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1.
Int J Mol Sci ; 25(7)2024 Apr 06.
Artigo em Inglês | MEDLINE | ID: mdl-38612889

RESUMO

The ubiquitin-proteasome system (UPS) is a pivotal cellular mechanism responsible for the selective degradation of proteins, playing an essential role in proteostasis, protein quality control, and regulating various cellular processes, with ubiquitin marking proteins for degradation through a complex, multi-stage process. The shuttle proteins family is a very unique group of proteins that plays an important role in the ubiquitin-proteasome system. Ddi1, Dsk2, and Rad23 are shuttle factors that bind ubiquitinated substrates and deliver them to the 26S proteasome. Besides mediating the delivery of ubiquitinated proteins, they are also involved in many other biological processes. Ddi1, the least-studied shuttle protein, exhibits unique physicochemical properties that allow it to play non-canonical functions in the cells. It regulates cell cycle progression and response to proteasome inhibition and defines MAT type of yeast cells. The Ddi1 contains UBL and UBA domains, which are crucial for binding to proteasome receptors and ubiquitin respectively, but also an additional domain called RVP. Additionally, much evidence has been provided to question whether Ddi1 is a classical shuttle protein. For many years, the true nature of this protein remained unclear. Here, we highlight the recent discoveries, which shed new light on the structure and biological functions of the Ddi1 protein.


Assuntos
Complexo de Endopeptidases do Proteassoma , Ubiquitina , Citoplasma , Proteínas Ubiquitinadas , Divisão Celular , Saccharomyces cerevisiae
2.
Pediatr Neurosurg ; 55(6): 374-379, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33242866

RESUMO

INTRODUCTION: We present a very rare case of ruptured superior hypophyseal artery (SHA) aneurysm that presented as an acute subdural hematoma (SDH) discussing its initial presentation, diagnosis, and treatment modalities. To our knowledge it is one of very few if any cases of a ruptured aneurysm in infants regarding that specific vascular location. CASE REPORT: A 5-month-old boy was referred to our department due to acute SDH over the right cerebral hemisphere without significant mass effect nor hydrocephalus. Further evaluation revealed a right internal carotid artery (ICA) aneurysm arising from the SHA segment. Microsurgical clip ligation using a fenestrated, angled clip was performed with simultaneous subdural clot removal and proximal control of the ICA dissected in the neck. Our patient made an excellent recovery without any complicating features. CONCLUSION: Surgical management seems to be a better option in this subgroup of patients given the long life expectancy and durability of microsurgical clip ligation. We believe that our brief case report would add some insight into the management of this rare subgroup of patients, leading to better decision-making and outcome.


Assuntos
Aneurisma Roto , Hematoma Subdural Agudo , Aneurisma Intracraniano , Aneurisma Roto/diagnóstico por imagem , Aneurisma Roto/cirurgia , Artéria Carótida Interna , Criança , Hematoma Subdural Agudo/diagnóstico por imagem , Hematoma Subdural Agudo/cirurgia , Humanos , Lactente , Aneurisma Intracraniano/complicações , Aneurisma Intracraniano/diagnóstico por imagem , Aneurisma Intracraniano/cirurgia , Masculino , Instrumentos Cirúrgicos
3.
Cent Eur J Immunol ; 45(1): 48-55, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32425679

RESUMO

INTRODUCTION: Hydrocephalus is a common disorder of the central nervous system (CNS) in the pediatric population. Surgical treatment options involve ventriculoperitoneal shunt (VPS) placement. VPS infection is the most common complication of surgically treated hydrocephalus in pediatric patients [1, 2],which may lead to neuronal damage. Myelin basic protein (MBP) has been proposed as a marker of neuronal injury in a variety of contexts, and MBP levels in the cerebrospinal fluid (CSF) may be used to assess the severity of neuronal damage [1, 3, 4]. Therefore, the aim of this study was to evaluate the CSF level of myelin basic protein (MBP) in a group of pediatric patients with VPS infection. MATERIAL AND METHODS: Thirty CSF samples were collected from pediatric patients with VPS infection. CSF levels of MBP were measured at three time points, marked by contamination detection, obtention of the first sterile CSF culture, and VPS shunt implantation. The collected data were compared with those of the control group composed of children with active congenital hydrocephalus and valid CSF values. RESULTS: The MBP level in the study group was higher than the corresponding control values in the second and third measurements. The highest MBP level was reached in the study group in the second and third measurements. CONCLUSIONS: The lack of normalization of MBP level in the CSF of children with shunt infection could be connected with ongoing brain damage. It takes longer than the normalization of CSF protein level and pleocytosis. The delay is associated with a prolonged reaction of the immunological system.

