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Biomedical subjects

W Tulzer

Publications and source records attributed to W Tulzer.

At least 19 recordsLinked to original sources
PubMed1993

[Zellweger syndrome, neonatal adrenoleukodystrophy or infantile Refsum's disease in a case with generalized peroxisome defect?].

An eleven month-old boy presented clinically with craniofacial dysmorphia, severe psychomotor retardation, neurological deterioration, no response to visual and acoustic stimuli, failure to thrive, hepatomegaly and adrenal insufficiency. Specific biochemical markers for a peroxisomal deficiency disorder (Zellweger's syndrome, neonatal adrenoleukodystrophy, infantile Refsum's disease) revealed pathological results for very long chain fatty acids, phytanic acid, pristanic acid, plasmalogen biosynthesis and catalase, thus confirming the clinical diagnosis. Comparison of clinical and biochemical findings in the patient with the characteristics of the three peroxisomal deficiency disorders showed overlapping with each of these disorders, which corresponds to the current view that these three peroxisomal disorders differ only with respect to onset and severity of the clinical manifestations, but not with regard to the biochemical defects.

Adrenoleukodystrophy↗
PubMed1992

[Schoenlein-Henoch syndrome with abdominal manifestations without skin involvement].

The etiology of Schoenlein-Henoch' Syndrome has not yet been fully clarified [10, 15]. An allergically toxic genesis is under discussion [8, 19]. The classical combination of symptoms consists of urticariel efflorescences, bleeding of skin and lining tissue and arthralgies [8, 19]. Involvement of kidneys and abdomen may occur [8, 19]. Our case report concerns exclusively an abdominal form with colics, vomiting and diarrhea [10, 18, 16]. Chemical tests reveal a reduction of factor XIII [1, 5, 21]. There is no involvement of kidneys, skin or joints. With reference to literature and this particular case, etiology, diagnosis and therapy are dealt with.

Child↗
PubMed1992

[Long-term therapy of immune neutropenia with high-dose 7S immunoglobulin in a child].

A 15 month old girl with suspected immune neutropenia was treated with high dose immunoglobulin because of recurrent infections over a period of 9 months. Before therapy her neutrophil counts were below 0.5 G/l. With monthly intravenous immunoglobulin her neutrophils remained above 0.5 G/l with one exception. Even after 9 months the patient responded well to therapy, she did not experience new episodes of infections. We suggest that in case of recurrent infections high dose immunoglobulin therapy should be considered in patients with immune neutropenia before the occurrence of life threatening infections.

Autoantibodies↗
PubMed1992

[Polyglandular type I autoimmune syndrome].

Two HLA-identical sisters have developed the full picture of type I polyglandular autoimmune syndrome over a period of 12 years. Both girls have hypoparathyroidism and Addison's disease. One of them additionally developed diabetes mellitus, hypergonadotropic hypogonadism and hypothyroidism. Autoantibodies to the adrenal, parathyroid and thyroid glands are present in both patients, as well as antinuclear antibodies. HLA associations have been described recently for the type I polyglandular autoimmune syndrome, but this could not be confirmed in the present two cases. Although we assume that the same genetic defect is present in both girls, additional factors to the genetic disposition are important for the clinical expression of the disease. The linkage of the disease-causing gene with the HLA region is not very close.

Addison Disease↗
PubMed1991

[Rare indications for iron chelation therapy with desferrioxamine].

In beta thalassaemia patients the subcutaneous desferrioxamine chelation therapy is performed routinely. Rare indications are hypoplastic anaemia, congenital dyserythropoetic anaemia and Fanconi anaemia. We initiated the chelation agent in three patients with the above mentioned diagnoses. The beginning of treatment in hypoplastic anaemia depends upon the quantity of red cell transfusions, whereas in dysterythropoetic anaemia the increased intestinal iron absorption has to be taken into consideration. Aim of the therapy is a negative iron balance. The evaluation of the iron balance is relatively simple. The girl with hypoplastic anaemia has been treated for 1.5 years. Within this time she received 11,680 mg iron by blood transfusions. The urinary iron output was 7112 mg. Depending on the analyzing method, the mean fecal iron excretion amounts 36.5% or 61% of the global excretion. Laboratory findings and clinical course are in favour to the aimed negative iron balance in two patients.

