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The one-step synthesis of [14 C]potassium cyanate from [14 C]urea is described with product characterization by gravimetric specific activity as well as a novel TLC system. The storage, stability, and repurification of [14 C]potassium cyanate are also discussed.
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Radioisótopos de Carbono/química , Cianatos/química , Cianatos/síntese química , Ureia/química , Técnicas de Química Sintética , RadioquímicaRESUMO
BACKGROUND: Evidence for the relationship between antidepressant use during pregnancy and the risk of attention-deficit/hyperactivity disorder (ADHD) in the children is conflicting. OBJECTIVE: To assess the association between fetal exposure to antidepressant drugs and the subsequent development of ADHD. SEARCH STRATEGY: A systematic literature search was conducted in PubMed, EMBASE, PsycINFO, and CINAHL databases to identify relevant cohort studies published from inception until October 2017. SELECTION CRITERIA: Cohort studies, identifying children with ADHD diagnosis and linking antidepressant use during pregnancy in their mothers. DATA COLLECTION: Two reviewers independently abstracted data and assessed study quality. MAIN RESULTS: The literature search identified six relevant cohort studies with association between antidepressant exposure during pregnancy and the risk of ADHD in children [hazard ratio (HR) 1.34; 95% confidence interval (CI) 1.14-1.57]. However, the association was not statistically significant when the reference group was mothers with psychiatric disorders not treated during pregnancy (HR 0.96; 95% CI 0.76-1.2; n = 2 studies). Moreover, preconception exposure to antidepressants was significantly associated with increased risk of ADHD (HR 1.82; 95% CI 1.54-2.15; n = 3 studies). CONCLUSIONS: The significant association between antidepressant exposure during pregnancy and ADHD in the children can be partially explained by confounding by indication. Given the low number of included studies, further studies with prospective designs that use validated measurements and controls for important confounders are needed to verify our findings. TWEETABLE ABSTRACT: Antidepressant use during pregnancy may be not associated with ADHD in the offspring.
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Antidepressivos/efeitos adversos , Transtorno do Deficit de Atenção com Hiperatividade/induzido quimicamente , Depressão/tratamento farmacológico , Complicações na Gravidez/tratamento farmacológico , Efeitos Tardios da Exposição Pré-Natal/induzido quimicamente , Criança , Estudos de Coortes , Feminino , Humanos , Gravidez , Complicações na Gravidez/psicologia , Modelos de Riscos ProporcionaisRESUMO
Hypopituitarism in leukemia is very rare. In addition, central nervous system (cns) relapse and leukemic retinopathy in childhood acute lymphoblastic leukemia (all) have declined with the use of modern systemic chemotherapy that includes cns prophylaxis. Here, we report the case of a 4-year-old girl who received chemotherapy and intrathecal therapy without cns radiation after a diagnosis of B-precursor all without cns involvement. Three months after chemotherapy completion, she presented with lower-extremity weakness and was diagnosed with an isolated cns relapse. Concurrent hypopituitarism and leukemic retinopathy were also found. After receiving craniospinal radiotherapy and systemic chemotherapy, her retinopathy and vision improved. She is now in complete remission, and she is still on chemotherapy according to the guideline from the Pediatric Oncology Group. Although rare, hypopituitarism and leukemic retinopathy should be taken into consideration in patients with cns involvement by leukemia.
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BACKGROUND AND OBJECTIVES: Ael is a rare blood type that is characterized by weak agglutination of RBCs when reacts with anti-A antibody in adsorption-elution test. Although IVS6 + 5GâA mutation is known to associate with the Ael blood type, genetic and mechanistic evaluation for the weak agglutination of Ael with IVS6 + 5GâA mutation has not yet been completely addressed. MATERIALS AND METHODS: In this study, five cases of confirmed Ael individuals were analysed. The cDNAs for the A(el) alleles were obtained by cloning method for sequence analyses. The erythroleukemia K562 cells were used as the cell study model and were transfected with the A(el) expression construct. Flow cytometry analysis was then performed to determine the levels of surface antigen expression. RESULTS: The results indicated that IVS6 + 5GâA attributes to all cases of Ael . RT-PCR analyses revealed the presence of at least 10 types of aberrant A(el) splicing transcripts. Most of the transcripts caused early termination and produced non-functional protein during translation. Nevertheless, the transcript without exons 5-6 was predicted to generate functional Ael glycosyltransferase lacking 57 amino acids at the N-terminal segment. When the exons 5-6 deletion transcript was stably expressed in the K562 cells, weak agglutination of the cells can be induced by adding anti-A antibody followed by adsorption-elution test. CONCLUSION: This study demonstrates that aberrant splicing of A transcripts contributes to weak A expression and the weak agglutination of Ael -RBCs, adding to the complexity for the regulatory mechanisms of ABO gene expression.
