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J S van den Berg

Publications and source records attributed to J S van den Berg.

18 recordsLinked to original sources
PubMed2001

Type III collagen deficiency in a family with intracranial aneurysms.

We present a family with 2 female cousins with intracranial aneurysms and type III collagen deficiency. DNA analysis revealed no mutations in the COL3A1 gene, encoding type III collagen, and including the segment encoding the C-propeptide of type III collagen. The 2 patients with low type III collagen production and intracranial aneurysms had inherited different type III collagen alleles. The type III collagen deficiency in this family may results from defects during posttranslational modification or from an altered collagen metabolism.

Adult↗
PubMed1999

Neuroinvasion by human herpesvirus type 7 in a case of exanthem subitum with severe neurologic manifestations.

A 19-month-old girl presented with severe neurologic symptoms associated with exanthem subitum. Human herpesvirus type 7 (HHV-7) DNA was detected in the CSF and serum, and supported by serologic studies. The patient was diagnosed with encephalopathy due to an acute HHV-7 infection. Neuron-specific enolase in the CSF was strongly elevated during the acute stage of infection, suggesting that the encephalopathy was due to viral invasion of the brain.

Brain Diseases↗
PubMed1999

Type III collagen deficiency in saccular intracranial aneurysms. Defect in gene regulation?

BACKGROUND AND PURPOSE: We sought to determine whether there are mutations in the COL3A1 gene in patients with saccular intracranial aneurysms with a type III collagen deficiency and whether there is an association between a marker in the COL3A1 gene and saccular intracranial aneurysms. One of the heritable factors possibly involved in the pathogenesis of saccular intracranial aneurysms is a reduced production of type III collagen, demonstrated earlier by protein studies. METHODS: We analyzed the type III collagen gene in a group of 41 consecutive patients with an intracranial aneurysm, of whom 6 patients had shown a reduced production of type III collagen in cultured diploid fibroblasts from a skin biopsy. RESULTS: No mutations could be demonstrated in the COL3A1 gene, especially not in the globular N- and C-terminal regions. A null allele was excluded in 25 patients, including 1 patient with a decreased type III collagen production. No differences were found between 41 patients and 41 controls in allele frequencies of a DNA tandem repeat polymorphism located in the COL3A1 gene. CONCLUSIONS: It is concluded that the COL3A1 gene is not directly involved in the pathogenesis of most of intracranial aneurysms. The reduced type III collagen production in cultured fibroblasts found in some patients with an intracranial aneurysm is not explained by the present study and needs further exploration.

Alleles↗
PubMed1998

The role of type III collagen in spontaneous cervical arterial dissections.

A case-control study was carried out to investigate whether type III collagen deficiency plays a role in the pathogenesis of spontaneous cervical arterial dissections. In 16 patients with spontaneous cervical arterial dissections and in 41 healthy controls, protein analysis of type III collagen (ratio of type III/type I collagen) was performed. Furthermore, single-stranded conformation polymorphism/heteroduplex analysis was used to investigate the type III collagen gene in the 16 patients with spontaneous cervical dissections to detect mutations. The ratios of type III/type I collagen in the controls ranged from 5.5 to 19.8% (median, 10%). The ratios of type III/type I collagen in the patients with spontaneous cervical arterial dissections ranged from 3.2 to 17.9% (median, 9.3%). Two patients had a low ratio of type III/type I collagen (<5.5%). No abnormalities suggesting a mutation in the gene of type III collagen were demonstrated in any of the 16 patients. Our findings are in keeping with the hypothesis that a reduced production of type III collagen may contribute to the formation of spontaneous cervical arterial dissections in some patients. The absence of a responsible mutation indicates that the coding sequence of the type III collagen gene is not involved.

Adult↗
PubMed1998

Water and electrolyte intake and output in conditioned Thoroughbred horses transported by road.

