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Biomedical subjects

J H Lewis

Publications and source records attributed to J H Lewis.

At least 163 records · Page 9Linked to original sources
PubMed1984

T lymphocyte colony assay in hemophiliacs.

Unexplained lymphadenopathy, with or without accompanying symptoms, known as the "lymphadenopathy syndrome," has been recognized in groups at risk for acquired immune deficiency syndrome (AIDS), namely, homosexuals and hemophiliacs. To date, however, no test has been defined that discriminates between asymptomatic individuals and those with adenopathy in these high-risk groups. The T colony assay, which measures T lymphocyte growth in soft agar and which allows selective T cell proliferation with minimal cell-cell contact, was evaluated in asymptomatic hemophiliacs. Significantly lower mean colony counts were found in eight hemophiliacs with adenopathy (HA), 763 +/- 348 (+/- SEM), than in 16 healthy hemophiliacs (HH) 3,044 +/- 661 (P less than .005), or than in 24 heterosexual control subjects, 3,964 +/- 395 (P less than .005). The in vitro addition of exogenous interleukin-2 (IL-2) restored normal colony growth in the HA population. These results indicate that the T colony assay can detect abnormal cell-mediated immunity among hemophiliacs and specifically discriminates between asymptomatic hemophiliacs (HH) and those with adenopathy (HA). In addition, IL-2 may be of potential benefit in improving T cell defects in AIDS or the "lymphadenopathy syndrome"; however, this remains to be proven.

Cell Division↗
PubMed1984

Intestinal obstruction due to bezoars.

A patient with acute intestinal obstruction due to unrecognized gastric phytobezoars is presented. There was no history of prior gastric or other abdominal surgery. At laparotomy several fragments of rubbery material were discovered obstructing the ileocecal valve. Exploration of the upper gastrointestinal tract revealed two partially fragmented bezoars in the stomach which were removed through a gastrotomy. After surgery the patient recounted the ingestion of persimmons a few weeks before the onset of her symptoms. A review of the literature reveals bezoars to be an infrequent, although not an altogether rare, cause of small bowel obstruction in patients without antecedent gastric surgery. A careful dietary history should accompany all patients presenting with acute intestinal obstruction.

Bezoars↗
PubMed1983

Mutation of antitrypsin to antithrombin. alpha 1-antitrypsin Pittsburgh (358 Met leads to Arg), a fatal bleeding disorder.

Our previous studies predicted a functional relationship between the plasma proteins alpha 1-antitrypsin and antithrombin III. To elucidate this relationship we investigated the plasma of a 14-year-old boy who had died from an episodic bleeding disorder. A variant alpha 1-antitrypsin was identified in which the methionine at position 358 had been replaced by an arginine. This had converted the alpha 1-antitrypsin from its normal function as an inhibitor of elastase to that of an inhibitor of thrombin. This finding indicates that the reactive center of alpha 1-antitrypsin is methionine 358, which acts as a bait for elastase, just as the normal reactive center of antithrombin III is arginine 393, which acts as a bait for thrombin. The independence of the new thrombin inhibitor from heparin control explains the bleeding disorder; it also indicates that heparin normally acts directly on antithrombin III, revealing its inherent inhibitory activity. The episodic nature of the bleeding was a consequence of the mutant protein's being an acute-phase reactant, the level of which increased several-fold after trauma.

Adolescent↗
PubMed1983

Turcot's syndrome. Evidence for autosomal dominant inheritance.

A case of Turcot's syndrome (colonic polyposis plus a malignant central nervous system tumor) occurring in a kindred with autosomal dominant colonic polyposis is presented. It is proposed that Turcot's syndrome patients can be classified into Type I where only siblings are affected and Type II where two or more generations have colonic polyposis. A third nonfamilial group cannot be classified into Type I or II based on available information. Evidence is presented suggesting Turcot's syndrome is best considered an additional phenotype of familial polyposis and is most likely inherited in an autosomal dominant manner.

Cerebellar Neoplasms↗
PubMed1983

Acquired-immunodeficiency-like syndrome in two haemophiliacs.

The immunological status of two multiply transfused patients with severe haemophilia A and diffuse lymphadenopathy was evaluated. But patients appeared clinically well and denied homosexuality or intravenous drug abuse. Immunological studies revealed depressed cellular immune function with anergy, relative lymphopenia, decreased T helper cells, and increased T suppressor cells in both patients and an altered T-helper-cell/T-suppressor-cell ratio in one. Hyperresponsiveness of the humoral immune system was demonstrated by elevated IgG and IgM. Lymph-node biopsy revealed benign nonspecific hyperplasia in both patients. The lymphadenopathy and immunological features in these two haemophiliacs bear a striking resemblance to the acquired immunodeficiency syndrome (AIDS) of homosexuals, intravenous drug abusers, and Haitian immigrants. These findings may represent a prodromal phase or a forme fruste of AIDS. Transmission of an infectious agent in blood products seems likely.

