Years Of Clinical Observation Research Articles

Congenital central hypoventilation syndrome in korea: 20 years of clinical observation and evaluation of the ventilation strategy in a single center

Congenital central hypoventilation syndrome (CCHS) is a rare genetic disorder characterized by hypoventilation due to impaired breathing control by the central nervous system and other symptoms of autonomic dysfunction. Mutations in paired-like homeobox 2 B (PHOX2B) are responsible for most cases of CCHS. Patients with CCHS have various phenotypes and severities, making the diagnosis difficult. This study aimed to present a comprehensive single-center experience of patients with CCHS, including key clinical features, treatment strategies, and outcomes. A retrospective chart review was performed for patients diagnosed with CCHS between January 2001 and July 2023 at Seoul National University Children’s Hospital. Finally, we selected 24 patients and collected their demographic data, genotypes, ventilation methods, and clinical features related to autonomic dysfunction. The relationship between the clinical manifestations and genotypes was also examined. All patients used home ventilators, and tracheostomy was performed in 87.5% of patients. Fifteen (62.5%) patients had constipation and nine (37.5%) were diagnosed with Hirschsprung disease. Arrhythmia, endocrine dysfunction, and subclinical hypothyroidism were present in nine (37.5%), six patients (25.0%), and two patients (16.7%), respectively. A significant number of patients exhibited neurodevelopmental delays (19 patients, 79.2%). There was a correlation between the phenotype and genotype of PHOX2B in patients with CCHS. (r = 0.71, p < 0.001). Conclusion: There was a positive correlation between paired-like homeobox 2 B mutations (especially the number of GCN repeats in the polyalanine repeat mutations sequence) and clinical manifestations. This study also demonstrated how initial treatment for hypoventilation affects neurodevelopmental outcomes in patients with CCHS.What is Known:• Congenital central hypoventilation syndrome is a rare genetic disorder characterized by hypoventilation and dysfunction of autonomic nervous system.• The disease-defining gene of CCHS isPHOX2Bgene – most of the cases have heterozygous PARMs and the number of GCN triplets varies among the patients(20/24 – 20/33).What is New:• We have noted in the Korean patients with CCHS that there is a correlation between genotype (number of GCN repeats) and severity of phenotype.• National support for rare diseases allowed for a prompter diagnosis of patients with CCHS in Korean population.

Expert consensus on clinical application of Lixuwang~® Xuesaitong Soft Capsules

Lixuwang~® Xuesaitong Soft Capsules(referred to as &quot;Xuesaitong Soft Capsules&quot;) have the effects of promoting blood circulation, resolving blood stasis, and dredging meridians and collaterals. They are widely used in the prevention and treatment of cardiovascular and cerebrovascular diseases in clinical practice. Through years of clinical observation, they have shown significant efficacy in ischemic stroke, coronary heart disease, and other diseases, and have been recommended by multiple guidelines, consensus statements, and monographs. Based on the summary of clinical application experience by doctors and existing evidence-based research, following the Technical Specifications for Consensus Development of Chinese Patent Medicine by Clinical Experts issued by Standardization Office of the Chinese Association of Traditional Chinese Medicine, a nominal group method was used to reach 19 recommended opinions/consensus suggestions. This document proposes the timing of medication, syndrome differentiation for medication, therapeutic effects, dosage and administration, treatment duration, economic considerations, and safety considerations in the use of Xuesaitong Soft Capsules for the treatment of ischemic stroke and angina pectoris in coronary heart disease. It is intended for doctors in internal medicine, encephalopathy(neurology), cardiovascular medicine, geriatrics, emergency medicine, general practice, and traditional Chinese medicine departments of various medical institutions, as well as pharmacists in hospitals and pharmacies, as a medication reference when using Xuesaitong Soft Capsules. It is hoped that the widespread application of this consensus can improve the clinical efficacy of Xuesaitong Soft Capsules in the treatment of ischemic stroke and coronary heart disease, promote rational drug use, and reduce medication risks. This consensus has been reviewed and published by the China Association of Traditional Chinese Medicine, with the identification number GS/CACM 323-2023.