The objectives of this trial were to measure the water and electrolyte intake and loss, of horses during road transportation in relatively hot environmental conditions. Six mature, Thoroughbred horses in full training were used in a balanced crossover design. The horses were conditioned on a treadmill for 6 weeks before the start of the trial in order to simulate the type of horse that is transported most often over long distances in South Africa. The horses were assigned randomly to one of 2 treatment groups. On a particular day 3 horses were transported, while the other horses served as controls. One month was allowed before the crossover. Horses in the 'transport group' were transported by tarred road in a circular route over 600 km. This allowed the standardisation of measurements and use of the same instruments in both groups. Data were collected in each treatment group during transportation that lasted for 8 h (transport phase) and for 6 h after travelling (recovery phase). The following data were collected or calculated: Water and electrolyte (sodium, potassium and chloride) intake and output, changes in bodyweight and feed consumption. Although water was always available, the transport group failed to drink during transit. Based on bodyweight, the transported horses were 3% dehydrated at the end of transit. This bodyweight loss was corrected within one hour after their return due to a significantly higher water intake compared to control horses. The feed intake in the transported horses was unaffected during travelling, but was decreased for 6 h following transportation. Urinary water loss was similar in the 2 treatment groups during and following transportation. The faecal water loss decreased in the transported horses and remained lower than the control group for 6 h following transit. Total sodium and chloride intake were unaffected, while the potassium intake was decreased during transportation. Sodium and potassium loss via the faeces and urine during the transport period were similar in the 2 treatment groups, whereas potassium output in the transport group was significantly decreased during the recovery period of the study. It was concluded that transportation by road affected the water and electrolyte balance of conditioned horses for a period up to 6 h after travelling.

Animals↗
PubMed1998

Treatment of heartwater: potential adverse effects of furosemide administration on certain homeostatic parameters in normal sheep.

Diuretics, in particular furosemide, are generally recommended as a supportive treatment in the advanced stages of heartwater in ruminants. However, after what appeared to be possible adverse effects accompanying its use in field cases of heartwater, the effects of this drug on certain blood and urine parameters were investigated in normal sheep at the same dose rates. Diuresis with concomitant natriuresis was significant after furosemide administration, as was the expected plasma volume decrease. Other significant changes included metabolic alkalosis, hypokalaemia and reduced blood ionised calcium. The difference in duration of the diuretic effect and the duration of the changes in blood parameters from c. 3 h and c. 6 h respectively make it difficult to determine a time interval between successive treatments with furosemide. It appears that the probable cause of death of sheep with heartwater is a drastic reduction in blood volume and decreased cardiac output that leads to general circulatory failure. A therapeutic approach that involves further loss of plasma volume due to diuresis appears contradictory. The added effects of potentiating respiratory alkalosis and the terminal drop in blood ionised calcium seen in heartwater-affected animals indicate that the use of furosemide in supportive treatment of this disease is not warranted.

Alkalosis↗
PubMed1997

Some patients with intracranial aneurysms have a reduced type III/type I collagen ratio. A case-control study.

A reduced production of type III collagen has been reported in previous studies to be associated with intracranial aneurysms. The purpose of this prospective case-control study was to assess the possible role of a reduced type III collagen production as a risk factor for having an intracranial aneurysm. The study group consisted of 41 consecutively admitted patients with intracranial aneurysms. Intracranial aneurysms were demonstrated by intraarterial digital subtraction cerebral angiography or during operation. The control group consisted of 41 healthy volunteers matched for age and sex. Fibroblasts were cultured from skin biopsies from patients and control subjects, and the type III/type I collagen ratios were determined. The type III/type I collagen ratios in the controls ranged from 5.5 to 19.8%, with a median ratio of 10%, and none had a ratio below 5.5%. The type III/type I collagen ratios in patients ranged from 1.1 to 25.1%, with a median ratio of 10.5%, and eight patients (19.5%) had a low (< 5.5%) ratio (p = 0.005, Fisher's exact test). Our findings support the hypothesis that a reduced production of type III collagen may contribute to the formation of intracranial aneurysms in some patients.

Adult↗
PubMed1997

The effect of diminazene aceturate on cholinesterase activity in dogs with canine babesiosis.

