Therapeutic results with apl 93 protocol in acute promyelocytic leukemia (34 cases)

BACKGROUND: Acute promyelocytic leukaemia (APL) account for approximately 10% to 15% of all AML in most reports. Clinical features includes the presence in 80% to 90% of patients of a severe hemorrhagic syndrome, a specific balanced translocation between chromosomes 15 and 17 with a fusion of a large pert of the retinoic acid receptor a gene (RARa) on chromosome 17 to a part of the promyelocytic leukaemia (PML) gene on chromosome 15.More than 75% of patients (under 65 years of age) can be cured, with the application of a combination of anthracyclines and all-trans retinoic acid (ATRA), followed by maintenance therapy. AIM: of the study was to assess of the therapeutic management of APL 93 protocol in acute promyelocytic leukemia. METHODS: We present here the results of a retrospective study concerning 34 patients with APL included between 1998 and 2004 in the APL 93 protocol : 20 in group B and 14 in group C. CR was 82 %. RESULTS: Failure is only due to toxic death (18%) Event free survival at 4 years is 63,47% with relapse rate at 14.25%. Overall survival at 4 years is 69,72%. Our results are acceptable and can be improved with reduction of failure due to toxic death, probably with omission of cytarabine from induction and consolidation adapted by the Spanish PETHEMA Group.     

Retroiliac ureter imaging. A case report

The retroiliac ureter is a rare congenital anomaly with no specific clinical and biologic signs. The authors reports a case of right retroiliac ureter diagnosed in a 41-year-old male with an isolated renal colic. Diagnosis was confirmed by abdominal CT-scan. The different investigations did not show other associated congenital abnomaly. Therapeutic decision was to establish a clinical, biologic and sonographic surveillance.     

Is treatment adherence associated with better quality of life in children with sickle cell disease?

The association of treatment adherence with quality of life (QOL) and the role of sickle cell disease complications were explored in children with sickle cell disease. Primary caregivers of 43 children, ages 5 years and older, and 21 children, ages 8 years and older, completed a standardized measure of QOL during an admission for pain or fever to the hematology acute care unit. Adherence was measured through medical staff ratings, caregiver-report of sickle cell disease-related care activities, and matching of medical staff standard recommendations for treatment of pain and fever with sickle cell disease-related care activities. Sickle cell disease complications were assessed via medical file review. Pearson correlation coefficients indicated that better adherence was associated with poorer overall QOL. In follow-up analyses, although sickle cell disease complications were associated with adherence, it did not explain the negative correlation of adherence with QOL. Higher treatment adherence may interfere with activities that contribute to QOL for some children. Further research to investigate the role of sickle cell disease complications, as well as psychosocial factors, in determining both treatment adherence and QOL is suggested.     

Postoperative cognitive dysfunction after cardiac surgery

Prolonged postoperative cognitive dysfunction (POCD) is reported to occur frequently after cardiac surgery. However, it is rarely assessed in routine clinical practice and receives little attention. Although the cerebral consequences of cardiopulmonary bypass have been measured clinically, insights into the resulting molecular and pathologic events within the brain have only begun to be investigated. POCD is likely to impair quality of life and constitutes a large burden on society when elderly patients prematurely lose their independence. Numerous studies have reported that neurocognitive deficit is associated with heightened mortality, increased length of hospital stay, and discharge to a nursing home. This is linked with a tremendous demand for health-care resources. Because of the magnitude of the clinical problem, serious consideration must be directed toward understanding its etiology and the development of neuroprotective strategies. Clearly identifying the mechanisms of POCD is challenging. The purpose of this review is to discuss recent developments in our understanding of the pathophysiologic mechanisms, prevention, and treatments that have been designed to ameliorate brain dysfunction after cardiac surgery.     