Linguistic profile automated characterisation in pluripotential clinical high-risk mental state (CHARMS) conditions: methodology of a multicentre observational study

IntroductionLanguage is usually considered the social vehicle of thought in intersubjective communications. However, the relationship between language and high-order cognition seems to evade this canonical and unidirectional description (ie, the...

Expert consensus on clinical application of Compound Congrong Yizhi Capsules in treatment of vascular dementia

Compound Congrong Yizhi Capsules is widely used in clinic for the long-term treatment and synergistic treatment of vascular cognitive impairment. After years of clinical observation, it has an obvious curative effect on the treatement of vascular cognitive impairment and has been recommended by multiple guidelines, consensuses, and series. This consensus was formulated for the treatment of vascular dementia. On the basis of summarizing the application experience of clinicians, and combined with the existing evidence-based evidence, 11 recommendations/consensus recommendations were finally reached through the nominal group method. The indications, usage and dosage, course of treatment, medication time, concomitant medication, and precautions of Congrong Yizhi Capsules in the treatment of vascular dementia were proposed, and the safety of the clinical application was described. This consensus is applicable to the use of Compound Congrong Yizhi Capsules in the treatment of patients with vascular dementia, and can be used by clinicians from the departments of encephalopathy(neurology), geriatrics, and traditional Chinese medicine in general hospitals. This consensus has been approved by China Association of Chinese Medicine, with the number of GS/CACM 298-2022.

Replacement of Two Missing Adjacent Teeth with Two Single-Retainer Resin-Bonded Fixed Dental Prostheses with Improved Proximal Design.

To present a minimally invasive treatment approach for the replacement of two missing adjacent teeth with two single-retainer resin-bonded fixed dental prostheses (RBFDPs). Two missing adjacent premolars were restored by two RBFDPs with an innovative design of the proximal section. Both RBFDPs were digitally designed and milled from monolithic 3Y-TZP zirconia ceramic using CAD/CAM. Following construction of the anterior RBFDP, a shallow interlock was designed in the area of proximal contact in the same insertion direction as the posterior RBFDP. In this way, different paths of insertion of the RBFDPs could be provided without compromising the proximal hygiene capability. The presented minimally invasive restorations were successful over 5 years of clinical bservation without any complications. The presented restoration design secures the transversal position of the RBFDPs while maintaining physiologic tooth mobility. In addition, it allows for varying paths of insertion while improving hygienic conditions. Last, in case of a unilateral debonding of one retainer wing, rebonding might be possible. Int J Prosthodont 2023;36:155-160. doi: 10.11607/ijp.7711.

Xin Su Ning-A Review of Basic and Clinical Pharmacology Integrated With Traditional Chinese Medicine Antiarrhythmic Theory.

Xin Su Ning (XSN) is a patented multicomponent medicine, which was certified in 2005 by the China State Food and Drug Administration to be produced pharmaceutically and to be used clinically. The XSN capsule was developed from an effective formula composed by Prof. Shuwen Ding of Shandong University of Traditional Chinese Medicine. Through more than 30 years of clinical observation, Prof. Ding concluded that XSN has a significant effect on arrhythmia with phlegm-heat heart-disturbed syndrome according to the traditional Chinese medicine (TCM) diagnosis. XSN, derived from a classical TCM formula Huanglian Wen Dan Decoction, is formulated with 11 Chinese herbal medicines to treat cardiac ventricular arrhythmia. Clinical evidence suggests that it is particularly efficacious for the arrhythmias induced by cardiac ischemia and viral myocarditis without obvious adverse reactions being reported. Cellular electrophysiological studies in ventricular myocytes revealed that XSN prolongs the duration and suppresses the amplitude of the action potential (AP), which is supported by the blockage of sodium and potassium channels indicating the characteristics of class I and III antiarrhythmic drugs. A recently reported double-blind, placebo-controlled, multicenter clinical trial of XSN enrolled 861 patients (ChiCTR-TRC-14004180) and showed that XSN significantly inhibited premature ventricular contraction (PVC). The cellular electrophysiological discoveries provided the mechanistic evidence for the clinical efficacy on inhibition of PVC by XSN as demonstrated in the clinical trial. These studies, for the first time, provided exclusive evidence that multicomponent TCM antiarrhythmic medicine can be evaluated using conventional research methods that have been used for antiarrhythmic drug discoveries for decades. We aimed to give a comprehensive review on XSN including its origin with the support of TCM theory, its pre-licensing clinical use and development, and its pharmacological and clinical study discoveries. The review will be summarized with the discoveries reported in a novel network pharmacological study that introduced a weight coefficient, which made it possible to evaluate the pharmacological properties of the TCM formula with regard to its formation based on TCM theory. Limitations regarding XSN’s basic and clinical research and possible future studies are listed. We hope that the advances in how XSN was studied may offer useful guidance on how other TCM could be studied with respect to the integrity of the TCM formulas.