A clinical trial was designed to evaluate the effects of diminazene aceturate and its stabiliser antipyrine on serum pseudocholinesterase (PChE) and red blood cell acetylcholinesterase (RBC AChE) in dogs with babesiosis. The trial was conducted on naturally occurring, uncomplicated cases of babesiosis (n = 20) that were randomly allocated to groups receiving a standard therapeutic dose of diminazene aceturate with antipyrine stabiliser (n = 10) or antipyrine alone (n = 10). Blood was drawn immediately before and every 15 minutes for 1 hour after treatment. Plasma PChE showed a 4% decrease between 0 and 60 min within the treatment group (p < 0.05). No statistically significant differences were found between the treatment and control groups at any of the time intervals for PChE. There was an increase in RBC AChE activity at 15 min in the treatment group (p < 0.05). No significant differences were found between the treatment and control groups at any time interval for RBC AChE. In view of the difference in PChE, samples from additional, new cases (n = 10) of canine babesiosis were collected to identify the affect of the drug over 12 hours. No significant depression was identified over this time interval. The results suggests that the underlying mechanism in producing side-effects, when they do occur, is unlikely to be through cholinesterase depression.

Acetylcholinesterase↗
PubMed1996

Is Marfan syndrome associated with symptomatic intracranial aneurysms?

BACKGROUND AND PURPOSE: Marfan syndrome is a heritable disorder of connective tissue caused by a deficiency of the glycoprotein fibrillin. In several publications and neurological textbooks, a relationship between Marfan syndrome and intracranial aneurysms has been assumed. METHODS: The records of 135 patients classified as having Marfan syndrome who visited the Amsterdam Marfan clinic or were admitted to the departments of neurology and neurosurgery and the records of all patients with a subarachnoid hemorrhage or intracranial aneurysm who visited or were admitted to the departments of neurology and neurosurgery between January 1, 1982, and January 1, 1994, were retrieved. The literature was reviewed regarding Marfan syndrome and intracranial aneurysms. RESULTS: No patient visiting the Marfan clinic had a symptomatic intracranial aneurysm. No patient with Marfan syndrome had been admitted with a ruptured intracranial aneurysm at the departments of neurology or neurosurgery in this period, while during that period 826 patients with symptomatic intracranial aneurysms had been admitted. During follow-up of 129 of the 135 patients with Marfan syndrome (2850 retrospective patient observation years and 581 prospective patient observation years), none presented a symptomatic intracranial aneurysm. The suggested relationship between Marfan syndrome and intracranial aneurysms is based mainly on 10 case reports. However, the diagnosis of Marfan syndrome is doubtful in several of these reports. Several large studies of patients with Marfan syndrome did not mention a ruptured intracranial aneurysm as a clinical manifestation. CONCLUSIONS: We conclude that there is insufficient evidence to presume a relationship between symptomatic intracranial aneurysms and Marfan syndrome on the basis of currently available data.

Adolescent↗
PubMed1995

[Wilson's disease; diagnosis with the aid of magnetic resonance tomography].

In a 21-year-old woman with a smaller and sloppy handwriting, drooling especially when stooping, sporadic choking, clumsiness, and frequent stumbling, Wilson's disease was diagnosed. The medical history disclosed a short period of haemolytic anaemia with transient hepatic failure, and irregular menstruation periods with infertility. On examination there were no signs of liver or spleen enlargement. She was slow, had an expressionless face and mild dysarthria, and slight impairment of the coordination of the limbs. Magnetic resonance imaging of the brain showed bilateral hyperintensive lesions of the basal ganglia on T2W images. Zinc therapy induced a good biochemical response and there was also some clinical improvement. Linkage analysis within the family identified one other asymptomatic homozygotically affected sister. A diagnostic delay occurs frequently due to relative unfamiliarity with this rare disease and due to its variable clinical expression.

Adult↗
PubMed1995

Single and multiple dose pharmacokinetics of gentamicin administered intravenously and intramuscularly in adult conditioned thoroughbred mares.

