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

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 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.

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 SAT0495 LONG TERM OUTCOME OF JUVENILE IDIOPATHIC ARTHRITIS IN ADULTHOOD; THE MONOCENTRIC EXPERIENCE OF 520 PATIENTS FOLLOWED FOR 20 YEARS IN A TRANSITION TERTIARY CLINIC OF PEDIATRIC RHEUMATOLOGY.

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1204.2-1204
Author(s):  
I. Pontikaki ◽  
S. Carbogno ◽  
F. Corona ◽  
A. Petaccia ◽  
R. Cimaz
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Educational Level ◽  
Disease Duration ◽  
Age At Onset ◽  
Disease Onset ◽  
Clinical Manifestations ◽  
Term Outcome ◽  
Long Term Outcome

Background:Juvenile Idiopathic Arthritis (JIA) is a chronic pediatric inflammatory disease that shows many differences compared to adult-onset arthritis. The different clinical manifestations, the assessment and the management of JIA is the reason that the transition from childhood to adulthood is an important multidimensional process that emphasizes a lot of aspects.Objectives:To describe the long-term outcome of JIA.Methods:Five-hundred and twenty patients affected by JIA and referred to a transition care rheumatology tertiary centre were considered between 1999 and 2019. The outcome assessment included remission, disease duration, medications, number of prosthesis implantation, pregnancies, mortality and social integration (employment status and educational level).Results:A hundred and thirty-eight (26%) males and 382 (73%) females were included; 157 (30%) patients were lost to follow up. The mean age of the patients was 27 (18-57) years, with a mean age at onset of 8 years and an average disease duration of 19 years. Subtypes of JIA at disease onset included 252 (48%) oligoarthritis, 134 (26%) polyarthritis, 64 (12%) systemic arthritis, 22 (4%) psoriatic arthritis, 43 (8%) enthesitis related arthritis and 1 (0.1%) undifferentiated arthritis. Ninty-three (18%) patients suffered of uveitis. Ninty-five implant prosthesis and 16 arthrodesis were recorded. At follow up 198 (38%) patients were on remission of which 107 (20%) off medication. Among the 322 patients still on medication, 84 (16%) were under treatment with oral steroids, 226 (43%) with sDMARDs and 249 (40%) with bDMARDs. Five deaths (1%) occurred in this cohort. Two hundred and thirty-five subjects had a higher educational level, 327 had an employment. We have data of twenty-nine pregnancies. The transition age was considered after the age of 16 years old. The key word for the management of this cohort was the multidisciplinary approach towards each patient, with the collaboration of other specialists (ophthalmologist, orthopedic, dermatologist, gastroenterologist, obstetric and psychologist).Conclusion:In the era of biologic therapy the long-term outcome of JIA underwent an outstanding improvement regarding a lot of variables. Two hundred and thirty-two patients were still followed, not only because of the continuation of the biological therapy, but also for a multidisciplinary care even during remission. JIA often persists over the adulthood, therefore the long term follow-up and care of these patients needs to be conducted by a rheumatologist expertized in JIA in collaboration with other specialists.Disclosure of Interests:None declared

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 follow-up of meningiomas of the cavernous sinus after surgical treatment alone

2007 ◽  
Vol 107 (5) ◽  
pp. 937-944 ◽  
Author(s):  
Marc Sindou ◽  
Ernesto Wydh ◽  
Emmanuel Jouanneau ◽  
Mustapha Nebbal ◽  
Thomas Lieutaud
Keyword(s):  
Cavernous Sinus ◽  
Secondary Growth ◽  
Clinical Manifestations ◽  
Tumor Control ◽  
Ocular Motility ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Group 1

Object The authors report on the long-term outcome in 100 consecutive patients with meningiomas arising from the cavernous sinus (CS) with compressive extension outside the CS. The treatment in all cases was surgery alone without adjuvant radiosurgery or radiotherapy. The aim of this study was to evaluate the percentage of patients in whom surgery alone was able to produce long-term tumor control. Methods All 100 patients harbored meningiomas with supra- and/or laterocavernous extension, and 27 had petro-clival extension. Surgery was performed via frontopterionotemporal craniotomy associated with orbital and/or zygomatic osteotomy in 97 patients. Proximal control of the internal carotid artery at the foramen lacerum was undertaken in 65 patients; the paraclinoid carotid segment was exposed extradurally at the space made by the anterior clinoidectomy in 81 patients. For the petroclival tumor extension, a second-stage surgery was performed via a presigmoid–retro-labyrinthine or retrosigmoid approach in 13 and 14 patients, respectively. Results The mortality rate was 5% and two patients had severe hemiplegic or aphasic sequelae. The creation or aggravation of disorders in vision, ocular motility, or trigeminal function occurred in 19, 29, and 24% of patients respectively, with a significantly higher rate of complications when resection was performed inside the CS (p < 0.05). Gross-total removal of both the extra- and intracavernous portions was achieved in 12 patients (Group 1), removal of the extracavernous portions with only a partial resection of the intracavernous portion in 28 patients (Group 2), and removal only of the extracavernous portions was performed in 60 patients (Group 3). The follow-up period ranged from 3 to 20 years (mean 8.3 years). There was no tumor recurrence in Group 1. In the 83 surviving patients in Groups 2 and 3 combined, the tumor remnant did not regrow in 72 patients (86.7%); regrowth was noted in 11 (13.3%). Conclusions The results suggest that there is no significant oncological benefit in performing surgery within the CS. Because entering the CS entails a significantly higher risk of complications, radiosurgical treatment should be reserved for remnants with secondary growth and clinical manifestations.

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