4.
Acta Obstet Gynecol Scand ; 98(9): 1172-1177, 2019 09.
Artigo em Inglês | MEDLINE | ID: mdl-30977122

RESUMO

INTRODUCTION: Severe fetal cerebral ventriculomegaly, observed in about 1 in 1000 newborns, is associated with a high risk of perinatal death and neurodevelopmental delay in survivors. The objective of this study was to evaluate the efficiency of ventriculo-amniotic shunting for drainage of severe fetal cerebral ventriculomegaly and the neurodevelopment of survivors at the age of 2 years. MATERIAL AND METHODS: This was a retrospective study of 44 fetuses with severe bilateral ventriculomegaly treated with ventriculo-amniotic shunting in a tertiary fetal therapy center between 2010 and 2015. RESULTS: Shunt insertion was successfully carried out at a median gestational age of 25 weeks (range 20-33 weeks). There were three fetal deaths within 24 hours of the procedure and 41 live births at a median gestational age of 37 weeks (range 28-39 weeks). Neurodevelopment at 2 years of age was evaluated using the Bayley scale in the 38 survivors. In the 27 cases with isolated ventriculomegaly 19 (70.4%; 95% confidence interval [95% CI] 51.5%-84.2%) had normal or mild neurodevelopmental delay and 8 (29.6%; 95% CI 15.6%-48.5%) were moderately or severely delayed. In the 11 with non-isolated ventriculomegaly 2 (18.2%; 95% CI 5.1%-4.8%) had normal or mild neurodevelopmental delay and 9 (81.8%; 95% CI 52.3%-94.9%) babies were moderately or severely delayed. CONCLUSIONS: Ventriculo-amniotic shunting is an option for the management of severe ventriculomegaly and results in normalization of the ventricular diameter. However, a high proportion of survivors have neurodevelopmental delay and the possible beneficial effect of ventriculo-amniotic shunting needs to be assessed by randomized studies.


Assuntos
Terapias Fetais/métodos , Hidrocefalia/cirurgia , Adulto , Feminino , Morte Fetal , Idade Gestacional , Humanos , Lactente , Recém-Nascido , Masculino , Gravidez , Resultado da Gravidez , Diagnóstico Pré-Natal , Estudos Retrospectivos
5.
Childs Nerv Syst ; 35(2): 237-243, 2019 02.
Artigo em Inglês | MEDLINE | ID: mdl-30564912

RESUMO

OBJECTIVE: The aim of this paper was to evaluate the outcomes of surgical treatment for post-inflammatory hydrocephalus in pediatric patients. The patient's age, surgical technique and type of implants, revision rate (depending on the cause for revision and shunt type), and final outcome measured with Neurologic Outcome Scale for Infants and Children (NOSIC) scale were evaluated. METHODS: We performed a retrospective analysis of 101 patients with post-inflammatory hydrocephalus, treated in Polish Mother's Memorial Hospital Research Institute since 2005. Children with comorbidities (e.g., tumors or hemorrhages) were excluded from the study. The assessment included patient age, surgical technique (ventriculoperitoneal shunt (VPS) or neuroendoscopy) and type of implant, revision rate (considering its cause), and final outcome measured in Neurologic Outcome Scale for Infants and Children (NOSIC) scale. RESULTS: VPS implantation was the most common surgical technique. It was performed in 66.33% (n = 67) of cases. Neuroendoscopic procedure was used in 33.66% of cases (n = 34). Revision rate of VPS was 52.23% (n = 35). Endoscopic third ventricle ventriculostomy (ETV) was efficient only in 5 cases (14.7%), whereas in 29 cases (85.3%), it was followed by VPS implantation. Revision rate in VPS implantation after ETV reached 55.17% (n = 16). In all age groups, VPS implantation was the most frequently used procedure. Revisions of the shunt systems occurred most frequently in the 1-3 (n = 21 41.18%) and < 1 (n = 12, 23.53%) age ranges. The type of valve that most often underwent dysfunction was flow-regulated type (n = 23, 62.16%). The type of valve that was the least frequently revised was differential pressure type (n = 11, 17.18%). In all age groups, mechanical dysfunction was the most frequent cause of shunt disability. Average NOSIC score ranged from 39 to 98 (average 80.58, standard deviation ± 13.34). NOSIC result relative to individual operational techniques was as follows: ETV + VPS-80.17 (n = 29, standard deviation ± 11.44), VPS-80.44 (n = 67, standard deviation ± 14.30), and ETV-80.80 (n = 5, standard deviation ± 11.62). There was no difference between the outcome of the NOSIC and the type of implanted valve or its dysfunction. CONCLUSIONS: In our analysis, post-inflammatory hydrocephalus accounts for 11.7% of all hydrocephalus types. Of post-inflammatory hydrocephalus, multiloculated type accounts for 14.9%. The most common type of surgery in these patients is implantation of the ventriculoperitoneal system. The most frequent revisions of the VPS system occur in the group of the younger children (< 3). The most common type of a dysfunction shunt is the differential pressure valve, and the rarest type the flow-regulated type. In the case of mechanical dysfunction, occlusion of the intraventricular catheter is the most common reason. ETV does not affect the frequency of VPS revisions. The average NOSIC score in children treated with hydrocephalus is below normal, and the best results are observed in the youngest children.


