Biomedical subjects

R Dickerhoff

Publications and source records attributed to R Dickerhoff.

28 records · Page 2Linked to original sources

PubMedDate not supplied

[Splenic sequestration in patients with sickle cell disease. ].

Splenic sequestration is a potentially life threatening event that is characteristic for sickle cell disease. For reasons unknown a fraction of or even the entire blood volume is trapped in the splenic sinuses within a few hours and thus is no longer available for circulation. The result is splenomegaly, hypovolemia, anemia and extreme reticulocytosis. If the sequestered blood volume is very large the patient goes into fatal hypovolemic shock unless transfused instantly. If the sequestered volume is small there is a chance of spontaneous resolution. The etiology of splenic sequestration is not known. Children with homozygous sickle cell disease (HbSS) are at risk until age 6 years while individuals with compound heterozygous disease (HbSbetaThal, HbSC, HbSD) may develop splenic sequestration even in adulthood. Parents of infants and toddlers with sickle cell disease need to learn how to palpate the spleen in order to detect splenomegaly as early as possible and take the child to the hospital. Splenic sequestration with a drop in hemoglobin of more than 3 g/dl below the patient's usual hemoglobin level is a clear indication for splenectomy regardless of the patient's age as splenic sequestration tends to recur.

Adolescent↗

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↗

PubMedDate not supplied

[Active chickenpox vaccination of children with acute leukemia or other neoplastic diseases].

26 patients with acute leukemia and other malignancies susceptible to varicella were vaccinated during maintenance chemotherapy. Vaccination was done with OKA strain of live attenuated varicella vaccine developed by Takahashi 1974. All recipients showed no adverse clinical reactions. There was no spread of vaccine virus to others. Seroconversion was 94% in seronegative patients. In those having low antibody titers before vaccination in 56% booster effect was demonstrable. None of the seroconverted recipients contracted varicella in spite of documented contact exposure. Vaccine zoster was not observed. The results suggest that in immunocompromised children live varicella vaccination has a protective effect against varicella infection which has a described mortality rate up 7% in this patients.

Acute Disease↗

PubMedDate not supplied

[Manifestations of sickle cell disease in adolescents and young adults. Clinical aspects and therapy references].

In Germany about 300 sickle cell patients are being seen at more than 100 different hospitals. One third of these patients are adolescents and young adults. Since this is a congenital chronic disease, the majority of these teenagers and young adults are being cared for by pediatricians. Sickle cell disease in patients older than 15 years is characterized by the development of chronic organ damage, in addition to the occurrence of acute manifestations of disease such as pain crises, splenic sequestration, aplastic crises and Acute Chest Syndrome. Pediatricians who care for older sickle cell patients have to handle not only internal medicine problems but also to answer questions concerning pregnancy and contraception. In this paper the specific problems of adolescents and young adults with sickle cell disease are presented and suggestions are offered for the care of this group of patients.

Adolescent↗

PubMedDate not supplied

[Successful therapy of local recurrence of congenital mesoblastic nephroma].

A 3 1/2 month old girl was found to have a large abdominal tumor originating in the upper pole of the right kidney. At laparotomy the tumor had infiltrated the perirenal fat, the right lobe of the liver and the diaphragm. Partial nephrectomy was performed and the tumor was completely resected. However, an adequate safety margin could not be achieved. Histology showed a congenital mesoblastic nephroma of the cellular subtype. Postoperatively no chemotherapy was considered necessary. 11 months after diagnosis the patient had an extensive local recurrence with infiltration of the perirenal fat, mesenterium and colon. Complete resection could not be achieved and the tumor was classified as stage III. There was a striking morphological change from spindle cells in the initial tumor to malignant round cells in the relapse specimen. The patient was treated with Vincristine, Actinomycin-D and Adriblastin. Radiotherapy was not given. 38 months after relapse the patient is free of disease and developing normally. Our patient obviously had an aggressive variant of CMN. The significance of the potentially aggressive variant of CMN, atypical mesoblastic nephroma, is discussed and possibilities are suggested for management.

Antineoplastic Combined Chemotherapy Protocols↗

PubMedDate not supplied

[Immune thrombocytopenia in childhood--how much diagnosis and therapy is reasonable?].

