Πέμπτη 10 Δεκεμβρίου 2020

Oxytocin in young children with Prader‐Willi syndrome

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Abstract

Context

Prader‐Willi syndrome (PWS) is characterized by hypothalamic dysfunction, hyperphagia and a typical behavioral phenotype, with characteristics of autism spectrum disorder (ASD) like stubbornness, temper tantrums and compulsivity. It has been suggested that the oxytocin system in patients with PWS is dysfunctional. In ASD, intranasal oxytocin treatment has favorable effects on behavior.

Objective

To evaluate the effects of 3 months of twice daily intranasal oxytocin (dose range 16‐40 IU/day), compared to placebo, on behavior and hyperphagia in children with PWS.

Design

Randomized, double‐blind, placebo‐controlled, crossover study in the Dutch PWS Reference Center.

Patients

26 children with PWS aged 3‐11 years

Main outcome measures

(Change in) behavior and hyperphagia measured by Oxytocin Questionnaire and Dykens hyperphagia questionnaire.

Results

In the total group, no significant effects of oxytocin on social behavior or hyperphagia were found. However, in boys, the Oxytocin Questionnaire scores improved significantly during oxytocin treatment, compared to a deterioration during placebo (4.5 (‐0.8‐15.3) vs. ‐4.0 (‐11.3‐0.8)), p=0.025). The Dykens hyperphagia questionnaire scores remained similar during oxytocin treatment, while there was a deterioration during placebo (0.0 (‐0.8‐4.3) vs. ‐3.5 (‐6.0‐0.0), p=0.046). Patients with a deletion had significant improvements in both questionnaire scores during oxytocin treatment, but deteriorations during placebo. Oxytocin treatment was well‐tolerated and there were no serious adverse events.

Conclusions

Intranasal oxytocin treatment has positive effects on social and eating behaviour in 3‐11 years aged boys with PWS and in children with a deletion without safety concerns. Intranasal oxytocin in children with PWS might be considered, but individual effects should be carefully evaluated and treatment discontinued if no effects are found.

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Association of urinary free cortisol with bone formation in patients with mild autonomous cortisol secretion

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Abstract

Context

Mild autonomous cortisol secretion (ACS) is associated with an increased risk of vertebral fractures (VFx). However, the influence of this condition on bone turnover or its association with mild ACS is still controversial.

Objective

This study aimed to evaluate the impact of mild ACS on bone quality among patients living with the disease.

Design and setting

A retrospective study was conducted using data from 55 mild ACS and 12 nonfunctioning adrenal tumor (NFT) patients who visited Chiba University Hospital, Japan, from 2006 to 2018.

Patients and main outcome measures

We analyzed clinical features and bone‐related factors, including bone mineral density (BMD) and VFx, performed blood tests to assess bone metabolism markers in patients with mild ACS and NFT, and assessed the associations between bone‐related markers and endocrinological parameters in patients with mild ACS.

Results

No significant differences between mild ACS and NFT patients were observed with respect to the presence or absence of VFx and BMD. Urinary free cortisol (UFC) was higher in mild ACS patients with VFx than those without (P = 0.037). The T‐score and young adult mean (YAM) of the BMD of the femoral neck in mild ACS patients with a body mass index < 25 were positively correlated with dehydroepiandrosterone‐sulfate levels (ρ: 0.42, P = 0.017; ρ: 0.40, P = 0.024, respectively). Pearson's correlation analysis showed that bone‐specific alkaline phosphatase was negatively correlated with UFC in the patients with mild ACS (ρ: −0.37, P = 0.026).

Conclusions

These results suggest that urinary free cortisol may be useful for predicting bone formation in mild ACS patients.

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SRY‐negative 46,XX testicular/ovotesticular DSD: long‐term outcome and early blockade of gonadotropic axis

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ABSTRACT

Objective

SRY‐negative 46,XX testicular and ovotesticular disorders/differences of sex development (T/OTDSD) represent a very rare and unique DSD condition where testicular tissue develops in the absence of a Y chromosome. To date, very few studies have described the phenotype, clinical and surgical management and long‐term outcomes of these patients. Particularly, early blockade of the gonadotropic axis in patients raised in the female gender to minimize postnatal androgenization has never been reported.

Design

Retrospective description of sixteen 46,XX T/OTDSD patients.

