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Biomedical subjects

W Y Wong

Publications and source records attributed to W Y Wong.

At least 19 recordsLinked to original sources
PubMed2007

Ethyl acetate extract of Chinese medicinal herb Sarcandra glabra induces growth inhibition on human leukemic HL-60 cells, associated with cell cycle arrest and up-regulation of pro-apoptotic Bax/Bcl-2 ratio.

Sarcandra glabra (Thunb.) Nakai, colloquially known as Caoshanhu, is a Chinese medicinal herb with reported anti-tumor, anti-inflammatory, anti-viral and non-specific immunoenhancing properties. Although the plant has been clinically used for treating a variety of diseases, its bioactive ingredients are largely unknown and its mode of action has never been investigated. In this study, the anti-tumor property of ethyl acetate (EA) extract of S. glabra was investigated by determining its in vitro growth-inhibitory effects on a panel of human cancer cell lines of different histotypes. Growth inhibition of the EA extract on the cancer cells seemed to be selective, and the leukemic HL-60 was found to be the most responsive after 48 h of treatment (IC50=58 microg/ml). Flow cytometric studies further illustrated that the extract might interfere with DNA replication and thus arrested the cell cycle at S phase in the leukemic cells, followed by DNA fragmentation and loss of phospholipid asymmetry in the plasma membrane after 72 h of treatment. Concurrently, the pro-apoptotic Bax/Bcl-2 ratio was also up-regulated by more than 178% of the control level. All these findings suggested that the extract had initiated apoptosis to kill the leukemic cells. Results from this pioneer study help to establish a scientific foundation for future research and development of the bioactive ingredients in EA extract of S. glabra as efficacious anti-cancer agents.

Acetates↗
PubMed2006

A randomised trial of carbetocin versus syntometrine in the management of the third stage of labour.

OBJECTIVE: Syntometrine is an effective uterotonic agent used in preventing primary postpartum haemorrhage but has adverse effects including nausea, vomiting, hypertension and coronary artery spasm. Carbetocin is a newly developed long-acting oxytocin analogue that might be used as an uterotonic agent. We compare the efficacy and safety of intramuscular (IM) carbetocin with IM syntometrine in preventing primary postpartum haemorrhage. DESIGN: Prospective, double-blinded, randomised controlled trial. SETTING: Delivery suite of a university-based obstetrics unit. POPULATION: Women with singleton pregnancy achieving vaginal delivery after and throughout 34 weeks. METHODS: Three hundred and twenty-nine eligible women were randomised to receive either a single dose of 100 microgram IM carbetocin or 1 ml IM syntometrine (a mixture of 5 iu oxytocin and 0.5 mg ergometrine) at the end of second stage of labour. MAIN OUTCOME MEASURES: Difference in haemoglobin drop measured 2 days after delivery between the two groups. RESULTS: There was no difference in the drop of haemoglobin concentration within the first 48 hours between the two groups. The incidence of additional oxytocic injections, postpartum haemorrhage (blood loss > or = 500 ml) and retained placenta were also similar. The use of carbetocin was associated with significant lower incidence of nausea (relative risk [RR] 0.18, 95% confidence interval [CI] 0.04-0.78), vomiting (RR 0.1, 95% CI 0.01-0.74), hypertension 30 minutes (0 versus 8 cases, P < 0.01) and 60 minutes (0 versus 6 cases, P < 0.05) after delivery but a higher incidence of maternal tachycardia (RR 1.68, 95% CI 1.03-3.57). CONCLUSIONS: IM carbetocin is as effective as IM syntometrine in preventing primary postpartum haemorrhage after vaginal delivery. It is less likely to induce hypertension and has a low incidence of adverse effect. It should be considered as a good alternative to conventional uterotonic agents used in managing the third stage of labour.

Adult↗
PubMed2004

Frequency and causes of hospitalization in HIV-negative children and adolescents with haemophilia A or B and its effect on academic achievement.

The aim of the study is to determine the causes and frequency of hospitalization in HIV-negative boys and adolescents with haemophilia and evaluate their impact on academic achievement. One hundred and twenty-six HIV-negative boys and adolescents were followed prospectively from 1989-96, at 14 comprehensive haemophilia treatment centres. One hundred and fifteen participants with haemophilia A or B were included in the investigation. These participants contributed an average of 57.8 months of follow-up. There were 203 hospitalizations in 65 participants and 50 participants were never hospitalized. Haemarthroses and soft tissue bleeds accounted for 46 and 44 causes of hospitalization. Central line infection was the third most common cause. Participants with inhibitor had the majority of central line infections and hospitalizations. Intracranial haemorrhage resulted in five hospitalizations in two participants. Other causes of bleeding accounted for 22% of hospitalizations. The median number of hospitalizations per year was 0.18. Duration of hospital stay was significantly related to lower spelling scores. Acute and chronic joint problems and soft tissue bleeds still account for the majority of hospitalizations. Positive inhibitor status was associated with higher numbers of hospitalizations and central line infections. Academic achievement was affected, to some degree, by length of hospital stay.

