This study was designed to analyze the spectrum of central nervous system (CNS) disease at diagnosis, traumatic lumbar puncture (TLP), role of cranial irradiation, prognostic parameters, and survival outcome in patients with CNS involvement amongst 747 patients with acute lymphoblastic leukemia managed at our center. Twenty-five and six patients had CNS disease and TLP, respectively. Patients with CNS involvement had significantly higher mean presenting leukocyte count (p = 0.021) and incidence of hyperleukocytosis (p = 0.01) compared to those without it. The outcome was poor with three patients in continuous complete-remission, nine relapsers, eight deaths, and eight therapy defaulters. Three patients did not opt for therapy. CNS involvement was significantly associated with inferior survival by log-rank (p = 0.03) analysis but not by Cox-multivariate (p = 0.145) analysis. CNS involvement is a high-risk indicator. Poor outcome in our cohort indicates the need for the revaluation of our treatment protocols with the inclusion of risk-stratified systemic therapy, categorization of CNS involvement into CNS1/CNS2/CNS3, and appropriate use of intrathecal therapy.

The development of microbubbles has had considerable impact on the field of diagnostic ultrasound. These minute, gas- or lipid-filled spheres have enabled imaging in the kidney, liver, heart, and myocardium with resolutions that were previously unachievable. Insonation of these microagents generates high-energy cavitational oscillations, which, in addition to providing contrast, can increase local drug diffusivity through microstreaming or bubble collapse. The ability of focused insonation to induce such collapse on ligated microbubbles offers opportunities to deliver targeted therapies in novel ways. Microbubbles have had considerable impact on cancer research in terms of both imagining tumors and through the delivery of therapeutic agents and on the delivery of substances across biological obstructions such as the blood-brain barrier. This review offers a discussion of current approaches used in microbubble construction, of the underlying physics involved in their creation and destruction, and of current applications for these particles, the latter demonstrating their importance for high-contrast medical ultrasound. Challenges to overcome and future areas of microbubble application are also discussed.

BACKGROUND: There is paucity of data on long-term probability of remission in chronic idiopathic thrombocytopenic purpura (ITP). Aim was to study the course and factors influencing remission of chronic ITP. Chronic ITP was defined as thrombocytopenia persisting >6 months following initial diagnosis. PROCEDURES: Case-records of children with chronic ITP, aged <14 years, were reviewed in this retrospective study (1987-2006). RESULTS: Two hundred seventy children were followed. Median age at diagnosis was 6 years. Median duration of follow up was 30 months (range 6-166). Isolated thrombocytopenia (even if <10 x 10(9)/L) in the absence of "significant" bleeds, by itself was not considered an indication for drug therapy. Sixty-seven (24.8%) children attained complete remission (CR) over a median period of 18 months (range 7-120). The probabilities of remission at 5 years for males and females were 24% and 39.6%, respectively (P = 0.01). The probability of achieving remission at 10 years in children <8 and >/=8 years was 51.2% and 34%, respectively (P = 0.02). The probability of remission at 5 years for children who received some treatment, versus no treatment was 31.4% and 27%, respectively (P = 0.8). Nine of 18 children, who underwent splenectomy, achieved CR. Intracranial hemorrhage (ICH) occurred in 11 (4%) cases. The time of occurrence of ICH from onset of symptoms varied from 6 to 55 months. CONCLUSIONS: The predicted spontaneous remission rate with chronic ITP was 30% and 44% at 5 and 10 years, respectively. Platelet count at diagnosis and the treatment administered did not influence remission outcomes. Age <8 years and female gender were predictors of a favorable outcome. Pediatr Blood Cancer.(c) 2009 Wiley-Liss, Inc.

Hyponatraemia is common, affecting about one in five of all hospitalized patients. Minor degrees of chronic hyponatraemia cause cognitive and motor impairment, and severe hyponatraemia is associated with substantial morbidity and mortality. Syndrome of inappropriate antidiuretic hormone secretion (SIADH) is a common cause of hyponatraemia and is often poorly understood and inappropriately treated. Clinical evaluation and simple biochemical assessment should guide management. The introduction of vasopressin antagonists, or vaptans, into clinical practice heralds the beginning of a new and exciting era for this important group of disorders.