Assuntos
Hidrocefalia/cirurgia , Complicações Pós-Operatórias/epidemiologia , Derivação Ventriculoperitoneal , Ventriculostomia , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Hidrocefalia/etiologia , Lactente , Recém-Nascido , Inflamação/complicações , Masculino , Neuroendoscopia/efeitos adversos , Neuroendoscopia/métodos , Complicações Pós-Operatórias/etiologia , Reoperação , Estudos Retrospectivos , Derivação Ventriculoperitoneal/efeitos adversos , Derivação Ventriculoperitoneal/métodos , Ventriculostomia/efeitos adversos , Ventriculostomia/métodos
6.
Childs Nerv Syst ; 34(12): 2425-2430, 2018 12.
Artigo em Inglês | MEDLINE | ID: mdl-30003328

RESUMO

PURPOSE: The aim of the study is to use Raman spectroscopy to analyze the biochemical composition of medulloblastoma and normal tissues from the safety margin of the CNS and to find specific Raman biomarkers capable of differentiating between tumorous and normal tissues. METHODS: The tissue samples consisted of medulloblastoma (grade IV) (n = 11). The tissues from the negative margins were used as normal controls. Raman images were generated by a confocal Raman microscope-WITec alpha 300 RSA. RESULTS: Raman vibrational signatures can predict which tissue has tumorous biochemistry and can identify medulloblastoma. The Raman technique makes use of the fact that tumors contain large amounts of protein and far less lipids (fatty compounds), while healthy tissue is rich in both. CONCLUSION: The ability of Raman spectroscopy and imaging to detect medulloblastoma tumors fills the niche in diagnostics. These powerful analytical techniques are capable of monitoring tissue morphology and biochemistry. Our results demonstrate that RS can be used to discriminate between normal and medulloblastoma tissues.


Assuntos
Neoplasias Cerebelares/diagnóstico por imagem , Meduloblastoma/diagnóstico por imagem , Neuroimagem/métodos , Análise Espectral Raman/métodos , Humanos
7.
Childs Nerv Syst ; 34(12): 2399-2405, 2018 12.
Artigo em Inglês | MEDLINE | ID: mdl-30032408

RESUMO

PURPOSE: The aim of the article is to describe an immunological reaction to shunt infection in children with hydrocephalus. The main cause of shunt infection involves methicillin resistant Staphylococcus epidermidis (Bhatia et al. Indian J Med Microbiol 35:120-123, 2017; Hayhurst et al. Childs Nerv Syst 24:557-562, 2008; Martínez-Lage et al. Childs Nerv Syst 26: 1795-1798, 2010; Simon et al. PLoS One, 2014; Snowden et al. PLoS One 8:e84089, 2013; Turgut et al. Pediatr Neurosurg 41:131-136, 2005), a bacterial strain which is responsible for the formation of biofilm on contaminated catheters (Snowden et al. PLoS One 8:e84089, 2013; Stevens et al. Br J of Neurosurg 26: 792-797, 2012). METHODS: The study group involved 30 children with congenital hydrocephalus after shunt system implantation, whose procedures were complicated by S. epidermidis implant infection. Thirty children with congenital hydrocephalus awaiting their first-time shunt implantation formed the control group. The level of eosinophils in peripheral blood was assessed in both groups. Cerebrospinal fluid (CSF) was examined for protein level, pleocytosis, interleukins, CCL26/Eotaxin-3, IL-5, IL-6, CCL11/Eotaxin-1, CCL3/MIP-1a, and MBP. Three measurements were performed in the study group. The first measurement was obtained at the time of shunt infection diagnosis, the second one at the time of the first sterile shunt, and the third one at the time of shunt reimplantation. In the control group, blood and CSF samples were taken once, at the time of shunt implantation. RESULTS: In the clinical material, the highest values of eosinophils in peripheral blood and CSF pleocytosis were observed in the second measurement. It was accompanied by an increase in the majority of analyzed CSF interleukins. CONCLUSION: CSF pleocytosis observed in the study group shortly after CSF sterilization is presumably related to an allergic reaction to Staphylococcus epidermidis, the causative agent of ventriculoperitoneal shunt infection.