Adolescent↗
PubMed2004

Extinction paradox and actual power scattered in light beam scattering: a two-dimensional study.

The extinction paradox is examined by applying partial-wave analysis to a two-dimensional light beam interacting with a long transverse cylinder without absorption, assuming always short wavelengths. We show that the (conventional) power scattered, Psca, except for a very narrow beam hitting a transparent cylinder on axis, is always double the power directly intercepted by the scatterer, Pitc, including a zero result for Psca when the incident beam is basically off the material surface. This contradicts the interpretation that attributes one half of Psca to edge diffraction by the scatterer. Furthermore, we identify the shadow-forming wave (SFW) from the partial-wave sum in the forward direction and show that the actual power scattered or, equivalently, the power depleted from the incident beam is equal to one unit of Pitc for a narrow beam, gets larger for a broader beam, and approaches 2Pitc for a very broad beam. The larger value in the latter cases is due to the extent of divergence of the SFW beam out of the incident beam at distances well beyond the Rayleigh range.

Journal Article↗
PubMed2001

Progressive decreases in nuclear retinoid receptors during skin squamous carcinogenesis.

Retinoids are essential for normal skin growth, differentiation, and apoptosis and are active pharmacologically in the prevention and treatment of skin cancers and other lesions. Retinoid effects are mediated mainly by retinoic acid receptors (RARs) and retinoid X receptors (RXRs), which act as transcription factors to alter gene expression. Using in situ hybridization, we analyzed the expression of RARs and RXRs in normal sun-exposed skin (n = 85), squamous cell carcinoma (SCC; n = 28), and actinic keratosis [AK (a precursor to SCC); n = 38]. The expressions of five receptors (RAR-alpha and -gamma and RXR-alpha, -beta, and -gamma) were moderate to very strong in normal skin, with higher expressions in spinous and granular layers than in the basal layer. RAR-beta expression was weak or absent in normal and lesion samples. All five receptors expressed in the skin were suppressed progressively from normal skin to premalignant skin (AK) to invasive skin SCC. Specific receptor decreases in lesions relative to normal skin ranged from 75% (RXR-beta) to 96% (RAR-alpha) in SCC and from 37% (RAR-gamma) to 68% (RXR-beta) in AK. The degree of suppression of RXR-alpha and RAR-gamma, the two predominant retinoid receptors in skin, was relatively less for RXR-alpha (58% versus 86%; P = 0.015) and relatively greater for RAR-gamma (37% versus 89%; P = 0.0001) between AK and SCC, suggesting that suppression of RXR-alpha may be an earlier event and expression of RAR-gamma may be a later event of multistep squamous skin carcinogenesis. Our results indicate that suppressed expression of retinoid receptors occurs early (in AK) and is associated with progression of squamous skin carcinogenesis to SCC.

Aged↗
PubMed2001

Relation between HIV-1 and hepatitis C viral load in patients with hemophilia.

Coinfection with hepatitis C virus (HCV) and HIV-1 is common in patients with hemophilia and in intravenous drug users. Little, however, is known about the relation between HIV-1 and HCV coinfection and the effects on HCV clearance and pathogenesis. We examined data from 207 HIV-1-infected and 126 HIV-1-uninfected patients with hemophilia enrolled in the multicenter Hemophilia Growth and Development Study. Participants were observed during prospective follow-up for approximately 7 years with annual measurements of alanine aminotransferase (ALT), CD4+ cells, and HCV and HIV-1 RNA levels. Clearance of HCV was more likely to occur in those uninfected with HIV-1 (14.3 versus 2.5%; odds ratio [OR] 4.79; 95% confidence interval [CI], 1.63-14.08, p =.005) and was more common with decreasing age (OR, 1.23; 95% CI, 1.04-1.47; p =.017). HCV RNA levels were higher throughout the 7 years of follow-up in those HIV-1-infected (p <.001). In the HIV-1-infected participants, baseline CD4+ cells were inversely related to HCV RNA with every 100-cell increase associated with a 0.19 log10 copy/ml decrease in HCV RNA (p =.002), and HIV-1 and HCV RNA levels were directly related (p =.008). Increasing HCV RNA levels were also associated with significantly higher ALT levels regardless of HIV-1 infection status. These results demonstrate that HIV-1/HCV co-infection is associated with a reduced likelihood of HCV clearance and that higher levels of HCV RNA are associated with increased hepatic inflammation.

