Biomedical subjects
Publications and source records attributed to B Horn.
A short information about importance of fever in general practice. Workup of fever between "watchful waiting" and diagnostic eagerness. Priority of a careful patient history and clinical exam. Management of the febrile patient.
Chronic disease management by primary care physician is one of the outstanding challenges in family medicine. Regular checking of possible interactions between different diagnoses and treatments is of great opportunity. Optimal communication and cooperation between doctors in hospital and primary care is a continuous commitment.
Plague epidemy in 1669 in the valley of Grindelwald (Bernese Oberland/ Switzerland) has been described with acrimony by the priest of the village. Despite of success of modern medicine, numerous problems stay practically unchanged for centuries and history will be an important teacher even in the future.
The major problems with busulfan/cyclophosphamide (Bu/Cy)-containing conditioning regimens are acute toxicities and graft failure. To decrease acute toxicities, we have prospectively evaluated a reduced intensity conditioning (RIC) regimen using targeted dosing of i.v. busulfan, fludarabine, and rabbit ATG (Bu/Flu/rATG) in children with diagnoses that historically would have been conditioned with Bu/Cy regimens. Nineteen pediatric patients were enrolled in the study. The donors included HLA-matched and one antigen-mismatched unrelated volunteers (n = 11), unrelated cord blood (n = 1), and related donors (n = 7). Four patients developed graft failure, which occurred between 1 and 8.5 months post transplant. All four of them underwent a second transplantation and 3/4 are alive without evidence of disease. The mean follow-up of living patients is 29.5 +/- s.d. 11 months. Despite excellent 2-year post-transplant overall survival (89 +/- s.d.7%) and event-free survival (74 +/- s.d.10%), the study was closed prematurely due to high graft failure rate (21%). Receiving a transplant from a mismatched unrelated donor was identified as a risk factor for graft failure. The Bu/Flu/rATG RIC regimen was very well tolerated, resulted in excellent overall survival, and provided sustained engraftment in patients undergoing transplant from matched sibling and unrelated donors. However, it did not provide sustained engraftment in the majority of children with nonmalignancies undergoing mismatched unrelated donor transplants.
BME and vocational training, legal, insurance, financial and social problems. Standards and guidelines, publicity, emergency equipment, emergencies in GP's office and at home, patient information, problems of elderly patient, ethical aspects. Some "key messages".
The use of peripheral blood stem cells (PBSC) for allogeneic transplants in adults has greatly increased. This trend is reflected in pediatrics, where healthy children increasingly are donating PBSC or donor lymphocyte infusion (DLI) via apheresis for use by ill siblings. There is a potential concern that the risks of PBSC collection may differ for pediatric donors. However, no large studies have assessed safety issues in this population. To address this need, we reviewed 218 (213 PBSC, five DLI) collections in 201 normal pediatric donors (8 months to 17 years, median 11.8 years) at 22 institutions in the Pediatric Blood and Marrow Transplant Consortium. Donors received a median of 4 days of growth factor, and mean collection yield was 9.1 x 10(6) CD34+ cells/kg recipient weight. Younger age, days of apheresis, and male gender predicted increased yield of CD34+ cells/kg donor weight. Growth factor-induced pain was mild and reported in less than 15% of patients. Most donors <20 kg (23/25, 92%) required PRBC priming of the apheresis machine. This experience with over 200 collections demonstrates that PBSC collection is safe in normal pediatric donors and desired CD34 cell yields are easily achieved. Younger children utilize more medical resources and children <20 kg usually require a single blood product exposure.
In this review, narcolepsy is described based on personal experience of the author with this disease, especially with imperative day-somnolence and cataplexy. Narcolepsy is frequently underestimated and repressed. The economical (disability) and ethical (traffic accidents) implications of the disease may be significant. Optimal treatment and changes of behaviour are important.
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Acute embolic or local thrombotic ischaemia of the upper limbs can be treated by embolectomy or by endovascular techniques. We report here on the endovascular thrombectomy of acute embolic occlusions of subclavian and axillary arteries in two patients using a rotational thrombectomy device and give an overview about the actual literature. Two female patients, each with a history of multivessel coronary disease and intermittent atrial fibrillation, complained of sudden onset of pain at rest and paleness of the left and right arm, respectively. Duplex ultrasound showed a localized embolic occlusion of the left subclavian artery and the bifurcation of the brachial artery in the first patient and a localized embolic occlusion of the distal right subclavian and axillary artery in the second patient. In the first patient, the left subclavian artery was reopened using a 8F-Rotarex device via the femoral access, while the bifurcation of the brachial artery was reopened by local thrombolysis using 25 mg rt-PA because of the insufficient length of the thrombectomy device of 80 cm. In the second patient, the right subclavian and axillary arteries were reopened using a 6F-Rotarex device. Follow-up examinations before discharge and after 6 months showed normalized perfusion of the arms of both patients.
