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Biomedical subjects

S Y Ha

Publications and source records attributed to S Y Ha.

At least 19 recordsLinked to original sources
PubMed2006

Successful treatment of spinal cord compression secondary to extramedullary hematopoietic mass by hypertransfusion in a patient with thalassemia major.

A 15-year-old girl with thalassemia major who suffered from paraparesis with a history of progressive lower limb weakness for 2 years immigrated from mainland China to Hong Kong. She had not received regular blood transfusion since the age of 6 years after splenectomy. MRI of the spine showed thoracic spinal cord compression secondary to extramedullary hematopoietic mass. She made significant recovery with hypertransfusion therapy alone. MRI of the spine repeated 3 months later showed nearly complete resolution of the extramedullary hematopoietic mass.

Adolescent↗
PubMed2006

Carotid intima-media thickness is increased and related to arterial stiffening in patients with beta-thalassaemia major.

An increased iron store has been linked to the risk of atherosclerosis. The carotid intima-media thickness (IMT), stiffness index, Young's elastic modulus (YEM), and augmentation index were determined in 20 patients with beta-thalassaemia major. Compared with controls, patients had greater IMT (P < 0.001), YEM (P = 0.005), and stiffness (P = 0.009) and augmentation (P = 0.03) indices. The odds ratio for increased IMT in patients was 39. Carotid IMT correlated with YEM (P = 0.036), stiffness index (P = 0.036) and augmentation index (P = 0.006). Multivariate analysis identified patient status (P < 0.001) as the only significant determinant of IMT. These findings suggest the possibility of premature atherosclerosis in beta-thalassaemia major patients.

Adult↗
PubMed2006

Improved outcome of acute lymphoblastic leukaemia treated by delayed intensification in Hong Kong children: HKALL97 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 HKALL97 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.

Adolescent↗
PubMed2005

Hyperbilirubinemia and cholelithiasis in Chinese patients with hemoglobin H disease.

Hemoglobin H disease (HbH) is a hemoglobinopathy peculiar to parts of the world with high incidence alpha-thalassemia mutations. Among 90 HbH cases, 50 cases suffered from clinically significant jaundice (bilirubin >30 mmol/l), including 14 with severe jaundice (bilirubin >60 mmol/l). Cholelithiasis was found in 38 cases. The incidence is roughly eight times higher than that in background control population but 50% lower than that in beta-thalassemia. The risk of gallstones was related to higher bilirubin levels but not alpha-globin genotype, sex, ferritin, and hemoglobin levels. Homozygotes or double heterozygotes for Gilbert alleles (17.2%), but not heterozgyotes (42.2%), were found to have a significantly increased risk of gallstones and jaundice. However, common Chinese Gilbert syndrome alleles do not completely explain the variable risks.

Alleles↗
PubMed2005

Use of midazolam and ketamine as sedation for children undergoing minor operative procedures.

OBJECTIVES: We used intravenous midazolam and ketamine for children undergoing minor operative procedures with satisfactory results. We aimed to further evaluate its efficacy and adverse effects in pediatric ward setting. METHODS: This was a prospective study of all children undergoing minor operations with sedation in our pediatric general and oncology wards from July 1998 to June 1999. The procedures included lumber puncture+/-intrathecal chemotherapy, bone marrow aspiration+/-trephine biopsy, central venous catheter removal, skin biopsy, or their combination. All sedation procedures were started with midazolam 0.1 mg/kg and ketamine 1 mg/kg; they were increased gradually to 0.4 and 4 mg/kg, respectively, if necessary. Heart rate and SaO2 were continuously monitored. RESULTS: Altogether, 369 minor operations were performed in 112 patients (male:female=2:1, median age 6 years, range 5 months-17 years). All achieved adequate sedation, with 96% within 30 s and 75% required just the starting dose. Younger children required a higher dosage (p=0.003 for midazolam, p<0.001 for ketamine). The median recovery time was 87 min, with no association with age, sex, or dosage of sedation, but was longer in patients having hallucination (p=0.001). Adverse effects included tachycardia (27.9%), increased secretion (17.6%), agitation (13.6%), nausea and vomiting (9.2%), hallucination (8.7%), desaturation (8.4%), and cataleptic reaction (0.8%). All desaturation episodes were transient and responded to oxygen supplement alone. None developed bronchospasm or convulsion. Some adverse effects were dose-related. Half of the children who received 0.3 mg/kg midazolam developed desaturation. CONCLUSIONS: Intravenous midazolam-ketamine can provide rapid, effective, and safe sedation for children undergoing minor operations in ward setting. Adverse effects are mild. Midazolam above 0.3 mg/kg should be used with caution.