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Sistema ABO de Grupos Sanguíneos/genética , Mutação , Fenótipo , Alelos , Tipagem e Reações Cruzadas Sanguíneas , Linhagem Celular Tumoral , Éxons , HumanosRESUMO
Imatinib has improved outcomes in patients with Philadelphia chromosome-positive (Ph+) acute lymphoblastic leukemia (all). Minimal residual disease (mrd) is a useful tool for predicting leukemia relapse. However, there is no consensus on how to treat children with elevation of BCR-ABL transcripts but no evidence of hematologic relapse during chemotherapy combined with imatinib. Here, we report the case of a child with Ph+ all who had persistent elevation of mrd, but no evidence of hematologic relapse while receiving imatinib plus intensive chemotherapy. Dasatinib was substituted for imatinib because no suitable donor for allogeneic hematopoietic stem-cell transplantation (hsct) was available. Less-intensive chemotherapy with methotrexate and 6-mercaptopurine was administered concomitantly. No serious adverse events were encountered. With continuous dasatinib combined with chemotherapy, but no allogeneic hsct, our patient reached complete molecular remission and has been in complete molecular remission for more than 13 months. This report is the first about the long-term use of dasatinib in patients with Ph+ all and mrd elevation but hematologic remission during imatinib chemotherapy. In a similar situation, chemotherapy combined with dasatinib instead of allogeneic hsct could be considered to avoid hsct-related mortality and morbidity. Clinical trials are needed.
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Cerebrospinal fluid (CSF) amino acid concentrations were measured in 45 children with acute lymphoblastic leukemia (ALL). Central nervous system (CNS) disease was absent in 34 and present in 11 (Groups L and M, respectively) at diagnosis. Thirty-two otherwise healthy children with febrile convulsions were studied for comparison. Results from this study show that glutamine levels at Day 0 were significantly higher in patients than in controls. Patients in Group M had elevated glutamine levels compared to Group L. In comparison, at Day 14, concentrations of glutamine and asparagine decreased, while glutamic acid amounts increased significantly in Group L. Glutamine levels fell at Day 42 in Group M, which may have resulted from more intensive treatment. From this study we hypothesise that higher baseline glutamine levels are indicative of a greater risk for CNS leukemia. Large-scale prospective trials are required to confirm increased baseline CSF glutamine levels in ALL patients, to identify glutamine as a marker for CNS disease and to clarify underlying mechanisms regulating glutamine in ALL.
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Aminoácidos/líquido cefalorraquidiano , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Leucemia-Linfoma Linfoblástico de Células Precursoras/líquido cefalorraquidiano , Adolescente , Criança , Feminino , Humanos , Lactente , Masculino , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamento farmacológico , Estatísticas não ParamétricasRESUMO
Current body mass index (BMI) norms for children and adolescents are developed from a reference population that includes obese and slim subjects. The validity of these norms is influenced by the observed secular increase in body weight and BMI. We hypothesized that the performance of children in health-related physical fitness tests would be negatively related to increased BMIs, and therefore fitness tests might be used as criteria for developing a more appropriate set of BMI norms. We evaluated the existing data from a nation-wide fitness survey for students in Taiwan (444 652 boys and 433 555 girls) to examine the relationship between BMI and fitness tests. The fitness tests used included: an 800/1600-m run/walk; a standing long jump; bent-leg curl-ups; and a sit-and-reach test. The BMI percentiles developed from the subgroup whose test scores were better than the 'poor' quartile in all four tests were compared with those of the whole population and linked to the adult criteria for overweight and obesity. The BMIs were significantly related to the results of fitness testing. A total of 43% of students had scores better than the poorest quartile in all of their tests. The upper BMI percentile curves of this fitter subgroup were lower than those of the total population. The 85th and 95th BMI percentile values of the fitter 18-year-old-students (23.7 and 25.5 kg m(-2) for boys; 22.6 and 24.6 kg m(-2) for girls) linked well with the adult cut-off points of 23 and 25 kg m(-2), which have been recommended as the Asian criteria for adult overweight and obesity. Hence, the BMI norms for children and adolescents could be created from selected subgroups that have better physical fitness. We expect that the new norms based on this approach will be used not only to assess the current status of obesity or overweight, but also to encourage activity and exercise.
