Latest Visit Research Articles

Long-term prognosis and factors associated with damage accrual in Japanese patients with systemic lupus erythematosus

Using a single-center cohort of Japanese patients with SLE, we attempted to clarify the long-term outcome and factors associated with damage accrual using the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI). We examined a cohort of 557 patients who had been referred to Niigata University Hospital and diagnosed as having SLE between 1961 and 2013. The patients' data at the latest visit were collected from their clinical records, and causes of death were defined on the basis of those data. Survival from the time of diagnosis was calculated by the Kaplan-Meier method. The SDI was calculated and analyzed using Spearman's correlation coefficient and stepwise multiple regression analysis to reveal the factors associated with any organ damage. Data from 458 of the patients were successfully obtained. The overall 5-year survival rate was 92.2%, and patients diagnosed after 2000 had a significantly high 5-year survival rate of 96.4%. Stepwise multiple regression analysis selected serum creatinine levels (B=0.6051, p<0.0001), age (standardized beta=0.2762, p<0.001), hypertension (standardized beta=0.2267, p<0.001), and antiphospholipid antibody syndrome (standardized beta=0.1533, p=0.005) as positive independent variables, whereas administration of bisphosphonate (standardized beta=-0.1295, p=0.016) was selected as a negative independent variable. These results suggest that Japanese patients with SLE have a favorable long-term prognosis, and also indicate that disease control as well as management of chronic complications such as hypertension and osteoporosis has possible effects for prevention of organ damage.

Long-term follow-up of clinical trial patients treated for chronic HCV infection with daclatasvir-based regimens.

Background & AimsDaclatasvir has achieved high sustained virologic response (SVR) rates in diverse hepatitis C virus (HCV) populations. This study evaluated the long‐term efficacy and safety of daclatasvir‐based regimens administered during clinical studies.MethodsPatients enrolled within 6 months of parent study completion or protocol availability at the study sites. The primary objective was durability of SVR at follow‐up Week 12 (SVR12). Secondary objectives included analysing HCV sequences in non‐responders or responders who relapsed, and characterization of liver disease progression.ResultsBetween 24 February 2012 and 17 July 2015, this study enrolled and began following 1503 recipients of daclatasvir‐based regimens (follow‐up cut‐off, 13 October 2015); 60% were male, 18% aged ≥65 years, 87% had genotype‐1a (42%) or ‐1b (45%) infection, and 18% had cirrhosis. Median follow‐up from parent study follow‐up Week 12 was 111 (range, 11‐246) weeks. 1329/1489 evaluable patients were SVR12 responders; 1316/1329 maintained SVR until their latest visit. Twelve responders relapsed by (n = 9) or after (n = 3) parent study follow‐up Week 24; one was reinfected. Relapse occurred in 3/842 (0.4%) and 9/487 (2%) responders treated with interferon‐free or interferon‐containing regimens, respectively. Hepatic disease progression and new hepatocellular carcinoma were diagnosed in 15 and 23 patients, respectively. Among non‐responders, emergent non‐structural protein‐5A (NS5A) and ‐3 (NS3) substitutions were replaced by wild‐type sequences in 27/157 (17%) and 35/47 (74%) patients, respectively.ConclusionsSVR12 was durable in 99% of recipients of daclatasvir‐based regimens. Hepatic disease progression and new hepatocellular carcinoma were infrequent. Emergent NS5A substitutions persisted longer than NS3 substitutions among non‐responders.

Bariatric surgery in HIV patients: experience of an Obesity Reference Center in France