Ten years of clinical observation of olfactory ensheathing cell transplantation in patients with spinal cord injury

Objective:To evaluate the long-term curative efficacy and safety of olfactory ensheathing cell (OEC) transplantation by 10 years of follow-up investigation.Methods:A follow-up observation was done on 13 patients with allograft olfactory bulb-derived OEC transplantation from September 2005 to September 2007 at the Second Affiliated Hospital of Xi’an Jiaotong University. After cell purification, amplification, and identification, a 2 × 107/mL cell suspension was prepared for transplantation. In the posterior horn of the spinal cord 0.5 cm distal and proximal to the spinal cord injury zone, 4 needle points were selected to avoid the blood vessels. The needle depth was 3 mm, and the injection volume per point was 10 μL. Postoperatively and at 1 week, 4 weeks, 12 weeks, 24 weeks, 1 year, 3 years, 5 years, and 10 years after the surgery, the patient’s American Spinal Injury Association (ASIA) score, adverse reactions, and other minor observations were assessed.Results:All the patients did not have serious complications. No gliomas or other new organisms formed during the 10-year observation period. Eight of 13 patients had improvement in sensory function, and 5 patients showed improvement in motor function. The ASIA acupuncture, light touch, and exercise scores improved significantly 1 year after the surgery, and this improvement continued until the 10-year follow-up period. Three of 13 patients had improvement in defecation and urination, and 1 patient had improved neuralgia after spinal cord injury.Conclusion:OEC transplantation is safe and effective in treating spinal cord injury. The observation period of OEC transplantation is 1 to 3 years.

Laminoplasty with selective fusion at unstable segment versus laminectomy with fusion for multilevel cervical myelopathy: a case\u2010control study

BackgroundSegmental cervical instability is a risk factor for the progression of osteophytic bone spurs and development of myelopathy, and is treated as a relative contraindication of cervical laminoplasty. The aim of this study was to compare laminoplasty with selective fixation (LPSF) versus laminectomy with fusion (LCF) in patients with multilevel cervical myelopathy accompanied by segmental instability.MethodsA case-control study was conducted by reviewing data from 63 patients who underwent LPSF (n = 30) or LCF (n = 33). Cervical alignment, range of motion (ROM), neurologic status and axial symptom severity pre-operation, 3-days after operation, and at the final follow-up (minimum 24 months) were measured and compared between groups.ResultsPostoperation, patients in the LPSF group lost 31.1 ± 17.3 % of cervical lordosis and 43.2 ± 10.9 % cervical ROM while patients in the LCF group lost 5.7 ± 8.2 % and 67.9 ± 15.5 %, respectively. Both LPSF and LCF groups significantly improved neurologic status and axial symptom severity at the final follow-up with similar between-group results(P > 0.05). Blood loss, operation time, hospital stay, and medical cost in the LPSF group were significantly less than in the LCF group(P < 0.05).ConclusionsIn 2 years of clinical observation, LPSF was effective in maintaining the stability of the cervical spine with less sacrifice of mobility and surgical trauma for multilevel myelopathy with segmental instability compared to LCF.

Ten-Year Clinical Survival of Anterior Cantilever Resin-Bonded Fixed Dental Prostheses: A Retrospective Study.

