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Biomedical subjects

U Martinowitz

Publications and source records attributed to U Martinowitz.

107 records · Page 6Linked to original sources
PubMed1986

The short foot syndrome--an unfortunate consequence of neglected raised intracompartmental pressure in a severe hemophilic child: a case report.

The vast majority of bleeding episodes in hemophilia occur in the musculoskeletal system. When bleeding occurs, within a closed compartment, the possibility of neurovascular compromise must be suspected. We present an interesting case where this danger was overlooked in a young hemophiliac boy and resulted in bone growth retardation and permanent deformity.

Adult↗
PubMed1986

Intracranial hemorrhage in patients with hemophilia.

Intracranial hemorrhage (ICH) is a life-threatening complication of hemophilia. Seven of the 288 hemophiliacs living in Israel suffered eight episodes of ICH during the years 1972 to 1982. All episodes occurred in hemophilia A patients, with a higher incidence among patients with factor VIII inhibitor. Diagnosis was confirmed by computed tomographic scan in seven of the eight episodes. Four of the 7 patients died despite adequate factor replacement and supportive therapy, probably due to a conservative and hesitant neurosurgical approach. The correction of factor VIII to hemostatic level alone is inadequate in the majority of cases, and there is sudden deterioration in the patient's condition and death. Operation is strongly recommended when no improvement is noted within a few hours.

Adult↗
PubMed1986

Effect of cardiac operation on platelets.

The effect of extracorporeal circulation on platelet count and size (mean platelet volume) was studied in 65 patients (nine bleeders and 56 nonbleeders). In addition to the above, in 20 of the patients platelet aggregation response to adenosine diphosphate, collagen, and ristocetin was measured. Platelet counts dropped postoperatively both in the bleeder and in the nonbleeder groups. The difference between them was not significant. However, the bleeders had a significantly lower mean platelet volume (7.7 +/- 0.84 versus 8.68 +/- 1.1 fl) and lower volume percentage of platelets in whole blood (0.075% +/- 0.02% versus 0.116% +/- 0.04%) (p less than 0.05) than the nonbleeders. None of the bleeders had a volume percentage of platelets in whole blood higher than 0.095%. All 20 patients studied for platelet functions had an abnormal postoperative aggregation response to adenosine diphosphate, collagen, and ristocetin. Three patterns of disturbed response to ristocetin were observed: grade I, delayed onset (14 patients); grade II, incomplete aggregation (five patients); and grade III, total lack of aggregation (one patient). All patients with delayed-onset response to ristocetin had a normal bleeding time, whereas the six patients with grade II and III responses had prolonged bleeding times and three of them had clinically significant bleeding. Factor VIII procoagulant activity, factor VIII-related antigen, factor VIII-ristocetin cofactor, and factor VIII two-dimensional electrophoresis were found normal, which suggests that the von Willebrand-like reaction to ristocetin observed in this study was caused by a defect in platelet membrane rather than by factor VIII changes.

Adolescent↗
PubMed1986

Platelet size and mass as an indicator for platelet transfusion after cardiopulmonary bypass.

Platelet count, mean platelet volume (MPV), and plateletcrit (PCT) were studied in 51 patients after cardiopulmonary bypass (CPB). MPV was significantly lower in 10 patients who developed postoperative bleeding (bleeders) compared to 41 with no significant bleeding (nonbleeders) (7.7 +/- 0.86 vs 8.5 +/- 1.2 fl, p less than .05). Postoperative platelet count was significantly lower in the group of bleeders (93.3 +/- 22.4 vs 127.5 +/- 43 X 10(9)/liters, p less than .02). A cutoff point of MPV or platelet count that would include bleeders and exclude nonbleeders could not be found due to the large overlap between the two groups. However, such a cutoff point does exist for PCT (PCT = total platelet mass). PCT was significantly lower among the bleeders (0.072 +/- 0.02% vs 0.108 +/- 0.036%, p less than .05) and a cutoff point of PCT less than 0.1% included all the bleeders and excluded 65% of nonbleeders. The low PCT and bleeding tendency can be corrected by platelets transfusion. In 15 patients (eight bleeders and seven nonbleeders) with low postoperative PCT (0.078 +/- 0.014), transfusion of 10 platelet units increased platelet count from 101 +/- 32 to 169 +/- 22 X 10(9)/liter, increased PCT to 0.128 +/- 0.2%, and stopped bleeding in all bleeders. A finding of PCT less than 0.1% after CPB is a clear indication for platelet transfusion in patients who develop post-CPB bleeding. This supports the observation that large platelets are more active than smaller ones, and that PCT, rather than PLT counts, predicts the risk of bleeding in patients with thrombocytopenia.

