PubMed Health⌕ Search

Biomedical subjects

B Otten

Publications and source records attributed to B Otten.

At least 19 recordsLinked to original sources
PubMed2004

Hypovitaminosis D-related myopathy in immigrant teenagers.

INTRODUCTION: Vitamin D deficiency resulting in a limb-girdle muscle weakness was diagnosed in three veiled immigrant teenage girls. PATIENTS: Three girls had a progressive muscle weakness and pain during a period varying from 6 months to two years. On examination limb girdle muscle weakness, predominantly of the lower extremities, without other neurological abnormalities was found. Serum examination showed a decreased level of vitamin D and phosphate and an increased alkaline phosphatase, and in two girls decreased calcium and increased parathyroid hormone levels were found. After supplementation with vitamin D, the pain subsided and muscle strength increased within weeks. Serum examination of the female relatives revealed eight persons with hypovitaminosis D, without any complaints. CONCLUSIONS: Vitamin D deficiency can result in a limb-girdle myopathy in veiled immigrant teenagers in the Netherlands. Vitamin D supplementation leads to rapid recovery of the muscle strength. The female relatives of these patients should be examined too.

Adolescent↗
PubMed2001

Fluctuant, progressive hearing loss associated with Menière like vertigo in three patients with the Pendred syndrome.

OBJECTIVE: To evaluate vestibular and long-term audiometric findings in patients with Pendred syndrome. STUDY DESIGN: Retrospective analysis of long-term clinical data. SETTING: University hospital department. PATIENTS: Three patients with Pendred syndrome caused by a mutation in the SLC26A4 gene. METHODS: Perchlorate discharge test, mutation analysis of the SLC26A4 gene, MR imaging of temporal bones, vestibular function test (in two cases) and serial audiometry. A saturation hyperbola with onset age was fitted to the audiometric threshold-on-age data using a nonlinear regression method. The residues remaining after regression were analyzed in a correlation analysis to detect significant ipsilateral or contralateral cofluctuation. RESULTS: All three patients had a mutation in the SLC26A4 gene and bilateral enlarged vestibular aqueduct; two of them had a positive perchlorate discharge test but in one of two siblings this test was negative. Hearing loss was significantly progressive with significant ipsilateral and contralateral cofluctuation in all evaluable cases, combined with episodes of Menière like vertigo in two cases. The episodes of vertigo are as seen in Menière disease. One case had unilateral caloric areflexia and one had bilateral vestibular hyporeflexia, proven to be progressive in a repeat examination. CONCLUSIONS: Patients with Pendred syndrome may exhibit progressive and fluctuant hearing loss with episodes of vertigo.

Adult↗
PubMed2001

A computer study of biodegradable plates for internal fixation of mandibular angle fractures.

PURPOSE: This computer-based study was performed to determine the suitability of small biodegradable plate systems for mandibular angle fractures. MATERIALS AND METHODS: In a 3-dimensional computer model of the mandible, fracture mobility and plate strain were calculated for bite forces applied on 13 bite points on the dental arch. The angle fracture was fixed with 2 polylactide (PLA) midiplates or with 2 PLA maxiplates. The first plate was positioned buccally on the external oblique ridge. Two positions of the second plate were studied: halfway up the height of the mandible or on the lower border. Maximum fracture mobility was set at a limit of 150 microm to enable undisturbed fracture healing. Maximum plate strain was set at the yield strain of PLA. RESULTS: Fixation with the PLA maxiplates, with the second plate positioned halfway up the height of the mandible, resulted in fracture mobility below the set limit for all bite points. For the other PLA fixation strategies, fracture mobility exceeded the set limit. Fixation with the second plate positioned halfway up the height of the mandible generally resulted in less fracture mobility than with the plate positioned on the lower border. The yield strain of PLA was not exceeded in any of the fixation strategies. CONCLUSIONS: Based on the computer model, 2 PLA maxiplates are suitable for fixation of mandibular angle fractures. One plate should be positioned buccally on the external oblique ridge, and the other should be positioned halfway up the height of the mandible.

Absorbable Implants↗
PubMed2000

Rodent therapeutics.

Although the number of rodents kept as pets is increasing in the United States, much of the veterinary information that is known regarding their care is derived from laboratory animal medicine. This article describes individual species variation of medication delivery methods and the associated risks of antibiotic usage. A comprehensive formulary is also included.

Animals↗
PubMed1999

Patterns of cognitive functioning in school-aged children with Noonan syndrome associated with variability in phenotypic expression.

