Segmental intestinal muscular thinning: a possible cause of intestinal obstruction in the newborn

Intestinal obstruction proximal to a transition zone without an interposed physical barrier usually indicates Hirschsprung disease. The authors report one case of focal small bowel muscular thinning just distal to a transition zone that produced clinical and radiographic findings that simulated long-segment Hirschsprung disease in a 2-day-old infant.     

Common bile duct calculi: updated experience with dissolution with methyl tertiary butyl ether

The authors describe their experience with methyl tertiary butyl ether (MTBE) in a larger series of patients than previously reported in order to acquaint physicians with both its effectiveness for dissolution of common bile duct calculi and the limitations of its use. Ten patients with 13 biliary calculi underwent percutaneous stone dissolution treatment with the experimental cholesterol solvent, MTBE. Three stones completely dissolved within 30 minutes, seven were reduced in size, and three were visibly unaffected. All stones not completely dissolved were easily extracted by means of a stone basket except for one in a patient taken to surgery. Although MTBE perfusion is an effective technique for management of biliary calculi, practitioners should be aware that its use is quite time consuming and its odor difficult to control.     

The lipoprotein-associated coagulation inhibitor that inhibits the factor VII-tissue factor complex also inhibits factor Xa: insight into its possible mechanism of action

Blood coagulation is initiated when plasma factor VII(a) binds to its essential cofactor tissue factor (TF) and proteolytically activates factors X and IX. Progressive inhibition of TF activity occurs upon its addition to plasma. This process is reversible and requires the presence of VII(a), catalytically active Xa, Ca2+, and another component that appears to be associated with the lipoproteins in plasma, a lipoprotein-associated coagulation inhibitor (LACI). A protein, LACI(HG2), possessing the same inhibitory properties as LACI, has recently been isolated from the conditioned media of cultured human liver cells (HepG2). Rabbit antisera raised against a synthetic peptide based on the N-terminal sequence of LACI(HG2) and purified IgG from a rabbit immunized with intact LACI(HG2) inhibit the LACI activity in human serum. In a reaction mixture containing VIIa, Xa, Ca2+, and purified LACI(HG2), the apparent half-life (t1/2) for TF activity was 20 seconds. The presence of heparin accelerated the initial rate of inhibition threefold. Antithrombin III alpha alone had no effect, but antithrombin III alpha with heparin abrogated the TF inhibition. LACI(HG2) also inhibited Xa with an apparent t1/2 of 50 seconds. Heparin enhanced the rate of Xa inhibition 2.5-fold, whereas phospholipids and Ca2+ slowed the reaction 2.5-fold. Xa inhibition was demonstrable with both chromogenic substrate (S-2222) and bioassays, but no complex between Xa and LACI(HG2) could be visualized by sodium dodecyl sulfate-polyacrylamide gel electrophoresis (SDS-PAGE). Nondenaturing PAGE, however, showed that LACI(HG2) bound to Xa but not to X or Xa inactivated by diisopropyl fluorophosphate. Thus, LACI(HG2) appears to bind to Xa at or near its active site. Bovine factor Xa lacking its gamma-carboxyglutamic acid-containing domain, BXa(-GD), through treatment with alpha-chymotrypsin, was used to further investigate the Xa requirement for VIIa/TF inhibition by LACI(HG2). LACI(HG2) bound to BXa(-GD) and inhibited its catalytic activity against a small molecular substrate (Spectrozyme Xa), though at a rate approximately sevenfold slower than native BXa. Preincubation of LACI(HG2) with saturating concentrations of BXa(-GD) markedly retarded the subsequent inhibition of BXa. The VII(a)/TF complex was not inhibited by LACI(HG2) in the presence of BXa(-GD), and further, preincubation of LACI(HG2) with BXa(-GD) slowed the inhibition of VIIa/TF after the addition of native Xa. The results are consistent with the hypothesis that inhibition of VII(a)/TF involves the formation of a VIIa-TF-XA-LACI complex that requires the GD of XA.(ABSTRACT TRUNCATED AT 400 WORDS)     

The biochemical and clinical consequences of 2'-deoxycoformycin in refractory lymphoproliferative malignancy

