[Do behavioural problems existing at referral become worse in subsequent years? A study of young persons referred to the outpatient unit of the mental health service]. / Wordt het probleemgedrag bij aanmelding in de loop der jaren erger? Een onderzoek bij jeugdigen verwezen naar de ambulante ggz.

BACKGROUND: Although young persons with severe and complex emotional and behavioural problems are often referred to the outpatient unit of the mental health service, little information is available about whether these problems increase over the years. This information is urgently needed in order to ensure that the mental health service provides adequate care. AIM: To obtain more insight into any increase in young persons' emotional and behavioural problems that may occur over a period of six years following referral to an outpatient unit of the mental health service. METHOD: The nature, severity and complexity of the emotional and behavioural problems of 123 young persons (1999) and of 149 young persons (2005) at the time of the referral - as rated by their parents on the basis of the Child Behavior Checklist (CBCL) - were assessed; the young persons' records were also checked for background characteristics. RESULTS: Compared to 1999, the year 2005 saw a slight decrease in the severity of the problems existing at referral; social problems also declined significantly compared to 1999. Problems identified in the 2005 group often seemed less complex than in 1999. The severity of delinquent behaviour as measured on the Delinquent Behaviour Scale seems to have risen in the 12 to 18 age group in 2005, whereas the severity declined in the 4 to 11-year olds. CONCLUSION: Emotional and behavioural problems as reported by the parents at the time their children were referred to the mental health service do not increase.

Family contexts, parental behaviour, and personality profiles of children and adolescents with Prader-Willi, fragile-X, or Williams syndrome.

The personality profiles for youths with Prader-Willi, fragile-X, or Williams syndrome were compared to three matched groups attending regular schools. Using the California Child Q-Set (CCQ), both of the parents of the 39 children with Prader-Willi syndrome, 32 boys with fragile-X syndrome, 28 children with Williams syndrome, and children in the comparison groups provided independent personality descriptions in terms of the Big Five personality factors of Extraversion, Agreeableness, Conscientiousness, Emotional Stability, and Openness, along with Motor Activity and Irritability. Specific personality phenotypes for each of the three syndrome groups were found to be differentially related to parental behaviours (i.e. control and anger) and family contexts (i.e. experienced family stress, marital conflict, and parental consistency).

Problem behaviors and personality of children and adolescents with Prader-Willi syndrome.

OBJECTIVE: Compare behavioral and emotional problems of children and adolescents with Prader-Willi Syndrome (PWS) and clients consulting mental health centers (MHC) and related behavioral and emotional problems to the children's personality in the PWS group. METHODS: Participants were 39 children with PWS and 585 matched MHC clients. Child Behavior Checklist (CBCL) syndromes were related to the Big-Five personality factors measured with the California Child Q-sort (CCQ). RESULTS: Mean CBCL Total Problems scores were not different for the PWS and MHC groups, but differences were found for several of the CBCL subscales. Patterns of correlations among CBCL scales were similar in both groups, although coefficients were generally higher in the PWS group, indicating higher comorbidity or co-absence of CBCL syndromes in children and adolescents with PWS. Personality profiles were specific for internalizing and Externalizing problems of children and adolescents with PWS.

Mannose transporter of Escherichia coli. Backbone assignments and secondary structure of the IIA domain of the IIABMan subunit.

The mannose transporter of Escherichia coli consists of two transmembrane and one peripheral protein subunit. The complex acts by a mechanism which couples translocation of the substrate with substrate phosphorylation. The peripheral IIABMan is a homodimer. The IIABMan monomer itself contains two domains which are linked by an Ala-Pro-rich hinge and which are both transiently phosphorylated at histidyl residues. The IIA and IIB domains can be separated by limited proteolysis. The IIA domain has a dimer molecular mass of 2 x 14 kDa. Almost complete 1H, 13C, and 15N NMR assignments of the backbone resonances of IIAMan have been achieved using 3D and 4D double-and triple-resonance techniques. Secondary structure elements were derived from NOE data. The IIA domain consists of a central beta-sheet of four parallel and one antiparallel strand (strand order 5 4 3 1 2) with helices on both sides of the sheet. The active-site His-10 is located in a loop at the C-terminus of beta-strand 1. This loop and the loop after strand 3 are at the topological switch point of the sheet.

Synaptonemal complex studies in male mice treated with cyproterone acetate or with testosterone.

