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Biomedical subjects

R G Long

Publications and source records attributed to R G Long.

90 records · Page 5Linked to original sources

Assessment of the (14C) aminopyrine breath test in liver disease.

Different methods of performing the (14C) aminopyrine breath test have been assessed. A tracer dose of 2 muCi without a loading dose and with a single breath collection at two hours was the method selected, since it gave the best discrimination between patients with hepatocellular diseases and normal subjects (5.2 +/- 0.2%, mean +/- SEM). Reduced values occurred in patients with chronic active hepatitis (with and without cirrhosis) (1.5 +/- 0.2%), alcoholic cirrhosis (1.7 +/- 0.4%) and hepatitis (2.5 +/- 0.3%), and late primary biliary cirrhosis suggesting defective microsomal function with respect to demethylation. Normal results were common in early primary biliary cirrhosis. Two weeks of prednisolone therapy caused some improvement in the breath test in nine of 10 patients with chronic active hepatitis. It is concluded that the (14C) aminopyrine breath test is a simple test for detecting hepatocellular dysfunction, but has no obvious diagnostic advantage over the determination of serum aspartate transaminase and two hour post-prandial bile-acids.

Adolescent↗

Clinical, biochemical, and histological studies of osteomalacia, osteoporosis, and parathyroid function in chronic liver disease.

Twenty of 32 patients with either chronic cholestatic or hepatocellular liver disease had bone pain or recent fractures. On bone biopsy five patients had normal bone, 15 had osteomalacia, five had osteoporosis, and seven had a combination of osteomalacia and osteoporosis. In the presence of osteoporosis, osteomalacia was minimal or absent. There was no biochemical, radiological, or histological evidence of excess parathyroid activity. No significant correlations were demonstrated between the plasma and urinary biochemical findings and the presence of either osteoporosis or osteomalacia and bone biopsy was essential for correct diagnosis. There was no statistical relationship between low serum 25-hydroxy vitamin D values and the presence of osteomalacia. Bone disease was not prevented by regular intramuscular vitamin D2, although biochemical changes were improved. Drugs such as corticosteroids and cholestyramine may be important aetiological factors in hepatic osteodystrophy.

Adult↗

Hepatic osteodystrophy.

Hepatic osteodystrophy consists of three types: osteomalacia, osteoporosis, and periosteal reaction with new bone formation. Secondary hyperparathyroidism is very rare, if it occurs at all. The cause of osteomalacia appears to be vitamin D deficiency due to a lack of vitamin D substrate. In the presence of adequate substrates, 25-OHD and dihydroxy vitamin D metabolites are formed. The vitamin D deficiency results in osteomalacia and malabsorption of calcium and phosphorus. The osteomalacia can be treated successfully with vitamin D supplements. In some patients calcium, phosphorus, and magnesium supplements may be required. The aetiology and treatment of the osteoporosis and the periosteal reactions remain obscure.

Bone and Bones↗

25-Hydroxylation of vitamin D in primary biliary cirrhosis.

The ability to 25-hydroxylate vitamin D was investigated in thirty-nine patients with symptomatic primary biliary cirrhosis (P.B.C.). In seven previously untreated patients serum-25-hydroxy-vitamin-D (25-OHD) concentration increased after regular monthly injections of vitamin D. After a single injection of vitamin D in eight P.B.C. patients serum-25-OHD did not change significantly over 12 days; in contrast there were significant increases in eight normal subjects and in seven patients with nutritional osteomalacia. Twenty-three of twenty-five P.B.C. patients on regular vitamin-D therapy had normal serum-25-OHD values. These results indicate that serum-25-OHD concentrations become normal in P.B.C. if adequate amounts of vitamin D are presented to the liver as substrate.

Adult↗

Adult Niemann-Pick disease: its relationship to the syndrome of the sea-blue histiocyte.

Three unrelated female patients with adult Niemann-Pick disease are described. All the patients had reduced coagulation factors and involvement of the marrow, liver, spleen and lungs. Two patients were shown to have abnormal platelet function; two patients also had pingueculas and a late onset of a menarche. Foam cells and sea-blue histiocytes were seen in the marrow and livers in all three patients, in the spleen in two patients in the lymph nodes in one patient. The clinical presentation, the histologic appearance, the histochemical staining reactions, the lipid analysis and the ultrastructure were all consistent with a diagnosis of adult Niemann-Pick disease. On the basis of these observations, it is clear that adult Niemann-Pick disease is a cause of the syndrome of the sea-blue histiocyte. The existence of the syndrome of the sea-blue histiocyte as a separate entity is also questioned.

Adolescent↗

Pre-eclampsia presenting with deep jaundice.