BackgroundFew data on bariatric surgery are available regarding obese human immunodeficiency virus (HIV)-infected patients. SettingsAntoine Beclere hospital, Clamart, Paris-sud University, France MethodsProspective observational follow-up study recruited HIV-infected patients who underwent bariatric surgery from 2009 to 2015. Baseline demographic characteristics, surgery characteristics, perioperative outcomes, changes in weight loss, HIV markers, antiretroviral drug plasma levels are described. ResultsThere were 10 patients followed before and after sleeve gastrectomy: 2 men and 8 women; 50% of African origin; median age, 48.5 years, median time since HIV infection, 7.5 years; median body mass index, 48.5 kg/m2. Of patients, 8 had co-morbidities. All except 2 patients received antiretroviral drugs at the time of surgery with a median CD4 cell count at 709/mm3. There was no death or postoperative infectious complications. The median follow-up was 18 months (range, 15–55). The median postoperative weight loss was 43 kg (range, 17–83). Median percentage of excess weight loss was 82.5% (range, 35–119) at the latest visit after surgery. All co-morbidities were resolutive with weight loss. We observed no significant modification of CD4 cell count before and after surgery. Pharmacokinetics of antiretroviral drugs remains adequate and efficacious. ConclusionOur prospective series is the largest one on sleeve gastrectomy procedures performed on obese-treated HIV-infected patients. The sleeve generates good results in weight loss, with no significant impact on HIV infection, and with improvement of obesity-associated co-morbidities. Optimal management of HIV-infected patients with morbid obesity may include classical surgical procedures.

Minimal Invasive Reconstruction of Neglected Achilles Tendon Rupture

Category: Sports Introduction/Purpose: Recently minimal invasive reconstruction using semitendinosus tendon autograft was shown to be safe and successful for neglected Achilles tendon ruptures. We hypothesized that allograft could also result in similar result like in the reconstruction of anterior cruciate ligament of the knee joint. Methods: Between December 2013 and September 2015, 19 consecutive neglected Achilles tendon rupture cases underwent minimally invasive reconstruction using semitendinosus allograft tendon regardless of rupture time and gap. Out of them, 5 patients had flexor hallucis longus tendon transfer additionally. Thirteen were male, and the average age at the time of surgery was 63(30~82). MRI was checked to confirm the diagnosis in all cases. The Achilles tendon total rupture score(ATRS) was measured for clinical outcome at both pre and post surgery. Thompson squeezing test, knee flexion test, and single-heel-rise test were recorded. Isokinetic strength of plantar flexion by peak torque with plantar flexion at speeds of 30 and 120°/sec was measured to check triceps muscle power after surgery. Results: The mean follow-up period was 13.6(6~26) months. The ATRS was improved from 34(7~72) to 70(48~97) after the surgery(p&lt;0.0001). At the latest visit, all patients showed negative Thompson squeezing test, and in knee flexion test 16 patients showed normal ankle plantar flexion. Thirteen patients could raise their affected heel at the time. In the affected leg at speeds of 30 and 120°/sec, the isokinetic strength was 14.9(6.0~31.9) and 10.0 Nm (2.8~ 19.0) Nm preoperatively, and 37.3(12.1~90) and 19.6(7.8~47) Nm at the latest follow-up. (P=0.012, 0.017) There was no wound problem, rerupture or other complications related to the surgery. Conclusion: Minimally invasive reconstruction using semitendinosus allograft tendon seems to safe and effective for the treatment of neglected Achilles tendon without sacrificing other healthy tissues. We think that this technique can provide a significant improvement of symptoms and function with fewer complications.

Glycemic control in Type 2 Diabetes patients attending a clinic in Vijayawada, a South Indian city

Objective: Andhra Pradesh (A.P), a southern state in India, is faced with a significant burden of diabetes. Few studies are there mentioning the prevalence in some parts of the state and as a state as a whole. There is limited information regarding the glycemic control of these patients. We analyzed the status of glycemic control in a sample of patients attending a diabetic clinic, based at Vijayawada, a major city in A.P. Methods: Data from patient records was collected for those with first visit during 2006 and analyzed for glycemic parameters at first visit and at 10 year follow up. Results: A total of 104 Type 2 Diabetes Mellitus patients details have been considered for the study. Mean age of the sample at first visit is 51.2 years. Mean duration of follow up is 9.7 years. During this follow up the mean HbA1C is reduced significantly from 9.5 % at first visit to 7.8% at latest visit (?HbA1C=1.72, P<0.001). FBS and PPBS are also reduced significantly by 17.6 mg/dl (P<0.01) and 28.2 mg/dl (P<0.01) respectively. Proportion of patients achieving HbA1C target of less than 6.5%, 7% and 7.5% are 18%, 29% and 30% respectively at the latest visit. There is a slight increase in weight by 2 kg and a change of BMI by 0.98 kg/m 2 between first and latest visit. Conclusion: Glycemic control in this clinic is satisfactory in terms of HbA1C control and proportion of patients achieving target.

