HUNTERS SYNDROME AND COOLEY'S ANAEMIA IN THE SAME PATIENT Effect of Multiple Transfusions

It has been reported that plasma and leukocyte infusions have a beneficial effect on patients with Hurler's and Hunter's syndromes. A 5-year-old boy with Hunter's syndrome and Coo-ley's anaemia is presented. This boy received a total of 38 transfusions over a 4-year period. The usual clinical manifestations of the Hunter's syndrome were fully developed, and were not, in any obvious way, ameliorated by the transfusions.     

Power Doppler sonography in the diagnosis of hemophilic synovitis – a promising tool

Summary. Background: Recurrent hemarthroses in hemophilia results in synovitis and joint arthropathy. Primary prophylaxis when universally instituted at current doses can prevent joint deterioration but is expensive. Alternatively, the selective implementation of prophylaxis would require a more sensitive tool for detecting synovitis than possible with clinical surveillance or plain radiographs. Magnetic resonance imaging (MRI) is such a tool and is utilized for the evaluation of hemophilic joint disease (HJD). However, it is expensive, and requires sedation in younger children precluding its utility for monitoring of synovitis. Ultrasonography (USG) with power Doppler (USG‐PDS) has been utilized to detect and quantitate synovial vascularity in other arthritides and could provide an equally effective but less costly tool for HJD, particularly in children who would not require sedation. Objectives: To determine whether USG‐PDS is comparable to MRI in the evaluation of hemophilic synovitis. Patients: A prospective cohort of 31 subjects including 33 joints (knees, elbows, ankles) underwent dynamic contrast enhanced (DCE)‐MRI and USG‐PDS. Results: USG‐PDS measurements of synovial thickness(r = 0.70, P < 0.0001) and synovial vascularity (r = 0.73, P < 0.0001) correlated strongly with those obtained with DCE‐MRI. A cutoff of PDS intensity of 1.3 decibels (dB) per mm² was found to yield a sensitivity of 100% and a specificity of 94.1% in 17 joints with/without a history of hemarthroses. Pettersson radiographic scores correlated significantly with synovial thickness in adults but not children. Conclusions: Our data suggest that USG‐PDS may be an inexpensive and easily implemented imaging tool for detecting hemophilic synovitis and could be useful in tailoring effective prophylaxis.     

Sigmoidorectal (Mainz II) pouch for continent urinary diversion in bladder cancer

BACKGROUND: We aimed to assess the feasibility, safety, and outcome of the sigmoidorectal (Mainz II) pouch for urinary diversion in patients with invasive bladder cancer. METHODS: Twenty-nine patients (25 men and four women), aged 65-76 years, who had undergone radical cystectomy and the sigmoidorectal pouch procedure for invasive bladder cancer were included in this study. Postoperative evaluations included metabolic testing, standard laboratory screening, renal ultrasonography, pouchography, and intravenous urography or pouchoscopy when indicated. RESULTS: The median operative time was 175 min. Urine leakage was encountered in two patients (6.8%), deep vein thrombosis in one (3.4%), and ileus necessitating surgery in another one. Two patients developed pyelonephritis due to ureterocolonic stricture, which was treated with antegrade balloon dilatation. No local relapses of bladder cancer were found. All patients were continent during the day, but one patient was occasionally incontinent during the night. In the long term, six patients (20.6%) developed metastatic disease, and five patients (17.2%) died because of cancer-related causes. Overall survival was 100, 96 and 60% at 1, 2 and 3 years after the operation, respectively. The mean survival was 36.8 +/- 1.9 months, which was statistically significantly associated with the M stage (P < 0.001), but not with the T (P = 0.091) or N (P = 0.081) stages. CONCLUSIONS: The sigmoidorectal (Mainz II) pouch seems to be a feasible, safe and effective method for continent urinary diversion. It is able to provide good quality of life, and ensure good overall survival rates.     

