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1.
Medicine (Baltimore) ; 102(31): e34462, 2023 Aug 04.
Artigo em Inglês | MEDLINE | ID: mdl-37543796

RESUMO

Polycythemia vera (PV) diagnosis remains a difficult task despite various updates in the 2016 World Health Organization (WHO) diagnostic criteria compared to 2008 criteria. This study aimed to examine the biochemical and clinical features of patients diagnosed with PV using the WHO 2016 criteria but would have been missed by the WHO 2008 criteria, and to ascertain the impact of the lowered thresholds on PV diagnosis. A total of 229 patients with suspected myeloproliferative neoplasms were included in this cross sectional study. The study group was divided with regard to hemoglobin values. Group A consisted of 126 patients with hemoglobin values of ≤ 18.5 g/dL in males and ≤ 16.5 g/dL in females. Group B comprised 103 patients with hemoglobin values of > 18.5 g/dL in males and > 16.5 g/dL in females. The number of PV diagnoses increased to 145 from 87 (increased by 66.67%) when the 2016 diagnostic criteria were employed rather that the 2008 criteria. Mean age and the frequency of female subjects were lower in Group A compared to Group B. The groups were similar in terms of chronic obstructive pulmonary disease/obstructive sleep apnea syndrome, spleen status, smoking status, and mean corpuscular volume, white blood count, neutrophil, eosinophil and platelet values. red blood cells and lactate dehydrogenase values were significantly higher, while lymphocyte counts were significantly lower in Group B. With the introduction of WHO 2016 criteria, we found a significant increase in the number of patients who were candidates for PV testing and were ultimately diagnosed with PV. These findings support the diagnostic value of the 2016 WHO criteria, and by extension, the lowered thresholds for detection of patients requiring further analysis.


Assuntos
Transtornos Mieloproliferativos , Policitemia Vera , Masculino , Humanos , Feminino , Policitemia Vera/diagnóstico , Estudos Transversais , Transtornos Mieloproliferativos/diagnóstico , Hemoglobinas , Organização Mundial da Saúde
2.
Mult Scler Relat Disord ; 77: 104847, 2023 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-37393803

RESUMO

BACKGROUND: Neuromyelitis optica spectrum disorders (NMOSD) are immune-mediated inflammatory disorders of the central nervous system (CNS) mostly presenting as optic neuritis and acute myelitis. NMOSD can be associated with seropositivity for aquaporin 4 antibody (AQP4 IgG), myelin oligodendrocyte glycoprotein antibody (MOG IgG), or can be seronegative for both. In this study, we retrospectively examined our seropositive and seronegative pediatric NMOSD patients. METHOD: Data were collected from all participating centres nationwide. Patients diagnosed with NMOSD were divided into three subgroups according to serology: AQP4 IgG NMOSD, MOG IgG NMOSD, and double seronegative (DN) NMOSD. Patients with at least six months of follow-up were compared statistically. RESULTS: The study included 45 patients, 29 female and 16 male (ratio:1.8), mean age 15.16 ± 4.93 (range 5.5-27) years. Age at onset, clinical manifestations, and cerebrospinal fluid findings were similar between AQP4 IgG NMOSD (n = 17), MOG IgG NMOSD (n = 10), and DN NMOSD (n = 18) groups. A polyphasic course was more frequent in the AQP4 IgG and MOG IgG NMOSD groups than DN NMOSD (p = 0.007). The annualized relapse rate and rate of disability were similar between groups. Most common types of disability were related to optic pathway and spinal cord involvement. Rituximab in AQP4 IgG NMOSD, intravenous immunoglobulin in MOG IgG NMOSD, and azathioprine in DN NMOSD were usually preferred for maintenance treatment. CONCLUSION: In our series with a considerable number of double seronegatives, the three major serological groups of NMOSD were indistinguishable based on clinical and laboratory findings at initial presentation. Their outcome is similar in terms of disability, but seropositive patients should be more closely followed-up for relapses.


