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Biomedical subjects

H Verhelst

Publications and source records attributed to H Verhelst.

10 recordsLinked to original sources
PubMed2003

Peliosis hepatis presenting as a multifocal hepatic pseudotumor: MR findings in two cases.

Two cases of pseudotumoral peliosis hepatis are presented with emphasis on MRI findings. One patient had four individual lesions, the other had two. Image characteristics in both were: heterogeneic signal intensity on T1-weighted images (T1WI) with areas of high-, intermediate, and low signal intensity; heterogeneic signal intensity on T2WI with presence of numerous intralesional "cystic" hyperintense areas with a hypointense border. Signal intensities on T1WI were iso- to hypointense in one case and mild central hyperintensities were demonstrated in the other case, probably due to intra-lesional hemorrhage or diffuse accumulation of fresh clotting within the sinusoids. One case demonstrated early enhancement of the peripheral borders in the arterial phase, and both demonstrated enhancement in the portovenous and late phases. One case was studied with Gd-BOPTA and iron oxides and demonstrated enhancement with both products, suggestive for the presence of hepatocytes and Kupffer cells. This is the first report of the use of hepato-specific contrast agents in this entity. Spontaneous regression of the lesions was demonstrated on a follow-up MR examination in one case.

Aged↗
PubMed2003

Anti-epileptogenesis research: the clinical relevance.

In recent years, different research lines have examined the epileptogenic process in order to understand the different stages in this process, and with the hope that early recognition and intervention could prevent chronic epilepsy in patients with epileptic seizures. In animals, acquired epilepsy is studied most commonly with kindling models, status epilepticus models and traumatic brain injury models. Molecular genetic studies substantially help to understand age-specific channel and receptor abnormalities. Major progress has been made in recent years and we are now waiting for the first large scale multi-center clinical trials that test the possible anti-epileptogenic properties of anti-epileptic drugs or other compounds in well defined patient groups. In clinical practice, a structured diagnostic work-up in all patients with recurrent seizures is a first and necessary step in the recognition of patients at risk for developing chronic and refractory epilepsy.

Animals↗
PubMed2001

A new mitochondrial point mutation in the transfer RNA(Leu) gene in a patient with a clinical phenotype resembling Kearns-Sayre syndrome.

OBJECTIVE: To report on the molecular identification of a novel heteroplasmic G-to-A transition at mitochondrial DNA position 3249 in transfer RNA(Leu) gene in a patient with a clinical phenotype resembling Kearns-Sayre syndrome. PATIENT AND METHODS: A 34-year-old patient had been suffering for more than 10 years from progressive visual failure, neurosensorial hearing loss, exercise intolerance, muscle weakness, paresthesia in the lower limbs, and difficulties swallowing. Clinical examination revealed generalized muscle wasting, ptosis, external ophthalmoplegia, and ataxia. Ophthalmologic examination showed dystrophic features in the cornea and retina. In skeletal muscle, morphologic and biochemical studies of the respiratory chain complexes were performed. Polymerase chain reaction, single-strand conformation polymorphism, and direct sequencing were used to screen for mutations in the 22 mitochondrial transfer RNA genes. RESULTS: In skeletal muscle, a significantly decreased catalytic activity of complex I was detected by spectrophotometric analysis and numerous cytochrome c oxidase-negative ragged-red fibers were seen on morphologic examination. A G-to-A substitution 3249 (G3249A) mutation was found in the transfer RNA(Leu) gene of the patient and mutant mitochondrial DNA represented 85% of the total in skeletal muscle but only 45% in leukocytes. The mutation was shown to be present in a small fraction in leukocytes from the unaffected mother and to be absent in leukocytes from the healthy sister. CONCLUSIONS: A causal relationship between a heteroplasmic G3249A transfer RNA(Leu) mutation in a patient suffering from progressive external ophthalmoplegia, retinal dystrophy, ataxia, neurosensorial hearing loss, and muscle wasting is postulated. To our knowledge, the G3249A mutation has never previously been described and was not detected in control subjects.

Adult↗
PubMed2001

Blue native polyacrylamide gel electrophoresis: a powerful tool in diagnosis of oxidative phosphorylation defects.