Adolescent↗
PubMed2005

Ventriculo-vascular interactions in patients with beta thalassaemia major.

OBJECTIVES: To determine potential interactions between the heart and arterial system in patients with beta thalassaemia major. DESIGN AND PATIENTS: Vascular compliance, systemic vascular resistance, and left ventricular (LV) contractility was determined in 34 asymptomatic thalassaemia patients at 2-4 hours after blood transfusion and also in 34 age and sex matched controls using a non-invasive device. The results were compared between groups and inter-relationships between LV contractility and indices of vascular load were explored. SETTING: Tertiary paediatric cardiac centre. RESULTS: When compared with controls, patients had greater systemic vascular resistance (1633 (259) v 1377 (276) dynes/s/cm5, p < 0.001) and effective arterial elastance (E(a)) (1.86 (0.25) v 1.65 (0.29) mm Hg/ml, p = 0.001), an index of combined pulsatile and static vascular load. On the other hand, their systolic blood pressure (104 (9) v 112 (13) mm Hg, p = 0.006), pulse pressure (45 (9) v 57 (10) mm Hg, p < 0.001), adjusted systemic vascular compliance (1.21 (0.09) v 1.37 (0.14), p < 0.001), adjusted brachial artery distensibility (21 (0.29) v 7.95 (0.29)%/mm Hg, p < 0.001) and LV+dP/dt (1059 (183) v 1239 (237) mm Hg/s, p = 0.001) were significantly lower. Significant determinants of LV contractility, as reflected by LV+dP/dt, were age (standardised beta = -0.24, p = 0.003), body mass index (standardised beta = -0.34, p = 0.004), systolic blood pressure (standardised beta = 0.90, p < 0.001), and effective E(a) (standardised beta = -0.50, p < 0.001) (model R2 = 0.69). No significant correlation existed between serum ferritin concentration and any of the cardiac or vascular indices. CONCLUSION: An unfavourable ventriculo-vascular interaction, as characterised by impaired cardiac contractility and increased static and pulsatile vascular load, occurs in patients with beta thalassaemia major.

Arteries↗
PubMed2004

Bone marrow transplantation for therapy-related acute myeloid leukemia in congenital retinoblastoma associated with 13q deletion syndrome.

Children with constitutional deletion of the long arm of chromosome 13 are at risk for retinoblastoma (RB) due to loss of the RB tumor suppressor gene. The prognosis is poor since the tumors are often bilateral, aggressive, and recurrent and the patients often harbor other congenital abnormalities. One further complication is that of therapy-related malignancies later in life. We report a case of allogeneic stem cell transplantation for therapy-related acute myeloid leukemia in an 8-year-old girl after multimodality treatment for refractory bilateral relapsing RB, with excellent outcome in both the ophthalmic and marrow disease.

Acute Disease↗
PubMed2004

Comparison of subcutaneous infusion needles for transfusion-dependent thalassemia patients by the intrapersonal cross-over assessment model.