Assuntos
Eosinofilia/etiologia , Hidrocefalia/cirurgia , Complicações Pós-Operatórias/imunologia , Infecções Estafilocócicas/imunologia , Derivação Ventriculoperitoneal/efeitos adversos , Pré-Escolar , Feminino , Humanos , Hidrocefalia/congênito , Lactente , Masculino , Resistência a Meticilina , Staphylococcus epidermidis
8.
Childs Nerv Syst ; 34(5): 845-851, 2018 05.
Artigo em Inglês | MEDLINE | ID: mdl-29322338

RESUMO

INTRODUCTION: Recognizing patients with ventriculomegaly who are at risk of developing acute hydrocephalus presents a challenge for the clinician. The association between disturbed cerebrospinal fluid flow (CSF) and impaired brain compliance may play a role in the pathogenesis of hydrocephalus. Phase contrast MRI is a noninvasive technique which can be used to assess CSF parameters. The aim of the work is to evaluate the effectiveness of phase contrast MRI in recognizing patients at risk of acute hydrocephalus, based on measuring the pulsatile CSF flow parameters in the Sylvian aqueduct and prepontine cistern in children with ventriculomegaly. AIM: The aim of the work is to characterize the parameters of cerebrospinal fluid (CSF) flow in the Sylvian aqueduct and prepontine cistern in children with ventriculomegaly with regard to patient age and symptoms. We hypothesize that the relationship between CSF flow parameters in these two regions will vary according to analyzed factors and it will allow to recognize children at risk of hydrocephalus. MATERIALS AND METHODS: A group of 26 children with ventriculomegaly (five girls and 21 boys) underwent phase contrast MRI examinations (Philips 3T Achieva with Q-flow integral application). Amplitudes of average and peak velocities of the CSF flow through the Sylvian aqueduct and prepontine cistern were used to calculate ratios of oscillation and peak velocities, respectively. The relationship between the oscillation coefficient, the peak velocity coefficient, and stroke volume was then assessed in accordance with age and clinical symptoms. RESULTS: The peak velocity coefficient was significantly higher in patients with hyper-oscillating flow through the Sylvian aqueduct (3.04 ± 3.37 vs. 0.54 ± 0.28; p = 0.0094). Moreover, these patients tended to develop symptoms more often (p = 0.0612). No significant age-related changes were observed in CSF flow parameters. CONCLUSION: Phase contrast MRI is a useful tool for noninvasive assessment of CSF flow parameters. The application of coefficients instead of direct values seems to better represent hemodynamic conditions in the ventricular system. However, further studies are required to evaluate their clinical significance and normal limits.


Assuntos
Tronco Encefálico/diagnóstico por imagem , Aqueduto do Mesencéfalo/diagnóstico por imagem , Líquido Cefalorraquidiano/fisiologia , Hidrocefalia/diagnóstico por imagem , Imageamento por Ressonância Magnética , Aqueduto do Mesencéfalo/patologia , Criança , Pré-Escolar , Meios de Contraste/farmacocinética , Feminino , Humanos , Lactente , Masculino
9.
Childs Nerv Syst ; 32(11): 2225-2231, 2016 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-27669697

RESUMO

PURPOSE: The aim of the article is to present the new extrapedicular percutaneous technique for posttraumatic vertebral column fracture. METHODS: A 15-year-old boy needed a surgical Th8 posttraumatic vertebral body (VB) compressive fracture reduction due to insufficient conservative treatment and consistent severe clinical symptoms. After 6 months of external Jevett long-roll brace stabilization, progressive sagittal balance disturbance of thoracic kyphosis was measured and persistent clinical symptoms were observed. It was decided to present a surgical technique method allowing to attempt to reduce VB fracture, rebalance the vertebral column (VC) without any motion limitation, and decrease clinical symptoms. The procedure was performed percutaneously from extrapedicular approach with intravertebral implant (Spine Jack®-Vexim™) and cement (Interface®-Vexim™) under fluoroscopic imaging (Ziehm™ 8000®). RESULTS: The whole procedure was uneventful. Now, the child is free from clinical symptoms and the partial reduction of VB fracture was achieved. The patient has been followed for 3 months. In the control CT scans, the VB fracture reduction is stable and no progression of thoracic kyphosis angle is observed. Furthermore since the surgical procedure, the patient is clinical symptom free. CONCLUSION: The extrapedicular percutaneus technique of VB fracture reduction with intravertebral fixation allowed to partially reduce the VB compressive fracture, rebalance the VC without any motion limitation, avoid external long-roll brace, and eliminate clinical symptoms. The procedure is minimally invasive, fast, and clinically effective. However, the technique should be restricted only to carefully selected clinical cases.