Acute and chronic ITP in childhood are both relatively mild diseases that only rarely result in live threatening complications. In most cases diagnostic measures can be limited to a detailed history, thorough physical examination, a complete blood count and evaluation of platelet size on smear. A bone marrow aspirate is only necessary if the diagnosis of ITP is not straightforward. Because of large platelet size and vascular stability bleeding tendency in childhood ITP is mild even with very low platelet counts. 90% of children with acute IPT recover spontaneously within 12 months. Therefore therapy can safely be limited to a few situations: necessary surgical intervention during thrombocytopenia, live threatening bleeding, major trauma. In most instances optimal management of ITP consists in a "wait and see" approach in addition to giving detailed and thorough information to patients or parents about the benign nature of the disease, the likelihood of spontaneous recovery and the importance of avoiding aspirin and contact sports. Controversies in regards to diagnosis and therapy of ITP in childhood are discussed and the various therapeutic possibilities are presented.

Adolescent↗

PubMedDate not supplied

[Pain crises in patients with sickle cell diseases. Pathogenesis, clinical aspects, therapy].

About 70% of all patients with sickle cell disease suffer from pain crises. Pain crises are recurrent episodes of pain that range in severity from mild to severe, usually occur very abruptly and are often localized around joints. Pain crises are caused by vaso-occlusions in the vascular bed of the bone marrow, leading to necrosis, edema and increased pressure. For effective analgesia morphine or morphine analogues are often required. When treating a pain crisis the patient's complaints need to be taken seriously and analgesic therapy should be started promptly with analgesics in proportion to the severity of the patient's pain. With mild pain oral non-opioid analgesics are sufficient, in moderate pain they are given in combination with oral codeine. Severe pain requires IV morphine, also combined with a non-opioid analgesic. Intravenous morphine makes a thorough monitoring of ventilation and level of consciousness mandatory. Sickle cell patients do not become drug dependent if given morphine for adequate analgesia. While bone marrow transplantation has become an accepted treatment modality for sickle cell patients with severe pain crises, treatment with hydroxyurea to increase HbF levels and reduce incidence and severity of pain crises, however, is still experimental.

Analgesics, Opioid↗

PubMedDate not supplied

[Indications for bone marrow transplantation in sickle cell anemia].

Sickle cell disease is a hemoglobin disorder which is characterized by live-threatening organ damage, both acute and chronic. It can be cured by bone marrow transplantation (BMT), mortality and severe morbidity being 10-15%. Severity of clinical manifestations in sickle cell disease varies considerably from patient to patient. As there are as yet no reliable criteria to predict a patient's individual course there is a dilemma when it comes to selecting patients for BMT. The presently existing protocols for BMT in sickle cell patients, Belgian, American and a British, are selecting patients who already had severe clinical problems without, however, showing signs of irreversible organ damage. As the number of sickle cell patients in Germany is very small (ca. 250) we plan to join one of the existing BMT studies.

Adolescent↗

PubMedDate not supplied

[Cerebral infarct and hemorrhage in patients with sickle cell disease].

Two sickle cell patients with cerebral accidents, infarct and bleeding, are presented. Up to 15% of all sickle cell patients suffer cerebral events, 75% of which are infarcts, 25% intracranial hemorrhages. Infarcts occur predominantly in children with a clustering around age 7, while bleeding predominates in adult life. Cerebral events are due to intimal changes, proliferation and finally occlusion of both small and large cerebral arteries. Infarcts present with hemiparesis, aphasia, loss of vision and seizures whereas intracranial bleedings are associated with severe headache and/or loss of consciousness and coma. When cerebral infarct is suspected, magnetic resonance imaging is the diagnostic method of choice. Intracranial bleeding is best diagnosed by computed tomography or angiography. Partial exchange transfusion is indicated in both events to be followed by a chronic transfusion program of as yet undetermined length. Routine magnetic resonance angiogram and/or transcranial doppler sonography in young asymptomatic sickle cell patients may make it possible in the future to detect patients at risk and institute treatment prior to a cerebral accident.

Anemia, Sickle Cell↗