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1.
BMC Pediatr ; 23(1): 110, 2023 03 08.
Artículo en Inglés | MEDLINE | ID: mdl-36890482

RESUMEN

BACKGROUND: Neurofibromatosis type 1 and pseudoachondroplasia are both rare autosomal dominant disorders, caused by pathogenic mutations in NF1 and COMP genes, respectively. Both neurofibromin 1 and cartilage oligomeric matrix protein (COMP) play a role in the development of the skeleton. Carrying both germline mutations has not been previously reported; however, it can affect the developing phenotype. CASE PRESENTATION: The index patient, an 8-year-old female presented with several skeletal and dermatologic anomalies resembling the coexistence of multiple syndromes. Her mother had dermatologic symptoms characteristic for neurofibromatosis type 1, and her father presented with distinct skeletal anomalies. NGS-based analysis revealed a heterozygous pathogenic mutation in genes NF1 and COMP in the index patient. A previously unreported heterozygous variant was detected for the NF1 gene. The sequencing of the COMP gene revealed a previously reported, pathogenic heterozygous variant that is responsible for the development of the pseudoachondroplasia phenotype. CONCLUSIONS: Here, we present the case of a young female carrying pathogenic NF1 and COMP mutations, diagnosed with two distinct heritable disorders, neurofibromatosis type 1 and pseudoachondroplasia. The coincidence of two monogenic autosomal dominant disorders is rare and can pose a differential diagnostic challenge. To the best of our knowledge, this is the first reported co-occurrence of these syndromes.


Asunto(s)
Acondroplasia , Neurofibromatosis 1 , Humanos , Femenino , Neurofibromatosis 1/complicaciones , Neurofibromatosis 1/diagnóstico , Neurofibromatosis 1/genética , Acondroplasia/diagnóstico , Acondroplasia/genética , Mutación , Fenotipo
2.
Int Orthop ; 37(1): 99-104, 2013 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-23223970

RESUMEN

PURPOSE: The purpose of the study was to evaluate the treatment of five patients with proximal tibial epiphysis preserving resection for malignant tumours of the tibia. METHOD: Three patients suffered from Ewing sarcoma, two had osteosarcoma. The proximal level of the resection was in the line of the growth plate in four patients, and 2 cm below of the growth plate in one patient. The distal resection level was in the diaphysis, depending on the tumour border. The resected part of the tibia was substituted by both the ipsilateral and contralateral fibulas. The two fibulas were proximally built into the epiphysis, the contralateral, freely transplanted fibula was placed distally into the tibial diaphysis. The leg was fixed in an Ilizarov frame. RESULTS: The follow-up period was 4.9 years on average (range, 3.5-8 years). Neither local recurrence nor metastasis was noticed in patients with tibia tumour resection during the observation time. Proper fixation of the transplanted fibula with all of the patients, both proximal to the tibia epiphysis and distal to diaphysis, was experienced. The range of motion of the knee was 122° on the average. Two patients can walk without support and three can walk with an orthosis fully weight bearing. The limb shortening of the affected side was 2.6 cm on average (range,1.5-4.5 cm). In three patients the transplanted opposite side fibula has broken. In one patient, chronic osteomyelitis developed at the distal part of the transplanted fibula. CONCLUSIONS: The difficulties of intercalary segment supplementation were present to a greater extent because of the small size of proximal epiphysis, but the preserved knee joint can produce better function for the patient in this limb-saving surgery.


Asunto(s)
Neoplasias Óseas/cirugía , Epífisis/cirugía , Osteosarcoma/cirugía , Sarcoma de Ewing/cirugía , Tibia/cirugía , Adolescente , Neoplasias Óseas/patología , Trasplante Óseo , Niño , Preescolar , Epífisis/patología , Femenino , Peroné/trasplante , Estudios de Seguimiento , Humanos , Técnica de Ilizarov , Recuperación del Miembro , Masculino , Osteosarcoma/patología , Complicaciones Posoperatorias , Sarcoma de Ewing/patología , Tibia/patología , Resultado del Tratamiento , Soporte de Peso
3.
J Minim Access Surg ; 9(2): 84-6, 2013 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-23741116

RESUMEN

Lymphangioleiomyomatosis with tuberous sclerosis complex is a rare disease. One of the most frequent complications of lymphangioleiomyomatosis is pleural effusion (chylothorax) wich can be treated with the use of VATS. Authors report a case of pulmonary lymphangioleiomyomatosis in a 56-year-old female patient with tuberous sclerosis complex with an 8-week history of recurrent chylothorax, dyspnea and debilitating weakness. By CT scan a flat tissue proliferation was seen in the site of the thoracic duct and it was supposed to be the reason for the pleural effusion. A VATS resection of this laesion and ligation of the thoracic duct was performed successfully. Chylothorax is often associated with pulmonary lymphangioleiomyomatosis. Lymphangioleiomyomatosis combined with tuberous sclerosis complex is extremely rare. In case of chylothorax VATS treatment is successful and may be the first choice.