SIGNS OF EARLY COGNITIVE DECLINE WITHIN CONNECTED SPEECH: EVIDENCE FROM THE WISCONSIN REGISTRY FOR ALZHEIMER'S PREVENTION (WRAP)

Alzheimer's disease (AD) is primarily characterized by early memory impairment; however, many patients show deficits in language across all stages of AD. Typical language testing is insensitive to early language problems that may manifest in discourse. We examined connected speech longitudinally in a group of late-middle-aged adults at risk for AD. We hypothesized that those with early Mild Cognitive Impairment (eMCI) would show decline in connected speech over time compared to cognitively healthy (CH) adults. First, we submitted connected speech measures from a picture description task to exploratory and confirmatory factor analyses (EFA, CFA) from a subgroup of cognitively healthy WRAP participants (n=399; mean age=60±7, 67% female). Next, we examined resulting factor scores as outcomes in a subset of participants with speech samples obtained at 2 time points (n=219), using a linear mixed effects model (LME), with fixed effects of time point and baseline cognitive status (CH/eMCI), and a random effect of intercept nested within subjects. Secondary analyses used logistic regression to investigate the effect of speech factors on cognitive status at latest visit. The EFA/CFA factor structure (Fig 1, Table 1) met goodness-of-fit criteria. Participant characteristics for longitudinal analyses are presented in Table 2. Cognitive status at time 1 was a significant predictor of Semantic (p =.007) and Syntax (p <.001) factors, with a significant interaction of time and cognitive status for the Semantic factor (p =.04) (Table 3, Fig.2). Syntax was a significant predictor of eMCI (p <.0001), as was change in Fluency (p=.01) (Table 4). This is the first study to demonstrate a confirmed factor structure of connected speech measures in a prospective AD-risk-enriched cohort. Evident are baseline differences between eMCI and CH in syntax complexity. Baseline syntax and change in Fluency were significant predictors of eMCI at the latest visit. Unexpectedly, participants with eMCI performed higher on the Semantic factor at baseline; however, they declined more steeply than the CH group over time. Future analyses will continue to examine longitudinal relationships among speech factors and subtypes of cognitive impairment (memory, executive function, language), which may contribute to early identification of and intervention for AD. Conceptual model submitted to confirmatory factor analysis using the cross-validation subsample(n=149). Note: CFA met the following satisfactory fit indices: χ2=35.63, CFI = .99, NNFI = .99, RMSEA=.04, 90% confidence interval=.00, .08, GFI=.98, AGFI=.97, SRMR = .07. Definitions: SUID is Semantic Unit Idea Density (number of information units/total words). Type-token ratio: number of unique words/total words. Pronoun index: total pronouns divided by nouns + pronouns. Maze Index: total number of revisions, repetitions, and filled pauses/total words. Verb Index: total number of verbs/utterances. Grammatical complexity: The number of grammatical relations that mark syntactic embeddings divided by the total number of grammatical relations. Density: ratio of the propositions corresponding to verbs, adjectives, adverbs, prepositions and conjunctions to the total number of words (excluding repetitions and fillers). Boxplots of Connected Speech Factor by Visit and Consensus Diagnosis at Speech Time 1 (unadjusted means).

Deep brain stimulation for Tourette syndrome: a single-center series.

