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Biomedical subjects

I D Mutz

Publications and source records attributed to I D Mutz.

At least 19 recordsLinked to original sources

Neuroblastoma in Europe: differences in the pattern of disease in the UK. SENSE. Study group for the Evaluation of Neuroblastoma Screening in Europe.

BACKGROUND: Neuroblastoma is a major contributor to childhood cancer mortality, but its prognosis varies with age and stage of disease, and some tumours regress spontaneously. Urinary screening programmes or clinical examination may detect the disease before symptoms appear, but the benefit of early diagnosis is uncertain. We examined the incidence, pattern, and presentation of neuroblastoma in four European countries. METHOD: Population-based incidence rates were derived for France, Austria, Germany, and the UK. Age, sex, and stage distribution were analysed by Mantel-Haenszel techniques and Poisson regression. The proportion of incidental diagnoses (cases without symptoms found at routine health checks or during investigation of other disorders) and mortality rates were also compared. FINDINGS: Between 1987 and 1991, 1672 cases of neuroblastoma were diagnosed in children under 15 years old (France, 624; Austria, 69; Germany, 493; UK, 486). Age-standardised annual incidence was significantly lower in the UK (10.1/million) than in France (12.5) and Germany (11.4). In the UK a deficit of low-stage disease in infants was accompanied by an excess of stage IV in older children. The UK had significantly fewer incidental diagnoses (8%) than Austria (27%) and Germany (34%). UK mortality rates were significantly higher than German or French rates. INTERPRETATION: In the UK, neuroblastoma diagnosis is delayed, possibly because of a less rigorous system of health checks for children. Although some overdiagnosis occurs in mainland Europe, our data suggest that in the UK some low-stage cases, undetected in infancy, may later present as advanced disease. This finding has implications for screening programmes and organisation of routine surveillance of infant health in the UK.

Adolescent↗

Whistling face syndrome. A case report and literature review.

The cranio-carpo-tarsal or "whistling face" syndrome was first described by Freeman and Sheldon in 1938. More than 60 cases with great variability of expression are known till now and autosomal dominant as well as recessive inheritance and sporadic cases suggest a genetic heterogeneity. We review 60 well-documented cases of the literature and present a patient with a severe form, who died of bronchopneumonia at the age of 9 months. The facial stigmata of his mother and the ulnar deviations of his maternal grandfather support the autosomal inheritance of the syndrome.

Abnormalities, Multiple↗

[Congenital neuroblastoma and placental metastases].

Thus far ten patients with connatal neuroblastoma and metastases in the placenta have been reported. Our patient suffered from neuroblastoma stage IV-S and expired on the first day of life due to hepatic failure from massive metastatic involvement of this organ. Metastases from the placenta into the mother have thus far never occurred, neither in our case nor in any patient reported in the literature.

Adrenal Gland Neoplasms↗

A trial of RIT-4237 rotavirus vaccine in 1-month-old infants.

In a double blind placebo-controlled study rotavirus vaccine RIT 4237 was offered to young infants after the fourth week of life. The vaccine was very well tolerated. Fifty-seven out of 100 vaccine recipients and 10 out of 103 placebo recipients developed rotavirus-IgM-antibodies during the 1 month follow-up period. During a 6 month follow-up, stool samples from 2 out of 12 vaccinees and from 5 out of 12 placebo recipients contained rotavirus.

Antibody Formation↗

[Ski camps for juvenile diabetics. Experiences with 2 vacation ski courses].

In 1980 and 1981 a 10 and 14 day skiing instruction course for 22 diabetic children was held in Styria. The age range of the children was between four and a half and seventeen years. The main purpose of this course was education of diabetic children in the management of their disease during sports activities. The experience about insulin and caloric requirements are described.

Cooperative Behavior↗

[Results of Primary chemotherapy and Delayed Surgical Resection in Osteosarcoma (author's transl)].

Primary systemic therapy with delayed surgical intervention has been employed for the treatment of malignant bone tumours since 1977 at the Universitäts-Kinderklinik and III. Medizinische Abteilung Graz. Improved therapeutic results, as originally reported on a large number of patients at the Memorial Sloan Kettering Cancer Center (MSKCC) seem to have been attained with a small group of patients (n = 5).

Adolescent↗

[Bone marrow transplantation for aplastic anaemia in a ten-year-old girl (author's transl)].