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Índice de Massa Corporal , Exercício Físico , Obesidade/epidemiologia , Aptidão Física/fisiologia , Adolescente , Distribuição por Idade , Criança , Feminino , Inquéritos Epidemiológicos , Humanos , Masculino , Obesidade/classificação , Distribuição por Sexo , Taiwan/epidemiologiaRESUMO
OBJECTIVE: To assess the effects of early postnatal dexamethasone therapy on hematologic values in preterm infants. MATERIALS AND METHODS: We reviewed the hematologic data of 179 preterm infants who participated in a double-blind clinical trial of early postnatal dexamethasone therapy (<12 hours after birth) for the prevention of chronic lung disease. One group (86 infants) received saline and the other group (93 infants) received dexamethasone. Dexamethasone was given intravenously every 12 hours in tapering doses: 0.25 mg/kg on days 1 to 7, 0.12 mg/kg on days 8 to 14, 0.05 mg/kg on days 15 to 21, and 0.02 mg/kg on days 21 to 28. Blood samples were obtained on days 0, 3, 7, 10, 14, 21, and 28. None of the infants received prenatal steroid therapy. RESULTS: Multiple regression analysis revealed significant differences in the values versus time curves of the white blood cell, neutrophil, lymphocyte, basophil, and eosinophil counts between the two groups. The white blood cell count was significantly higher in the dexamethasone group on days 7 through 14, and this was associated with significantly higher numbers of segmented neutrophils and band forms and significantly lower numbers of lymphocytes and eosinophils. The hematocrit and platelet counts were similar in the two groups throughout most of the trial. Except for platelet count, steroid therapy did not alter the hematologic values for infants with bacteremia. CONCLUSION: Dexamethasone affects white blood cell, segmented neutrophil, lymphocyte, basophil, and eosinophil counts in neonates. This should be taken into consideration when evaluating preterm infants who are receiving dexamethasone.early dexamethasone therapy; neonatal blood count; preterm infant; respiratory distress syndrome.
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Contagem de Células Sanguíneas/efeitos dos fármacos , Dexametasona/uso terapêutico , Glucocorticoides/uso terapêutico , Recém-Nascido Prematuro/sangue , Pneumopatias/prevenção & controle , Síndrome do Desconforto Respiratório do Recém-Nascido/tratamento farmacológico , Bacteriemia/sangue , Doença Crônica , Dexametasona/administração & dosagem , Método Duplo-Cego , Feminino , Glucocorticoides/administração & dosagem , Humanos , Recém-Nascido , MasculinoRESUMO
Aberrant transcripts of FHIT and TSG101 using nested RT-PCR were reported in many human tumours. The role of these aberrant transcripts in tumourigenesis is not clear. We, therefore, analyzed the aberrant transcripts of FHIT, TSG101 and PTEN/MMAC1 in peripheral mononuclear cells of normal individuals using nested RT-PCR to explore the role of these genes in cancer development. The results showed that there are at least five types of aberrant transcripts: type I is the deletion at junction located in-between normal exon and intron; type II has deletion of some bases and subsequent insertion of several bases in the deletion area; type III accommodates splicing donor or acceptor site-like sequence; type IV has homologous sequences near the deleted junction; and type V comprises the homologous sequences at the deletion junction. A normal healthy person can have more than one aberrant transcripts of FHIT, TSG101 and PTEN/MMAC1 genes. The size and the number of the transcripts vary and the diversity is unconstrained. It is not depended on the time, condition of the reaction, or the isolation method. From these results, we suggested that the aberrant transcripts of FHIT, TSG101 and PTEN/MMAC1 genes may be the imperfect products of splicesome which occur one in every thousands, ten thousands or more. As a result, these data implied no direct association between the aberrant transcripts and tumourigenesis.