Clinical review: Interpretation of arterial pressure wave in shock states

In critically ill patients monitored with an arterial catheter, the arterial pressure signal provides two types of information that may help the clinician to interpret haemodynamic status better: the mean values of systolic, diastolic, mean and pulse pressures; and the magnitude of the respiratory variation in arterial pressure in patients undergoing mechanical ventilation. In this review we briefly discuss the physiological mechanisms responsible for arterial pressure generation, with special focus on resistance, compliance and pulse wave amplification phenomena. We also emphasize the utility of taking into consideration the overall arterial pressure set (systolic, diastolic, mean and pulse pressures) in order to define haemodynamic status better. Finally, we review recent studies showing that quantification of respiratory variation in pulse and systolic arterial pressures can allow one to identify the mechanically ventilated patients who may benefit from volume resuscitation.     

Neurohormonal activation in severe scorpion envenomation: correlation with hemodynamics and circulating toxin

We studied the effects of scorpion (Androctonus australis hector) venom on hemodynamics and on the release of catecholamines, neuropeptide Y (NPY), endothelin-1 (ET-1) and atrial natriuretic peptide (ANP) in dog model of severe scorpion envenomation. Nine mongrel anesthetized dogs were submitted to mechanical ventilation through intubation and were administered intravenously purified dried scorpion venom (Androctonus autstralis) 0.05 mg/kg. Measurements including pulmonary artery catheter derived parameters, serum toxin levels and humoral variables were performed at baseline (before venom injection) and 5, 15, 30 and 60 min after venom injection. Humoral variables included: serum lactate, epinephrine (EP), norepinephrine (NE), NPY, ET-1 and ANP plasma concentrations. Scorpion venom caused rapid and transient increase of mean arterial pressure (MAP) and PAOP associated with a marked and sustained decline in cardiac output (-55% at 60 min; P < 0.001). Hemodynamic changes were associated with a rapid and significant increase of all measured hormones. The highest increase was for NE (28-fold) and EP (25-fold). MAP was closely correlated with NE and less significantly correlated with toxin levels. Similarly, significant correlation was observed between PAPO and ANP plasma levels. These findings support the implication of excessive catecholamines release in hemodynamic disturbances of severe SE and suggest that NPY and ET-1 could be involved in this process. Serum toxin does not appear to consistently contribute to these effects. Through its correlation with PAOP, ANP could be a reliable and useful marker of cardiac dysfunction in SE.     

Chronic pancreatitis in the ventral pancreas in pancreas divisum

Pancreas divisum, partial or non fusion of the dorsal and ventral pancreatic ductal system, affects up to 10 percent of the population. Approximately 25 percent of patients with pancreas divisum will develop complications such as recurrent pancreatitis. Surgical and endoscopic therapy usually include minor papilla sphincterotomy or sphincteroplasty to facilitate drainage of the dorsal ductal system. The following case represents an unusual complication of the pancreas divisum with primarily ventral duct disease and pancreaticolithiasis.     

Role of MRCP in assessment of biliary variants in living donor liver transplantation

Introduction

Liver transplantation can be done by three different methods: Cadaveric, heterotopic and living donor liver transplantation (LDLT). In LDLT usually the right lobe of a donor liver is transplanted into the patient after proper volumetric studies. The native patient bile ducts are connected to the biliary tree of the transplanted liver segment.

Patients and methods

From January 2010 to August 2011, 50 potential LDLT donors were evaluated with preoperative MRCP.

Results

The radiologist evaluated the visualization of the common duct, right and left intra-hepatic ducts, and insertion of the right posterior lobe duct. The data were classified according to Huang and Hakki classifications. According to the more detailed classification of Hakki 13 patients (26%) were Type K1; 15 patients (30%) were Type K2a; three patients (6%) were Type K2b; 11 patients (22%) Type K3a; four patients (8%) were Type K3b; two patients (4%) were Type K4 and no patients were Type K5. Two patients with unclassified anatomical biliary pattern were encountered. In conclusion, this study has shown an extremely high accuracy of MRCP in preoperative assessment of live hepatic donors, which aided in the style and procedure of the operation and ultimately to a high success rate of the transplantation procedures.