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1.
Inflammation ; 45(2): 627-638, 2022 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-34637032

RESUMO

We investigated plasma YKL-40 levels and chitotriosidase (CHIT1) activity in patients with cystic fibrosis (CF) lung disease and evaluated clinically relevant factors that may affect their levels. Plasma samples were obtained from pediatric (n = 19) and adult patients (n = 15) during exacerbation, discharge, and stable period of the disease. YKL-40 levels and chitotriosidase activity were measured by enzyme-linked immunosorbent assay and fluorometric assay, respectively. Data were compared with healthy children and adults of similar age. YKL-40 levels of pediatric and adult CF patients at all periods were significantly higher than controls (p < 0.001 and p < 0.05). CHIT1 activities of adult patients at all periods were significantly higher compared to controls (p < 0.05). On the other hand, CHIT1 activities of pediatric CF patients were similar with controls. YKL-40 levels of exacerbation period of adult CF patients were negatively correlated with forced vital capacity (FVC) (r = - 0.800, p = 0.014) and forced expiratory volume in 1 s (FEV1) (r = - 0.735, p = 0.008). YKL-40 levels in the exacerbation period of pediatric CF patients were negatively correlated with FVC (r = - 0.697, p = 0.0082) and FEV1 (r = - 0.720, p = 0.006). CHIT1 activity may be a valuable marker of chronic inflammation in adult CF patients who suffer from CF for a longer period compared to pediatric patients. Increased YKL-40 levels in both pediatric and adult patients compared to controls may point to a role in between CF pathology.


Assuntos
Fibrose Cística , Adulto , Criança , Proteína 1 Semelhante à Quitinase-3 , Hexosaminidases , Humanos , Testes de Função Respiratória
2.
Turk J Pediatr ; 62(1): 156-159, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32253884

RESUMO

Primary ciliary dyskinesia (PCD) is a rare, genetic disease characterized by ciliary dysfunction. Patients may present with respiratory distress during neonatal period; chronic sinopulmonary disease, bronchiectasis, recurrent otitis media, sinusitis and infertility in later periods. About 50% of PCD patients have situs inversus totalis and 6-12% have situs ambiguous known as heterotaxy syndromes. Herein, we present a case of PCD and accompanying situs inversus who had acute abdominal pain and was diagnosed with torsion of one of the multiple spleens. Evaluation of acute abdominal pain in these patients has great importance since the internal organs are not at their typical locations.


Assuntos
Síndrome de Kartagener , Otite Média , Sinusite , Situs Inversus , Dor Abdominal/diagnóstico , Dor Abdominal/etiologia , Humanos , Recém-Nascido , Síndrome de Kartagener/complicações , Síndrome de Kartagener/diagnóstico , Situs Inversus/complicações , Situs Inversus/diagnóstico
3.
Lipids ; 55(3): 213-223, 2020 05.
Artigo em Inglês | MEDLINE | ID: mdl-32120452

RESUMO

We investigated plasma sphingomyelin (CerPCho) and ceramide (Cer) levels in pediatric patients with cystic fibrosis (CF) and primary ciliary dyskinesia (PCD). Plasma samples were obtained from CF (n = 19) and PCD (n = 7) patients at exacerbation, discharge, and stable periods. Healthy children (n = 17) of similar age served as control. Levels of 16-24 CerPCho and 16-24 Cer were measured by LC-MS/MS. Concentrations of all CerPCho and Cer species measured at exacerbation were significantly lower in patients with CF than PCD. 16, 18, 24 CerPCho, and 22, 24 Cer in exacerbation; 18, 24 CerPCho, and 18, 20, 22, 24 Cer at discharge; 18, 24 CerPCho and 24 Cer at stable period were significantly lower in CF patients than healthy children (p < 0.001 and p < 0.05). All CerPCho and Cer levels of PCD patients were significantly higher except 24 CerPCho and 24 Cer during exacerbation, 24 CerPCho at discharge, and 18, 22 CerPCho levels at stable period (p < 0.001 and p < 0.05) compared with healthy children. There was no significant difference among exacerbation, discharge, and stable periods in each group for Cer and CerPCho levels. This is the first study measuring plasma Cer and CerPCho levels in PCD and third study in CF patients. The dramatic difference in plasma levels of most CerPCho and Cer species found between two diseases suggest that cilia pathology in PCD and CFTR mutation in CF seem to alter sphingolipid metabolism possibly in opposite directions.


