Biomedical subjects

A Lindinger

Publications and source records attributed to A Lindinger.

26 records · Page 2Linked to original sources

PubMedDate not supplied

[Arterial tortuosity syndrome].

The arterial tortuosity syndrome is a rare congenital disorder characterized by elongation and generalized tortuosity of the major arteries including the aorta. Associated clinical features consist of excessively stretchable skin and joint laxity which is indicative of a connective tissue disorder such as Ehlers-Danlos or Cutis laxa syndrome. The gene locus of the arterial tortuosity syndrome has recently been localised on chromosome 20q13; inheritance ist autosomal recessive. - We report on a newborn with arterial tortuosity syndrome and hiatal hernia, bilateral hip dislocation, inguinal hernias and diffuse tortuosity of the great arteries including the aorta. Known gene loci involved in Ehlers-Danlos syndrome, cutis laxa syndrome and other connective tissue disorders were excluded by specific DNA markers. By homozygosity mapping with polymorphic microsatellite markers it was possible to confirm the gene locus for the ATS on chromosome 20q13. In addition to the presentation of this patient, a review of the literature is presented.

Abnormalities, Multiple↗

PubMedDate not supplied

Severe valproic acid intoxication is associated with atrial tachycardia: secondary detoxication by hemoperfusion.

Valproic acid is an anticonvulsant drug which is associated with serious toxicity including fatal outcome in case of severe intoxication. Secondary detoxication by hemodialysis or hemoperfusion has been employed successfully in valproic acid intoxication. Cardiac arrhythmias have only been described rarely in valproic acid intoxication in humans. We report on a 15 year-old boy with severe valproic acid intoxication (valproic acid plasma level on admission: 1 150 mg/l) who presented with coma, hypernatremia and atrial tachycardia. The patient was successfully treated with hemoperfusion and intensive supportive care without implementation of a specific antiarrhythmic therapy. We conclude that patients with severe valproic acid intoxication may benefit from secondary detoxication. In addition to generally known symptoms valproic acid intoxication may also be associated with cardiac arrhythmias.

Adolescent↗

PubMedDate not supplied

[Mediastinal cystic lymphangioma as a cause of hydrops fetalis].

This report is upon a case with fetal hydrops on the base of a paracardiac cystic lymphangioma in the mediastinum. The newborn which had hypoplastic lungs and multiple pneumatothoraces died because of a pulmonary insufficiency. An overview is given about the most common causes of the nonimmune fetal hydrops, and the pathomechanisms are discussed.

Female↗

PubMedDate not supplied

[Kawasaki syndrome in infants in the first 6 months of life].

Diagnosis in Kawasaki disease usually is made on the so called main symptoms and on laboratory findings. The analysis of three cases in the first six months of life demonstrated that the typical symptoms concerning the mucosa and the lymphatic nodes are rare in this age group, whereas drug resistant fever and polymorphic exanthema was present in all patients. Besides that accessory symptoms like abacterial meningitis and pathologic urine analysis seemed to be frequent signs. Laboratory findings such as pathologic blood sedimentation rate, elevated CRP and thrombocytosis on the other hand were helpful in making the diagnosis.

Aneurysm↗

PubMedDate not supplied

[Modification of cardiovascular dynamics in orthostatic dysregulation in childhood by oxilofrin].

In 10 children with clinically and hemodynamically proven orthostatic dysregulation of the sympathetic type, non invasive hemodynamics using sphygmographic techniques as well as systolic time intervals were evaluated. Measurements were taken during 10 minutes in the supine and a 65 degrees position on a tilting table before and after acute administration of Oxilofrin, 20 mg as well as after 2 and 4 weeks of chronic treatment with 20 mg t.i.d. During orthostatic stress, Oxilofrin caused a significant increase in pulse pressure, cardiac output, stroke volume, and systolic ejection rate with a reduction in peripheral vascular resistance compared to control. Pre-ejection period and isovolumic contraction time, while prolonged on baseline orthostatic stress, were only slightly shortened by Oxilofrin. These hemodynamic actions of the drug are explained by a stimulation of adrenergic beta and alpha receptions with the consequence of decreasing venous capacitance and increased venous return, thus providing a useful hemodynamic profile for the treatment of orthostatic dysfunction.

Adolescent↗

PubMedDate not supplied

[Compression syndrome of the upper thoracic outlet (thoracic outlet syndrome)--report on a 13-year-old girl].

The thoracic-outlet-syndrome, a symptom complex consisting of arterial, venous and neural disorders of the upper extremity, is caused by compression of the neurovascular structures between the clavicle and the first rib. Other reasons are congenital abnormalities like a cervical rib, an abnormal first rib, acquired anatomic lesions like fractures and traumata or functional factors. The younger-and middle-age groups are most commonly involved. The symptoms are depending upon the point at which compression occurs and include pain, paresthesias, numbness, fatigue, pallor, coolness or swelling and enlargement of the arms. At physical examination a systolic murmur is heard in the supraclavicular region in 30 percent and the pulse of the upper extremity often is reduced. Diagnostic measurements include roentgenogram of the chest and the cervical spine, pulsed doppler-ultrasonography, nerve conduction-velocity testing and an arterio-venography. Treatment is nonoperative in patients with mild symptoms (shoulder-girdle-exercises) and operative in patients with severe or persistent symptoms. The operative treatment of choice is resection of the first rib by an axillary route. Risks are minimal, and the results are good to excellent in 90% of the cases. Because the thoracic outlet syndrome is very rare in childhood, we report a 13 year old girl which has undergone bilateral operation with excellent result.

Adolescent↗

PubMedDate not supplied

[Churg-Strauss syndrome in a 15-year old boy].

A 15 year-old boy was admitted in our hospital with the clinical signs of cardiopulmonary deterioration. His history showed bronchial asthma since 3 years. Furthermore he revealed signs of polyneuropathy, exanthema and a transitory pulmonary infiltration. Elevation of eosinophilic granulocytes and IgE confirmed the diagnosis of a Churg-Strauss syndrome. A rapid improvement of the symptoms was achieved by high-dose steroids and symptomatic therapy. The impaired systolic and diastolic myocardial function improved, but a cardiomyopathy with a shortening fraction below 25% persisted. The Churg-Strauss syndrome is a rare disease in childhood and is classified as a systemic granulomatosis of the vessels. The marked perimyocarditis with consecutive restrictive cardiomyopathy is a rare manifestation in childhood and adolescence. The prognosis of this disease is mainly influenced by the cardiac involvement.

Adolescent↗

PubMedDate not supplied

[Ventricular tachycardia after erythromycin administration in a newborn with congenital AV-block].

UNLABELLED: This report is on a newborn with congenital complete av-block due to a maternal collagenosis. The intravenous application of erythromycin produced premature ventricular beats and non sustained ventricular tachycardias by prolongation of the QT interval. After discontinuation of the erythromycin application, the QT-interval normalised. CONCLUSION: In atrioventricular conduction disorders with severe bradycardia and prolongation of the QT interval, the application of erythromycin--if unavoidable--should be managed by slow intravenous infusion and with permanent ECG monitoring.

Anti-Bacterial Agents↗