Paroxysmal Dysarthria and Raynaud's Phenomenon in the Tongue

Abstract Three patients suffering from systemic scleroderma and Raynaud's phenomenon in the digits as well as the tongue are reported. Following exposure to cold, a vasospasm was observed in the digits and the tongue accompanied by severe dysarthria. These striking oral symptoms had been overlooked for years in the medical ward. It is recommended to question all patients with Raynaud's phenomenon about visceral manifestations during the digital attacks.     

No Liver 99Tc-tin-colloid Uptake in Primary Sclerosing Cholangitis

Abstract A ⁹⁹Tc-tin-colloid scintigraphy showed no uptake of the radiopharmaceutical in the liver of a patient with jaundice. The diagnosis was later established as primary sclerosing cholangitis     

Collagen biosynthesis enzymes in serum and hepatic tissue in liver disease

Abstract. Serum immunoreactive prolyl hydroxylase protein was measured in sixty-five patients with liver disease, and liver prolyl hydroxylase activity, immunoreactive prolyl hydroxylase protein and collagen hydroxyproline in forty of these patients. Serum immunoreactive prolyl hydroxylase protein was above the 95% confidence limit of the controls in most patients with primary biliary cirrhosis, portal cirrhosis, acute hepatitis and cancer with liver metastases, but below this in most patients with fatty liver, chronic active hepatitis, extrahepatic cholestasis, cholangitis, cancer without liver metastases and other malignant diseases. Elevated serum immunoreactive prolyl hydroxylase protein decreased rapidly with time in acute hepatitis but not in primary biliary cirrhosis. Liver prolyl hydroxylase activity and immunoreactive prolyl hydroxylase protein were elevated in the same diseases as serum immunoreactive prolyl hydroxylase protein, and correlated significantly with the latter whereas no correlation was found between serum immunoreactive prolyl hydroxylase protein and collagen hydroxyproline. Serum immunoreactive prolyl hydroxylase protein correlated highly significantly with serum alkaline phosphatase and weakly with serum aspartate aminotransferase in primary biliary cirrhosis, but not in any other disease. No correlation was found between serum immunoreactive prolyl hydroxylase protein and other tests of liver function. The results suggest that changes in serum immunoreactive prolyl hydroxylase protein in liver disease primarily reflect changes in this enzyme in the hepatic tissue, and that assays of serum immunoreactive prolyl hydroxylase in liver disease may give useful information on the actual hepatic collagen synthesis.     

THE USE OF IMMUNOHISTOCHEMISTRY TO IMPROVE SENSITIVITY AND SPECIFICITY IN THE DIAGNOSIS OF SYSTEMIC MYCOSES IN PATIENTS WITH HAEMATOLOGICAL MALIGNANCIES

The original histomorphological diagnoses in a series of 34 mycotic lesions from 23 patients with haematological malignancies were re-evaluated by immunohistochemistry. A panel of antibodies was used to identify the agents of aspergillosis, candidosis, fusariosis, scedosporiosis (pseudallescheriosis), and zygomycosis. Apart from improving the diagnosis of aspergillosis, candidosis, and zygomycosis, the application of immunohistochemistry also disclosed three lesions of aspergillosis which had been overlooked during the original screening. It is concluded that the use of immunohistochemistry for the diagnosis of common opportunistic mycoses will not only increase diagnostic specificity, but will also reveal more tissue infections than the conventional histomorphological examination of traditionally stained sections. © 1997 by John Wiley & Sons, Ltd.     

Pulmonary Arteriovenous Fistulas in Hereditary Hemorrhagic Telangiectasia

ABSTRACT. We reviewed all available chest X-rays of 95 patients with hereditary hemorrhagic telangiectasia (HHT). The diagnosis of pulmonary arteriovenous fistula (PF) had previously been made in 13 patients, nine of whom had been operated on. Another three cases were found in the review. The 16 patients with HHT and PF came from eight families, one of which contributed seven patients. Median observation time between the first and the latest chest X-ray examination was 11 years (range 1/2–35). Growth of the PF was seen in four patients and spontaneous regression in one. Four of the 16 patients with PF had symptoms consistent with cerebral embolism, while only two of the 79 patients without PF had such symptoms. This study is part of an epidemiological investigation of HHT—to our knowledge not carried out before. The calculated period prevalence of simultaneous HHT and PF in the decade 1964–74 in the county of Fyn (429 207 inhabitants) was 2.6 per 100 000.     

