PubMed Health⌕ Search

Biomedical subjects

R Dickerhoff

Publications and source records attributed to R Dickerhoff.

At least 19 recordsLinked to original sources
PubMed2000

Impact of bone marrow transplantation for symptomatic sickle cell disease: an interim report. Multicenter investigation of bone marrow transplantation for sickle cell disease.

Fifty children who had symptomatic sickle cell disease received matched sibling marrow allografts between September 1991 and March 1999, with Kaplan-Meier probabilities of survival and event-free survival of 94% and 84%, respectively. Twenty-six patients (16 male, 10 female) had at least 2 years of follow-up after transplantation and were evaluated for late effects of transplantation and for its impact on sickle cell-related central nervous system (CNS) and pulmonary disease. Patients ranged between 3.3 and 14.0 (median, 9. 4) years of age and had a median follow-up of 57.9 (range 38-95) months after transplantation. Among 22 of 26 patients who had stable donor engraftment, complications related to sickle cell disease resolved, and none experienced further episodes of pain, stroke, or acute chest syndrome. All 10 engrafted patients with a prior history of stroke had stable or improved cerebral magnetic resonance imaging results. Pulmonary function tests were stable in 22 of the 26 patients, worse in two, and not studied in two. Seven of eight patients transplanted for recurrent acute chest syndrome had stable pulmonary function. Linear growth measured by median height standard deviation score improved from -0.7 before transplantation to -0.2 after transplantation. An adverse effect of busulfan conditioning on ovarian function was demonstrated in five of seven evaluable females who are currently at least 13 years of age. None of the four males tested had elevated serum gonadotropin levels. These data confirm that allogenic bone marrow transplantation establishes normal erythropoiesis and is associated with improved growth and stable CNS imaging and pulmonary function in most patients. (Blood. 2000;95:1918-1924)

Adolescent↗
PubMed1995

Association of Hb S/Hb lepore and delta beta-thalassemia/Hb lepore in Sicilian patients: review of the presence of Hb lepore in Sicily.

The hemoglobin (Hb) lepore-Boston is a beta-globin structural variant, produced in a reduced amount and formed from the fusion of N-terminus delta-(residues 1-87) and C-terminus beta-chains (residues 116-146). This type of fusion protein is quite common in Southern Italy (Campania, Calabria, and Sicily). We report here the hematological and hemoglobin data on 96 unrelated Sicilians with Hb lepore trait. Particularly interesting are the subjects where Hb lepore occurs with Hb S or Sicilian type delta beta-thalassemia. In these individuals, striking features are clinical variability and different hematological pictures. These observations underscore the importance of thalassemia screening in these geographic areas, such as Southern Italy, principally Sicily, where the mutations in globin gene clusters are especially prevalent. Moreover, as from the second half of the last century, owing to high migratory flux from Sicily to Northern Europe, North and South America, and Australia, the Hb lepore, as well as other hemoglobin variants, have become prevalent, making the identification of the heterozygotes a problem of general interest.

Adolescent↗
PubMed1995

Enumeration of platelets by multiparameter flow cytometry using platelet-specific antibodies and fluorescent reference particles.

The correct enumeration of platelets is still an elusive matter. This is mainly due to the fact that commercial instruments which are used for platelet counting cannot discriminate platelets from other cellular particles and precipitates that cause similar signals. Visual (chamber counting) methods are still frequently used in routine laboratories to verify low automated platelet counts (< 50 x 10/l) despite obvious technical and statistical drawbacks. The following report shows how platelet counts can be measured by multiparameter flow cytometry with the help of reference particles (fluorescent latex beads) and platelet-specific antibodies i.e. anti-GPIIb/IIIa(CD41a), anti-GP Ib-alpha (CD42b) and anti-GP IIIa (CD61). The linearity of this method was highly satisfactory and the observed imprecision was within acceptable limits. At a platelet concentration of 10 x 10(9)/l the coefficient of variation (CV, n = 10) ranged from 5.3% (PCV = 0.456) to 5.6% (PCV = 0.148). Accuracy was evaluated by comparing results to the ICSH-selected method for platelet counting. The correlation of both methods was significant (P < 0.005) and Passing-Bablok's linear regression analysis showed no systematic differences between the two methods. Comparisons of this new platelet counting technique were also performed with routine visual methods, automated blood analysers (Technicon H-1, Sysmex E-5000) and a different flow cytometric method using only forward and side light scatter properties of platelets for their discrimination. The linear correlation of all methods was significant (P < 0.01) at platelet concentrations above 50 x 10(9)/l. At lower platelet concentrations, our new platelet counting technique correlated significantly only with the visual and the forward/side scatter methods.(ABSTRACT TRUNCATED AT 250 WORDS)

Antibodies↗
PubMed1993

[Monitoring pregnant patients from risk countries with sickle cell disease and thalassemia. Clinical aspects, screening and prenatal diagnosis].