Male mice were injected either with cyproterone acetate (CyAc) or with testosterone enanthate (TE) separately or in combination with estradiol benzoate (E2B) from the neonatal period on or during adulthood. Neonatally treated animals were killed on d28 or d60, whereas all treated adults were killed on d60. Synaptonemal complexes (SCs) were analyzed under the electron microscope. Various SC anomalies were recorded. The frequency of SC alterations was higher in neonatally treated mice injected with E2B alone or in combination with CyAc (range 9.6-23.7%) than in those injected separately with TE or CyAc (range 2-3.9%). In treated neonates, combination of CyAc and E2B treatment increased the prevalence of synaptic impairments (range 11-23.5%). However, whenever TE was added to E2B treatment a significant reduction of these impairments was observed (range 11-2.4 and 23.7-9.7% respectively). In treated adult mice the prevalence of SC anomalies was low (range 2.4-3.1%) whatever hormone was injected. Furthermore, the combination of estradiol with CyAc had no increasing effect on the induction of synaptic alterations. In E2B-treated animals, those injected during the neonatal period were more susceptible than those treated in adulthood. In mice injected separately with TE or CyAc such a difference of sensitivity between neonates and adults was not noticeable. This work demonstrates that testosterone deficiency is partly responsible for E2B-induced SC alterations. However, inhibition of testosterone action at the level of testicular target cells, such as that resulting from administration of cyproterone acetate, is less harmful to the synaptic process of meiosis.

Increased sister chromatid exchanges and tumor markers in workers exposed to elemental chromium-, cobalt- and nickel-containing dusts.

Sister-chromatid exchange (SCE) in blood lymphocytes, serum tumors markers, carcinoembryonic antigen (CEA) and tissue polypeptide antigen (TPA), and urinary excretion of chromium, cobalt and nickel were determined in 26 male workers occupationally exposed to chromium, cobalt and nickel dust and in 25 controls matched for age and smoking habits. The differences in the urinary excretion of metals between exposed persons and controls were statistically significant. An analysis of variance on the SCE rank values revealed that both exposure status (exposed persons vs. controls) and smoking habits (smokers and former smokers vs. never smokers) had a statistically significant effect. For the tumor markers, the analysis of variance did not reveal a statistically significant difference between exposed persons and controls. However, CEA serum levels were significantly correlated not only with smoking habits but also with duration of exposure. As cobalt is only weakly mutagenic, these results suggest that the small amount of absorbed chromium and nickel may have been sufficient to induce SCE. The hypothesis that tumor markers may be increased in groups of subjects exposed to genotoxic substances deserves further study.

Synaptonemal complex alterations in X-irradiated and in oestrogen-treated mice: a comparative study.

Synaptonemal complexes (SCs) were analysed in male NMRI mice either X-irradiated or treated with oestradiol benzoate (E2B). Animals 30 days old underwent a single X-ray exposure of either 5, 7.5 or 10 Gy and were killed at different times after exposure, i.e., 24 h, 1, 4, 12 and 16 weeks. E2B was injected daily to adult mice from day 30 to day 60 or up to day 90 of age. Oestradiol was also administered during the neonatal period and animals were examined on days 28, 60 and 90 of age. Different SC alterations were found in X-irradiated and in E2B-treated mice. SC lesions were rare in oestrogen-treated adult mice. Among SC anomalies, asynapsis and fragmentation of SC were common lesions. However, the former was more frequent in E2B-treated mice, whereas the latter was more frequent in X-irradiated mice. Quadri- or multi-valents, bridges between bivalents, rings and loops were exclusively encountered in the latter, whereas heterotelomeric associations seemed to be specific in E2B-treated animals. The mechanisms of the different SC lesions are discussed.

Effect of estradiol treatment on male mice synaptonemal complexes: difference of sensitivity between neonates and adults.

