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Traumatic brain injury (TBI) is a leading cause of morbidity and mortality in children. Head computed tomography (CT) is frequently utilized for evaluating trauma-related characteristics, selecting treatment options, and monitoring complications in the early stages. This study assessed the relationship between cranial CT findings and early and late neurological outcomes in pediatric TBI patients admitted to the pediatric intensive care unit (PICU). The study included children aged 1 month to 18 years who were admitted to the PICU due to TBI between 2014 and 2020. Sociodemographic data, clinical characteristics, and cranial CT findings were analyzed. Patients were categorized based on their Glasgow Coma Scale (GCS) score. Of the 129 patients, 83 (64%) were male, and 46 (36%) were female, with a mean age of 6.8 years. Falls (n = 51, 39.5%) and in-vehicle traffic accidents (n = 35, 27.1%) were the most common trauma types observed. Normal brain imaging findings were found in 62.7% of the patients, while 37.3% exhibited intracranial pathology. Hemorrhage was the most frequent CT finding. Severe TBI (n = 26, p = 0.032) and mortality (n = 9, p = 0.017) were more prevalent in traffic accidents. The overall mortality rate in the study population was 10.1%. In children with TBI, cranial CT imaging serves as an essential initial method for patients with neurological manifestations. Particularly, a GCS score of ≤ 8, multiple hemorrhages, diffuse cerebral edema, and intraventricular bleeding are associated with sequelae and mortality.
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Lesões Encefálicas Traumáticas , Escala de Coma de Glasgow , Tomografia Computadorizada por Raios X , Humanos , Feminino , Masculino , Lesões Encefálicas Traumáticas/diagnóstico por imagem , Lesões Encefálicas Traumáticas/mortalidade , Criança , Pré-Escolar , Adolescente , Lactente , Estudos Retrospectivos , Unidades de Terapia Intensiva PediátricaRESUMO
BACKGROUND: Liver involvement is an important cause of morbidity and mortality in patients with cystic fibrosis (CF). While liver biopsy is the gold standard for demonstrating involvement, its invasiveness prompts a search for noninvasive alternatives. OBJECTIVE: To evaluate liver involvement in pediatric patients with CF (versus healthy controls) using magnetic resonance (MR) elastography/spectroscopy and to correlate the imaging findings with clinical/laboratory characteristics. MATERIALS AND METHODS: This was a single-center, prospective cross-sectional study conducted between April 2020 and March 2022 in patients with CF versus healthy controls. Patients with CF were divided into two subgroups: those with CF-related liver disease and those without. MR images were acquired on a 1.5-tesla machine. Kilopascal (kPa) values were derived from processing MR elastography images. MR spectroscopy was used to measure liver fat fraction, as an indication of hepatosteatosis. Groups were compared using either the Student's t test or the MannâWhitney U test. The chi-square test or Fisher's exact test were used to compare qualitative variables. RESULTS: Fifty-one patients with CF (12 ± 3.3 years, 32 boys) and 24 healthy volunteers (11.1 ± 2.4 years, 15 boys) were included in the study. Median liver stiffness (P=0.003) and fat fraction (P=0.03) were higher in the CF patients than in the controls. Median liver stiffness values were higher in CF patients with CF-related liver disease than in those without CF-related liver disease (P=0.002). Liver stiffness values of CF patients with high alanine aminotransferase (ALT), high gamma-glutamyl transferase, and thrombocytopenia were found to be higher than those without (P=0.004, P<0.001, P<0.001, respectively). Only the high ALT group showed a high fat fraction (P=0.002). CONCLUSIONS: Patients with CF had higher liver stiffness than the control group, and patients with CF-related liver disease had higher liver stiffness than both the CF patients without CF-related liver disease and the control group. Patients with CF had a higher fat fraction than the control group. Noninvasive assessment of liver involvement using MR elastography/spectroscopy can support the diagnosis of CF-related liver disease and the follow-up of patients with CF.
