Stress-induced polymorphous ventricular tachyarrhythmias in two brothers: Unusual pattern of inheritance in the long QT syndrome

Two brothers, 10 and 9 years old, are reported who were treated for years with antiepileptic drugs until the cardiac origin of their syncopal attacks was discovered. Our findings are consistent with the diagnosis of long QT syndrome, although no other family members are affected and only intermittent and mostly borderline QT prolongation could be found. Diagnostic difficulties and genetic implications are discussed.     

The significance of the systemic inflammatory response syndrome (SIRS) in 1278 trauma patients

Summary The association between the increasing severity of systemic inflammatory response syndrome (SIRS) and the incidence of posttraumatic complications and mortality was retrospectively investigated in 1278 injured patients. Patients were divided into three groups according to their Injury Severity Score (ISS) (group A: ISS L 9 K16 points (n = 626); group B: ISS > 16 < 40 points (n = 589); group C: ISS L 40 points (n = 63)). SIRS was defined according to the criteria of the American Consensus Conference. The number of fulfilled criteria determined its severity: moderate SIRS: 2 criteria fulfilled, intermediate SIRS: 3 criteria fulfilled, severe SIRS: 4 criteria fulfilled. Additionally, acute respiratory distress syndrome (ARDS) was defined according to the Murray-Score and the multiple organ dysfunction syndrome (MODS) according to the Goris-Score. The incidence of SIRS was 42 % in group A, 70 % in group B and 100 % in group C (p < 0.05). The severity of SIRS increased with severity of trauma. Moreover, 178 of all injured patients (14 %) developed septic complications. In parallel to SIRS, the incidence of these septic complications correlated with the severity of trauma. The occurrence and severity of ARDS and MODS correlated with increased severity of SIRS and septic complications. Among patients without SIRS 15 % developed ARDS and 21 % MODS. In contrast, patients with severe SIRS and septic complications demonstrated ARDS in 99 % and MODS in 97 %. In these patients, no correlation was found between the ISS and the incidence of ARDS or MODS. There were also stepwise increases in mortality rates in the hierarchy from SIRS to septic shock. While 13 of patients with modest SIRS (5 %) and 32 of patients with intermediate SIRS (13 %) died, the mortality rate of patients with severe SIRS was 19 % (p < 0.05). In addition, a significant correlation between the incidence of septic complications and mortality was found. Injured patients with sepsis died in 13 %, those with severe sepsis in 23 %, and patients with septic shock in 33 % (p < 0.05). Thus, the increasing severity of SIRS was associated with the occurrence of posttraumatic ARDS, MODS, and mortality. Using the number of fulfilled SIRS criteria for classifying systemic inflammation, its severity may be predictive for posttraumatic complications and outcome of injured patients.      

Annotated Bibliography: Pulmonary Disease

Hoidal JR, Niewoehner DF: Pathogenesis of emphysema. Chest 83:679-685, 1983.     

Isoelectric focusing of Protac, the protein C activator from copperhead (Agkistrodon contortrix) venom: a note on experimental problems

The isoelectric point of Protac was recently estimated to be at pH 3. However, further investigations using different experimental procedures revealed that Protac, due to its particular binding properties, is able to form complexes with carrier ampholytes. Thus, the actual isoelectric point of Protac was found to be in the basic region.     

Chromogenic proteinase substrates as possible tools in the characterization of Crotalidae and Viperidae snake venoms

Proteinase activities have been determined photometrically in 25 different Crotalidae and Viperidae snake venoms by using five different chromogenic proteinase substrates. The activity profiles obtained by listing the identity numbers of substrates hydrolyzed by the venoms in a decreasing potency order remained quite stable within a given snake population. Submission of a venom to various physical and chemical treatments did not alter its activity profile. It is therefore concluded that this simple method of determining proteinase activities could be of help in snake venom characterization, as well as in studying the influence of metallic ions and inhibitors of snake venom proteinases.