Biomedical subjects
Publications and source records attributed to S Satyanath.
Schistosomiasis is one of the commonest parasitic diseases affecting mankind. Egyptian schistosomiasis (mansoni and haematobium) is capable of producing fibrosis and/or calcification, resulting in striking radiological features in target organs. Our two cases with biliary and pancreatic calcification provide a combination of features not documented previously.
Calcification of the ureters, bladder and seminal vesicles was detected in six patients studied on computed tomography (CT) for evaluation of genitourinary schistosomiasis and in four others examined for intestinal schistosomiasis. Other findings included pyelocaliectasis in ten kidneys, vesical carcinoma in one, seminal vesicle granuloma in one and non-visceral calcification in pelvic tissue in one patient.
In four out of six gallbladders with schistosomal infestation, calcified granulomas were dense enough to be seen on abdominal radiographs. In one patient, faint calcification seen on a specimen of the excised gallbladder was not visible on the abdominal radiograph. Calcification on abdominal radiographs, especially serpiginous, seen in the region of the neck of gallbladder, appears to be the clue to the diagnosis of gallbladder schistosomiasis in people from endemic areas.
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We report a sibship of two brothers and one sister with the osteoporosis-pseudoglioma syndrome and congenital heart disease. They presented in infancy with visual impairment and psychomotor retardation. Major features included bilateral cataracts, generalised osteopenia, severe platyspondyly, borderline mental retardation, muscular hypotonia, joint laxity, and ventricular septal defect. Parental consanguinity and affected sibs of both sexes strongly suggested autosomal recessive inheritance. Analysis of the present and previously reported cases showed a wide range of interfamilial variability which may point to the existence of multiple allelism or genetic heterogeneity in this syndrome.
We present an Arab family with some features of Kniest disease. The proband was a six year old boy with rhizomelic short limbed dwarfism, 'dish-like' facies, cleft palate, deafness, and camptodactyly. Most radiological changes were compatible with Kniest disease. Two younger sibs, similarly affected, had died at a few months old, and the pedigree shows strong evidence of autosomal recessive inheritance, unlike previously reported cases of Kniest disease which have shown autosomal dominant inheritance.
In a 6-month period, 25 appendices with histological involvement by schistosomiasis were radiographed. Eleven showed varying degrees of radiographically detectable calcification having a spotty, linear, or amorphous pattern affecting either a short segment or the whole appendix. Four of these appendices had a mixed pattern and one also had nodular calcification in the mesoappendix. In only 1 patient was the appendiceal calcification visible on preoperative abdominal radiographs. In 5 asymptomatic patients, not submitted to surgery, appendiceal calcification was detected during radiologic evaluation of urinary schistosomiasis.
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Schistosomal periportal fibrosis produced a typical pattern on computed tomography in five patients. Low-density periportal tissue, present throughout the liver, enhanced strongly after the administration of contrast medium. While rounded in cross section, the thickened periportal tissue produced linear and branching patterns when imaged in longitudinal section. In all cases, the sonographic features were typical of schistosomal periportal fibrosis. A lack of awareness of the distinctive features of periportal fibrosis may result in a mistaken diagnosis of hepatic metastases.
Rectocolonic calcification was detected radiographically in 17 sites in 14 patients undergoing excretory urography for the assessment of urinary schistosomiasis. The right colon was involved in 11 sites, the rectum in four, and the left colon in two. The pattern of calcification varied according to the degree of bowel distension. A laminar pattern was common to all sites and occurred when the rectum or colon was distended with air, feces, or barium. A laminar or irregular amorphous density was found in the empty colon, whereas the calcified, empty rectum had a corrugated pattern. Rectocolonic calcification is probably the most common radiographic manifestation of schistosomal infestation of the gastrointestinal tract.
The hepatic sonograms of 22 patients with the features of schistosomal periportal fibrosis (PPF) were reviewed retrospectively. Dense echogenic bands were seen when scanning along the long axis of the intrahepatic portal vein radicles. Rounded densities resulted from scanning across these bands as they radiated from the porta hepatis to the liver's edge. Scanning across central and peripheral bifurcation points of the portal veins produced a branching pattern of high echogenicity. Associated findings were ascites and splenomegaly secondary to the liver disease. The hepatic sonographic features were characteristic of portal tract thickening from schistosomal PPF.