At the present time, about 3.5 million people from Turkey, Greece, Italy, the Middle East, Africa and Asia are living in Germany. They are potential carriers of beta-thalassaemia and haemoglobinopathies such as sickle cell disease. These diseases are new for most of us and represent a challenge to physicians, taking care of these patients. Not only do we have to learn about the clinical problems of homozygous patients and how to handle them, we also have to become acquainted with the problems related to the heterozygous carrier stage. The large number of asymptomatic pregnant carriers of beta-globin anomalies is a particular challenge for obstetricians. They need to identify carriers through haemoglobin electrophoresis screening, inform the carrier about the meaning of being a carrier, screen the woman's partner, refer for genetic counselling and suggest and explain prenatal diagnosis in case the partner is also a carrier. There is as yet no cure for thalassaemia and sickle cell disease, except for bone marrow transplantation in a few selected cases. Therefore, prenatal diagnosis presents a valuable method of preventing severe chronic diseases. Screening does not only allow genetic counselling, the information gained has also clinical implications for carriers of beta-thalassaemia. In this paper a summary is given of the pathophysiological and clinical features of thalassaemia and sickle cell disease and molecular biology methods to diagnose thalassaemias and sickle cell disease are discussed. In addition, a screening programme for pregnant women from countries at risk is suggested to enable physicians to give optimal care and initiate prenatal diagnosis.

Anemia, Sickle Cell↗
PubMed1993

[Hemoglobin SD disease].

A 7 year old Kurdish girl presented with a cerebral infarction and a resulting hemiparesis following a blood transfusion. Examination of the blood film suggested sickle cell anaemia. However a simultaneously carried out haemoglobin electrophoresis showed haemoglobin S and haemoglobin D. The diagnosis of haemoglobin SD disease was thereby established. This kind of haemoglobin disorder usually shows a mild clinical manifestation. According to our knowledge such serious cases have not been published before. With this disease the main emphasis is on the prevention of recurrent cerebral infarctions bay a long-term transfusion programme whereas the effects of the cerebral infarction are treated in the usual way.

Anemia, Sickle Cell↗
PubMed1991

[Disorders of consciousness as an initial symptom of intestinal invagination].

Two infants with intussusception were presented with lethargy and vomiting. Initially, the classical abdominal manifestations of the disease were missing. In order to avoid a delay of the correct treatment, intussusception should be considered in infants with altered consciousness and ultrasonography of the abdomen should be included in the diagnostic work up.

Consciousness Disorders↗
PubMed1990

[Sickle cell disease and pregnancy].

Three million out of 4.5 million foreigners in Germany are from countries, where sickle cell anaemia is found. Today, this disease occurs in Germany with sufficient frequency and this makes it imperative for every physician to be familiar with it. Most of the sickle cell patients today attain the reproductive age. During pregnancy, sickle cell patients need very thorough medical care, because of the high foetal and maternal morbidity in this patient group. The main features of sickle cell disease are discussed and a survey of complications in pregnant sickle cell patients is presented. Suggestions are made regarding contraception and prenatal care.

Anemia, Sickle Cell↗
PubMed1989

Qualitative and quantitative changes in platelets after coronary-artery bypass surgery may help identify thrombotic complications and infections.

We studied the effect of coronary-artery bypass surgery on blood cells and platelets. Hematological parameters of eighty-three patients were measured by an automated cell counting and sizing analyzer. Sampling time was from 24 h prior to 10 days after surgery. During this time leukocytes and platelets showed characteristic changes in numbers and size, whereas red blood cells revealed no typical modifications. Even though it seems clear that changes of hematological parameters occur after bypass surgery, it is important to be aware of the actual extent of such changes. Therefore the data of 50 patients who had had no post-operative clinical complications were combined to generate diagrams of those parameters that had changed in a characteristic fashion. The diagrams showing average changes, and 99% confidence intervals in mean platelet volume and platelet count were able to identify seven (out of 7) cases with complications up to 48 h before clinical signs were apparent. Complications ranged from mild (3 cases with infections) to severe (4 cases with thrombosis, embolic thrombosis and/or reinfarction). Diagrams showing changes in leukocyte parameters were able to identify only two cases with infections. Even though the number of cases is yet small, the results suggest that surveillance of platelet parameters may be useful in postoperative care. Furthermore, this study was able to confirm the recent findings of Trowbridge and Martin that an abnormal increase in platelet volume distribution width and low platelet counts found in patients with coronary heart disease may serve as good indicators for the prethrombotic state and the risk of myocardial infarction.

Adult↗
PubMed1987

Acute aphasia and loss of vision with desferrioxamine overdose.