To assess the 10-year clinical retention and survival rates of metal-ceramic (MC) and all-ceramic (AC) cantilever resin-bonded fixed dental prostheses (CRBFDPs). Forty CRBFDPs constructed from either cobalt-chromium ceramic (MC group; n = 20) or glass-infiltrated alumina ceramic (AC group; n = 20) were placed in 40 patients between August 2007 and December 2009. After baseline recordings, the patients were followed up using modified United States Public Health Services (USPHS) criteria after 6 months and thereafter annually for 10 years. Data were statistically analyzed using Kaplan-Meier estimation with log-rank (Mantel-Cox) test. The 10-year clinical retention rate was 95.0% in the MC group and 70% in the AC group. The difference was statistically significant (P = .02, log-rank test). Three all-ceramic CRBFDPs fractured at 6, 12, and 84 months after insertion. No statistically significant difference in survival rate was observed between the MC and AC groups over the 10 years of clinical observation (MC: 100%; AC: 85%; P = .075) (Kaplan-Meier method, confidence interval = 92.5% to 97.5%). Although glass-infiltrated alumina ceramic anterior CRBFDPs exhibited a lower clinical retention rate compared to metal-ceramic CRBFDPs, the debonded prostheses were recemented and continued in function over the observation period. Additionally, an acceptable 10-year clinical longevity was recorded.

Growing up with Bilateral parieto-occipital injury: over ten years of clinical observation and neuropsychological follow-up

ABSTRACTThis paper presents a follow-up of a child with Balint’s syndrome over more than a decade. The patient experienced traumatic brain injury before age 12, resulting in bilateral occipito-parietal infarctions and a clinical presentation of Balint’s syndrome. Neuropsychological assessments at three time points showed average verbal abilities alongside persistent difficulties in visual orientation, mirrored in the patient’s daily life. Her outstanding compensatory abilities in the face of these impairments are discussed with respect to the recruitment of the ventral visual stream and the role of top-down processing. This profile may help to determine interventions for younger patients with similar lesions.

Outcome of posterior fixed dental prostheses made from veneered zirconia over an observation period of up to 13 years

ObjectivesIt was the aim of this clinical study to evaluate the long-term outcome of 3- to 4-unit posterior all-ceramic fixed dental prostheses (FDPs) made from veneered zirconia ceramic. MethodsBetween June 2003 and February 2005, 48 patients received 58 restorations. Twenty-four FDPs had a fixed-to-fixed design (FF) and thirty-four FDPs had a cantilever design (CA). Frameworks were scanned and milled out of 3 mol% yttrium-oxide partially-stabilized zirconia ceramic (CerconBase30, Degudent) and were veneered with feldspathic ceramic (CerconCeramS, Degudent). All FDPs were cemented with glass-ionomer cement (Ketac-Cem, 3 MEspe) after air-abrading the inner crown surfaces. ResultsThe mean observation period was 85.4 ± 54 (FF) and 91.7 ± 50 (CA) months, respectively. Ten cantilever FDPs and 5 fixed FDPs were lost due to biological problems of the abutment teeth. Two cantilever FDPs and 4 fixed-to-fixed FDPs were lost due to technical failures of the ceramic materials. The cumulative 13-year survival rate was 43.2% (FF) (CI: 22.8–66.2%) and 52.5% (CA) (CI: 32.5–71.8), respectively. In 13 cases, intraoral repair or polishing of the fractured feldspathic ceramic veneers was needed (7 in group FF, 6 in CA). Eight abutments of six restorations (4 in FF, 4 in CA) had to be treated endodontically and caries therapy was needed in 6 abutment teeth (4 in group FF, 2 in CA). The cumulative 13-year success rate was 29.5% (FF) (CI: 12.1–55.9%) and 22.5% (CA) (CI: 7.9–49.3%). ConclusionsWithin the limitations of the present investigation, posterior fixed dental prostheses made from veneered zirconia with either a fixed-to fixed or a cantilever design show comparable survival and success rates after 13 years of clinical observation. Clinical SignificanceFDPs made from veneered zirconia ceramic present high failure and complication rates, irrespective of the design (fixed-to-fixed or cantilevered).