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Fibrose Cística , Técnicas de Imagem por Elasticidade , Hepatopatias , Masculino , Humanos , Criança , Técnicas de Imagem por Elasticidade/métodos , Cirrose Hepática/patologia , Estudos Prospectivos , Fibrose Cística/diagnóstico por imagem , Fibrose Cística/patologia , Estudos Transversais , Fígado/diagnóstico por imagem , Fígado/patologia , Hepatopatias/patologia , Imageamento por Ressonância Magnética/métodosRESUMO
Congenital hepatic fibrosis (CHF) is a developmental liver disease that is caused by mutations in genes that encode ciliary proteins and is characterized by bile duct dysplasia and portal fibrosis. Recent work has demonstrated that mutations in ANKS6 can cause CHF due to its role in bile duct development. Here, we report a novel ANKS6 mutation, which was identified in an infant presenting with neonatal jaundice due to underlying biliary abnormalities and liver fibrosis. Molecular analysis revealed that ANKS6 liver pathology is associated with the infiltration of inflammatory macrophages to the periportal fibrotic tissue and ductal epithelium. To further investigate the role of macrophages in CHF pathophysiology, we generated a novel liver-specific Anks6 knockout mouse model. The mutant mice develop biliary abnormalities and rapidly progressing periportal fibrosis reminiscent of human CHF. The development of portal fibrosis in Anks6 KO mice coincided with the accumulation of inflammatory monocytes and macrophages in the mutant liver. Gene expression and flow cytometric analysis demonstrated the preponderance of M1- over M2-like macrophages at the onset of fibrosis. A critical role for macrophages in promoting peribiliary fibrosis was demonstrated by depleting the macrophages with clodronate liposomes which effectively reduced inflammatory gene expression and fibrosis, and ameliorated tissue histology and biliary function in Anks6 KO livers. Together, this study demonstrates that macrophages play an important role in the initiation of liver fibrosis in ANKS6-deficient livers and their therapeutic elimination may provide an avenue to mitigate CHF in patients.
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Proteínas de Transporte/metabolismo , Colestase/patologia , Cirrose Hepática/metabolismo , Fígado/metabolismo , Macrófagos/metabolismo , Animais , Modelos Animais de Doenças , Expressão Gênica/fisiologia , Inflamação/metabolismo , Inflamação/patologia , Fígado/patologia , Cirrose Hepática/patologia , Macrófagos/patologia , Camundongos , Camundongos Endogâmicos C57BL , Camundongos Knockout , Monócitos/metabolismo , Monócitos/patologiaRESUMO
PURPOSE/BACKGROUND: This study aimed to compare the frequency, clinical findings, treatment practices, and outcomes of toxicity to old-generation (OG) and new-generation (NG) antidepressants in our pediatric intensive care unit (PICU) by year-to-year. METHODS/PROCEDURES: The study included patients hospitalized for antidepressant poisoning during the 11-year period of January 2010 through December 2020. Antidepressants were classified as OG and NG. The groups were compared in terms of patient demographic characteristics, type of poisoning (accidental/suicidal), clinical findings, supportive and extracorporeal treatments received, and outcomes. FINDINGS/RESULTS: The study included 58 patients (NG, n = 30; OG, n = 28). The median age of the patients was 178 months (range, 13.6-215 months) and 47 patients (81%) were female. Patients admitted for only antidepressant poisoning constituted 13.3% of all poisoning cases (58/436). Of these, 22 cases (37.9%) were accidental and 36 (62.3%) were suicidal. The most common cause of poisoning was amitriptyline (24/28) in the OG group and sertraline (13/30) in the NG group. Neurological symptoms were significantly more common in the OG group (76.2% vs 23.8%), while gastrointestinal involvement was more common in the NG group (82% vs 18%; P = 0.001 and P = 0.026, respectively). Old-generation antidepressant poisoning was associated with more frequent intubation (4 vs 0 patients, P = 0.048) and longer length of PICU stay (median, 1 day [range, 1-8] vs 1 day [range, 1-4], P = 0.019). Rates of therapeutic plasma exchange and intravenous lipid emulsion therapy did not differ ( P = 0.483 and P = 0.229, respectively). IMPLICATIONS/CONCLUSIONS: In poisoned patients, proper evaluation and management of patients requiring PICU admission are vital for favorable patient outcomes.
