scholarly journals Clinical presentations and long term prognosis of childhood onset polyarteritis nodosa in single centre of Korea

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Jeong-Seon Lee ◽  
Joong-Gon Kim ◽  
Soyoung Lee
Keyword(s):  
Polyarteritis Nodosa ◽  
Clinical Manifestations ◽  
Long Term Outcomes ◽  
Childhood Onset ◽  
Laboratory Findings ◽  
Clinical Presentations ◽  
Long Term Prognosis ◽  
Digital Amputation

AbstractChildhood-onset polyarteritis nodosa (PAN) is a rare and systemic necrotising vasculitis in children affecting small- to medium-sized arteries. To date, there have been only a few reports because of its rarity. Thus, we aimed to investigate the clinical manifestations, laboratory findings, treatment, and long-term outcomes in patients with childhood-onset PAN and to evaluate the usefulness of the paediatric vasculitis activity score (PVAS). We retrospectively analysed the data of nine patients with childhood-onset PAN from March 2003 to February 2020. The median ages at symptom onset, diagnosis, and follow-up duration were 7.6 (3–17.5), 7.7 (3.5–17.6), and 7.0 (1.6–16.3) years, respectively. All patients had constitutional symptoms and skin manifestations, while five exhibited Raynaud’s phenomenon. Organ involvement was observed in one patient. The median PVAS at diagnosis was 7 (range: 2–32). Prednisolone was initially used for induction in all patients, and other drugs were added in cases refractory to prednisolone. All patients survived, but three patients with high PVAS at diagnosis experienced irreversible sequelae, including intracranial haemorrhage and digital amputation. In conclusion, early diagnosis and treatment may minimise sequelae in patients with childhood-onset PAN. This study suggests that high PVAS score at diagnosis may be associated with poor prognosis.

scholarly journals Anti-NMDAR encephalitis

2019 ◽  
Vol 7 (1) ◽  
pp. e633 ◽  
Author(s):  
Xiaolu Xu ◽  
Qiang Lu ◽  
Yan Huang ◽  
Siyuan Fan ◽  
Lixin Zhou ◽  
...  
Keyword(s):  
Ovarian Teratoma ◽  
Long Term Outcomes ◽  
First Line ◽  
Factors Affecting ◽  
First Line Therapy ◽  
Nmdar Encephalitis ◽  
Clinical Presentations ◽  
Long Term Prognosis

ObjectiveTo describe the detailed clinical characteristics, immunotherapy, and long-term outcomes of patients with anti-NMDA receptor (NMDAR) encephalitis in China.MethodsA single-center, prospective study. Patients who met the diagnostic criteria were enrolled from 2011 to 2017 and followed up. The clinical features, treatment, and long-term outcomes were collected prospectively. Factors affecting the long-term prognosis were analyzed.ResultsThe study included 220 patients. The most common clinical presentations were psychosis (82.7%) and seizures (80.9%). Of the patients, 19.5% had an underlying neoplasm; of which ovarian teratoma was 100% of tumors in females and only one male had lung cancer. Most patients (99.5%) received first-line therapy (glucocorticoids, IV immunoglobulin, or plasmapheresis alone or combined), and only 7.3% received second-line immunotherapy (rituximab, cyclophosphamide alone, or combined). Long-term immunotherapy (mycophenolate mofetil or azathioprine >1 year) was administered to 53.2% of patients. During the first 12 months, 207 (94.1%) patients experienced improvement, and 5 (2.3%) died, whereas 38 (17.3%) experienced relapses. At 12-month follow-up, 92.7% had favorable clinical outcomes (modified Rankin Scale score ≤2).ConclusionsPatients in China present with psychosis and seizure frequently but have a low percentage of underlying neoplasms. Re-enforced first-line immunotherapy is effective in managing anti-NMDAR encephalitis in the acute phase. Although relapse is relatively common, with combined first-line and long-term immunotherapy, most patients reached favorable outcomes.

scholarly journals Clinical presentations and long-term prognosis of childhood-onset polyarteritis nodosa in a single centre: a retrospective study

2020 ◽  
Author(s):  
Jeong-Seon Lee ◽  
JoongGon Kim ◽  
Soyoung Lee
Keyword(s):  
Polyarteritis Nodosa ◽  
Treatment Guidelines ◽  
Clinical Manifestations ◽  
Diagnosis And Treatment ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Childhood Onset ◽  
Single Centre ◽  
Laboratory Findings ◽  
Paediatric Patients