Assuntos
Neuromielite Óptica , Masculino , Feminino , Humanos , Aquaporina 4 , Estudos Retrospectivos , Imunoglobulina G , Glicoproteína Mielina-Oligodendrócito , Autoanticorpos/líquido cefalorraquidiano
3.
Langmuir ; 38(20): 6281-6294, 2022 05 24.
Artigo em Inglês | MEDLINE | ID: mdl-35549265

RESUMO

Cellular energy required for the maintenance of cellular life is stored in the form of adenosine triphosphate (ATP). Understanding cellular mechanisms, including ATP-dependent metabolisms, is crucial for disease diagnosis and treatment, including drug development and investigation of new therapeutic systems. As an ATP-dependent metabolism, endocytosis plays a key role not only in the internalization of molecules but also in processes including cell growth, differentiation, and signaling. To understand cellular mechanisms including endocytosis, many techniques ranging from molecular approaches to spectroscopy are used. Surface-enhanced Raman scattering (SERS) is shown to provide valuable label-free molecular information from living cells. In this study, receptor-mediated endocytosis was investigated with SERS by inhibiting endocytosis with ATP depletion agents: sodium azide (NaN3) and 2-deoxy-d-glucose (dG). Human lung bronchial epithelium (Beas-2b) cells, normal prostate epithelium (PNT1A) cells, and cervical cancer epithelium (HeLa) cells were used as models. First, the effect of NaN3 and dG on the cells were examined through cytotoxicity, apoptosis-necrosis, ATP assay, and uptake inhibition analysis. An attempt to relate the spectral changes in the cellular spectra to the studied cellular events, receptor-mediated endocytosis inhibition, was made. It was found that the effect of two different ATP depletion agents can be discriminated by SERS, and hence receptor-mediated endocytosis can be tracked from single living cells with the technique without using a label and with limited sample preparation.


Assuntos
Endocitose , Análise Espectral Raman , Trifosfato de Adenosina/metabolismo , Células HeLa , Humanos , Masculino , Azida Sódica/farmacologia , Análise Espectral Raman/métodos
4.
Turk J Pediatr ; 58(2): 136-144, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-27976553

RESUMO

Beginning school is an important milestone for children. Children's readiness for school involves cognitive, physical, and emotional development. Certain school programs allow children to start first grade after 66 months of age, together with 72 month-old children. In order to estimate school readiness, we screened children before starting first grade and compared their school performance according to their age and socio-demographic characteristics. Marmara School Readiness, Denver II developmental screening, and language assessment tests were applied. Language delays were more frequent and school readiness test scores were lower in the younger group compared to older children. However, school achievement did not differ between the two age groups. Preschool education, parental income and education affected performance in most tests. Preschool screening seems effective in detecting children with lower than average developmental skills, and the school system may provide a practical opportunity for providing support to those children.


Assuntos
Deficiências do Desenvolvimento/epidemiologia , Transtornos do Desenvolvimento da Linguagem/epidemiologia , Programas de Rastreamento/métodos , Criança , Desenvolvimento Infantil , Pré-Escolar , Feminino , Humanos , Idioma , Masculino , Instituições Acadêmicas
5.
Arch Med Sci ; 12(4): 697-703, 2016 Aug 01.
Artigo em Inglês | MEDLINE | ID: mdl-27478448

RESUMO

INTRODUCTION: In this study we aimed to detect paraoxonase 1 (PON-1) activity in iron deficiency anemia (IDA) and to compare it with healthy controls by observing the change after iron therapy. MATERIAL AND METHODS: In this study, 50 adult patients with IDA and 40 healthy subjects were enrolled. All patients were analyzed at the beginning and after treatment according to laboratory assessments. RESULTS: Mean paraoxonase and arylesterase activities in the iron deficiency anemia group were significantly lower than mean activities of the control group (102.4 ±19.2 U/l and 163.3 ±13.68 U/l, respectively and 157.3 ±26.4 U/l and 256.1 ±24.6 U/l, respectively; p = 0.0001 for both). Paraoxonase and arylesterase activities significantly increased after treatment for IDA (143.2 ±13.9 and 197.6 ±27.9 U/l, respectively, p = 0.0001). Mean activities after treatment with iron were significantly lower than mean activities in the control group (p = 0.002; p = 0.0001 respectively). CONCLUSIONS: Paraoxonase and arylesterase activities in patients with IDA significantly increased after treatment with iron therapy. In adults IDA may also be one of the factors associated with increased risk of atherosclerosis.