4.
Orv Hetil ; 164(16): 610-617, 2023 Apr 23.
Artículo en Húngaro | MEDLINE | ID: mdl-37087731

RESUMEN

INTRODUCTION: Caregiver Priorities and Child Health Index of Life with Disabilities (CPCHILD) questionnaire is a measurement tool for assessing health status and wellbeing of disabled children, which evaluates children's quality of life from the caregiver point of view. OBJECTIVE: The aim of our work was the Hungarian translation and cultural adaptation of the questionnaire and also validation of the CPCHILD on Hungarian cerebral palsy patients. Further aim was to test the eligibility of the questionnaire for superficial assessment of caregiver's psychological attitudes. METHOD: Translation of the questionnaire was carried out according to the Beaton's guidelines. Test-retest, interrater reliability (ICC) and also internal consistency (Cronbach-alpha) were calculated for reliability. The importance of the questions was assessed for face validity and known group validity test was done to measure construct validity. For examining parental attitudes, the patients were divided into ambulatory and non-ambulatory groups and the 36 quality of life questions of the 7th domain were used to find relations. RESULTS: During test-retest reliability measurements, the ICC was 0.96 (95% CI: 0.88-0.98), and Cronbach-alpha exceeded the minimal expected value of 0.7 (0.74-0.97) except in the 5th domain (0.67), while measuring interrater reliability the ICC was 0.87 (95% CI 0.70-0.94). Face validity was above the 2.0 threshold in every question (2.6-4.5; mean: 3.4 ± 1.34) and the known group validity calculations showed significant differences between the CPCHILD scores of ambulatory and non-ambulatory groups. Examining parental attitudes, a significant difference was also shown among the parents of ambulatory and non-ambulatory children in assessing the importance of sitting in the quality of life of their children (2.89 ± 1.28 vs. 3.51 ± 0.82; p<0.01). CONCLUSION: The final outcome of our study is that CPCHILD questionnaire has become widely accessible in Hungarian language. Our result, that the answers referring to the sitting abilities and the activities should be carried out in sitting position, was significantly different among the caregivers of the ambulatory and non-ambulatory children, showing that the parents of the GMFCS IV and V category children evaluate the importance of sitting ability higher compared to those parents who care for GMFCS I, II and III category children. Furthermore, the results draw attention to the wellbeing and health of the children measurable with CPCHILD as well as that parental caregiver attitudes can be recognized which may give further help in finding the balance between expectations and possibilities during the rehabilitation of cerebral palsy children. Orv Hetil. 2023; 164(16): 610-617.


Asunto(s)
Parálisis Cerebral , Niños con Discapacidad , Niño , Humanos , Calidad de Vida , Cuidadores , Salud Infantil , Parálisis Cerebral/psicología , Reproducibilidad de los Resultados , Hungría , Encuestas y Cuestionarios , Lenguaje
5.
Orv Hetil ; 153(5): 163-73, 2012 Feb 05.
Artículo en Húngaro | MEDLINE | ID: mdl-22275731

RESUMEN

In the last two decades, the treatment paradigms for Crohn's disease and ulcerative colitis have significantly changed inclusive of a continuously increasing role of biological therapy (anti TNFs). Some patients, however, experience lack or loss of response to biological treatment, and in such cases the management of patients is often empirical. In this review, the authors aim to summarize the available data regarding epidemiology and predictors of loss of response to biological therapy considering the clinical factors and the relationship between serum concentrations, antibodies against biological agents, respectively. Monitoring drug levels and antibodies is expected to play an important role in the management of loss of response (i.e. to confirm adherence, allow dose adjustment, or provide rationale for switching to another biological agent or to a different class of biological agent) in the coming years. The optimal method of detection and cut-off values are, however, not clear. In clinical practice, meticulous complex assessment of clinical symptoms, confirmation of active disease by endoscopic or radiological imaging, and excluding complications remain necessary.


Asunto(s)
Antiinflamatorios/farmacología , Anticuerpos/sangre , Fármacos Gastrointestinales/farmacología , Factores Inmunológicos/farmacología , Inmunoterapia , Enfermedades Inflamatorias del Intestino/tratamiento farmacológico , Enfermedades Inflamatorias del Intestino/inmunología , Adalimumab , Antiinflamatorios/administración & dosificación , Antiinflamatorios/sangre , Antiinflamatorios/inmunología , Anticuerpos Monoclonales/farmacología , Anticuerpos Monoclonales Humanizados/farmacología , Colitis Ulcerosa/tratamiento farmacológico , Colitis Ulcerosa/inmunología , Enfermedad de Crohn/tratamiento farmacológico , Enfermedad de Crohn/inmunología , Tolerancia a Medicamentos , Fármacos Gastrointestinales/administración & dosificación , Fármacos Gastrointestinales/sangre , Fármacos Gastrointestinales/inmunología , Humanos , Factores Inmunológicos/administración & dosificación , Factores Inmunológicos/sangre , Factores Inmunológicos/inmunología , Inmunoterapia/métodos , Infliximab , Metotrexato/farmacología , Ensayos Clínicos Controlados Aleatorios como Asunto , Factores de Riesgo , Insuficiencia del Tratamiento , Factor de Necrosis Tumoral alfa/antagonistas & inhibidores
6.
Int Orthop ; 35(5): 755-9, 2011 May.
Artículo en Inglés | MEDLINE | ID: mdl-20306192