Adolescent↗
PubMed2001

Flow pattern dynamics in convecting liquid helium.

We present experimental data which correlate thermal measurements and flow visualization in convecting liquid 4He. For a small range R(C) R1, generating thermal oscillations as in earlier reports. At higher R values the time dependence becomes aperiodic with the surprising appearance of spiral-defect chaos at an aspect ratio smaller than has previously been reported.

Journal Article↗
PubMed2001

Hepatitis C virus load is associated with human immunodeficiency virus type 1 disease progression in hemophiliacs.

Hepatitis C virus (HCV) and human immunodeficiency virus type 1 (HIV-1) coinfection is common in hemophiliacs and injection drug users. To assess the interaction between HCV load and HIV-1 disease progression, we examined 207 HIV-1/HCV-coinfected patients. Patients were followed prospectively for approximately 7 years, and annual measurements of CD4(+) cell counts and HCV and HIV-1 loads were obtained. Survival analysis was used to define the independent effects of HCV load on HIV-1 progression. After controlling for CD4(+) cell count and HIV-1 RNA level, every 10-fold increase in baseline HCV RNA was associated with a relative risk (RR) for clinical progression to acquired immunodeficiency syndrome (AIDS) of 1.66 (95% confidence interval [CI], 1.10-2.51; P=.016) and an RR for AIDS-related mortality of 1.54 (95% CI, 1.03-2.30; P=.036). These findings emphasize the need for further research regarding the use of HIV-1- and HCV-specific therapy in coinfected individuals.

Adolescent↗
PubMed2001

Therapeutic choices for patients with hemophilia and high-titer inhibitors.

Effective treatment of bleeding episodes in hemophilia with high titer inhibitors (HTI) remains a challenge, despite the fact that the therapeutic armamentarium has expanded considerably over the past few years. Treatment safety has improved with the availability of porcine factor VIII (FVIII) and bypassing products such as recombinant factor VIIa (rFVIIa), and plasma-derived activated Prothrombin Complex Concentrates (aPCCs) that are virally inactivated. The major drawbacks of rFVIIa and aPCCs are their unpredictable hemostatic effect, lack of laboratory assays to monitor efficacy and dosing frequency, and the risk of thrombosis. The proceedings of a one-day workshop of physicians who specialized in treating patients with hemophilia held in Vienna on May 13, 2000 have been summarized. In making a decision regarding the choice of product, physicians often consider the type of bleeding episode (life or limb threatening), age of the patient, volume of the reconstituted product, previous exposure to plasma derived products, cost, efficacy, and safety. For plasma naïve patients, to achieve rapid hemostasis a majority of the panelists used porcine FVIII (for patients who lack porcine inhibitory antibodies) or rFVIIa. For patients previously treated with plasma derived factors, in addition to the above concentrates, aPCCs were recommended. Although no data exists regarding safety and efficacy, switching products was routinely practiced either because of availability or cost. Furthermore, the panelists were uncertain about the efficacy of bypassing agents in the prevention of joint disease in inhibitor patients. The workshop participants felt that future research offers the best solution to resolve some of the dilemmas faced by clinicians and may help individualise treatment in a hemophilia patient with a high titer inhibitor.

Animals↗
PubMed2001

Coeliac disease and reproductive disorders: a neglected association.

Coeliac disease is a chronic disease caused by a permanent intolerance to ingested gluten resulting in immunologically mediated inflammatory damage of the small-intestinal mucosa. The wide spectrum of clinical symptoms is partly due to the malnourished state caused by the malabsorption of macro- and micronutrients. Fertility problems, sexual dysfunction and obstetrical complications are more frequently observed in patients with coeliac disease. These reproductive disorders may be a consequence of the endocrine derangements caused by selective nutrient deficiencies. Nowadays, the early diagnosis and treatment of coeliac disease is possible and not very costly. Therefore, coeliac disease must be seriously considered in the preconceptional screening and treatment of patients with reproductive disorders.

