Lo shock settico nei primi tre mesi di vita

2021 ◽  
Vol 40 (9) ◽  
pp. 567-575
Author(s):  
Sara Crestani ◽  
Erica Passini ◽  
valentina Spaggiari ◽  
Carlotta Toffoli ◽  
Alessandra Boncompagni ◽  
...  
Keyword(s):  
Septic Shock ◽  
Clinical Manifestations ◽  
Signs And Symptoms ◽  
Epidemiological Data ◽  
International Studies ◽  
Term Outcome ◽  
Adequate Treatment ◽  
Long Term Outcome ◽  
Sepsis And Septic Shock

Shock is a generalized tissue hypoperfusion that leads to severe cellular distress and complicates some cases of paediatric and neonatal sepsis. Although a reduction in associated sepsis mortality has been observed in the last decades, it remains one of the most important causes of death or long-term neurodevelopmental disabilities in children. Prompt recognition of this condition is therefore essential to improve survival and long-term outcome. The paediatrician and the neonatologist must therefore be able to promptly recognize the signs and symptoms of sepsis and septic shock to set up an adequate treatment according to the most recent international guidelines. This article provides epidemiological data from Italian and international studies, describes the pathophysiology and clinical manifestations of sepsis and septic shock, as well as the therapeutic indications according to very recent recommendations.

Long-Term Outcomes of Patients with Hydrocephalus Secondary to Tectal Plate Glioma versus Idiopathic Aqueductal Stenosis: Results from a Single Center

2021 ◽  
pp. 1-11
Author(s):  
Hamid Reza Niknejad ◽  
Melissa Frederickx ◽  
Emiel Salaets ◽  
Jurgen Lemiere ◽  
Lieven Lagae ◽  
...  
Keyword(s):  
Aqueductal Stenosis ◽  
Maximum Diameter ◽  
Benign Tumors ◽  
Term Outcome ◽  
Adequate Treatment ◽  
Long Term Outcome ◽  
Tectal Plate ◽  
The Mean

<b><i>Introduction:</i></b> Tectal plate gliomas (TPG) constitute a distinct entity of benign tumors of the brain stem which show an indolent clinical course. Adequate treatment of secondary hydrocephalus is undoubtedly a major factor in the outcome. However, little is known about to what degree the tumor itself determines the long-term outcome of these patients. <b><i>Methods:</i></b> We retrospectively analyzed and compared the clinical and radiological data of 16 pediatric TPG patients with data of 12 pediatric idiopathic aqueductal stenosis (IAS) patients treated in our center from 1988 to 2018. For both groups, we assessed the long-term outcome in terms of hydrocephalus management, and for the TPG group, we assessed tumor growth during follow-up. In a separate prospective part of the study, we performed a neuropsychological evaluation in a subgroup of patients using a standardized testing battery, covering intelligence, learning, memory, executive functions, and an inventory on depression. <b><i>Results:</i></b> In the TPG group, the mean clinical and radiological follow-up was 84 and 70 months, respectively. On average, the maximum diameter of the tumor increased by 11% (<i>p</i> = 0.031) and the estimated tumor volume with 35% (<i>p</i> = 0.026) on radiological follow-up. The fronto-occipital horn ratio (FOHR) decreased by 23% on average after treatment. In the IAS group, the mean clinical and radiological follow-up was 117 and 85 months, respectively. In this group, the FOHR decreased by 21% on average. Neurocognitive testing revealed significant higher scores in the TPG group on global intelligence (TPG = 109, IAS = 85.5, <i>U</i> = 3, <i>p</i> &#x3c; 0.01, <i>z</i> = −2.71), performance (TPG= 100, IAS = 85, <i>U</i> = 7, <i>p</i> = 0.03, <i>z</i> = −2.2), and verbal intelligence (TPG = 122, IAS = 91.5, <i>U</i> = 2, <i>p</i> &#x3c; 0.00, <i>z</i> = −2.87) as well as working memory (TPG = 109.5, IAS = 77, <i>U</i> = 0.5, <i>p</i> = 0.01, <i>z</i> = −2.46). <b><i>Conclusion:</i></b> Our results suggest that the long-term outcome in TPG patients is acceptable and that cognition is substantially better preserved than in patients with IAS. This puts the idea of a significant contribution of the tumoral mass to disease outcome on the long term in question. Adequate and prompt management of hydrocephalus is the most important factor in long-term cognitive outcome.