RESUMEN

The purpose of this study was to establish a nomogram in order to predict limb length discrepancies in children with unilateral fibular hemimelia more accurately. In 31 children with unilateral fibular hemimelia the femoral-tibial length and skeletal age were determined an average of seven times per case by sequential radiographs during growth. From the data, a skeletal age nomogram was developed which shows a steeply declining mean skeletal age pattern in unilateral fibular hemimelia (the slope in girls was -0.59 and in boys -0.64). This nomogram crosses the normal mean skeletal age line of the Moseley straight-line graph at 10.5 years in girls and at 12 years in boys, and continues to decline until maturity. The results demonstrate an abnormal skeletal maturation process in patients with unilateral fibular hemimelia. The consistently declining steep skeletal age nomogram in unilateral fibular hemimelia makes prediction of skeletal maturity and limb length discrepancy inaccurate by the standard predictive methods particularly when using early skeletal ages. The skeletal age nomogram from our data determines skeletal maturation in children with unilateral fibular hemimelia more accurately, and allows a correct prediction of limb length discrepancy.


Asunto(s)
Determinación de la Edad por el Esqueleto/métodos , Ectromelia/diagnóstico , Peroné/patología , Diferencia de Longitud de las Piernas/diagnóstico , Pierna/crecimiento & desarrollo , Niño , Preescolar , Errores Diagnósticos , Ectromelia/complicaciones , Ectromelia/diagnóstico por imagen , Femenino , Peroné/diagnóstico por imagen , Humanos , Pierna/diagnóstico por imagen , Diferencia de Longitud de las Piernas/diagnóstico por imagen , Diferencia de Longitud de las Piernas/etiología , Modelos Lineales , Masculino , Nomogramas , Valor Predictivo de las Pruebas , Tibia/patología
7.
Orv Hetil ; 152(9): 344-8, 2011 Feb 27.
Artículo en Húngaro | MEDLINE | ID: mdl-21324806

RESUMEN

Buschke-Löwenstein tumor (verrucous carcinoma, giant condylomata) of the anal margin is a locally invasive, destructively growing carcinoma that does not metastasize. The lesions are rare despite the increased incidence of anal condylomata and anal carcinomas. Authors report a case of a 63-year-old woman suffering from verrucous carcinoma (Buschke-Löwenstein tumor) of the anal margin. The tumor invaded the rectal sphincter and extended beyond the muscle, infiltrating the lower abdomen. Infiltration of the perivesical soft tissue caused bilateral hydronephros. Because both under- and overdiagnosis of anal cancer and precancer may lead to inappropriate treatment, it is important to perform adequate sampling for histology. Non-representative superficial biopsies may result underdiagnosis of the disease.


Asunto(s)
Neoplasias del Ano/diagnóstico , Biopsia , Carcinoma Verrugoso/diagnóstico , Neoplasias del Ano/patología , Neoplasias del Ano/cirugía , Tumor de Buschke-Lowenstein , Carcinoma Verrugoso/patología , Carcinoma Verrugoso/cirugía , Condiloma Acuminado/diagnóstico , Condiloma Acuminado/patología , Condiloma Acuminado/cirugía , Femenino , Humanos , Hidronefrosis/etiología , Persona de Mediana Edad , Neoplasias Pélvicas/complicaciones , Neoplasias Pélvicas/diagnóstico por imagen , Neoplasias Pélvicas/secundario , Neoplasias del Pene/diagnóstico , Neoplasias del Pene/patología , Neoplasias del Pene/cirugía , Tomografía Computarizada por Rayos X
8.
Front Cell Infect Microbiol ; 11: 660347, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-33937100

RESUMEN

Mucormycosis is a life-threatening opportunistic infection caused by certain members of the fungal order Mucorales. This infection is associated with high mortality rate, which can reach nearly 100% depending on the underlying condition of the patient. Treatment of mucormycosis is challenging because these fungi are intrinsically resistant to most of the routinely used antifungal agents, such as most of the azoles. One possible mechanism of azole resistance is the drug efflux catalyzed by members of the ATP binding cassette (ABC) transporter superfamily. The pleiotropic drug resistance (PDR) transporter subfamily of ABC transporters is the most closely associated to drug resistance. The genome of Mucor circinelloides encodes eight putative PDR-type transporters. In this study, transcription of the eight pdr genes has been analyzed after azole treatment. Only the pdr1 showed increased transcript level in response to all tested azoles. Deletion of this gene caused increased susceptibility to posaconazole, ravuconazole and isavuconazole and altered growth ability of the mutant. In the pdr1 deletion mutant, transcript level of pdr2 and pdr6 significantly increased. Deletion of pdr2 and pdr6 was also done to create single and double knock out mutants for the three genes. After deletion of pdr2 and pdr6, growth ability of the mutant strains decreased, while deletion of pdr2 resulted in increased sensitivity against posaconazole, ravuconazole and isavuconazole. Our result suggests that the regulation of the eight pdr genes is interconnected and pdr1 and pdr2 participates in the resistance of the fungus to posaconazole, ravuconazole and isavuconazole.