INTRODUCTION: The frequency of ovarian cysts in pregnancy is reported to be 1 in 1000 pregnancies. Laparoscopic ovarian cystectomy has been described in the literature as case series but this is the first case where an ovarian cyst at 20 weeks' gestation impacted in the pouch of Douglas was managed exclusively by laparoscopy. CASE PRESENTATION: A 31-year-old primigravida woman was diagnosed as having an 11 cm ovarian cyst at 20 weeks' gestation. At the 19th week routine ultrasound scan, the mass appeared to be cystic with no solid component. She was asymptomatic. In view of the size of the cyst, options were discussed with her including laparoscopic ovarian cystectomy which she agreed to. Laparoscopic surgery during pregnancy is reported to be safe and beneficial for pregnant women. At laparoscopy, a transvaginal scan was performed to localize the cyst and an ovarian cystectomy was carried out. The patient had an uneventful recovery and subsequent antenatal period. She had a lower segment caesarean section for non-progress of labour when both ovaries were found to be normal and mobile. CONCLUSIONS: Laparoscopic surgery during pregnancy has numerous advantages compared to open laparotomy. This is a rare example of an ovarian cyst in the pouch of Douglas impacted behind the uterus which was managed by laparoscopy and shows the safety of the technique in the presence of an expert laparoscopic surgeon.

Acute lymphoblastic leukemia (ALL) often presents with osteoarthritic manifestations which may lead to misdiagnosis with juvenile rheumatoid arthritis (JRA). This study was designed to identify ALL patients with initial diagnosis of JRA, compare their clinicolaboratory characteristics and outcome with other ALL patients treated at our center. Case records of 762 patients with ALL were analyzed. Information regarding the clinical-demographic profile, therapy and outcome were recorded. Of the children, 49 (6.4%) had initial presentation mimicking JRA. Asymmetric oligoarthritis was the most common pattern of joint involvement. Majority presented with fever, pallor, arthritis, night pain, and bone pain. None of the routine prognostic factors including age, gender, lymphadenopathy, hepatosplenomegaly, total leukocytes count (TLC), and platelet count were significantly associated with relapse/death. The mean symptom-presentation interval (SPI), hemoglobin was significantly higher whilst the TLC was significantly lower in these patients compared to other ALL patients. The 5 year overall-survival was better than other patients with ALL (p = 0.06, by logrank test). Significantly longer SPI in these patients underscores the need for prompt and early investigations to rule out ALL in patients of JRA with atypical features and pointers of ALL. Children with ALL-mimicking JRA may belong to a subgroup of ALL with a better prognosis.

The study was designed to determine the pattern of relapsed disease and identify problem areas in management. Relapse occurred in 111 (23.9%) of the boys and 16 (13.0%) of the girls. The majority relapsed while on chemotherapy. Isolated relapse in the marrow and in the CNS was seen in 51 (40.8%) and 24 (18.9%) patients, respectively. Isolated testicular relapse was seen in 17 (15.3%) of the 111 boys who suffered a relapse. Age and TLC at initial presentation and gender in relapsers and nonrelapsers were compared. Multivariate regression analysis showed that gender (p =.03) and TLC (p =.001) were significant predictors of relapse. Relapse of disease while on chemotherapy and high incidence of CNS and testicular relapse indicate the need for reappraisal of treatment protocols.

Deferiprone (L1) has been used in several countries for iron chelation therapy for over one decade. Long-term results on the drug are lacking. In the present study, data of 110 patients on deferiprone (L1) for up to 17 years were analyzed. On a mean L1 dose of 70.2 mg/kg/day (range 44-100), serum ferritin level showed a very steady decrease with time from an initial mean (+/-SD) of 3,033.61 +/- 1,468.04 ng/ml to final of 1,665.08 +/- 949.93 ng/ml after a mean (+/-SD) of 6.1 +/- 3.8 years. In total, 13 patients discontinued L1 therapy. Major complications of L1 requiring permanent discontinuation of treatment included arthropathy (n = 8, 7.2%) and neutropenia/agranulocytosis (n = 5, 4.5%). Lesser complications permitting continued L1 treatment included transient mild leucopenia or thrombocytopenia (n = 3) and gastrointestinal problems (n = 5). There were a total of three deaths attributed to agranulocytosis. Although the complications associated with L1 treatment are significant and require close monitoring, they do not preclude effective long-term therapy in the vast majority of patients. A longer duration of therapy is required for effective response in chronically iron-overloaded patients. Further well-controlled prospective studies of L1 are required to identify factors affecting individual response to therapy.