Results

Sixteen 46,XX SRY‐negative T/OTDSD were included. Most (12/16) were diagnosed in the neonatal period. Sex of rearing was male for six patients and female for ten, while the clinical presentation varied, with an external masculinization score from 1 to 10. Five patients raised as girl were successfully treated with GnRH analog to avoid virilization during minipuberty. Ovotestes/testes were found bilaterally for 54% of the patients and unilaterally for the others (with a contralateral ovary). Gonadal surgery preserved appropriate tissue in the majority of cases. Spontaneous puberty occurred in two girls and one boy, while two boys required hormonal induction of puberty. One of the girls conceived spontaneously and had an uneventful pregnancy. DNA analyses (SNP‐array, Next‐Generation Sequencing and Whole‐Exome Sequencing) were performed. A heterozygous frameshit mutation in the NR2F2 gene was identified in one patient.

Conclusions

This study presents a population of patients with 46,XX SRY‐negative T/OTDSD. Early blockade of gonadotropic axis appears efficient to reduce and avoid further androgenization in patients raised as girls.

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Classification and morphology of middle mesial canals of mandibular first molars in a southern Chinese subpopulation: a cone-beam computed tomographic study

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Abstract

Background

Cone-beam computed tomography (CBCT) was employed to study the morphology and curvature of middle mesial canals (MMCs) of mandibular first molars (MFMs).

Methods

CBCT scanning was performed on MFMs of 1100 patients. Patients' images that met the inclusion criteria were divided into group A (< 40 years old) and group B (≥ 40 years old) for further study. The images were used to study the incidence of MMCs at different ages, measure the curvature of MMCs in the mesiodistal and buccolingual directions using the Schneider method, and observe the anatomical morphology of the mesial root canal system. All statistical analyses were performed by using SPSS 21.0 software. Quantitative data were presented as mean ± standard deviation. Student's t-test was used to calculate the statistical significance. P < 0.05 was considered statistically significant.

Results

In 875 patients, 1750 MFM images met the inclusion criteria. Among these cases, 158 MFMs contained an MMC, yielding an incidence rate of 9.03%. The incidence rate of MMCs was 11.22% in group A and 6.61% in group B, and this difference was statistically significant (P < 0.05). The curvature in the mesiodistal direction was 29.39 ± 8.53° in group A and 26.06 ± 8.50° in group B, and this difference was also significant (P < 0.05). The curved regions in groups A and B were often located in the middle 1/3 of canal. No significant difference in the distance between MMC orifices and mesiobuccal canal orifices or mesiolingual canal orifices was noted (P > 0.05). The most common mesial root canal morphological type was type II (3-2) (53.80%).

Conclusion

The incidence of MMCs in MFMs declined as age increased. The canal systems of MMCs were varied and complex, mainly exhibiting an obvious mesiodistal curve. CBCT is an outstanding method to help guide root canal therapy.

*Yeqing Yang and Buling Wu have contribute equally to this article.

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Peroneal Flap – How to Harvest and Clinical Appraisal for Head and Neck Reconstruction

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A peroneal flap, the boneless version of fibula flap, is considered as the equivalent of radial forearm flap of the lower leg. Because it is thinner than an anterolateral thigh (ALT) flap, the use of a peroneal flap is a viable option for the repair of soft tissue defects when a thin flap could bring about better functional and cosmetic outcomes. In this article, we describe the details of peroneal flap harvest and present our experience with the use of peroneal flaps for head and neck reconstruction.
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Inferior Alveolar Nerve Reconstruction in Extensive Mandibular Resection: Technical Notes

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The inferior alveolar nerve (IAN) is a sensitive branch of the mandibular nerve innervating the lower lip, the chin, the buccal mucosa and the teeths. Lesions of the IAN are reported to occur in the 64,4% of maxillo-facial procedures, leading to anesthesia, hypoestesia and/or neurogenic discomfort. An extensive segment of the nerve can be moreover removed during mandibular resection for benign or malignant pathologies. Nervous grafts can be used in these cases to restore the nerve continuity. In order to optimize the procedure and to allow a concomitant mandibular osseous reconstruction, the Authors identified several standardized steps.
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Technical Principles and Clinical Workflow of Transcranial MR-Guided Focused Ultrasound

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Transcranial MR-guided focused ultrasound (MRgFUS) is a rapidly developing technology in neuroscience for manipulating brain structure and function without open surgery. The effectiveness of transcranial MRgFUS for thermoablation is well established, and the technique is actively employed worldwide for movement disorders including essential tremor. A growing number of centers are also investigating the potential of microbubble-mediated focused ultrasound-induced opening of the blood-brain barrier (BBB) for targeted drug deli very to the brain. Here, we provide a technical overview of the principles, clinical workflow, and operator considerations of transcranial MRgFUS procedures for both thermoablation and BBB opening.
Stereotact Funct Neurosurg
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The human melanocyte and melanoblast populations per unit area of epidermis in the rete ridge are greater than in the inter‐rete ridge