OBJECTIVE: To evaluate the cognitive profiles of children with Noonan syndrome (NS) and to relate these profiles to measures of overall clinical severity. STUDY DESIGN: Thirty-five children with NS between the ages of 7 and 18 years were tested on their intellectual, psychosocial, and academic functioning. The diagnosis of NS was established on the presence of a typical face, the characteristic heart defect, thorax deformity, short stature, affected first-degree relative(s), and cryptorchidism in male subjects. RESULTS: The total group of children with NS (n = 35) achieved significantly lower mean full-scale IQ, verbal IQ (VIQ), and performance IQ (PIQ) scores (between 85.9 and 89.3) than expected based on normative data. The individual full-scale IQ scores varied between 48 and 130. Because of this wide range of individual scores, the mean group values are not extremely informative. The mean full-scale IQ for the group with moderate NS (n = 19) is 90.8; for the children with severe NS (n = 16) the mean full-scale IQ is 80.6. The patterns of discrepancies between VIQ and PIQ are: (1) an extreme discrepancy between VIQ and PIQ is most likely to emerge in children with severe NS with (low) average intellectual abilities; (2) children with moderate NS are more likely to attain similarities in VIQ and PIQ scores; and (3) children with moderate NS demonstrate a particular pattern of discrepancy between VIQ and PIQ (ie, VIQ > PIQ). CONCLUSION: For children with NS, the findings on physical examination are indicative of the pattern of cognitive abilities. NS is not associated with substantial deficits in the level of intellectual functioning or with a single/unitary cognitive pattern. Severe NS expression, however, predicts in part a specific pattern of deficits and capacities in cognitive functioning.

Adolescent↗
PubMed1999

A computer study of fracture mobility and strain on biodegradable plates used for fixation of mandibular fractures.

PURPOSE: This computer-based study was done to determine whether a small biodegradable plate system was suitable for internal fixation of mandibular fractures. MATERIALS AND METHODS: In a three-dimensional computer model of the mandible, fracture mobility and plate strain were calculated for bite forces applied on 13 bite points on the dental arch. Simulated solitary angle, body, and symphysis fractures were fixed with one titanium miniplate, one polylactide (PLA) midiplate, one PLA maxiplate, or two PLA midiplates. Fractures with and without interfragmentary bone contact were studied. In the case of fractures with bone contact, the loads were transmitted through the fracture surfaces and the plate; when there was no contact, the loads were transmitted only through the plate. Maximum fracture mobility was set at 150 microm. Maximum plate strain was set at the yield strain of PLA and titanium. RESULTS: For fractures without interfragmentary bone contact, all plate fixations resulted in a fracture mobility and plate strain higher than the limits set, except for the symphysis fracture fixed with two PLA midiplates. Interfragmentary bone contact significantly reduced fracture mobility and plate strain. For the angle fracture with bone contact, all PLA plate fixations resulted in a fracture mobility above the limit, whereas the titanium miniplate fixation had a fracture mobility below the limit. For the body and symphysis fracture with bone contact, only double PLA midiplate fixation resulted in a fracture mobility below the limit. CONCLUSIONS: From a mechanical point of view, based on the computer model, small PLA plates are only suitable for symphysis fractures with and without interfragmentary bone contact and for body fractures with interfragmentary contact. However, fixation with two PLA plates is always necessary to provide sufficient reduction of fracture mobility and plate strain.

Absorbable Implants↗
PubMed1998

Two frequent missense mutations in Pendred syndrome.

Pendred syndrome is an autosomal recessive disorder characterized by early childhood deafness and goiter. A century after its recognition as a syndrome by Vaughan Pendred, the disease gene ( PDS ) was mapped to chromosome 7q22-q31.1 and, recently, found to encode a putative sulfate transporter. We performed mutation analysis of the PDS gene in patients from 14 Pendred families originating from seven countries and identified all mutations. The mutations include three single base deletions, one splice site mutation and 10 missense mutations. One missense mutation (L236P) was found in a homozygous state in two consanguineous families and in a heterozygous state in five additional non-consanguineous families. Another missense mutation (T416P) was found in a homozygous state in one family and in a heterozygous state in four families. Pendred patients in three non-consanguineous families were shown to be compound heterozygotes for L236P and T416P. In total, one or both of these mutations were found in nine of the 14 families analyzed. The identification of two frequent PDS mutations will facilitate the molecular diagnosis of Pendred syndrome.

Adolescent↗
PubMed1997

Growth before and during growth hormone treatment in children operated for craniopharyngioma.

