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Biomedical subjects

W Sorgo

Publications and source records attributed to W Sorgo.

9 recordsLinked to original sources

Gonadal agenesis in a 46,XY female with multiple malformations and positive testing for the sex-determining region of the Y chromosome.

A full-term 46,XY female newborn presented with respiratory failure due to a right-sided diaphragmatic hernia. During surgical repair, exploration revealed isolated dextrocardia and hypoplasia of the right lung. Neither gonads nor wolffian or müllerian structures could be palpated. Cardiac catheterization demonstrated defects of the ventricular septum, hypoplasia of the right pulmonary artery, persistence of the left vena cava superior and a patent ductus arteriosus. Anthropometric data were normal at birth, but fell below the 3rd percentile during follow-up. Body proportions displayed a predominance of the upper compared to the lower segment. Endocrine studies indicated no defect of steroid biosynthesis and no functional gonadal tissue. Using genetic analyses of various loci within the testis-determining region of the Y chromosome, a mutation could not be detected. The patient died from pneumonia at the age of 19 months. Postmortem examination confirmed the diagnosis of gonadal agenesis.

Abnormalities, Multiple

Testosterone-secreting adrenocortical tumor in a pubertal girl. Case report and review of the literature.

A girl aged 12 years and 10 months presented with deepening of the voice first noted 7 months earlier. Pubertal development was almost completed. The girl had regular monthly menses and no signs of hirsutism, clitoris enlargement or Cushing's disease. Serum testosterone was about threefold above normal, whereas dehydroepiandrosterone was in the upper normal range. The 17-ketosteroids as well as the gas-chromatographically analyzed 5 alpha and 5 beta derivatives of testosterone from urine were slightly increased. Other serum and urinary steroids were normal. Dynamic tests of the endocrine function exhibited inconclusive results. Ultrasonography revealed no ovarian cysts. A small, left-sided adrenal mass was identified by computed axial tomographic scan and removed by surgery. There were no signs of local metastasis nor of vascular extension. The histopathological diagnosis was adrenocortical carcinoma. 5 months after surgery, the preoperatively elevated steroid levels had returned to normal.

Adolescent

The effects of cyproterone acetate on statural growth in children with precocious puberty.

Forty-four patients (42 f, 2 m) with precocious puberty (31 idiopathic, 1 familial, 7 cerebral, 5 McCune-Albright) were treated with cyproterone acetate for periods of 1-8.75 years in different (P less than 0.05) daily dosages of 117 +/- 6.1 mg/m2 per day (mean +/- SEM, group A, N = 20) and 60.8 +/- 2.42 mg/m2 per day (group B, N = 24). Thirty-three girls had experienced menarche before therapy at a mean age of 4.89 +/- 0.42 years. Treatment was started at a chronologic age of 5.45 +/- 0.33 years in the girls and 5.74 +/- 1.34 years in the boys. At the time of evaluation, 31 of our patients had reached final height. With respect to the effects of treatment on statural growth, the Standard Deviation Scores were retrospectively determined for height, weight, and growth velocity. The initial Bayley-Pinneau height predictions were compared with final height and target height, and the skeletal maturation was studied. There were no significant differences between those parameters in the patients of group A and B or between treated and untreated subjects as far as final height and target height were concerned. It is concluded that cyproterone acetate administered orally at daily doses from 50-150 mg/m2 does not improve statural growth of patients with precocious puberty.

Body Height

[Diphtheria].

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Ampicillin

[T3-thyrotoxicosis in a 12 year old girl (author's transl)].

Isolated Triiodothyronine (T3) hyperthyroidism in childhood is rate. The incidence among hyperthyroid children is about 10%. The authors present a case history of a 12 2/12 year old girl with T3-thyrotoxicosis. Cause, diagnosis and therapy are thoroughly discussed. The importance to estimating serum T3 concentration for establishing the diagnosis of T3 hyperthyroidism is emphasized.

Child

[Minor diphtheria epidemic in a Cologne children's home (author's transl)].

Between May and July 1972, 12 children aged from 7 to 14 were admitted to the Cologne University Children's Hospital suspected of having diphtheria. In 10 cases the tonsils or pharyngeal cavity were affected. One case of wound diphtheria and one of nasal diphtheria were observed. The clinical suspicion was confirmed in 8 of 12 cases by detection of the pathogen Coryne-bacterium diphtheroides mitis. 11 patients were discharged from the hospital after being treated for between 2 to 6 weeks, but 5 of them needed ambulant cardiac observation. One patient died of malignant diphtheria with symptoms of cardiovascular failure. He had not been treated specifically for 1 week at first. Not one of the 12 patients had been satisfactorily immunized by active inoculation with diphtheria toxoid.

Adolescent

Asymmetry of the lipid-bilayer of Sindbis virus.

The organization of the lipid bilayer of the enveloped Sindbis virus has been studied. In the model membrane which consists only of two virus specific glycoproteins and host derived lipids the latter were radioactively labelled with 14C-palmitic acid by prelabelling their BHK 21 host cell lipids. The purified virus particles were submitted to neuramidase, bromelain and combromelain-neuraminidase treatment. It could be demonstrated that N-acetyl neuraminic acid residue of the total hematoside present in the virion is hydrolyzed by neuraminidase leaving the particles fully intact. Proteolysis of the spikes leads to particle aggregation yet an unchanged hematoside content. This was fully transformed into ceramidelactoside by subsequent neuraminidase treatment. The analyses of the ceramide species present in hematoside of the control particles and ceramidelactoside derived thereof by neuraminidase hydrolysis are in very close agreement. From these experiments it is concluded that all hematoside molecules are organized in the outer half of the bilayer of the envelope.

Bromelains

[Diagnosis and therapy of gigantism].

Some of the most important types of nonfamilial tall stature are discussed by stressing the clinical features, diagnostic aspects and therapeutic possibilities. The indications of treatment, the diagnostic procedures and steroid therapy of the familial type of tall stature, the predominant variant of tall stature, are presented in detail. The effects, side effects and contraindications of high dose steroid treatment are described. The important prerequisite for the evaluation of psychosomatic problems of tall stature is the understanding of the dynamics and variations of normal growth. This and the knowledge of methods concerning growth analyses continue to be the basis of an accurate diagnosis.

Body Height