scholarly journals Cutaneous neonatal Langerhans cell histiocytosis: a systematic review of case reports

F1000Research ◽  
2019 ◽  
Vol 8 ◽  
pp. 13
Author(s):  
Victoria Venning ◽  
Evelyn Yhao ◽  
Elizabeth Huynh ◽  
John W. Frew
Keyword(s):  
Systematic Review ◽  
Langerhans Cell Histiocytosis ◽  
Case Reports ◽  
Systemic Disease ◽  
Langerhans Cell ◽  
Multiple Lesions ◽  
Risk Of Mortality ◽  
Cochrane Database ◽  
Chi Squared ◽  
Worse Prognosis

Background: Cutaneous langerhans cell histiocytosis (LCH) is a rare disorder characterized by proliferation of cells with phenotypical characteristics of Langerhans cells. Although some cases spontaneously resolve, no consistent variables have been identified that predict which cases will manifest with systemic disease later in childhood. Methods: A systematic review (Pubmed, Embase, Cochrane database and all published abstracts from 1946-2018) was undertaken to collate all reported cases of cutaneous LCH in the international literature. This study was registered with PROSPERO (CRD42016051952). Descriptive statistics and correlation analyses were undertaken. Bias was analyzed according to GRADE criteria. Results: A total of 83 articles encompassing 128 cases of cutaneous LCH were identified. Multiple lesions were weakly associated with an increased length of survival (R=0.304 (p<0.05)), Worse prognosis was associated with internal organ involvement with a statistically significant chi squared statistic (χ2=14.96, 2DF p<0.001) and an elevated odds ratio ((OR)= 12.30 95% CI=2.67-56.74). Vesicular lesions (OR=10.8 95% CI=2.83-41.26), but not ulceration (OR=0.53 95% CI 0.12-2.05) were associated with greater risk of mortality. Conclusions: Congenital and neonatal LCH most commonly presents as multiple lesions in multiple anatomical sites at birth. Significant differences, including the associations of mortality with lesion morphology and number were seen in this neonatal cohort compared to overall pediatric LCH. These findings require validation in a large prospective cohort.

scholarly journals Unusual sites of bone involvement in Langerhans cell histiocytosis: a systematic review of the literature

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Nahid Reisi ◽  
Pouran Raeissi ◽  
Touraj Harati Khalilabad ◽  
Alireza Moafi
Keyword(s):  
Systematic Review ◽  
Dendritic Cells ◽  
Rare Disease ◽  
Langerhans Cell Histiocytosis ◽  
Case Reports ◽  
Langerhans Cell ◽  
The Other ◽  
Bone Involvement ◽  
Intracranial Extension ◽  
Pathologic Fractures

Abstract Background Langerhans cell histiocytosis (LCH) is a rare disease that originates from the uncontrolled proliferation and accumulation of bone marrow-derived immature myeloid dendritic cells. Dendritic cells are a type of histiocyte that play an important role in the human immune system and are found in the bone, skin, stomach, eyes, intestines, and lungs. Objective This systematic review aimed to collect and report published case reports of rare bone disease caused by LCH to avoid misdiagnoses or delays in diagnosis. Methods We systematically searched Scopus, PubMed, Embase, and Web of Sciences from August 1, 2000 to December 31, 2019. Studies reporting cases of LCH with rare bone involvement were included. Results We identified 60 articles including 64 cases. Of the identified cases, 31 (48.4%) involved children, and 33 (51.6%) involved adults. Additionally, 46.9% (30 individuals) were from Asian countries. The mean age of the children was 7.6 ± 4.3 years and that of the adults was 36 ± 12 years. The findings indicated that unifocal bone involvements were the most prevalent form of the disease (68.7%), and, overall, the skull and chest wall were the most commonly affected bones in both adults and children. The spine and long bones were the second most commonly affected bones in children, and the spine and jaw were the second most commonly affected bones in adults. Pain and swelling were the most frequent presenting signs among the investigated cases, and loss of consciousness, myelopathy, nerve palsy, visual loss, torticollis and clicking sounds were rare signs. Osteolytic lesions were the most frequent radiologic feature (62.5%), and intracranial hemorrhage, fluid–fluid level, dura and intracranial extension and pathologic fractures were rare radiological features. Total excision, curettage and observation in the unifocal group of patients and systemic chemotherapy in the other groups (i.e., multifocal and multisystem) were the most frequent management approaches. The recovery rates of the unifocal and multifocal groups were 77.3% and 81.8%, respectively, while that of the multisystem group was 55.5%. The rates of recurrence and mortality in the multisystem group were 11% and were higher than those in the other groups. Conclusions LCH is a rare disease that can affect any organ in the human body. However, bone is the most commonly involved organ, and rare bone involvements may be the first or only symptom of the disease due to the rarity of such lesions; a lack of familiarity with them may result in misdiagnosis or delayed diagnosis.