Adolescent↗
PubMed1986

Hemophilic arthropathy resulting in a locked shoulder.

A 14-year-old boy with severe hemophilia had a swollen immobile left shoulder joint. A roentgenogram showed a severely deformed humeral head that had interlocked onto the glenoid ring. After manipulation, the motion promptly returned. Incongruency of the shoulder in adolescence poses a serious therapeutic problem.

Adolescent↗
PubMed1985

Mechanism of warfarin potentiation by amiodarone: dose--and concentration--dependent inhibition of warfarin elimination.

Potentiation of the anticoagulant-effect of warfarin by amiodarone was studied in 30 patients. Thirteen received both drugs concurrently, and 17 received warfarin alone and the combination sequentially. Warfarin doses were adjusted to maintain the prothrombin time between 25-30% of control and its kinetics were compared to those in 20 control patients who received warfarin alone. Potentiation occurred in 28/30 patients, presenting as a 35%-65% reduction in the required dose of warfarin, and was correlated with the dose of amiodarone (r = 0.77, p less than 0.01). The free warfarin fraction was not affected by amiodarone (1.8% vs 1.6% in the controls). Warfarin clearance was lower in amiodarone-treated patients than in the controls (1.4 vs 3.1 ml/min, p less than 0.01) with similar plasma concentrations (1.5 vs 1.2 micrograms/ml) despite administration of lower doses (23.3 vs 39 mg/week respectively). The amiodarone concentration was significantly correlated with the warfarin concentrations independent of the effect of amiodarone on the dose of warfarin. Amiodarone hat no effect on prothrombin other than through its actions on the dose and plasma concentration of warfarin. The mechanism of the amiodarone-warfarin interaction is pharmacokinetic through dose - and concentration - dependent inhibition of warfarin elimination.

Adult↗
PubMed1985

Leg-length inequality in hemophilia. An interesting case report.

A hemophiliac child with flexion contracture of the knee and symptoms of leg length difference was treated with a shoe lift on the affected side, which failed to aid the problem. Further investigation revealed that the affected limb was actually longer than the contralateral side. The chronic synovitis of the knee that had resulted in the contracture also caused supplementary bone growth around the distal femoral epiphysis, resulting in the lengthening of that leg.

Child, Preschool↗
PubMed1982

Ilio-psoas hematoma--its detection, and treatment with special reference to hemophilia.

Pain around the hip joint and femoral neuropathy in a patient with a bleeding coagulopathy should alert the physician to the possibility of an iliopsoas haematoma. Unfortunately this diagnosis is often missed. Clinically, reduced hip motion, a mass in the iliac fossa and alteration in the motor and sensory function of the femoral nerve may be noted. If the pain is on the right side, appendicitis must be excluded. Ultrasound is the diagnostic aid of choice, being more sensitive than computerized tomography or radioisotope investigations. Treatment should be gradual, gentle and protracted to avoid recurrent bleeding episodes.

Hematoma↗
PubMed1982

Haemophiliac hands--a three year follow-up study.