Abstract Background: Polyarteritis nodosa (PAN) is a systematic necrotizing vasculitis involving medium-sized arteries. Childhood-onset PAN ranges from mild to severe systematic disease causing damage and early mortality. As it is a rare disease in children, there is less study on clinical features, diagnosis and treatment than adults. And diagnosis occurs relatively late in most paediatric patients with polyarteritis nodosa. Therefore, this study aimed to investigate clinical manifestations, laboratory findings, treatment strategies, and long-term outcomes among patients with childhood-onset polyarteritis nodosa treated at a single-centre in Korea. We further aimed to evaluate the usefulness of the Birmingham Vasculitis Activity Score (BVAS) and Five-Factor Score (FFS) in paediatric patients.Methods: We retrospectively analysed data collected from patients with childhood-onset PAN treated at our institution from March 2003 to February 2020. Results: Nine patients (6 male and 3 female) were included in the study. The median ages at symptom onset and diagnosis were 7.6 (3–17.5) and 7.7 (3.5–17.6) years, respectively. The median follow-up duration was 7.0 (1.6–16.3) years. Eight patients were diagnosed with systemic PAN, while one was diagnosed with cutaneous PAN. All patients exhibited skin manifestations, while five exhibited Raynaud’s phenomenon. Organ involvement was observed in one patient. Constitutional symptoms such as fever, arthralgia, myalgia, and weight loss were also observed. Antineutrophil cytoplasmic antibodies were absent in all patients. The median BVAS at diagnosis was 8 (range: 2–29), and one patient had an FFS of 1. Prednisolone was initially used for induction in all patients, and other drugs were added in cases refractory to prednisolone. All patients survived despite some complications such as intracranial haemorrhage or digital necrosis requiring amputation.Conclusions: Early diagnosis and treatment may minimise sequelae in patients with childhood-onset PAN. Further multicentre studies are required to clarify the unique characteristics of childhood-onset PAN and establish treatment guidelines, early detection strategies, and biomarkers. Trial registration: Retrospectively registered.

scholarly journals Neonatal Outcomes in Pregnant Women Infected with COVID-19 in Babol, North of Iran: A Retrospective Study with Short-Term Follow-Up

2021 ◽  
Vol 2021 ◽  
pp. 1-5
Author(s):  
Zahra Akbarian-Rad ◽  
Mohsen Haghshenas Mojaveri ◽  
Zinatossadat Bouzari ◽  
Farzin Sadeghi ◽  
Yousef Yahyapour ◽  
...  
Keyword(s):  
Pregnant Women ◽  
Clinical Characteristics ◽  
Neonatal Outcomes ◽  
Long Term Outcomes ◽  
Short Term ◽  
Reactive Protein ◽  
Clinical Presentations ◽  
North Of Iran

During the coronavirus disease 2019 (COVID-19) pandemic, the number of pregnant women and neonates suffering from COVID-19 increased. However, there is a lack of evidence on clinical characteristics and neonatal outcomes in pregnant women with COVID-19. We evaluated short-term outcomes (4 weeks postdischarge) and symptoms in neonates born to mothers infected with COVID-19. In this retrospective cohort study, we included all neonates born to pregnant women with COVID-19 admitted to Ayatollah Rohani Hospital, Babol, Iran, from February 10 to May 20, 2020. Clinical features, treatments, and neonatal outcomes were measured. Eight neonates were included in the current study. The mean gestational age and birth weight of newborns were 37 ± 3.19 weeks (30₊6-40) and 3077.50 ± 697.64 gr (1720-3900), respectively. Apgar score of the first and fifth minutes in all neonates was ≥8 and ≥9 out of 10, respectively. The most clinical presentations in symptomatic neonates were respiratory distress, tachypnea, vomiting, and feeding intolerance. This manifestation and high levels of serum C-reactive protein (CRP) in three infants are common in neonatal sepsis. The blood culture in all of them was negative. They have been successfully treated with our standard treatment. Our pregnant women showed a pattern of clinical characteristics and laboratory results similar to those described for nonpregnant COVID-19 infection. This study found no evidence of intrauterine or peripartum transmission of COVID-19 from mother to her child. Furthermore, the long-term outcomes of neonates need more study.

scholarly journals Long-Term Outcomes and Predictors of Childhood-Onset Schizophrenia: A Naturalistic Study of 6-year Follow-Up in China

