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Biomedical subjects

H H Brackmann

Publications and source records attributed to H H Brackmann.

126 records · Page 7Linked to original sources
PubMed1987

FcR-mediated clearance in thrombopenic and non-thrombopenic patients with hemophilia A and possible relation of thrombopenia to HIV seropositivity.

As morbidity of thrombopenia in hemophilia A patients is increasing, the pathogenetic influence of the reticuloendothelial system (RES) was measured using autologous anti-Rh0(D)-coated erythrocytes (EA) in 17 patients with or without thrombopenia. Mean survival of EA in patients was reduced to 53% of healthy controls (53.2 +/- 46.1 min vs 100.5 +/- 12.2 min; patients vs controls, mean +/- S.D.). Survival of EA was not significantly different either in thrombopenic vs non-thrombopenic nor anti-HIV (human immunodeficiency virus) positive vs negative patients. Thrombopenia, elevated serum IgG and circulating immune complexes were related to the presence of anti-HIV antibodies. EA survival was also decreased in the absence of anti-HIV antibodies. This indicates activation of RES by a mechanism different from retroviral infection by HIV (1).

Acquired Immunodeficiency Syndrome↗
PubMed1987

Virus safety of beta-propiolactone treated plasma preparations. Clinical experiences.

Sterilization of human plasma with beta-propiolactone and UV-irradiation (cold sterilization) has been shown to be effective for a number of common pathogenic viruses. We have published data on the hepatitis safety of cold sterilized factor IX concentrates (PPSB) in healthy volunteers. This study has now been extended to include 6 virgin hemophilia B patients, who have been treated with cold sterilized PPSB for a period of up to 5 years. None of these volunteers or patients exhibited clinical symptoms or laboratory data indicating the transmission of either viral hepatitis (B or NANB) or acquired immunodeficiency syndrome (AIDS). Hyperimmunoglobulin (HIg) preparations from cold sterilized plasma, offering protection against hepatitis B and NANB are also safe regarding HTLV-III virus transmission, even though these preparations may contain HTLV-III antibody titers up to 1:1000.

Acquired Immunodeficiency Syndrome↗
PubMed1986

Hemophilic patients with an inhibitor to factor VIII treated with high dose factor VIII concentrate. Results of a collaborative study for the evaluation of factor VIII inhibitor titer, recovery and half life of infused factor VIII.

The long term treatment of hemophilic patients with an inhibitor to Factor VIII has been difficult although some success with immunosuppressive agents has been reported. Eighteen hemophilic patients, mainly from Bonn in Germany, but also from other countries, have completed a high dose Factor VIII treatment in an attempt to reduce their inhibitor titer and induce "immune tolerance" to Factor VIII. Plasma samples from the 18 patients collected before and after infusion of 50 units Factor VIII/kg body weight were sent to five laboratories to evaluate inhibitor titer, Factor VIII recovery and half life. This collaborative study demonstrated a close correlation from one laboratory to another concerning inhibitor titration and Factor VIII recovery. The conclusion of the study is that all patients treated with this protocol showed an undetectable or low inhibitor titer against Factor VIII indicating that they can be efficiently treated with Factor VIII.

Adolescent↗
PubMed1982

[Hemophilic pseudotumors].

A review of the literature was made with special interest in morphology, pathogenesis and treatment of hemophilic pseudotumors. 110 reports of hemophilic pseudotumors were evaluated and compared with 25 own cases. The separation according to the inflicted site and the primarily involved anatomical structure revealed three types of hemophilic pseudotumors: 1. Intramuscular hematomas which enter secondarily into the periosteum. a) In the diaphyses of long tubular bones (femur 49, lower leg 14, humerus 4, forearm 2). b) In the iliac bone (37). 2. Intraspongious cysts in short cancellous bones (calcaneus 14, talus 5, scapula 3). 3. Direct subperiostal hematomas in the phalanges and metacarpals in the hand (thumb 5, finger 1, metacarpal 1). Concerning treatment, there is to state, that in general a conservative approach (substitution of the missing clotting factor and immobilization) is promising. The operative indications are demonstrated and determined, using case reports.

Adult↗
PubMed1980

[Regional anaesthesia in haemophiliacs (author's transl)].

Since the middle of 1976 spinal and peridural anaesthesia are also being used for orthopaedic surgery of the lower extremities in haemophilic persons, the most important pre-requisites for the use of these techniques are: that it is a case of genuine haemophilia, that the deficiency is fully made up before and during anaesthesia and that the most important coagulation parameters are closely watched in co-operation with the Hemophilia Centre during the entire period. 46 haemophiliacs were given spinal and epidural anaesthesia via a catheter. A great advantage of these long-acting techniques is that less analgesics are needed during the postoperative period. Continuous peridural anaesthesia ensures freedom from pain without interfering with motoricity. The treatment of contractures of the joint by active and passive exercises has thus a better chance of success. Provided factors VIII/IX were kept normal there have so far not been any haemophilia-related haemorrhages into the spinal canal or cord or neurological disturbances that could be attributed to these anaesthetic techniques.