OBJECTIVE Tourette syndrome (TS) is a complex neuropsychiatric disorder characterized by multiple motor and phonic tics. While pharmacological and behavioral therapy can be effective in most patients, a subset of patients remains refractory to treatment. Increasing clinical evidence from multiple centers suggests that deep brain stimulation (DBS) of the medial thalamus can be effective in many cases of refractory TS. METHODS The authors retrospectively reviewed outcomes in 13 patients with refractory TS who underwent medial thalamic DBS performed by their team over a 7-year period. Patients were evaluated by a multidisciplinary team, and preoperative objective assessments were performed using the Yale Global Tic Severity Scale (YGTSS) and Yale-Brown Obsessive Compulsive Scale. YGTSS scores were calculated at visits immediately postoperatively and at the most recent follow-up in patients with a minimum of 6 months of postoperative follow-up. Coordinates of the active DBS contacts were calculated and projected onto each patient's pre- and postoperative images. RESULTS Patients showed an average decrease of 37% (p = 0.0063) in the total tic severity at their first postoperative visit. At their latest visit, their scores achieved significance, decreasing from preoperative scores by an average of 50% (p = 0.0014). The average position of the active contact was noted to be at the junction of the posterior ventralis oralis internus/centromedian-parafascicular nuclei. Device-related complications occurred in 2 patients, necessitating additional surgeries. All patients continued to use the system at last follow-up. CONCLUSIONS The authors' data are consistent with the small but growing body of literature supporting DBS of the ventralis oralis internus/centromedian-parafascicular thalamus as an effective and relatively safe treatment for severe, refractory TS.

Social differences in who receives questions and advice about smoking habits when visiting primary care - Results from a population based study in Sweden in 2012.

The aim of this study was to examine whether there are social disparities in who receives questions and advice on smoking habits when visiting primary care and whether these disparities can be explained by differences in smoking habits. The study is based on 30,188 individuals aged 16–84 years who responded to a population survey questionnaire in 2012 in four counties in mid-Sweden (response rate 51%). Multivariate logistic regression models were used in statistical analyses. A total of 32% of those who visited a health care centre during the last three months reported that they were asked about their smoking habits during their latest visit, 6% received advice. In general, daily smokers received more often questions, and especially advice, than non-smokers. Persons with low education received more advice than persons with high education due to higher smoking prevalence. However, persons on disability pension and the unemployed were less frequently asked about their smoking habits than employees even though they smoke more. Women received less often questions and advice than men. Persons born outside the Nordic countries received advice twice as often as native Swedes regardless of whether they were daily smokers or not. In Sweden, those who are asked and, in particular, receive advice about changing their smoking habits while visiting primary care are mainly those who need it most. But the findings also imply that measures to reduce smoking should be intensified for women and are perhaps too intense for persons born outside the Nordic countries.

Clinical outcome of artificial urinary sphincter implantation to treat complex urinary incontinence

Objective To present the experience of artificial urinary sphincter implantation as a part of urinary tract reconstruction for patients with refractory urinary incontinence. Methods Between April 2002 and April 2016, a total of 30 patients (median age, 40.2 years, 29 males, 1 female ) with urinary incontinence had accepted artificial urinary sphincter placement during urinary tract reconstruction. Assessments included case selection, perioperative management, urinary continence, artificial urinary sphincter status, complications, quality of life and additional procedures. Results The mean follow-up time was 52.8 months ranged from 25 months to 13 years. At the latest visit, 23 patients (76.7%) maintained the primary artificial urinary sphincter. Four patients (13.3%) had artificial urinary sphincter revisions. Explantations were performed in three patients. Twenty-two patients were socially continent, of which 14 patients were totally dry, leading to the overall success rate as 73.3%. There was a significant reduction in pad count from 3.8±0.3 to 1.1±0.3 diapers per day (P<0.001). There was a significant reduction on the impact of urinary incontinence on quality of life, with a decrease from 6.9 ± 0.3 to 1.9 ± 0.5 (P<0.001) on a visual analogue scale (VAS). The complication rate was 26.7%; including infections (n=4), erosions (n=3), and mechanical failure (n=1). Conclusions Artificial urinary sphincter implantation is an effective treatment as a key procedure for urinary tract reconstruction, especially in complicated urinary incontinence cases. Other than post-prostatectomy incontinence cases, the quality and choice of management modalities should be tailored to the unique needs of each individual with caution. Key words: Artificial urinary sphincter; Urinary incontinence; Urinary tract reconstruction; Complication; Follow up

Survivor pain: Experience at an oncology palliative care clinic.

