using the deuterium dilution method. STATISTICAL ANALYSES PERFORMED: Unpaired t tests were used for comparisons between subjects groups and the Wilcoxon signed-rank test was used for comparison of nutrient intakes with RDAs. RESULTS: The subjects with Down syndrome were significantly shorter (P < .01) than control subjects; however, body composition did not differ between the groups. Reported energy intake was lower in subjects with Down syndrome. In addition, several micronutrients were consumed, especially among nonobese subjects with Down syndrome, at less than 80% of the RDA. APPLICATIONS: To avoid lowering already inadequate intakes of several vitamins and minerals, we suggest that treatment of obesity in children with Down syndrome combine a balanced diet without energy restriction, vitamin and mineral supplementation, and increased physical activity.     

IGF-I levels in prepubertal and pubertal children with Down syndrome

Medical behavior for intensive examination after colo-rectal cancer screening was examined in comparison with that after stomach cancer screening. Examinees who were positive in colo-rectal or stomach cancer screening, provided by an Occupational Health Organization from April 1993 to March 1994, were monitored for their medical behavior through notifications from physicians. The main results were as follows: 1. The proportion of those with the notification from a physician after colo-rectal cancer screening was half of that after stomach cancer screening. 2. Among those notified the rate of those who undertook intensive examination by the end of 12 weeks after the screening was 79.7% for a colo-rectal site and 87.0% for the stomach. The time interval from the screening to the intensive examination for the colo-rectal site was significantly longer than that for stomach. 3. For colo-rectal cancer screening, the proportion of those with the notification was lower in females than in males, and in those aged less than 50 than in those aged 50 or older. There were no differences in the proportion between those screened at the work site and those at the occupational health service center, and between those positive in the fecal occult blood test and those negative in the test but positive in the health interview. These results indicated, assuming that the probability of the physicians' notification was unrelated to the type of examination, a smaller proportion of the examinees positive in the colo-rectal cancer screening consulted a physician for the intensive examination later than those positive in the stomach cancer screening. It is therefore necessary to make colo-rectal cancer screening effective to establish a collaborating system which effectively facilitates the medical behavior of the screening positives.     

Prenatal screening for Down syndrome

OBJECTIVE: To investigate the fetal angiotensin II type 1 receptor genotype in pre-eclampsia. DESIGN: Case-control study. POPULATION: Forty-one maternal-fetal pairs from pre-eclamptic pregnancies and 80 maternal-fetal pairs from normotensive pregnancies. METHODS: Maternal and fetal DNA was genotyped at three diallelic polymorphisms, at nucleotides 573, 1062, and 1166, in the coding exon of the angiotensin II type 1 receptor gene, and at a dinucleotide repeat polymorphism in its 3' flanking region. RESULTS: Allele and genotype frequencies at the four polymorphic regions investigated did not differ between pre-eclamptic and normotensive groups, in either fetal or maternal samples. Mothers heterozygous for the dinucleotide repeat allele designated A4 transmitted this allele to the fetus in 15 of 18 informative pre-eclamptic pregnancies and in eight of 26 normotensive pregnancies. This was greater than the expected probability in pre-eclamptic pregnancies (p=0.04) and less than expected in normotensive pregnancies (p<0.005). The 573T variant, which is in partial linkage disequilibrium with the A4 allele, showed a similar distortion of maternal-fetal transmission. CONCLUSION: Angiotensin II type 1 receptor gene expression in the fetus may contribute to the aetiology of pre-eclampsia. It is unclear whether susceptibility is conferred by the fetal genotype acting alone, or by allele sharing by mother and fetus. Possible mechanisms for the effect of the angiotensin II type 1 receptor gene are suggested by the association of the 573T variant with low levels of surface receptor expression on platelets. If receptor expression is similarly genetically determined in the placenta, responsiveness to angiotensin II may be affected, with the potential to influence placentation or placental prostaglandin secretion.