The effect of estrogen on pachytene spermatocytes was studied with the assistance of the synaptonemal complex analysis under electron microscopy. Male NMRI mice were injected with estradiol benzoate from birth onwards and allotted to different groups according to the dose administered: 1) three injections of either 12.5 micrograms or 25 micrograms or 50 micrograms on d0, d5 and d10; 2) single injections of 50 micrograms either on d0 or on d5 or on d10; 3) double injections of 50 micrograms on d0 and d5; and 4) daily injection at the dose of 0.5 micrograms/g BW from d0 to d27. Animals were sacrificed on day 28, 60 and 90. Adult male mice were treated daily with E2B (0.5 micrograms/g BW) for one (from d30 to d60) or two months (from d30 up to d90) to test the age-related sensitivity to estrogen. A number of different SC anomalies were observed at each harvest time. Among all the anomalies, pairing failure (asynapsis) was predominant followed in decreasing order of importance by SC breakage (fragmentation of SCs), and heterotelomeric associations resulting either in quadrivalent-like figures or in trivalents. In E2B treated neonates the frequency of SC anomalies, which was less than 2% in controls, varied from 3.6 to 27% of pachytene cells regardless of the harvest time. In E2B treated adult mice, the SC anomalies were rare (< 4%), but significantly different from controls in which the frequency of SC aberrations did not exceed 1% of pachytene cells. The prevalence of anomalies appeared to be independent of the TW decrease. Our observations suggest that estrogens act indirectly on SCs. Different mechanisms of action are discussed.

Experimental diabetes impairs rat embryo development during the preimplantation period.

Congenital malformations and early fetal losses are still the main complications of diabetic pregnancy. Whether the diabetic state affects the early embryo development during the preimplantation period is not known. To understand better the early steps of embryo growth, we collected the embryonic structures from the uterine horns of pregnant diabetic rats on day 5 of pregnancy. Diabetes was induced by streptozotocin (50 mg/kg) injection, 7, 14 or 21 days before mating. The morphological analysis revealed a lower rate of blastocysts (72% of all structures) and an increased rate of morulae (19.5%) in diabetic rats, compared to control animals (86.7 and 7.9% respectively). Hence, diabetic rats had fewer blastocysts (5.5 +/- 2.9 per rat) and more morulae (1.5 +/- 1.7) than control animals (7.2 +/- 2.7 and 0.66 +/- 1.2 respectively). Moreover, blastocysts from diabetic rats had fewer nuclei (26.9 +/- 7.3 per blastocyst) than blastocysts from control animals (31 +/- 6.1). In another set of experiments, subdiabetogenic doses of streptozotocin were administered. In rats injected with 25 mg/kg, neither the glycaemia, nor the morphological aspects of the embryos, nor the number of blastocyst nuclei differed from the control animals. In the animals receiving 35 mg/kg, the glycaemia was increased to approximately twice the control group value. However, the embryonic morphology and the nuclei counting of the blastocysts were similar to those of the fully diabetic group injected with 50 mg of streptozotocin. These results show that experimentally induced diabetes, even of a rather mild degree, affects the embryo development during the preimplantation period. The recovered embryos appear less mature and less developed.(ABSTRACT TRUNCATED AT 250 WORDS)

Familial phaeochromocytoma: successful treatment with 131I-MIBG.

We report the case of a family in which the mother died of hypertensive encephalopathy following the relapse of a phaeochromocytoma. Two of her children are still alive. Both children had malignant phaeochromocytomas that have been treated by surgery and 131I-MIBG. The first child presented with phaeochromocytoma of the right suprarenal gland at the age of 7 years. Surgery was performed. At the age of 14 years, he developed a tumour of the left suprarenal gland and two pulmonary metastases demonstrated by 131I-MIBG. The three tumours were removed, but new lesions occurred. The boy then was treated with 200 mCi (7,400 MBq) of 131I-MIBG given twice, and is now free of disease more than 2 years after treatment. His sister presented at the age of 12 years with phaeochromocytoma of the left suprarenal gland, the only lesion recognized by 131I-MIBG. The tumour was removed, but 5 months later, she developed phaeochromocytoma in the right suprarenal gland. She was treated with 200 mCi (7,400 MBq) of 131I-MIBG and surgery was performed 6 months later. Histology of the suprarenal gland could not demonstrate the persistence of phaeochromocytoma cells. The child is now free of disease more than 2 years after treatment.

Acute non-immune haemolytic anaemia during infectious mononucleosis unmasking spherocytosis.

We report a case of acute haemolytic anaemia complicating infectious mononucleosis in a 19-year old male. There was no evidence for an immune haemolysis and red cell studies revealed a previously undiagnosed congenital spherocytosis. We discuss this rare presentation.

Response of human and rat small intestinal mucosa to oral administration of Saccharomyces boulardii.