A deficiency of adenosine deaminase, an enzyme important in purine nucleoside catabolism, is associated with a severe combined immunodeficiency disease in children. Inhibition of this enzyme in vitro and in vivo results in an impairment in lymphoblast proliferation. We have investigated the pharmacologic inhibition of this enzyme by 2'-deoxycoformycin in 15 patients with hematologic malignancies. Biochemical consequences of the administration of this agent were closely monitored in erythrocytes, nucleated peripheral blood and bone marrow cells, serum, and urine. A marked rise in erythrocyte dATP was accompanied by a depletion of ATP in those patients exhibiting toxicity. Most patients excreted large amounts of deoxyadenosine but not adenosine in the urine. Serum deoxyadenosine rose in patients demonstrating a marked decrease in cell mass. The biochemical disturbances and clinical toxicity, including hepatic, renal, and conjunctival abnormalities, were usually reversible. Central nervous system toxicity, which potentially was the most serious consequence, was associated with high erythrocyte dATP/ATP ratios and high levels of cerebrospinal fluid deoxyadenosine. In patients with lymphoma and leukemia, objective responses were observed but were short- lived. Patients with chronic lymphocytic leukemia receiving weekly low doses of the drug demonstrated minimal toxicity and some efficacy. The chemotherapeutic potential o 2'-deoxycoformycin, as either a single agent or in combination with Ara-A, merits further exploration.     

Recent advances in T-cell regulation relevant to inflammatory dermatopathology

Inflammatory dermatoses encompass an enormous area of dermatopathology. Our understanding of the subject comes from combination of histopathological observations and relevant clinical information. Diagnoses are generally reached at the hematoxylin and eosin (H&E) level by using various pattern recognition approaches including one devised by Dr Ackerman et al. ¹ Recent advances in cell biology and immunology especially the field of T-cell regulation shed light to the intricate cellular interactions, associations and connect to inflammatory dermatopathology. This review attempts to identify and put into context the most significant advances in cellular biology relevant to the topic. Most of the information presented here is not necessarily relevant to our regular work at the moment; however, the new information will surely channel into our practice to provide a better, more accurate, semi-individualized diagnostic approach in the not too far future.     

Differences in biopsy techniques of actinic keratoses by plastic surgeons and dermatologists: a histologically controlled pilot study

OBJECTIVE: To compare differences in biopsy techniques of actinic keratoses between dermatologists and plastic surgeons. DESIGN: Blinded, comparative, retrospective study. SETTING: Dermatopathology laboratory at a major academic medical center with referral of outside cases.Intervention We reexamined the histopathologic slides of 405 actinic keratosis biopsy specimens obtained by plastic surgeons and dermatologists from January 1, 1992, through May 31, 2002. We were specifically interested in the type of biopsy technique (shave, punch, or excisional biopsy) used for the surgical management of actinic keratoses by both groups of physicians. We also recorded the clinical diagnoses rendered on the dermatopathology request form and compared them with the histopathologic diagnoses. RESULTS: Excisional biopsies were performed by plastic surgeons in 50.0% of the cases, compared with only 1.4% by dermatologists. In contrast, shave biopsies of actinic keratoses were performed by plastic surgeons in only 32.4% of the cases, compared with 89.4% by dermatologists. Only 1 (0.5%) of the 198 dermatopathology request forms submitted by the plastic surgeons mentioned actinic keratosis, compared with 82 (39.6%) of 207 histopathologic evaluation requests submitted by dermatologists. CONCLUSIONS: The predominance of excisional biopsies of actinic keratoses by plastic surgeons may be related to a different ability in the clinical recognition of actinic keratoses compared with that of dermatologists. The surgical approach of dermatologists to shave diagnostically uncertain cutaneous lesions is less invasive than that of plastic surgeons and is more likely to achieve a better cosmetic outcome.     

Core strength: A new model for injury prediction and prevention

Objective  

Many work in injury prone awkward positions that require adequate flexibility and strength in trunk stabilizer muscle groups. Performance on a functional movement screen (FMS) that assessed those factors was conducted and an intervention was designed.