Needle-induced trauma is one of the major contributing factors for poor compliance in patients with thalassaemia major on iron chelation therapy. A new generation of needles is currently available on the market, but their theoretical advantages have not been tested clinically. We performed a study to compare the pros and cons of the representative prototypes from each of the new (Thalaset needle) and old (butterfly scalp vein needle) generations of needles. Patients with thalassemia major who had been receiving subcutaneous iron chelation therapy for at least 2 years were recruited. Patients using butterfly needles were instructed to switch to the newer form of needle (Thalaset) for 2.5 months and then to change back to butterfly needles for another 2.5 months. Comparison was done by the intrapersonal cross-over model using three identical sets of questionnaires collected at the beginning of the study and after the use of Thalaset and butterfly needles, respectively. Fifty-four (22 females; 32 males) patients were included in the statistical analysis. The median age was 24.1 years (range = 7.6-47.2 years). Local reactions such as pain, itchiness, tenderness, and swelling were significantly different among the three evaluation periods and were all in favor of the Thalaset needle (all with P < 0.001), even after adjusting for the intention-to-treat calculation. The Thalaset needle is significantly better than the butterfly needle in reducing needle-related trauma. It induced fewer local skin reactions such as pain, itchiness, tenderness, and swelling. However, recommendations for its routine clinical use require further cost-effectiveness analysis.

Adolescent↗
PubMed2004

Magnetic resonance screening of iron status in transfusion-dependent beta-thalassaemia patients.

The clinical utility of dual sequence (T1- and T2-weighted) magnetic resonance (MR) imaging in estimating liver iron concentration (LIC) in 32 transfusion-dependent beta-thalassaemia major (24 females; age 18.5+/-5.9 years) patients on desferrioxamine was evaluated. Signal intensity ratios (SIR) between liver, spleen and pancreas to psoas muscle were determined on both sequences. Relationships between clinical and MR parameters, and accuracy of SIR thresholds in determining adequacy of chelation from LIC were analysed. Liver T1- and T2-SIR were related to LIC (P < 0.001). T1-SIR < 0.60 predicted severe iron overload (LIC > 15 mg/g) with sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) of 100%, 87%, 33% and 100% respectively. T2-SIR < 0.1 yielded 100% sensitivity, 93% specificity, 50% PPV and 100% NPV. T1-SIR > or = 1.1 predicted LIC < 7 mg/g with 69% sensitivity, 88% specificity, 85% PPV and 74% NPV. T2-SIR > or = 0.20 yielded 56.5% sensitivity, 94% specificity, 90% PPV and 71% NPV. LIC correlated with liver T1-SIR, liver T2-SIR and serum ferritin (r = -0.76, -0.65, 0.47, respectively; P < 0.01). Serum ferritin was inversely related to liver T1-SIR, liver T2-SIR and spleen T2-SIR (r = -0.35, -0.43, -0.40, respectively; P < 0.05). Mean total transfusion burden was not related to any MR parameter. Although neither MR sequence was a highly accurate predictor of LIC, SIR thresholds are useful to determine presence of iron overload and adequacy of chelation treatment.

Adolescent↗
PubMed2004

Chronic benign neutropenia among Chinese children.

OBJECTIVE: To delineate the clinical behaviour of chronic benign neutropenia in Chinese children in Hong Kong. DESIGN: Retrospective study. SETTING: University teaching hospital, Hong Kong. PATIENTS: All infants and children with absolute neutrophil count of 1.5 x 10(9) /L or lower for more than 3 months. MAIN OUTCOME MEASURES: Development of significant infection, and achievement of remission. RESULTS: Twenty-four children with chronic benign neutropenia were identified between 1992 and 2001. Their median age of diagnosis was 9 months. The mean (standard deviation) initial absolute neutrophil count was 0.28 x 10(9) /L (0.24 x 10(9) /L). Twenty-three patients presented with infection. Of the 19 patients tested, four (21%) were positive for anti-neutrophil antibodies. Bone marrow examination was performed in 17 patients: nine had normal results, but six showed evidence of peripheral consumption, one showed late maturation arrest at band stage, and one showed phagocytosis of myeloid cells by histiocytes. The overall hospitalised infection rate was 51.6 episodes per 1000 patient-months. Ten percent of cases were considered 'significant' infections and required hospital admission with either surgical intervention or intravenous therapy (antibiotics or fluid replacement). In the first year of diagnosis, more than 80% of patients had their lowest absolute neutrophil count (mean, 0.16 x 10(9) /L; standard deviation, 0.11 x 10(9) /L). Granulocyte-colony stimulating factor was used to treat three patients and induced transient elevation of absolute neutrophil count in all three. The projected remission rate was 55.4% at 3 years. Even for those with persistent disease, there was significant recovery in absolute neutrophil count to a mean of 0.5 x 10(9) /L (P<0.01). CONCLUSIONS: Patients with chronic benign neutropenia experienced a relatively benign clinical course regardless of their remission status. Only a small proportion of patients developed significant infections. A multi-centre prospective study may help identify predictive factors of remission.