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Hidrolases Anidrido Ácido , Proteínas de Ligação a DNA/genética , Leucócitos Mononucleares/metabolismo , Proteínas de Neoplasias , Monoéster Fosfórico Hidrolases/genética , Proteínas/genética , Fatores de Transcrição/genética , Proteínas Supressoras de Tumor , Adulto , Sequência de Bases , DNA/análise , Complexos Endossomais de Distribuição Requeridos para Transporte , Humanos , Dados de Sequência Molecular , PTEN Fosfo-Hidrolase , RNA Mensageiro/análise , RNA Mensageiro/genética , Valores de Referência , Reação em Cadeia da Polimerase Via Transcriptase ReversaAssuntos
Coagulantes/uso terapêutico , Fator VIII/uso terapêutico , Hemofilia A/tratamento farmacológico , Sacarose/uso terapêutico , Adolescente , Adulto , Idoso , Criança , Pré-Escolar , Humanos , Masculino , Pessoa de Meia-Idade , Vigilância de Produtos Comercializados , Taiwan , Resultado do Tratamento , Adulto JovemRESUMO
The chromatographic identity of a compound can be determined by four parameters, namely, I, A, Z and (GRF). These are interrelated in a linear regression equation, given in the paper as Eq. 8. The retrieval of structural information from retention data requires the introduction of a new meaning to the Kováts retention index, the use of column difference (delta I) to characterize functional groups, the redefinition of the role of electronegative oxygen and nitrogen atoms, and the division of retention index (I) into contributions from atoms and from functional groups. The separation of retention index (I) into molecular and interaction contributions is a necessary condition for retention index prediction from structure and also for structure information retrieval from retention data. According to Eq. 8 the retention index is uniquely determined by three parameters, namely A, Z and (GRF). For prediction of retention index, the A value is assigned a value of 100 index units (i.u.), the Z value is obtained directly from the compound, and the (GRF) value is pre-calibrated. In Eq. 10, the m and n values represent the pre-calibrated terms for a quantitative structure-retention index relationship. These terms account for the positive and negative retention contributions from polar and polarizable atom groups. All atom groups that are different from methylene and methyl groups will interact with the stationary phase and contribute to retention. The m and n values for various functional, polar and polarizable atom groups and their column differences (delta I values) are the results of interactions between the solute and the stationary phase and are structure dependent. The interaction increases with increasing polarities of the solute and the stationary phase. The column difference not only reflects the strength of the interaction, but is also characteristic of the functional and polarizable groups. The retrieval of structural information from retention data is equivalent to obtaining Z and (GRF) values from known I and delta I values, which is straightforward for monofunctional compounds. For multi-functional compounds, additional data will be needed for retrieval of structural information. These can be obtained from derivatization of the unknown compound, from its chemical reactions with other reagents, from GC-MS analysis and from structure match using internal or external standards. The additional data required will depend upon the complexity of the unknown structure. This approach demonstrates that a system can be devised to utilize GC retention characteristics uniquely for structure elucidation.
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Físico-Química/instrumentação , Cromatografia , Físico-Química/normas , Humanos , Estrutura MolecularRESUMO
The retention index increment for addition of a methylene group to an analyte molecule is shown for 1-halo-n-alkanes to be different from 100 i.u., a value that is customarily assigned according to the current convention in retention index prediction. In temperature-programmed gas chromatography using linearly interpolated retention index I, a linear regression equation, I=AZ+(GRF), with the number of atoms (Z) in the molecule as variable can describe the retention of 16 homologous series of organic compounds on non-polar and polar columns with characteristic A (linear regression coefficient) and (GRF) (group retention factor) values. A molecular model of retention on the basis of electron density and electron density distribution relative to that of n-alkane is proposed. This model brings out the inter- and intramolecular electronic effects in the analyte molecule and its dipole-dipole interaction with the stationary liquid phases, as variations in the A value. The (GRF) value varies with the connectivity ability of a functional group for extended conjugation, substitution, etc., but is most influenced by hydrogen bonding (H-bonding) with the stationary liquid phase. One can estimate the sequence of elution of a mixture of organic compounds from any two of the three parameters on the right-hand side of the above equation or retrieve the retention indexes of an entire homologous series from its A and (GRF) values. The fact that each analyte molecule has its own A value on different columns makes column difference (deltaI) compound-specific rather than column-specific, a departure from previous assumptions.