7.
Ren Fail ; 38(5): 781-6, 2016 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-27050633

RESUMO

OBJECTIVE: Altered paraoxonase (PON) and arylesterase (ARE) activities have been shown in anemic chronic kidney disease (CKD) patients and in iron deficiency anemia (IDA) patients. Whether accompanying anemia alone is responsible for this diminished PON and ARE activities in CKD patients or an additive factor for this is not well studied. Therefore, we tried to clarify this issue here. METHODS: A total of 82 subjects that consisted of 19 patients with IDA (group 1), 23 anemic CKD patients (group 2), and 40 age and sex matched healthy subjects (group 3) were enrolled. Carotid intima media thickness (CIMT), serum total thiol (-SH), PON, and ARE activities of the participants were analyzed. RESULTS: Group 2 patients had significantly lowest serum levels of Total -SH, PON and ARE. Further comparison showed that total -SH, PON and ARE levels were lower in group 1 than group 3 (p = 0.0001 in both). Regarding comparison of group 1 and 2, only serum ARE levels were significantly lower in group 2 (p = 0.001). PON activity was not different between group 1 and group 2 whereas ARE activity was lower in group 2 than groups 1 and 3. In addition, correlation analysis showed that CIMT was negatively correlated with PON and ARE. CONCLUSIONS: This markedly decreased ARE activity in CKD patients, which could not be explained by the anemia alone, may have a role in the pathogenesis of increased atherosclerosis in such patients. Still further studies are needed to certain this.


Assuntos
Anemia Hipocrômica , Anemia Ferropriva , Arildialquilfosfatase/metabolismo , Hidrolases de Éster Carboxílico/metabolismo , Insuficiência Renal Crônica/complicações , Adulto , Anemia Hipocrômica/diagnóstico , Anemia Hipocrômica/etiologia , Anemia Hipocrômica/metabolismo , Anemia Ferropriva/diagnóstico , Anemia Ferropriva/metabolismo , Feminino , Humanos , Masculino , Reprodutibilidade dos Testes , Estatística como Assunto
8.
J Child Neurol ; 31(7): 929-31, 2016 06.
Artigo em Inglês | MEDLINE | ID: mdl-26887413

RESUMO

To determine the prevalence of celiac disease in children and adolescents with migraine, the authors investigated serum levels of tissue transglutaminase antibody immunoglobulin A and total immunoglobulin A from 81 children with migraine and in a healthy control group of 176 children. Study participants who were positive for tissue transglutaminase immunoglobulin A antibodies underwent a duodenal biopsy. Two patients in the migraine group (2.5%) and 1 in the control group (0.57%) tested positive for serum tissue transglutaminase immunoglobulin A antibodies (P > .05). Duodenal biopsy did not confirm celiac disease in both groups, and these patients were considered "potential celiac" cases. In the present study, children with migraine did not exhibit a higher prevalence rate of celiac disease compared with healthy controls. Therefore, the screening test for celiac disease is not a necessary part of the management of migraine in children.


Assuntos
Doença Celíaca/sangue , Doença Celíaca/complicações , Transtornos de Enxaqueca/sangue , Transtornos de Enxaqueca/etiologia , Adolescente , Autoanticorpos/sangue , Biomarcadores/sangue , Doença Celíaca/diagnóstico , Doença Celíaca/epidemiologia , Criança , Estudos de Coortes , Duodeno/patologia , Feminino , Humanos , Imunoglobulina A/sangue , Masculino , Transtornos de Enxaqueca/epidemiologia , Prevalência , Transglutaminases/sangue , Transglutaminases/imunologia
9.
Respiration ; 90(3): 191-8, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-26137891