Assuntos
Ceramidas/sangue , Transtornos da Motilidade Ciliar/sangue , Transtornos da Motilidade Ciliar/genética , Fibrose Cística/sangue , Esfingomielinas/sangue , Adolescente , Estudos de Casos e Controles , Criança , Cromatografia Líquida , Fibrose Cística/genética , Regulador de Condutância Transmembrana em Fibrose Cística/genética , Feminino , Humanos , Masculino , Proteínas Associadas aos Microtúbulos/genética , Mutação , Estudos Prospectivos , Espectrometria de Massas em Tandem
4.
J Trop Pediatr ; 62(4): 324-7, 2016 08.
Artigo em Inglês | MEDLINE | ID: mdl-27080471

RESUMO

Gastroesophageal disorders such as achalasia can be associated with pulmonary disorders because of non-tuberculous mycobacteria, frequently masquerading as aspiration pneumonia. The optimal therapeutic regimen and duration of treatment for non-tuberculous mycobacteria lung disease is not well established. Here, we present an 11 year old male patient with Mycobacterium abscessus pulmonary disease and underlying triple A syndrome, who was successfully treated with 2 months of imipenem, amikacin, clarithromycin and continued for long-term antibiotic treatment.


Assuntos
Insuficiência Adrenal/complicações , Antibacterianos/uso terapêutico , Acalasia Esofágica/complicações , Infecções por Mycobacterium não Tuberculosas/tratamento farmacológico , Micobactérias não Tuberculosas/isolamento & purificação , Amicacina/uso terapêutico , Criança , Claritromicina/uso terapêutico , Humanos , Imipenem/uso terapêutico , Masculino , Infecções por Mycobacterium não Tuberculosas/patologia , Resultado do Tratamento
5.
Clin Respir J ; 10(2): 153-62, 2016 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-25069394

RESUMO

BACKGROUND AND AIMS: Thalassemia major (TM) is characterized by abnormal hemoglobin synthesis, which results in decreased oxygen delivery to the tissues, ineffective erythropoiesis and iron overload. The purpose of this study was to find out the predominant type of lung mechanical abnormalities in TM patients, the prevalence of the change in pulmonary diffusing capacity and to search the association of cytokines with pulmonary function tests (PFTs). METHODS: TM patients (n = 26) who were on regular transfusion program were included in the study. Pretransfusional and posttransfusional PFTs were performed. Before transfusion, blood samples were collected for complete blood count, serum ferritin, zinc, transforming growth factor-ß1 (TGF-ß1), interleukin (IL)-8 and IL-10 levels. RESULTS: Obstructive lung pattern was the predominant pulmonary sequela. Male gender was the main risk factor for pulmonary function abnormality. Because of very low values, serum IL-10 levels of all patients were undetectable, and serum IL-8 levels could be measured only in six patients. Median serum IL-8 level was higher in patients with abnormal PFT. Pretransfusional single breath diffusion of carbon monoxide (DLCO ) correlated negatively with serum TGF-ß1 and ferritin levels. After transfusion, there was a statistically significant decrease in forced expiratory volume in 1 s, forced expiratory flow between 25% and 75% of vital capacity, and airway conductance but significant increase in DLCO . CONCLUSIONS: Although they are mostly asymptomatic, TM patients have important PFT abnormalities. Blood transfusion may have an acute deleterious effect on pulmonary functions. The disturbed pulmonary functions were found to correlate with IL-8 and TGF-ß levels. Relations of different cytokines with different PFT parameters suggest that the immune system is effective pulmonary dysfunction.


Assuntos
Transfusão de Sangue , Citocinas/sangue , Pulmão/fisiopatologia , Talassemia beta/terapia , Adolescente , Criança , Feminino , Ferritinas/sangue , Humanos , Interleucina-10/sangue , Interleucina-8/sangue , Masculino , Testes de Função Respiratória , Fator de Crescimento Transformador beta1/sangue , Adulto Jovem , Talassemia beta/sangue , Talassemia beta/imunologia , Talassemia beta/fisiopatologia
6.
Pediatr Pulmonol ; 49(8): 829-33, 2014 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-24574160

RESUMO

Pulmonary hemangioma is a rare benign tumor of the lungs. Airway hemangiomas in particular may lead to feeding difficulties, barking cough, stridor, respiratory distress, and even acute airway obstruction. Hence, such hemangiomas usually require early and aggressive treatment; however, the treatment modalities employed so far have been associated with their own co-morbidities and potential long-term adverse effects for the developing child. Here, we report a case of 9-month-old girl who presented with dyspnea and diagnosed pulmonary hemangioma in the lower lobe of left lung. Propranolol is now used as a first line treatment for infantile hemangiomas by many practitioners so we experienced propranolol treatment. This case report argues the first case of pulmonary hemangioma treated with propranolol successfully.