Myelodysplasia, Leukemia, Lymphoid Malignancies, and Other Cancers in Patients with Severe Chronic Neutropenia

Background: Since 1994 the Severe Chronic Neutropenia International Registry (SCNIR) has enrolled children and adults with > 3 absolute neutrophil counts (ANCs) < 0.5 x 109/L during a 3 month period to understand the pathobiology, natural history and treatment responses for severe chronic neutropenia. We have previously reported on the frequency and risk of myelodysplasia (MDS) and acute myeloid leukemia (AML) in patients with congenital neutropenia. For this report we reviewed patterns of hematological complications and malignancies occurring in all patients enrolled through the North American office of the SCNIR.Methods: Enrollment required informed consent, and patients and their physicians provided demographic, clinical and laboratory data including bone marrow results. Genetic testing was not required. Patients were followed with annual reports on blood counts, infections, malignancies and hospitalizations.Results: From 1994 to 2018 the Seattle SCNIR office has enrolled 1672 patients in the following categories: congenital 637 (38%), cyclic 259 (15%), and idiopathic / autoimmune 776 (47%), and many have been followed now for more than 15 years. There are approximately 17,577 person years of the observational data in this Registry. The congenital category now includes patients with mutations in ELANE, SBDS, TAZ, COH1, CXCR4, SLC37A4, G6PC3, WAS, CSF3R, SRP54, GFI1, VPS45, JAGN1, HAX1 and also patients with severe neutropenia from an early date in childhood without a genetic diagnosis. Cyclic neutropenia patients have demonstrated oscillations in ANC. The idiopathic and autoimmune category includes children and adults including some with large granular lymphocytes (LGL) syndrome without recognized features of a lymphoproliferative disorder. Most patients in all categories have been treated with granulocyte colony-stimulating factor (G-CSF).Findings: MDS or AML has occurred in 70 of the 1672 patients; 99% have clinical diagnosis of a hereditary type of neutropenia: severe congenital neutropenia (55), glycogen storage disease 1b (3), congenital immunodeficiency (2), Shwachman-Diamond syndrome (SDS) (5), WHIM syndrome (1),Wiskott-Aldrich syndrome (2), cyclic neutropenia (1) and idiopathic neutropenia (1). The median age at diagnosis of AML/MDS was 15.3 years (mean 18.3, +/- 1.79 SEM [range 0.40 - 70.6]); 69 of 70 were treated with G-CSF, median dose = 7.1 mcg/kg/day (mean 7.3, +/- 1.3 SEM )(range 0.18 - 100). One Shwachman-Diamond patient never received G-CSF. Outcomes for AML/MDS patients receiving chemotherapy with HSCT before 2000 were poor with 3/17 (18%) survivors. Since 2000 there were 35/53 (66%) survivors. Five patients developed myelofibrosis (4 congenital and 1 idiopathic). Two of the congenital patients later developed AML (1 living after treatment with a HSCT, 1 deceased). The clinical diagnosis of cyclic neutropenia has a favorable prognosis with G-CSF treatment, with only one probable case in 3,833 person years of clinical observation. 1Twelve patients developed T-cell lymphoproliferative disorder (1 autoimmune neutropenia, 3 congenital neutropenia, 8 idiopathic neutropenia (4 with LGL features)). Five of these patients are living, all in the idiopathic group, 3 of 5 living patients have features of the LGL syndrome.Ten patients have reported other hematological malignancies; CML in a congenital patient after treatment with HSCT (living), CLL in a cyclic patient (living), CMML in an idiopathic patient after treatment with a HSCT (deceased). Six of the 10 patients have developed lymphoma; cyclic neutropenia (1), idiopathic/autoimmune neutropenia (5). Only one SDS patient has developed aplastic anemia.Other cancers/non-hematological malignancies have occured mostly in older patients: breast cancer (15) colon cancer (6), dermatological malignancies (13), hepatoma (1), lung cancer (1), prostate cancer (1), thyroid cancer (1).Conclusions: The hematological consequences of severe chronic neutropenia depend on the underlying etiology. MDS and AML occur largely in patients with the congenital or hereditary neutropenias. The diagnosis of cyclic neutropenia and chronic idiopathic / autoimmune neutropenia portends a favorable prognosis, based on a total of 10482 person years of observation. Marrow failure and aplastic anemia are not expected consequences of severe chronic neutropenia. DisclosuresDale:Athelas, Inc.: Equity Ownership; Amgen: Consultancy, Research Funding; Sanofi-Aventi: Consultancy, Honoraria; Cellerant: Other: Scientific Advisory Board; Hospira: Consultancy; Prolong: Consultancy; Beheringer-Ingelheim: Consultancy; Coherus: Consultancy. Newburger:X4 Pharmaceutics: Consultancy, Honoraria; TransCytos LLC: Consultancy; Janssen Research & Development, LLC: Consultancy, Honoraria.

