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W Kaulfersch

Publications and source records attributed to W Kaulfersch.

At least 37 records · Page 2Linked to original sources
PubMed1987

[Changes in the vertebrae as an initial symptom of leukemia].

Back pain due to vertebral changes as early feature of acute lymphocytic leukemia (ALL) in childhood has been infrequently reported. There are 8 previously described patients with similar clinical and laboratory data, suggesting a biologically unique subset of ALL. Characteristic findings of this rare primary manifestation of leukemia are lack of significant organomegaly or lymphadenopathy, normal or low white blood cell count with predominance of lymphocytes and rarely circulating lymphoblasts, normal platelet count, uric acid and lactate dehydrogenase values. In the following report we make a further attempt to confirm the hypothesis of a subset of ALL, demonstrating two additional patients with characteristic features of ALL presenting with vertebral changes and low leukemic burden.

Child, Preschool↗
PubMed1986

Immunoglobulin and T-cell receptor gene rearrangement and expression in human lymphoid leukemia cells at different stages of maturation.

The use of probes to genes (IG and TCRB) encoding immunoglobulins (IG) and the beta chain of the T-cell antigen receptor (TCRB), respectively, have become a sensitive means to assess clonality and lineage in lymphoid malignancies. It has become apparent that some individual cases show rearrangements of both IG and TCRB genes. In an attempt to more accurately define cell lineage we have analyzed cells from patients with B- or T-cell leukemia (n = 26) at various stages of maturation with probes to two additional TCR genes, TCRG and TCRA (encoding the TCR gamma and alpha chains, respectively), as well as the IG heavy chain joining region (IGHJ) and TCRB genes. On Southern blot analysis, the mature T-cell leukemia cells studied had rearranged TCRG and TCRB while IGHJ remained as in the germ line. The mature B-cell leukemia cells studied had rearranged IGHJ with germ-line TCRG and TCRB. These data suggest that, in the majority of more mature leukemias, cells have rearranged IG or TCR genes but not both. In contrast, cells from five of nine precursor B-cell leukemia patients and cell lines from one of four precursor T-cell leukemia patients had rearranged both IGHJ and TCR genes. TCRG and TCRB mRNAs were expressed in the cells of precursor T- but not B-cell leukemia patients studied. The spectrum of leukemia cells studied within the T-cell series permitted an assessment of the order of TCR gene rearrangements. Two of 13 patients had cells with germ-line TCRG and TCRB, 2 patients had cells with rearranged TCRG alone, and the remainder had cells with rearranged TCRG and TCRB. TCRG and TCRB mRNAs were expressed in precursor T-cell leukemia cells, whereas TCRB and TCRA were expressed in mature T-cell leukemia cells. These results parallel observations from mouse studies on gene expression and support the view of a hierarchy of TCR gene rearrangements in T-lymphocyte ontogeny. TCRG genes are rearranged first, subsequently TCRB genes are rearranged, followed by TCRA gene activation.

Cell Differentiation↗
PubMed1986

[Treatment of stage IV neuroblastoma with high-dose melphalan and autologous bone marrow transplantation following in vitro preliminary treatment of the bone marrow with the active cyclophosphamide derivative Asta Z-7654].

The case of a 4 year 8 months old boy with neuroblastoma of unknown primary, metastatic to the bone and to the bone marrow is presented. After achieving a partial remission with six cycles of conventional chemotherapy, the patient was given supraconventional chemotherapy (melphalan 220 mg/m2 bolus i.v.) in an effort to eliminate residual disease. Prior to the administration of the drug, 560 cc of autologous bone marrow, morphologically free of tumor was harvested (total 110 X 10(8) nucleated cells) and concentrated to a mononuclear cell fraction with a total of 10 X 10(8) cells. After in vitro purging with the stable metabolite of 4-hydroperoxycyclophosphamide ASTA Z 7654 (40 micrograms/2 X 10(7) mononuclear cells/ml), the mononuclear cell suspension was retransfused 10 hours following the application of high dose melphalan. Hemopoietic reconstitution was delayed with a platelet count reaching 70,000/microliter only after seven months. At the time of this writing (20 months after diagnosis and 16 months after autologous bone marrow transplantation) there is no evidence for active disease according to the bone scan and multiple bone marrow biopsies. In view of the dismal prognosis of patients with neuroblastoma, stage IV it is recommended that further patients should be treated with a slightly modified protocol of the cooperative austrian neuroblastoma study.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1986

[Intracranial hemorrhages in mature and premature infants. Computer tomography and clinical follow-up studies].