RESUMO

BACKGROUND: Tumor necrosis factor (TNF)-α inhibitors are known to increase the risk of tuberculosis (TB). OBJECTIVES: To examine the factors associated with an increased risk of TB in patients receiving anti-TNF-α treatment (aTNF-α-T). METHOD: Of 3,094 patients who received aTNF-α-T between 2003 and 2013, a total of 1,964 subjects with a follow-up time longer than 6 months were identified and included in this retrospective analysis. Potential risk factors for the development of TB in patients receiving aTNF-α-T were evaluated. RESULTS: Of the 1,964 patients, 1,009 (51%) were male and 955 (49%) were female, with a mean age of 39.7 ± 13.9 years. The primary conditions requiring aTNF-α-T included ankylosing spondylitis (n = 875), rheumatoid arthritis (n = 711), Behçet's disease (n = 83), and others (n = 295). Sixteen patients [8 (50%) males and 8 (50%) females; 5 (31.2%) with pulmonary TB and 11 (68.8%) with extrapulmonary TB] developed TB, with a corresponding TB incidence of 466/100,000. No significant associations were found between age, gender, smoking history, pack-years of smoking, isoniazid (INH) chemoprophylaxis, type of anti-TNF-α agent, use of other immunosuppressive drugs, and the risk of TB (p > 0.05). Multivariate logistic regression analysis showed a significantly higher risk of TB in patients diagnosed with Behçet's disease, and a significantly lower risk of TB in patients with a tuberculin skin test wheal ≥10 mm in diameter (p < 0.05). CONCLUSION: aTNF-α-T is associated with an increased risk of pulmonary or extrapulmonary TB, even when follow-up protocols and INH chemoprophylaxis are implemented, and TB often develops in the later stages of treatment. The risk of TB was higher in patients with Behçet's disease and lower in patients who had a strong tuberculin skin test reaction.


Assuntos
Artrite Reumatoide/tratamento farmacológico , Síndrome de Behçet/tratamento farmacológico , Espondilite Anquilosante/tratamento farmacológico , Tuberculose/induzido quimicamente , Fator de Necrose Tumoral alfa/antagonistas & inibidores , Adulto , Distribuição por Idade , Animais , Estudos de Coortes , Modelos Animais de Doenças , Feminino , Seguimentos , Humanos , Hospedeiro Imunocomprometido , Incidência , Modelos Logísticos , Masculino , Camundongos , Pessoa de Meia-Idade , Análise Multivariada , Estudos Retrospectivos , Medição de Risco , Distribuição por Sexo , Tuberculose/epidemiologia , Tuberculose/imunologia , Fator de Necrose Tumoral alfa/efeitos adversos
10.
J Craniomaxillofac Surg ; 43(7): 1059-64, 2015 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-26116303

RESUMO

OBJECTIVE: The aim of this study was to evaluate the effect of climate and altitude differences on the volume of paranasal sinuses and on the frequency of anatomic variations by comparing the paranasal sinus tomograms (PNSCT) of patients who were born and living in a cold, dry climate at high altitude with those of patients who were born and living on the coast at sea level in a temperate climate. We also aimed to determine differences relating to gender. MATERIAL AND METHODS: A total of 55 PNSCTs of 55 patients from the city center of Antalya and 60 PNSCTs of 60 patients from the city center of Agri were evaluated and compared prospectively. The study included a total of 115 patients with a mean age of 44.75 ± 9.64 years (range, 27-63 years). Group 1 (Antalya) comprised 26 females (47.3%) and 29 males (52.7%) with a mean age of 36.7 ± 12.4 years. Group 2 (Agri) comprised 25 females (41.7%) and 35 males (58.3%) with a mean age of 35.1 ± 13.4 years. Maxillary sinus volumes were 18.27 cm(3) (range, 5.04-37.62) and 15.06 cm(3) (4.11-41.40); sphenoid sinus volumes were 7.81 cm(3) (1.80-20.63) and 6.35 cm(3) (0.54-16.50); frontal sinus volumes were 5.51 cm(3) (0.50-29.25) and 3.76 cm(3) (0.68-22.81) respectively. RESULTS: There was no statistically significant difference between the groups in term of volumes (p > 0.025). Both maxillary and frontal sinus volumes were greater in males compared to females (p < 0.025). The mean value of the maxillary sinus volume was 15.7 ± 5.3 cm(3) and was significantly larger in males than in females (p = 0.004). There was no statistically significant correlation between the volume of maxillary sinuses with age or side. There was no statistically significant difference between the groups in terms of septum deviation and concha bullosa rates (p = 0.469 and p = 0.388). CONCLUSION: There have been many studies of nasal cavity changes due to climatic conditions but this is the first study to measure the difference of paranasal sinus volumes. No difference was determined in the anatomic variations and volumes of the maxillary, frontal, sphenoid sinuses on PNSCT of patients from different climates and altitudes.