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Antidepressivos , Hospitalização , Humanos , Criança , Feminino , Masculino , Estudos Retrospectivos , Unidades de Terapia Intensiva Pediátrica , Cuidados CríticosRESUMO
BACKGROUND: This study's objective was to investigate the incidence of acute kidney injury (AKI) in children with severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) and multisystem inflammatory syndrome (MIS-C) and to report our clinical experience. METHODS: Acute COVID-19 and MIS-C-diagnosed patients observed in two pediatric intensive care units (PICUs) between 2019 and 2021 were examined for AKI and retrospectively compared to children with AKI. RESULTS: The study comprised 163 children, of whom 98 (60.1%) were diagnosed with acute COVID-19 and 65 (39.9%) with MIS-C. AKI was observed in 40 (40.8%) of the acute COVID-19 patients and 18 (27.7%) of the MIS-C patients. Low calcium level and hypotension were linked with AKI at initial presentation (OR: 0.56, 95% CI: 0.369-0.560, p = 0.006 and OR: 3.64, 95% CI: 1.885-7.152, p = 0.001, respectively). A history of nephrotoxic medication usage played an essential role in the development of AKI in patients who acquired AKI after hospitalization (p = 0.001, odds ratio: 9.32, confidence interval: 3.106-27.973). In clinical practice, individuals with respiratory distress and cough had a high chance of having AKI (OR: 4.47, 95% confidence interval: 2.25-8,892 and OR: 3.48, 95% confidence interval: 1.76-6.88). AKI patients had a greater demand for respiratory assistance and a longer period of stay in the PICU. CONCLUSIONS: AKI in the COVID-19 and MIS-C patient groups is related with increased mortality and extended hospitalization, according to the findings. These statistics imply that identifying and preventing risk factors is necessary. A higher resolution version of the Graphical abstract is available as Supplementary information.
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Injúria Renal Aguda , COVID-19 , Humanos , Criança , COVID-19/complicações , COVID-19/epidemiologia , SARS-CoV-2 , Estudos Retrospectivos , Estado Terminal , Injúria Renal Aguda/epidemiologia , Injúria Renal Aguda/etiologia , Injúria Renal Aguda/diagnóstico , Fatores de RiscoRESUMO
BACKGROUND: The neutrophil-to-lymphocyte ratio (NLR) is a simple and inexpensive inflammation biomarker that reflects systemic inflammation based on complete blood count values. AIMS: In our study, we aimed to compare the NLR values in pediatric inflammatory bowel disease (IBD) and in healthy controls, and to define NLR levels in children with IBD during diagnosis, active disease, and remission. METHODS: NLR values of patients with IBD at diagnosis, remission, and active disease of the patients were recorded retrospectively. Age- and sex-matched healthy subjects enrolled as the control group. RESULTS: Sixty-three patients with IBD and 92 healthy subjects as the control group enrolled. The mean age of the patients with IBD was 9.31 ± 5.24 years, and 57.1% were males. The mean NLR values of the patients with IBD at diagnosis and remission were significantly higher than that of healthy controls (p < 0.001). The mean NLR values of the patients at diagnosis and active disease were significantly higher than that of during remission (p < 0.001). The best cutoff of NLR for prediction of diagnosis of IBD in children was 1.46 with a sensitivity of 86.2% and specificity of 93.5%. There was no significant difference regarding NLR between patients with IBD with and without associated diseases. At diagnosis the mean NLR level of patients with Crohn's disease was significantly higher than that of ulcerative colitis (p = 0.019). CONCLUSIONS: It was shown for the first time that NLR levels were significantly increased at diagnosis and active disease of childhood IBD, compared to the remission period. We believe that NLR can be a non-invasive inflammatory biomarker that should be used in the initial evaluation and follow-up of the disease activity in children.