Height and weight growth before and during treatment with human growth hormone (hGH) was studied in 46 Dutch patients treated for craniopharyngioma. Weight was expressed as body mass index (BMI, weight/height). At the time of tumor treatment mean +/- SD height standard deviation score (SDS) was -1.22 +/- 1.38 and BMI SDS was 0.56 +/- 1.32. The initial height SDS was inversely related to age (r = -0.38; p < 0.02). Before hGH treatment height SDS decreased to - 1.57 +/- 1.08 (p < 0.05) and BMI SDS increased to 1.54 +/- 1.58 (p < 0.005) during the first year after tumor treatment. Changes in height SDS correlated positively with basal prolactin (PRL) levels (r = 0.46; p < 0.05). Neither tumor localization nor treatment mode was related to changes in height SDS and BMI SDS. Forty patients were treated with hGH, started a median interval of 2.0 years after tumor treatment. At the time of the start of hGH treatment height SDS in these patients was -1.70 +/- 1.13, and BMI SDS was 1.44 +/- 1.79. During treatment with hGH, height SDS increased to -1.05 +/- 1.10 (p < 0.001) in the first, and to -0.80 +/- 1.04 (p < 0.001) in the second year. BMI SDS did not change during hGH therapy. In conclusion, there is a large variation in height SDS and BMI SDS at the time of initial presentation as well as during spontaneous growth after tumor treatment. Spontaneous growth is related to serum PRL concentrations. Treatment with hGH significantly increased height SDS during the first 2 years, whereas BMI SDS did not change.

Adolescent↗
PubMed1997

GH and TSH deficiency.

Hypothyroidism is a recognised complication of GH therapy in GH deficient children. The mechanisms involved include direct effects on thyroid function but also result from the close interrelationship of pituitary cell-lines that differentiate during embryonic development of the anterior pituitary gland. Among numerous pituitary transcriptionfactors that orchestrate pituitary organogenesis Pit-1 was the first to be recognised and is the most extensively studied. Mutations in the Pit-1 gene account for a form of combined pituitary hormone deficiency for GH, Prolactin (Prl) and TSH (CPHD). Despite the variability of the clinical presentation of this syndrome at the time of initial diagnosis, all forms finally result in severe retardation of growth and development due to GH-deficiency and hypothyroidism. More than half of the families with a combined pituitary hormone deficiency have not disclosed any Pit-1 abnormalities. Evidence is accumulating that Prop-1, a transcriptionfactor expressed temporarily in the fetal anterior pituitary, could be a candidate for patients with a Pit-1 phenotype without any Pit-1 gene abnormalities.

DNA-Binding Proteins↗
PubMed1996

A regression method including chronological and bone age for predicting final height in Turner's syndrome, with a comparison of existing methods.

A total of 235 measurement points of 57 Dutch women with Turner's syndrome (TS), including women with spontaneous menarche and oestrogen treatment, served to develop a new Turner-specific final height (FH) prediction method (PTS). Analogous to the Tanner and Whitehouse mark 2 method (TW) for normal children, smoothed regression coefficients are tabulated for PTS for height (H), chronological age (CA) and bone age (BA), both TW RUS and Greulich and Pyle (GP). Comparison between all methods on 40 measurement points of 21 Danish TS women showed small mean prediction errors (predicted minus observed FH) and corresponding standard deviation (ESD) of both PTSRUS and PTSGP, in particular at the "younger" ages. Comparison between existing methods on the Dutch data indicated a tendency to overpredict FH. Before the CA of 9 years the mean prediction errors of the Bayley and Pinneau and TW methods were markedly higher compared with the other methods. Overall, the simplest methods--projected height (PAH) and its modification (mPAH)--were remarkably good at most ages. Although the validity of PTSRUS and PTSGP remains to be tested below the age of 6 years, both gave small mean prediction errors and a high accuracy. FH prediction in TS is important in the consideration of growth-promoting therapy or in the evaluation of its effects.

Adolescent↗
PubMed1996

Final height in central precocious puberty after long term treatment with a slow release GnRH agonist.

OBJECTIVE: To study the resumption of puberty and the final height achieved in children with central precocious puberty (CPP) treated with the GnRH agonist triptorelin. PATIENTS: 31 girls and five boys with CPP who were treated with triptorelin 3.75 mg intramuscularly every four weeks. Girls were treated for a mean (SD) of 3.4 (1.0) years and were followed up for 4.0 (1.2) years after the treatment was stopped. RESULTS: The rate of bone maturation decreased during treatment and the predicted adult height increased from 158.2 (7.4) cm to 163.9 (7.5) cm at the end of treatment (p < 0.001). When treatment was stopped bone maturation accelerated, resulting in a final height of 161.6 (7.0) cm, which was higher than the predicted adult height at the start of treatment (p < 0.001). Height at the start of treatment was the most important factor positively influencing final height (r = 0.75, p < 0.001). Bone age at cessation of treatment negatively influenced final height (r = -0.52, p = 0.03). A negative correlation between bone age and height increment after discontinuation of treatment was observed (r = -0.85, p = 0.001). Residual growth capacity was optimal when bone age on cessation of treatment was 12 to 12.5 years. Body mass index increased during treatment and remained high on cessation. At final height, the ratio of sitting height to subischial leg length was normal. Menarche occurred at 12.3 (1.1) years, and at a median (range) of 1.1 (0.4 to 2.6) years after treatment was stopped. The ovaries were normal on pelvic ultrasonography. CONCLUSIONS: Treatment of CPP with triptorelin increases final height, with normal body proportions, and seems to increase body mass index. The best results were achieved in girls who were taller at the start of treatment. Puberty was resumed after treatment, without the occurrence of polycystic ovaries.

