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Biomedical subjects

M Boel

Publications and source records attributed to M Boel.

13 recordsLinked to original sources
PubMed2003

Spirometry in young children: should computer-animation programs be used during testing?

Currently, computer-animation programs are frequently used to instruct and stimulate young children in performing maximal expiratory flow/volume (MEFV) curves. The reproducibility and maximal performance of MEFV manoeuvres with and without the use of two computer-animation programs (the "candles" and the "balloon" programs) were evaluated. Eighty-eight children, aged 4-8 yrs, were randomly assigned to one of the two animation programs. All children performed two series of at least three technically acceptable curves, one series with the incentive and one without, in random order. With the use of computer-animation programs, a lower proportion of children were able to fulfil international criteria for forced vital capacity (FVC) and forced expiratory volume in one second (FEV1) reproducibility. The use of incentives improved reproducibility and performance of peak expiratory flow (PEF). Performance of FVC decreased significantly in 6-8-yr-old children using the animation programs. Training with a program for a short period of time before the formal lung-function test may be valuable. According to the results, however, the use of these programs during tests under the guidance of an experienced lung-function technician cannot be routinely recommended because of possible deteriorating effects on reproducibility and performance of forced expiratory manoeuvres.

Child↗
PubMed1996

Add-on therapy of fenfluramine in intractable self-induced epilepsy.

Eleven institutionalized children (7 girls, 4 boys) with refractory epilepsy received fenfluramine as add-on therapy. Besides therapy resistance, the patients manifested self-induction of seizures. Eight patients were photosensitive and/or pattern sensitive. All antiepileptic drugs of doubtful efficacy were withdrawn. During withdrawal, patients did neither improve nor show an increase in seizure frequency. Fenfluramine was then added and administered once to three times daily at dosages between 0.5 and 1 mg/kg.day. Side effects were mild and transient. Of the 11 patients, 7 became seizure-free and in 4 patients a more than 75% decrease of all seizures was obtained.

Adolescent↗
PubMed1989

Continuous spikes and waves during slow sleep: a 30 months follow-up study of neuropsychological recovery and EEG findings.

An eight-year-old boy is reported who presented with a progressive mental deterioration in the years following a nocturnal asymmetrical generalized tonic-clonic seizure. The diagnosis of continuous spikes and waves during slow sleep (CSWS) was made. Once a month a sleep recording was made during twenty-two consecutive months and detailed neuropsychological studies were made over a period of 30 months. Intensive antiepileptic treatment resulted in the disappearance of the CSWS and in a recovery from intellectual-, language- and behavioural disturbances. The good results were still present at a follow-up examination 30 months later.

Child↗
PubMed1988

Familial periodic ataxia responsive to flunarizine.

A ten-year-old boy is reported who presented with periodic ataxia. The diagnosis is based on family history and on the observation of an evoked paroxysm. The differential diagnosis is discussed and successful treatment with flunarizine is described.

Child↗
PubMed1985

Variability of outcome in Joubert syndrome.

Two children with Joubert syndrome are reported. Patient one is the first case with Joubert syndrome where CT-findings are confirmed by autopsy. Until now only three cases with necropsy findings were reported. Patient two shows a remarkable clinical outcome not previously mentioned.

Cerebellum↗
PubMed1985

Schinzel acrocallosal syndrome: a variant example of the Greig syndrome?

A 5-month-old male is reported with clinical and radiological findings identical to those present in the Schinzel acrocallosal syndrome. The similarity with the Greig syndrome is discussed and the question is raised whether both syndromes are variant examples of the same autosomal dominant condition.

Abnormalities, Multiple↗
PubMed1984

Paroxysmal kinesigenic choreoathetosis.

A twelve and a half-year-old boy is reported who presented with paroxysmal kinesigenic choreoathetosis. Family history lead to the diagnosis. The differential diagnosis to paroxysmal dystonic choreoathetosis of Mount and Reback (1940) is discussed, and treatment is commented upon.

Athetosis↗
PubMed1981

Ultrastructural abnormalities of bronchial cilia in children with recurrent airway infections and bronchiectasis.

Anomalies of the bronchial cilia were studied in 5 children with recurrent pulmonary infections. Case 1 had Kartagener's syndrome and an absence of the inner and outer dynein arms in most cilia, although a few shortened and even some normal arms could be seen. Cases 2 and 3 had unilateral bronchiectasis without family history of Kartagener's syndrome. Serial studies of the bronchial epithelium at times showed a bilateral lack of the inner dynein arms and a partial lack of outer arms. These abnormalities persisted in these 2 children after they had recovered from the acute pulmonary infection but disappeared after 6-8 months of antibiotic treatment. Cases 4 and 5 had recurrent pulmonary infections without bronchiectasis and many shortened outer dynein arms could be seen, but these anomalies disappeared after recovery. In all 5 children such architectural ciliary anomalies were present as megacilia, fused cilia, naked cilia, and completely disorganised axonemas. These architectural defects were particularly numerous in the children without bronchiectasis. Our observations suggest that anomalies of the bronchial ciliary microtubular system may not only be congenital but may also be acquired; this might well help to explain some cases of repeated respiratory tract infection and bronchiectasis.

Adolescent↗
PubMed1981

[Progressive bulbar paralysis in childhood with pyramidal signs (author's transl)].

We report on a 8 year-old boy who presented with progressive bulbar paralysis. Remarkable were the presence of pyramidal signs, the visual disturbances, the peculiar gait and the intermittent progression of the disease. Our case supports the idea that spinal muscular atrophies form a group of diseases with variable expression. We can classify our patient between the juvenile form and the adult form of spinal muscular atrophy with progressive bulbar palsy. We could follow this boy for almost 4 years. He died at the age of twelve years. Post mortem examination was not permitted.

Bulbar Palsy, Progressive↗