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Colite Ulcerativa , Doenças Inflamatórias Intestinais , Masculino , Humanos , Criança , Pré-Escolar , Adolescente , Feminino , Neutrófilos , Estudos Retrospectivos , Doenças Inflamatórias Intestinais/diagnóstico , Linfócitos , Inflamação , BiomarcadoresRESUMO
Effects of Caffeic acid phenethyl ester (CAPE) and/or PD98059 (PD) on the gene expression of Caveolin-1 (CAV1) and reduced glutathione (GSH), malondialdehyde (MDA), copper-zinc superoxide dismutase (CuZn-SOD), and catalase (CAT) enzyme activities were investigated in an experimental chronic renal failure model in rats. Eighty Wistar rats were divided into eight groups for a 28-day study: Control, CsA (Cyclosporine A), CsA-V (CsA solvent), CsA + PD (CsA + PD98059), CsA + PD + CAPE, CsA + CAPE, CAPE-V (CAPE solvent), and PD-V (PD98059 solvent). Serum blood urea nitrogen and creatinine levels, as well as histopathological findings indicated the development of renal failure in the CsA group. Kidney GSH levels decreased while MDA levels, CuZn-SOD, and CAT activities increased significantly in the CsA group compared to control indicating oxidative stress. CAV1 gene expression significantly decreased in the CsA group compared to the control. PD98059 and CAPE applications made positive improvements in the levels of the parameters investigated. PD98059 and CAPE applications in CsA given animals increased GSH and CAV1 gene expressions and decreased CuZn-SOD and CAT levels compared to the CsA group. In conclusion, it was shown that PD98059 and CAPE could attenuate the effects of chronic renal failure, and CAV1 is suggested as a therapeutic target and the inhibition of the p44/42 MAPK pathway may be a new approach for the treatment of renal degenerations.
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Antioxidantes , Falência Renal Crônica , Ratos , Animais , Antioxidantes/farmacologia , Caveolina 1/genética , Ratos Wistar , Falência Renal Crônica/tratamento farmacológico , Falência Renal Crônica/genética , Expressão Gênica , Superóxido DismutaseRESUMO
BACKGROUND: In this study, we aimed to evaluate the duration of extracorporeal membrane oxygenation (ECMO) and its effect on outcomes. Also, we sought to identify hospital mortality predictors and determine when ECMO support began to be ineffective. METHODS: This was a single-center, retrospective cohort study conducted between January 2014 and January 2022. The prolonged ECMO (pECMO) cut-off point was accepted as 14 days. RESULTS: Thirty-one (29.2%) of 106 patients followed up with ECMO had pECMO. The mean follow-up period of the patients who underwent pECMO was 22 (range, 15-72) days, and the mean age was 75 ± 72 months. According to the results of our heterogeneous study population, life expectancy decreased dramatically towards the 21st day. Hospital mortality predictors were determined in the logistic regression analysis in all ECMO groups in our study as high Pediatric Logistic Organ Dysfunction (PELOD) two score, continuous renal replacement therapy (CRRT) use, and sepsis. The pECMO mortality was 61.2% and the overall mortality was 53.0%, with the highest mortality rate in the bridge-to-transplant group (90.9%) because of lack of organ donation in our country. CONCLUSIONS: In our study, the PELOD two score, presence of sepsis, and use of CRRT were found to be in the predictors of in-hospital ECMO mortality model. Considering the complications, in the COX regression model analysis, the factors affecting the probability of dying in patients followed under ECMO were found to be bleeding, thrombosis, and thrombocytopenia.
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BACKGROUND: The initial extracorporeal membrane oxygenation (ECMO) configuration is inefficient for patient oxygenation and flow, but by adding a Y-connector, a third or fourth cannula can be used to support the system, which is called hybrid ECMO. METHODS: This was a single-center retrospective study consisting of patients receiving hybrid and standard ECMO in our PICU between January 2014 and January 2022. RESULTS: The median age of the 12 patients who received hybrid ECMO and were followed up with hybrid ECMO was 140 (range, 82-213) months. The total median ECMO duration of the patients who received hybrid ECMO was 23 (8-72) days, and the median follow-up time on hybrid ECMO was 18 (range, 3-46) days. The mean duration of follow-up in the PICU was 34 (range, 14-184) days. PICU length of stay was found to be statistically significant and was found to be longer in the hybrid ECMO group (p = 0.01). Eight (67%) patients died during follow-up with ECMO. Twenty-eight-day mortality was found to be statistically significant and was found to be higher in the standard ECMO group (p = 0.03). The hybrid ECMO mortality rate was 66% (decannulation from ECMO). The hybrid ECMO hospital mortality rate was 75%. The standard ECMO mortality rate was 52% (decannulation from ECMO). The standard ECMO hospital mortality rate was 65%. CONCLUSIONS: Even though hybrid ECMO use is rare, with increasing experience and new methods, more successful experience will be gained. Switching to hybrid ECMO from standard ECMO at the right time with the right technique can increase treatment success and survival.