Body Height↗
PubMed1996

Pit-1: clinical aspects.

Pit-1 is a transcription factor which is expressed in the somatotrope, lactotrope, and thyrotrope cell population of the anterior pituitary gland from early fetal development throughout life. Mutations in the Pit-1 gene result in insufficient expression of this factor, accounting for a form of combined pituitary hormone deficiency for growth hormone (GH), prolactin, and thyroid-stimulating hormone. Clinical presentation at diagnosis can be variable, although all forms finally result in severe growth retardation due to GH deficiency and hypothyroidism. The clinical variability is due to other factors than the exact location of the mutation; however, the type of inheritance seems to correlate well with the genotype. Early detection of Pit-1 abnormalities might prevent the sequelae associated with some early and severe presentations of this disorder.

Animals↗
PubMed1993

Antibody deficiency and isolated growth hormone deficiency in a girl with Mulibrey nanism.

A combination of humoral immunodeficiency and isolated growth hormone deficiency was observed in a girl with Mulibrey nanism. The humoral immunodeficiency consisted of subnormal concentration of serum IgG, in particular IgG2 and IgG4, and low concentration of serum IgM. Serum IgA and IgD were elevated, IgE was absent. Antibody response in vivo was very low or absent and opsonization in vitro was defective. Total B-cell number was low. In addition, the serum kappa/lambda light chain ratios within the immunoglobulin classes G, A, and M were abnormal. The defective antibody response may be linked to the abnormal kappa/lambda light chain ratios. Endocrine functions were normal except for isolated growth hormone deficiency. Therapy with human growth hormone resulted in increased growth velocity but did not improve humoral immune functions.

Child, Preschool↗
PubMed1983

[A new catheter set for continuous axillary plexus anesthesia].

A new device for continuous axillary plexus block is described. Using the "loss-of-resistance"-method as in peridural anesthesia it allows a safe introduction of a catheter within the neurovascular axillar sheath. Danger of puncture of blood vessels or of neural injury is minimized by a specially developed FEP-teflon-cannula. The main field of application for this device is seen in the management of pain postoperatively, mobilisation of contracted joints as well as in sympathetic block for peripheral vascular disease of various etiology. Furthermore, its allows the anaesthesia of the axillary plexus for operations of long duration (i.e. plastic surgery).

Axilla↗
PubMed1981

[Peridural morphine-analgesia. II. Respiratory depression (author's transl)].

In nine healthy young patients ventilatory CO2-response curves and mouth-occlusion pressure were measured after peridural injection of 5 mg morphine hydrochloride diluted in 10 ml saline solution in the lumbar region. Patients were not premedicated. Prior to measurements lower extremity operations were performed under peridural anaesthesia. Five minutes after peridural morphine injection there was a slight reduction in ventilatory response to increasing CO2 (94.7% +!- 15%), which was even more pronounced one hour later (74.5% +/- 14.7%). Mouth occlusion pressure decreased to 86.7% +/- 27.4% after 5 min to 72.4% +/- 13.4% after 60 min. Changes in the relation of the central stimulus P100 to the resulting flow (VT/Ti) was 96.7% +/- 32.5% after 5 min and 95.0% +/- 31.4% after 60 min. These results demonstrate a central respiratory depression after peridural morphine injection with a consequent reduction in ventilatory CO2-response.

Adult↗
PubMed1981

[Epidural morphine analgesia. Postoperative period (author's transl)].

50 patients after abdominal surgery received peridural morphine for postoperative pain relief. At the end of operation, 2-3 mg morphine in 10-15 ml 0,9% saline were injected peridurally by a catheter, which was placed preoperatively. The injection was followed by a dissociated analgesia. Diffuse pain and wound pain were diminished. Pin prick and temperature were still perceived. Analgesia started within 2-5 min after injection and lasted 14 h in the mean (range 1-49 h). The degree of analgesia was determined by a visual analogue scoring system. The patients reported a marked pain relief. A new dose of 2 mg morphine was given on demand, when the patients felt pain again. This procedure was continued until the second postoperative day. The overall amount of morphine by peridural application was 8 mg within 3 days. Neither clinically nor by blood gas analyzes here were signs of respiratory depression. There were no severe side effects. The advantages of epidural morphine analgesia are: low dose, long duration, lack of sedation and motor block, lack of respiratory depression, tachyphylaxia and sympathetic block. No pneumonia were seen in the epidural morphine group, whereas 4 cases of pneumonia were seen in the control group (n = 50) with conventional analgetics.

Abdomen↗