Celiac Disease↗
PubMed2001

Semen parameters, including WHO and strict criteria morphology, in a fertile and subfertile population: an effort towards standardization of in-vivo thresholds.

In this study, the semen analysis results of a fertile population were compared with those from a subfertile population, in order to establish normal cut-off values for the standard semen parameters with the aid of receiver operating characteristic (ROC) curve analysis. The fertile group comprised healthy males (n = 107) without any history of fertility problems, the partners of whom had had a spontaneous pregnancy within one year of unprotected intercourse and were pregnant at the time of the male's inclusion into the study. A total of 103 males from couples attending the infertility clinic, and with an initial sperm count of <20x10(6)/ml were recruited to form the subfertile population. The best discriminating parameter between the two populations was sperm morphology evaluated according to WHO criteria at a cut-off point of 31% normal spermatozoa. The other cut-off values were at 8% for the acrosome index, 45% for motility, and 4% normal spermatozoa for strict criteria. Recalculating the ROC curve cut-off values based on an assumed 50% prevalence of subfertility in an assisted reproductive setting, the cut-off points were reduced to 21% and 3% normal spermatozoa for WHO and strict criteria respectively. For motility, the new cut-off value was at 20% motile spermatozoa, for motility quality at 3.5 (on a scale of 1-6), the acrosome index at 3% normal acrosomes, and the teratozoospermia index at 2.09.

Acrosome↗
PubMed2001

Prevention and management of infection in children with sickle cell anaemia.

Sickle cell anaemia (SCA) predisposes a child to infections for various reasons, including increased bone marrow turnover, poor perfusion and functional asplenia leading to decreased opsonisation of polysaccharide encapsulated organisms. Bacteria and viruses that most frequently cause serious infections in children with sickle cell disease are Streptococcus pneumoniae, Haemophilus influenzae type b, Salmonella spp., Escherichia coli, Staphylococcus aureus, Mycoplasma pneumoniae, Chlamydia pneumoniae, parvovirus B19 and hepatitis A, B and C viruses. Penicillin prophylaxis has decreased the incidence of infection-related morbidity and mortality significantly in children with SCA. Children <3 years of age are administered oral penicillin 125mg twice daily, and the dose is increased to 250mg twice daily for the >3 to 5 year age group. Adherence to the penicillin prophylactic regimen is recommended for children with SCA who are >5 years of age. For children with SCA who have recurrent invasive pneumococcal infections, an effort is made to keep the child on penicillin prophylaxis indefinitely. The administration of various childhood vaccines has also made an appreciable impact on the overall morbidity and mortality associated with infection in children with SCA. The administration of the heptavalent conjugate pneumococcal vaccine (PCV7) has provided control of invasive pneumococcal infections, and the prophylactic use of the H. influenzae type b conjugate vaccine has reduced the incidence of septicaemia and meningitis caused by this organism. Other vaccines used prophylactically in children with SCA include hepatitis A and B, and vaccines against influenza and varicella viruses. The immediate administration of intravenous antibacterials, after appropriate blood and urine cultures, is of great importance in the treatment of the febrile child with SCA. Ceftriaxone and cefotaxime have been recommended for the treatment of septic episodes in SCA associated with S. pneumoniae, Haemophilus and Salmonella spp. Infection with Yersinia enterocolitica may be treated with cefotaxime or an aminoglycoside. The prevalence of Helicobacter pylori infection in SCA is unknown. Effective therapies include metronidazole, tetracycline or amoxicillin. Parvovirus infections require supportive care and specific antiviral therapy is not indicated. The judicious use of antimicrobials is encouraged in view of the worldwide emergence of multidrug-resistant strains. The long term sequelae associated with infections in children with SCA can be decreased with the implementation of immunisation programmes and effective and prompt treatment with appropriate antibacterials.

Anemia, Sickle Cell↗
PubMed2001

Intermediate probability lung scans (IPLS): retrospective review of 82 cases.