A variant form of X-linked chronic granulomatous disease with normal nitroblue tetrazolium slide test and cytochrome b

Chronic granulomatous disease was diagnosed in a boy who suffered from severe generalized infections. Family investigations revealed the inheritance of the disease to be X-linked. However, unlike other cases of X-linked chronic granulomatous disease, the membrane oxidase of the neutrophils from this patient was not totally defective and sufficient activity was left to result in a normal phorbol myristate acetate-stimulated nitroblue tetrazolium slide test. Also, unlike the usual findings in X-linked chronic granulomatous disease, cytochrome b was present in normal amounts in the neutrophils from this patient. The cytochrome was normal, judged from its midpoint potential of -245 mV and its ability to bind CO. It is thus apparent that X-linked chronic granulomatous disease may result from at least two different defects and that the phorbol myristate acetate stimulated nitroblue tetrazolium slide test fails to detect some cases.     

Hydrogen bond connectivity patterns and hydrophobic interactions in crystal structures of small,acyclic peptides

Crystal structures of all available unblocked linear peptides with two to five residues were retrieved from the Cambridge Structural Database and their intermolecular contacts and packing modes studied using molecular graphics. This survey reveals that interactions between hydrophobic portions of the molecules are critically important in determining the overall features of their crystal packing patterns. Distinct hydrophobic columns or layers are observed in almost all crystal structures. Analyses of the relationships between these interactions and crystal growth properties of small peptides are given. It is suggested that needle growth is promoted by hydrophobic packing, usually along a short crystallographic axis (4.6-6.0 Å). Also contributing to these morphologic characteristics are entropic factors associated with hydrophobic aggregation as well as tightly bound water molecules on hydrophobic faces. The paper also provides a comprehensive overview of hydrogen bond patterns in acyclic peptide crystals. It is demonstrated that one of their primary roles is to provide a scaffolding within which hydrophobic groups can aggregate. Even though there is a high density of hydrogen bonds in the crystals, often with complex patterns and networks, certain motifs are found to recur in a number of structures indicating specific hydrogen bond preferences. Water, for example, is an integral part of the hydrogen bond networks in these crystals, usually acting as the primary donor for main-chain carboxylate groups in peptide hydrates.     

Papillary Muscle Rupture as a First Event in Acute Myocardial Infarction

Abstract. Cardiogenic shock caused by papillary muscle rupture in acute myocardial infarction is potentially reversible by surgical treatment. A case of inferior myocardial infarction in a 56-year-old previously healthy man is reported, in which the first event was papillary muscle rupture. The patient was in shock and had a mitral insufficiency murmur. The diagnosis was made by echocardiography and ventriculography. A St. Jude valve was implanted, and the patient was discharged in good health. It is suggested that routine echocardiography be carried out on patients with sudden cardiogenic shock, when a mitral murmur is present.     

Salmonella or Campylobacter gastroenteritis prior to a cancer diagnosis does not aggravate the prognosis: a population‐based follow‐up study

Gradel KO, Nørgaard M, Schønheyder HC, Dethlefsen C, Ejlertsen T, Kristensen B, Nielsen H. Salmonella or Campylobacter gastroenteritis prior to a cancer diagnosis does not aggravate the prognosis: a population‐based follow‐up study. APMIS 2010; 118: 136–42. We hypothesized that preceding zoonotic Salmonella or Campylobacter gastroenteritis aggravated the prognosis in cancer patients. Exposed patients comprised all of those diagnosed with first‐time Salmonella/Campylobacter gastroenteritis from 1991 and with first‐time cancer diagnosis thereafter (through 2003) in two Danish counties. These patients were matched for main cancer type, gender, age and calendar period to unexposed cancer patients, i.e. those without Salmonella/Campylobacter gastroenteritis. We compared cancer stage by age‐ and comorbidity‐adjusted logistic regression analysis, survival by comorbidity‐adjusted Cox’s regression analysis and mortality dependent on the time period between Salmonella/Campylobacter gastroenteritis and cancer by spline regression curves. The study cohort comprised 272 Salmonella/Campylobacter‐exposed cancer patients and 2681 unexposed cancer patients. Prevalence odds ratios [95% confidence intervals (CI)] in exposed as compared with unexposed patients were 0.96 (0.74–1.25) for localized tumours, 1.15 (0.87–1.54) for regional spread and 1.14 (0.84–1.55) for metastases. Adjusted mortality rate ratios (95% CI) were 0.93 (0.75–1.16) for 0–1 year, 1.08 (0.84–1.39) for 2–5 years and 1.02 (0.60–1.73) for the remaining period. Mortality estimates did not change in relation to the time period between gastroenteritis and cancer. Salmonella/Campylobacter gastroenteritis prior to cancer was associated with neither the cancer stage nor a poorer prognosis.