Pre-eclampsia complicated by deep jaundice occurred in a previously healthy primigravida. The main aetiological factor was disseminated intravascular coagulation; this caused both haemolysis and liver cell necrosis. Liver biopsy showed fibrin thrombi in the capillaries with microhaemorrhages and loss of periportal liver cells. The jaundice was attributed to both haemolytic and hepatocellular processes. Organs other than the liver were relatively unaffected.

Adult↗

Percutaneous transhepatic cholangiography using the "Chiba" needle--80 cases.

Using the Chiba needle for percutaneous transhepatic cholangiography, bile ducts have been visualized radiographically in 80% of 80 patients with cholestatic jaundice. The success rate was 94.4% in the 54 patients with dilated bile ducts due to an extrahepatic bilary obstruction, and 50% of the 20 patients with undilated ducts. Four patients developed ascending cholangitis with septicaemia on the same day as the procedure, and early surgery after visualization of obstructed bile ducts is recommended. The technique was successful in three of five patients with sclerosing cholangitis. Percutaneous cholangiography using the Chiba needle is a convenient method of opacifying the biliary system in patients with severe large bile duct obstruction.

Cholangiography↗

Presentation and course of asymptomatic primary biliary cirrhosis.

Twenty patients without symptoms of hepatobiliary disease were diagnosed as having asymptomatic primary biliary cirrhosis. In every patient liver histology was diagnostic of, suggestive of, or compatible with the diagnosis. Eighteen had a positive serum mitochondrial antibody, 18 had raised serum immunoglobulin M, and 17 had markedly raised serum alkaline phosphatase values. A mean of 4.5 years after diagnosis, 10 of the patients had not developed hepatobiliary symtoms; 4 of these 10 patients have survived 6 to 10 years. Ten patients developed symptoms after a mean of 2.2 years from initial diagnosis; 7 are still alive but 3 have died of liver failure. The development of symptoms could not be predicted by either serum biochemical tests or hepatic histology. It is concluded that the diagnosis of asymptomatic primary biliary cirrhosis is compatible with 10 or more asymptomatic years.

Adult↗

Serum-25-hydroxy-vitamin-D in untreated parenchymal and cholestatic liver disease.

Serum-25-hydroxy-vitamin-D (25 OHD) concentration has been measured in 106 patients with untreated parenchymal and cholestatic liver disease. Low mean values were found in groups of patients with alcoholic hepatitis and cirrhosis, non-cirrhotic active chronic hepatitis, lupoid and cryptogenic cirrhosis, symptomatic primary biliary cirrhosis, and acute and chronic biliary disease. In a group of patients with presymptomatic biliary cirrhosis the mean value was not significantly different from normal. It is concluded that in the presence of significant parenchymal or cholestatic liver disease serum-25-OHD concentrations are usually low. The mechanisms for the reduction remain to be clarified, but low serum-25-OHD values may play a contributory role in the aetiology of osteomalacia in chronic liver disease.

Acute Disease↗

Percutaneous transhepatic obliteration of gastro-oesophageal varices.

Percutaneous transhepatic portal-vein catheterisation was attempted to obliterate the major variceal venous supply in 13 decompensated cirrhotic patients, who continued to bleed after conservative therapy. Obliteration was achieved and bleeding stopped in 7 patients. In 5 patients obliteration was technically unsuccessful. The remaining patient had an unsuspected portal-vein block diagnosed by the transhepatic technique. 1 patient with successfully obliterated varices died after a haemothorax and haemorperitoneum developed. Follow-up splenic venography at three to six months in the 6 successfully thrombosed patients showed that 4 had persistent obliteration and had not re-bled. 2 patients re-bled from incompletely obliterated varices. It is concluded that selective obliteration of the major variceal supply is effective in stopping acute gastro-oesophageal variceal bleeding, but that greater experience is necessary before the long-term effectiveness of the procedure can be determined.

Adult↗

Scleritis and temporal arteritis.

Thirty consecutive patients with severe scleritis or episcleritis were admitted as in-patients to the Medical Ophthalmology Unit and assessed for systemic disease. There were seventeen women and thirteen men. The mean age was 53 with a median of 57 (range 23-83). Eighteen of the patients had scleritis: eleven of these had evidence of connective tissue disease and three of them had temporal arteritis. Twelve patients had episcleritis: six of them had a collagen disease and one of them developed temporal arteritis. This high incidence of temporal arteritis in association with scleritis has not been previously reported. It is important to diagnose and treat overt temporal arteritis early with parenteral steroids so that ischaemic papillopathy can be avoided. A higher incidence of collagen diseases than previously described is reported in episcleritis. It is thought that this is secondary to selection since patients with the usual self-limiting episcleritis are not normally referred for further in-patient investigation. In no patient was more than one significant diagnosis made. There was no significant medical illness in only 11% of patients with scleritis and 33% of patients with episcleritis. The majority of the non-collagen diseases (e.g. hypertension) were not previously recognized. In none of the patients with temporal arteritis was the diagnosis made before admission. It is concluded that full examination and investigation for underlying disease is indicated in both scleritis and severe episcleritis.

