Hypereosinophilic syndrome: diagnosis and treatment

PURPOSE OF REVIEW: Hypereosinophilic syndrome is increasingly recognized as a heterogeneous group of disorders, in some cases with precisely defined pathogenesis, which has led to changes in diagnostic approaches and therapeutic strategies. An update on causes and modern therapy is presented here. RECENT FINDINGS: Clonal eosinophilias belong to the group of myeloid malignancies. Karyotypically occult FIP1L1- platelet-derived growth factor receptor alpha and beta rearranged eosinophilic disorders respond to imatinib mesylate with almost 100% efficacy. If standard therapies fail, the FIP1L1- platelet-derived growth factor receptor-negative cases of hypereosinophilic syndrome should also be considered for treatment with imatinib. The recognition of acquired resistance to imatinib has aroused interest in developing new tyrosine kinase inhibitors. Other subgroups of clonal eosinophilias have been molecularly defined, but the curative verification of pathogenetic relevance has not been certified. Hypereosinophilic syndrome patients with abnormal T-cell populations have benefited from treatment with anti IL-5 monoclonal antibodies. SUMMARY: The FIP1L1- platelet-derived growth factor receptor alpha and beta-positive patients, and those with abnormal T-cell populations are currently the only clearly defined treatable subgroups of hypereosinophilic syndrome. The FIP1L1- platelet-derived growth factor receptor alpha-negative responders to imatinib pose a question as to the existence of subentities with unrecognized tyrosine kinases-based mutation. The search for such cases and other treatable subgroups of hypereosinophilic syndrome has already begun.     

Fatal human herpesvirus 6-associated multifocal meningoencephalitis in an adult female patient

Human herpesvirus 6 (HHV 6) is a known cause of central nervous system infection in immunocompromised patients. Less is known about the clinical course of HHV 6 encephalitis in immunocompetent patients. We report a case of meningoencephalitis in a 42-y-old immunocompetent patient associated with HHV 6 infection.     

Clinical Significance of Laboratory-determined Aspirin Poor Responsiveness After Primary Percutaneous Coronary Intervention

Aims

The objective of the present substudy was to examine whether aspirin poor/high responsiveness (APR/AHR) is associated with increased rates of major adverse cardiovascular events (MACE) and serious bleeding after primary percutaneous coronary intervention (PPCI).

Methods

We analyzed 961 consecutive ST-elevation acute myocardial infarction patients who underwent PPCI between February 2008 and June 2011. Multiplate analyser (Dynabite, Munich, Germany) was used for the assessment of platelet reactivity. APR/AHR were defined as the upper/lower quintiles of ASPI values, determined 24 h after aspirin loading. APR patients were tailored using 300 mg maintenance dose for 30 days. The co-primary end points at 30 days were: MACE (death, non-fatal infarction, ischemia-driven target vessel revascularization and ischemic stroke) and serious bleeding according to the BARC classification.

Results

One hundred and 90 patients were classified as APR, and 193 patients as AHR. At admission, compared with aspirin sensitive patients (ASP), patients with APR had more frequently diabetes, anterior infarction and heart failure, while AHR patients had reduced values of creatine kinase, leukocytes, heart rate and systolic blood pressure. Compared with ASP, the rates of 30-day primary end points did not differ neither in APR group including tailored patients (MACE, adjusted OR 1.02, 95%CI 0.47-2.17; serious bleeding, adjusted OR 1.92, 95%CI 0.79-4.63), nor in patients with AHR (MACE, adjusted OR 1.58, 95%CI 0.71-5.51; serious bleeding, adjusted OR 0.69, 95%CI 0.22-2.12).

Conclusions

The majority of APR patients were suitable for tailoring. Neither APR including tailored patients nor AHR were associated with adverse 30-day efficacy or safety clinical outcomes.

     

Staphylococcus sciuri: an unusual cause of pelvic inflammatory disease

We present the case of polymicrobial pelvic inflammatory disease (PID) that involved Staphylococcus sciuri, S. epidermidis, and Streptococcus agalactiae. In order to determine the frequency of S. sciuri isolation from the female lower genital tract, 3415 vaginal samples were analysed during the one-year study period. S. sciuri was isolated from three (0.09%) samples. In all the three cases, S. sciuri was obtained in mixed culture from outpatients without symptoms of infection. While the origin of S. sciuri in the female genital tract remains to be elucidated, the present study showed that this bacterium may colonize vagina and, moreover, may be involved in the pathogenesis of an infection as serious as PID. The low rate of isolation we established, however, indicates infrequent and, most probably, transient colonization of the female genital tract by S. sciuri.     

Partial left ventriculectomy improves left ventricular end systolic elastance in patients with idiopathic dilated cardiomyopathy

OBJECTIVE: To assess the effect of partial left ventriculectomy (PLV) on estimate of left ventricular end systolic elastance (Ees), arterial elastance, and ventriculoarterial coupling. PATIENTS: 11 patients with idiopathic dilated cardiomyopathy before and two weeks after PLV, and 11 controls. INTERVENTIONS: Single plane left ventricular angiography with simultaneous measurements of femoral artery pressure was performed during right heart pacing before and after load reduction. RESULTS: PLV increased mean (SD) Ees from 0.52 (0.27) to 1.47 (0.62) mm Hg/ml (p = 0.0004). The increase in Ees remained significant after correction for the change in left ventricular mass (p = 0.004) and end diastolic volume (p = 0.048). As PLV had no effect on arterial elastance, ventriculoarterial coupling improved from 3.25 (2.17) to 1.01 (0.93) (p = 0.017), thereby maximising left ventricular stroke work. CONCLUSION: It appears that PLV improves both Ees and ventriculoarterial coupling, thus increasing left ventricular work efficiency.     