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BACKGROUND: Nutritional status in primary immunodeficiencies (PID) is a major factor influencing immune defense. We aimed to evaluate the nutritional status of patients with PID. METHODS: Demographic findings and anthropometric measurements of 104 patients were recorded for this cross-sectional study. RESULTS: Combined immunodeficiencies (n = 49), predominantly antibody deficiencies (n = 28) and phagocytic system disorders (n = 17), were the major disease groups. In total, 44 (42.3%) patients had at least one anthropometric measurement below -2 standard deviations. Chronic, acute, and mixed-type malnutrition were detected in 18.3%, 16.3%, and 7.7% of the patients, respectively. No significant difference was detected among groups regarding anthropometric measurements however higher malnutrition rates were observed in 'combined immune deficiency less profound than severe combined immuno deficiency' (52%), chronic granulomatous disease (66.6%), and X-linked agammaglobulinemia (50%) patients. Severe malnutrition was present in 22 (21.2%) of the patients, although it was not significant. It was more common in the phagocytic system disorder group. All patients in the severe combined immunodeficiency group had undergone hematopoietic stem cell transplantation and 50% of them had malnutrition. There was also no significant difference regarding age, sex, anthropometric indexes (Weight for age, lenght/height for age body mass index Z-scores), malnutrition types, and prevalence of malnutrition among three major disease groups. Only the hospitalization history inversely related to body mass index and weight for age Z-scores (P < 0.0001). In patients with malnutrition, daily caloric intake was at least 20% or more below the requirement. CONCLUSIONS: Regardless of the type of immunodeficiency, nutritional status was poor in PID and hospitalization is the most important determinant of nutritional status. Even after hematopoietic stem cell transplantation, nutritional support should be continued.
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Desnutrição , Estado Nutricional , Antropometria , Estatura , Estudos Transversais , HumanosRESUMO
Regional citrate anticoagulation (RCA) is an option but citrate accumulation is risk and it is a giving up cause for this situation. This retrospective study was conducted in the pediatric intensive care unit (PICU) between May 2019 and April 2021. We investigated 47 patients with liver failure (LF) in our PICU, and RCA during continuous renal replacement therapy (CRRT) was applied to 10 (21.3%) of them. Half of them were male (n: 5/10), their mean age was 104.7 ± 66.20 months. Nine of them needed vasoactive support during follow-up. The most common indication for CRRT was hepatorenal syndrome (40%). There was no significant difference between liver transaminases and liver function tests before and after CRRT (p > 0.05). In terms of citrate toxicity of the patients, there was no significant difference between total calcium/ionized calcium, lactate level, pH and bicarbonate values before and after CRRT (p > 0.05). The mean total CRRT time was 110.2 ± 118.2 h, and the mean circuit lifespan was 43.8 ± 48.7 h; the mean number of circuits was 2.7 ± 2.4. Total Ca/ionized Ca >2.5 was a clinically relevant endpoint, but no patient interrupted dialysis for this cause. There was no complication about RCA. This study did not observe any adverse effects on acid-base status, transaminases, an increase in bilirubin during RCA-CRRT treatment in pediatric patients with LF. Total calcium/ionized calcium ratio, serum lactate level and prothrombin time level should be closely monitored daily in terms of citrate accumulation in this patient group.
