Influence of Blood Count,Cardiovascular Risks,Inherited Thrombophilia,and JAK2 V617F Burden Allele on Type of Thrombosis in Patients With Philadelphia Chromosome Negative Myeloproliferative Neoplasms

Introduction

Thrombosis is the most common complication in Philadelphia chromosome negative (Ph?) myeloproliferative neoplasms patients.

(Mis)placed central venous catheter in the left superior intercostal vein

BACKGROUND: Chest X-ray is routinely performed to check the position of the central venous catheter (CVC) inserted through the internal jugular or subclavian vein, while the further evaluation of CVC malfunction is usually performed by contrast venography. In patients with superior vena cava obstruction, the tip of the catheter is often seen in collateral mediastinal venous pathways, rather than in the superior vena cava. In such cases detailed knowledge of thoracic vessel anatomy is necessary to identify the exact location of the catheter. CASE REPORT.: We report a case of 32-year-old female patient with relapsing mediastinal lymphoma and previous superior vena cava obstruction with collateral azygos-hemiazygos venous pathways. The patient had CVC inserted through the left subclavian vein and its position was detected by CT to be in the dilated left superior intercostal vein and accessory hemiazygos vein. Considering that dilated accessory hemiazygos vein can tolerate infusion, the CVC was left in place and the patient had no complaints related to CVC (mal)position. Furthermore, we present anatomical and radiological observations on the azygos-hemiazygos venous system with the special emphasis on the left superior intercostal vein. CONCLUSIONS: Non-contrast CT scans can be a valuable imaging tool in the detection of the CVC position, especially in patients with renal insufficiency and contrast media hypersensitivity.     

Polycyclic aromatic hydrocarbons in the air near gas field Molve

Small-volume air samples (approximately 7 m3 per 24 h) of airborne PM10 particle fraction were collected on quartz fibre filters at two measuring sites in the vicinity of the gas field Molve in April and July 2006. It took five to seven days for each sample to collect and one month to collect five to seven samples. Mass concentrations of PM10 fractions were determined by gravimetry while PAHs were analysed using a HPLC with a fluorescent detector. The analysis included fluoranthene (Flu), pyrene (Pyr), benzo(a)anthracene (BaA), chrysene (Cry), benzo(b)fluoranthene (BbF), benzo(k)fluoranthene (BkF), benzo(a)pyrene (BaP), benzo(ghi)perylene (BghiP) and indene(1,2,3-cd)pyrene (Ind). Average concentrations of all PAHs in April at site A were slightly higher than at site B while in July they were lower and similar on both sites. Average BaP concentration measured at site A in April was 0.156 ng m(-3) and at site B 0.129 ng m(-3), while July BaP averages were 0.022 ng m(-3) at both sites. In both months, the mass concentration of BaP was lower than the limit value (1 ng m(-3)) and well below the tolerant value (2 ng m(-3)) set by a Croatian regulation of 2005. This suggests that the air near gas field Molve was of acceptable quality in respect to BaP at the time of the measurement.     

Gastric outlet obstruction in children: an overview with report of Jodhpur disease and Sharma's classification

Background/Purpose

Gastric outlet obstruction (GOO) is overwhelmingly described as infantile hypertrophic pyloric stenosis that overshadows other causes; thereby, there is a need to develop an elaborate and comprehensive classification. We present the same and establish a new disease called primary acquired gastric outlet obstruction during infancy and childhood that was reported for the first time by us in 1997, reviewing the literature published since then, with report of our new cases.

Methods

Eight patients of this disease presented at our center from 1996 to May 2008, and these were analyzed epidemiologically and clinically. Other reports published in literature were compared, and all reported patients were compiled.

Results

All 8 cases presented at age ranging from 1 month to 6 years, with mean age of 2.9 years; there was predilection for male sex (male, 7; female, 1). Presenting symptoms and signs were nonbilious vomiting, weight loss, dehydration, dyselectrolytemia, abdominal pain, and visible gastric peristalsis. Upper gastrointestinal contrast study showed large stomach and increased gastric emptying time. Endoscopy displayed normal stomach without intraluminal lesion. Exploration revealed a dilated stomach, without any pyloric muscular hypertrophy, scarring, inflammation, or obstruction externally or intraluminally. Result of histopathologic examination was normal. All patients except 1 (died of unrelated cause) were cured by Heineke-Mikulicz pyloroplasty.

Conclusions

These patients represent a new disease entity designated as primary acquired gastric outlet obstruction during infancy and childhood. Author's classification is complete and scientific, encompassing all rare causes of GOO. We nickname this idiopathic variety of GOO the Jodhpur disease, the place of the first cases.     

