Duodenal and jejunal atresia with agenesis of the dorsal mesentery: "apple peel" small bowel.

Two newborn infants with duodenal and jejunal atresia and agenesis of the dorsal mesentery represent our surgical experience with "apple peel" small bowel or "christmas tree" demormity. The first patient had the typical appearance of this condition. The postoperative course was complicated by hyperbilirubinemia, septicemia, and disseminated intravascular coagulation. The infant is in satisfactory condition 1.5 years after operation. The second patient had agenesis of the dorsal mesentery without spiraling of the bowel around its vascular stalk. The child died after 1 month, with complete absence of extrahepatic bile ducts as seen at a second laparotomy. Neither child had been subjected to gastrostomy.     

Osteoplastische Behandlung der Osteomyelitis

Zusammenfassung Nach kurzer Einführung folgt die Begriffsbestimmung der osteoplastischen Behandlung der Osteomyelitis, bei der die Knochentransplantation nicht nur Knochenersatz leistet, sondern darüber hinaus ein wesentlicher Faktor der Heilung des osteomyelitischen Prozesses ist. Der Autor geht auf die Fragen der Indikationen, Eigenschaften des Knochenlagers, Wesen, Struktur und Konservierungsart des Transplantates, immunologische Probleme, Anwendungsmethoden, Dynamik des Umbaues des Transplantates und Ergebnisse ein. Er schlägt eine Skala für eine Qualifizierung des Empfängerlagers vor. Der Autor hat die osteoplastische Behandlung der Osteomyelitis im breiten Rahmen der verschiedenen Anwendungsmethoden entwickelt. Es wird zur Analyse und Diskussion ein Krankengut von 602 Fällen vorgeführt. Das Krankengut ist nach Kriterien, Alter, Lokalisation, Morphologie, Infektion und Operationsverfahren untersucht. Weiterhin ist die Charakteristik des Transplantates, Entnahmestellen und seine Konservierungsart aufgezeigt. Der Autor hat die Ausfüllung der Defekte an der Entnahmestelle des Autotransplantates (Spongiosa, Span) durch Allospongiosa eingeführt.Bei Ausfüllungen von 285 Entnahmestellen hat er keinerlei Komplikationen beobachtet.Die Beurteilung der Ergebnisse werden im Zusammenhang mit den Komplikationen bzw. Erkrankungen des Transplantates analysiert. Andererseits werden die Ergebnisse und die Komplikationen im Zusammenhang mit dem Alter, dem Wesen und der Struktur des Transplantates sowie in Beziehung zum Operationsverfahren beurteilt. Gesondert sind die Reoperationen aufgestellt. Es haben sich bei 436 Kranken mit hämatogener Osteomyelitis 58 und bei 166 Kranken mit traumatischer eitriger Ostitis 34 Reoperationen als notwendig erwiesen. Die Heilungsquote nach der osteoplastischen Behandlung ist für die hämatogene Osteomyelitis 86%, für die traumatische Ostitis 79%. Einschließlich der Reoperationen steigt die Heilungsquote auf entsprechend 97% und 98%.Der Autor führt eine neue Stadienskala für den Umbau des Transplantates ein, die 6 Stadien enthält und die den Verhältnissen bei der Osteomyelitis entspricht.

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Osteoplastic treatment of the osteomyelitis

Summary After a short introduction, a definition of the nature of the osteoplastic treatment is given, in which the bone grafting assures not only a bonesubstitution, but it is the motive factor for the treatment of the osteomyelitis.The author gives a discussion on the problems of indications, properties of the bone-bed, structure and methods of conservation of the graft, the immunological problems, methods of application, the dynamics of the graft's reorganization and the results. He proposes a scale for the properties of the bed-recipient. Osteoplastic treatment of the osteomyelitis is developed by the author in a large plan of different surgical-operative methods. A clinical material of 602 patients is included for analysis and discussion. Criteria for the clinical material are as follows: age, localization, morphology, infection and method of conservation are given. The author starts using allografting to replace the donor's damage, when the auto-bone is taken. There are any complications after 285 such substitutions. The results are analysed in connection with the complications, typical of the bone grafting add of the diseases of the grafts respectively. On other hand the results are analysed in connection with the age of the patient, nature and structure of the graft and the type of operation. The reoperations are analysed separately. In 436 patients with haematogenic osteomyelitis the reoperations are 58, and in 166 patients with traumatic purulent ostitis they are 34. With the osteoplastic treatment of the haematogenic osteomyelitis the curability is 86 per cent, and in the traumatic purulent ostitis is 79 per cent. After the reoperations these percentages ware corrected to 97 for the haematogenic osteomyelitis and 98 per cent for the traumatic purulent ostitis, respectively. The author introduces a new scale for the stages of reorganization of the graft, including 6 stages with good correspondation to the conditions of the osteomyelitis.