scholarly journals B Cell Activating Factor, Renal Allograft Antibody-Mediated Rejection, and Long-Term Outcome

2018 ◽  
Vol 2018 ◽  
pp. 1-5 ◽  
Author(s):  
Haiyan Xu ◽  
Xiaozhou He ◽  
Renfang Xu
Keyword(s):  
B Cell ◽  
Renal Allograft ◽  
Delayed Diagnosis ◽  
Clinical Manifestations ◽  
Direct Proof ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Antibody Mediated Rejection ◽  
B Cell Activating Factor

Antibody-mediated rejection (ABMR) of renal allograft lacks typical phenotypes and clinical manifestations, always resulting in delayed diagnosis and treatment. It has been considered to be an elemental factor influencing the improvement of the long-term outcome of renal allograft. The B cell activating factor (BAFF) signal plays a fundamental function in the process of antibody-mediated immune response. Data from recipients and the nonhuman primate ABMR model suggest that the BAFF signal participates in the ABMR of renal allograft, while there are objections. The challenges in the diagnosis of ABMR, different study population, and details of research may explain the discrepancy. Large quantities of dynamic, credible data of BAFF ligands and their association with renal allograft pathological characteristics would constitute a direct proof of the role of BAFF in the progression of renal allograft ABMR.

scholarly journals Clinical and Biochemical Profiles of Filipino Patients with Distal Urea Cycle Disorders Detected by Abnormal Expanded Newborn Screening

2020 ◽  
Vol 54 (4) ◽  
Author(s):  
Michelle E. Abadingo ◽  
Mary Ann R. Abacan ◽  
Mary Anne D. Chiong ◽  
Leniza G. De Castro-Hamoy
Keyword(s):  
Newborn Screening ◽  
Neurodevelopmental Outcome ◽  
Clinical Manifestations ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Expanded Newborn Screening ◽  
Biochemical Profiles ◽  
Cycle Disorders ◽  
Argininosuccinate Synthetase Deficiency

Objective. The study is a retrospective review which provides preliminary data on the correlation between biochemical profiles and initial clinical manifestation of patients diagnosed to have argininosuccinate synthetase deficiency (ASSD) and argininosuccinate lyase deficiency (ASLD) detected by expanded newborn screening (ENBS).Methods. This is a study of five distal UCD patients initially detected by elevated citrulline on ENBS. Medical charts of the patients were reviewed. The initial clinical manifestations of the patients were correlated with results of biochemical tests.Results. There were four cases of ASLD and one case of ASSD reviewed in this study. All cases of ASLD were confirmed by the presence of argininosuccinic acid (ASA) in the urine metabolic screen (UMS). The plasma citrulline level of the ASSD patient is significantly elevated as compared to the ASLD patients (2,690 µmol/L; NV: 10-45 µmol/L). The ASSD patient and one ASLD patient were symptomatic within the first six days of life. Both presented with significantly elevated plasma ammonia, citrulline and glutamine levels. Three ASLD patients were asymptomatic on initial screening.Conclusion. ENBS has shown importance in the early detection and management of ASSD and ASLD. Early initiation of management may prevent hyperammonemic crises. Long term outcome studies are needed to look into the correlation of neurodevelopmental outcome with lifelong accumulation of citrulline and glutamine in ASSD and ASA in ASLD.

scholarly journals Protracted Bacterial Bronchitis, it is a myth?