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Terapia de Substituição Renal Contínua , Hepatopatias , Falência Hepática , Anticoagulantes/efeitos adversos , Cálcio , Criança , Citratos/efeitos adversos , Ácido Cítrico/uso terapêutico , Feminino , Humanos , Lactatos , Hepatopatias/complicações , Falência Hepática/induzido quimicamente , Falência Hepática/complicações , Falência Hepática/terapia , Masculino , Diálise Renal , Estudos Retrospectivos , TransaminasesRESUMO
OBJECTIVES: Colchicine intoxication is rare but potentially fatal. The toxic dose of colchicine is not well established; it has been reported that major toxicity starts after doses of 0.5 mg/kg. We aimed to evaluate the demographic, clinical aspects, treatments, and outcome of colchicine toxicity cases in the pediatric intensive care unit (PICU). METHODS: We collected the data of patients aged between 0 and 18 years, admitted to Ankara University Faculty of Medicine PICU for colchicine poisoning (n = 22), from October 1999 to January 2020, retrospectively. Data extracted from the cases included age, sex, chronic condition, time between intake of drug and admission to PICU, source of drug, amount of drug ingested, other drug intake, symptoms, clinical findings, cardiac involvement, laboratory results, time of stay in PICU, treatment, and outcome. RESULTS: Patients' age ranged from 7 months to 17 years. Median age was 86 months. The most common symptom at time of admission was vomiting, occurring in 13 (59%) of the patients. Two of the patients presented with change in mental status. Time between taking medication and applying to the hospital ranged from half an hour to 4 days. Medication intake of 3 of 22 patients was more than 0.5 mg/kg. One patient whose parents' best estimate of dose ingested was 0.48 mg/kg died because of the development of multiorgan failure. One patient who ingested 0.4 mg/kg of colchicine underwent plasma exchange and recovered without any complications. CONCLUSIONS: Colchicine poisoning has a high risk of mortality, and death can be seen in doses less than a single acute dose of 0.5 mg/kg. These patients need close monitoring because there is always a risk of them to require aggressive support. Prognosis is poor in patients who have rapidly developing hemodynamic failure.
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Unidades de Terapia Intensiva Pediátrica , Intoxicação , Adolescente , Criança , Pré-Escolar , Colchicina , Humanos , Lactente , Recém-Nascido , Intoxicação/epidemiologia , Intoxicação/terapia , Prognóstico , Estudos Retrospectivos , VômitoRESUMO
OBJECTIVE: Critically ill children often require transport to tertiary centers where higher levels of care can be provided. In this study, we aimed to evaluate the reasons for transport, complications that occurred during transport, and the clinical outcomes of the patients transferred to our tertiary pediatric intensive care unit (PICU). METHODS: This retrospective study was conducted in a tertiary pediatric hospital with 250 beds and 20 tertiary PICU beds. RESULTS: During the study period, 108 patients were transferred to our PICU. The mean age was 75.0 ± 70.5 months (range, 1-211 months), and 59 were female. Most patients (82.4%) were transported by land; 17.6% were transported by air ambulance. Fourteen patients were referred for liver transplantation, and 7 patients were referred because of a need for extracorporeal membrane oxygenation support and heart transplantation or left ventricular assist device placement. Two patients were transported by air while on extracorporeal membrane oxygenation. Complications occurred in 25% of patients. CONCLUSION: Vital signs were assesed, and certain critical interventions such as intravenous fluids and respiratory support were provided more frequently during air transport, possibly due to the fact that physicians were always present during air transport. Quality improvement of transport teams and multicenter and nationwide studies on PICU transport are needed.
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Oxigenação por Membrana Extracorpórea , Unidades de Terapia Intensiva Pediátrica , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Estudos Retrospectivos , Centros de Atenção TerciáriaRESUMO
BACKGROUND AND AIM: We aimed to examine the frequency and the characteristics of immunoglobulin G4 (IgG4)-associated autoimmune hepatitis among pediatric patients with autoimmune hepatitis. METHODS: Immunostaining for IgG and IgG4 was performed in liver biopsies of 40 pediatric patients with autoimmune hepatitis. The patients with more than 10 IgG4-positive plasma cells/high-power field were defined as IgG4-associated autoimmune hepatitis. Clinic, laboratory, and histopathological results were compared between groups. RESULTS: Among the 40 pediatric patients, 34 patients were type 1 and 6 patients were type 2 autoimmune hepatitis. Six patients (15%), four of the type 1 and two of the type 2 autoimmune hepatitis patients, were diagnosed with IgG4-associated autoimmune hepatitis. Clinical, laboratory, and histopathological data were initially similar in both forms. There was a positive correlation between IgG4-positive plasma cell count and degree of portal (r: 0.406, P: 0.009) and lobular inflammation (r: 0.37, P: 0.019), grade of interface hepatitis (r: 0.33, P: 0.03), and fibrosis (r: 0.318, P: 0.046). Time required for normalization of liver transaminases and serum IgG level was significantly shorter in IgG4-associated autoimmune hepatitis (3.3 ± 0.5 vs 6.6 ± 3.5 for alanine aminotransferase, 3.7 ± 0.8 vs 6.7 ± 1.2 for aspartate aminotransferase, 4.3 ± 1.2 vs 7.1 ± 2.7 for gamma-glutamyl transpeptidase, and 7.2 ± 3.1 vs 12.8 ± 4.5 for IgG). CONCLUSIONS: Immunoglobulin G4-associated autoimmune hepatitis can be found in pediatric age group and also in type 2 autoimmune hepatitis patients. As steroid response may be better in IgG4-associated autoimmune hepatitis, biopsy specimens should be evaluated for this entity at diagnosis.