What is the Core Symptom and Neural Mechanism of Asperger Syndrome? From Our Ten Years of Clinical Observation of Adults with Autism Spectrum Disorder

In 2008, we launched a new outpatient clinic and day care service designed exclusively for adults with autism spectrum disorder (ASD). Since then, more than 6000 people visited our facility. We have also launched the Medical Institute for Developmental Disabilities Research (MIDDR) at Showa University, where neuroimaging studies with a 3.0 Tesla MRI scanner and rehabilitation studies intended to support people with ASD for job seeking were conducted for the past five years. As our subjects were found to have normal or high intelligence, we present here our research studies on Asperger syndrome patients with the focus on their resting-state functional connectivity network. We also review hypotheses of brain functioning in Asperger syndrome.

Oral health-related quality of life as a predictor of subjective well-being among older adults-A decade-long longitudinal cohort study.

The objective of this study was to evaluate possible longitudinal associations between oral health-related quality of life (OHRQoL) and subjective well-being among quinquagenarians and septuagenarians over up to 10years of clinical observation. This research is part of the "Interdisciplinary Study on Adult Development and Aging (ILSE)." All participants lived in an urban region of southwest Germany. One hundred and fifty-two participants born in 1930-1932 (older cohort, OC; n=54) and 1950-1952 (younger cohort, YC; n=98) underwent comprehensive psychological, medical and dental examinations at baseline and up to 10years thereafter. The Geriatric Oral Health Assessment Index (GOHAI) was used to assess OHRQoL. The Philadelphia Geriatric Center Morale Scale (PGCMS), general life satisfaction (GLS) and individuals' general satisfaction with their health (SWH) were used to evaluate subjective well-being. Regression models and structural equationmodelling (SEM) were used to analyse the longitudinal associations between OHRQoL and subjective well-being. A cross-lagged prediction model revealed a positive association between baseline OHRQoL and subjective well-being across 10years for both birth cohorts, OC [0.49 (0.21; 0.69)] and YC [0.36 (0.24; 0.52)]. However, higher subjective well-being at baseline was not a predictor of better OHRQoL 10years later for either cohort. OHRQoL seems to be a substantial predictor of subjective well-being in quinquagenarians and septuagenarians across the rather long observational period. Further studies with larger sample sizes are required to confirm this finding.

Risk factors for tooth loss in middle and older age after up to 10 years: An observational cohort study

ObjectiveThe objective of this research was to identify risk factors for tooth loss in two birth cohorts, quinquagenarians and septuagenarians, after up to 10 years of clinical observation. DesignOne hundred and twenty-three participants were recruited from the Interdisciplinary Study of Adult Development (ILSE) and examined at baseline and up to 10 years after. Thirty-nine and 84 participants belonged to the older (OC; born in 1930/32) cohort and younger (YC; born in 1950/52) cohort, respectively. Each participant underwent a dental examination comprising evaluation of the dental status (number of teeth, prosthetic restorations), Plaque Index (PI), Gingival Index (GI), DMF-S, periodontal probing depths (PD) and tooth mobility (TM). Incidence of tooth loss over the study period was calculated for both cohorts as well as for the grouped dental target variables. A logistic regression model for tooth loss (0=tooth present/1=tooth lost) was compiled with possible binary confounders. ResultsDuring the study period (eight years in mean), 1.2 (1.9) and 2.6 (2.6) teeth were lost in YC and OC, respectively, reflecting correspondent loss rates of 5% and 14% (p<0.001). However, primarily TM >1 merged into substantial tooth loss (60% lost). The regression analysis confirmed the bivariate findings. Older age and worse oral health issues were identified as risk factors for tooth loss(p<0.05). ConclusionsBoth quinquagenarians and septuagenarians show relevant tooth loss over a period of up to 10 years but more in septuagenarians. The predominant predictor for tooth loss seems to be greater tooth mobility. With the rising challenges due to aging in several societies, knowing the risks might help clinicians when weighing treatment strategies and should encourage refining preventive measures for older patients.