2021 ◽  
Vol 12 ◽  
Author(s):  
Zheng Liangrong ◽  
Zhang Guican ◽  
Zhu Qi ◽  
Yang Weirui ◽  
Zhang Yaqi ◽  
...  
Keyword(s):  
Depressive Symptoms ◽  
Long Term Outcomes ◽  
Childhood Onset ◽  
Occupational Education ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Childhood Onset Schizophrenia ◽  
Education Status

Objectives: The long-term outcome of childhood-onset schizophrenia (COS) and its influencing factors remain unclear. The current study aimed to assess the long-term outcomes of COS and identify possible outcome predictors.Methods: We retrospectively investigated 276 patients with COS. Diagnosis made according to the ICD-10 criteria for schizophrenia, and the age of the first onset was ≤ 14 years. Follow-up was completed for 170 patients, with a median follow-up period of 5.6 years. Outcome variables included occupational/education status and readmission. Spearman correlation was performed to assess the relationship between predictors and outcome variables. Binary logistic regression was conducted to detect possible predictor variables for outcome variables.Results: At the end of the follow-up, 89 patients (52.3%) were at school, 70 patients (41.2%) were employed, and only 11 patients (6.5%) were dropped out of school or unemployed. The duration to the first admission and depressive symptoms were identified as predictors of occupational/educational status. The length of follow-up and obsessive-compulsive symptoms (OCS) were distinguished as predictors of readmission. Duration to the first admission and length of follow-up were risk factors, and depressive symptoms and OCS were protective factors for the outcomes of COS.Conclusion: We found a favorable long-term outcome on occupational/education status in COS, and depressive symptoms and OCS may be associated with more positive long-term outcomes in COS. Our findings suggest that COS patients may benefit from early intervention and require appropriate treatment.

scholarly journals Prevalence and Prognostic Significance of Left Ventricular Dysfunction in Patients Presenting Acutely with Atrial Fibrillation

2010 ◽  
Vol 4 ◽  
pp. CMC.S4106 ◽  
Author(s):  
Chin Lin ◽  
Colin Edwards ◽  
Guy P. Armstrong ◽  
Anthony Scott ◽  
Hitesh Patel ◽  
...  
Keyword(s):  
Systolic Dysfunction ◽  
Management Strategies ◽  
Newly Diagnosed ◽  
Imaging Studies ◽  
Long Term Outcomes ◽  
Prognostic Importance ◽  
Long Term Prognosis ◽  
Repeat Imaging

The prevalence and prognostic importance of CM occurring as a consequence of AF is poorly defined. This study investigated the incidence of CM in patients with AF, its clinical features and long-term outcomes. We demonstrated that CM is common in patients presenting acutely with newly diagnosed rapid AF, and carries a worse long-term prognosis. Systolic dysfunction was reversible in an important proportion of patients, suggesting a greater prevalence of rate-related CM in AF than has previously been postulated. This underscores the importance of appropriate rhythm management strategies and repeat imaging studies. Background Atrial fibrillation (AF) may precipitate LV dysfunction, potentially leading to cardiomyopathy (CM). The prevalence and prognostic importance of CM occurring as a consequence of AF is poorly defined. We investigated the incidence of CM in patients with AF, its clinical features and long-term outcomes. Methods We reviewed 292 consecutive patients (average age 72 ± 13yrs) presenting acutely with AF and tachycardia over a 3 year period from June 2004. Clinical details were obtained from medical records. CM was defined as ejection fraction (EF) ≤ 50% on index admission. Results Echo was performed 93% of patients at index admission, and 69 (24%) had CM (average EF% = 37 ± 11), 60 of which were newly diagnosed. Patients with CM had significantly higher presenting heart rate (141 ± 19 vs. 132 ± 23 bpm), larger end-diastolic (5.7 vs. 5.2 cm) and end-systolic (4.5 vs. 3.2 cm) dimensions, and larger left atrial size (4.6 vs. 4.3 cm) ( P < 0.05 for all). They were also statistically more likely ( P < 0.05) to be male, present with breathlessness, have a history of coronary disease, and be treated with digoxin and warfarin. Follow-up echo between 6 and 12 months was performed in 46% of patients with new CM, and average EF rose to 53 ± 12%. At an average follow-up of 2.5 years, there was a significant increase in mortality in CM patients (16% vs. 9.5%, P < 0.05). Conclusion CM is common in patients presenting acutely with newly diagnosed rapid AF, and carries a worse long-term prognosis. Systolic dysfunction was reversible in an important proportion of patients, suggesting a greater prevalence of rate-related CM in AF than has previously been postulated. This underscores the importance of appropriate rhythm management strategies and repeat imaging studies.