We retrospectively analyzed data on T- and B-cell reconstitution and infectious complications in 58 children undergoing ABMT, in order to evaluate post-transplant supportive care measures used during the study period. Normalization of T-cell number and lymphocyte proliferative responses to phytohemagglutinin (PHA) and alloantigen (MLC) occurred in two-thirds of children by 6 months post transplant. Normal IgM levels developed in 75% of children by 6 months post transplant. A total of 34 children (59%) developed 39 episodes of infection between neutrophil engraftment and 1 year post transplant. The most common infections included bacteremia, varicella-zoster virus infection and pneumonia, which represented 46, 23 and 9% of infections, respectively. All patients with bacteremia had a central venous catheter in place at the time of infection. Most infections (77%) developed by 6 months post transplant. In this small patient cohort, time to normalization of tests of T- and B-cell function was not significantly different between patients with and without infection. Earlier removal of an indwelling central venous catheter may decrease the risk of bacteremia post transplant. Post-transplant supportive care measures may be discontinued at 6 months post ABMT in most children, as the risk of infection decreases after that time.
We retrospectively analyzed the incidence and risk factors for veno-occlusive disease (VOD) in 83 consecutive children with solid tumors, who underwent autologous blood or bone marrow (BM) transplantation at UCSF between 1992 and 2000. Forty-one patients were diagnosed with neuroblastoma and 42 had another solid tumor (Ewing's sarcoma, soft tissue sarcomas, germ cell tumors, etc). Patients with neuroblastoma were more likely than patients with other solid tumors (ST) to be < or =7 years of age, to have a decreased serum albumin level, and to have received abdominal radiation and surgery prior to transplant. Patients with neuroblastoma received a different conditioning regimen and a purged stem cell product. Twenty patients (24%) developed VOD. VOD was self-limited in 15 (75%) patients and severe in five (25%) patients. Univariate analysis identified the following risk factors for VOD: diagnosis of neuroblastoma (odds ratio 6.1, P < 0.01), abdominal radiation (odds ratio 4.1, P < 0.01), abdominal surgery (odds ratio 4.1, P < 0.01), and age < or =7 years of age (odds ratio 3.3, P = 0.02). Disease status at transplant, intensity of previous chemotherapy, conditioning regimen, progenitor cell source, ALT, AST, albumin level, renal function prior to transplant, or use of amphotericin, growth-factor or heparin during transplant, did not affect the incidence of VOD. On multivariate analysis, only the diagnosis of neuroblastoma remained significant (odds ratio 7.8, P = 0.03). Larger studies of patients with neuroblastoma are necessary in order to confirm our findings and better define the risk factors for VOD development in neuroblastoma patients.
BACKGROUND: To evaluate the efficacy, safety and limitation of a new 6F-compatible nitinol stent (Dynalink(TM). MATERIAL AND METHODS: We treated 50 patients (17 women, 33 men, mean age 72 +/- 8 years) by implanting 80 Dynalink(TM)-stents into 75 vessels during a 6-month period. Target lesions were: iliac artery: 25, femoral artery: 31, popliteal artery: 16, femoro-popliteal bypass: 5, subclavian vein: 3. Preinterventional Rutherford classifications: Class 1 : 3 legs (5 %), class 2 : 27 legs (51 %), class 3 : 16 legs (31 %), class 4 : 2 legs (4 %), class 5 : 5 legs (9 %). 40 % stents each were implanted ipsilateral, 60 % cross-over. RESULTS: All interventions were successful regardless of a sometimes anatomically difficult access to the lesion. The device was characterised by a high flexibility and radial force and the stent did not shorten. COMPLICATIONS: One distal stent dislocation during placement occurred, no puncture site complication. The mean diameter stenosis was reduced from 91 +/- 10 % (75 - 100 %) to 4 +/- 8 % (0 - 30 %). The ankle-brachial index was improved from 0.46 +/- 0.22 to 0.75 +/- 0.23 (p < 0.001). Post-interventional Rutherford classifications: Class 0 : 43 legs (81 %), class 1 : 5 legs (4,5 %), class 5 : 5 legs (4,5 %). CONCLUSIONS: The new 6F-sheath compatible nitinol stent is characterised by a good flexibility, radial force, and a lack of shortening. By the reduction of the diameter of the device to 6F, the potential risk of a local bleeding complication may be reduced and 6F sealing devices will be usable. Disadvantages are the 0.018 inch guide-wire lumen and the limited stent sizes.