Assuntos
Antagonistas Adrenérgicos beta/uso terapêutico , Hemangioma/tratamento farmacológico , Neoplasias Pulmonares/tratamento farmacológico , Propranolol/uso terapêutico , Feminino , Hemangioma/diagnóstico por imagem , Humanos , Lactente , Neoplasias Pulmonares/diagnóstico por imagem , Radiografia , Resultado do Tratamento
7.
Respir Med ; 106(9): 1278-85, 2012 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-22784405

RESUMO

BACKGROUND: Niemann-Pick disease (NPD) and Gaucher disease (GD) are well-known lysosomal storage diseases. Respiratory system involvement is an important cause of morbidity and mortality in patients with NPD and GD. OBJECTIVES: We tried to assess the clinical, radiological, and histological features of GD and NPD patients with lung involvement. METHODS: We reviewed medical history, physical examination, radiological, and histological data of 10 NPD and 7 GD patients. RESULTS: The most common respiratory symptoms were recurrent lung infection and dyspnea. Although lung examination results in 6 NPD patients were normal, they had lung involvement; 3 patients were diagnosed as NPD directly via lung biopsy during investigation of recurrent lung infection or interstitial lung disease. All GD patients but 1 had respiratory system symptoms at the time of diagnosis. Hepatopulmonary syndrome was present in 4 GD patients. A ground-glass pattern and atelectasis were 2 important high-resolution computed tomography features in the NPD and GD patients. Flexible bronchoscopy and bronchoalveolar lavage were used for emergency extraction of bronchial casts in 1 NPD patient. CONCLUSIONS: Lung involvement in NPD and GD patients should be included in the differential diagnosis of interstitial lung disease. Besides interstitial appearance on HRCT, atelectasis related to bronchial cast and bronchiectasis are other radiological findings in these group of patients. Analysis of bronchoalveolar fluid and lung biopsy provide very important clues for diagnosis. Hepatopulmonary syndrome is an important vascular complication observed in GD patients.


Assuntos
Doença de Gaucher/complicações , Pneumopatias/etiologia , Doenças de Niemann-Pick/complicações , Transtornos Respiratórios/etiologia , Infecções Respiratórias/etiologia , Criança , Pré-Escolar , Feminino , Doença de Gaucher/diagnóstico por imagem , Doença de Gaucher/patologia , Humanos , Lactente , Pneumopatias/diagnóstico por imagem , Pneumopatias/patologia , Masculino , Doenças de Niemann-Pick/diagnóstico por imagem , Doenças de Niemann-Pick/patologia , Radiografia , Transtornos Respiratórios/diagnóstico por imagem , Transtornos Respiratórios/patologia , Infecções Respiratórias/diagnóstico por imagem , Infecções Respiratórias/patologia , Estudos Retrospectivos
8.
Pediatr Pulmonol ; 45(5): 514-7, 2010 May.
Artigo em Inglês | MEDLINE | ID: mdl-20425862

RESUMO

Pulmonary alveolar microlithiasis (PAM) is a rare disease with alveolar microliths mainly composed of calcium phosphate. The gene responsible for the disease is SLC34A2, which encodes a type-IIb sodium phosphate cotransporter, has been described recently. Treatment of this disease is not clearly defined. Disodium etidronate is a member of bisphonates and it has been administered in these patients due to its inhibitory effect on the precipitation of hydroxyapatite microcrystals. Here, clinical and radiological improvement of two patients with PAM who were treated with disodium etidronate for 9 and 11 years, respectively, are presented. The pathogenetic mechanism of this treatment on the genetic basis of disease is discussed.


Assuntos
Difosfonatos/uso terapêutico , Ácido Etidrônico/uso terapêutico , Litíase/tratamento farmacológico , Pneumopatias/tratamento farmacológico , Alvéolos Pulmonares , Fosfatos de Cálcio/análise , Criança , Pré-Escolar , Feminino , Humanos , Litíase/diagnóstico por imagem , Litíase/genética , Pneumopatias/diagnóstico por imagem , Pneumopatias/genética , Radiografia , Proteínas Cotransportadoras de Sódio-Fosfato Tipo IIb/genética
9.
Pediatr Surg Int ; 24(8): 965-9, 2008 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-18587587

RESUMO

H-type tracheoesophageal fistula (TEF) may lead to chronic respiratory disease if the diagnosis is delayed. Long-standing fistula causes esophageal distention which is named as pneumoesophagus or megaesophagus and possibly affects the motility of the esophageal body which may also be encountered as a part of tracheoesophageal anomalies. Both dysmotility and megaesophagus may mimic achalasia radiologically and the patient may be advised an unnecessary esophagocardiomyotomy. The authors report a 15-year-old adolescent with H-type TEF who has been diagnosed during investigations for chronic respiratory disease due to presumptive diagnosis of achalasia. The authors emphasize that a complete anatomical and functional evaluation of the upper gastrointestinal tract should be done before recommending operation for achalasia in patients with chronic respiratory disease. H-type TEF should be investigated to avoid unnecessary cardiomyotomy.


Assuntos
Acalasia Esofágica/diagnóstico , Fístula Traqueoesofágica/diagnóstico , Adolescente , Broncoscopia , Diagnóstico Diferencial , Endoscopia Gastrointestinal , Acalasia Esofágica/etiologia , Seguimentos , Humanos , Masculino , Pneumonia Aspirativa/diagnóstico , Pneumonia Aspirativa/etiologia , Radiografia Torácica , Fatores de Tempo , Tomografia Computadorizada por Raios X , Fístula Traqueoesofágica/complicações
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