The total absence of literature regarding the affects of haemophilia on the hand prompted this three year study at the Israel National Haemophilia Centre. The hands of all haemophiliacs were examined and X-rayed at the time of their annual check-up. Apart from a number of established Volkmann's contractures in elderly patients and multiple bone cysts of the carpus, surprisingly little pathology was found. When considering the extensive damage done to the musculoskeletal system by this haemarthritic pathogenesis it is astounding how the hand, with its multiple small joints, constant movement and trauma, escapes significant damage.

Adult↗
PubMedDate not supplied

Treatment of patients with antiphospholipid antibodies during pregnancy.

Most authors agree upon the causal association between antiphospholipid antibodies [lupus anticoagulant (LAC) and/or anticardiolipin antibodies] and adverse pregnancy outcome. Placental insufficiency, caused by thrombosis, infarction and maldevelopment, is thought to be the main cause of fetal loss in patients with LAC. Therapy given thus far to prevent fetal loss can be divided into (1) immunosuppression by corticosteroids, azathioprine, or intravenous gamma globulin (IVGG), (2) anti-aggregants to overcome imbalance of thromboxane/prostacycline production in patients with LAC, and (3) anticoagulants to neutralize the possible impairment of clotting inhibitor systems. Different therapeutic success rates have been reported by various authors who used the same combination of therapy. We report the results of different therapy regimens in 154 pregnancies in 31 women with LAC. These patients suffered from SLE with LAC or from APLA syndrome and experienced either recurrent miscarriages or thromboembolic phenomena in the past. With no therapy there were seven (6.8%) live births and 95 (93.2%) failures. Various combinations of corticosteroids, anti-aggregants and anticoagulants were used for treatment. Of 52 treated pregnancies, 27 (51.9%) were successful. Sixteen (69.1%) of 23 pregnancies treated by all three modalities ended in live births. Four of these successful pregnancies occurred after failure of treatment by prednisone and anti-aggregants only. In order to minimize osteoporosis caused by the combination of steroids and heparin, we have used warfarin in the second trimester and have lately substituted low molecular weight heparin for heparin. In the absence of a therapeutic schedule predicated on a large prospective study, therapy during pregnancy in patients having LAC should be individualized according to their obstetric and medical history.(ABSTRACT TRUNCATED AT 250 WORDS)

Anticoagulants↗
PubMedDate not supplied

Prevalence of a CCR5 gene 32-bp deletion in an Israeli cohort of HIV-1-infected and uninfected hemophilia patients.

OBJECTIVE: The recently discovered connection of chemokines and their receptors to HIV pathogenesis, and the description of the 32-bp deletion in the CCR5 gene (delta 32 CCR5), led to heightened excitement and numerous reports regarding their role in HIV transmission and disease progression. The populations in most of these reports, except for one, consisted of homosexual men. Our objective was to investigate the significance of delta 32 CCR5 in hemophilia patients in Israel. STUDY DESIGN/METHODS: We have determined by polymerase chain reaction (PCR) the prevalence of delta 32 CCR5 in 34 HIV-seropositive Israeli patients with hemophilia A and compared them with a control group of 42 HIV-seronegative hemophilia patients. RESULTS: Thirteen heterozygotes were identified among the 76 hemophilia patients tested (allelic frequency, 8.5%), 5 (14.7%) among the HIV-seropositive patients, and 8 (19%) among the noninfected. CONCLUSIONS: No protective advantage to delta 32 CCR5 heterozygosity was seen as far as infection with HIV is concerned. However, a trend of a slower progression to AIDS in delta 32 CCR5 heterozygotes compared with wild-type homozygotes may be apparent, although no absolute correlation could be made.

Cohort Studies↗
PubMedDate not supplied

Glanzmann's thrombasthenia: the use of autologous fibrin glue in tooth extractions.

Three teeth were extracted in three sessions in a 15-year-old girl with Glanzmann's thrombasthenia who suffered in the past severe bleeding and required platelet transfusions. The extraction sites were treated locally with autologous fibrin glue in adjunct of tranexamic acid swish and swallow rinses. No systemic platelet infusion was employed as a preventive measure. No postoperative bleeding occurred.

Adolescent↗