MUe effect of 0.1, 0.4, 1.0, 2.0 and 5.0 micrograms kg-1 h-1 somatostatin on food-induced rise of serum gastrin concentrations was studied in 4 dogs. Whereas it has been shown previously that these doses of somatostatin reduce postprandial release of other gastroenteropancreatic hormones, there was no evidence of suppression of serum gastrin levels. A dose of 12.6 micrograms kg-1 h-1 somatostatin abolished the gastrin response completely. The study shows that low doses of somatostatin are not a uniform suppressor of gastroenteropancreatic hormones.

The signs of congenital erythropoietic porphyria (Günther) had their onset at the age of two in a male patient born in 1934: there were blisters and scars after exposure to sunlight, red urine, marked cutaneous pigmentation, acral mutilation, lanugo and erythrodontia. Porphyrin levels in red blood cells and urine confirmed the diagnosis. Splenectomy was performed in 1966 for hypersplenism and haemolysis. No transfusion has been necessary since and the haemolysis has completely disappeared, but signs of ineffective erythropoiesis have continued. Cholecystectomy with choledochal revision was performed because of right upper abdominal colics, fever and biliary stasis in 1977: five partly pigmented, partly mixed gall-stones were removed. The latter had a black, friable centre with high porphyrin content and a cholesterol coating. Thus, as in other chronic haemolyses, pigmented gall-stones may also occur in congenital erythropoetic porphyria.

In all affected members of a family with Gardner's syndrome the complete triad of skin tumours, osteomas and polyps of the colon has developed since 1968 when examination revealed an apparently bisymptomatic variant of Gardner's syndrome. In this family bony changes consisting of circumscript osteomas, diffuse sclerosing zones within certain bones, and ridge-like widening of the corticalis were noted, having developed during puberty but remaining constant thereafter. Similarly, no significant new skin tumours appeared after puberty. Most colonic polyps however, occurred in adolescence or thereafter, with malignant transformation after a variable interval of several years. The necessity of long-term observation of the colon, also in patients presenting with a bi- or monosymptomatic form, is stressed. Coloscopy has proven to be more useful diagnostically than radiological methods.

No abstract

No abstract