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Cromatografia Gasosa/métodosRESUMO
AIM: To compare the efficacy and safety of an indomethacin treatment strategy based on serial echocardiographic measurement of patent ductus arteriosus (PDA) flow pattern with a standard protocol. METHODS: Neonates weighing less than 1500 g at birth, who required respiratory support, and who had developed symptomatic PDA, were studied. PDA was confirmed in all infants using colour Doppler echocardiography, and serial observations of the ductal flow pattern were made. Infants randomly assigned to receive conventional indomethacin treatment (protocol group) were given an initial dose of 0.2 mg/kg, followed by 0.1 or 0.2 mg/kg, depending on age, 12 hourly for two further doses, and were eligible for a second course. Those randomly assigned to the ductal flow pattern assessment (ECHO group) received further doses of indomethacin after 24 hours, only if their flow pattern was "pulsatile" or "growing." RESULTS: There was no significant difference in the primary outcome measures between the two groups. The closure rate was 89.1% and 87.2%, respectively, in the protocol and ECHO groups. The mean (SD) doses of indomethacin were significantly higher in the protocol group: 3.2 (1.4) doses compared with 1.6 (0.9) doses. There was a significantly higher incidence of hypoglycaemia, impaired urine output, and gastrointestinal bleeding in the protocol group. CONCLUSIONS: An indomethacin treatment strategy for PDA based on measurement of the ductal flow pattern is associated with a reduction in the total doses of indomethacin administered, and a reduced rate of complications, compared with a conventional protocol. There is no difference in closure rate.
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Anti-Inflamatórios não Esteroides/uso terapêutico , Permeabilidade do Canal Arterial/tratamento farmacológico , Indometacina/uso terapêutico , Permeabilidade do Canal Arterial/diagnóstico por imagem , Ecocardiografia Doppler em Cores , Humanos , Recém-Nascido , Recém-Nascido Prematuro , Recém-Nascido de muito Baixo Peso , Reprodutibilidade dos TestesRESUMO
This retrospective study sought to determine the characteristics of community-acquired methicillin-resistant Staphylococcus aureus (CA-MRSA) infections in patients younger than 18 years without known risk factors who were treated at a teaching hospital in central Taiwan. Epidemiological and clinical data were collected from medical charts. Possible risk factors included hospitalization within the past 6 months, transfer from other hospitals or nursing homes, and having underlying illness. A total of 173 isolates of community-acquired S. aureus were analyzed. Seventeen (9.8%) of these 173 isolates were methicillin-resistant S. aureus collected from patients without risk factors, 31 (17.9%) were methicillin-resistant S. aureus from patients with risk factors, and the other 125 (72.3%) were methicillin-susceptible S. aureus. Most isolates of community-acquired methicillin-resistant S. aureus collected from patients without risk factors (14/17, 82.4%) were obtained from the infected wounds of skin or soft tissues. Only 4 (23.5%) in 17 patients with isolates resistant to methicillin were prescribed antimicrobial therapy with glycopeptides. Nevertheless, all patients recovered without any long-term sequelae. These results highlight the fact that community-acquired methicillin-resistant S. aureus infections occur frequently in Taiwan among patients who have no established risk factors for this infection.