Adolescent↗
PubMed1978

[Haemophilic pseudo-tumours and iliac haematomas: radiological and clinical findings (author's transl)].

Between 1971 and 1977, 648 patients with haemophilia were seen; of these 38 had pseudo-tumours and 163 iliac haematomas. Amongst the latter 153 were cured by the administration of the appropriate factor. In ten the symptoms persisted. In the early stages the diagnosis cannot be made radiologically. Large haematomas produce indirect evidence of their presence by displacing the bladder. Particularly with small haematomas, computer tomography has proved a reliable and accurate diagnostic measure. Without treatment, a haemophilic pseudo-tumour may develop. Its radiological differential diagnosis includes malignant and benign bone tumours, inflammatory and parasitic disease. Therapy includes conservative measures (factor concentrates) or operative intervention (removal of the haematoma).

Bone Cysts↗
PubMed1977

Reconstructive surgery in the lower limb in hemophiliacs.

During the years 1973-76, we performed 76 operations on the lower limbs of hemophiliacs in an attempt to correct the disability due to intraarticular hemorrhages. The ways in which rehabilitative surgery can improve function and retard further damage are discussed in the light of the pathogenesis of disability due to intraarticular and intramuscular hemorrhage. Three particularly successful operative procedures are discussed: 1) supracondylar correction of knock-knee, 2) intertrochanteric correction of coxa valga, and 3) evacuation of iliacus hematoma in patients with paralysis of the femoral nerve.

Adolescent↗
PubMed1977

[Orthopedic problems in hemophilia. Pathophysiology and results of operations (author's transl)].

During the last 3 years we have treated over 400 patients with severe hemophilia A or B of all ages. They were looked after by the Institute for Experimental Hematology and Blood-Transfusion of the University at Bonn, for prevention, rehabilitation and operation. The specific pathophysiology of disability of the hemophiliac is shortly outlined, the chances of operative rehabilitation and their indications are discussed. The results show that--with experience--extensive operative rehabilitation can be achieved also in hemophiliacs.

Adolescent↗
PubMed1975

[Treatment of hemophilia A with actor VIII concentrate in urological operations and injuries of the urinary tract].

A congenital tencency to bleeding can nowadays be successfully treated by substitution of the missing clotting factors. One patient with a slight hemophilia A had a renal calculus removed without complications by pyelotomy, using an antihemophilic Factor VIII concentrate. A 16-year-old boy with a severe form of hemophilia A suffered a renal trauma with retroperitoneal hemorrhage. He was treated conservatively with Factor VIII concentrate and partial function of the kidney was retained. Substitution therapy for operative or conservative procedures and the requirements and expense are discussed.

Adolescent↗
PubMedDate not supplied

Male-to-female transmission of HIV in a cohort of hemophiliacs--frequency, risk factors and effect of sexual counseling.

The incidence of male-to-female transmission of HIV infection was studied in a population of 198 sexual partners of hemophiliacs who tested HIV positive since 1984. The follow-up observation period was 1987-1992. Transmission occurred in 20 (10%) cases. The analysis of risk factors for transmission was performed in a subgroup of 57 hemophiliacs with seronegative sexual partners as compared to eight transmitters. Transmitters showed a significantly more advanced immune depletion at enrollment as well as at the end of the observation period. Furthermore, transmitters had a more advanced disease at the end of the study (75% vs. 29% CDC IV; p < 0.01). Also virus cultures were more frequently positive in the transmitters than in the non-transmitters (71% vs. 42%). Regular sexual counseling was offered to all couples. After 1987, no new seroconversions were detected. However, two seroconversions in female partners of hemophiliacs outside the initial study population were observed. Both transmissions occurred during a period of severe clinical and immunological deterioration. This study shows that sexual partners of HIV-infected hemophiliacs with more advanced disease are at higher risk of infection with HIV. The frequency of male-to-female transmission of HIV in long-term monogamous sexual relationships practicing safer sex is low. Overall, disease awareness and counseling for safer sex seem to be effective in reducing transmission rates.

Adult↗
PubMedDate not supplied

Immune thrombocytopenia in severe hemophilia A treated with high-dose intravenous immunoglobulin.

Five patients with severe hemophilia A receiving long-term treatment with commercial factor VIII concentrates developed severe immune thrombocytopenia (ITP, platelet counts less than 20 X 10(9)/l). Concomitantly, they presented with a marked elevation of serum IgG concentrations (mean, 2364 mg/dl;range, 1712-2954 mg/dl). In four patients, the T helper to suppressor cell ratio was below 1. Treatment with high-dose intravenous immunoglobulin (IgG, 7s) at doses of 0.2g (n = 2) or 0.4g (n = 3) per kg of body weight on 5 consecutive days was effective immediately. The bleeding tendency ceased and platelet counts rose transiently. In three cases, treatment was repeatedly effective and patients underwent uneventful splenectomies. Thus, high-dose IgG therapy may serve as a life-saving agent in patients with severe hemophilia complicated by ITP.

Adolescent↗