Clinical Management Protocol for Dental Implants Inserted in Patients with Active Lichen Planus.

To propose a treatment protocol for management of implant candidates suffering from active lichen planus. Twenty-three patients who were diagnosed with active lichen planus were referred to a dental practice seeking dental implants. Patients received dental implants using open flap technique and sequential osteotomy. After a healing time of 6 weeks, implants were put into occlusal contact using interim prostheses. Of the 55 inserted implants, 42 failed after a short clinical service time (7 to 11 weeks). Failed implants were surgically removed, and osteotomy sites were carefully cleaned using manual curettes and scrubbing of osteotomy walls. CD4/CD8 cell count and gingival biopsies were collected from the surgical sites. Patients were put on an ascending dose (5 mg/10 days) of oral corticosteroids until a daily dose of 20 mg/day was achieved and maintained for 2 weeks. All patients received ten sessions of low-energy soft tissue laser irradiation at the implant insertion sites to assess soft tissue healing through improving blood circulation, reduction of inflammation, and sterilization of the osteotomy region. After approximately 8 weeks, a new set of 42 implants was placed, and definitive restorations were cemented using early loading technique. Marginal bone levels around inserted implants were periodically evaluated every 3 months using digital periapical radiographs (α = 0.05). Primary blood tests revealed a high CD8 cell count, while soft tissue biopsies revealed infiltration of inflammatory cells at the epithelium soft tissue interface of the examined tissue. Repetition of immunological investigations after administration of the oral corticosteroids and soft tissue laser irradiation revealed reduction of CD8 cell counts and disappearance of inflammatory cell infiltration of the examined soft tissue biopsy. Patients reported marked improvement of the oral mucosa after treatment. All implants (n = 42) were functional after 3 years of clinical observation. There was an initial reduction in marginal bone level after 3 months from insertion time, after which it remained constant during the following observation periods. Active lichen planus should be managed with oral corticosteroids and soft tissue laser irradiation before insertion of dental implants.

Regard sur l’expression de la souffrance psychique de l’enfant en Martinique aujourd’hui

In 25 years of clinical observation in ambulatory practice, there has been a radical change in ways children express symptoms of distress. Overview of clinical aspects and questions.

Course of Atypical Manifestations of a Case of Charcot-Marie-Tooth Disease over 35 Years of Clinical Observation

Introduction: Atypical manifestations of peripheral neuropathy are not rare, challenging the differential diagnosis. In the past, the diagnosis of hereditary neuropathy was mainly based on the clinical and electromyographic (EMG) findings and, occasionally, biopsy. Nowadays, the genetic tests allow us to identify more than 40 different genes/loci associated with Charcot-Marie-Tooth (CMT) disease, although some subtypes are clinically indistinguishable. We have followed a patient with a clinical diagnosis of apparent sporadic and atypical CMT and recently diagnosed genetically as distal hereditary motor neuropathy, type V (dHMN-V). Case Report: Thirty-five years ago, a 16 years old patient complained muscular weakness and wasting at the hands small muscles. Sporadic fasciculations were observed whereas deep tendon reflexes and sensation were normal. EMG examination revealed neurogenic muscular denervation in the distribution of C7, C8 and T1 segments bilaterally. Muscular biopsy of the left Biceps Brachii showed rare atrophic fibers and some cellular atypia. The disease has undergone a clinical and EMG progression and diffusion over the years, involving the lower limbs and leading to a bilateral steppage. A more slight diffuse axonal motor neuropathy was also identified in the proband’s son and second cousin. The genetic study found a known missense mutation in BSCL2 gene related to a dHMN-V. Interestingly, there was a remarkable intra-familiar phenotypic variability, especially in the clinical onset and severity. Discussion: Atypical manifestations of hereditary neuropathies often overlap with other conditions. The present case highlights how a comprehensive clinical evaluation and a careful follow-up have led to a correct diagnosis even 35 years later and have allowed to identify other affected family members. The apparent lack of familiarity was probably due to the very soft presentation in the proband’s relatives. Although the genetic study was not available at that time, the first clinical diagnosis was not disavowed. The main differential diagnoses and a brief review of similar reported cases are discussed.