Asunto(s)
Azoles , Mucor , Antifúngicos/farmacología , Farmacorresistencia Fúngica , Proteínas Fúngicas , Humanos , Pruebas de Sensibilidad Microbiana
9.
Magy Seb ; 63(3): 112-7, 2010 Jun.
Artículo en Húngaro | MEDLINE | ID: mdl-20570783

RESUMEN

AIMS: Videothoracoscopy plays a leading role in the management of spontaneous pneumothorax. This study evaluates various treatment strategies based on early results. PATIENTS AND METHODS: In a five-year period 243 (184 male, 59 female, mean age: 37.1 years) patients were treated with spontaneous pneumothorax in 302 cases. In case of partial ptx observation was used in 24 (8%) and aspiration in 6 (2%) patients. Chest drain was inserted in 241 (67.6%) cases. Indications for surgery were recurrence of disease, previous contralateral pneumothorax and failure of drainage. Videothoracoscopy was indicated in 71 (23.5%), Jakoscopy in 5 (1.7%), thoracotomy in 13 (4.3%) cases. The operation was completed with partial pleurectomy in 25, talcum or mechanical pleurodesis in 32 and 3 cases, respectively. RESULTS: Conservative treatment was successful in 24 (80%) of the 30 conservatively treated cases, while chest drainage succeeded in 204 (84.7%) of the 241 cases. Conversion was needed in 8 (11.3%) cases of the 71 VATS. The remaining 63 patients recovered. Thoracotomy and Jakoscopy were successful in all cases. The postoperative complication rate was 6.3% after VATS, and 7.7% after thoracotomy. Reoperation was performed because bleeding in one case after VATS and thoracotomy. In one case empyema, and in another patient pneumonia developed after VATS. Postoperative bleeding occurred in one case after thoracotomy. The mean hospitalization was 8.5 days after drainage, 9.1 days after VATS and 11.3 days after thoracotomy. The postoperative mortality rate was 1.3% (4 patients). CONCLUSIONS: In case of spontaneous pneumothorax the first choice of therapy is chest tube drainage. VATS is indicated in case of recurrence, failure of drainage and previous contralateral pneumothorax.


Asunto(s)
Neumotórax/cirugía , Cirugía Torácica Asistida por Video , Adulto , Anciano , Tubos Torácicos , Drenaje , Femenino , Humanos , Masculino , Persona de Mediana Edad , Pleura/cirugía , Pleurodesia , Neumotórax/terapia , Hemorragia Posoperatoria/etiología , Hemorragia Posoperatoria/cirugía , Reoperación , Cirugía Torácica Asistida por Video/efectos adversos , Cirugía Torácica Asistida por Video/métodos , Toracotomía/efectos adversos , Resultado del Tratamiento
10.
Magy Seb ; 63(3): 121-4, 2010 Jun.
Artículo en Húngaro | MEDLINE | ID: mdl-20570785

RESUMEN

Spontaneous rupture of the oesophagus is an extremely serious condition with high morbidity and mortality. Primary surgical repair may be followed by numerous complications. A 29-year-old man had undergone primary surgical repair due to spontaneous rupture of the lower third of the oesophagus. After the operation he developed suture insufficiency, which could not have been stented, and transgastric drainage was performed therefore. After a slow healing of the fistula, we could avoid another operation and his oesophagus was preserved. Fortunately, a stricture did not develop either. Transgastric drainage of the oesophagus could be a good therapeutic choice in selective and complicated cases.


Asunto(s)
Drenaje/métodos , Perforación del Esófago/terapia , Estómago , Dehiscencia de la Herida Operatoria/terapia , Adulto , Perforación del Esófago/diagnóstico por imagen , Perforación del Esófago/cirugía , Humanos , Laparotomía , Masculino , Rotura Espontánea/complicaciones , Dehiscencia de la Herida Operatoria/etiología , Tomografía Computarizada por Rayos X
11.
Int Orthop ; 33(2): 561-6, 2009 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-18259704

RESUMEN

This study evaluated the histological changes in muscle tissue after limb lengthening in skeletally mature and immature rabbits and assessed the most vulnerable level of striated muscle. Twenty-three male domestic white rabbits, divided into six groups, were operated on and different lengthening protocols were used in the mature and immature rabbits. The histopathological changes were analysed by a semi-quantitative method according to the scoring system of Lee et al. (Acta Orthop Scand 64(6):688-692, 1993). After the evaluation of the five main degenerative parameters (muscle atrophy, muscle nuclei internalisation, degeneration of the muscle fibre, perimysial and endomysial fibrosis, haematomas), it is evident that the adults lengthened at a rate of 1.6 mm/day showed more degenerative changes than those lengthened at 0.8 mm/day. The adult 1.6 mm/day lengthened group presented significantly higher damage in the muscle and lower regenerative signs compared with the young 1.6 mm/day lengthened group, according to the summarised degenerative scores.