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Infecções Comunitárias Adquiridas/etiologia , Resistência a Meticilina , Infecções Estafilocócicas/etiologia , Adolescente , Criança , Pré-Escolar , Infecções Comunitárias Adquiridas/tratamento farmacológico , Feminino , Humanos , Lactente , Masculino , Estudos Retrospectivos , Fatores de Risco , Infecções Estafilocócicas/tratamento farmacológico , Staphylococcus aureus/efeitos dos fármacosRESUMO
We used a polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) method for DNA-based typing of Duffy, Yt and Colton blood groups in Taiwanese, Filipinos and Thais. A total of 200 Taiwanese, 115 Filipinos and 105 Thais were studied. In the Duffy blood group in Taiwanese, 180 cases (90%) were homozygote of Fya, 18 cases (9%) were double heterozygote of Fya and Fyb, and 2 cases (1%) were homozygote of Fyb. In Filipinos, 98 cases (85.2%) were homozygote of Fya, 16 cases (14.0%) were double heterozygote of Fya and Fyb and 1 case (0.8%) was homozygote of Fyb. In Thais, 87 cases (82.9%) were homozygote of Fya, 18 cases (17.1%) were double heterozygote of Fya and Fyb, and no case of Fyb was found. These results correlate well with serological phenotype. For the Yt blood group, only YT1 was found in Taiwanese. Among Filipinos, 114/115 (99.1%) was YT1/1 and 1/115 (0.9%) was YT1/2. In Thais, 103/105 (98.1%) was YT1/1 and 2/105 (1.9%) was YT1/2. For the Colton blood group, the results showed that there was only Coa allele in these three populations. Our results provide the first data of the Yt and Colton blood groups in these three populations.
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Antígenos de Grupos Sanguíneos/genética , Sistema do Grupo Sanguíneo Duffy/genética , Genótipo , Humanos , Filipinas , Reação em Cadeia da Polimerase , Polimorfismo de Fragmento de Restrição , Taiwan , TailândiaRESUMO
Twenty seven newborn infants with persistent hypoxemia in the first 3 days after birth were enrolled for hemodynamic assessment using echocardiography. Measurements included pulmonary arterial pressure (peak velocity of tricuspid regurgitation (TR), patent ductus arteriosus (PDA) flow pattern, interatrial shunting flow pattern and pulmonary flow velocity ratio (the time to peak velocity/right ventricle ejection time ratio (TPV/RVET)) and left ventricular ejection fraction. The estimated systolic pulmonary arterial pressure and the systemic arterial pressure determined via an indwelling arterial line were recorded at the time of echocardiographic examination, and pulmonary arterial pressure/systemic arterial pressure was calculated. Nineteen infants (70.4%) had a TR sufficient to estimate systolic pulmonary arterial pressure. The median value of pulmonary arterial pressure/systemic arterial pressure was 1.02 (range, 0.68 to 1.78). Twenty two infants (81.5%) had a PDA and flow patterns indicating pulmonary arterial pressure above or approaching systemic arterial pressure. All infants had a foramen ovale and flow patterns were bi-directional or pure right-to-left. TPV/RVET had a wide range of values (0.23 to 0.55), and only 44.5% of infants had high pulmonary arterial pressure as reflected by low TPV/RVET ratio. Eleven infants (40.7%) had an ejection fraction below the normal range. Results of 17 survivors were compared with 8 deceased infants (2 infants of birth weight less than 1000 gm were excluded who died of massive pulmonary hemorrhage). There were no significant differences for any parameter of pulmonary arterial pressure, but ejection fraction was significantly lower in deceased infants. This study has demonstrated that it is possible to evaluate pulmonary arterial pressure noninvasively by using echocardiography in most newborn infants with clinical evidence of persistent pulmonary hypertension of the newborn (PPHN). Ejection fraction is an echocardiographic parameter which can significantly predict mortality.
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Ecocardiografia Doppler/métodos , Recém-Nascido Prematuro , Síndrome da Persistência do Padrão de Circulação Fetal/diagnóstico por imagem , Síndrome da Persistência do Padrão de Circulação Fetal/mortalidade , Feminino , Testes de Função Cardíaca , Hemodinâmica/fisiologia , Humanos , Incidência , Recém-Nascido , Unidades de Terapia Intensiva Neonatal , Masculino , Estudos Prospectivos , Valores de Referência , Fatores de Risco , Sensibilidade e Especificidade , Análise de Sobrevida , Taiwan/epidemiologiaRESUMO
The microorganisms, outcome of infections and the risk factors were evaluated in 39 patients with beta-thalassemia who received frequent blood transfusions. Among these patients, thirteen developed 22 episodes of infections, and bacteremia accounted for 72.7% (16/22) of all infections. Three patients developed meningitis, two patients had liver abscesses, three patients had soft tissue infections, one patient had a urinary tract infection and one patient had lobar pneumonia. Interestingly, a large proportion of the patients were infected by Gram-negative bacteria. Patients who were implanted with intravascular catheters were most susceptible to bacterial infection (1.70 episodes/patient) (P = 0.0069). So were patients with ferritin levels over 2,000 ng/mL (1.18 episodes/patient) (P = 0.028). The frequency of bacterial infections in patients with splenectomies (1.08 episode/patient) was also significantly higher than that of the average patient (P = 0.025). In conclusion, three major risk factors for bacterial infection were identified in this group of patients: intravascular catheterization, high serum ferritin levels (> or = 2,000 ng/mL) and splenectomy. The infection rate of these patients (0.45 episode/100 patient-year) is about 20-fold higher than that of general pediatric patients (0.023 episode/100 patient-year).