Catalytic activity of oxidative phosphorylation complexes is maintained following separation by Blue Native polyacrylamide gel electrophoresis (BN-PAGE). In BN-PAGE gels, using histochemical staining methods, we have demonstrated enzymatic activity of the complexes I, II, IV, and V in heart and skeletal muscle, liver, and cultured skin fibroblasts. The combination of BN-PAGE and catalytic staining can be successfully applied for detection of complex deficiencies. Tissues from 18 patients with deficiency in the oxidative phosphorylation as detected by spectrophotometric assay were used (10 patients complex IV, three patients complex I, one patient complex II, one patient complex I+III, three patients complex I+IV). The gene defect was located in nuclear DNA in five patients and mitochondrial DNA in one patient. In samples from patients with a severe deficiency, almost complete absence of the corresponding enzyme band is observed after catalytic staining in the gel. In patients with known partial deficiency, a milder decrease of the corresponding enzyme band is demonstrated. The amount of protein in complexes I, V, and III can easily be evaluated in samples from heart and skeletal muscle after separation by BN-PAGE using silver or Coomassie staining. The protein amount in complex IV is difficult to visualize by silver staining but easier by the Coomassie technique. In samples from liver and cultured skin fibroblasts, evaluation of protein amount is more difficult due to high background staining. In these tissues, immunoblotting can be done after BN-PAGE and subsequent transfer to a nitrocellulose membrane.

Adolescent↗
PubMed2001

Retrospective study of topiramate in a paediatric population with intractable epilepsy showing promising effects in the West syndrome patients.

Topiramate (TPM) is a new anti-epileptic drug with proven efficacy against partial seizures in adults. Its use in children is less well documented. In a retrospective study, 41 patients with intractable childhood epilepsy were treated with TPM as add-on therapy for an average period of 15 months. They were classified according to seizure type and etiology. The dose was titrated for effect and ranged between 2 and 24 mg/kg/d. Of the 41 patients being treated, six became seizure free, ten had a seizure reduction of more than 75% and eight a seizure reduction of between 50 and 75%. The most remarkable effect was seen in seven patients with West syndrome. Of these, four patients became seizure free and one had more than 75% seizure reduction. Adverse effects including sedation, fatigue, difficulties with verbal expression and anorexia were noted in 15 patients. None of these effects were important enough to interrupt treatment. We conclude that TPM as adjunctive therapy is a promising drug in children with intractable epilepsy, especially in the patients with West syndrome.

Adolescent↗
PubMed1999

Fibromuscular dysplasia of the external iliac artery.

A 47-year-old female with fibromuscular dysplasia of both external iliac arteries was treated by percutaneous balloon catheter angioplasty. The immediate result was unsatisfactory. On discharge from hospital, the patient was only able to walk one kilometer, and had reduced ankle:arm blood pressure indices. Conservative treatment with anticoagulants for three months, followed by ticlopidine, and exercise for six months led to complete recovery.

Angioplasty, Balloon↗
PubMed1998

The use of extracorporeal membrane oxygenation in postpneumonectomy pulmonary oedema.

A 59-year-old male developed a severe adult respiratory distress syndrome following a right pneumonectomy for pulmonary cancer. Extracorporeal membrane oxygenation for 11 days was life-saving. The operation was considered curative, but the patient died nine months later with multiple metastases. The pathogenesis and treatment for postpneumonectomy pulmonary oedema and an explanation for rapid dissemination of the cancer are stated.

Adenocarcinoma↗
PubMedDate not supplied

Midgut volvulus in an adult patient.

The authors report on a case of midgut volvulus in a 27-year-old man who presented with bilious vomiting and acute abdominal pain. US demonstrated a reversal of the normal relationship between the superior mesenteric artery (SMA) and superior mesenteric vein (SMV). A clockwise whirlpool sign, diagnostic for midgut volvulus, was not visualised. In a further assessment, upper gastrointestinal series demonstrated obstruction in the second part of the duodenum highly suspicious of Ladd's bands. Malpositioning of bowel structures, as already suggested by the reversal of the SMA and SMV on ultrasound, and a distinctive whirl pattern due to the bowel wrapping around the SMA was demonstrated on CT. Furthermore angiography revealed focal twisting of the SMA. US is the first imaging modality to perform in suspicion of midgut volvulus. When inconclusive, CT is in our opinion the next stage in the diagnostic work-up.

Abdomen, Acute↗
PubMedDate not supplied

Cystic insulinoma of the pancreas in a patient with myotonic dystrophy: correlation of imaging and pathologic findings.

We present a case of cystic islet cell tumor of the pancreas in a 60-year old male with myotonic dystrophy. A cystic lesion was found with a thick peripheral rim, a few intralesional septae and a fluid-debris level on CT and MRI. Image characteristics are compared with the pathological findings. MRI demonstrated the internal architecture of the lesion more clearly.

Humans↗