Asunto(s)
Alargamiento Óseo/efectos adversos , Alargamiento Óseo/métodos , Células Musculares/patología , Músculo Esquelético/patología , Atrofia Muscular/patología , Factores de Edad , Animales , Biopsia con Aguja , Modelos Animales de Enfermedad , Humanos , Inmunohistoquímica , Fibras Musculares Esqueléticas/patología , Probabilidad , Conejos , Distribución Aleatoria , Regeneración/fisiología , Factores de Riesgo , Sensibilidad y Especificidad , Estadísticas no Paramétricas
12.
Magy Seb ; 62(6): 353-6, 2009 Dec.
Artículo en Húngaro | MEDLINE | ID: mdl-19945939

RESUMEN

Orthopedic surgeons apply metallic pins to stabilize the clavicule and humerus on a daily basis. Migration of these pins into the thoracic cavity is rare. We present the case of an elderly female patient, whose right humeroscapular joint was fixed with Kirschner wires due to recurrent luxation. Six weeks later, a follow-up X-ray revealed that the pins have migrated into the right thoracic cavity, confirmed by a CT chest. Videothoracoscopic removal of the metallic pins was not possible because of dense adhesions. Right anterolateral thoracotomy was carried out, and after pneumolysis one pin was taken out from the 2nd lung segment. The other one, which was running along the cupola and entering the spinal cord, was also removed. There was no postoperative surgical complication. The authors review the literature of this rare complication and point out that pins migrating into the thoracic cavity should be removed to avoid life threatening complications.


Asunto(s)
Hilos Ortopédicos/efectos adversos , Remoción de Dispositivos , Migración de Cuerpo Extraño/diagnóstico por imagen , Migración de Cuerpo Extraño/cirugía , Luxación del Hombro/cirugía , Articulación del Hombro/cirugía , Anciano , Femenino , Humanos , Húmero/cirugía , Radiografía , Escápula/cirugía , Cirugía Torácica Asistida por Video , Resultado del Tratamiento
13.
Orv Hetil ; 160(28): 1105-1111, 2019 Jul.
Artículo en Húngaro | MEDLINE | ID: mdl-31280598

RESUMEN

Introduction: Botulinum toxin is used for decreasing spasticity, improving gait pattern and preventing secondary deformities and orthopedic surgeries in children with cerebral palsy. Despite its wide use, there is no evidence for the long-term beneficial effect of the toxin. Aim: The authors focused on the short-term effects of the toxin and on the subjective evaluation done by the parents about the botox treatment. Method: First, the calf muscle of 18 children was treated and casting was also performed. In our second patient group, multilevel lower limb injection was done in 12 cases. Joints' range of motion, muscle tone and spasticity were assessed before and 4-6 weeks after treatment. In ambulatory children, gait analysis was done. Side effects were recorded and parental opinion about the treatment was requested. Results: Increased ankle range of motion and decreased muscle tone and spasticity were seen in our first patient group. In the second group, hip flexion contracture became milder and hip abduction and the popliteal angle improved. However, gait analysis results only slightly changed after botox treatment. Occasional mild and transient adverse effects were observed during the pharmacologically active period of the toxin. Associate beneficial effects were also reported such as better comfort, easier movements, improving function of the non-injected upper limb, decreasing dysarthria and dysphagia. Conclusion: Our study strengthens the observation that botox treatment of the spastic calf muscle together with plaster casting can result in improved ankle dorsiflexion. Multilevel botox treatment can improve lower limb joints range of motion, however, gait pattern remains unchanged. The administration of botulinum toxin with respect to the guidelines has no major adverse effects. Further studies are needed to clarify the observed beneficial associate effects of the toxin. Orv Hetil. 2019; 160(28): 1105-1111.


Asunto(s)
Toxinas Botulínicas Tipo A , Parálisis Cerebral , Espasticidad Muscular , Toxinas Botulínicas Tipo A/uso terapéutico , Parálisis Cerebral/tratamiento farmacológico , Niño , Humanos , Extremidad Inferior , Espasticidad Muscular/tratamiento farmacológico , Rango del Movimiento Articular , Resultado del Tratamiento
14.
J Cancer Res Clin Oncol ; 134(4): 439-51, 2008 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-17922141