Adolescent↗
PubMed1990

[Initial therapeutic experiences in AIDS in childhood].

The acquired immuno-deficiency-syndrome in children is a diagnostic and therapeutic challenge. The classification of pediatric AIDS follows the recommendation of the C.D.C., Atlanta. We report on three children infected by their HIV-positive mothers. All three mothers were intravenous drugaddicts, one of them already died of AIDS. One child (S.M.) is classified P2-AB, two children (K. C., D. M.) suffering from lymphoid interstitial pneumonia belong to category P2-C. Patient D. M. was treated with prednisolone following the recommendation of Rubinstein. The pulmonary condition improved remarkably. All three patients are being under treatment with oral antimycotics and prophylactically with Cotrimoxacole for pneumocystis pneumonia. Positive experiences in U.S.A. and Europe have encouraged the use of parenteral immunoglobuline-therapy in three of our patients to minimize additional viral or bacterial infections. We report on our first experiences with this therapeutic regimen. The dosage being used is 0.4 g/kg body weight every month.

Child, Preschool↗
PubMed1988

[AIDS in childhood].

1% of registered AIDS cases are children, 80% have been perinatally infected. Special diagnostic criteria have to be applied for infants. Intrauterine infection can produce a specific malformation syndrome. Chronic pulmonary illness can be caused by pulmonary lymphoid hyperplasia and-less frequently-by Pneumocystis carinii pneumonia. Pulmonary lymphoid hyperplasia is responsive to corticoids. HIV infected mothers should refrain from breast feeding. Teenagers have to be informed adequately about the risks of and how to avoid HIV infection.

AIDS Serodiagnosis↗
PubMed1988

[Smoking in childhood and adolescence].

Well documented reports point out, that most children smoking their first cigarette are younger than 15 years of age. Smoking habits are usually fixed till age 20. Withdrawal treatment shows a bad prognosis and a great number of relapses. Therefore preventive measures must start early at school and should include all social environmental factors.

Adolescent↗
PubMed1987

[Drug therapy of chronic juvenile arthritis].

Children suffering from juvenile chronic arthritis need a longterm therapy and guidance for many years, in course of which drug therapy is only one part of the whole treatment regimen. This article gives a review about the actual available drugs with mode of action, indications and side effects. Finally follow some suggestions for practicable therapeutic guidelines on different courses of juvenile chronic arthritis which include also the possibility for the necessary individual adaptation to the patient.

Adrenal Cortex Hormones↗
PubMed1985

[Experiences with growth hormone therapy in pituitary dwarfism].

The recommendations of the growth hormone dose for the treatment of growth hormone deficiency are similar, but there is still some uncertainty about the question which dose is optimal for the patients needs. The results of 10 patients with growth hormone deficiency are discussed. The indication to change the weekly dose from 8 IU to 12 IU growth hormone was dependent on the growth velocity. The mean growth velocities were: 7.61 cm for the first year, 6.22 cm for the second year, 6.21 cm for the third year and 6.66 cm for the fourth year. At any time, when we increased the dose, we saw an increment of the height afterwards. It remains to be determined, whether it means, that the genetically existing growth potential can be stimulated any time. When we compare the mean height velocity for the first 4 years with the results of other groups, we can see, that we did not lose growth power despite the late change to a third growth hormone injection. The mean growth velocity of the first 4 treatment years was 6.67 cm.

Adolescent↗
PubMed1985

[Chronic juvenile arthritis. Control of long-term therapy].

Children suffering from juvenile chronic arthritis need a longterm therapy and guidance for many years. Drug therapy is only part of the whole treatment regimen and has to be permanently adapted to the actual state of the disease. Drug induced side effects must be strictly differentiated from exacerbations of the rheumatic disease. Short time clinical controls, some few laboratory tests and many detailed talks with the patient and his parents are basic requirements for successful treatment and lead in more than 80% of the juveniles to a normal school education and adequate vocational training.

Adrenal Cortex Hormones↗
PubMed1984

[Second tumor following Hodgkin's disease].

