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Biomedical subjects

F M Slijper

Publications and source records attributed to F M Slijper.

At least 37 records · Page 2Linked to original sources

Psychosocial effects of two years of human growth hormone treatment in Turner syndrome. The Dutch Working Group: Psychologists and Growth Hormone.

Thirty-eight girls with Turner syndrome were treated for 2 years with human growth hormone. Both parents and patients carried out assessments of the effects of treatment on various aspects of psychosocial functioning. The children used the Piers-Harris Self-Concept Scale and the Social Anxiety Scale for children, while parents were interviewed and filled in the Child Behavior Checklist. Both parents and children used the Therapy Evaluation Scale and the Silhouette Apperception Technique. Teachers completed the Teacher Rating Form. Results showed no significant changes with treatment in self-concept or social anxiety, or in behavioural problems, though immature behaviour was less common after the treatment period. Therapy was evaluated positively, and improvements were apparent in both social and emotional functioning; about a quarter of the patients became more independent, happier and more involved in social interactions. Unrealistic perceptions of present height and expectations of future height were expressed by a large proportion of both children and parents.

Anxiety↗

[Intellectual development of patients with phenylketonuria; 15-year national screening in The Netherlands].

OBJECTIVE: To evaluate the intellectual development of patients with phenylketonuria (PKU) or hyperphenylalaninaemia (HPA). DESIGN: Longitudinal follow-up study. SETTING: University Children's Hospitals of Amsterdam, Groningen, Nijmegen, and Rotterdam. METHODS: In September 1974 a nationwide neonatal screening for PKU started in the Netherlands. We obtained data on the intellectual development of 116 patients (101 classical PKU, 15 HPA), all detected and treated in the first 15 years of this screening. The children were tested at 6 ages with different instruments for the assessment of intellectual functions, and the scores were compared with the test norms. Performance and verbal capacities were determined as well as possible sex-related differences. RESULTS: Our data show that the levels of intellectual functioning of PKU and HPA patients do not differ from test norms until the age of 8 yr 6 months. In the older PKU patients there appears to be a trend towards lower IQ scores. We found no sex differences in the mental functioning of PKU patients. CONCLUSION: Continuation of this study is necessary in order to investigate possible negative effects of PKU on cognitive functioning, especially in the older age groups.

Child↗

Implicit memory tested in children during inhalation anaesthesia.

Memory for stimuli presented during inhalation anaesthesia was tested in 80 children undergoing eye surgery. Two groups were exposed, in a random double-blind study, to repeated neutral phrases including either the colour orange or green. A postoperative colouring task was used as a test of implicit memory to detect any preference for the colour named under anaesthesia. No colour preference attributable to implicit memory could be demonstrated. One patient had a distinct preference for the named colour. No patient remembered any intra-operative events.

Anesthesia, Inhalation↗

Evaluation of young women with congenital adrenal hyperplasia: a pilot study.

To evaluate the outcome of medical and surgical treatment of 10 young women with congenital adrenal hyperplasia (CAH), final height, weight, body proportions and body hair growth as well as aspect and functionality of the external genitalia were assessed. The final height was below-2 SD of the target height in 55%, though the body proportions were in the normal range. Only 3 patients were menstruating regularly. Scores for 'hormonal' body hair growth were in a higher range. Gynaecological examination revealed that in 5 out of 9 patients, the genitalia were functionally acceptable. This pilot study stresses that from birth into adulthood of female patients with CAH there is a need for a combined therapeutic approach by a paediatric endocrinologist, a surgeon and a psychologist. This will improve the physical and psychosocial well-being of these patients.

Adolescent↗

[Good things come in small packages? Psychosocial aspects of small stature].

Short stature is considered as a social disadvantage. This review deals with the findings of studies on cognitive and socio-emotional functioning in children with growth hormone deficiency, constitutional short stature, Turner syndrome, skeletal dysplasias and chronic illnesses. The consequences of short stature in adulthood are also described. The short term effect of growth hormone treatment on psychosocial functioning appears positive, but long term results have to be awaited before conclusions can be drawn.

