Biomedical subjects
Publications and source records attributed to R Kurz.
Preliminary results are indicating, that disorders of the control of respiration during sleep may be an explanation of the sudden infant death syndrome (SIDS). It should be possible to determine individual risk factors for SIDS by means of improved and extended studies, leading finally to a successful prevention of infant's death.
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Severe bacterial infections accompanying conditions during childhood which demand for surgical intervention mostly are caused by Staph. aureus, E. coli, Klebsiella/Aerobacter or Ps. aeruginosa. 20 patients, 10 of them suffering from a putrid peritonitis, showed a good efficacy of Optocillin (Bay 1-1330), a combination of 6-((R)-2-[3-methylsulfonyl-2-oxo-imidazolidine-1-carboxamido]-2-phenyl-acetamido)-penicillanic acid sodium salt (mezlocillin, Baypen) and 5-methyl-3-phenyl-4-isoxazolylpenicillin (oxacillin, Stapenor), in 85%. Bacteria were eliminated in 79%.
The effect of combined iron-vitamin therapy and iron therapy only was studied in 14 respectively 14 children with hypochromic anemia. Pyridoxalphosphate in serum, activity of red cell glutamic oxaloacetic transaminase and excretion of 4-pyridoxic acid were measured as indices of vitamin B6 nutriture before therapy was started, four and seven days under therapy once more. Erythrocytes, reticulocytes, concentration of hemoglobin were simultaneously counted, whereas serum iron and transferrin have been measured before and after therapy. A group of 22 hematologically healthy children was studied as controls. After iron therapy a decrease of vitamin B6 body pool came off as a consequence of increased requirement of pyridoxalphosphate for heme synthesis. Additional dosage of vitamins compensated the biochemical B6 deficit and had an accelerating effect on heme synthesis.
In 47 patients with acute lymphoblastic leukemia surface markers were evaluated on mononuclear cells of the peripheral blood as well as in some cases on bone marrow lymphocytes. The lymphocytes were characterized by their binding capacity for sheep red blood cells, the demonstration of Fc-receptors, complement receptors as well as surface immunoglobulins. In 6 of 23 untreated patients the blasts bound sheep red blood cells spontaneously (T-ALL), in two of these six cases the lymphoblasts had simultaneously receptors for complement. In a further patients the lymphoblasts had complement- and Fc-receptors. The blasts of 16 of 23 patients were negative in respect to the markers tested (O-ALL). By comparing two groups of patients--one with positive cells, one unreactive--the clinical features differed: the marker positive group showed a predominance of male patients, 5 of 7 patients had a massive mediastinal mass and the remission rate was lower than in the group with positive blasts. 24 patients in remission under maintance treatment had a decreased percentage of rosette forming lymphocytes as well as lymphocytes with surface immunoglobulins and Fc-receptors. There existed some correlation between the percentage of rosette forming lymphocytes and the clinical course: patients with complications had lower percentages of rosette forming lymphocytes than patients with a favourable course.
The percentage of T- and B-lymphocytes was determined by means of surface markers in 190 patients suffering from various lymphoproliferative disease. Characteristic T-cell lymphomas were diagnosed in 8 patients: 2 of these patients suffered from acute leukaemia, 2 from lymphoblastic lymphoma, 3 from mycosis fungoides and one from the Sézary syndrome. The clinical course of the disease in these patients, the clinical picture and the results obtained with various surface markers and with unspecific mitogens are described and discussed.
Within the last 20 years 43 children with Wilms' tumour were seen at the Paediatric Departments of the University Hospitals of Graz, Innsbruck and Vienna. Case histories, clinical details and diagnostic procedures are discussed. Since 1969 19 out of 21 children were treated according to the modern atandard regimen (operation, irradiation and cytostatic therapy for 2 years except in infants with stage I). Since 1969 the survival rate has been higher (17 out of 21 children: 81%) than in the period 1956 to 1968 (7 out of 22 children: 31.8%), when only one child (in stage I) was treated according to current concepts. The better prognosis noted in young infants of this series, as in the literature was due to the earlier stage of the disease in these infants. A further improvement in the survival rate of children with Wilms' tumour should be achieved by earlier diagnosis, thereby ensuring operability, and by cytostatic therapy during the following 2 years. This will only be possible when there is closer cooperation between surgeon, radiotherapist and oncologist. It should be possible to lower the long-term therapeutic complication rate with even more stringent observation measures and with increasing expertise of all doctors concerned.
Renal clearance and intestinal absorption studies were performed to investigate the renal and intestinal handling of phosphate under various conditions in two female patients with VDRR. The two types of transepithelial phosphate transport in the renal proximal tubule and intestinal mucosa are discussed. The defective parathormone sensible transport mechanism is slightly depressed under the influence of parathormone and activated after treatment of vitamin D in one patient, in the other no further inhibition by PTH could be observed. Electrolyte excretion in urine remains stable during treatment with vitamin D 3. Therapy consists of administration of oral phosphate and of pharmacologic doses of vitamin D.