scholarly journals Efficacy and safety of intravenous immunoglobulins for the treatment of viral encephalitis: a systematic literature review

2021 ◽  
Author(s):  
Judith N. Wagner ◽  
Annette Leibetseder ◽  
Anna Troescher ◽  
Juergen Panholzer ◽  
Tim J. von Oertzen
Keyword(s):  
Systematic Review ◽  
Viral Encephalitis ◽  
Viral Infections ◽  
Case Reports ◽  
Ivig Therapy ◽  
Intravenous Immunoglobulins ◽  
Efficacy And Safety ◽  
Serious Adverse Events ◽  
Optimal Dosing ◽  
Cochrane Database

Abstract Background For most viral encephalitides, therapy is merely supportive. Intravenous immunoglobulins (IVIG) have been used as a prophylactic and therapeutic approach. We conduct a systematic review on the safety and efficacy of IVIG in viral encephalitis. Methods We conducted a systematic review assessing PubMed, Cochrane Database, Biosis Previews and the ClinicalTrials.gov website to identify all reports on patients with viral encephalitis treated with IVIG as of May 31, 2019. The main outcomes assessed were therapeutic efficacy and safety. For an increased homogeneity of the population, atypical viral infections were excluded, as were reports on prophylactic IVIG use, intrathecal application of immunoglobulins, or use of antibody-enriched IVIG-preparations. Data were extracted from published studies. Descriptive statistics were used. Results We included a total of 44 studies (39 case reports). The case reports cover a total of 53 patients. Our search retrieved two prospective and three retrospective studies. These show heterogeneous results as to the efficacy of IVIG therapy. Only one study reports a significant association between IVIG-use and death (odds ratio 0.032; 95% confidence interval 0.0033–0.3024; p = 0.0027). None of the studies report significant differences in the number of serious adverse events. Conclusion Data on the efficacy of IVIG-therapy is heterogeneous. While it seems generally safe, evident superiority compared to supportive treatment has not been demonstrated so far. Future trials should also investigate the optimal dosing and timing of IVIG and their benefit in the immunosuppressed.

Langerhans cell histiocytosis in monocygote twins: case reports

1996 ◽  
Vol 38 (2) ◽  
pp. 163-165 ◽  
Author(s):  
I. Mader ◽  
K. W. Stock ◽  
E. W. Radue ◽  
W. Steinbrich
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Case Reports ◽  
Langerhans Cell

scholarly journals Langerhans cell histiocytosis of the mandible: two case reports and literature review

2019 ◽  
Vol 45 (3) ◽  
pp. 167
Author(s):  
Dae-Seok Hwang ◽  
Jun Sang Lee ◽  
Uk-Kyu Kim ◽  
Hae Ryoun Park ◽  
Mi Heon Ryu ◽  
...  
Keyword(s):  
Literature Review ◽  
Langerhans Cell Histiocytosis ◽  
Case Reports ◽  
Langerhans Cell

scholarly journals Unrecognized oral manifestations of Langerhans cell histiocytosis which progressed to systemic disease

2006 ◽  
Vol 42 (1) ◽  
pp. 10-13 ◽  
Author(s):  
I. Alajbeg ◽  
V. Vucicevic Boras ◽  
R. Femenic ◽  
A. Cekic-Arambasin ◽  
M. Anicic ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Systemic Disease ◽  
Langerhans Cell ◽  
Oral Manifestations

scholarly journals Pediatric Langerhans Cell Histiocytosis (LCH) Clinical Outcome in Hospital Civil De Guadalajara, México

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 4951-4951 ◽  
Author(s):  
Katterine Rojas Rodríguez ◽  
Veronica Soto ◽  
Carlos Rodriguez-Galindo ◽  
Paola M. Friedrich ◽  
Edwin Guzmán ◽  
...  
Keyword(s):  
Clinical Outcome ◽  
Langerhans Cell Histiocytosis ◽  
Disease Risk ◽  
Conflicts Of Interest ◽  
Systemic Disease ◽  
Giant Cells ◽  
Langerhans Cell ◽  
Induction Treatment ◽  
Multisystem Disease ◽  
Clonal Proliferation

