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Biomedical subjects

H Stroink

Publications and source records attributed to H Stroink.

42 records · Page 3Linked to original sources
PubMed1989

[Children with autism and related contact disorders: medical aspects].

In children with infantile autism or atypical pervasive developmental disorders somatic aspects play an important role. A review is presented of important hereditary, pre-, peri- and neonatal factors, findings at neurological examination, specific medical disorders and neurochemical and neurophysiological findings. Results of the medical examination of 15 children with autistic or atypical developmental disorders are presented. It is concluded that extensive medical examination of these children is indicated: in 8 out of 15 children a clinically relevant chromosomal, neurological or biochemical disorder could be detected.

Autistic Disorder↗
PubMed1989

[X-linked adrenoleukodystrophy and other peroxisomal diseases caused by a failing peroxisomal beta-oxidation system: clinical expression, diagnosis and treatment].

X-linked adrenoleukodystrophy (ALD) is a sex-linked, neurodegenerative disorder which in its most stereotypic form manifests itself in a boy who progresses normally for the first years of life and then presents with CNS signs and symptoms such as behavioural abnormalities, visual and auditory disturbances and an abnormal gait. The course of the disease is progressive, culminating within a few years in dementia, blindness, quadriplegia and death. Recently it has become clear that ALD is caused by an impairment in the peroxisomal beta-oxidation system leading to the accumulation of very-long-chain fatty acids. Accumulation occurs not only in plasma but also in brain giving rise to increasing myelin instability and subsequent demyelination. Apart from X-linked ALD there are a number of other disorders all characterized by a deficient peroxisomal beta-oxidation activity. It is remarkable that the clinical presentation of these different inborn errors of peroxisomal beta-oxidation differs markedly. In this paper the inborn errors of peroxisomal beta-oxidation known at present will be discussed with particular emphasis on ALD.

Adrenoleukodystrophy↗
PubMed1987

Fontanelle pressure monitoring in infants with the Rotterdam Teletransducer: a reliable technique.

Intracranial Pressure (ICP) monitoring is important in patients at risk for raised ICP. In infants non-invasive methods for measuring ICP are to be preferred, and hence Anterior Fontanelle Pressure (AFP) measurements have been employed. So far, techniques used were not reliable since application of a transducer to the fontanelle generally influences the recorded pressure value. For the purpose of non-ambiguous ICP recording the Rotterdam Teletransducer was fitted in a special light weight skill adaptor, provided with a special fixation frame. With this adaptor the transducer can be set and maintained at accurate depth in the fontanelle according to the pressure depth curve. The plateau in the pressure depth curve represents actual ICP. The development of this reliable and reproducible technique is discussed. The results of instantaneous and continuous monitoring of AFP in 70 children with- and without neurological diseases are presented.

Brain Diseases↗
PubMedDate not supplied

Phakomatosis pigmentovascularis.

We report a patient with phakomatosis pigmentovascularis IIb and numerous iris hamartomas. Phakomatosis pigmentovascularis IIb is characterized by the simultaneous occurrence of a nevus flammeus, a mongolian spot, and sometimes a nevus anemicus in the same individual, with systemic involvement. To our knowledge, the association with multiple iris hamartomas has been reported only once. This second patient suggests that the association might be more common. Additional reports will indicate if such an association is more frequent than is now assumed.

Child, Preschool↗
PubMedDate not supplied

Follow-up of 146 children with epilepsy after withdrawal of antiepileptic therapy.

The relapse rate after discontinuation of antiepileptic drug treatment was investigated in 146 children with epilepsy, in whom medication was withdrawn according to a predesigned protocol, after a seizure-free period of at least 2 years and normalization of the EEG. The cumulative probability of remaining seizure-free in this series was 74.5%. Three-quarters of the relapses occurred during the withdrawal period and in the 2 years thereafter. From multivariate analysis, the factors indicating a significantly higher relapse risk were seizures with a known cause and female sex. In primary generalized epilepsy, no factor significantly increased the likelihood of a recurrence. In partial epilepsy, significant factors predictive of recurrence were the presence of a neurological deficit (focal neurological signs and/or mental retardation), female sex, a positive family history for epilepsy, and the number of drugs necessary for control of the seizures. The present results are compared with the available literature data. It is argued that using multivariate analysis after elimination of EEG variables uncovers significant clinical predictive factors that in other studies may have remained hidden. Finally, it is argued that statistical analysis may be used to enable the clinician to predict the likelihood of recurrence in individual children with a given set of relevant predictive factors.

Adolescent↗