Assuntos
Fixação Interna de Fraturas/métodos , Fraturas por Compressão/cirurgia , Fraturas da Coluna Vertebral/cirurgia , Vertebroplastia/métodos , Fixação Interna de Fraturas/instrumentação , Humanos , Masculino , Próteses e Implantes
10.
Childs Nerv Syst ; 30(10): 1729-32, 2014 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-25119152

RESUMO

PURPOSE: The aim of the article is to present the new hybrid technique for ventriculoatrial shunt implantation. METHODS: Two-and-a-half-year-old boy needed ventriculoatrial shunt implantation due to communicating hydrocephalus and impaired absorptive ability of the peritoneum. Because of a complete occlusion of the right internal jugular vein and critical stenosis of a distal part of the left internal jugular vein, the procedure was performed under fluoroscopy guidance in the catheterisation laboratory equipped with a 3-dimensional single plane angiography machine (Philips Allura--The Netherlands). At the level of critical stenosis of the left jugular vein, it was decided to perform a percutaneous venous balloon angioplasty. This procedure allowed inserting the ventriculoatrial shunt into the right atrium. RESULTS: The whole postoperative period was uneventful. Now, the child is free from symptoms of increased intracranial pressure. The boy has been followed for 9 months. In the control MRI examination, the ventricular system did not change as compared with the previous study. CONCLUSION: The disadvantage of the hemodynamic technique is a higher dose of X-ray irradiation in comparison to other techniques. The hybrid technique should be reserved only to very complicated cases.


Assuntos
Ventrículos Cerebrais/cirurgia , Derivações do Líquido Cefalorraquidiano/métodos , Hidrocefalia/cirurgia , Cateterismo , Pré-Escolar , Humanos , Hidrocefalia/diagnóstico por imagem , Masculino , Flebografia , Tomografia Computadorizada por Raios X
11.
Ginekol Pol ; 85(12): 16-22, 2014 Dec.
Artigo em Polonês | MEDLINE | ID: mdl-25669060

RESUMO

OBJECTIVE: The aim of the study was to establish optimal diagnostic and therapeutic scheme and to assess the efficacy of intrauterine therapy of hydrocephalus. MATERIAL AND METHODS: The study was carried out between 1992-2012 on the total of 222 fetuses with hydro- cephalus, using Orbis-Sigma and ACCU-Flow valves (168 cases) and Cook8 shunts, according to a strictly defined diagnostic and therapeutic scheme. RESULTS: In the first stage of the study (between 1992-2001), a total of 168 fetuses with prenatally diagnosed hydrocephalus received intrauterine therapy In 91.6% of the cases the therapy resulted in a decreased size of cerebral ventricles. The valve dislocated in 23 cases (13.6%). Preterm delivery occurred in 44% of the affected neonates. Severe mental impairment occurred in 17.76%, average in 36.8%, and slight in 32.9% of the infants. Normal mental development at the age of 3 was observed in 12.5% of the children. A total of 11.2% of chldren did not require further neurosurgical treatment. In the second stage of the study (between 2006-2012) after therapy the size of the right lateral cerebral ventricle decreased by 54.76% (average of27.54 mm to 12.46 mm) and the left lateral cerebral ventricle decreased by 53.12% (average of 26.41 mm to 12.38 mm) (p=0.0018). The maximum and minimum width of the cerebral cortex increased by 23.06% and 27% (average of 9.04 mm to 11.75 mm vs. 3.65 mm to 5 mm), respectively Early complications were observed in 22% of the cases: PROM (6), intrauterine fetal death (4), intrauterine infection (1), and premature detachment of the placenta (1). Average gestational age at delivery was 34 weeks, and 24% of the patients delivered at term. CONCLUSIONS: Implantation of ventriculoamniotic shunts proved to be an effective form of therapy resulting in normalization of intracranial pressure. In both stages of therapy reduction of ventricular size in patients with hydrocephalus and good neurological outcome (45.4% in I stage, 60% in II stage) were observed. In the second stage of therapy the size of lateral brain ventricles after fetal therapy was significantly lower (54%). A total of 18% of the neonates did not require neurosurgical treatment.


Assuntos
Derivações do Líquido Cefalorraquidiano/métodos , Doenças Fetais/cirurgia , Terapias Fetais/métodos , Hidrocefalia/cirurgia , Resultado da Gravidez , Feminino , Humanos , Hidrocefalia/embriologia , Recém-Nascido , Gravidez , Resultado do Tratamento
12.
Diagnostics (Basel) ; 14(10)2024 May 13.
Artigo em Inglês | MEDLINE | ID: mdl-38786301

RESUMO

We present a case of a neonate born with prenatal diagnosis of Cantrell syndrome and ectopia cordis. This extremely rare congenital disorder underscores the significant need for multimodality imaging to plan further management. The aim of the study was to present the thoracoabdominal syndrome using a three-dimensional computed tomography angiography. The CT scans confirmed complex intracardiac defects consisting of tetralogy of Fallot, total anomalous pulmonary venous return and persistent left superior vena cava. In conclusion, Cantrell syndrome necessitates a multidisciplinary approach, from the onset of the prenatal diagnosis followed by prompt medical imaging and surgical interventions after birth. The thoracoabdominal wall defect including complete ectopia cordis is an extremely rare disorder with a fatal outcome.