Autoimmune Diseases↗
PubMed2003

Klebsiella infection in patients with thalassemia.

Klebsiella infection has previously been reported in a few patients with transfusion-dependent thalassemia. The incidence and clinical spectrum of this infection in our cohort of patients were reviewed retrospectively. Among 160 patients observed for 12 years, there were 15 episodes of Klebsiella infection that occurred in 12 patients (7.5%), resulting in an incidence of 0.78 infections per 100 patient-years. The clinical spectrum included sinusitis (4 cases), intracranial infection (5 cases), septicemia (4 cases), and abscesses of the liver, lung, kidney, and parotid gland (1 case each). Three patients had recurrent infections involving different sites, 2 (16%) died of fulminant septicemia, and 3 (25%) had significant permanent neurological deficits. The antibiotic susceptibility pattern for the isolates was similar to the pattern for isolates recovered in the community. With regard to predisposing factors, iron overload and liver function derangement were found to be significant on univariate analysis (P=.046 and P=.049, respectively) but insignificant on multivariate analysis. Klebsiella infection was a serious and frequently encountered complication in our patients with transfusion-dependent thalassemia, resulting in high mortality and morbidity rates.

Adolescent↗
PubMed2003

Treatment of acute lymphoblastic leukemia in Hong Kong children: HKALL 93 study.

A population-based multicentre study for childhood acute lymphoblastic leukemia (ALL) was conducted in Hong Kong from 1993 to 1997. One hundred and forty-five newly diagnosed ALL patients were treated by the HKALL 93 protocol. Patients were stratified into three risk groups according to age, presenting white cell count, immunophenotyping and cytogenetic study. The patients received the same induction and early and late intensification at week 5 and week 20. Fifty-eight standard risk (SR) patients received regular intrathecal methotrexate as CNS preventive therapy, while 49 intermediate risk (IR) patients received high dose intravenous methotrexate and regular intrathecal methotrexate. Thirty-eight high risk (HR) patients were treated with prophylactic cranial irradiation and an additional intensification block at week 35. The induction remission rate was 97.2% with 2% induction death. Two patients died during first complete remission. Relapse occurred in 20.7, 42.9 and 42.1% of SR, IR and HR patients respectively. By multivariate logistic regression, age> or =10 years and white cell count> or =100 x 10(9)/l were the two significant variables accounting for mortality. The 5-year overall and event-free survival of the whole group was 81.3 and 62.6% respectively. According to risk groups, the event-free survival was 79, 49 and 61% for SR, IR and HR patients respectively, while the overall survival was 96, 73 and 68% for SR, IR and HR patients respectively. In conclusion, the treatment protocol had low treatment-related mortality but was associated with a rather high relapse rate, especially in IR patients. Salvage therapy achieved sustained second remission in some patients. More intensive treatment especially a late intensification is required to improve the outcome.

Adolescent↗
PubMed2002

Arterial stiffness and endothelial function in patients with beta-thalassemia major.