The findings of CT-follow-up-studies on a group of 94 infants, who had suffered intracranial hemorrhage, were correlated with the clinical and neurological outcome. Purpose of the retrospective study was to work out prognostic criteria dependent on the typ and location of the hemorrhage. Among the infants with intraventricular hemorrhage, there was a direct relationship between the severity of the hemorrhage and major handicaps on the one hand and the mortality rate on the other hand. Neurologic sequelae were noted in 16.5% of the infants with tentorial hemorrhages, in 56% of the infants with intracerebral hemorrhages and 37.5% of the infants with subarachnoid hemorrhages. Our results indicate, that the outcome of infants with intracranial hemorrhages is not dependent on the typ and severity of the bleeding only, but is also influenced by additional factors, like perinatal asphyxia.

Brain Damage, Chronic↗
PubMed1985

[Ultrasound diagnosis of malignant diseases in pediatrics].

Grey scale abdominal ultrasound was used in a total of 146 children for primary diagnostic evaluation of abdominal masses and for follow-up of patients with malignant diseases. Of 93 patients examined for a suspected abdominal mass, 6 showed to have an intraabdominal tumour. In each case the site of tumour origin could be ascertained by ultrasound. The remaining 87 patients showed no pathological findings and have been tumour-free at routine follow-up studies. Sonography proved to be also useful in the follow-up of patients with malignant diseases. Local recurrence or metastases could be ruled out on the basis of sonographic findings alone in 32 out of 35 surgically treated patients, in 3 children with non-resectable tumours, changes in tumour size during radio- and chemotherapy could be studied. Diagnostic ultrasound was also of great importance in the assessment of complications after surgery, radio- and chemotherapy. So the early detection of hydronephrosis in 2 patients with treated nephroblastoma prevented a damage of the remaining kidney. Due to the high accuracy of sonography, invasive diagnostic methods could be restricted to a small number of patients in whom ultrasound failed to provide adequate information.

Abdominal Neoplasms↗
PubMed1985

Hematological and oncological indications for splenectomy in children.

The most common hematologic and oncologic indications for splenectomy in childhood are hereditary spherocytosis, chronic idiopathic thrombocytopenic purpura, hypersplenism, and Hodgkin's disease. Because of the increased incidence of septic complications after splenectomy, benefits to be gained from the operation should be weighed against the risks. A retrospective study was done on the charts of 42 consecutive children with hematologic and oncologic disorders, who underwent splenectomy between 1967 and 1982. The incidence of septic complications after splenectomy was 12%; sepsis, however, only occurred in patients with severe underlying diseases (three patients with Hodgkin's disease, one patient with systemic lupus erythematosus, and one patient with chronic pseudo-malignant immunoproliferation). In contrast, none of the patients who were splenectomized for other reasons (mainly hereditary spherocytosis and chronic immune thrombocytopenic purpura) had a septic complication. Two patients with end-stage Hodgkin's disease (5%) experienced fatal septic complications. Although splenectomy is well established for diagnostic and therapeutic considerations in patients with Hodgkin's disease, not all of them might benefit from this operation, and studies with a more limited approach to splenectomy might prove to be of the same therapeutical value.

Adolescent↗
PubMed1985

[Computed tomographic and clinical follow-up studies in intraventricular hemorrhage].

The investigation comprises 41 children with intraventricular hemorrhage (IVH). The CT-findings were divided into 4 groups, according to Papile. A CT and clinical follow-up study was performed on the survivors. The grade of IVH correlated with the rate of mortality and with neurological deficits. It is demonstrated in 3 cases that the CT-scan is only part of a prognostic aspect which is remarkable influenced by clinical events.

Cerebral Hemorrhage↗
PubMed1985

[Supportive measures in aggressive cytostatic therapy].

