Partial splenectomy in Gaucher's disease

In 11 children with hypersplenism due to Gaucher's disease, partial splenectomy was planned with the aim to prevent the development of postsplenectomy sepsis and also to slow the advance of the disease in the rest of the reticuloendothelial system by permitting continuing accumulation of the beta-glucocerebroside in the remaining splenic tissue. In seven children, partial splenectomy was performed successfully, the weight of the splenic tissue removed ranging from 400 to 3,680 g. The postoperative course was uneventful and the average duration of hospitalization was 12 days. In subsequent follow-up, isotope scanning demonstrated continuing growth of the splenic remnant and there were no episodes of postsplenectomy sepsis nor evidence of increased accumulation of beta-glucocerebroside in the liver or bones. These children showed a marked improvement in the growth curve and dramatic improvement in the hematologic picture. Of the four remaining children, in two, partial splenectomy was followed by complete removal of the remaining spleen due to necrosis, whereas in two, total splenectomy was performed since the huge spleens were extensively infarcted. Our experience suggests that partial splenectomy is the treatment of choice in the management of young patients with hypersplenism due to Gaucher's disease.     

Bladder dysfunction and neurosyphilis

Neurosyphilis today is a rare problem. We describe a man who presented with organic brain syndrome, psychosis and incontinence, and diagnosis was neurosyphilis with resultant bladder dysfunction. Urodynamic studies defined the voiding dysfunction as detrusor areflexia with a positive bethanechol test. This case reminds us of the necessity of obtaining a test for venereal disease to rule out neurosyphilis in patients with idiopathic voiding dysfunction.     

Correlation of functional parameters derived by equilibrium radionuclide angiocardiography and left ventricular size in patients with old myocardial infarction

A total of 131 patients with old (over 6 months) myocardial infarction (MI) and 18 normal subjects underwent equilibrium radionuclide angiocardiography at rest (rERNA). The following rERNA parameters were assessed: left ventricular ejection fraction (LVEF), peak ejection rate (PER), peak filling rate (PFR), regional wall motion and a left ventricular size index. The patients with old MI were divided into four groups (I to IV) according to increasing left ventricular (LV) size, and the behaviour of the numerical parameters (LVEF, PER, PFR) was evaluated in each group. LVEF proved to be the most sensitive numerical parameter of overall LV performance. PFR decreased significantly from group I to group III but not from group III to group IV, suggesting that for extreme degrees of left ventricular enlargement some compensatory mechanism acts to prevent a too large fall in LV compliance. The effects of the site of the previous MI on LV performance were also evaluated. Both LV size and performance were least affected by postero-inferior MI. The LVEF was, however, a better predictor of LV size than the site of the MI.     

Comparison of primary orchiectomy with oestrogen therapy in advanced prostatic cancer. A 2-year follow-up report of a national, prospective prostatic cancer study

Two hundred and seventy-seven patients with advanced prostatic cancer were treated by either orchiectomy or oestrogen. During the 2-year follow-up period, the response to treatment was considered more favourable in the oestrogen group, and this response was particularly emphasised in patients with poorly differentiated tumour and metastases at the time of diagnosis. Further evaluations included the cardiovascular side effects of oestrogen therapy.     

Accuracy of an anthropometric estimate of the muscle and bone area in a transversal cross-section of the arm

To standardize the maximal static force (Fo) of the arm flexors, the accuracy of an anthropometric method for estimating the mid-arm cross-sectional muscle and bone area (MBA) was investigated. This was done by comparing the anthropometrically determined area (MBA.A) with the area measured by means of computerized tomography (MBA.S). In the same way, the accuracy of Heymsfield's equations (Heymsfield et al., 1982) for predicting MBA (MBA.H) from anthropometric measures was tested. MBA.A was significantly larger than MBA.S, the relative difference increasing with the thickness of the subcutaneous fat layer. This difference was attributed to a 27% underestimation of the fat layer thickness as measured with the skinfold caliper. Women being fatter than men, this caused the standardized maximal static force (Fo/MBA) to be lower in women than in men. MBA.H was 12% smaller than MBA.S. This may have been due to a difference in the way of measuring the arm circumference between the present authors and Heymsfield et al.     