AIM: In coronary heart disease, the surgical therapy of choice is the construction of an artenal bypass of the left anterior descending artery (LAD) using the internal thoracic artery 8ITA). We define age dependent normal values for the ITA such as diameter of the lumen (LD), peak systolic (Vs) and diastolic (Vd) flow velocity and the ratio of these two values (SDR) measured by duplex ultrasound. Furthermore, the modification and pathological changes of the Doppler curve after minimal invasive bypass of the LAD (MIDCAB) are described. MATERIAL AND METHODS: 96 people, age 18 - 87 years, subdivided into 6 age groups were examined by duplex to define the normal values of the ITA, and 55 patients (mean age 63 +/- 10 years, 48 men, 7 women) were evaluated after MIDCAB-surgery to describe the postoperative modification of the Doppler curve. The examinations were performed using a 4 - 7 MHz linear ultrasound transducer in the right and left 1. or 2. intercostal space parasternally. RESULTS: In all 96 people, the ITA was detectable on both sides presenting a typical bi- or triphasic Doppler flow profile. A linear age dependent increase in the LD of 1.95 +/- 0.15 mm (right ITA) and 1.93 +/- 0.27 mm (left ITA) respectively to 2.65 +/- 0.48 mm and 2.55 +/- 0.43 mm was found. No significant side difference was found for Vs and Vd, nor were there any age dependent differences for Vs. The SDR showed an age dependent linear increase on the right side from 3.5 +/- 1.1 to 6.1 +/- 2.2, p < 0.0012, and on the left from 3.9 +/- 1.2 to 6.7 +/- 1.7, p < 0.0001. Postoperatively, the Doppler spectrum was modified into a mono- or biphasic Doppler curve with a reduced Vs and an increased Vd resulting in a significantly decreased SDR on the left side compared with the right side (1.3 +/- 0.8 vs. 5.4 +/- 2.0, p < 0.00001). Three patients with angiographically proven graft failure had an SDR of 2.6 - 5.2 (mean 3.8), as opposed to a value of < 2.0 in case of a patent bypass. An SDR > 2.0 has a sensitivity of 100 % and a specificity of 97 % in the detection of a haemodynamically relevant (>70 %) ITA-graft-stenosis. CONCLUSIONS: With rising age, there is an increase in the LD of the ITA and the peripheral resistance, expressed as SDR. After MIDCAB surgery the former triphasic Doppler flow curve changes into a mono- or biphasic curve corresponding to the coronary blood flow. An SDR >2.0 is a strong indicator of bypass failure.
BACKGROUND: Evaluation of the acute and long-term results after recanalization of thrombotic occlusions of infra-aortic native arteries, stented arteries and bypass-grafts using a rotational thrombectomy device (8F Straub-Rotarex TM). MATERIALS AND METHODS: From July 2000 to February 2002, in 98 patients (64 % male, mean age 66 +/- 9 (range 47 to 90) years, mean duration of occlusion 31 +/- 33 (range 0 to 140) days, mean occlusion length 21 +/- 11 [range 2 to 40] cm) 100 vessel recanalizations were performed. 42 % of the interventions were performed during the first 14 days after the onset of the symptoms (acute occlusions). These patients were classified according to the after the TASC protocol modified SVS/ISCVS-classification: Class I: n = 22 (52 %); class lla: n = 13 (32 %); class llb: n = 6 (14 %); class III: n = 1 (2 %). Subacute and chronic occlusions were classified according to Rutherford (58 %): Stage 1: n = 5 (9 %); stage 2 and 3: n = 39 (68 %); stage 4: n = 6 (10 %); stage 5: n = 8 (13 %). RESULTS: Primary success rate was 96 % (ipsilateral interventions 99 %, cross-over 40 %). The restenosis rate after a mean follow-up of 13 +/- 4 months was 33 % for native arteries (group 1), 74 % for instent-recanalizations (group 2) and 86 % for bypass-graft occlusions (group 3). 3 % severe complications occurred: two amputations below the knee after unsuccessful recanalizations of a native artery and a bypass-graft, respectively. One death because of multiorgan failure in patient who was already in cardiac shock before the successful intervention. Further 16 complications could be solved periinterventionally. The ancle-brachial index was significantly improved during follow-up (from 0.37 +/- 0.19 to 0.82 +/- 0.22 before discharge, 0.70 +/- 0.21 after 12 months, p < 0.001). CONCLUSION: The Straub-Rotarex TM device is useful in the treatment of acute and subacute thrombotic occlusions of infra-aortic arterial and bypass-graft occlusions, with promising long-term results in native vessels but unchanged high restenosis of stented arteries and bypass-grafts.