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Hepatite Autoimune/diagnóstico , Hepatite Autoimune/patologia , Doença Relacionada a Imunoglobulina G4/diagnóstico , Doença Relacionada a Imunoglobulina G4/patologia , Adolescente , Biomarcadores/metabolismo , Criança , Pré-Escolar , Feminino , Hepatite Autoimune/imunologia , Humanos , Imunoglobulina G/sangue , Doença Relacionada a Imunoglobulina G4/imunologia , Fígado/enzimologia , Fígado/imunologia , Testes de Função Hepática , Masculino , Estudos Retrospectivos , Fatores de Tempo , Transaminases/metabolismoRESUMO
BACKGROUND: This study evaluated cardiac function using tissue Doppler echocardiography and assessed electrocardiographic findings in children diagnosed with Wilson's disease. METHOD: Asymptomatic patients with a diagnosis of Wilson's disease (n = 43) were compared to healthy controls (n = 37) that were age and gender matched. RESULTS: The standard electrocardiographic and conventional echocardiographic examinations were similar in both groups. The left ventricular ejection fraction, shortening fraction, and diastolic function were not significantly different between the two groups. The Tei index for mitral lateral, mitral septal, tricuspid lateral, tricuspid septal, and inter-ventricular septum on tissue Doppler echocardiography was higher in the patient group, yet it did not reach statistical significance. Mitral lateral and septal systolic annular velocity values were significantly lower in the patient group when compared to the control group (p = 0.02 and 0.04, respectively). Also, mitral lateral and septal isovolumetric contraction time values were higher in the patient group (p = 0.04). Although the left ventricular values were not significantly different, relative left ventricular wall thickness was higher in the patient group when compared to the control group, and concentric remodelling in the left ventricle was found in 7 (16%) of 42 patients. QT interval (p = 0.02) and P-wave dispersion values (p = 0.04) were significantly higher in the patient group compared to the control group, and these tend to predict arrhythmias. CONCLUSION: Our study based on the tissue Doppler echocardiography assessment indicated a subclinical systolic, rather than diastolic, dysfunction in the myocardium with increased QT interval and P-wave dispersion, despite the young age of the patients and short disease duration.
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Ecocardiografia Doppler de Pulso/métodos , Eletrocardiografia/métodos , Ventrículos do Coração/fisiopatologia , Degeneração Hepatolenticular/fisiopatologia , Contração Miocárdica/fisiologia , Volume Sistólico/fisiologia , Função Ventricular Esquerda/fisiologia , Adolescente , Doenças Assintomáticas , Criança , Pré-Escolar , Diástole , Feminino , Seguimentos , Ventrículos do Coração/diagnóstico por imagem , Degeneração Hepatolenticular/diagnóstico , Humanos , Lactente , Masculino , Estudos Prospectivos , Sístole , Adulto JovemRESUMO
Our aim was to investigate the effects of Achillea millefolium on wound healing in second-degree burns formed in diabetic rats. This study was conducted at the Kafkas University Experimental Research Center. 20 Sprague Dawley rats were divided into 2 groups. Group I (n=10 rats) was the Control group and contained the diabetic rats with burn injury. Group II (n=10 rats) was the group where burn injury was created and Achillea millefolium was administered to diabetic rats. The backs of the rats were shaved so as to include 30% of the body area. A 10% lanolin extract was administered for 14 days after the burn injury was created. Tissue was obtained from the burn area of the rats sacrificed. No significant difference was found in Group II in terms of the severity of the dermatitis and inflammatory cell reactions when compared to Group I. No significant difference was observed between FGFR1immunoreactivity in the epidermis and dermis in Group I. While FGFR1immunoreactivity in 3 rats in Group II was similar to Group I, strong immunore activity that was more prominent in the epidermis was found in 7 rats in Group II. We believe that Achillea millefolium contributes to wound healing in burn injury due to its antioxidant and anti-inflammatory properties.