RESUMEN

PURPOSE: Carboxypeptidase-M (CPM) is a membrane-bound peptidase that metabolizes peptides, and is present in pneumocytes. CPM hydrolyses the C-terminal arginine of epidermal growth factor (EGF) resulting in des-Arg53-EGF which binds to the EGF receptor (EGFR) with an equal or greater affinity than native EGF. Therefore, this study focused on the possible presence of CPM in human lung adenocarcinomas (ADC) and evaluated the relationship between CPM and EGFR by assessing the impact of expressions on patient clinical outcome. METHODS: This is a retrospective study of 110 patients who underwent resection of the primary tumour (92) or metastatic tissues (18) for treatment or diagnosis. Immunohistochemistry (IHC) for CPM and EGFR was made in serial sections using standard methods. RESULTS: This study demonstrates for the first time that 23.6% of ADCs express carboxypeptidase-M (26/110), mainly in membrane-bound forms. The amounts and the extent of CPM within tumours vary from low levels to obviously overexpressed forms. The immunohistochemical positivity (+) for CPM in ADCs negatively correlated with disease survival. In addition, 80% of CPM+ adenocarcinomas (21/26) showed a coexpression with EGFR suggesting a high prevalence for coexistence. The follow up data indicated a significantly shorter 5-year survival time for patients with CPM+-EGFR+ (double-positive) tumours compared to those harbouring neoplasias negative for both proteins (9.5 vs. 60.4% survivals, P < 0.001). CONCLUSION: The fact that CPM+ ADCs often co-express with EGFR suggests a functional-regulatory link between these proteins which might have therapeutical consequences. The present novel data could lead to improved IHC tests in lung adenocarcinomas for EGFR expression.


Asunto(s)
Adenocarcinoma/química , Receptores ErbB/análisis , Neoplasias Pulmonares/química , Metaloendopeptidasas/análisis , Adenocarcinoma/mortalidad , Adenocarcinoma/patología , Femenino , Proteínas Ligadas a GPI , Humanos , Inmunohistoquímica , Neoplasias Pulmonares/mortalidad , Neoplasias Pulmonares/patología , Masculino , Estadificación de Neoplasias , Estudios Retrospectivos
15.
J Laparoendosc Adv Surg Tech A ; 18(5): 735-7, 2008 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-18803519

RESUMEN

OBJECTIVE: Intubation of the tracheobronchial tree is the most common type of malposition during the placement of narrow-bore enternal tubes. CASE REPORT: In addition to other treatment components in a 65-year-old female, nasojejunal feeding was started to treat her for severe acute pancreatitis. After the placement of the narrow-bore feeding tube, she developed dyspnea and huskiness. On auscultation and X-ray investigation, the right pneumothorax was detected and the tube was found in the chest cavity. The diagnosis was confirmed by bronchoscopy. Videothoracoscopic resection and closure of the lacerated lung, using a tube thoracostomy, were performed. The patient recovered after postoperative conservative treatment for her pancreatitis. CONCLUSION: Pneumothorax and laceration of the lung-caused by the malposition of narrow-bore enternal tube-can be successfully treated by applying videothoracoscopy.


Asunto(s)
Nutrición Enteral/efectos adversos , Intubación Gastrointestinal/efectos adversos , Neumotórax/etiología , Cirugía Torácica Asistida por Video/métodos , Toracostomía/métodos , Anciano , Extravasación de Materiales Terapéuticos y Diagnósticos/diagnóstico por imagen , Femenino , Humanos , Pancreatitis/dietoterapia , Neumotórax/diagnóstico por imagen , Radiografía
16.
Magy Seb ; 61 Suppl: 37-40, 2008.
Artículo en Húngaro | MEDLINE | ID: mdl-18504235

RESUMEN

Myasthenia is a rare autoimmune disease characterized by fluctuating muscle weakness and fatigability due to a reduction in available acetylcholine receptors at the neuromuscular junction. Data of 186 patients suffering from myasthenia were collected retrospectively. All patients underwent thymectomy over a 23 years period from 1981 to 2006 without surgical mortality. Postoperative ventilation was required for more than 24 hours in seven patients and one patient needed postoperative ventilatory support more than seven days. Thymectomy for myasthenia was performed using promethazine and atropine in general anaesthesia. Introduction could be facilitated with propofol, etomidate or thiopental and sevoflurane, avoiding use of any muscle relaxants. Non-depolarizing muscle relaxants were not used during the procedures. Adequate surgical conditions were provided by short-acting inhaled anaesthetics (sevoflurane) and small doses of opiates. 95% of the narcotized patients were immediately extubated after the procedure in the operating room. Length of stay in intensive care unit could have been reduced without any postoperative ventilatory support. Nonsteroid analgesics and nalbuphine were used for pain relief. Anaesthesia of thymectomy is based on volatile gases. Airway complications can be prevented with use of small amounts of anticholinergic drugs, perhaps steroids.