2021 ◽  
Vol 20 (4) ◽  
Author(s):  
Ahmad Fadzil
Keyword(s):  
Chronic Cough ◽  
Clinical Presentation ◽  
Signs And Symptoms ◽  
Medical Conditions ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Protracted Bacterial Bronchitis

Protracted wet and productive chronic cough is a symptom that indicate there is an underlying cause and the possibility vary from a simple to serious medical conditions. If the cough is accompanied with specific pointers in signs and symptoms, the diagnosis is apparent. Nevertheless, the challenge is to identify the diagnosis in children who cough without specific pointers. Recently, protracted bacterial bronchitis is one of the conditions that was proposed and postulated to conceive this clinical presentation. Since then, the understanding of PBB has escalated and deepened. However, there is still no unanimous consensus in definition, pathophysiology, diagnostic, treatment, long term outcome and even the entity itself. Further research is required to refine understanding of this condition in several facets therefore patients can be treated accordingly.

scholarly journals Mycobacterium tuberculosisInfection following Kidney Transplantation

2013 ◽  
Vol 2013 ◽  
pp. 1-9 ◽  
Author(s):  
Karima Boubaker ◽  
Tahar Gargah ◽  
Ezzedine Abderrahim ◽  
Taieb Ben Abdallah ◽  
Adel Kheder
Keyword(s):  
Demographic Data ◽  
Clinical Manifestations ◽  
Treatment Protocol ◽  
Renal Transplant Recipients ◽  
Transplant Recipients ◽  
Graft Dysfunction ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Post Transplant

Introduction and Aims. Post-transplant tuberculosis (TB) is a problem in successful long-term outcome of renal transplantation recipients. Our objective was to describe the pattern and risk factors of TB infection and the prognosis in our transplant recipients.Patients and Methods. This study was a retrospective review of the records of 491 renal transplant recipients in our hospital during the period from January 1986 to December 2009. The demographic data, transplant characteristics, clinical manifestations, diagnostic criteria, treatment protocol, and long-term outcome of this cohort of patients were analyzed.Results. 16 patients (3,2%) developed post-transplant TB with a mean age of 32,5 ± 12,7 (range: 13–60) years and a mean post-transplant period of 36,6months (range: 12,3 months–15,9 years). The forms of the diseases were pulmonary in 10/16 (62,6%), disseminated in 3/16 (18,7%), and extrapulmonary in 3/16 (18,7%). Graft dysfunction was observed in 7 cases (43,7%) with tissue-proof acute rejection in 3 cases and loss of the graft in 4 cases. Hepatotoxicity developed in 3 patients (18,7%) during treatment. Recurrences were observed in 4 cases after early stop of treatment. Two patients (12.5%) died.Conclusion. Extra pulmonary and disseminated tuberculosis were observed in third of our patients. More than 9months of treatment may be necessary to prevent recurrence.

scholarly journals Improved Survival beyond 28 Days up to 1 Year after CytoSorb Treatment for Refractory Septic Shock: A Propensity-Weighted Retrospective Survival Analysis

2020 ◽  
pp. 1-7
Author(s):  
Willem P. Brouwer ◽  
Servet Duran ◽  
Can Ince
Keyword(s):  
Septic Shock ◽  
Positive Predictive Value ◽  
Predictive Value ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Factors Associated ◽  
Icu Admission ◽  
Lactate Levels