Acquired Immunodeficiency Syndrome↗
PubMed1983

Budd-Chiari syndrome associated with oral contraceptive steroids. Review of treatment of 47 cases.

Oral contraceptive steroids (OCS) have been implicated as the cause of a number of instances of hepatic vein thrombosis (Budd-Chiari syndrome). Survival appears to be related to early diagnosis and treatment, but there does not appear to be a consensus as to the most appropriate management of these patients. Portosystemic shunting has frequently been advocated, although the results have been quite variable. Some patients appear to do well with conservative measures only. In the effort to obtain a clearer understanding of the effects of different medical and surgical therapies in this disorder, we analyzed the treatment of 47 cases associated with OCS, 29 of which were found in the literature and 18 additional cases identified through a questionnaire survey mailed to members of the AASLD. Surgery had been performed in 27 of these 47 patients (57%); 17 patients had been treated medically (36%); two individuals had received unspecified treatment, and one patient died before any treatment could be initiated. In the surgical group 13 patients underwent portosystemic shunt surgery with six surviving up to 5 years. Two patients survived more than 15 months following orthotopic liver transplant and one patient is well after partial hepatectomy. Of 10 patients who underwent exploratory laparotomy (three with the intent to perform a shunt), seven died postoperatively. Mean survival for the surgically treated group was 19.4 months (range 10 days to 7 years). Of those patients treated medically with combinations of diuretics, anticoagulants, antiplatelet agents, fibrinolytic agents, and peritoneovenous shunts to control ascites, 11 (65%) have survived from 3 months to 6 years (mean survival 29.0 months). We conclude that a satisfactory response may accompany either medical or surgical management of patients with Budd-Chiari syndrome associated with OCS. Patients with severe occlusive disease may benefit most from surgical decompression of the hepatic veins. However, for those with mild to moderate disease, the proper role for operative intervention remains to be defined.

Adult↗
PubMed1983

A hemophiliac dog colony: genetic studies and coagulation findings in hemophiliac and normal dogs.

A colony of hemophiliac dogs was developed as descendants of a single affected Pomeranian. Over a 13 year period, a total of 29 hemophiliac dogs survived to the age of 6 months or longer. Eleven were female and 18 were male. All five of the possible matings in a sex-linked recessive inheritance pattern were accomplished and the offspring fell into the expected sex and disease groups. Twenty-one dogs died from untreated hemorrhages which usually occurred in loose tissues or body cavities during the night. Clinically, there were no hemarthroses but 22 other hemorrhages responded promptly to treatment with dog cryoprecipitate. Coagulation studies showed that the hemophiliac dogs averaged about 0.23 U/ml equivalents of human F VIII:C while normal dogs averaged 8.0 U/ml equivalents of human. F's V, VII, II, X, IX, XI and XII were higher than human in both normal and hemophiliac dogs. Dog fibrinogen fell within the human range and Fletcher F was very low.

Animals↗
PubMed1983

Indexes of intravascular hemolysis, quantification of coagulation factors, and platelet survival in patients with porcine heterograft valves.

Ten patients with porcine heterograft valves who were not receiving anticoagulant agents were evaluated to determine the effect of the valve on red blood cell survival and on platelet activation and consumption as measured by (1) quantification of the coagulation mechanism, (2) platelet function studies, and (3) 51-chromium platelet survival time. There was no evidence of significant intravascular hemolysis as determined by the reticulocyte count, serum iron and iron binding capacity, serum bilirubin level, or lactic dehydrogenase activity. The coagulation profile and the platelet function studies were normal. No statistically significant difference was found in the platelet survival time in the 10 patients with porcine heterograft valves (half-life 3.2 +/- 0.8 days) and the 11 normal control subjects (half-life 3.6 +/- 0.6 days) (p greater than 0.2). The finding of a normal platelet survival time in patients with porcine heterograft valves is consistent with clinical experience indicating that this device is associated with a low incidence of systemic embolization, approximating 3% per year.

Adult↗
PubMed1983

Ascites-induced LeVeen shunt coagulopathy.

