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Biomedical subjects

D Harms

Publications and source records attributed to D Harms.

At least 181 records · Page 10Linked to original sources

Cellular congenital mesoblastic nephroma in a newborn.

Typical nephroblastoma (Wilms'tumor) is uncommon within the first 6 months of life. Renal tumors most commonly found in this age constitute of two groups which have a better and worse prognosis, respectively, than typical nephroblastomas. The group of tumors with a better prognosis encompasses congenital mesoblastic nephroma (CMN), fetal rhabdomyomatous nephroblastoma and cystic, partially differentiated nephroblastoma. The group of tumors with a worse prognosis consists of rhabdoid tumor of the kidney and bone metastasizing renal tumor (clear cell sarcoma) of childhood. Adequate therapy for these two neoplasms has as yet to be developed. Among the low-grade malignant tumors congenital mesoblastic nephroma can be successfully treated with simple nephrectomy. There, are however, variants of CMN which may differ in clinical behavior from the typical form of CMN. These variants include the cellular CMN2 and, possibly, the malignant mesenchymal nephroma of infancy. A case of cellular congenital mesoblastic nephroma is presented here. Its clinical and pathologic features are discussed.

Female↗

Influence of two-stage anatomic correction on size and distensibility of the anatomic pulmonary/functional aortic root in patients with simple transposition of the great arteries.

To evaluate the results of the two-stage anatomic correction of simple transposition of the great arteries the size, distensibility, and histologic characteristics of the anatomic pulmonary root, which arises from the anatomic left ventricle and which we termed the functional aortic root after anatomic correction, were determined in seven patients before and twice after anatomic correction (mean 43 and 671 days) and the results were compared with those in normal control subjects. The diameter of the systolic sinus of the anatomic pulmonary root increased after banding on the average to 140% of normal, whereas the diameter of the diastolic sinus of the functional aortic root increased after anatomic correction on the average to 150% of normal. Diameters of both the systolic and diastolic sinuses of the functional aortic root remained 30% to 55% larger than normal after anatomic correction. Growth potential of the functional aortic root after anatomic correction was normal, whereas its distensibility, as assessed by determination of the percent change in radius and pressure-strain elastic modulus (stiffness index), was decreased after anatomic correction. This pressure-strain elastic modulus was directly related to the corresponding body surface area and age at banding. In four of five specimens of the anatomic pulmonary arterial wall that were obtained at the time of anatomic correction, fragmentation and shortening of elastic fibers were observed. The histologic characteristics of the pulmonary root in the patient with the smallest body surface area at banding and normal distensibility of the anatomic pulmonary/functional aortic root before and after anatomic correction revealed normal aortic configuration of the elastic tissue.(ABSTRACT TRUNCATED AT 250 WORDS)

Aorta↗

Prenatal diagnosis of a short-rib-polydactylia syndrome type Saldino-Noonan at 17 weeks' gestation.

A case is described in which the family history and the sonographic findings during the 17th gestational week led to the suspicion of a short-rib-polydactylia syndrome of the Saldino-Noonan type and an abortion was induced. The ultrasound image disclosed cystic structures in the topographic area of the kidney, marked ascites and oligohydramnios and indicated a severe fetal developmental disturbance. Due to the oligohydramnios the extremities could not be interpreted sonographically. The pathological-anatomical findings confirmed the suspected short-rib-polydactylia syndrome of the Saldino-Noonan type. Typical skeletal changes, dysgenetic cystic kidneys and pancreatic fibrosis were especially indicative of the syndrome. The complete agenesia of the islets of Langerhans, present in our observation, has, as far as we know, previously not been reported in the case of short-rib-polydactylia syndromes.

Abnormalities, Multiple↗

[Congenital nesidioblastosis. Successful treatment with total pancreatectomy].

A 6,410 g newborn baby suffered from severe hypoglycemia despite therapy with high doses of diazoxide and glucagon as well as intravenous application of glucose. There was no persistent response of blood glucose to continuous infusion of somatostatin. A 85% pancreatectomy was performed at the age of 6 weeks, after biochemical findings had indicated hyperinsulinism. As the hypoglycemia reappeared postoperatively, the child underwent total pancreatectomy. Now, at the age of 9 months, the baby's growth and development is normal under substitution therapy with Pankreon and depot-insulin 0.2 U/kg/day.

Diabetes Mellitus, Type 1↗

Malignant rhabdoid tumor of the kidney. Histopathology, ultrastructure and comments on differential diagnosis.

Clinical and histopathological features of two cases of malignant rhabdoid tumor of the kidney are presented. One of these cases was also studied by electron microscopy. Histologically, both tumors consisted of an admixture of undifferentiated polygonal or elongated cells and cells with abundant eosinophilic cytoplasm frequently containing hyaline globular structures. Ultrastructurally, these cytoplasmic inclusions were composed of large masses of actin-size and intermediate-size filaments. The poor prognosis of this type of tumor is emphasized and histological criteria for differential diagnosis from other malignant renal tumors of childhood and adolescence are discussed.

Diagnosis, Differential↗

[Hearing defects after purulent meningitis (author's transl)].

80 children in the age of 4 to 20 years who got over a purulent meningitis in infancy or childhood were examined by means of several audiological methods. Two children (2,5 percent) were found to be deaf. In 4 cases (5 percent) deafness was detected only unilateral not preferring the right or left ear. Hearing impairment after the eights cranial nerve damage was found in four children. 4 children suffered from severe to profound hearing defects on one side or bilateral. No correlation could be found between incidence and severity of the hearing defects to the total dose of the antibiotics that were penicillin, ampicillin and gentamycin. Before the background of the literature reported on this subject and the presented results hearing impairment after purulent meningitis seems to be more likely a sequela of inflammation than a toxic side-effect of the antibiotics.

Adolescent↗

[Vigilance and beta blockade. The influence of the beta blocker atenolol and other substances on the reaction time of the visual system].

Visual reaction times as a measure of vigilance and of the psycho-physiological condition of subjects was determined after combined physical and mental stress to examine beta-blocker influence. Using the technique of electrooculography 40 subjects aged 25,7 +/- 6 with a mean blood pressure to 126/79 mmHg were measured in a double-blind cross-over design after application of placebo or 50 mg of atenolol (Tenormin) for 3 days. Visual reaction time was defined as the time between display of a peripheral light signal and the start of the eye movement that shifts the direction of gaze from the reference point to the stimulus. The results of the study show, that under these experimental conditions there is a positive effect of beta-blocker medication on vigilance. Findings of other authors are discussed. To prove the sensitivity of the test method in a preliminary study, the effects of the well-described drugs fenetyllinhydrochloride, diazepam, oxazepam and alcohol on visual reaction time were investigated.

Adrenergic beta-Antagonists↗

[Dedifferentiating intracerebral neuroblastoma resistant to x-ray and drug therapy -- a case report (author's transl)].

A new case of primary intracerebral neuroblastoma is reported. The patient, a 2 year old boy, was subjected to temporary successful craniotomy, postoperative irradiation and cytostatic therapy without any influence on the recurrence of the tumor. In contrast to the possible well-known maturation of neuroblastomas a dedifferentiating process occurred. The boy died 8 1/2 months after diagnosis.

Brain Neoplasms↗