Effects of summer acupoint application therapy in reducing exacerbation frequency of chronic lung diseases: protocol of a retrospective and prospective study

Chronic lung diseases, including bronchial asthma, chronic obstructive pulmonary disease (COPD), chronic bronchitis, allergic rhinitis and repeated respiratory tract infection (RRTL) in infants, exacerbate frequently in winter because of respiratory viral infections and low temperature. Summer acupoint application therapy (SAAT) is thought to be effective in reducing exacerbation frequency of chronic lung diseases in winter. It is a kind of therapy using a herbal mixture for external application on special acupoints during summer. The herbal mixture basically contains Semen Sinapis Albae, Herba Asari, Radix Euphorbiae Kansui and Rhizoma Corydalis. The acupoints include Feishu (BL13), Dazhui (GV14) and Danzhong (CV17). Through a large-scale multicenter trial based on three years of clinical observation, and retrospective and prospective analyses, this study aims to explore the efficacy of SAAT. In this clinical observation trial, patients who have been diagnosed with bronchial asthma, COPD, chronic bronchitis, allergic rhinitis or RRTL will be enrolled from 13 centers. All patients enrolled will be treated with SAAT over a two-year period by medical practitioners. After this, an initial case report form (CRF) will be completed and forwarded to the central study site (China Academy of Chinese Medical Sciences, Beijing, China). The CRF is designed to investigate patients' history of medical treatment (including SAAT) and chronic lung disease exacerbation, also self-reported health condition. For retrospective analyses, the authors will focus on those who have accepted SAAT before enrollment and will collect their SAAT history and chronic lung disease exacerbation history, to evaluate the effects of SAAT. For prospective analyses, medical students will follow up with patients by phone interviews in winter once a year. The primary outcome is frequency of chronic lung disease exacerbation in winter. The secondary outcomes include conditions of lung disease recovery, non-SAAT therapy, and a self health report. The authors aim to collect 7 400 patients from July 2008 to August 2009. The final follow-up has been completed in December 2010. To reduce the selection bias, a total of 13 clinical centers from different areas of China have participated in this study. The results from this study will provide a high-quality evidence base for evaluating the efficacy of SAAT in reducing exacerbation frequency of chronic lung diseases in winter. This trial has been registered in Chinese Clinical Trial Registry (Identifier: ChiCTR-TNRC-10001292).

Vaccination with apoptosis colorectal cancer cell pulsed autologous dendritic cells in advanced colorectal cancer patients: Report from a clinical observation

To investigate vaccination with apoptosis colorectal cancer (CRC) cell pulsed autologous dendritic cells (DCs) in advanced CRC, 14 patients with advanced colorectal cancer (CRC) were enrolled and treated with DCs vaccine to assess toxicity, tolerability, immune and clinical responses to the vaccine. No severe toxicity was observed and the vaccine was well tolerated. The levels of interleukin-2 (IL-2), interleukin-12 (IL-12) and interferon-γ (IFN-γ) significantly increased after DCs vaccination (P < 0.05). Mean of carcinoembryonic antigen (CEA) lever decreased from 99.5 to 71.4 ng/ml. Five patients showed a positive skin response to the apoptotic cells loaded DC vaccines in the delayed type hypersensitivity (DTH). In the five DTH positive patients, we found a patient with a lymphoglandulae coeliacae metastasis; temporary seroperitoneum regression was demonstrated by CT scan after full immunizations during 12 weeks. However, a rescan after 6 weeks showed new lesions on the abdomen as well as re-growth of the primary lesion. During the two years of clinical observation, all patients survived the first year, while the survival rate decreased from 80 to 20% during the second year owing to the advancement of CRC. In conclusion, the strategy for vaccination of apoptosis colorectal cancer cell pulsed-DCs may raise the clinical response of CRC patients by activating Th1 responses. Furthermore, indications of both immunologic and clinical effect were found in heavily pretreated patients with advanced colorectal cancer. Key words : Dendritic cells, immunotherapy, colorectal cancer.