Childhood acute lymphoblastic leukaemia masquerading as primary skeletal problem

Acute lymphobiastic leukaemia (ALL) usually presents with signs of bone marrow failure, Occasionally ALL in children may present with non-specific or misleading clinical features. In a retrospect analysis of 25 consecutive cases of childhood ALL, we identified three children who were initially managed and discharged as acute osteomyelitis, juvenile chronic arthritis and postinfectious arthralgia respectively. They had mild normochromic, normocytic anaemia and two had transient leucopenia. Their platelet counts were normal and none had circulating blasts. The severity of their skeletal symptoms was out of proportion to the clinical and radiological finding. They failed to respond to the initial management and diagnosis was delayed for an average of 42 days and eventually confirmed on the marrow aspirate. Complete remission was attained in all cases with standard chemotherapy and had been maintained for an average of 29 months. ALL should be considered as a differential diagnosis in children complaining of multiple or persistent joint pains, especially in the presence of atypical clinical features or poor response to treatment.published_or_final_versio

Reversible posterior leukoencephalopathy syndrome in Chinese children induced by chemotherapy: A review of five cases

This is a retrospective review of the clinico-radiological features and neurological outcomes of reversible posterior leukoencephalopathy syndrome episodes in Chinese cancer children receiving chemotherapy in a regional hospital in Hong Kong from 1998 to 2008. Five children (3 males and 2 females) with a mean age of 7 years were identified, four of whom had acute lymphoblastic leukaemia and one had a central nervous system germ cell tumour. Presenting symptoms included seizures (100%), altered mental function (100%), headache (40%), and visual disturbance (60%). The mean systolic blood pressure at presentation was 158 mm Hg. Approximately 80% had typical radiological features of reversible posterior leukoencephalopathy syndrome. All showed complete recovery after the acute stage, but one subsequently developed epilepsy. Two patients ultimately died of refractory malignant disease. Two others were followed up for a mean of 6 years, and remained neurologically normal. This report was the first case review documenting reversible posterior leukoencephalopathy syndrome in Chinese cancer children. The clinico-radiological features and neurological outcomes were similar to those reported in western series. Early recognition of the syndrome is important to facilitate appropriate treatment. The central nervous system damage may not be reversible and thus long-term follow-up is warranted.published_or_final_versio

Age as a factor in severe bacterial infection in transfusion-dependent patients with thalassemia major [2] (multiple letters)

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Improved outcome of acute lymphoblastic leukaemia treated by delayed intensification in Hong Kong children: HKALL 97 study

Objective. To study the outcome of children with acute lymphoblastic leukaemia who were treated using a protocol including one or two delayed intensifications. Design. Prospective single-arm multicentre study. Setting. Five designated children cancer units of the Hospital Authority of Hong Kong. Patients. Children aged between 1 and 17.9 years with newly diagnosed acute lymphoblastic leukaemia seen from November 1997 to December 2002. Intervention. Chemotherapy was modified from a German Berlin-Frankfurt-Muenster 95 (BFM95) protocol that included a delayed intensification similar to the induction phase repeated 5 months after diagnosis. High-risk patients were given double delayed intensification. Main outcome measures. Overall survival and event-free survival of the whole group and the three risk groups (standard-, intermediate-, and high-risk groups), and comparison with historical controls. Results. A total of 171 patients were recruited with a median age at diagnosis of 5.57 years (range, 1.15-17.85 years). The induction remission rate was 95.3% and non-leukaemia mortality during remission was 2.3%. At 4 years, the relapse rate of this (HKALL97) study was significantly lower than that of the HKALL93 study (15.7 vs 37.3%; P<0.001). The 4-year overall survival of HKALL97 and HKALL93 studies were 86.5% and 81.8%, respectively (P=0.51). The 4-year event-free survival for HKALL 97 and HKALL93 studies were 79% and 65%, respectively (P=0.007). Nonetheless the difference of event-free survival was most remarkable in the intermediate-risk group: 75.6% and 53.1% for HKALL97 and HKALL93 studies, respectively (P=0.06). Conclusion. A more intensive delayed consolidation phase improved the outcome for children with acute lymphoblastic leukaemia by reducing relapses at 4 years. The early treatment complications were manageable and non-leukaemia mortality during remission remained low.published_or_final_versio

Minimal residual disease-based risk stratification in Chinese childhood acute lymphoblastic leukemia by flow cytometry and plasma DNA quantitative polymerase chain reaction

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Internet addiction and its psychosocial risks (depression, anxiety, stress and loneliness) among Iranian adolescents and young adults: a structural equation model in a cross-sectional study

Internet addiction has become an increasingly researched area in many Westernized countries. However, there has been little research in developing countries such as Iran, and when research has been conducted, it has typically utilized small samples. This study investigated the relationship of Internet addiction with stress, depression, anxiety, and loneliness in 1,052 Iranian adolescents and young adults. The participants were randomly selected to complete a battery of psychometrically validated instruments including the Internet Addiction Test, Depression Anxiety Stress Scale, and the Loneliness Scale. Structural equation modeling and Pearson correlation coefficients were used to determine the relationship between Internet addiction and psychological impairments (depression, anxiety, stress and loneliness). Pearson correlation, path analysis, multivariate analysis of variance (MANOVA), and t-tests were used to analyze the data. Results showed that Internet addiction is a predictor of stress, depression, anxiety, and loneliness. Findings further indicated that addictive Internet use is gender sensitive and that the risk of Internet addiction is higher in males than in females. The results showed that male Internet addicts differed significantly from females in terms of depression, anxiety, stress, and loneliness. The implications of these results are discussed

A Genome-Wide Association Study of Diabetic Kidney Disease in Subjects With Type 2 Diabetes

dentification of sequence variants robustly associated with predisposition to diabetic kidney disease (DKD) has the potential to provide insights into the pathophysiological mechanisms responsible. We conducted a genome-wide association study (GWAS) of DKD in type 2 diabetes (T2D) using eight complementary dichotomous and quantitative DKD phenotypes: the principal dichotomous analysis involved 5,717 T2D subjects, 3,345 with DKD. Promising association signals were evaluated in up to 26,827 subjects with T2D (12,710 with DKD). A combined T1D+T2D GWAS was performed using complementary data available for subjects with T1D, which, with replication samples, involved up to 40,340 subjects with diabetes (18,582 with DKD). Analysis of specific DKD phenotypes identified a novel signal near GABRR1 (rs9942471, P = 4.5 x 10(-8)) associated with microalbuminuria in European T2D case subjects. However, no replication of this signal was observed in Asian subjects with T2D or in the equivalent T1D analysis. There was only limited support, in this substantially enlarged analysis, for association at previously reported DKD signals, except for those at UMOD and PRKAG2, both associated with estimated glomerular filtration rate. We conclude that, despite challenges in addressing phenotypic heterogeneity, access to increased sample sizes will continue to provide more robust inference regarding risk variant discovery for DKD.Peer reviewe