260 Background: As of January 2014 there are an estimated 14.5 million cancer survivors in the United States. In observational studies of cancer survivors up to 40% report chronic pain. Opioids remain an important and effective treatment for active cancer pain. For survivors, however, we must take into account the paucity of data supporting long term benefits of opioids on pain and functional outcomes, and extensive evidence of potential harms. We present data from our palliative care clinic where we have established a nuanced approach to pain management in this population, which includes risk mitigation, consistent education, emphasis on non-pharmacological and non-opiate medications, and attempts to down-taper opioid analgesics when feasible. Methods: Retrospective, observational chart review of 62 patients identified as cancer survivors, who have been managed for pain, at an academically affiliated palliative care clinic embedded in the oncology department. Demographic information, baseline and latest visit pain scores and functional assessments, as well as pain regimen data were collected. Means for pain and functionality scores were compared for those patient’s with differing opioid regimens and nonpharmacological treatments. Results: Of the 62 patients reviewed, 48 were tapered off opioid medication. In the patients who were tapered off opioid medications, 8 (16%) showed an increase in average pain scores, 11 (22%) showed no change in average pain scores, and 29 (60%) showed improvement in average pain score. Conclusions: In 82% of the patients whose opioid medications were decreased, average pain scores either remained the same or actually improved over time. This is encouraging data to support the theory that decreasing opioids in cancer survivors does not worsen pain control in the majority of that population.

Mid-to-long-term follow-up results of transcatheter closure of atrial septal defect in patients older than 40years.

We investigated the mid-to-long-term results of transcatheter closure of atrial septal defect (ASD) in patients ≥40years since there are issues with patients presenting with pulmonary hypertension and arrhythmia at the time of closure. In an 8.8 year period, transcatheter closure of ASD was successful in 179 patients aged ≥40years, but failed in 2. Of the 179 patients (44 males, median 53years), NYHA functional class, presence of arrhythmia and severity of pulmonary hypertension were compared before and after closure. Patients with pulmonary hypertension (n=43, 24%) were significantly older (60±11 vs. 52±9years, p<0.01) and required larger devices (27±7 vs. 24±7mm, p=0.04) than those without. Arrhythmia before intervention was documented in 31 patients (17.3%): 22 atrial fibrillation (AF), 4 atrial flutter and 5 supraventricular tachycardia. Patients with AF or atrial flutter (n=26) were significantly older (63±10 vs. 53±10years, p=0.048) and had a higher pulmonary artery mean pressure (29.2±12.6 vs. 20.2±7.6mmHg, p=0.041) than those without. The mean follow-up period was 3.8±2.1years. Early new-onset arrhythmia was documented in 23 patients of whom 1 had persistent AF, 1 developed sick sinus syndrome and others were in sinus rhythm at latest visit. There was significant improvement in NYHA functional class after closure (p<0.001). Of the 22 patients with AF, 10 were in sinus rhythm, 1 had paroxysmal AF, and 11 had persistent AF. Pulmonary hypertension persisted in 13 patients. Transcatheter closure of atrial septal defect in patients above 40years is beneficial in terms of NYHA functional class, pulmonary artery pressure and cardiac rhythm.

The Mid-Term Outcome of Total Knee Arthroplasty in Patients With Prior High Tibial Osteotomy: A Prospective Study