A distinct form of autosomal recessive T-B- severe combined immunodeficiency disease occurs with a high frequency among Athabascan-speaking Native Americans (SCIDA), including Navajo and Apache Indians from the southwestern US and Dene Indians from the Canadian Northwest Territories. The SCIDA gene has been linked to markers on chromosome 10p although its identity and role in the pathogenesis of this disease are unknown. We report our experience in treating 18 Navajo and Dene children with SCIDA between 1984 and 1999; 16 underwent bone marrow transplants (BMT). All children were symptomatic within 2 months of birth, had the T-B- NK(+)SCID phenotype and 67% presented with oral and/or genital ulcers. Three children had evidence of maternal engraftment prior to transplant. Two children died shortly after diagnosis. Three children required more than one BMT and 12 are alive with T cell reconstitution at a median follow-up of 7 years. Three children developed normal B cell immunity, two of whom received ablative conditioning therapy with either radiation or busulfan. Three of the four children who died received therapy with either radiation or busulfan and two of eight long-term survivors who were also recipients of cytotoxic chemotherapy have failed to develop secondary teeth. These results demonstrate the efficacy of BMT in treating infants with this distinct form of SCID, although B cell reconstitution remains a problem even with HLA-matched donors. Without conditioning, T cell engraftment is likely when closely HLA-matched donors are used. With T cell depletion of haplocompatible marrow, conditioning with immunosuppressive therapy may be necessary; however, children with SCIDA who were treated with intensive immunosuppressive and myeloablative therapy had a poor outcome.
Nonpeptide orally active angiotensin II type 1 (AT1) receptor antagonists are the most specific means presently available to block the renin-angiotensin enzymatic cascade. Six of these drugs have already been licensed in Europe and in the United States for the treatment of high blood pressure, and additional candidates are in the pipeline. The World Health Organisation has also recently endorsed their use for this condition. Inasmuch as AT1 receptor antagonists have proven themselves the equals of angiotensin converting enzyme inhibitors with respect to antihypertensive efficacy, but demonstrated better safety profiles, this class of drugs may be considered to be a qualitative improvement in the treatment of essential hypertension. Interestingly, the six agents now on the market diverge considerably with respect to their pharmacokinetic and pharmacodynamic properties, although it is not certain whether such differences are clinically relevant. A considerable number of large, multicentre trials are in progress to ascertain the possible longer-term organoprotective effects of these substances on cardiovascular morbidity and mortality. Because of their noteworthy safety record to date, and simple once-a-day dosage regimen, AT1 receptor antagonists have the potential to improve compliance in patients with chronic hypertension.
BACKGROUND: To evaluate the efficacy, safety and limitations of a new rotational thrombectomy device (Straub-Rotarex) in clinical practice. MATERIAL AND METHODS: The Straub-Rotarex catheter is a new, wire-guided rotational thrombectomy device for the treatment of acute and subacute occlusions of the femoro-popliteal arteries. Over a 6-month period, 28 patients (64% male, mean age 69 +/- 9.5, 51-91 years) with 31 legs, mean duration of occlusion 4.4 +/- 4 (0-20) weeks, mean occlusion length 22 +/- 11 (5-40) cm were treated with the device, 5 of them in a cross-over technique. Target lesions: Aortic-femoral bypass, common iliac artery, external iliac artery, common femoral artery: 1 each, superficial femoral artery: 23, popliteal artery: 17. Initial stage of claudication: IIa: 6%, IIb: 72%, III: 16%, IV: 6%. RESULTS: Primary success rate: 90% (ipsilateral: 100%, cross-over: 40%). Stage of claudication after intervention: I: 85%, IIa: 7%, III: 4%, IV 4%, one amputation. 3-months follow-up: stage I: 86% (n = 24), IIa: 14% (n = 4), one femoro-popliteal bypass. Restenosis rate 18%. 6-months follow-up: stage I: 56% (n = 9), IIa: 31% (n = 5), IIb: 13% (n = 2). Restenosis rate 56%. COMPLICATIONS: 32% (5 perforations, three cases of embolism after PTA, one wire-induced dissection, one retroperitoneal bleeding coming from the puncture site). CONCLUSIONS: The new device is a useful tool for the treatment of (sub)acute long-distance occlusions of the SFA and popliteal artery and in-stent restenosis as well in antegrade technique. Main complications are perforations. Cross-over interventions can only be done in special cases.
We here report on a new HLA-A19 allele (A*2416) found in a German kidney recipient. Serological class I typing revealed HLA-A11,19 without clear definition of the A19 split antigen. As with serology, polymerase chain reaction (PCR)-based typing also revealed inconclusive results. We therefore sequenced the gene from the 5' flanking region through the 3'-end of exon 4 of this allele after haplotype-specific PCR amplification. The sequence analysis revealed a new HLA-A allele which is identical to A*3101 with the exception of the 3' half of exon 2 which is identical to the common A9 alleles. The phylogenetic analysis constructed with the nearest-neighbor algorithm and based on exons 1-4 or introns 1-3 clearly indicated, that A*2416 belongs unequivocally to the A19 lineage.