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Infecções Bacterianas/epidemiologia , Reação Transfusional , Talassemia beta/microbiologia , Adolescente , Adulto , Infecções Bacterianas/etiologia , Criança , Pré-Escolar , Feminino , Ferritinas/sangue , Humanos , Masculino , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/microbiologia , Fatores de Risco , Esplenectomia , Taiwan , Talassemia beta/terapiaRESUMO
To determine the incidence and classification of chronic lung disease (CLD) in extremely low birth weight (ELBW) infants, a 2-year retrospective analysis was performed. From January 1997 to December 1998, 117 infants weighing less than 1000 g were enrolled. The survival rate beyond 28 days was 60.7% (71/117). CLD was defined as a supplemental oxygen requirement at 28 days of age, with symptoms of persistent respiratory distress and chest radiograph showing characteristic appearance. In addition to the common finding of CLD, infants with bronchopulmonary dysplasia (BPD) had history of respiratory distress syndrome (RDS), infants with Wilson-Mikity syndrome (WMS) had no RDS but had early appearance of bubbly lung on chest x-ray, and infants with chronic pulmonary insufficiency of prematurity (CPIP) had only hazy appearance on chest x-ray. The incidence of CLD in infants who survived beyond 28 days was 50.7% (36/71). Among the 36 infants with CLD, 17 (47%) had BPD, 4 (11%) had WMS and 15 (42%) had CPIP. The median (min, max) days of mechanical ventilation were 45 (9, 112), 45.5 (45, 50) and 7.5 (0, 40) days in BPD, WMS and CPIP groups, respectively. The median (min, max) days of oxygen requirement were 73 (28, 120), 149 (70, 211) and 52.5 (38, 90) days, respectively. The infants still requiring oxygen at post-conceptional age of 36 weeks are significantly more in BPD (14 (82.4%)) and in WMS (4 (100%)) than in CPIP (3 (20%)). Two (1 BPD, 1 WMS) were discharged and received oxygen therapy at home. Four infants with BPD died of respiratory failure. CLD includes a wide range of conditions, from BPD or WMS with severe respiratory morbidity and mortality to no residual problems. Such information is important for design of appropriate strategies to prevent CLD.
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Recém-Nascido de muito Baixo Peso , Pneumopatias/epidemiologia , Displasia Broncopulmonar/epidemiologia , Doença Crônica , Humanos , Incidência , Lactente , Recém-Nascido , Pneumopatias/mortalidade , Pneumopatias/terapia , Estudos Retrospectivos , Taxa de SobrevidaRESUMO
Mutation analysis of CYP21A2 gene was performed in seven patients with congenital adrenal hyperplasia (CAH) by combining differential long template polymerase chain reaction (PCR) amplification and amplified created restriction site (ACRS) methods. All mutations were identified, including five alleles of deletions, three alleles of splicing (IVS2-12[C/A] > G), four alleles of Ile172Asn, and two alleles of Arg356Trp. During the course of genetic analysis of CYP21A2, we found that misgenotyping of CAH by PCR-based method is possible if both alleles of a CAH patient were deletion mutations and at least one of them carried a CYP21A1P-CYP21A2 fusion gene. We also found a patient's mother was misgenotyping as IVS2-12[C/A] > G homozygous due to "allele dropout" in the PCR amplification process. We present in this article evidences of mis-genotyping by PCR-based amplification method. Due to the pitfalls observed in this study, we recommend that more methods, including microsatellite linkage analysis and direct sequencing, should be performed with direct amplification of known mutations in prenatal diagnosis of CAH to avoid misdiagnosis.