Search

To assess the prevalence of impaired homocysteine metabolism in young adults with arterial occlusive disease, 80 consecutive patients under 45 years old were screened. Various laboratory blood investigations and a standardized methionine loading test were performed. In the first 52 patients plasma levels of free homocysteine were determined; thereafter the levels of total homocysteine (a more sensitive measure of impaired homocysteine metabolism) were measured. The methionine loading test was abnormal in 15 patients (19 per cent) who did not differ from the other 65 with respect to prevalence of other risk factors, clinical characteristics, and electrocardio-graphic and angiographic findings. Blood levels of glucose, vitamins B6and B12, folate, protein C and protein S, fibrinogen and low-density lipoprotein cholesterol did not differ significantly between the two groups. The prevalence of impaired homocysteine metabolism in young patients with arterial occlusive disease is greater than the 1–2 per cent found in the normal population.

In an attempt to stimulate bone resportion, a 10-week-old infant with malignant infantile osteopetrosis was treated with high doses of calcitriol, a potent bone resorption stimulatory agent, combined with a low calcium diet to prevent hypercalcaemia. Although calcitriol administration was initiated at this very young age, our patient did not show any clinical, radiological, or histological improvement. Despite reports of positive results of this treatment in the literature, our patient did not reveal any signs of bone resorption. She eventually died from the complications of osteopetrosis at the age of 6 months after 88 days of therapy.

In a 2-year-old boy with untreated cystic fibrosis, an acute deterioration of his chronic respiratory insufficiency developed due to bilateral pneumonia. This condition caused acute right-sided heart failure and non-transmural myocardial infarction of the inferior wall. In concordance with this diagnosis, a marked increase of CPK-MB levels combined with transient severe ischemia on the ECG and the absence of myocardial injury at echocardiography was seen. At 3 years follow-up, he was in good clinical condition.

In a 2-year-old boy with untreated cystic fibrosis, an acute deterioration of his chronic respiratory insufficiency developed due to bilateral pneumonia. This condition caused acute right-sided heart failure and non-transmural myocardial infarction of the inferior wall. In concordance with this diagnosis, a marked increase of CPK-MB levels combined with transient severe ischemia on the ECG and the absence of myocardial injury at echocardiography was seen. At 3 years follow-up, he was in good clinical condition.

A male patient with pycnodysostosis suffered from chronic respiratory insufficiency and pulmonary hypertension. This was caused by concomitant upper airway obstruction, resulting from a low implanted uvula and a long soft palate, in combination with glossoptosis and retrognathia due to the flattened mandibular angles. An inter-current respiratory infection gave rise to an acute deterioration, with right-sided heart failure, severe liver damage and coma. Surgical shortening of the soft palate was performed, after which the blood gas values returned to normal.

In 70 patients with proven giardiasis the efficacy and cost-effectiveness of the diagnostic procedure were studied retrospectively. In 53% of the patients, complaints existed for more than 6 months. Parasitological examination of three stool specimens on non-consecutive days showed a sensitivity of 95.7%. The false negative rate was 2.8%, as giardiasis was found in 3 out of 109 patients in whom duodenal biopsy was performed after a negative “triple faecal test”. Of other laboratory investigations performed before the results of faecal screening were known, 75% gave normal results, thus not contributing to the diagnosis. A proposal for a cost-effective diagnostic procedure is presented.

Thirty-eight children (21 male, 17 female, age 3–18 years), treated for Crohn disease in two Dutch university centres, were retrospectively studied in order to evaluate the results of conservative treatment and to find out in what way surgical treatment in this age group may have differed from treating adults with this disease. Both groups had an equal distribution of age and sex. Diarrhoea with discharge of blood and mucus, abdominal pain, nausea/vomiting, weight loss, fever and general discomfort were the most frequent presenting symptoms. Twenty-three children (60%) showed signs of malabsorption; 4 children (10%) had growth retardation. In 27 children (70%), 63 surgical procedures were performed (2.4 operations per child). There was no surgical mortality. Most operations were performed for ileocolitis and colon-only localizations needed most re-operations. Of the surgical procedures performed, 55% were excisional procedures. Already 3 years after the onset of symptoms, 50% of all children had had their first resection, whereas in adults, 50% of the patients undergo surgery 8 years after disease onset. Eight children were treated with split ileostomy. In only one of these children, operated for non-toxic colitis and severe steroid-dependent growth retardation, could the colon eventually be saved. The time between the onset of symptoms and the first operation seems to be shorter in children compared to adults. Severe malabsorption and growth retardation are additional specific indications for surgery for Crohn disease in childhood. The latter combined with non-toxic colitis, may perhaps be the only indication left for performing split ileostomy in Crohn disease.

The role of conjugated bilirubin in liver regeneration after partial hepatectomy (PH) was studied in Gunn rats, in transport-mutant (TR-) rats, and in rats with extrahepatic biliary obstruction. Ornithine decarboxylase (ODC) and thymidine kinase (TK) activities in liver homogenates and immunohistochemistry ofin vivo bromodeoxyuridine (BrdU) incorporation in hepatic DNA were followed as regeneration parameters at 24 and 48 hr after PH. The results relative to TK activity and BrdU incorporation were consistent with significantly delayed hepatic DNA synthesis in Gunn rats in comparison to control Wistar and TR- rats. This delay in DNA synthesis was not reflected in the hepatic ODC activity. After one week of complete common bile duct obstruction (CBO), an increased TK activity and BrdU incorporation was seen. PH following CBO resulted in a further increase in ODC activity and BrdU incorporation. TK activity did not change, however. These data relative to the regulation of hepatic DNA synthesis after PH in Gunn rats and in rats with extrahepatic biliary obstruction suggest a possible stimulatory role for conjugated bilirubin in hepatic regeneration; however, the normal hepatic DNA synthesis in TR- rats studied before PH and the subnormal DNA synthesis seen 24hr after PH in TR- rats and in rats with CBO indicate that conjugated bilirubin does not stimulate hepatic DNA synthesis.