13.
Life (Basel) ; 14(6)2024 Jun 10.
Artigo em Inglês | MEDLINE | ID: mdl-38929723

RESUMO

BACKGROUND: Costello syndrome (CS) is a rare genetic syndrome in which, due to the occurrence of a mutation in the HRAS gene on chromosome 11 that causes the manifestation, a set of features such as a characteristic appearance, many congenital defects, intellectual disability and a genetic predisposition to cancer, friendly personality, and others can be identified. CS is very rare, with an incidence of ~1/300,000, but it belongs to one of the largest groups of congenital syndromes, called RASopathies, occurring with an incidence of 1/1000 people. Scoliosis and kyphosis, as well as other spinal defects, are common, in 63% and 58% of patients, respectively, and a study conducted among adult patients showed the presence of scoliosis in 75% of patients; there may be excessive lordosis of the lumbar section and inverted curvatures of the spine (lordosis in the thoracic section and kyphosis in the lumbar section). The aim of our study is to present a case report of treatment of severe scoliosis of 130 degrees in a 14-year-old patient with Costello syndrome, with coexisting Chiari II syndrome and syrinx in the absence of skeletal maturity. This patient underwent foramen magnum decompression 3 months before planned surgical correction for severe scoliosis. The patient was qualified for surgical treatment using magnetically controlled growing rods (MCGR). After spine surgery using MCGR, we gradually performed MCGR distraction over the next 2 years; we performed the final surgery, conversion to posterior spinal fusion (PSF) with simultaneous multi-level Ponte osteotomy, which gave a very good and satisfactory surgical result. In the perioperative period, two serious complications occurred: pneumothorax caused by central catheter and gastrointestinal bleeding due to previously undiagnosed gastrointestinal varices. This case shows that the treatment of severe and neglected scoliosis is complicated and requires special preparation and a surgical plan with other cooperating specialists. The scoliosis was corrected from 130 degrees to approximately 48 degrees, sagittal balance was significantly improved, and the surgical outcome was very pleasing, significantly improving quality of life and function for the patient.

14.
PLoS One ; 19(8): e0308131, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-39121090

RESUMO

Shunt infection is one of the most common complications of conventional hydrocephalus treatment. The route of invasion of a pathogen can modify the immune response of the CNS. The aim of the study is to analyze the immune response to shunt infection caused by S. epidermidis in children with hydrocephalus. The immune response to the pathogen will be analyzed on the basis of, inter alia, simple laboratory test results, such as changes in the pattern of white blood cells, including neutrophils, monocytes, and lymphocytes. The entire study analyzes changes in general parameters of the cerebrospinal fluid (pleocytosis, protein level, glucose level) and in levels of selected interleukins (IL-6, CXCL8 / IL-8, CCL3 / MIP-1a) in the cerebrospinal fluid. The clinical material analyzed in the study was collected in 2010-2014. The study group consisted of 30 patients, who were admitted to the hospital due to their first-ever episode of valve dysfunction caused by S. epidermidis infection. The control group consisted of 30 children who also suffered from congenital hydrocephalus but had not been operated on before. The most pronounced response to CSF infection in the study group was a significant increase in the counts of all investigated WBC lines in the samples collected immediately after the patients' admission to the ward. The earliest aberration of the CSF was a significant increase in protein level. An infection of a ventriculoperitoneal shunt caused by S. epidermidis evokes a very early peripheral blood response. In children affected by a ventriculoperitoneal valve infection, the humoral immune response detected in the cerebrospinal fluid precedes the increase in the level of pleocytosis. The highest level of cytokines in the cerebrospinal fluid is achieved when the pathogens are cleared. Phagocytes, and, in particular, monocytes, play an important role in the normalization of the cerebrospinal fluid parameters after the elimination of S. epidermidis. The local immune response of the central nervous system plays an important role in extinguishment of the inflammatory process.