The impact of KIR2DS4 gene on clinical outcome after hematopoietic stem cell transplantation

Killer cell immunoglobulin-like receptors (KIR) are a family of inhibitory/activating receptors expressed on NK cells. Interactions of KIR receptors with KIR ligands have been shown to modify hematopoietic stem cell transplantation (HSCT) outcome. The aim of this research was to determine the KIR2DS4 allele variants distribution among 111 patients with different hematological malignancy who underwent HSCT and their donors, and to evaluate KIR2DS4 alleles’ impact on HSCT outcome. The KIR gene frequency analysis showed a significantly higher incidence of full-length KIR2DS4 alleles among patients. The impact of KIR2DS4 alleles on transplantation outcomes revealed that donors’ full-length KIR2DS4 alleles is associated with lower overall survival rates, higher risk of GVHD and higher relapse incidence. The expression of full-length KIR2DS4 allele variants may contribute to a worse clinical outcome after HSCT. KIR typing for KIR2DS4 could be used as an additional criterion for selecting suitable donors in cases when more than one HLA identical donor is identified for a specific patient.     

Injuries of the retrohepatic inferior vena cava and the liver

BACKGROUND: Injuries of the retrohepatic inferior vena cava, and the liver have mortality rate up to 71-78%. We presented a patient with combined injury of the retrohepatic inferior vena cava, liver, craniocerebral and thoracic traumas, inflicted in a traffic accident. CASE REPORT: Man, 20 years old has been injured in a traffic accident. At admission, 20 minutes after the injury, the patient was comatose and hypotensive. Bloody content was obtained by abdominal tracer. The patient underwent emergent laparotomy, utilizing trifurcated incision and cell saver device. Abdominal exploration revealed two litres of free blood and massive retroperitoneal hematoma. Manual compression of the liver was done, as well as perihepatic packing, complete hepatic vascular exclusion and mobilization of the right liver lobe. Due to impressive chemodynamic instability supraceliac aortic clamping was performed. Upon exposure of the retrohepatic inferior vena cava and right liver lobe, multiple lacerations of retrohepatic inferior vena cava and right hepatic vein, and right hepatic vein avulsion were found. We also identified an injury of VII and VIII segments of the liver (grade V according to the Moore's classification). Nonexpansive hepatoduodenal ligament hematoma and the injury of II and III segments of the liver group II/III according to Moore were found. Venorrhaphy of the inferior vena cava was done in the area of circumference of the right hepatic vein, a portion of which served as autologous vein patch. Continuous prolene 3/0 venorrhaphy of the distal caval laceration was done. Total caval and aorta clamping time of the inferior vena cava was 41 minutes. Atypical resection, debridment, of hepatic segments was done by using a harmonic scalpel. Hepatoduodenal ligament was declamped after 65 minutes. Flbrin glue was applied on the resectioned area of liver. The patient received 3.2 1 of autologuos blood transfusion with 5 units of packed red blood cells, 6 units of fresh frozen plasma, 13 units of concentrated thrombocytes and 15 units of cryoprecipitates. Due to coagulopathy, factor rVIIa was administered. Bilateral toracal drainage was done. Small bilateral contusions of the frontal part of the brain were noticed but the patient successfully recovered and was dismissed after three weeks. CONCLUSION: Combined injuries of the inferior vena cava and the liver befall into the most complex vascular traumas, thus representing a challenge for any complete medical team to manage them. The patient presented in our study was urgently transported to the hospital, immediately operated on applying modern doctrines of anesthesiology, transfusiology and vascular surgery that, all together, resulted into favorable treatment outcome with no distant complications.     

Point-of-care i-STAT cardiac troponin I for assessment of patients with symptoms suggestive of acute coronary syndrome

BACKGROUND: Few studies have investigated the role of cardiac troponin point-of-care (POC) testing for predicting adverse outcomes in acute coronary syndrome (ACS) patients. We investigated the use of a POC cTnI assay in ACS patients. METHODS: We studied consecutive patients (n = 367) presenting with symptoms suggestive of ACS who were admitted through the emergency department. We measured plasma cTnI with the i-STAT assay. Patients were risk-stratified based on cTnI concentrations defined by the predetermined 99th percentile reference limit for plasma (0.04 microg/L). Patients were followed for 60 days. We computed survival and event curves with the Kaplan-Meier method and compared risk stratification groups with the log-rank test. RESULTS: Acute myocardial infarction (MI) was diagnosed in 8.1% of patients. Odds ratios and 95% confidence intervals for all-cause death (ACD), MI or ACD, MI or cardiac death, and cardiac death at 60 days were all statistically significant after adjustment for age, diabetes, hypertension, and history of renal failure as follows: 2.54 (1.24-5.20), P = 0.009; 2.76 (1.37-5.58), P = 0.003; 5.98 (1.65-21.7), P = 0.008; and 2.54 (1.24-5.20), P = 0.009. Kaplan-Meier curves showed early separation between patients with increased vs. reference concentrations before 30 days for ACD, MI or ACD, and MI or cardiac death. CONCLUSION: The i-STAT POC cTnI assay can be added to the list of assays for risk stratification.     

Clinical comparison of V122I genotypic variant of transthyretin amyloid cardiomyopathy with wild-type and other hereditary variants: a systematic review

Heart Failure Reviews - V122I genotype variant (pV142I) is the most common hereditary transthyretin amyloidosis (hATTR) in the USA, with 3–3.5% of African-Americans being the carriers of this...