Assuntos
Altitude , Clima , Seios Paranasais/anatomia & histologia , Adulto , Variação Anatômica , Clima Frio , Feminino , Seio Frontal/anatomia & histologia , Seio Frontal/diagnóstico por imagem , Humanos , Masculino , Seio Maxilar/anatomia & histologia , Seio Maxilar/diagnóstico por imagem , Pessoa de Meia-Idade , Septo Nasal/anatomia & histologia , Septo Nasal/diagnóstico por imagem , Oceanos e Mares , Tamanho do Órgão , Seios Paranasais/diagnóstico por imagem , Estudos Prospectivos , Fatores Sexuais , Seio Esfenoidal/anatomia & histologia , Seio Esfenoidal/diagnóstico por imagem , Tomografia/métodos , Conchas Nasais/anatomia & histologia , Conchas Nasais/diagnóstico por imagem , Turquia , Adulto Jovem
11.
Int J Surg Case Rep ; 5(12): 972-4, 2014.
Artigo em Inglês | MEDLINE | ID: mdl-25460450

RESUMO

INTRODUCTION: Testicular cancer has high cure rates, especially after the adjuvant use of chemotherapy. Secondary tumors may develop months and years after the primary tumor. We aimed to report a case of cutaneous Ewing's sarcoma at the site of surgery 3 years after BEP chemotherapy. PRESENTATION OF CASE: 21 year old male underwent radical orchiectomy in 2008. After one year surgical site complaints brought him to same hospital. A limited surgical resection was made. As his complaints continued he applied to our clinic. We resected the lesion with a 5cm safety margin with the light of previous medical history. Pathology revealed cutaneous Ewing's sarcoma, and patient received VACD-IE chemotherapy. He is free of recurrence till now. DISCUSSION: Chemotherapy may cause secondary cancer especially in long term. In this case secondary tumor is diagnosed three years after surgery. Patient underwent therapeutic surgery and received chemotherapy (VACD-IE) for secondary Ewing's sarcoma. Early diagnosis and definitive treatment provide recurrence free survival in the patient. CONCLUSION: Secondary tumors can emerge months or years after primary tumor therapies, and are not related with the primary tumors. Any lesion or sign should be investigated carefully. Early diagnosis and correct treatment could prevent dramatic results.

12.
J Coll Physicians Surg Pak ; 24 Suppl 1: S46-7, 2014 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-24718004

RESUMO

Schwannomas are solitary benign tumours derived from peripheral nerve sheath that are difficult to diagnose using imaging only. Diagnoses are commonly confirmed with conventional surgical resection. Small proportion of cases are formed by cellular schwannoma usually seen at paravertebral, pelvic, retroperitoneal, or mediastinal location. This patient had a 3 x 2 cm hard mass at the dorsal side of proximal phalanx of the right third toe. Bone destruction was seen on radiography and MR imaging. Curettage after excision and grafting were performed. No complication was seen after surgery. The histopathology result was reported as cellular schwannoma. It was an atypical location for cellular schwannoma not previously described in literature.


Assuntos
Neoplasias Ósseas/diagnóstico , Neurilemoma/diagnóstico , Falanges dos Dedos do Pé/diagnóstico por imagem , Neoplasias Ósseas/cirurgia , Humanos , Masculino , Neurilemoma/cirurgia , Radiografia , Resultado do Tratamento , Adulto Jovem
13.
Pediatr Hematol Oncol ; 31(4): 318-26, 2014 May.
Artigo em Inglês | MEDLINE | ID: mdl-23988130

RESUMO

Hyperleukocytosis in patients with leukemia is associated with early mortality, especially due to the pulmonary and neurological complications of leukostasis. The prompt use of leukapheresis may improve patients' survival in the initial treatment period. The medical records of all previously untreated acute leukemia patients were reviewed to determine whether there was hyperleukocytosis at presentation. This study summarizes a single-center experience of leukapheresis that was applied to 12 children with acute leukemia and hyperleukocytosis. The median leukocyte count at diagnosis was 589,000/mm(3) (range: 389,000-942,000/mm(3)) for ALL patients and 232,000/mm(3) (range: 200,000-282,000/mm(3)) for AML patients. A central venous catheter (CVC) was inserted, and leukapheresis procedures were repeated at 12-hour intervals. A total of 29 leukapheresis cycles were performed on 12 children. The median number of cycles of leukapheresis required by each patient was two (range: 1-4). The median absolute and percentage reductions in white blood cell (WBC) count after the first cycle of leukapheresis were 113,000/mm(3) (range: 55,000-442,000/mm(3)) and 36% (range: 16-57.4%), respectively. As a laboratory finding, mild hypocalcemia was the most frequently observed complication. No patients developed any other problem related to the procedure. Our results showed that leukapheresis is a safe and effective procedure if performed by experienced staff.