To evaluate the response of the small intestinal mucosa to Saccharomyces boulardii (S.b.), a yeast widely used in some countries as an adjuvant drug with oral antimicrobial therapy, seven healthy adult volunteers were treated with high doses of lyophilized S.b. (250 mg four times per day) for 2 wk. A peroral jejunal suction biopsy was performed on days 0 and 15 of the study. Compared to the initial biopsy, histological examination of the posttrial biopsy revealed no morphological alteration nor change in villus height or crypt depth. After treatment, the specific activity (per U protein) of sucrase, lactase, and maltase was, respectively, increased by 82% (p less than 0.05) 77% (p less than 0.05), and 75% (p less than 0.05) over the basal activity of the enzymes measured on day 0, whereas mucosal protein content remained unchanged. Similar findings were found in the jejunum of adult rats treated for 5 days with either viable or killed S.b. cells. The changes in total enzyme activity (per jejunal segment) paralleled the changes in specific enzyme activity. In vitro assays on freshly prepared suspensions of S.b. (6.0 X 10(8) viable cells/ml) evidenced a high activity for sucrase (mean +/- SE: 8 364 +/- 1280 U X g X protein-1) but no maltase, neutral lactase, acid beta-galactosidase, or aminopeptidase activity. To determine whether treatment with S.b. could influence the incorporation rate of neutral lactase into the brush border membrane, 14-day-old sucklings treated either with saline or with S.b. were given intraperitoneally a dose of 20 microCi D-[1(14)C] glucosamine 3 hours before sacrifice.(ABSTRACT TRUNCATED AT 250 WORDS)

[S-100 protein in amniotic fluid of the ancencephalic fetus]. / La protéine S-100 dans le liquide amniotique de foetus anencéphales.

S-100 protein, essentially of astrocytic origin, is present in the amniotic fluid of anencephalic fetuses. Its detection reflects tissue necrosis and could be complementary for prenatal diagnosis.

Role of dietary iron in maturation of rat small intestine at weaning.

The weanling process is characterized by the transition from a liquid diet poor in iron (rat milk) to a solid diet high in iron (chow pellets). To examine the effects of iron content of the weanling diet on terminal maturation of rat small intestine, suckling pups, nursed by iron-sufficient mothers, were weaned by day 16 onto a solid basal diet that was either deficient [low-iron diet (LID): 0.5 mg iron/100 g solid] or high [high-iron diet (HID) controls: 30 mg iron/100 g solid] in iron. The animals were studied during or at the end of the 4th postnatal wk. By day 17 rats weaned onto the LID exhibited an initial rise in jejunal sucrase activity as did their controls, but the activity plateau of the enzyme was reduced to a level 60% of the controls. On day 28 iron-deprived rats were anemic and showed significant decreases (P less than 0.01 compared with HID rats) in the activity of jejunal sucrase (-57%), neutral lactase (-83%), and maltase (-46%), whereas villus height, crypt depth, mucosal mass parameters, ileal acid beta-galactosidase activity, mucosal protein, and DNA synthesis rates were equivalent in LID and HID groups. The concentration of the secretory component, a glycoprotein synthesized by the intestinal crypt cell, was markedly depressed (P less than 0.01 vs. controls) in the jejunum (-54%) and ileum (-79%) of iron-deprived rats. When D-[1-14C]glucosamine was injected intraperitoneally, incorporation of the label into jejunal and ileal brush-border proteins was two to three times lower for iron-deficient rats than for controls.(ABSTRACT TRUNCATED AT 250 WORDS)

Intestinal development in the suckling rat: effects of weaning, diet composition, and glucocorticoids on thymidine kinase activity and DNA synthesis.

The present study was designed to determine whether the ontogenic changes in the salvage pathway for DNA synthesis of suckling rat intestine could be causally related to physiologic events during the weaning period. The intestinal activity of soluble thymidine kinase, extremely low in young sucklings (d 11, 0.057 +/- 0.007 nmol X min-1 X g tissue-1), increased dramatically between d 18 and 22 postpartum and reached a plateau (19.8 +/- 0.5 nmol X min-1 X g tissue-1) at the fourth postnatal week. Rat pups prevented from weaning showed an initial rise in the enzyme synthesis by d 18 as did their littermates weaned onto an adult diet. Compared with 22-d-old rats weaned onto chow pellets (14.1 +/- 2.1 nmol X min-1 g tissue-1), thymidine kinase concentration was reduced by one-half in rats of the same age, fed on mother's milk alone (6.5 +/- 0.7 nmol X min-1 X g tissue-1) or on a liquid artificial diet either high in fat (mimicking rat milk, 7.4 +/- 0.6 nmol X min-1 X g tissue-1) or high in carbohydrate mimicking chow, 6.4 +/- 1.3 nmol X min-1 X g tissue-1). The relative proportions of fat and carbohydrate in the diet had little or not effect on growth rate, intestinal wt, and DNA content per centimeter. In a second experiment, 9-d-old sucklings were treated for four consecutive d with either saline or hydrocortisone hemisuccinate at doses assumed to be in the physiologic (0.5 mg/100 g body wt per day) and pharmacologic (2.0 mg/100 g body wt per day) range.(ABSTRACT TRUNCATED AT 250 WORDS)