Adult↗

Growth analysis up to final height and psychosocial adjustment of treated and untreated patients with precocious puberty.

Thirty-four patients (27 girls and 7 boys) with precocious puberty who had reached final height were examined in order to analyze their growth. Thirty patients were diagnosed as having idiopathic precocious puberty, while 4 patients had cerebral organic disorders. Twenty-two patients were treated with cyproterone acetate, 3 with medroxyprogesterone acetate, and 9 patients remained untreated. Growth data of these three groups showed no differences, and final height was not affected by treatment. In patients with precocious puberty, adult height was significantly more often in the lower percentiles (less than or equal to P25, corresponding to an SDS of -0.67) than expected. In both treated and untreated patients, any negative influence of the early onset of puberty on well-being in later life could not be established.

Adaptation, Psychological↗

[Intellectual development of phenylketonuria children treated early--a longitudinal study. 10 years' concomitant psychological study in The Netherlands].

Since 1st September 1974 almost all newborns in The Netherlands have been screened for phenylketonuria (PKU). Data collected between then and 1 October 1984 are presented, with respect to the intellectual functioning of 69 patients who received early treatment for PKU. Wherever possible, psychological testing was carried out at four key ages, namely 1.0 years, 2.0 years, 4.6 years, 6.6 years. The results of the tests revealed no differences in the mental levels of these patients and healthy children at any of the key ages. Also no sex differences at key ages 1 and 2 years related to this variable were found. The longitudinal course of the intellectual development in the first four and a half years was stable. The necessity for the continuation of screening and the collection of psychosocial data is discussed.

Child↗

The effect of treatment with an LH-RH agonist (Buserelin) on gonadal activity growth and bone maturation in children with central precocious puberty.

Twenty-five children (23 girls and 2 boys) with central precocious puberty were treated with the LH-RH agonist D-Ser (TBU)6-LHRH (1-9) EA (HOE 766, Buserelin) by daily subcutaneous injection for a period of 11-18 months. Eight girls and 2 boys previously treated with cyproterone acetate (CPA, 100-150 mg/m2 body surface per day) and the first seven newly diagnosed patients received 2 X 10 micrograms Buserelin/kg bodyweight per day for 1 week, followed by a maintenance therapy of 1 X 10 micrograms/kg per day. After an initial marked increase, oestrogen (E2) serum levels in girls and testosterone (T) values in boys decreased. After a treatment period of 6-20 weeks the patients received 2 X 20 micrograms Buserelin/kg per day for 1 week and thereafter a maintenance dosage of 20 micrograms/kg per day to obtain full suppression (i.e. E2 less than 50 pmol/l; T less than 1 nmol/l). The remaining eight patients started directly on 2 X 20 micrograms Buserelin/kg per day followed by 1 X 20 micrograms/kg per day. All eight girls with menarche before therapy had no further menses. In all girls there was a reduction of palpable breast tissue. Decrease of pubic hair development was observed in 3 girls, an increase was seen in 5 girls, whereas in the remaining 15 girls no change was observed. Both boys had a reduction of testicular volume and of pubic hair.(ABSTRACT TRUNCATED AT 250 WORDS)

Bone Development↗

[Pseudo- and central precocious puberty due to adrenogenital syndrome].

We describe three patients in whom at the age of 4-7 yrs the diagnosis adrenogenital syndrome (21-hydroxylase deficiency) was made. There was no salt-loss. Two boys presented with precocious pubic hair development and increased growth velocity and bone maturation. The third patient was a severely virilized girl raised as a boy until she was 4 yrs old. Following an extensive psychiatric evaluation, it was decided to raise her as a girl and feminoplasty was performed. Clinical and biochemical evidence of central precocious puberty was present in one boy at the time of diagnosis at age 7 and developed under hydrocortisone substitution therapy in the two other patients. The precocious puberty was treated in two patients with the anti-androgen cyproterone acetate. In one boy suppression of pituitary gonadotropin secretion was obtained by LH-RH analogue (Buserelin) treatment.

Adrenal Hyperplasia, Congenital↗