Asunto(s)
Anestesia General/métodos , Anestésicos por Inhalación/administración & dosificación , Anestésicos Intravenosos/administración & dosificación , Hipnóticos y Sedantes/administración & dosificación , Miastenia Gravis , Timectomía , Adyuvantes Anestésicos/administración & dosificación , Adulto , Anciano , Electromiografía , Femenino , Humanos , Hungría , Masculino , Persona de Mediana Edad , Miastenia Gravis/diagnóstico , Miastenia Gravis/fisiopatología , Estudios Retrospectivos
17.
Magy Seb ; 60(1): 510-3, 2007 Jan.
Artículo en Húngaro | MEDLINE | ID: mdl-17474305

RESUMEN

AIMS: Video assisted thoracoscopy (VATS) is a more and more frequently used method in the diagnosis and treatment of blunt thoracic trauma. In some cases it has diagnostic and in others therapeutic effect. The authors analyze the role of VATS in the diagnosis and the treatment of 83 patients treated with haemothorax. PATIENTS AND METHODS: There were 83 patients treated (60 male, 23 female, mean age of 54.4 years) with haemothorax caused by blunt thoracic trauma. Chest tube drainage was used in 31 (37.3%) cases. Urgent thoracotomy was performed in only two (2.4%) cases. Elective, planned VATS was used in 11 (13.3) cases. All of these 3 were diagnostic and 8 were therapeutic procedures. In three cases rupture of the diaphragm was diagnosed with the use of VATS which were treated through thoracotomy. In the remaining 8 cases haematoma evacuation and in 3 cases intercostal artery bleeding were treated with VATS. RESULTS: No complications related to the procedure were observed. The thoracotomy in all 3 cases verified the diaphragmatic injuries. The 8 patients undergone therapeutic VATS recovered. The mean hospital stay after VATS was 7.8 days and 11.3 days after thoracotomy in the patients with diaphragmatic injury. CONCLUSIONS: The VATS has a significant role in the diagnosis of blunt thoracic, especially of diaphragmatic, injuries. In other cases the VATS has good therapeutic effect.


Asunto(s)
Traumatismos Torácicos/cirugía , Cirugía Torácica Asistida por Video , Heridas no Penetrantes/cirugía , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Niño , Preescolar , Diafragma/lesiones , Diafragma/cirugía , Procedimientos Quirúrgicos Electivos , Femenino , Hematoma/etiología , Hematoma/cirugía , Humanos , Tiempo de Internación , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Rotura/diagnóstico , Rotura/etiología , Resultado del Tratamiento
18.
Magy Seb ; 60(1): 514-7, 2007 Jan.
Artículo en Húngaro | MEDLINE | ID: mdl-17474306

RESUMEN

AIMS: The pyogenic infection of the sternoclavicular joint is a rare disease. The treatment can be conservative (antibiotics) or surgical (exposure and drainage of the joint or its resection). The authors analyze the causative and predisposing factors, the symptoms, the diagnosis, the questions of the therapy and its efficiency. PATIENTS AND METHODS: In the 6 cases (5 male, 1 female, average age: 56.8 years) the cause of the disease were trauma or metastatic septic focus in 2-2 cases and intraarticular injection or preceding radiotherapy in 1-1 case. Predisposing diseases (diabetes mellitus, gout, chronic alcoholism, liver cirrhosis) could be proved in two cases. The most frequent symptoms were the swelling and erythema of the joint, fever, pain and limitation of motion. In all cases the CT scan proved the destruction of the joint. In one case after unsuccessful conservative therapy and in 1-1 case after debridement and drainage because of concomitant multiple septic focuses and mediastinitis resection of the sternoclavicular joint was applied in a later second step. In the other three patients primary resection of the joint was performed. RESULTS: Both the conservative and drainage managements were insufficient. On the other hand the radical joint resection caused complete recovery in all cases. No intra and postoperative complications were observed. On an average 28.2 months after the radical operation the functional results were excellent. CONCLUSIONS: Relying upon the results, radical resection is supposed to be the most effective method. Conservative treatment or drainage are recommended only for cases associated with severe complications. Resection is worthy performing after the recovery of the concomitant illnesses.


Asunto(s)
Infecciones Bacterianas/cirugía , Artropatías/cirugía , Articulación Esternoclavicular/cirugía , Adulto , Anciano , Antibacterianos/uso terapéutico , Infecciones Bacterianas/complicaciones , Infecciones Bacterianas/tratamiento farmacológico , Infecciones Bacterianas/microbiología , Drenaje , Femenino , Humanos , Artropatías/complicaciones , Artropatías/tratamiento farmacológico , Artropatías/microbiología , Masculino , Persona de Mediana Edad , Factores Desencadenantes , Estudios Retrospectivos , Factores de Riesgo , Articulación Esternoclavicular/microbiología , Tomografía Computarizada por Rayos X , Resultado del Tratamiento
19.
Magy Seb ; 60(4): 199-204, 2007 Aug.
Artículo en Húngaro | MEDLINE | ID: mdl-17931996