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Achillea/química , Queimaduras/tratamento farmacológico , Diabetes Mellitus Experimental/tratamento farmacológico , Fitoterapia/métodos , Extratos Vegetais/uso terapêutico , Animais , Queimaduras/complicações , Queimaduras/metabolismo , Queimaduras/patologia , Diabetes Mellitus Experimental/complicações , Diabetes Mellitus Experimental/metabolismo , Masculino , Extratos Vegetais/química , Ratos , Receptor Tipo 1 de Fator de Crescimento de Fibroblastos/metabolismo , Cicatrização/efeitos dos fármacosRESUMO
The use of extended criteria donors who might have previously been deemed unsuitable is an option to increase the organ supply for transplantation. This report presents a pediatric case of a successful liver transplantation from a donor with ß-thalassemia intermedia. A patient, 6-year-old female, with a diagnosis of cryptogenic liver cirrhosis underwent deceased donor liver transplantation from a thalassemic donor. Extreme hyperferritinemia was detected shortly after transplantation. The most probable cause of hyperferritinemia was iron overload secondary to transplantation of a hemosiderotic liver. Hepatocellular injury due to acute graft rejection might have contributed to elevated ferritin levels by causing release of stored iron from the hemosiderotic liver graft. Iron chelation and phlebotomy therapies were started simultaneously in the early postoperative period to avoid iron-related organ toxicity and transplant failure. Follow-up with monthly phlebotomies after discharge yielded a favorable outcome with normal transplant functions. Thalassemia intermedia patients can be candidates of liver donors to decrease pretransplant waitlist mortality. After transplantation of a hemosiderotic liver, it is important to monitor the recipient in terms of iron overload and toxicity. Early attempts to lower iron burden including chelation therapy and/or phlebotomy should be considered to avoid organ toxicity and transplant failure.
Assuntos
Sobrecarga de Ferro/diagnóstico , Cirrose Hepática/congênito , Cirrose Hepática/cirurgia , Transplante de Fígado , Talassemia beta , Quelantes/química , Terapia por Quelação/métodos , Criança , Contraindicações , Desferroxamina/uso terapêutico , Feminino , Ferritinas/sangue , Humanos , Ferro/sangue , Quelantes de Ferro/uso terapêutico , Sobrecarga de Ferro/terapia , Seleção de Pacientes , Flebotomia , Doadores de TecidosRESUMO
BACKGROUND: The aim of this study was to investigate whether Helicobacter pylori plays a role in the pathogenesis and severity of non-cystic fibrosis bronchiectasis, and its relationship with gastroesophageal reflux (GER). METHODS: Forty-one patients and 16 controls between 5 and 18 years of age were enrolled. H. pylori was investigated on polymerase chain reaction and culture in gastric juice (GJ) and bronchoalveolar lavage fluid (BALF). Urea breath test (UBT) was also used for defining H. pylori infection. GER was detected on 24 h pH monitoring or scintigraphy. Computed tomography (CT) scoring was used to quantify the severity and extent of bronchiectasis. RESULTS: Nine patients (22%) in the bronchiectasis group (BG) and three patients (18.8%) in the control group (CG) had H. pylori-positive BALF. Sixteen BG patients (39%) and seven CG patients (43.8%) had H. pylori-positive GJ. UBT was positive in 11 BG patients (26.8%) and in three CG patients (18.8%). H. pylori positivity in BALF, GJ and UBT was not significantly different between the two groups (P > 0.05). Six patients with GER and five patients without GER in BG had H. pylori-positive BALF and GJ (P = 0.827). No association was found between BALF H. pylori positivity and forced expiratory volume in 1 s (FEV1 ) in BG. CT score was significantly higher in BG patients with H. pylori-positive compared with H. pylori-negative BALF (P < 0.05). CONCLUSIONS: Helicobacter pylori is not associated with the pathogenesis of bronchiectasis but it may be responsible for the severity of the disease.