Ten of 11 patients undergoing peritoneovenous (LeVeen) shunt placement for intractable ascites had disseminated intravascular coagulation (DIC) following the shunt procedure. Intraoperative ascitic fluid specimens revealed fibrin split products (FSP) in high titer (1:100-1:1600) in all patients. Endotoxin was found in 6 of 11 ascitic fluid samples but in no plasma samples. Activated clotting factors, clot inhibitors, excess protein, and fibrinolytic activity were not found in ascitic fluid. Clotting factor levels were much lower than in plasma. Bleeding occurred after operation in two patients; this appeared to be related to the severity of liver dysfunction as demonstrated by elevations of bilirubin, serum glutamic oxalocetic transaminase (SGOT), serum glutamic pyruvic transaminase (SGPT), and preoperative DIC. It is concluded that the LeVeen shunt coagulopathy is DIC, and may be related to exposure of the systemic circulation to FSP-rich ascitic fluid that may activate the coagulation mechanism. Bleeding complications do not appear to be related to the severity of the post shunt coagulopathy, but rather to the severity of liver dysfunction and presence of preoperative DIC (probably caused by the liver disease).

Adult↗
PubMed1983

Enflurane hepatotoxicity. A clinicopathologic study of 24 cases.

Analysis of 24 cases of enflurane anesthesia-associated hepatic injury shows that the clinical, biochemical, and histologic features are similar to those seen with halothane- and methoxyflurane-related hepatitis. Postoperative fever was the presenting symptom in 19 patients. Jaundice occurred in 19 patients after a mean latent period of 8 days. Sixteen patients had been previously exposed to enflurane or halothane, and the latent period from exposure to the onset of symptoms or jaundice was shortened in these patients. There were five fatalities among the entire group. Liver biopsy most characteristically showed centrilobular necrosis, occasionally with ballooning degeneration and fatty change. The presumed mechanism of injury is metabolic idiosyncracy, and prior exposure to a haloalkane anesthetic may increase the risk of hepatic injury after enflurane administration.

Adolescent↗
PubMed1983

Treatment of gastric ulcer. What is old and what is new.

The rational treatment of gastric ulcer (GU) requires both an understanding of the various causative factors responsible for what is best considered a spectrum of disorders, as well as a familiarity with the newer antisecretory and cytoprotective therapies that are available. Gastric ulcers that fulfill the criteria to be peptic ulcers (ie, occur in the antrum in the presence of excess luminal acid, with or without a coexisting duodenal ulcer) are best treated with a histamine H2-receptor antagonist. For GUs that develop in the setting of normal or reduced acid output, or those that occur as a result of direct (ie, drug-induced or bile acid-related) mucosal injury, use of a cytoprotective agent (eg, sucralfate) is the treatment of choice. Any GU that fails to heal within 12 to 15 weeks should be carefully examined to exclude the presence of a malignant neoplasm and should be considered for surgical resection.

Aged↗
PubMed1983

An immunologic study of spouses and siblings of asymptomatic hemophiliacs.

A transmissible agent may be responsible for the recent occurrence of the acquired immune deficiency syndrome (AIDS) in female heterosexual partners and infant contacts of AIDS victims or of persons at increased risk for AIDS. Therefore, we evaluated the clinical and immunologic status of 18 hemophiliac-spouse pairs and 19 hemophiliac-sibling pairs. Using surface marker assays of lymphocyte subpopulations, we found a mean T helper cell/T suppressor cell (H/S) ratio +/- SEM in 18 hemophiliac adults of 1.11 +/- 0.15 (11 less than 1.00), in their 18 spouses of 1.88 +/- 0.13 (none less than 1.00), in 19 hemophiliac children of 1.54 +/- 0.11 (3 less than 1.00), and in their 19 siblings of 1.87 +/- 0.11 (none less than 1.00). Both hemophiliac adult and hemophiliac children mean H/S ratios differed significantly (p less than 0.05) from the control mean ratio of 2.22 +/- 0.16 and from the mean of their respective nonhemophiliac spouse or sibling group (p less than 0.05). The mean ratio of hemophiliac adults differed significantly from that of hemophiliac children (p less than 0.05). These findings suggest that lymphocyte alterations in hemophiliacs are secondary to their therapy, are not influenced by genetic factors, and are not related to an infectious agent that is easily transmissible by close contact.

Acquired Immunodeficiency Syndrome↗
PubMed1982

Delta infection and liver disease in hemophilic carriers of hepatitis B surface antigen.