This article deals with the case of a three year old boys suffering from a M. Hodgkin stage IA who was given telecobalt radiation in 1975. A year later he was again given radiation because of a local recurrence and additionally treated by chemotherapy according to the MOPP scheme. Six and a half years after the first diagnosis we noticed the incidence of a not differentiated neurogenic intracerebral tumor. The question if chemotherapy and radiotherapy possibly cause the incidence of second malignancies is now under discussion.

Antineoplastic Agents↗
PubMed1983

[The problem of diagnosis and therapy of myotonic dystrophy].

Three cases of myotonia dystrophica are presented with special problems of diagnosis and treatment. Diagnosis at an early stage can be difficult because of a wide onset of the disease and varying symptomatology. Muscle biopsies should be taken from distal muscle groups since the proximal musculature may be involved at a later stage of the disease. The diagnosis can be established with a typical electromyographic finding. Therapeutic benefits can be obtained by membrane-stabilizing substances like anticonvulsants and antiarrhythmics, the dystrophic process however cannot be halted.

Adult↗
PubMed1982

[Progress in the treatment of juvenile leukemias].

Between January 1979 and December 1980 64 children with acute lymphoblastic leukemia were treated in 9 pediatric clinics in austria according to the BFM study 76/790-protocol. For remission induction all patients received an 8 week multidrug regimen (West-Berlin ALL-protocol). High risk patients were defined according to a risk score at diagnosis and additionally treated with a 6 week reinforced reinduction protocol during the first half year after diagnosis. Maintenance therapy was stopped after about 22 months. The life table-analysis after 30 months showed a 75.5% disease free survival for the total group of patients. Compared with a control group of 228 patients treated between 1974 and 1980 in 9 different clinics in Austria according to 3 consecutive national treatment regimens (modifications of Memphis protocol VII and VIII), therapeutic results were markedly improved. After a follow-up of 36 to 90 months the overall oumulative remission rate was 37.7%. The results could be improved especially in the group of high risk patients for replase by 35% in contrast to the historical studies. A prognostic difference between low- and high risk-patients was not seen in the BFM study (84.3% vs. 69.9%). Without doubt, the marked improvement of prognosis is due to the intensification of therapy.

Antineoplastic Agents↗
PubMed1982

[Child abuse].

Explore the source record for details and available documents.

Age Factors↗
PubMed1981

[Treatment of Wilms' tumor (author's transl)].

Uniform treatment based on the therapeutic approach of the 1st and 2nd US National Wilms' Tumor Study was decided on in March 1976 by paediatricians, surgeons, urologists and radiotherapists in Austria. Wilms' tumour was diagnosed in 34 children between 1 january 1976 an 29 february 1980 (stage I: n = 11, stage II: n = 8, stage III: n = 8, stage IV: n = 7). Parents of two children refused treatments; both children have since died of metastases. Of the remaining 32 children 29 (90.6%) are alive, 10 for more than 4, 15 for more than 3 and 19 for more than 2 years after diagnosis. 21 children are without need of treatment. Three children have died, one due to postoperative complications, one due to haemorrhagic chickenpox, but free of tumour, and one after insufficient treatment. Two of the five children with a recurrence between 2 1/4 to 15 months after diagnosis had been treated inadequately in the initial phase. The tumour free survival rate in 74.2%. Two children with early occurring or recurrent lung metastases have survived for 53 1/2 and 54 months up to now.

Age Factors↗
PubMed1981

[Human fascioliasis in Austria (author's transl)].

Two cases of human fascioliasis in Austria (a 3-year-old boy and a 4-year-ond girl) are reported. A description is given of the clinical picture and the diagnostic procedures employed in the two cases. The importance of the serodiagnosis of fascioliasis is demonstrated, especially in the case of liver flukes; 4 different serological tests, partly of high sensitivity, were performed. After unsuccessful therapy with Resochin both children were treated with dehydroemetine; after a 4-week interval during which no eggs of Fasciola were found in several stool samples from either child, ova were yet again detected in the faces of the boy. The problems concerning the chemotherapy of fascioliasis are discussed.

Antibodies↗