Localized osteolysis in stable, non-septic total hip replacement

We are reporting four cases of extensive, localized bone resorption adjacent to a rigidly anchored, cemented total hip replacement. None of these hips showed evidence of infection on clinical, bacteriological, or pathological evaluation. The tissue from the regions of osteolysis showed sheets of macrophages and foreign-body giant cells invading the femoral cortices. Abundant methylmethacrylate particulate debris was present in the tissues, but polyethylene wear debris was absent. The histological appearance of this tissue resembled that reported about loosened total hip implants with the exception of the synovial-like layer at the cement surface. The cases reported here show that aggressive bone lysis may occur around stable cemented total hip arthroplasties without the presence of sepsis or malignant disease.     

Computerized tomography diagnosis of diffuse intraperitoneal metastases after retroperitoneal lymphadenectomy for testicular carcinoma

We report 2 cases of diffuse intraperitoneal metastases from testicular carcinoma following transabdominal retroperitoneal lymphadenectomy. This is an unusual pattern of metastasis for nonseminomatous germ cell tumors and it is believed to be the result of direct seeding from lymphatic leakage secondary to surgery. The value of computerized tomography in diagnosing this entity is emphasized.     

Distribution and prevalence of Octomyomermis troglodytis (Nematoda: Mermithidae), a parasite of the western tree hole mosquito, Aedes sierrensis

Octomyomermis troglodytis was found infecting Aedes sierrensis larvae in 14.5% of 165 tree holes sampled between 1982 and 1986. Mermithid infections were detected in tree hole waters that ranged in pH from 6.5 to 9.3 and electrical conductivities between 0.10 and 5.11 mmhos/cm. Third and fourth instar larvae were most frequently infected, and most immatures that succumbed to infections died while in the fourth instar. Most hosts contained only one nematode. Infected adults were obtained from emergence traps over tree holes, from field-collected immatures reared in the laboratory, and from mosquito collections from sentinel humans. Octomyomermis troglodytis escaped from adults into water vials in the laboratory, suggesting that infected adult mosquitoes serve as dispersal agents for this parasite.     

Pharmacokinetics of intravenous bepridil in patients with coronary disease

The pharmacokinetics of intravenous bepridil (1-[2-(N-benzylanilino)-1-(isobutoxymethyl)ethyl]pyrrolidine ) were studied in 16 patients undergoing cardiac catheterization for evaluation of coronary disease, all with normal base-line hemodynamic and renal functions. Ten patients received 3 mg/kg and six patients received 4 mg/kg of bepridil infused over a period of 30 min. Plasma bepridil concentrations were measured by HPLC and analyzed by model-dependent and model-independent methods. The mean (+/- SD) maximum plasma bepridil concentrations at the end of the infusion were 2047 +/- 820 ng/mL (3 mg/kg) and 2478 +/- 1426 ng/mL (4 mg/kg). Postinfusion bepridil concentrations were best described by a two-compartment open model. The model-dependent harmonic mean distribution and elimination half-lives were 1.7 h (range: 1.1-2.2 h) and 19.7 h (range: 8.0-61.9 h), respectively. The harmonic mean elimination half-life from model-independent analysis was 14.9 h (range: 7.4-64.0 h). The arithmetic means of other model-independent kinetic parameters were systemic clearance, 0.524 +/- 0.215 L X kg-1 X h-1; Vd, 15.3 +/- 10.9 L/kg; and Vdss, 10.1 +/- 6.0 L/kg. Model-dependent and model-independent estimates of half-life and clearance agreed reasonably well. Bepridil was well tolerated, effecting little or no change in central hemodynamics or EKG intervals. The extensive distribution and relatively slow clearance of bepridil account for its long elimination half-life. Intravenous bepridil appears to be a safe calcium (II) antagonist that is suitable for once-a-day dosing.