Assuntos
Leucaférese/métodos , Leucocitose/terapia , Leucemia-Linfoma Linfoblástico de Células Precursoras/terapia , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Hipocalcemia/sangue , Hipocalcemia/etiologia , Contagem de Leucócitos , Leucocitose/sangue , Leucocitose/mortalidade , Masculino , Leucemia-Linfoma Linfoblástico de Células Precursoras/sangue , Leucemia-Linfoma Linfoblástico de Células Precursoras/mortalidade , Estudos Retrospectivos
14.
Am J Case Rep ; 14: 419-23, 2013.
Artigo em Inglês | MEDLINE | ID: mdl-24349604

RESUMO

PATIENT: Female, 58 FINAL DIAGNOSIS: Solitary paraganglioma of the hypoglossal nerve Symptoms: Neck pain Medication: - Clinical Procedure: Surgical resection Specialty: Otolaryngology. OBJECTIVE: Rare disease. BACKGROUND: Paragangliomas are rare neuroendocrine tumors originating in the neural crest. Only a few cases of hypoglossal paraganglioma have been reported in the published literature. The localization of hypoglossal paragangliomas close to the carotid artery precludes determination of tumor origin preoperatively. CASE REPORT: A 58-year-old female patient was admitted due to neck pain. During physical examination, a significant mass could not be palpated in the upper left part of the neck, despite sensitivity during palpation. Atrophy and left deviation of the left half of the tongue was observed. MRI of the neck revealed a lesion located superior to the carotid bifurcation between the left internal carotid artery and external carotid artery. There was atrophy in the left half of the tongue. The neck mass displaced the left internal carotid artery anteriorly and medially. The operation was performed with left lateral cervical access. This lesion, which derived from the hypoglossal nerve, was excised. Following histopathological evaluation, the lesion was diagnosed as paraganglioma. CONCLUSIONS: Hypoglossal paraganglioma is quite rare and there are no established criteria for preoperative diagnosis. Hypoglossal paraganglioma must be considered to determine treatment options if a lateral neck mass and ipsilateral tongue atrophy are present at the level of the 12th cranial nerve tract.

15.
Ren Fail ; 35(8): 1163-6, 2013 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-23885711

RESUMO

Intravascular lymphoma (IVL) is a rare extra nodal subtype (usually of B-cell origin) presenting with infiltration of large neoplastic lymphocytes into lumina of blood vessels, leading to vascular occlusion. The early diagnosis is very crucial, however it is usually diagnosed postmortem investigation in most of the cases. A 56-year-old female presented with elevated creatinine level, and anasarca-type edema that superimposed with hard, indurated, erythematous plaques extending to inguinal region, abdomen, anterior aspect of chest, and face. B-cell IVL was confirmed with skin biopsy. The patient had some degree of clinical improvement following chemotherapy. B-cell IVL presenting with anasarca edema was not previously reported in the literature. Even if its rarity, IVL should be considered in the differential diagnosis of renal failure with anasarca edema.


Assuntos
Injúria Renal Aguda/etiologia , Edema/etiologia , Linfoma Difuso de Grandes Células B/patologia , Neoplasias Cutâneas/patologia , Neoplasias Vasculares/patologia , Injúria Renal Aguda/patologia , Edema/patologia , Feminino , Humanos , Linfoma Difuso de Grandes Células B/complicações , Pessoa de Meia-Idade , Neoplasias Cutâneas/complicações , Neoplasias Vasculares/complicações
16.
J Craniomaxillofac Surg ; 41(3): 242-4, 2013 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-23127544

RESUMO

The nasal hamartoma is a rare non-neoplastic malformation or inborn error of tissue development, characterized by a mix of several tissue types. Folliculosebaceous cystic hamartoma is a distinct type of cutaneous hamartoma. We present a 3-month-old male infant who had nasal obstruction and who had a diagnosis of nasal folliculosebaceous cystic hamartoma after surgery.