<b><i>Background and Aims:</i></b> It is currently unknown whether CytoSorb treatment for septic shock improves long-term survival beyond 28 days from intensive care unit (ICU) admission and which factors determine outcome. <b><i>Methods:</i></b> This was a long-term follow-up retrospective analysis of patients with septic shock who were treated with continuous renal replacement therapy (CRRT) + CytoSorb (<i>n</i> = 67) or CRRT alone (<i>n</i> = 49). These patients were previously analyzed for 28-day mortality. The primary outcome was the time to long-term all-cause mortality. Factors associated with time to event were analyzed both weighted by stabilized inverse probability of treatment weights (sIPTW) as well as unweighted stratified by therapy received. <b><i>Results:</i></b> The median follow-up for the total cohort was 30 days (interquartile range [IQR]: 5–334, maximum 1,059 days) after ICU admission and 333 days (IQR: 170–583) for those who survived beyond 28 days (<i>n</i> = 59). Survival beyond 28 days was sustained up to 1 year after ICU admission for both treatment regimens: 80% (standard error [SE] 7%) vs. 87% (SE 7%), for CytoSorb vs. CRRT, respectively, <i>p</i> = 0.853. By sIPTW, CytoSorb was significantly associated with long-term outcome compared to CRRT (adjusted hazard ratio [aHR] 0.59, 95% confidence interval [CI] 0.37–0.93, <i>p</i> = 0.025). Independent factors associated with long-term outcome in CytoSorb-treated patients were baseline log<sub>10</sub> lactate levels (aHR 5.1, <i>p</i> = 0.002), age in the presence of comorbidity (aHR 2.60, <i>p</i> = 0.013), and presence of abdominal sepsis (aHR 0.34, <i>p</i> = 0.004). A lactate level above 6.0 mmol/L at the start of CytoSorb therapy had a positive predictive value of 79% for mortality (<i>p</i> = 0.013). <b><i>Conclusions:</i></b> Survival is achieved with CytoSorb and CRRT for patients with septic shock beyond 28 days from ICU admission and may be improved for CytoSorb treatment. Lactate levels above 6.0 mmol/L at the start of CytoSorb therapy are predictive of worse outcome with high specificity and positive predictive value.

Alternating hemiplegia of childhood: clinical manifestations and long-term outcome

2000 ◽  
Vol 23 (2) ◽  
pp. 134-141 ◽  
Author(s):  
Mohamad A Mikati ◽  
Uri Kramer ◽  
Mary L Zupanc ◽  
Robin J Shanahan
Keyword(s):  
Clinical Manifestations ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Alternating Hemiplegia Of Childhood

Clinical manifestations and long-term outcome of anti-Jo1 antisynthetase patients in a large cohort of Spanish patients from the GEAS-IIM group

2016 ◽  
Vol 46 (2) ◽  
pp. 225-231 ◽  
Author(s):  
Ernesto Trallero-Araguás ◽  
Josep María Grau-Junyent ◽  
Anne Labirua-Iturburu ◽  
Francisco José García-Hernández ◽  
Manuel Monteagudo-Jiménez ◽  
...  
Keyword(s):  
Clinical Manifestations ◽  
Large Cohort ◽  
Term Outcome ◽  
Long Term Outcome

scholarly journals Guillain-Barré syndrome in the elderly: clinical, electrophysiological, therapeutic and outcome features

2005 ◽  
Vol 63 (3b) ◽  
pp. 772-775 ◽  
Author(s):  
Marcondes C. França Jr ◽  
Leonardo Deus-Silva ◽  
Rafael de Castro ◽  
Solange G. Garibaldi ◽  
Beatriz H.M. Pfeilsticker ◽  
...  
Keyword(s):  
Epidemiological Data ◽  
The Elderly ◽  
Motor Deficit ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Sensory Motor ◽  
Short And Long Term ◽  
Prognostic Implications ◽  
Guillain Barré

There are few papers devoted to geriatric Guillain-Barré (GBS) and many related issues remain unanswered. OBJECTIVE: To describe clinical, electrophysiological and therapeutic features in this age. METHOD: Clinico-epidemiological data and therapy of GBS patients older than 60 years were reviewed. Hughes scores were used to quantify neurological deficit and define outcome. RESULTS: Among 18 patients (mean age 64.8 years), 9 had evident prodrome and 80% noticed initially sensory-motor deficit. Demyelinating GBS was found in 8 and axonal in 6 subjects. There was one Miller-Fisher and 3 unclassified cases. Plasmapheresis (PFX) was single therapy in 12 patients and intravenous immunoglobulin (IVIg) in 2. Disability scores just before therapy were similar in both groups, so as short and long term outcome. CONCLUSION: Axonal GBS seems to be more frequent in the elderly and this may have prognostic implications. PFX and IVIg were suitable options, but complications were noticed with PFX. Prospective studies are needed to better understand and manage GBS in the elderly.

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