BACKGROUND: Increased iron store has been linked to risk of cardiovascular disease. Structural alterations of arteries in beta-thalassemia major patients and in vitro functional disturbance of vascular endothelial cells by thalassemic serum have been described. We sought to determine whether arterial stiffness and endothelial function are altered in vivo. METHODS AND RESULTS: Thirty thalassemia patients (16 male) aged 22.2+/-7.4 years were recruited. Left ventricular (LV) mass and function were assessed echocardiographically. Carotid and brachioradial artery stiffness was assessed by stiffness index and pulse-wave velocity (PWV), respectively. Brachial artery endothelial function was assessed by vascular response to reactive hyperemia (flow-mediated dilation [FMD]) and sublingual glyceryl trinitrate. These indexes were compared with those of 30 age- and sex-matched controls. None of the patients had LV systolic or diastolic dysfunction. When compared with controls, patients had greater absolute (113.8+/-38.0 versus 109.0+/- 32.6 g, P=0.04) and indexed (82.4+/-17.5 versus 66.7+/-12.7 g/m(2), P<0.001) LV mass, carotid artery stiffness index (8.1+/-3.5 versus 5.5+/-1.6, P<0.001), and brachioradial PWV (8.9+/-2.4 versus 7.9+/-1.7 m/s, P= 0.03). Their FMD was impaired (3.5+/-3.3% versus 8.8+/-3.9%, P<0.001), whereas glyceryl trinitrate- mediated dilation was preserved (17.9+/-7.6% versus 16.3+/-6.1%, P=0.40). Both stiffness index and PWV correlated inversely with magnitude of FMD (r=-0.40, P=0.03; r=-0.41, P=0.03) and positively with indexed LV mass (r=0.50, P=0.005; r=0.40, P=0.027). Nonetheless, no significant correlation existed between ferritin level and carotid stiffness, PWV, or FMD. CONCLUSIONS: Increased arterial stiffness, endothelial dysfunction, and LV hypertrophy occur in patients with beta-thalassemia major, which may result in reduction of mechanical efficiency of the heart.

Adult↗
PubMed2002

Paediatric hepatoblastoma and hepatocellular carcinoma: retrospective study.

OBJECTIVES: To compare and contrast clinical characteristics and outcomes of hepatoblastoma or hepatocellular carcinoma in paediatric patients. DESIGN: Retrospective study. SETTING: University teaching hospital, Hong Kong. PATIENTS AND METHODS: Medical records of 22 paediatric patients with hepatoblastoma (n=11) or hepatocellular carcinoma (n=11) admitted to Queen Mary Hospital between 1989 and 2000 were reviewed. Data gathered included demographic data, results of liver function tests, hepatitis A, B, and C titres, and alpha-foetoprotein levels, and imaging studies including chest X-ray, ultrasound study, computed tomography scan, and magnetic resonance imaging/hepatic angiogram for tumour staging and resectability. RESULTS: The mean age of patients with hepatoblastoma was 18 months (range, 5 months to 3 years), while that of patients with hepatocellular carcinoma was 10.2 years (range, 2 to 16 years). Females predominated in the hepatoblastoma group (female:male, 8:3) and males in the hepatocellular carcinoma group (male:female, 10:1). None of the patients with hepatoblastoma were hepatitis B surface antigen positive, in contrast to 64% of the hepatocellular carcinoma group. Only 45% of the hepatocellular carcinomas were resectable at presentation and this figure remained unchanged following chemotherapy. A total of 91% of hepatoblastomas were resectable, four at presentation, and a further six after chemotherapy. Tumour rupture was more common in patients with hepatoblastoma than in those with hepatocellular carcinoma (36% versus 9% of cases, respectively). Mortality rates were considerably higher among the hepatocellular carcinoma group than the hepatoblastoma group in this series. CONCLUSION: Childhood hepatoblastoma and hepatocellular carcinoma differ with respect to age and tumour stage at presentation, hepatatis B surface antigen status, tendency to rupture, chemosensitivity, and prognosis.

Carcinoma, Hepatocellular↗