     

Free wall rupture (FWR) in patients with acute ST-elevation myocardial infarction (STEMI) receiving fibrinolytic therapy (FT): a 7-year prospective study

Previous studies have shown a paradoxical increase in early mortality in older patients (>70 years) with acute STEMI treated with fibrinolytic therapy (FT), which has been attributed to the development of free wall rupture (FWR). Our aim was to assess occurrence of FWR in STEMI patients receiving FT. In this 7-year prospective study, data from 1701 consecutive patients were obtained. We analyzed predictors of the in-hospital mortality in patients > 70 years old. The independent contribution of several variables to overall mortality and FWR development was assessed using multiple logistic regression analyses. The mortality of entire cohort was 18% (306/1701). Diabetes mellitus, anterior infarction, smoking, female gender and hypercholesterolemia were independent predictors of in-hospital mortality. FT was given to 18% of all patients (304/1701) of which 13% died (39/304). FWR was 18.4-times more often in patients who received FT. Among patients younger than 70 years who received FT there was no FWR, while in patients ≥70 years of age FWR was found in almost half of the deceased (30/68; 44%). Application of FT in STEMI patients is not associated with higher mortality, but significantly increases number of FWR, especially in patients over 70 years of age.     

Cardiologic predictors of sudden death in patients with myotonic dystrophy type 1

The aim of this study was to analyze survival, causes of death and cardiologic predictors of sudden death in a large cohort of patients with myotonic dystrophy type 1 (DM1). The study was comprised of 171 adult DM1 patients hospitalized at the Neurology Clinic in a 20-year period. Severe electrocardiographic (ECG) abnormality included at least one of the following: rhythm other than sinus, PR interval of ?240 ms, QRS complex duration of 120 ms or more, and second-degree or third-degree atrioventricular (AV) block. Survival data were analyzed by the Kaplan–Meier test, log–rank test and Cox regression analysis. During the mean follow-up period of 9.4 ± 5.4 years, a pacemaker was implanted in 5.8% of DM1 patients and 14% of patients died. The mean age at death was 55.6 ± 12.5 years. The most common causes of death in our cohort were sudden death (41.7%) and respiratory failure (29.2%). The presence of palpitations (hazard ratio [HR] = 4.7, p < 0.05) and increased systolic blood pressure (HR = 9.8, p < 0.05) were significant predictors of sudden death. Among ECG parameters, severe ECG abnormality (HR = 4.7, p < 0.05), right bundle branch block (RBBB; HR = 3.9, p < 0.05) and bifascicular block (HR = 5.8, p < 0.05) were significant predictors of sudden death.     

Clinical and epidemiological features of Guillain‐Barré syndrome in the Western Balkans

The aim of this study was to define features of Guillain‐Barré syndrome in a large cohort of patients from three Western Balkans countries. Data from adult Guillain‐Barré syndrome (GBS) cases from 2009 to 2013 were retrospectively obtained from all tertiary health care centers. During the 5‐year period, 327 new cases of GBS were identified with a male to female ratio of 1.7 : 1. The most common GBS variants were demyelinating (65%) and axonal (12%). At nadir 45% of patients were chair‐bound, confined to bed, or required assisted ventilation, while 5% died. The crude incidence of GBS in Serbia and Montenegro was 0.93 per 100,000 population, and age‐adjusted incidence according to the world standard population was 0.86 per 100,000. Incidence was particularly high in 50‐ to 80‐year‐old men. Statistically significant seasonal variations of GBS were not observed. This study of patients with GBS in the Western Balkans allows us to prepare the health system better and to improve the management of patients. This study also opens opportunities for international collaboration and for taking part in the multinational studies on GBS.     

Coenzyme specificity in fungal 17beta-hydroxysteroid dehydrogenase

The 17beta-hydroxysteroid dehydrogenase from the fungus Cochliobolus lunatus is an NADP(H)-dependent member of the short-chain dehydrogenase/reductase superfamily (SDR) that belongs to the cP1 classical subfamily. Here, we have created several mutants by site-directed mutagenesis, and through these we have studied the amino acid residues that are responsible for coenzyme binding and specificity. The Thr202Val and Thr202Ile mutants were inactive, thus confirming the importance of Thr202 for the appropriate orientation of the coenzyme that enables the hydride transfer. The Ala50Arg and Asn51Arg mutants had increased rates of NADPH dissociation, and thus an enhanced substrate oxidation with NADP+, while the Asn51Arg mutant also showed an increased rate of NADP+ dissociation, and thus an enhanced substrate reduction with NADPH. Addition of a negatively-charged amino acid residue at the first position after the second beta-strand (Tyr49Asp) affected the coenzyme specificity and turned the enzyme into an NAD+-dependent oxidase resembling the cD1d subfamily members.