Whipple's disease. A classic case report and review of the literature.

We report the case of a 43-year-old carpenter with abdominal complaints and progressive weight loss. The HLA-B27 positive male had been suffering migratory arthropathy for five years, only partially under control with corticosteroids and methotrexate therapy. Endoscopic investigation showed dark staining of the duodenal mucosa and the ileal mucosa had an erythematous aspect with multiple white spots. Abundant periodic acid Schiff positive macrophages were seen on histologic examination of biopsy samples. This is a classic presentation of Whipple's disease, a rare multisystemic disease caused by the Tropheryma whipplei. Typical symptoms are arthropathy, weight loss, abdominal pain and diarrhea, but also systemic and neurological manifestations may occur. The otherwise lethal disease can be treated with long term antibiotics.

[Evaluation of a decade of neonatal screening for congenital hypothyroidism in The Netherlands]. / Evaluatie van een decennium neonatale screening op congenitale hypothyreoïdie in Nederland.

OBJECTIVE: Evaluation of the Dutch screening programme for congenital hypothyroidism (CH). DESIGN: Descriptive. SETTING: Nationwide. METHODS: Data on the screening were obtained from the administration body for vaccination and screening results, laboratories and paediatricians to whom infants with positive screening values were referred, during the period from January 1st, 1981 to December 31st, 1990. RESULTS: Of all live births in the Netherlands, 99.5% (1,797,719) were screened for CH. During the study period, 10,165 children (0.57% of all screened infants) were referred. Of the referred children, 529 had primary CH and 53 had congenital thyrotropin deficiency syndrome (CTDS). The prevalences of primary CH and CTDS are 1:3,400 and 1:25,000, respectively. The sensitivity of the programme with respect to detection of primary CH and CTDS was 99% and 74%, respectively. For all forms of CH combined, the specificity of the programme was 99% and the positive predictive value 6%. The positive predictive value was especially low in the group of infants with low T4 and normal thyrotropin values. One of the goals of the programme is to realise the start of treatment in all patients before they reach the age of three weeks. Before the screening programme came into being, the cumulative proportion of patients treated on the 21st day was 6%. After the beginning of the programme, the proportion increased to 54%. In screened patients with a severe form of CH it is currently 72%. CONCLUSION: The screening programme has made a substantial and important contribution to early and effective identification of patients with CH. A number of measures to decrease the number of false-positive results have been taken and others are at present being investigated. Although patients are now treated much earlier than before the programme started, substantial improvement in this respect is still possible. This can only be achieved by a collective effort of performers of the heel puncture, laboratories, administration body for vaccination and screening results, general practitioners and pediatricians.

Patterns of use of obstetrical interventions in 12 countries.

Recent obstetrical practice trends in 12 countries were surveyed. There was a 3-fold difference in caesarean section rates and a 10-fold difference in instrumental vaginal delivery rates among countries. There was a net increase in the caesarean section rate of all countries over the study period and a net decrease in the instrumental vaginal delivery rate of some countries. There was a decrease in the caesarean section rate during the last year of observation in Australia, Denmark and Finland. In general, countries with high caesarean rates also had high instrumental vaginal delivery rates. There was no consistent relationship between use of caesarean section and use of instrumental vaginal delivery, although in several countries increasing use of caesarean section was accompanied by decreasing use of instrumental vaginal delivery. Oxytocin use rates were associated positively with instrumental delivery but not with caesarean section rates. While it was not possible to determine the proportions of women who received appropriate obstetrical care, we can infer that a significant proportion of interventions were unnecessary or only marginally beneficial. Continued increases in rates of obstetrical intervention are unlikely to result in improvements in birth outcome overall and may pose a risk to mothers and their newborns.

Youth health care in The Netherlands: a bird's eye view.

In the context of the conference on cross-national comparisons of child health, a short overview is given of youth health care in the Netherlands. After a brief explanation of the financing of the Dutch health care system, trends in child mortality are shown. A short description is presented of obstetrical care, which features independent midwives and 35% home deliveries; the organization of well baby clinics, which provide for 10 visits in the first 15 months and care for 95% of all children; and the school health services, which cover nearly all children.

The national immunization program of The Netherlands.

After a brief explanation of the immunization policy in the Netherlands, the national immunization program is described, with special attention given to coupling of the municipal population records with a computerized database of individual immunization records at the provincial level. The Dutch program achieves coverage rates greater than 90% for all routine immunizations. Participation in the program is free of charge to every child living in the country up to the age of 13 years, but there is no obligation or requirement to be immunized. Financing of the program is also discussed.

Children with special needs in The Netherlands: impaired hearing, adolescent pregnancy, and myelomeningocele.

In the Netherlands, treatment and management of children with special needs is characterized by the absence of financial barriers to care and the existence of a number of specialized services. For hearing-impaired children, there are special audiologic centers and special schools. Adolescents at risk of unintended pregnancy are protected through education and a dedicated system of family planning clinics. Children with multiple handicaps, such as those associated with myelomeningocele, face greater difficulties but still have an array of services available to them at little or no cost. Families are sheltered from excessive expense through social insurance.

Duplex Doppler evaluation of renal allograft dysfunction: Doppler signal quantitation and pathologic correlation.

The value of quantitative duplex Doppler sonography in discriminating the different possible causes of renal transplant dysfunction was prospectively studied in 60 patients during 65 episodes of renal function impairment. Final diagnosis at histology was acute rejection (n: 30), acute tubular necrosis (n: 4), cyclosporin nephrotoxicity (n: 16) and chronic rejection (n: 15). Duplex sonography was done the day a percutaneous biopsy was taken and before any therapy was started. Arterial Doppler signals obtained from the segmental, interlobar and arcuate arteries were both morphologically and quantitatively analysed. For quantitative analysis we used the resistive index as proposed by Pourcelot on the one hand, and introduced a variant resistive index on the other hand. Morphological analysis yielded no discriminative value. Comparing both quantitative methods--the resistive index of Pourcelot and the variant resistive index--clearly higher specificities--71% using the variant resistive index, 28% using the resistive index of Pourcelot--for excluding acute rejection from the other possible causes of renal function impairment could be achieved. A nephrectomy was done on 7 patients with severe transplant dysfunction. Microangiographies performed on these nephrectomy specimens were correlated with previous Doppler studies and with histology.

Balloon angioplasty of venous structures.

Percutaneous transluminal angioplasty was attempted in 20 patients with stenoses of venous structures. It concerned one stenosis in a native subclavian vein, 12 stenoses in venous bypass grafts and 7 stenoses in hemodialysis-access fistulas. Primary results were excellent for the procedures in the native vein and the venous bypass grafts with success in all patients (13/13). In the arteriovenous fistulas for hemodialysis, dilatation of the venous stenoses was only possible in 5 out of 7 patients. The subclavian vein stenosis did not recur within a follow-up period of 3 years. Of the stenoses in the venous bypass grafts, two lesions reoccluded within one week and 6 lesions recurred within one year. Two of these lesions were successfully redilated so that 6 lesions remain patent with a follow-up of more than one year. Of the 5 successful dilatations in hemodialysis-access fistulas, two lesions recurred within 2 months. Only 3 veins are still accessible for hemodialysis. It is concluded that attempts at balloon dilatation of stenoses in venous bypass grafts or hemodialysis-access fistulas are meaningful in order to prolong the life of these surgical procedures. Recurrence of stenoses is however likely to occur within one or two years.