Abstract Pediatric Langerhans cell histiocytosis (LCH) clinical outcome in Hospital Civil de Guadalajara, México. Introduction : LCH results from clonal proliferation of functionally and immunophenotyped inmature round Langerhans cells along with eosinophiles, machrophages, lymphocytes and ocasionally multinucleated giant cells (1). Its incidence is 2-10 cases by million of children below 15 yr in US (2). Our objective was to describe the clinical characteristics and treatment outcome of patients with LCH at Departement of Hematology-Oncology of Hospital Civil de Guadalajara México. Methods: It was a retrospective design and 41 pediatric patients below 18 yr were included. The diagnosis was corroborated by pathology and immunohistochemistry. Variables as age, gender, localised vs systemic disease, risk organ commitment, global survival (GS) and event free survival (EFS) were analysed. We used descriptive and inferencial statistics with SPSS program. Results: There were included 41 patients from January 1st 2012 to December 31st 2017. Relation male:female was 1.1:1. Mean presentation was localised disease (58%). Bone was the principal affected structure (34%) and it was 71% to be combined with lung, lymph node and CNS compromise. Risk organ commitment was presented in 32%, being more frecquent bone marrow and liver in 22% each one. Time induction treatment was equal or below 12 weeks in 66% of patients. The 25% of patients had reactivation of LCH, with similar lesions to the beggining in 19.5%. We found statistically significant differences between dead patients (DP) (14.6%) and not dead patients (NDP) (85.4%) in clinical presentation: localised (0% in DP vs 69% in NDP) and systemic disease (100% vs 31%) (p=0,003) and risk organ commitment (100% in DP vs 20% in NDP) (p=0,000). Median age of 13 vs 24 months was for DP and NDP respectively. Conclusion: Dead patients were younger than 13 months old, with systemic disease, and risk organ commitment. We found a later asking of medical advice in DP (6 months) vs NDP (2 months). Keys words: langerhans cell histiocytosis, multisystem disease, risk organ Figure. Figure. Disclosures No relevant conflicts of interest to declare.

scholarly journals Association between varying cut-points of intermediate hyperglycemia and risk of mortality, cardiovascular events and chronic kidney disease: a systematic review and meta-analysis

2021 ◽  
Vol 9 (1) ◽  
pp. e001776
Author(s):  
Unjali P Gujral ◽  
Ram Jagannathan ◽  
Siran He ◽  
Minxuan Huang ◽  
Lisa R Staimez ◽  
...  
Keyword(s):  
Systematic Review ◽  
Chronic Kidney Disease ◽  
Kidney Disease ◽  
English Language ◽  
Meta Analysis ◽  
Glycosylated Hemoglobin ◽  
Language Studies ◽  
Risk Of Mortality ◽  
Cochrane Database ◽  
Design And Methods

IntroductionWe conducted a systematic review and meta-analysis to evaluate the updated evidence regarding prediabetes for predicting mortality, macrovascular and microvascular outcomes.Research design and methodsWe identified English language studies from MEDLINE, PubMed, OVID and Cochrane database indexed from inception to January 31, 2020. Paired reviewers independently identified 106 prospective studies, comprising nearly 1.85 million people, from 27 countries. Primary outcomes were all-cause mortality (ACM), cardiovascular mortality (CVDM), cardiovascular disease (CVD), coronary heart disease (CHD) and stroke. Secondary outcomes were heart failure, chronic kidney disease (CKD) and retinopathy.ResultsImpaired glucose tolerance was associated with ACM; HR 1.19, 95% CI (1.15 to 1.24), CVDM; HR 1.21, 95% CI (1.10 to 1.32), CVD; HR 1.18, 95% CI (1.11 to 1.26), CHD; HR; 1.13, 95% CI (1.05 to 1.21) and stroke; HR 1.24, 95% CI (1.06 to 1.45). Impaired fasting glucose (IFG) 110–125 mg/dL was associated with ACM; HR 1.17, 95% CI (1.13 to 1.22), CVDM; HR 1.20, 95% CI (1.09 to 1.33), CVD; HR 1.21, 95% CI (1.09 to 1.33), CHD; HR; 1.14, 95% CI (1.06 to 1.22) and stroke; HR 1.22, 95% CI (1.07 to 1.40). IFG 100–125 mg/dL was associated with ACM; HR 1.11, 95% CI (1.04 to 1.19), CVDM; HR 1.14, 95% CI (1.03 to 1.25), CVD; HR 1.15, 95% CI (1.05 to 1.25), CHD HR; 1.10, 95% CI (1.02 to 1.19) and CKD; HR; 1.09, 95% CI (1.01 to 1.18). Glycosylated hemoglobin A1c (HbA1c) 6.0%–6.4% was associated with ACM; HR 1.30, 95% CI (1.03 to 1.66), CVD; HR 1.32, 95% CI (1.00 to 1.73) and CKD; HR 1.50, 95% CI (1.32 to 1.70). HbA1c 5.7%–6.4% was associated with CVD HR 1.15, 95% CI (1.02 to 1.30), CHD; HR 1.28, 95% CI (1.13 to 1.46), stroke; HR 1.23, 95% CI (1.04 to 1.46) and CKD; HR 1.32, 95% CI (1.16 to 1.50).ConclusionPrediabetes is an elevated risk state for macrovascular and microvascular outcomes. The prevention and management of prediabetes should be considered.

Sign in / Sign up

Export Citation Format

Share Document