A 10-year-old girl suffering from post-hepatitic aplastic anaemia received 21 x 10(10) (5 x 10(8)/kg body weight) nucleated cells from her brother, whose HLA typing, but not his blood group, was identical with the patient's. Conditioning had been carried out with cyclophosphamide according to the Seattle protocol (50 mg/kg body weight on four successive days). On day 9 she showed signs of early engraftment by the appearance of granulocytes and their precursor cells in the peripheral blood. The new blood group of the patient's erythrocytes and the male karyotype of her leucocytes demonstrate successful engraftment. The child is now in excellent health 18 months after transplantation and requires no treatment of any kind. This paper reports the first bone-marrow transplantation in Austria.

Adolescent↗

[Problems of Hodgkin's disease in childhood].

Many publications about treatment results of Hodgkin's disease in adults give a rather optimistic outlook. Our experience with the treatment results of 10 children between 1970 and 1980 is rather disappointing due to late side effects of the combined treatment modalities. These complications of therapy and guidelines form the management of various stages of Hodgkin's disease are discussed.

Child↗

[Ketamine anesthesia for outpatient procedures in children (author's transl)].

Ketamine anesthesia was used in children to perform 200 outpatient procedures (mainly spinal taps, bone marrow aspirations and biopsies). Atropine was administered in advance and a ketamine dose of 1.8--2.0 mg/kg i.v. was sufficient in most instances. The general anesthesia allows calm and accurate performance of the necessary procedures. Ketamine can be used repeatedly and was preferred by the majority of children for subsequent procedures. The application of Diazepam (2 mg i.v. or 5 mg rectally) reduced unpleasant wake-up dreams in older children. The children were under close observation until consciousness was regained. They left the outpatient clinic attended by their parents approximately one hour after the procedure.

Adolescent↗

Immunologic studies of prothrombin in newborns.

Prothrombin of newborns was investigated by means of the quantitative immunoelectrophoresis and the two-dimensional immunoelectrophoresis. The plasma of 10 of 24 healthy term newborns and of 5 preterm infants taken immediately after birth showed one prothrombin precipitate in the alpha-2-beta-globulin position when a buffer containing 25 mM calcium lactate was used. No differences in the mobility and form of the precipitate could be observed as compared to healthy adults. The plasma of the other 14 term newborns and one small-for-date infant showed two precipitates, one in the alpha-2-beta-globulin position and a second in the alpha-1-globulin position. Twenty-four hr after the injection of vitamin K, the precipitate in alpha-1-globulin position was no longer evident. When a buffer without calcium was used in the two-dimensional immunoelectrophoresis, only one precipitate in the alpha-1-globulin position was found in all newborns. In the quantitative immunologic determination, the prothrombin was found to be decreased as compared to that of adults. The quantitative immunologic determination and the activity assay gave equal results in those infants who had only one precipitate in the two dimensional immunoelectrophoresis. In those newborns, who had two precipitates, the quantitative immunologic determination gave higher results than the measurement of the activity assay. From this study, no evidence of differences between the prothrombin of newborns and that of adults could be derived. Vitamin K deficiency, as judged from the appearance of a second peak in alpha-1-globulin position in the two dimensional immunoelectrophoresis was a frequent findings in term newborns, even on the 1st day of life.

Adult↗

[Thrombasthenia in a newborn (author's transl)].

Thrombasthenia is an extemely rare hereditary disorder of platelet function characterized by impairment of adhesiveness, ADP-induced aggregation, and clot retraction. Petechiae and ecchymoses in a newborn infant followed by prolonged jaundice facilitated early diagnosis.

Blood Coagulation Factors↗

[Dimethyl-triazeno-imidazole-carboxamide (DTIC) in combination chemotherapy for childhood neuroblastoma (author's transl)].

Eight children with state III or IV neuroblastoma were treated with courses of chemotherapy consisting of nitrogen mustard (6 mg/m2), vincristine (2 X 1.5 mg/m2), doxorubicin (40 mg/m2) and dimethyl-triazeno-imidazolecarboxamide (850 mg/m2) every three weeks. Three children died after a median survival of 8 (+/-5) months. Five children (62%) have now survived for a medium duration of 21 (+/-16) months without clinical evidence of disease. These results are far better than previously-achieved survival rates at our department.

Child, Preschool↗