The prevalence of infection with hepatitis B virus (HBV)-associated delta (delta) agent was assessed in 277 treated hemophiliacs (primarily adolescents and adults) and 24 treated hemophilic children. Hemophiliacs who carry hepatitis B surface antigen (HBsAg) are at high risk of delta infection. Antibody to delta (anti-delta) was found in 14 (49%) of 29 HBsAg-positive adult or adolescent hemophiliacs and four (25%) of 16 HBsAg-positive hemophilic children; it was identified in none of the patients without serologic evidence of exposure to HBV and only occasionally and in low titers in hemophiliacs convalescent from HBV infection. Either histologic or biochemical evidence of chronic hepatitis was found in 10 (56%) of 18 HBsAg-positive hemophiliacs with anti-delta. In two patients with anti-delta a potentially pathogenic role for delta was suggested by the intrahepatic expression of delta antigen, detected by immunofluorescence. It appears that delta infection is a major cause of chronic liver disease in hemophiliacs.

Adolescent↗
PubMed1982

Bleeding and coagulation abnormalities in alcoholic cirrhotic liver disease.

Coagulation profiles were performed in 30 consecutive alcoholic cirrhotic patients without known infection, malignancy, recent surgery, transfusion, or alcoholic intake. Hemorrhagic phenomena were present in 70% and included gastrointestinal bleeding, oozing from venipuncture sites, bruising, and epistaxis. All 30 patients had multiple liver function and coagulation abnormalities, the most frequent of which were increases in F VIII components and decreases in F XI and F VII. Also decreased in half or more of the 30 patients were Fletcher F, F II, F X, prothrombin time (PT), partial thromboplastin time (APTT), thrombin time (TT), reptilase time (RT), anti-thrombin III, and plasminogen. When comparing cirrhotic bleeders with nonbleeders, four parameters were significantly different in those with a bleeding tendency: F VII, anti-thrombin III, plasminogen, and albumin. The prolonged APTT was associated in four cases with a blocking inhibitor of unknown etiology. The prolonged TT and RT, in the absence of fibrin split products, fibrin monomers, DIC, or shortened euglobulin lysis time in any patient were suggestive of an abnormal fibrinogen, a dysfibrinogen. In three other patients, there was an inhibitor of the TT. Further investigation of the suspected dysfibrinogen in 21 patients by SDS-polyacrylamide gel electrophoresis revealed that the molecular weights of the Aalpha, Bbeta, and gamma polypeptide chains of fibrinogen were not different from normal. Two-dimensional immunoelectrophoresis of the suspected dysfibrinogen was similar to normal in 18 of 21 patients, with loss of the initial shoulder in three.

Adult↗
PubMed1982

Gastric carcinoma in patients with unoperated duodenal ulcer disease.

Gastric cancer may develop in the presence of active or chronic duodenal ulcer disease in the unoperated stomach. This association is considered rare, especially since the presence of a duodenal ulcer is believed to protect against the development of a gastric malignancy. In this review, we describe two patients in whom gastric cancer was diagnosed after a long history of duodenal ulcer disease. Also presented is a review of 236 additional cases from the literature Patients with gastric cancer associated with duodenal ulcer disease tended to be younger than patients who develop gastric cancer not associated with duodenal ulcer. When compared to patients with gastric remnant (stump) cancer, the latency from onset of ulcer symptoms to the diagnosis of gastric cancer was shorter than the latency for stump cancer development, but the risk of developing stump cancer appears to be greater. The natural history and prognosis of this entity is otherwise similar to gastric cancer unassociated with duodenal ulcer and to stump cancer. A change in ulcer symptoms is an insensitive clue to the presence of gastric cancer in this group. The diagnosis may be delayed in some individuals whose gastric cancers respond to cimetidine or other agents.

Adenocarcinoma↗
PubMed1982

Pioneer growth cones in virgin mesenchyme: an electron-microscope study in the developing chick wing.

Axons grow out along predictable routes of specific destinations. An EM study of the early development of one of the main chick wing nerves - the interosseous nerve - helps to show how they do it. The growing tip of the nerve appears frayed, consisting of nerve fibres occurring singly or in small bundles, taking slightly divergent paths. Most of these pioneer nerve fibres have the characteristic appearance of growth cones. They are not seen to advance along fibrils of the intercellular matrix (as one might expect from some tissue culture experiments), but instead are generally in close contact over their whole surface either with mesenchyme cells or with other nerve fibres. The same is true of the axons at more proximal levels of the developing nerve: they too are never naked, but always in contact either with other axons or with mesenchyme cells. Later nerve fibres follow the pioneers: their growth cones travel out in contact with the pre-existing axons, within the primitive perineurium formed by the enveloping mesenchyme cells, and most often close to the periphery of the fascicle.

Animals↗