Effect of surgical incision on pain and respiratory function after abdominal surgery: a randomized clinical trial

BACKGROUND/AIMS: The aim of the present study was to assess the severity of respiratory disturbances occurring after abdominal surgery and to identify surgical incisions that entail the least respiratory complications and postoperative pain. METHODOLOGY: A total of 105 patients divided into two groups were included in this randomized clinical trial. Seventy-five patients in the first group underwent upper abdominal surgery, and were operated on by use of vertical, oblique, transverse and elliptic incision. The remaining 30 patients in the second group were submitted to low abdominal surgery by use of vertical and oblique incision. Arterial blood gases and pulmonary shunt development were determined at 12 h preoperatively, and at 6 h, 72 h and 144 h postoperatively. During the postoperative course, VAS-pain score and use of tramadol were observed according to the type of surgical incision employed. RESULTS: Most severe respiratory disturbances in terms of PaO2 decrease, activating compensatory hyperventilation, pulmonary shunt increase, the highest VAS-pain score and consumption of tramadol were induced by the following upper abdominal incisions: transversal laparotomy according to Orr, elliptic periumbilical laparotomy, upper midline laparotomy, transrectal laparotomy and subcostal laparotomy. In the group of low abdominal surgery (low midline laparotomy and inguinal incision) consumption of tramadol was statistically significantly lower, but no statistically significant differences were recorded between the baseline preoperative and postoperative values of PaO2, PaCO2 and pulmonary shunt. CONCLUSIONS: All low abdominal incisions were found to entail statistically significantly less respiratory disturbances, lower VAS-pain score and lower tramadol use when compared to upper abdominal incisions. The upper abdominal incisions observed caused substantial respiratory disturbances including hypoxia, hyperventilation and pulmonary shunt increase.     

Evaluation of the Glasgow coma scale score in critically Ill infectious disease patients

Summary In this prospective study the Glasgow Coma Scale (GCS) score was evaluated in 107 critically ill infectious disease (ID) patients admitted to the Intensive Care Unit (ICU) during a 1-year period. Patients were separated into two groups: those affected by central nervous system (CNS) infections and those affected by infections other than of the CNS. There were no apparent differences in the first ICU day GCS score values between the two groups (11±4 vs. 11±4, p=0.5318). Univariate logistic regression analysis confirmed a significant relationship between the first ICU day GCS score and the subsequent ICU mortality in the group of patients with CNS infections (r=0.3152, p=0.0015) but not in the group with infections not affecting the CNS (r=0.0919, p=0.1106). Our preliminary results suggest that the prognostic value of the GCS score is valid only in patients with CNS infections but not in other ID patients.

---

Evaluierung der Glasgow Coma Skala bei schwerkranken Patienten mit Infektionen

Zusammenfassung Die Glasgow Coma Scale (GCS) wurde bei 107 schwerkranken Patienten mit Infektionen im Rahmen einer prospektiven Studie evaluiert. Es handelte sich um Patienten, die während eines Jahres in der Intensivpflegestation behandelt wurde. Sie wurden nach dem Vorliegen oder nicht Vorliegen einer Infektion des Zentralnervensystems in zwei Gruppen eingeteilt. In den GCS-Scores am ersten Tag der Intensivpflegebehandlung wiesen die beiden Gruppen keine Unterschiede auf (11±4 und 11±4; p=0,5318). Die logistische Regressionsanalyse für eine Variable deckte eine signifikante Beziehung zwischen dem GCS Punktewert am ersten Tag und der folgenden Sterberate bei Patienten mit ZNS-Infektionen auf (r=0,3152; p=0,0015). Bei Patienten, deren Infektion das ZNS nicht betraf, war eine derartige Beziehung nicht zu erkennen (r=0,0919; p=0,1106). Diese vorläufigen Ergebnisse lassen darauf schließen, daß der Glasgow Coma Score nur bei Patienten mit ZNS-Infektionen eine valide Funktion hat, nicht aber bei Patienten mit anderen Infektionen.

     

Orthodontic anomalies and malocclusions in Late Antique and Early Mediaeval period in Croatia

ObjectiveMalocclusions are relative infrequently analysed in bioarchaeological investigations and if investigated the samples are very small. This research provides analysis of orthodontic anomalies of even 1118 individuals from the Late Antique (LA) and Early Mediaeval (EM) period. Aims were to describe the prevalence of orthodontic anomalies in this historical period and to analyse which orthodontic anomalies are best suitable for bioarchaeological investigations.Methods1118 skulls were examined for anomalies of tooth number, tooth displacement (rotation, malposition, diastema and crowding) as well as for malocclusions.ResultsThe prevalence of hypodontia in the LA was 41.02% and 30.61% in the EM sample. Tooth displacement was noticed in 15.63% individuals from the LA and in 12.42% individuals from EM. About 26% of the LA sample and 7.19% of the EM sample were affected with tooth crowding and the difference was statistically significant.ConclusionOrthodontic anomalies affecting only one tooth or group of teeth are more suitable for examination in bioarchaeological investigations than orthodontic features requiring presence of both jaws and all or almost all teeth. Clinical investigation protocols and methodology should be adopted for bioarchaeological researches and international standards and recommendations should be established for this kind of investigation on skeletal remains.     

Cutaneous tuberculosis

Tuberculosis continues to be a significant health problem in developing countries. Although cutaneous tuberculosis is uncommon, disseminating skin involvement may still be seen, especially patients from rural areas. A case is reported of disseminated tuberculosis presenting with different clinical forms of cutaneous lesions, pulmonary and liver involvement in an immunocompetent patient.