Assuntos
Hidrocefalia , Leucócitos , Staphylococcus epidermidis , Derivação Ventriculoperitoneal , Humanos , Hidrocefalia/cirurgia , Hidrocefalia/líquido cefalorraquidiano , Hidrocefalia/etiologia , Derivação Ventriculoperitoneal/efeitos adversos , Masculino , Feminino , Lactente , Pré-Escolar , Staphylococcus epidermidis/isolamento & purificação , Criança , Infecções Estafilocócicas/líquido cefalorraquidiano , Infecções Estafilocócicas/sangue , Infecções Estafilocócicas/microbiologia , Contagem de Leucócitos
15.
Diagnostics (Basel) ; 14(7)2024 Apr 05.
Artigo em Inglês | MEDLINE | ID: mdl-38611686

RESUMO

Open-source artificial intelligence models (OSAIM) find free applications in various industries, including information technology and medicine. Their clinical potential, especially in supporting diagnosis and therapy, is the subject of increasingly intensive research. Due to the growing interest in artificial intelligence (AI) for diagnostic purposes, we conducted a study evaluating the capabilities of AI models, including ChatGPT and Microsoft Bing, in the diagnosis of single-curve scoliosis based on posturographic radiological images. Two independent neurosurgeons assessed the degree of spinal deformation, selecting 23 cases of severe single-curve scoliosis. Each posturographic image was separately implemented onto each of the mentioned platforms using a set of formulated questions, starting from 'What do you see in the image?' and ending with a request to determine the Cobb angle. In the responses, we focused on how these AI models identify and interpret spinal deformations and how accurately they recognize the direction and type of scoliosis as well as vertebral rotation. The Intraclass Correlation Coefficient (ICC) with a 'two-way' model was used to assess the consistency of Cobb angle measurements, and its confidence intervals were determined using the F test. Differences in Cobb angle measurements between human assessments and the AI ChatGPT model were analyzed using metrics such as RMSEA, MSE, MPE, MAE, RMSLE, and MAPE, allowing for a comprehensive assessment of AI model performance from various statistical perspectives. The ChatGPT model achieved 100% effectiveness in detecting scoliosis in X-ray images, while the Bing model did not detect any scoliosis. However, ChatGPT had limited effectiveness (43.5%) in assessing Cobb angles, showing significant inaccuracy and discrepancy compared to human assessments. This model also had limited accuracy in determining the direction of spinal curvature, classifying the type of scoliosis, and detecting vertebral rotation. Overall, although ChatGPT demonstrated potential in detecting scoliosis, its abilities in assessing Cobb angles and other parameters were limited and inconsistent with expert assessments. These results underscore the need for comprehensive improvement of AI algorithms, including broader training with diverse X-ray images and advanced image processing techniques, before they can be considered as auxiliary in diagnosing scoliosis by specialists.

16.
J Clin Med ; 13(14)2024 Jul 09.
Artigo em Inglês | MEDLINE | ID: mdl-39064053

RESUMO

Background: Open-source artificial intelligence models (OSAIMs) are increasingly being applied in various fields, including IT and medicine, offering promising solutions for diagnostic and therapeutic interventions. In response to the growing interest in AI for clinical diagnostics, we evaluated several OSAIMs-such as ChatGPT 4, Microsoft Copilot, Gemini, PopAi, You Chat, Claude, and the specialized PMC-LLaMA 13B-assessing their abilities to classify scoliosis severity and recommend treatments based on radiological descriptions from AP radiographs. Methods: Our study employed a two-stage methodology, where descriptions of single-curve scoliosis were analyzed by AI models following their evaluation by two independent neurosurgeons. Statistical analysis involved the Shapiro-Wilk test for normality, with non-normal distributions described using medians and interquartile ranges. Inter-rater reliability was assessed using Fleiss' kappa, and performance metrics, like accuracy, sensitivity, specificity, and F1 scores, were used to evaluate the AI systems' classification accuracy. Results: The analysis indicated that although some AI systems, like ChatGPT 4, Copilot, and PopAi, accurately reflected the recommended Cobb angle ranges for disease severity and treatment, others, such as Gemini and Claude, required further calibration. Particularly, PMC-LLaMA 13B expanded the classification range for moderate scoliosis, potentially influencing clinical decisions and delaying interventions. Conclusions: These findings highlight the need for the continuous refinement of AI models to enhance their clinical applicability.

17.
Cancers (Basel) ; 16(12)2024 Jun 20.
Artigo em Inglês | MEDLINE | ID: mdl-38927985

RESUMO

Worldwide, lung cancer remains the predominant cause of cancer cases and deaths and poses significant health challenges, with surgical resection being a key treatment. Post-surgery, patients often experience functional impairments. This study aimed to develop a comprehensive ICF version for assessing the functional profile and disability in lung cancer patients post-thoracic surgery undergoing pulmonary rehabilitation using the ICF and WHODAS 2.0 tool. We analyzed the correlation between the ICF Core Set and WHODAS 2.0 data to understand the impact on daily functioning. This study included 50 patients (23 F, 27 M) from the Clinic of Thoracic Surgery and Respiratory Rehabilitation in Lodz. Essential ICF codes were determined using the Delphi method, and assessments were conducted on the third day post-operation. Statistical analyses included various tests with α = 0.05. The results showed no impairments in voice functions (b310), respiration rates (b4400), and diaphragm functions (b4451), but there were significant issues with chest pain (b28011), respiratory muscle functions (b445), exercise tolerance (b455), and muscle endurance (b740). In Activities and Participation and Environmental Factors, most codes were not problematic, except for employment (d845, d850) and atmospheric pressure (e2252). Significant correlations were found between mobility limitations (d410, d460) and self-care (d510, d540) with the WHODAS 2.0 results. The comprehensive ICF Core Set effectively described the functional profile of post-surgery patients, confirming its utility and highlighting the impact of disability on daily functioning.