Background: It is generally accepted that total knee arthroplasty (TKA) after high tibial osteotomy (HTO) is technically more difficult; however, there is still disagreement whether the subsequent arthroplasty is compromised. Objectives: The current case series study aimed to assess patients treated by knee arthroplasty after a previous high tibial osteotomy and to determine the influence of this osteotomy on the clinical and radiologic outcomes of subsequent arthroplasty. Methods: Up to April 2013, twenty-three patients with a history of twenty-five proximal tibial osteotomies prior to TKA were identified and compared with a matched group who underwent primary TKA in Shafa Orthopedic Hospital, Tehran, Iran. Demographic parameters of patients, surgical details, the knee range of motion (ROM), and American functional knee society (AKS) scores before and after TKA were recorded in the HTO and control groups. Weight bearing alignment radiographs were taken preoperatively and at the last follow-up. At the latest visit, patients’ satisfaction was recorded. Results: After an average of 49 months follow-up, all patients in the HTO and control groups were satisfied with their current level of function. In the HTO group, the rectus snip had to be used for better exposure in eight cases. There was no statistically significant difference between the two groups in postoperative knee ROM, mechanical leg alignment and mean functional knee society score, but the mean of operation time in the HTO group was significantly longer than that of the TKA time in the control group. Conclusion: Although knee arthroplasty after HTO is technically more challenging than a primary procedure, the results of clinical scores, postoperative ROM and radiological evaluation in the study subjects were comparable with those of the primary TKA in midterm follow-up. Rectus snip is a safe procedure in such difficult cases for better exposure.

Macular Hole Retinal Detachment Following Surgical Removal of Myopic Choroidal Neovascularization: A Case Report

AIM: To report a case of Japanese woman who developed macular hole retinal detachment (MHRD) following surgical removal of myopic choroidal neovascularization (CNV). PATIENT: The best-corrected visual acuity (BCVA) was 20/200 with -17.0 diopters in her left eye. She was diagnosed with juxtafoveal CNV and subretinal hemorrhage secondary to myopia. She underwent surgical removal of the CNV. Postoperatively, the CNV was not detected. 2.5 years postoperatively, the BCVA was 20/16. At 6.5 years postoperatively, however the patient presented with worsening vision. The BCVA was 20/220. Funduscopic examination as well as optical coherence tomography (OCT) revealed retinal detachment involving the posterior pole with a macular hole. The patient underwent a vitrectomy without removal of the internal limiting membrane (ILM), and silicone oil tamponade. Finally, Silicone oil was removed. At the latest visit, a flattened macula with a closed macular hole was observed by OCT. The BCVA was 20/100. CONCLUSION: In this case, the reason for the development of MHRD without vitreous and spontaneous closure of the macular hole without vitreous or removal of the ILM is unknown. A higher index of suspicion of MHRD may be warranted for patients who complain of sudden vision loss after surgical treatment for myopic CNV.

Syria

Syria

Eicosapentaenoic Acid as long-term secondary prevention after ischemic stroke.

BackgroundIt is sometimes difficult to choose anti-thrombotic agents for secondary prevention in stroke patients at high bleeding risk. Recently, Eicosapentaenoic Acid (EPA) was reported to reduce the recurrence of stroke in hypercholesterolemic patients without increasing hemorrhagic risk. In this study, we investigated the features of recurrent stroke patients during EPA medication as secondary stroke prevention.MethodsFollowing the approval of the ethical committee, stroke patients in the outpatient clinic were consecutively screened and patients who continuously take EPA were enrolled in this study (n = 71, average age 69.7 yo). Blood sample data was adopted from the latest visit or the admission at the stroke recurrence. According to the previous stroke history, all patients were classified into the hemorrhagic stroke (HS) group (n = 10) and the ischemic stroke, including asymptomatic infarction, (IS) group (n = 61).ResultAny stroke recurrence was not observed in the HS group. Whereas, ischemic stroke recurrence was observed in 6 patients in the IS group, although there was no hemorrhagic stroke recurrence. Recurrent stroke patients showed the higher serum level of cholesterol or the renal dysfunction. The stroke subtype of patients were 2 embolic strokes, 3 atherothrombotic infarctions (two were compromised with renal failure and one had insufficient amount of EPA) and one lacunar infarction (who showed high triglyceride level).ConclusionHemorrhagic stroke was not occurred in our observation of EPA prescribed patients. The clinical features of recurrent stroke patients were the existing complications of dyslipidemia and renal dysfunction.