Over the past years significant progress has been made in the treatment of childhood cancers due to newer and more intensive chemotherapeutic regimes. However, with the increased intensity of chemotherapy, more treatment related complications are seen, requiring also more aggressive supportive care. The major complications of the cytotoxic treatment are bone marrow aplasia, immunosuppression, vomiting, anorexia and weight loss and supportive measures as adequate blood component supply, prophylaxis, recognition and effective treatment of infections as well as parenteral hyperalimentation are corner stones of modern cancer therapy. Blood sampling, application of blood products or intravenous drugs and continuous parenteral nutrition is easily performed using a central venous line. Our experience with the continuous venous access of central lines in patients receiving aggressive cytotoxic treatment did not show a higher incidence of infectious complications but had significant advantages in the supportive care.

Anemia, Aplastic↗
PubMed1984

[Immunosuppressive treatment of severe aplastic anemia with high-dose methylprednisolone and antithymocyte globulin in 2 patients].

This article describes the treatment of two patients with severe aplastic anaemia (SAA) by means of high-dose methylprednisolone ( HDMP ) and antithymocyte globulin (ATG). A complete normalization of the haematological data was obtained in one patient, which has persisted now for 10 months. ATG and HDMP seem to provide an alternative mode of treatment of SAA to bone marrow transplantation if there is no histocompatible bone marrow donor available.

Adolescent↗
PubMed1984

[Myoclonic encephalopathy (Kinsbourne syndrome)].

Three children with myoclonic encephalopathy (Kinsbourne's disease) are described, in which one of them was shown to have ganglioneuroblastoma. Symptoms were opsoclonus, polymyoclonia of the striated muscles and cerebellar ataxia. Treatment consisted in corticosteroids and adrenocorticotropic hormone respectively in all patients, the patient with ganglioneuroblastoma also had a resection of the tumor. All patients responded to therapy, however recurrence of myoclonia and of the opsoclonus were seen after discontinuation or reduction of the corticosteroid dose, as well as following the course of intercurrent viral infections. Neurologic symptoms eventually disappeared after 3 1/2-5 1/2 years, however in two children behavioural abnormalities and disorders of speech and cognitive development remained.

Adrenal Cortex Hormones↗
PubMed1983

[Life-threatening hypernatremia caused by faulty preparation of fully adapted infant formula].

Two male newborns developed severe life threatening hypernatremia with serum sodium levels of 181 and 196 meq/l respectively. Both children were fed a two-to fivefold concentrated powdered infant formula since birth. Shortly before admission diarrhea developed in both patients. The children were admitted during the third week and fourth week respectively, of their lifes with clinical signs of severe hyperosmolar dehydration. In both, treatment of shock was instituted and was followed by rehydration therapy. Great care was taken to lower serum sodium by not more than 15 meq/l/day in order to prevent cerebral edema. Inspite of these measures, the first patient developed cerebral seizures 5 hours after initiation of fluid replacement. The patient was intubated, and measures aimed at prevention of cerebral edema were started (hyperventilation). The eventual outcome was satisfactory, on follow up the patient showed no signs of persistent cerebral damage. In the second patient the same management was started from the beginning and no cerebral problems occurred. Restoration of stable body functions with a normal electrolyte status was achieved within five days.

Brain↗
PubMed1983

[Pseudo-Perthes' disease caused by acute lymphatic leukemia].

A two year old boy was seen in the orthopedic clinics because of typical symptoms of Legg-Perthes disease, a scintigraphy with Technetium99m showed a distinct deficiency of nuclear activity in the femoral head which is characteristic of the early stage of Legg-Perthes disease. A routine blood count lead to the diagnosis of acute lymphoblastic leukemia. The boy was treated according to the Austrian cooperative leukemia protocol and complete remission was achieved. No orthopedic treatment of the femur head necrosis was done, after eight weeks of treatment with multiagent chemotherapy the boy started to walk again and subsequently became free of all symptoms of Legg-Perthes disease. A scintigraphy done eight weeks after the initial scintigraphy showed that the deficiency of radionuclear activity of the femoral head was nearly vanished. This case illustrates the variability of bone involvement in acute lymphoblastic leukemia, which often is the most prominent symptom at an early stage of the disease.

Antineoplastic Agents↗
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

[Biphasic splenic rupture in a hemophilic child (author's transl)].

This report describes a biphasic splenic rupture following trauma in a 12 1/2 years old boy with the severe form of hemophilia B. Immediately after the trauma the boy was treated with only one single dose of factor IX concentrate. He stayed free of symptoms for 9 days until finally severe abdominal bleeding occurred.

Child↗