OBJECTIVE: In the light of a reported 30-40% prevalence of pulmonary embolism (PE) in intermediate probability lung scans (IPLS) based on results of the Prospective Investigation of Pulmonary Embolism Diagnosis (PIOPED) study, we examined the frequency of documented PE in 82 patients with IPLS, the management strategy employed in these patients with regards to additional imaging (e.g. further evaluation with venous sonography or spiral computed tomographic angiography (CTA)), anticoagulation therapy, and subsequent follow-up outcomes. METHOD: Retrospective review of the medical records of 82 patients with intermediate probability ventilation-perfusion (V/Q) lung scans from January 1998 to July 1999. RESULTS: 14.1% of V/Q scans were reported as having an intermediate probability of PE. 72% of IPLS were subject to further evaluation with venous Doppler ultrasound and/or CTA, and 39% of these patients had evidence of thrombo-embolic disease. All patients with imaging evidence of thromboembolic disease were started on anticoagulation therapy. In addition, 19 patients were treated based on clinical judgement. Amongst the 35 patients who were not treated, 17 (49%) were based on clinical findings without further imaging. There was no mortality on follow-up of 28 cases of untreated IPLS. CONCLUSION: The majority of IPLS will have further imaging, out of which over one-third will have thrombo-embolic disease. Approximately half of IPLS cases will receive anticoagulation therapy. No mortality or PE was found on follow-up of patients who were not treated.

Adolescent↗
PubMed2000

Interaction of a bacterially expressed peptide from the receptor binding domain of Pseudomonas aeruginosa pili strain PAK with a cross-reactive antibody: conformation of the bound peptide.

The C-terminal receptor binding region of Pseudomonas aeruginosa pilin protein strain PAK (residues 128-144) has been the target for the design of a vaccine effective against P. aeruginosa infections. We have recently cloned and expressed a (15)N-labeled PAK pilin peptide spanning residues 128-144 of the PAK pilin protein. The peptide exists as a major (trans) and minor (cis) species in solution, arising from isomerization around a central Ile(138)-Pro(139) peptide bond. The trans isomer adopts two well-defined turns in solution, a type I beta-turn spanning Asp(134)-Glu-Gln-Phe(137) and a type II beta-turn spanning Pro(139)-Lys-Gly-Cys(142). The cis isomer adopts only one well-defined type II beta-turn spanning Pro(139)-Lys-Gly-Cys(142) but displays evidence of a less ordered turn spanning Asp(132)-Gln-Asp-Glu(135). These turns have been implicated in cross-reactive antibody recognition. (15)N-edited NMR spectroscopy was used to study the binding of the (15)N-labeled PAK pilin peptide to an Fab fragment of a cross-reactive monoclonal antibody, PAK-13, raised against the intact PAK pilus. The results of these studies are as follows: the trans and cis isomers bind with similar affinity to the Fab, despite their different topologies; both isomers maintain the conformational integrity of their beta-turns when bound; binding leads to the preferential stabilization of the first turn over the second turn in each isomer; and binding leads to the perturbation of resonances within regions of the trans and cis backbone that undergo microsecond to millisecond motions. These slow motions may play a role in induced fit binding of the first turn to Fab PAK-13, which would allow the same antibody combining site to accommodate either trans or cis topology. More importantly for vaccine design, these motions may also play a role in the development of a broad-spectrum vaccine capable of generating an antibody therapeutic effective against the multiple strains of P. aeruginosa.

Amino Acid Sequence↗
PubMed2000

Ruthenium(II) ammine and hydrazine complexes with [N(Ph2PQ)2]- (Q = S, Se) ligands.

Reactions of coordinatively unsaturated Ru[N(Ph2PQ)2]2(PPh3) (Q = S (1), Se (2)) with pyridine (py), SO2, and NH3 afford the corresponding 18e adducts Ru[N(Ph2PQ)2]2(PPh3)(L) (Q = S, L = NH3 (5); Q = Se, L = py (3), SO2 (4), NH3 (6)). The molecular structures of complexes 2 and 6 are determined. The geometry around Ru in 2 is pseudo square pyramidal with PPh3 occupying the apical position, while that in 6 is pseudooctahedral with PPh3 and NH3 mutually cis. The Ru-P distances in 2 and 6 are 2.2025(11) and 2.2778(11) A, respectively. The Ru-N bond length in 6 is 2.185(3) A. Treatment of 1 or 2 with substituted hydrazines L or NH2OH yields the respective adducts Ru[N(Ph2PQ)2]2(PPh3)(L) (Q = S, L = NH2NH2 (12), t-BuNHNH2 (14), l-aminopiperidine (C5H10NNH2) (15); Q = Se, L = PhCONHNH2 (7), PhNHNH2 (8), NH2OH (9), t-BuNHNH2 (10), C5H10NNH2 (11), NH2NH2 (13)), which are isolated as mixtures of their trans and cis isomers. The structures of cis-14 and cis-15 are characterized by X-ray crystallography. In both molecular structures, the ruthenium adopts a pseudooctahedral arrangement with PPh3 and hydrazine mutually cis. The Ru-N bond lengths in cis-14.CH2Cl2 and cis-15 are 2.152(3) and 2.101(3) A, respectively. The Ru-N-N bond angles in cis-14.CH2Cl2 and cis-15 are 120.5(4) and 129.0(2) degrees, respectively. Treatment of 1 with hydrazine monohydrate leads to the isolation of yellow 5 and red trans-Ru[N(Ph2PS)2]2(NH3)(H2O) (16), which are characterized by mass spectrometry, 1H NMR spectroscopy, and elemental analyses. The geometry around ruthenium in 16 is pseudooctahedral with the NH3 and H2O ligands mutually trans. The Ru-O and Ru-N bond distances are 2.118(4) and 2.142(6) A, respectively. Oxidation reactions of the above ruthenium hydrazine complexes are also studied.