Nurse-led outpatient clinics in oncology care – Patient satisfaction, information and continuity of care

PurposeThe aims of the present study were to investigate patients' satisfaction with nurse-led clinics, patients' perception of received information and associations between continuity of care and satisfaction with information. MethodsQuestionnaires on patient satisfaction were sent to consecutive samples of patients after they attended a nurse-led clinic at the Department of Oncology, Karolinska University Hospital in 2007, 2009, 2011 and 2013. Patients' perceptions of received information were evaluated in 2011 and 2013, by the EORTC QLQ-INFO25. Data on registered continuity of care were retrieved from the patients' medical record. ResultsA total of 962 patients responded (79%) to one of the four surveys. Patients' satisfaction with nurse-led clinics was stable over time. More than 90% rated nurses' interpersonal manners and the care at the clinic as “good”, the waiting time as “acceptable”, and the length of appointments as “sufficient”. Over 90% responded that it was important to meet the same nurse and 62% reported they actually did so and 52% stated they were assigned a named nurse navigator. More than 75% rated the information at their latest visit at a nurse-led clinic as “completely” sufficient. However, 48% expressed wish for more information “during the current disease”. No statistical significant associations were found between “satisfaction with information” and continuity of care. ConclusionsPatients' satisfaction with nurse-led clinics was stable over time with generally high figures with the exception for continuity of care and information, areas in which improvements are needed. The wide variety in information needs might require a person-centred approach.

Short Children with CHARGE Syndrome: Do They Benefit from Growth Hormone Therapy?

Aim: The aim of this study was to evaluate the response to recombinant growth hormone (GH) treatment in short children with CHARGE syndrome. Patients: We identified 51 children (28 boys and 23 girls) in KIGS (Pfizer International Growth Database). The median chronological age was 7.6 years at the start of GH therapy and 13.2 years at the latest visit. Evaluation for GH deficiency (n = 33) was based on the following: peak GH level 7.3 μg/l and IGF-I level -2.01 standard deviation score (SDS). Sixteen subjects (9 boys) were followed longitudinally for 2 years. Results: Birth length (median SDS, -0.47) and weight (-0.97) were slightly reduced. At the start of GH therapy, height was -3.6 SDS, BMI -0.7 SDS, and the GH dose was 0.26 mg/kg/week. At the latest visit after 2.7 years of GH therapy, height had increased to -2.2 SDS and BMI to -0.5 SDS. In the longitudinal group, height increased from -3.72 SDS at the start of GH therapy to -2.92 SDS after 1 year to -2.37 SDS after 2 years of therapy (start - 2 years: p < 0.05), height velocity increased from -1.69 to 2.98 to 0.95 SDS, and BMI and GH dose (mg/kg/week) remained almost unchanged. Conclusions: Our data show a positive effect of conventional doses of GH on short-term growth velocity for the longitudinal as well as for the total group, without any safety issues.

Endoresection technique with/without brachytherapy for management of high posterior choroidal melanoma: extended follow-up results.

To evaluate the long-term efficacy and safety of endoresection for high posterior choroidal melanoma. Retrospective nonrandomized interventional case series. Forty-one patients had endoresection as primary treatment for posterior choroidal melanoma. Of these, 21 patients had adjuvant brachytherapy. The inclusion criteria were tumor thickness ≥8 mm, base diameter <15 mm, and posterior tumors not extending anterior to the equator. Main outcomes measures were enucleation rate, visual outcome, surgical complications, local recurrence, metastasis, and mortality. Mean follow-up time was 102.5 months. Mean preoperative best-corrected visual acuity was 20/100. Mean tumor thickness was 9.8 mm (range, 7.7-13.5 mm; standard deviation, 1.7 mm), mean base diameter was 9.9 mm (range, 5-15 mm; standard deviation, 2.8 mm). At the latest visit, 36 patients (87.8%) still retained the eye. Mean postoperative best-corrected visual acuity was 20/1,625. Retinal detachment was the main postoperative complication (28.9%). At completion of follow-up, 12% of patients had phthisis bulbi, and 3 developed chronic hypotony. Five patients (12.2%) had local tumor recurrence; none of them had received brachytherapy as initial treatment. At 5 years of follow-up, 3 patients (7.3%) had liver metastasis. On Kaplan-Meier analysis at 10 years, all-cause mortality was 7.3% and specific mortality because of melanoma was 2.4%. Endoresection of high posterior melanomas was not associated with a higher risk of metastasis, death, or local recurrence than other reported techniques used to treat similar melanomas.