Assuntos
Hamartoma/diagnóstico , Doenças Nasais/diagnóstico , Adipócitos/patologia , Diagnóstico Diferencial , Seguimentos , Folículo Piloso/patologia , Humanos , Lactente , Masculino , Mesoderma/patologia , Obstrução Nasal/diagnóstico , Glândulas Sebáceas/patologia , Vênulas/patologia
17.
Case Rep Oncol ; 4(2): 323-6, 2011 May.
Artigo em Inglês | MEDLINE | ID: mdl-21738503

RESUMO

BCR-ABL fusion gene t(9;22)(q34;q11) occurs in only 3% of pediatric acute lymphoblastic leukemia (ALL) cases. Previously, less than 40% of Philadelphia-positive ALL patients were cured with intensive chemotherapy. The use of imatinib (340 mg/m(2)/day) added to an intensive chemotherapy regimen has improved the outcome in this population at 3 years to an event-free survival of 80%. Imatinib treatment alone was administered after remission induction chemotherapy to a patient with Philadelphia-positive ALL who presented with serious chemotherapy toxicity, so that intensive chemotherapy could not be maintained. This is the only patient in the literature who survived remission for more than 2.5 years with imatinib treatment only.

18.
Mycoses ; 54(3): 234-42, 2011 May.
Artigo em Inglês | MEDLINE | ID: mdl-19906090

RESUMO

Combination treatment of paediatric invasive fungal infections (IFIs) has rarely been reported. A total of 17 children with 19 IFI episodes were enrolled in the study. The median age of the patients was 5.3 (range 0.5-17) years. IFI was classified as proven in 4, probable in 12 and possible in 3 episodes. These patients received empiric antifungal treatment, which consisted of liposomal amphotericin B (LAmB) monotherapy for a median duration of 12 days (range 3-69 days). All patients were refractory to LAmB; therefore, caspofungin was added to the therapy in 11 patients. In the remaining six patients, LAmB was ceased and a combination of caspofungin and voriconazole was started. Among the patients who received caspofungin + LAmB, four did not show favourable response and the combination was switched to caspofungin + voriconazole. The median (range) and total duration of the therapy were 7 (3-14) days and 91 patient days for LAmB + caspofungin combination and 49 (7-126) days and 516 patient days for caspofungin + voriconazole combination. We found a favourable response rate of 68.4% in 16 proven or probable IFI episodes. Twelve-week survival rate of these patients was 75%. No serious side effect was observed among the patients. Our data suggest that combination antifungal therapy is safe and effective in children with haematological malignancies.


Assuntos
Anfotericina B/administração & dosagem , Farmacorresistência Fúngica , Equinocandinas/administração & dosagem , Neoplasias Hematológicas/complicações , Micoses/tratamento farmacológico , Pirimidinas/administração & dosagem , Terapia de Salvação/métodos , Triazóis/administração & dosagem , Adolescente , Anfotericina B/efeitos adversos , Antifúngicos/administração & dosagem , Antifúngicos/efeitos adversos , Caspofungina , Criança , Pré-Escolar , Quimioterapia Combinada/efeitos adversos , Quimioterapia Combinada/métodos , Equinocandinas/efeitos adversos , Feminino , Fungos/efeitos dos fármacos , Humanos , Lactente , Lipopeptídeos , Masculino , Pirimidinas/efeitos adversos , Terapia de Salvação/efeitos adversos , Resultado do Tratamento , Triazóis/efeitos adversos , Voriconazol
19.
Pediatr Neurol ; 41(3): 179-82, 2009 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-19664532

RESUMO

Oxidative damage plays a role in neurodegenerative diseases. Levels of cerebrospinal fluid nitrite and nitrate levels (oxidation products that provide an indirect estimation of nitric oxide) were investigated in relation to clinical and laboratory features in subacute sclerosing panencephalitis (n = 47) and age-matched control (n = 43) groups. Significantly decreased levels of nitrite (median, 4.91 micromol/L) and nitrate (median, 6.14 micromol/L) were found in the patients. Nitrite and nitrate levels did not correlate with clinical or laboratory findings, except for presence of myoclonus. Cerebrospinal fluid nitrite levels of subacute sclerosing panencephalitis patients without myoclonic jerks were significantly higher than in those with myoclonus (median, 15.63 vs 4.34 micromol/L, respectively). The higher levels of nitrite in these patients can be explained by short disease duration and early stages of disease. Nitrate levels in subacute sclerosing panencephalitis patients with myoclonus (median, 9.26 micromol/L) were higher than in those without myoclonus (median, 4.25 micromol/L). Microbleeding resulting in conversion of nitrite to nitrate and increased production of superoxide can be suggested as possible mechanisms underlying these findings.