The pharmacokinetics of gentamicin following single and multiple intravenous and intramuscular doses were compared in a two phase, randomised cross-over study in horses. Gentamicin was administered to 6 healthy, conditioned Thoroughbred mares at a dosage of 3.3 mg/kg body weight every 12 hours for 5 intravenous or intramuscular consecutive treatments. Equal numbers of horses were treated by either route during each phase. There was a wash-out period of 5 days between phases. During each phase serial blood samples were collected from each mare immediately before treatment and at 16 intervals following the first and fifth administrations. Blood samples were also collected immediately before treatment and at 30 and 60 minutes following doses 2 through to 4. Gentamicin plasma concentrations were determined by fluorescence polarisation immunoassay. Plasma gentamicin concentration versus time data for both single and multiple doses by either route was best described by a 2 compartmental open model with first order rate constants. A distribution half-life (T1/2 alpha) of 0.1 +/- 0.1 hours, terminal half-life (T1/2 beta) of 1.2 +/- 0.2 hours, mean residence time (MRT) of 1.4 +/- 0.1 hours and total body clearance (ClB) of 1.4 +/- 0.2 ml/kg/min were observed following multidose gentamicin intravenous administration. The volume of distribution at steady state (Vdss) was 117.6 +/- 10.8 ml/kg. No significant differences (P > 0.05) were observed for any of the parameters between single or multiple doses for either route of administration. Except for AUC, significant (P < or = 0.05) differences were observed between multiple intravenous and intramuscular treatments for all pharmacokinetic parameters determined.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗
PubMed1995

Pseudomonas spp. associated vegetative endocarditis in two horses.

This paper describes the case histories of two Thoroughbred horses, a 2-year-old colt in training and a 7-year-old broodmare, that were presented with histories of weight loss, exercise intolerance, intermittent fever, limb oedema and anaemia. Vegetative endocarditis of the mitral and tricuspid valves was diagnosed in the colt by means of echocardiography. Pseudomonas sp. endocarditis of the mitral valve was diagnosed in the mare using echocardiography and bacterial culture. The colt had secondary congestive heart failure and was euthanased on humane grounds. Pseudomonas cepacia was isolated from the vegetative lesions following the post mortem examination. The mare was sent home and treated with gentamicin at a dosage of 3.3 mg/kg intra-muscularly twice daily for ten days. Her condition improved and she returned to stud.

Animals↗
PubMed1993

Pulsed wave Doppler echocardiographic evaluation of intracardiac blood flow in normal sheep.

The blood flow velocities and flow patterns were evaluated in normal sheep across the four cardiac valves using pulsed wave spectral Doppler echocardiography. Merino ewes (n = 20) aged between 16 and 61 months with bodyweights ranging between 51 to 72 kg were evaluated. The sheep were fully conscious and required no sedation. The mean peak velocities for the left atrioventricular, right atrioventricular, aortic and pulmonary valves were 68.4, 51.9, 99.1 and 92.7 cm s-1, respectively. Additionally the wave forms were described and mean velocities and a variety of flow variables determined for each of these flow areas. The mean left atrioventricular valve E:A ratio was under 1 which differs from humans and dogs. Aortic and pulmonary flow variables were compared to each other.

Animals↗
PubMedDate not supplied

Prevalence of symptomatic intracranial aneurysm and ischaemic stroke in pseudoxanthoma elasticum.

BACKGROUND: Pseudoxanthoma elasticum (PXE) is an heritable connective tissue disorder with clinical manifestations of the ocular, dermal, and cardiovascular system. The purpose of this study was to investigate the prevalence of symptomatic intracranial aneurysms (IAs) and ischaemic stroke (IS) in PXE. METHODS: The records of 100 patients with PXE were retrieved. All patients were contacted and data on complications were collected. The literature was reviewed regarding PXE, ISs, and IAs. RESULTS: No patient with PXE had a symptomatic IA as presenting symptom. One patient presented with an IS. During follow-up of 94 of the 100 patients (mean follow-up 17.1 years, range 1-49 years), none presented a symptomatic IA (3,168 retrospective patient observation years and 1, 602 prospective patient observation years). Upper gastrointestinal haemorrhage during follow-up occurred in 17 patients, in 1 patient during aspirin use. One patient has IS as presenting symptom and a recurrence during follow-up, and 7 patients had IS during follow-up. All were caused by small-vessel disease. The relative risk of IS in PXE under 65 years compared with the general population was 3.6 (95% confidence interval 3.3-4.0). CONCLUSIONS: On the basis of the currently available data, an association between symptomatic IAs and PXE is unlikely. However, the incidence of IS, due to small-vessel disease, was increased. Antiplatelet therapy in patients with PXE may lead to a high incidence of upper gastrointestinal haemorrhages.

Adult↗