Within the last 20 years 56 children with neuroblastoma were seen at the Paediatric Departments of the University Hospitals of Graz, Innsbruck and Vienna. Case histories, clinical details and diagnostic procedures are discussed. The poor prognosis (only 18 out of the 56 children i.e. 32% are still alive) is due to the high incidence of metastasis (55% of the patients were admitted in stage IV of the disease). Diagnosis within the first year of life, mediastinal localization and histological differentialtion to ganglioneuroblastoma are good prognostic features, whereas therapeutic measures are of less importance. Radical surgery still provides the patient with the best chance of survival, but is rarely feasible (complete primary removal was possible in only 7 out of the 56 patients; in futher 2 patients the tumour proved to be resectable at a 2nd look operation). Cytostatic therapy has not really increased the survival rate. At present improvement in prognosis can only be expected in case of early diagnosis of the tumour.
The Arachnoiditis spinalis and the Pachymeningitis spinals are seldom the cause of neurologic deficit. During infancy both illnesses are extremely rare. One illness with a Arachnoiditis spinalis chronica adhaesiva cystica and, for the first time one with a Pachymeningitis spinalis hypertrophicans could be observed. The signs show parallels to spinal tumors, but when inflammation is the origin, the early starting symptoms of tumors appear relatively late. Symptoms of the radix remain dominant. Myelography shows a typical drop shaped block in the case of Arachnoiditis. Diagnosis can be asured only histologically. Surgery may prevent remaining defects of the spine by decompression and may obtain a permanent success by the removal of local processes. When expanded changes occur, an additional chemotherapy with Prednisolon, perhaps in combination with Endoxan may cure the patient, as shown in our two cases.
Two children with ALL receiving long-term therapy with methotrexate (50 mg/week i.v.) developed severe osteoporosis accompanied by swelling of the joints, gait disturbances and fractures after minimal trauma. Bone scans 85Sr disclosed a decreased input and increased turnover of calcium especially in the most used bones. The data of the calcium phosphorus metabolism are within the normal range. The minimal changes in the biopsy material of the iliac crest may be due to little mechanical use so that this part of the skeleton is not much affected by the osteoporosis. Since the controls receiving the same therapy did not have signs of such severe osteoporosis it is assumed that an additional, till unknown factor may play an additional role in this kind of methotrexate side effect.
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Burkitt's lymphoma occurs endemically in Central Africa, but is also found sporadically in areas with temperate climate. A case of Burkitt's lymphoma found in our area is described. The literature in regard to the clinical symptoms, etiology and treatment of Burkitt's lymphoma is reviewed. At the present time cyclophosphamide is considered to be the drug of choice; it leads to a remission rate of about 90%.
The formation of an Austrian study group for the treatment of leukemia in children with international cooperation is reported. An essential aim is the centralisation of treatment at the 3 Austrian University clinics, the St. Anna children's hospital in Vienna and other children's hospitals particularly interested in the treatment of leukemia. Secondly the group undertakes randomised therapeutic studies, at present on two new variants of the Pinkel treatment scheme. Within the first 9 months 33 cases were included in this study in cooperation with the Zagreb University children's clinic (Doz. TIEFENBACH).
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In a retrospective study 40 children were selected out of 53 cases of septicaemia with thrombocytopenia. They were divided into two coincidentally equally large groups of patients with consumption coagulopathy on the one side and patients with isolated thrombocytopenia without consumption coagulopathy on the other side. Both groups were of comparable age and sex distribution. Two-thirds of the children were under three months. For the differential diagnosis of both groups the activated partial thromboplastin time, the thrombotest, the factor V plasma concentration, the serum concentration of fibrin (fibrinogen) degradation products as well as control coagulation studies can be considered to have the greatest diagnostic value. The results of the study permit the following conclusions: 1. Platelet deficiency in sepsis does not prove the presence of consumption coagulopathy. 2. Consumption coagulopathy and isolated thrombocytopenia differ statistically significantly according to the bacteria cultured from the blood, the circulatory state and the pH of the blood. 3. The finding of thrombocytopenia in a patient with shock, acidosis and gramnegative septicaemia justify the suspicion of consumption coagulopathy.
In condition, it has been found that an acute infantile leukemia in its early stages, manifested itself clinically and morphologically as a reticulosarcome. Through cytochemical examinations, the malignant disease could be identified as an acute myeloid leukemia. The blasts in the tumourous tissues show less differentiation than the leukemic blood and bone marrow cells. The lack of maturation of the blasts is discussed as the cause of tumorlike cell growth.
Pernicious anemia is a rare disease of the pediatric age-group. Two kinds of pernicious anemia are known for this period, the "congenital" and the "iuvenile" form. Both are characterized by a megaloblastic anemia and a deficiency of the intrinsic-factor. The congenital form unusually begins before the second year of age and does not have changes of the mucous membranes of the stomach. The iuvenile form also called "adolescent or auto-immune pernicious anemia" has its beginning in the later childhood and shows like the adult form atrophy of the mucous membrane of the stomach, anacidity and antibodies against the intrinsic factor and parietalcells. A patient with congenital pernicious anemia is presented; the symptoms, diagnosis, differentialdiagnosis and therapy of this disease is reviewed.