Assuntos
Mioclonia/líquido cefalorraquidiano , Nitratos/líquido cefalorraquidiano , Óxido Nítrico/líquido cefalorraquidiano , Nitritos/líquido cefalorraquidiano , Panencefalite Esclerosante Subaguda/líquido cefalorraquidiano , Análise de Variância , Criança , Feminino , Febre/líquido cefalorraquidiano , Humanos , Imunoglobulinas/líquido cefalorraquidiano , Hipertensão Intracraniana/líquido cefalorraquidiano , Leucemia/líquido cefalorraquidiano , Masculino , Sarampo/imunologia , Índice de Gravidade de Doença , Fatores de Tempo
20.
Biol Blood Marrow Transplant ; 15(7): 817-26, 2009 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-19539213

RESUMO

Pulmonary complications (PC) remain a significant barrier to the success of allogeneic blood and marrow transplantation (BMT). Pretransplant pulmonary function tests (PFTs) have been correlated with risk of early respiratory failure and mortality in adult BMT recipients. There is limited data on their relationship to posttransplant outcomes in pediatric patients. We sought, in pediatric allo-BMT recipients (1) to analyze the spectrum of infectious and noninfectious PCs, (2) to evaluate the prevalence and course of PFT abnormalities before and after transplant, and (3) to correlate pretransplant PFT findings with patient outcomes, specifically risk of PC, respiratory failure, and death. We conducted a retrospective review of PC in all patients who underwent allo-BMT at Children's Hospital of Pittsburgh during 1996 to 2006. PFTs were performed in children 6 years and older pretransplant, 3, 6, 12, and 24 months after transplant. PCs occurring within 100 days of BMT were considered early. One hundred ten consecutive children who underwent allo-BMT were included (median age = 9.7 years; 67 males, 43 females). Seventy-five of 110 patients had 370 PFT studies performed; 62 of 73 patients >6 years of age (85%) underwent PFT studies pre-BMT. There was a significantly higher risk of early respiratory failure in patients with reduced pretransplant forced expiratory volume in 1 second (FEV(1)) (P = .0001, odds ratio [OR] 5.1) or forced vital capacity (FVC) (P = .0001, OR 8.5). Forty-three of 110 (39%) patients required mechanical ventilation, and in 30 episodes (70%), patients remained ventilator-dependent until time of death. Posttransplant, we observed statistically significant reductions in FEV(1), FVC, total lung capacity (TLC), and diffusing capacity of the lungs (DLco) at 3 months post-BMT and similar reductions at 6 months post-BMT except for DLco (not significant). Between 12 and 24 months, FEV(1), FVC, TLC, and DLco improved significantly from earlier declines post-BMT; however FEV(1) and FVC remained significantly below pretransplant values. At a median follow-up of 5.5 (1.6-11.6) years, 58 of 110 (53%) patients were surviving. The majority of the patients who died from transplant-related complications suffered from 1 or more PCs (31/32, 97%). Early PC was associated with over 4-fold reduction in probability of survival at 10 years (8/44, 18% with early PC versus 50/66, 76% without early PC). On multivariate analysis, risk of death was significantly associated with high-risk disease status (P = .015; hazard ratio [HR] = 2.5), unrelated donor (P = .03; HR = 2.1), early PC (P = .0001; HR = 7.7) and pathogen identification (P = .02; HR = 2.7). These results suggest that, in children undergoing allo-BMT (1) compromised pretransplant lung function is significantly correlated with risk of early respiratory failure but not of overall survival (OS), (2) reductions in lung volumes and diffusion capacity are common 3- to 6-month posttransplant with partial recovery by 12 to 24 months, (3) there is high mortality following mechanical ventilation, and (4) early PCs are associated with significantly worse OS.


Assuntos
Transplante de Medula Óssea , Insuficiência Respiratória/mortalidade , Doadores de Tecidos , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Testes de Função Respiratória , Insuficiência Respiratória/etiologia , Insuficiência Respiratória/fisiopatologia , Estudos Retrospectivos , Fatores de Risco , Fatores de Tempo , Transplante Homólogo
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