Reversible aphasia, loss of vision, bradycardia, and mild hypotension occurred in a 6-year-old girl with aplastic anemia and transfusion-related iron overload after inadvertent increase of her intravenous desferrioxamine drip. Signs and symptoms were reversed promptly by intravenous fluids, suggesting that desferrioxamine, when given as a sudden intravenous bolus, may upset vasoregulation. Three possible mechanisms are discussed that could explain this phenomenon. High-dose intravenous desferrioxamine ought to be given by pump infusion only, in order to avoid accidental overdose.

Anemia, Aplastic↗
PubMed1986

Malignant gastric sarcoma--diagnosis by ultrasound and endoscopy.

A 14-year-old boy was presented with a mass in the epigastrium. The tumor developed 13 years after removal of a neuroblastoma of the left adrenal gland. Therapy of the primary tumor had included irradiation and chemotherapy. Diagnosis of the gastric tumor was made by ultrasound and endoscopy. A total gastrectomy was performed showing a malignant undifferentiated sarcoma. Primary malignant tumors of the stomach such as gastric teratoma of the newborn [4], leiomyosarcoma [6], malignant lymphoma and carcinoma [1] are very rare in childhood. As cancer is treated more aggressively with radiation and cytostatics, more therapy-induced-tumors, including gastric sarcoma, must be expected in children.

Adolescent↗
PubMed1985

Selective hemisplenectomy for Hodgkin's disease.

Schellong's recommendation to do a selective splenectomy in Hodgkin's disease supported by our retrospective histological findings in biopsies of lymph nodes and spleens of 25 patients. In addition, we recommend a hemisplenectomy instead of a splenectomy in cases of involvement of the lymph nodes at the pancreatic tail and at the hilus of the spleen. Hemisplenectomy, which is not a more difficult or more time-consuming operation, provides more security in judging an invasion of the spleen.

Biopsy↗
PubMed1985

[Therapeutic experiences with high-dose ARA-C and L-ASP].

Five patients, 4 with ALL, 1 with AML received 8 cycles of HD-ARA-C (Capizzi protocol). One complete remission was achieved, 2 patients died shortly after cycle 1 and could not be evaluated. Two patients responded partially. Toxicity was considerable.

Adolescent↗
PubMed1985

Active immunization against varicella of children with acute leukaemia or other malignancies on maintenance chemotherapy.

Twenty-six patients with acute leukaemia and other malignancies susceptible to varicella were vaccinated with the Oka-strain live attenuated varicella vaccine during maintenance chemotherapy. All recipients showed no adverse clinical reactions. There was no spread of vaccine virus. Seroconversion was 94% in seronegative patients. Among those having low antibody titres before vaccination, a booster effect was demonstrable in 56%. None of the seroconverted recipients contracted varicella despite documented contact exposure. No case of herpes zoster occurred. The results suggest that, in immunocompromised children, live varicella vaccination has a protective effect against varicella infection which may result in a mortality rate of up to 7% in these patients.

Acute Disease↗
PubMedDate not supplied

[Transient myeloproliferation and acute myeloid leukemia in infants with Down's syndrome].

Transient neonatal myeloproliferative disorders (TMD's) indistinguishable from acute leukaemia by clinical and morphological criteria have been described in neonates with Down's syndrome. To analyse its clinical significance, 10 infants under 1 year of age presenting with Down's syndrome and the morphological picture of acute myelogenous leukaemia were reviewed. 3 of these children had true AML leading to death after 2, 8 and 11 months. In the other 7 children the diagnosis TMD was suggested as spontaneous or in one case interferon-induced remission occurred within 4 to 25 weeks after diagnosis. The interferon-treated patient died of SIDS at the age of 11 months. Another one of the TMD children developed fatal erythroleukaemia at the age of 2 years. Regarding initial clinical and haematological parameters, TMD was indistinguishable from true congenital leukaemie. In all patients classification according to the FAB criteria was difficult, as mainly undifferentiated or poorly differentiated myeloid blasts were seen, sometimes with erythro- or megakaryocytic features. Because of the difficulties in the differential diagnosis of TMD and true AML it is recommended to delay specific cytostatic therapy in neonates with Down's syndrome, until definite progression of the leukaemic process is observed or cytogenetic analyses suggesting true AML are available.

Antigens, CD↗
PubMedDate not supplied

[Visceral leishmaniasis. Personal observation and review of epidemiology, clinical aspects and therapy].

A 5 year old German girl contracted visceral leishmaniasis during a vacation in Spain, either 32 or 20 month prior to the manifestation of disease. She presented with fever, hepatosplenomegaly and pancytopenia. A bone marrow aspirate proved the diagnosis. Therapy with a pentavalent antimony drug brought about immediate improvement. Visceral leishmaniasis has to be suspected in individuals with fever, hepatosplenomegaly and pancytopenia who have resided in endemic areas (Mediterranean countries, India, East Africa, South America) during the previous years. If untreated, visceral leishmaniasis runs a fatal course. Therefore, early diagnosis by morphological and serological means and specific therapy with pentavalent antimony drugs are mandatory.

Animals↗