Journal Article↗
PubMed2000

Experimental investigation into the incineration of wool scouring sludges in a novel rotating fluidised bed.

The main purpose of this research was to investigate the possibility of incineration of wool scouring sludges in a novel vertical axis rotating fluidized bed (RFB). A small-scale RFB was designed and constructed with an internal diameter (ID) of 200 mm and height of 50 mm to carry out the experiments. In phase one of the experiments, a cold test was conducted to investigate the fluidization performance of the RFB, which eventually led to the optimisation of the operating parameters, i.e., sand particle size, rotation speed and bed loading (bed thickness) which ensures complete fluidization and minimum particle elutriation. Sand particle size of 0.5 to 0.6 mm, rotation speed of 200 to 400 rpm and bed loading of 1 kg (equivalent to bed thickness of 27 mm) were found optimal. These information generated were useful for the second phase of the experiments, which was the hot test, in investigating the possibility of incinerating wool scouring sludges in the RFB. Nine wool sludges from different process routes generated from the wool scouring industries were analysed for their compositions. Most of these sludges were highly moist, had high volatile matter and high ash content with low level of fixed carbon. These characteristics made incineration difficult. Hence, the effect of varying the moisture content, rotation speed and sludge feed rate on the incineration of the three selected sludges were studied in the hot test. With 5% support methane, all sludges with a maximum moisture up to 70% as-received could be successfully burned in the RFB at rotating speeds of 200 and 300 rpm. The combustion was found to be intense with a high efficiency due to the good turbulence and mixing in the RFB. The combustion gases produced, i.e., CO, CO(2) and NO(x) were reasonably low due to the high combustion intensity and efficiency. To study the dynamics of the bed and freeboard region in the RFB, the velocity flow field was simulated using a computational fluid dynamics (CFD) model to generate information of the flow pattern. The special advantages of swirling flow would benefit the gas combustion in the RFB. The experimental results obtained have suggested that the incineration was successful and the ash particles elutriated were fine due to the good mixing and turbulence in the RFB. This also reflects the RFB as an effective incinerator.

Animals↗
PubMed2000

Impact of bone marrow transplantation for symptomatic sickle cell disease: an interim report. Multicenter investigation of bone marrow transplantation for sickle cell disease.

Fifty children who had symptomatic sickle cell disease received matched sibling marrow allografts between September 1991 and March 1999, with Kaplan-Meier probabilities of survival and event-free survival of 94% and 84%, respectively. Twenty-six patients (16 male, 10 female) had at least 2 years of follow-up after transplantation and were evaluated for late effects of transplantation and for its impact on sickle cell-related central nervous system (CNS) and pulmonary disease. Patients ranged between 3.3 and 14.0 (median, 9. 4) years of age and had a median follow-up of 57.9 (range 38-95) months after transplantation. Among 22 of 26 patients who had stable donor engraftment, complications related to sickle cell disease resolved, and none experienced further episodes of pain, stroke, or acute chest syndrome. All 10 engrafted patients with a prior history of stroke had stable or improved cerebral magnetic resonance imaging results. Pulmonary function tests were stable in 22 of the 26 patients, worse in two, and not studied in two. Seven of eight patients transplanted for recurrent acute chest syndrome had stable pulmonary function. Linear growth measured by median height standard deviation score improved from -0.7 before transplantation to -0.2 after transplantation. An adverse effect of busulfan conditioning on ovarian function was demonstrated in five of seven evaluable females who are currently at least 13 years of age. None of the four males tested had elevated serum gonadotropin levels. These data confirm that allogenic bone marrow transplantation establishes normal erythropoiesis and is associated with improved growth and stable CNS imaging and pulmonary function in most patients. (Blood. 2000;95:1918-1924)

Adolescent↗