18.
Diagnostics (Basel) ; 13(12)2023 Jun 19.
Artigo em Inglês | MEDLINE | ID: mdl-37371003

RESUMO

We present a case of a child who was transported to the Neurosurgery Clinic from another hospital for the purpose of performing a surgical procedure of the spinal myelomeningocele. On the first day of the stay, a set of tests was performed, including an anterior-posterior (AP) projection X-ray, which clearly showed a developmental defect in the lumbar-sacral section of the spine. In the follow-up physical examination, there was a depression of the skin on the right side of the surgical scar after closing the open myelomeningocele. In the follow-up MRI of the lumbar-sacral section, an extremely rare congenital anterior dislocation of the sacrococcygeal bone was unexpectedly visualized. Despite recommendations for further diagnostics, the patient did not attend the required follow-up examinations. In the final section, we provide a general summary of the literature on rare developmental defects of the spine in children.

19.
Diagnostics (Basel) ; 13(13)2023 Jun 22.
Artigo em Inglês | MEDLINE | ID: mdl-37443536

RESUMO

Assessing severe scoliosis requires the analysis of posturographic X-ray images. One way to analyse these images may involve the use of open-source artificial intelligence models (OSAIMs), such as the contrastive language-image pretraining (CLIP) system, which was designed to combine images with text. This study aims to determine whether the CLIP model can recognise visible severe scoliosis in posturographic X-ray images. This study used 23 posturographic images of patients diagnosed with severe scoliosis that were evaluated by two independent neurosurgery specialists. Subsequently, the X-ray images were input into the CLIP system, where they were subjected to a series of questions with varying levels of difficulty and comprehension. The predictions obtained using the CLIP models in the form of probabilities ranging from 0 to 1 were compared with the actual data. To evaluate the quality of image recognition, true positives, false negatives, and sensitivity were determined. The results of this study show that the CLIP system can perform a basic assessment of X-ray images showing visible severe scoliosis with a high level of sensitivity. It can be assumed that, in the future, OSAIMs dedicated to image analysis may become commonly used to assess X-ray images, including those of scoliosis.

20.
J Pers Med ; 13(12)2023 Dec 09.
Artigo em Inglês | MEDLINE | ID: mdl-38138922

RESUMO

Open-source artificial intelligence models are finding free application in various industries, including computer science and medicine. Their clinical potential, especially in assisting diagnosis and therapy, is the subject of increasingly intensive research. Due to the growing interest in AI for diagnostics, we conducted a study evaluating the abilities of AI models, including ChatGPT, Microsoft Bing, and Scholar AI, in classifying single-curve scoliosis based on radiological descriptions. Fifty-six posturographic images depicting single-curve scoliosis were selected and assessed by two independent neurosurgery specialists, who classified them as mild, moderate, or severe based on Cobb angles. Subsequently, descriptions were developed that accurately characterized the degree of spinal deformation, based on the measured values of Cobb angles. These descriptions were then provided to AI language models to assess their proficiency in diagnosing spinal pathologies. The artificial intelligence models conducted classification using the provided data. Our study also focused on identifying specific sources of information and criteria applied in their decision-making algorithms, aiming for a deeper understanding of the determinants influencing AI decision processes in scoliosis classification. The classification quality of the predictions was evaluated using performance evaluation metrics such as sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), accuracy, and balanced accuracy. Our study strongly supported our hypothesis, showing that among four AI models, ChatGPT 4 and Scholar AI Premium excelled in classifying single-curve scoliosis with perfect sensitivity and specificity. These models demonstrated unmatched rater concordance and excellent performance metrics. In comparing real and AI-generated scoliosis classifications, they showed impeccable precision in all posturographic images, indicating total accuracy (1.0, MAE = 0.0) and remarkable inter-rater agreement, with a perfect Fleiss' Kappa score. This was consistent across scoliosis cases with a Cobb's angle range of 11-92 degrees. Despite high accuracy in classification, each model used an incorrect angular range for the mild stage of scoliosis. Our findings highlight the immense potential of AI in analyzing medical data sets. However, the diversity in competencies of AI models indicates the need for their further development to more effectively meet specific needs in clinical practice.

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