Expression of an X-linked muscular dystrophy in a female due to translocation involving Xp21 and non-random inactivation of the normal X chromosome.

A young female was diagnosed as having X-linked muscular dystrophy of the Duchenne type. Chromosome studies, including trypsin-Giemsa banding, Quinacrine fluorescence, and nucleolus organizer region (NOR) silver staining revealed an X-autosome reciprocal translocation t(X;21) (p21;p12). Utilizing both [3H] thymidine autoradiography and the BrdU-Hoechst 33258-Giemsa technique, lymphocytes and fibroblasts were found to show a preferential inactivation of the normal X suggesting the presence of a single mutant gene on the translocated X. This patient is one of seven reported cases of an X-linked muscular dystrophy associated with an X-autosome translocation. In all seven cases the exchange point in the X chromosome is in band p21 at or near the site of the Duchenne gene.

Pseudohypoparathyroidism: inheritance and expression of deficient receptor-cyclase coupling protein activity.

Pseudohypoparathyroidism, Type I (PSP-I) is a familial disorder characterized by secondary hyperparathyroidism, resistance of urinary cyclic adenosine-3', 5'-monophosphate (cAMP) excretion to exogenous parathyroid hormone (PTH), and by effects upon other hormones, including thyrotrophin (TSH) hyperresponsiveness to thyroliberin (TRH). In the present study, 12 PSP-I patients in five families exhibited partial deficiency of receptor-cyclase coupling protein (N protein) in blood cells, in association with the skeletal findings of Albright's hereditary osteodystrophy. In one father and six mothers of PSP-I patients, deficient N protein activity was associated with normal urinary cAMP responses to PTH. In this group of seven parents, five had Albright's osteodystrophy, two exhibited secondary hyperparathyroidism, and two had TSH hyperresponsiveness to TRH. In a sixth family with none of the features of Albright's osteodystrophy, N protein deficiency did not correlate with urinary cAMP responsiveness to PTH. In this kindred, one mother with N protein deficiency, but normal urinary cAMP responsiveness to PTH had raised serum levels of immunoreactive PTH. We conclude that in the majority of families with PSP-I the urinary cAMP response to PTH is an inadequate indicator of the genetic defect. In such families, deficiency of N activity more consistently points to metabolic defects, including secondary hyperparathyroidism and TSH hyperresponsiveness, even when urinary cAMP responses are normal.

Ontogeny of cell proliferation and DNA synthesis in rat colon: role of glucocorticoids.

This study was undertaken to determine whether the rat colon exhibits ontogenic changes in epithelial cell proliferation and DNA synthesis during growth. DNA synthesis was measured at intervals after birth in four colonic segments by the incorporation rates of [3H]thymidine. The labeled crypt cell index was determined by radioautography. New findings from our study are that 1) in each colonic segment of suckling rats, [3H]thymidine incorporation rate overshot the adult levels (49-119%) with a peak occurring at day 14 postpartum, 2) between days 14 and 20, the incorporation rates declined sharply to adult values and remained thereafter unchanged until adulthood; during the same period, the labeled and mitotic index decreased, respectively, from 52 to 19% and from 3.58 to 1.43%, 3) the decrease in DNA synthesis and in cell proliferation rates was concomitant with an upsurge in plasma total corticosterone initiated on day 14, and 4) treatment of 10-day-old sucklings with physiological doses of hydrocortisone for 4 consecutive days significantly depressed (P less than 0.01) colonic DNA content and DNA synthesis rates to levels about 45-67% of the control values. These data indicate that growth of the colon may be under the control of glucocorticoid secretion at the weaning period.