Mortality Rates in a Cardiovascular 'Low-risk' Population in Rural Crete

A research project in primary health care has been in progresssince 1988 in an agricultural area of Crete, with the aim ofsurveying the cardiovascular risk profile and following theincidence of cardiovascular disease in the population over time.In the present paper we have investigated the mortality ratesand the causes of death in 1989 and 1990 of the population ofthe Agios Vassilios county (n = 7758). Furthermore, wheneverpossible, we have checked the accuracy of the certified causesof death against the case record system introduced in 1988 inthe health centre. During 1989 and 1990, 267 subjects died inthe research area, 234 of whom were >65 years old. Diseasesof the circulatory system (mostly congestive heart failure)accounted for 46% of all deaths. The corresponding figure forneoplasms was 18%. The standardized mortality from ischaemicheart disease was found to be very low in both men and women,37.9 and 41.9 per 100 000 per year, respectively, in comparisonwith that of Greece as a whole in 1989, 140.0 and 59.5, respectively.These figures were considerably lower than those reported in1988 from for example Sweden, 303.6 and 153.3, respectively.The standardized mortality from cerebrovascular disease in AgiosVassilios county was found to be somewhat lower than the nationalfigures in 1989, 118.1 and 107.0 in males and females versus135.7 and 142.1, respectively. In our analyses we did not findany under- representation of deaths in circulatory diseaseswhen the death certificates were checked against the availableinformation in the case record system at the health centre,in those patients where this type of in formation was available(n = 128, 48%). In conclusion, the mortality from ischaemicheart disease still seems to be low in Cretans, as previousreports have shown. An upward trend may, however, be expectedin the future, maybe as a result of the change in lifestylenow taking place in Crete. The longitudinal observation of themortality of the Agios Vassilios population is expected to elucidatethis further in the future.     

Glucose-6-Phosphate Dehydrogenase Deficiency in Greece

The glutathione stability of red cells was estimated in 40 patients duringacute hemolysis induced by fava beans. There were wide individual differences but in all cases except one (Case 18) the post-incubation GSH fell tolevels below 40 mg. per cent packed RBC which is the lower normal limit.

The GSH stability on 44 mothers and 37 fathers gave results consistent withthe genetic hypothesis that in male patients the mother is the carrier of thebiochemical defect, while in female patients both parents are carriers, since,as a rule, only female homozygotes suffer from hemolytic episodes. However,in only 77.7 per cent of the mothers could the biochemical defect be proved bythis method.

The Motulsky test was performed in 30 of the 40 patients. It gave abnormaldecolorization times in 25 or 83 per cent of the cases. This test is thereforevaluable for diagnosing "sensitivity" during a hemolytic episode; it is, nevertheless, less sensitive than the GSH stability method.

The Motulsky test was also performed on 31 mothers, 18 fathers and 8siblings. It proved to be unreliable in the detection of female heterozygotes.

G-6-PD deficiency is widely disseminated in Greece; it is, however, notevenly distributed throughout the country.

The highest frequency of G-6-PD deficiency found so far in males was about3 per cent; the lowest was 0.7 per cent.

Submitted on August 1, 1960 Accepted on April 21, 1961     

IMMUNOGLOBULIN LEVELS IN CHILDREN WITH HOMOZYGOUS β-THALASSEMIA

Abstract. Patients with hereditary hemolytic anaemias have been known to have immunoglobulin alterations related to an increased susceptibility to infection. In the present series, immunoglobulins G, A and M have been estimated in 50 thalassemic children aged 10 months to 13 years. No significant difference was found in any of the immunoglobulins between patients and age-matched controls. No correlation could be shown between immunoglobulin levels and ( a ) the severity of anaemia, ( b ) the degree of hemosiderosis, and ( c ) the frequency of febrile infections per year. Against clinical impressions patients did not show any increased susceptibility to infections.     

蜂螫疗法与五行

探讨了涉及蜂王、养蜂及神经传递等9种要素的蜂螫疗法。第一次提出了蜂蜇疗法与中医五行的关系,即蜂毒、蜂蜜、蜂胶、蜂王浆与花粉分别与火、土、金、水、木相配合,构成了蜂蜇疗法与五行的系统联系。结合现代研究及传统的时间治疗学,提出了蜂蜇疗法的最佳治疗时间及治疗特点。