Widespread choroidal thickening and abnormal midperipheral fundus autofluorescence characterize exudative age-related macular degeneration with choroidal vascular hyperpermeability.

PurposeTo investigate the clinical findings that characterize exudative age-related macular degeneration (AMD) with choroidal vascular hyperpermeability (CVH).DesignRetrospective comparative study.ParticipantsForty-eight consecutive patients attending the outpatient clinic of Tokyo University Hospital between May 2013 and July 2013.MethodsThe presence or absence of CVH was determined with indocyanine green angiography performed at the latest visit. When CVH was observed, the eye was categorized as CVH(+) AMD, otherwise it was categorized as CVH(-) AMD. Using high-penetration optical coherence tomography, we measured choroidal thickness at the fovea and at four midperipheral areas (mean choroidal thickness at points on 6- and 9-papilla diameter circles superior, inferior, temporal, and nasal to the fovea). Ultrawide field retinal imaging was used to investigate abnormalities in midperipheral fundus autofluorescence (FAF). Choroidal thickness and the proportion of FAF abnormalities were compared between the CVH(+) AMD and CVH(−) AMD eyes and between eyes with polypoidal choroidal vasculopathy and typical AMD. Multiple regression analysis was used to control for treatment history and other characteristics.ResultsCVH was observed in 17 cases. Choroidal thickness was higher in the CVH(+) AMD eyes than in the CVH(−) AMD eyes at the fovea (325 μm versus 229 μm, respectively; P=0.0010, t-test), superior point (277 μm versus 215 μm, respectively; P=0.0021, t-test), inferior point (225 μm versus 161 μm, respectively; P=0.0002, t-test), and nasal point (202 μm versus 165 μm, respectively; P=0.042, t-test). The significance was maintained after controlling for possible confounders. The choroid was thicker at the fovea and at the inferior point in polypoidal choroidal vasculopathy than in typical AMD. The rate of midperipheral FAF abnormality was significantly higher in the CVH(+) AMD eyes than in the CVH(−) AMD eyes (82% versus 48%, respectively; P=0.031).ConclusionAMD with CVH is associated with widespread choroidal thickening and peripheral FAF abnormalities.

Efficacy of estrogen replacement therapy (ERT) on uterine growth and acquisition of bone mass in patients with Turner syndrome.

Estrogen replacement therapy (ERT) is necessary for uterine development and bone mass acquisition in women with Turner syndrome (TS) suffering from ovarian insufficiency. However, adequate ERT regimens have not yet been established. The aim of this study was to evaluate the efficacy of ERT for both uterine development and bone mass acquisition. One hundred TS patients from Yokohama City University Hospital (88 with primary amenorrhea (PA) and 12 patients with spontaneous menstrual cycles (MC)) were enrolled after obtaining consent. Clinical profiles, uterine length (UL) measured by ultrasonic examination, and bone mineral density (BMD) of the lumbar vertebrae (L2-4) assessed by DEXA were evaluated. At the time of the first visit, the ULs of patients in the PA group were significantly shorter than those in the MC group. After receiving ERT, there were no significant differences in UL between patients with PA and MC. Forty-seven patients for whom the ERT initiation age was known were investigated to clarify the influence on BMD. The results showed that the BMD in the late initiation (18 years or older) group at the latest visit (0.770 ± 0.107 g/cm2: n = 16) was significantly lower than that in the early initiation (under 18 years) group (0.858 ± 0.119 g/cm2: n = 21) or the MC group (0.941 ± 0.118 g/cm2: n = 10). No significant differences were seen between the early initiation and MC group. ERT was effective in increasing UL and BMD. However, early initiation of ERT is necessary to increase BMD.