RESUMEN

INTRODUCTION: Successful treatment of penetrating chest trauma largely depends on the accurate and rapid diagnostic work-up, as well as the adequate surgical management. The authors discuss current issues in the diagnosis and the treatment of penetrating chest injuries based on the analysis of 109 cases. PATIENTS AND METHODS: 82 men and 27 women with penetrating chest trauma were studied. The average age of the patients was 37.8 years. The injury was caused by stabbing in 104 cases (95.4%), gunshot in 4 patients (3.7%) and explosion in one case (0.9%). 41 patients had cardiac and pericardial injuries. In those, 19 (46.3%) patients had a chest X-ray, echocardiography was done in nine cases (22%), while CT scan and diagnostic VATS were performed in two patients, respectively. All patients underwent surgery except one, who was treated conservatively.In all of the 68 patients, who had no cardiac injuries, a chest X-ray was performed. Echocardiography was done in six (8.8%) cases, diagnostic VATS in four (5.9%) patients, and abdominal ultrasound scan in 3 (4.4%) cases. Chest tube was inserted in 13 patients (19.1%), an open surgery was performed in 51 cases, while in 4 cases VATS was carried out. RESULTS: In the group of patients with cardiac and pericardial injuries, the sensitivity of the chest X-ray, echocardiography and VATS were 57.9%, 88.9% and 100%, respectively. Further, specificity of the above were 26.3%, 88.9% and 100%, respectively. However, in patients with non-cardiac injuries, the sensitivity of the chest X-ray was 100%, and both the specificity and sensitivity of VATS was 100%. Postoperative complication rate was 12.6% overall (15% in cases with cardiac injury and 10.9% in the non-cardiac subgroup). Mortality rate was 7.3% among the patients with cardiac injury, while there was no mortality detected in the non-cardiac subgroup. The average mortality rate was 2.8%. CONCLUSION: Patients with penetrating chest trauma should undergo a rapid and accurate diagnostic work-up followed by an adequate surgical management in order to keep their prognosis relatively good.


Asunto(s)
Traumatismos Torácicos/diagnóstico , Traumatismos Torácicos/cirugía , Heridas Penetrantes/diagnóstico , Heridas Penetrantes/cirugía , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Niño , Preescolar , Ecocardiografía , Explosiones , Femenino , Lesiones Cardíacas/diagnóstico , Lesiones Cardíacas/cirugía , Humanos , Hungría/epidemiología , Puntaje de Gravedad del Traumatismo , Masculino , Persona de Mediana Edad , Traumatismo Múltiple/diagnóstico , Traumatismo Múltiple/cirugía , Radiografía Torácica , Estudios Retrospectivos , Traumatismos Torácicos/complicaciones , Traumatismos Torácicos/diagnóstico por imagen , Traumatismos Torácicos/mortalidad , Cirugía Torácica Asistida por Video , Procedimientos Quirúrgicos Torácicos/efectos adversos , Procedimientos Quirúrgicos Torácicos/mortalidad , Resultado del Tratamiento , Heridas por Arma de Fuego/diagnóstico , Heridas por Arma de Fuego/cirugía , Heridas Penetrantes/complicaciones , Heridas Penetrantes/diagnóstico por imagen , Heridas Penetrantes/mortalidad , Heridas Punzantes/diagnóstico , Heridas Punzantes/cirugía
20.
Orv Hetil ; 147(43): 2091-6, 2006 Oct 29.
Artículo en Húngaro | MEDLINE | ID: mdl-17297756

RESUMEN

The role of volume reduction surgery in the therapy of early stage chronic obstructive pulmonary disease. COPD is a leading cause of morbidity and mortality worldwide. Assessment of COPD severity is based on the level of patient's symptoms, the severity of the spirometric abnormality and the presence of complications, such as respiratory failure, and right heart failure. Management of mild to moderate COPD is complex, involves the avoidance of risk factors to prevent disease progression, pharmacotherapy as needed to control symptoms, physiotherapy, and rehabilitation. The bases of pharmacotherapy are bronchodilators, which represent the first line symptom based pharmacologic intervention in COPD. Surgical treatment of emphysema was basically designed to improve subjective dyspnoea, respiratory function and quality of life in severely disabled patients. Three surgical options are currently employed, namely bullectomy, lung volume surgery and lung transplantation. During volume reduction surgery the hyperinflated areas are resected, so the functional residual volume is getting reduced, the respiratory muscle function is increased; the quality of life is improved. The authors are report a young early stage COPD patient who underwent a volume reduction surgery because of decline in physical condition, improving her disability. In conclusion in properly selected patients, lung volume surgery can offer prolonged functional benefit and satisfactory long term survival, even in early stage. The invasive options has a risk of both mortality and morbidity, they are directed only at patients who remain symptomatic despite optimal medical treatment.


Asunto(s)
Neumonectomía , Enfermedad Pulmonar Obstructiva Crónica/fisiopatología , Enfermedad Pulmonar Obstructiva Crónica/cirugía , Factores de Edad , Broncodilatadores/uso terapéutico , Contraindicaciones , Femenino , Humanos , Trasplante de Pulmón/normas , Persona de Mediana Edad , Enfermedad Pulmonar Obstructiva Crónica/diagnóstico por imagen , Enfermedad Pulmonar Obstructiva Crónica/tratamiento farmacológico , Enfermedad Pulmonar Obstructiva Crónica/patología , Calidad de Vida , Pruebas de Función Respiratoria , Terapia Respiratoria , Índice de Severidad de la Enfermedad , Espirometría , Tomografía Computarizada por Rayos X
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