Biomedical subjects

T Kühne

Publications and source records attributed to T Kühne.

44 records · Page 3Linked to original sources

PubMed1991

Maternal vegan diet causing a serious infantile neurological disorder due to vitamin B12 deficiency.

We present a 9-month-old exclusively breast-fed baby of a strict vegetarian mother who had excluded all animal proteins from her diet. The patient's symptoms included dystrophy, weakness, muscular atrophy, loss of tendon reflexes, psychomotor regression and haematological abnormalities. Biochemical investigations revealed severe methylmalonic aciduria and homocystinuria in the patient, slight methylmalonic aciduria in the mother and low concentrations of serum vitamin B12 in both patient and mother.

Adult↗

PubMed1991

Repeated polymerase chain reaction complementary to other conventional methods for early detection of HIV infection in infants born to HIV-infected mothers.

The efficacy of a polymerase chain reaction (PCR) method for early detection of human immunodeficiency virus (HIV) in infants at risk for HIV infection was assessed. The PCR method was added to the routine laboratory test programme in these patients in 1988. PCR was performed in a total of 26 children at risk (age range 2 days to 58 months), including 17 infants born to HIV-infected mothers, who were followed up clinically from the time of birth for a mean period of 23 months (range 6 to 54) in a prospective study. Twelve children were PCR-positive. Eight had AIDS, ARC or symptoms suggestive of HIV infection. All these patients had at least one culture positive for HIV (6/8) and/or one positive serum p24-antigen test (5/8). One child was repeatedly PCR positive, but asymptomatic as well as virus- and antigen-negative. Three asymptomatic children with a single positive PCR result were PCR negative in subsequent tests. Fourteen children with negative PCR did not show clinical or immunological signs suggestive of HIV infection. Their cultures for HIV and antigen-p24 assays were negative. It is concluded that in addition to clinical and immunological parameters PCR is a useful technique for diagnosis of HIV infection in infants born to HIV-infected mothers. However, in case of negative HIV cultures and/or serum p24-antigen tests, single positive PCR results in asymptomatic patients must be interpreted with caution and should be confirmed by repeated tests.

Base Sequence↗

PubMed1983

Evidence against a scanning model of RNA splicing.

A cloned rabbit beta-globin gene was modified by introducing a DNA fragment containing the 5' splice region of the large intron upstream or downstream of its natural counterpart. Analogous constructions were carried out with the 3' splice region. The genes were linked to SV40 DNA, transiently expressed in HeLa cells and the transcripts analyzed by S1 mapping. In all cases, the splice site further removed from the intron was utilized to the complete exclusion of its counterpart. This finding argues persuasively against a simple scanning model of RNA splicing, in which the splicing enzyme(s) attaches at the 'donor' spliced region and moves along the intron until it encounters the closest 'acceptor' splice region. A model compatible with the currently known facts is presented.

Base Sequence↗

PubMedDate not supplied

Thrombolysis of prosthetic tricuspid valve thrombosis with human recombinant tissue plasminogen activator in an adolescent.

Prosthetic heart valve thrombosis is associated with a high mortality. Traditionally, thrombectomy or valve replacement is performed. Thrombolysis offers a promising alternative to surgery. Usually, streptokinase and urokinase are the preferred agents for thrombolysis; however, human recombinant tissue plasminogen activator (rt-PA) is increasingly used. Thrombosis of prosthetic valves in children and adolescents is rare and experience of thrombolysis for obstructed valves is limited. We report the successful lysis of a thrombosed prosthetic tricuspid valve in an adolescent using rt-PA. The outcome of our patient supports the assumption that rt-PA represents an adequate therapeutic option for thrombolysis of obstructed prosthetic heart valves in children and adolescents.

Adolescent↗

PubMedDate not supplied

Newly diagnosed idiopathic thrombocytopenic purpura in childhood: an observational study.

BACKGROUND: Diagnosis and management of idiopathic thrombocytopenic purpura (ITP) have been based primarily on expert opinion and practice guidelines rather than on evidence. We have used a registry to prospectively survey the presenting features and the diagnostic evaluation and management practices used for children with ITP worldwide. METHODS: We used the Intercontinental Childhood ITP Registry which had been widely advertised. 209 physicians from 136 institutions in 38 countries participated by submitting data for each of their newly diagnosed patients. Data from 2031 children with ITP was registered between June, 1997, and May, 2000, and we analysed 6-month follow-up data from 1496 children. FINDINGS: There was a peak in occurrence of childhood ITP during spring and a nadir in the autumn. Mean initial platelet count was 15.4x10(9)/L (SD 19.7). 1447 (73%) of 1976 children were admitted to hospital. Initial management consisted of no drug treatment in 612 (31%), intravenous immunoglobulin in 576 (29%), corticosteroids in 651 (33%), or both in 137 (7%) patients. Intracranial haemorrhage was reported in two patients. INTERPRETATION: The variable approaches to management of childhood ITP demonstrate the need for prospective clinical trials, which should be feasible within such a study group.

Adolescent↗

PubMedDate not supplied

Pediatric hematology and oncology at the University Children's Hospital, Basel, Switzerland.

The department offers the management and research of benign and malignant pediatric hematology and oncology at a comparable European standard. It serves as a referring department for BMT, thoracic and visceral pediatric surgery, and pediatric orthopedic surgery and is equipped with all facilities of modern oncology and surgery. Psychooncology is a continuing interest at our department and may help children and their families to better understand and accept their situation and to receive the support necessary for coping with the threat of malignant diseases. Our department aims at practicing a highly sophisticated communication culture essential for a multidisciplinary approach to the child with cancer, representing a central condition for the success of the treatment of malignant diseases.

Child↗

PubMedDate not supplied

Continuous infusion of von Willebrand factor and factor VIII after elective heart surgery in a 12-year-old girl with von Willebrand disease type 3.

The continuous infusion of von Willebrand factor (VWF) in a 12-year-old girl with type 3 von Willebrand disease is described. The patient had elective heart surgery with cardiopulmonary bypass for closure of her atrial septum defect. The surgical procedure lasted 3 h. A presurgical bolus followed by a postoperative continuous infusion of factor VIII/VWF concentrates was administered. During the continuous infusion of clotting factors, stable plasma levels of hemostasis and avoidance of dangerously low levels of factor concentrates were achieved. No peri- or postsurgical complications occurred. Continuous infusion of clotting factors allows constant and hemostatic factor concentrations to be maintained with the possibility of dose titration and adjustment.

Cardiac Surgical Procedures↗

PubMedDate not supplied

All-trans retinoic acid as an alternative to chemotherapy in the treatment of acute promyelocytic leukemia.

A 12-year-old girl with acute promyelocytic leukemia has been treated with conventional chemotherapy. The patient remained in complete remission for a year when the first bone marrow relapse occurred. Since reinduction chemotherapy was rejected by the parents, an alternative treatment with oral all-trans retinoic acid was administered. A second complete remission was achieved within 100 days. After 14 months a second bone marrow relapse occurred. All-trans retinoic acid and the combination with interferon-alpha failed.

Child↗