Aged↗

A randomized trial of percutaneous transhepatic cholangiography with the Chiba needle versus endoscopic retrograde cholangiography for bile duct visualization in jaundice.

Sixty consecutive patients, who were deeply jaundiced or in whom intravenous cholangiography had failed, were randomized to retrograde endoscopic cholangiography or percutaneous transheptic cholangiograhy with the "skinny" Chiba needle technique. Twenty-eight patients were assigned to retrograde cholangiography, which succeeded in 17 (65%). Percutaneous cholangiography was successful in 16 (50%) of the remaining 32 patients. When patients in whom the first procedure was unsuccessful were reinvestigated by the alternative technique, retrograde cholangiograms were obtained in 13 (81%) of 16, and percutaneous cholangiograms in 8 (73%) of 11. Thus, one or the other technique was successful in 54 (90%) of 60 patients. When the results were analyzed separately for extrahepatic (29 patients) or intrahepatic (31 patients) cholestasis, percutaneous cholangiography was successful in 95% of patients with extrahepatic cholestasis but in only 25% with intrahepatic cholestasis. Endoscopic retrograde cholangiography successded in 63% of patients with extrahepatic and 76% with intrahepatic causes of cholestasis. Complications occurred only in patients with extrahepatic cholestasis. Cholangitis and septicemia occurred in 1 patient after retrograde cholangiography and in 2 after the percutaneous technique. An intraperitoneal bile leak occurred in one other patient after percutaneous cholangiography. Percutaneous cholangiography with the narrow needle is a simple, inexpensive, and reliable method for demonstrating the biliary system and is usually successful when an extrahepatic cause of cholestasis is present. The occurrence of serious complications in patients with extrahepatic cholestasis, despite prophylactic antibiotics, makes provision for early surgery mandatory after both techniques.

Adult↗

The endoscopic appearance of duodenal folds is predictive of untreated adult celiac disease.

The loss of duodenal folds visible endoscopically has recently been reported as being a marker for celiac disease. We have investigated the sensitivity and specificity of this finding with a prospective study in 75 patients with symptoms or results of investigations compatible with celiac disease. Reported duodenal fold appearance was compared with histological findings, disaccharidase levels, and clinical diagnosis. Fifteen patients were found to have celiac disease and 11 had reduced or absent duodenal folds compared with only 2 of 60 patients who did not have celiac disease (p less than 0.0001). This finding has a sensitivity of 73%, specificity of 97%, and positive predictive value of 85%. Duodenal folds were not reported as being abnormal in seven patients with hypolactasia or two with giardiasis and did not appear to be influenced by age. A reduction in the number or height of duodenal folds as seen endoscopically in the second part of the duodenum is a specific and sensitive sign of celiac disease. Endoscopists should biopsy the duodenum for celiac disease whenever the duodenal folds appear to be reduced or absent.

Adult↗

Barium enema or colonoscopy for the investigation of iron deficiency anaemia?

OBJECTIVE: To determine whether double contrast barium enema is adequate for excluding carcinoma of the colon in patients with iron deficiency anaemia. DESIGN: Prospective audit. SUBJECTS: One hundred and twenty-three patients with iron deficiency anaemia. INTERVENTION: All patients had upper intestinal (GI) endoscopy, duodenal biopsy and double contrast barium enema. Malignant disease and other GI pathology were treated. Patients with recurrent or persistent anaemia at follow-up were colonoscoped. OUTCOME MEASURE: Colon cancer missed on barium enema examination but detected on colonoscopic examination. RESULTS: An identified GI lesion contributing to their anaemia and 11 colon cancers were found in 71% of patients, all identified on barium enema. Two pre-cancerous conditions were missed on barium enema examination. Only 45% colonoscopies inspected the caecum. CONCLUSION: Double contrast barium enema with sigmoidoscopy is probably sufficient for excluding carcinoma of the colon in iron deficiency anaemia.

Adolescent↗

Oral manifestations of systemic diseases.

Many systemic diseases have oral manifestations. The oral cavity might well be thought of as the window to the body because oral manifestations accompany many systemic diseases. These oral manifestations must be properly recognized if the patient is to receive appropriate diagnosis and referral for treatment. We have reviewed a series of recent articles and summarized known and newly described oral manifestations of several systemic diseases. The lesions of the oral mucosa, tongue, gingiva, dentition, periodontium, salivary glands, facial skeleton, extraoral skin and other related structures caused by some of the more common systemic diseases are highlighted.

Diagnosis↗