[Nielsen syndrome--a rare dysmorphia complex].
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Biomedical subjects
Publications and source records attributed to M Radke.
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In 19 children affected with acute leukaemia the serum immunoglobulin level was examined. An Ig deficiency treated with a single administration of IgA or IgM concentrate could be detected in 16 of these children. The impact of this single Ig substitution on the serum immunoglobulin level and on B-lymphocytes in the peripheral blood was thrice examined after this substitution. A significant increase of the corresponding Ig class in the serum and a significant decrease of B-lymphocytes in the peripheral blood could be observed.
In 15 children with a lowered gammaglobulin level a single substitution with HGG was made, with the impact of this substitution on the number of B-cells in the peripheral blood being examined by means of the direct fluorescence antibody technique. In 9 from 15 children the substitution had no influence on the number of B-cells. However, a significant increase of the number of B-cells could be observed in 6 from 15 children. 14 days after the substitution the number of B-cells lay within the normal range again.
By means of a direct immunofluorescence technique the numbers of B-lymphocytes in the peripheral blood were determined in healthy children of different age with anti-human IgG serum and anti-human globulin. Their small number in new borns and a marked increase in infancy were significant. In addition, the paper contains extensive methodical data.
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In comparison with infants not suffering from cyanotic heart failures a different size (width and length) as well as a delayed time of closure of the anterior fontanelle in infants with congenital cyanotic heart failures were detected. In these patients typical phenomenons by ultrasound examinations are described also.
Rhabdomyolysis can be caused by aetiologic completely different factors. It can occur only few hours following beginning of enteritis and refusal of ingestion. We report the case of a 5 month old male infant, which showed the clinical characteristics of a hypertonic dehydration on admission to hospital. Serum levels of transaminases, creatinine, urea, lactated hydrogenase, creatine kinase and urinary excretion of myoglobin were highly elevated. Forced diuresis, compensation of acidosis and electrolyte disturbances could prevent the development of an acute renal failure--the most important complication of rhabdomyolysis. The boy could be discharged without any residuals of this episode of disease.
A male infant with obstructive uropathy developed yeast cell agglomerations which were detectable macroscopically and by image-generating techniques within both renal pelvises after Candida albicans infection of the urinary tract. Therapy with flucytosine induced excretion of 'fungal balls' via the urethra. Continuation of therapy with liposomal amphotericin B (AmBisome) prevented a relapse after development of fungal resistance against flucytosine. Sonographically or radiographically detectable formation of 'concrements' within the urinary tract of patients with an immature or compromised immune system and additional features such as obstructive urinary tract should suggest a localized or systemic mycosis, particularly in the context of long-term antibiotic treatment.
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