scholarly journals Series of patients with febrile status epilepticus: clinical manifestations and long-term follow-up

2020 ◽  
Vol 15 (1) ◽  
pp. 28-39
Author(s):  
V. E. Kitaeva ◽  
A. S. Kotov
Keyword(s):  
Status Epilepticus ◽  
Clinical Manifestations ◽  
Epilepsy Syndrome ◽  
Metabolic Decompensation ◽  
Long Term Follow Up ◽  
Organic Brain ◽  
Long Term Prognosis ◽  
Febrile Status Epilepticus

Background. The conception of febrile status epilepticus includes many syndromes with seizures of different etiologies (fever, infection, autoimmune processes, etc.). Generally developing seizures have a severe course and are difficult to treat.Objective: to classify patients with febrile status epilepticus and study their long-term prognosis.Materials and methods. The study included 10 patients (children and adolescents), hospitalized in the intensive care unit, whose epileptic status were associated with a fever.Results. In the analysis of the patients, who were included in the study, 4 subgroups can be distinguished: 1) a short-term period of convulsion against the background of metabolic decompensation caused by a main disease (with a conditionally favorable prognosis for seizures); 2) seizures as a manifestation of organic brain damage or infectious diseases with a serious prognosis of neurological development and seizures; 3) the onset of seizures against the background of fever (absence of any other causes of seizures); the prognosis of getting rid of seizures is variable, long follow-up monitoring is necessary, probably for many years; 4) true febrile infection-related epilepsy syndrome (in our study 1 patient had its classical form, and 1 patient had its less typical form (the onset of the disease at 18 years old – not at school age)).Conclusions. Obtained data illustrate the whole severity of febrile status epilepticus in children, according to follow-up observation, seizures persisted in at least 3 out of 10 patients (2 of them with febrile infection-related epilepsy syndrome), at least three patients had a serious infectious or organic brain pathology.

Periprocedural risk and long-term outcome of intracranial angioplasty based on a single-centre experience

VASA ◽  
2013 ◽  
Vol 42 (4) ◽  
pp. 264-274
Author(s):  
Dagmar Krajíčková ◽  
Antonín Krajina ◽  
Miroslav Lojík ◽  
Martina Mulačová ◽  
Martin Vališ
Keyword(s):  
Death Rate ◽  
Long Term Outcomes ◽  
Single Centre ◽  
Intracranial Atherosclerotic Stenosis ◽  
Atherosclerotic Stenosis ◽  
Angioplasty And Stenting ◽  
Aggressive Medical Management ◽  
Stroke Death

Background: Intracranial atherosclerotic stenosis is a major cause of stroke and yet there are currently no proven effective treatments for it. The SAMMPRIS trial, comparing aggressive medical management alone with aggressive medical management combined with intracranial angioplasty and stenting, was prematurely halted when an unexpectedly high rate of periprocedural events was found in the endovascular arm. The goal of our study is to report the immediate and long-term outcomes of patients with ≥ 70 % symptomatic intracranial atherosclerotic stenosis treated with balloon angioplasty and stent placement in a single centre. Patients and methods: This is a retrospective review of 37 consecutive patients with 42 procedures of ballon angioplasty and stenting for intracranial atherosclerotic stenosis (≥ 70 % stenosis) treated between 1999 and 2012. Technical success (residual stenosis ≤ 50 %), periprocedural success (no vascular complications within 72 hours), and long-term outcomes are reported. Results: Technical and periprocedural success was achieved in 90.5 % of patients. The within 72 hours periprocedural stroke/death rate was 7.1 % (4.8 % intracranial haemorrhage), and the 30-day stroke/death rate was 9.5 %. Thirty patients (81 %) had clinical follow-up at ≥ 6 months. During follow-up, 5 patients developed 6 ischemic events; 5 of them (17 %) were ipsilateral. The restenosis rate was 27 %, and the retreatment rate was 12 %. Conclusions: Our outcomes of the balloon angioplasty/stent placement for intracranial atherosclerotic stenosis are better than those in the SAMMPRIS study and compare favourably with those in large registries and observational studies.

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