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Abstract

Objective

In human skin, melanocytes and melanoblasts are mostly located in the epidermis in addition to hair follicles. In hairy skin such as mouse skin, the inter‐follicular epidermis is generally flat. In contrast, in human skin, the epidermis is wavy and possesses well‐developed rete ridges. It is not well understood what difference exists in cell function between melanocytes present in the inter‐rete ridge and those in the rete ridge. To clarify this problem we first tried to determine the melanocyte and melanoblast populations per unit area of epidermis both in the rete ridge and inter‐rete ridge epidermis.

Methods

Thirty‐eight samples from normal skin sites of 28 patients (14 males and 14 females) aged from 5 to 76 years were fixed and processed to dopa and combined dopa‐premelanin reactions. The numbers of cells positive to the dopa (melanocytes) and combined dopa‐premelanin (melanoblasts and melanocytes) reactions were scored.

Results

The average melanocyte population/0.1 mm2 of the inter‐rete ridge was 74 cells, whereas that of the rete ridge was 147 cells. Moreover, the average melanoblast population/0.1 mm2 in the inter‐rete ridge was 43 cells, whereas that of the rete ridge was 131 cells. The melanoblast population in the rete ridge differed between female and male, but the melanocyte populations in the rete ridge and inter‐rete ridge as well as the melanoblast population in the inter‐rete ridge did not differ between female and male. However, no age difference in the melanocyte and melanoblast populations was observed between the rete ridge and inter‐rete ridge.

Conclusion

Human epidermal melanocytes and melanoblasts in the rete‐ridge exist more numerously than in the inter‐rete ridge.

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Efficacy and Safety of Mesenchymal Stem Cell Clusters in Patients With Critical Limb Ischemia

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Condition:   Critical Limb Ischemia
Interventions:   Biological: A cluster of adipose-derived mesenchymal stem cells (Dose: 1 x 10^7 cells/1 mL/vial);   Biological: A cluster of adipose-derived mesenchymal stem cells (Dose: 1 x 10^8 cells/1 mL/vial)
Sponsors:   S.Biomedics Co., Ltd.;   Dt&Sanomedics
Recruiting
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Efficacy and Safety of Pembrolizumab (MK-3475) Plus Lenvatinib (E7080/MK-7902) Plus Chemotherapy in Participants With Advanced/Metastatic Human Epidermal Growth Factor Receptor 2 (HER2) Negative Gastric/Gastroesophageal Junction (GEJ) Adenocarcinoma (MK-7902-015/E7080-G000-321/LEAP-015)

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Condition:   Advanced/Metastatic HER2 Negative Gastric/Gastroesophageal Junction Adenocarcinoma
Interventions:   Biological: Pembrolizumab;   Biological: Lenvatinib;   Drug: Oxaliplatin;   Drug: Capecitabine;   Drug: Leucovorin;   Drug: 5-FU
Sponsors:   Merck Sharp & Dohme Corp.;   Eisai Inc.
Not yet recruiting
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Pilot Study of a 1-Millimeter Resolution Clinical PET System in Head and Neck Cancer Imaging

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Condition:   Head and Neck Cancer
Intervention:   Device: 1-mm Resolution Clinical PET camera
Sponsors:   Stanford University;   Goldman Sachs Foundation;   Wallace H. Coulter Foundation;   Emerson Collective
Not yet recruiting
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Systemic Inflammation Response Index Is a Predictor of Poor Survival in Locally Advanced Nasopharyngeal Carcinoma: A Propensity Score Matching Study

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Introduction

Nasopharyngeal carcinoma (NPC) is a common malignancy in China and known prognostic factors are limited. In this study, neutrophil-to-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR), systemic immune inflammation index (SII), and systemic inflammation response index (SIRI) were evaluated as prognostic factors in locally advanced NPC patients.

Materials and Methods

NPC patients who received curative radiation or chemoradiation between January 2012 and December 2015 at the Second Xia ngya Hospital were retrospectively reviewed, and a total of 516 patients were shortlisted. After propensity score matching (PSM), 417 patients were eventually enrolled. Laboratory and clinical data were collected from the patients' records. Receiver operating characteristic curve analysis was used to determine the optimal cut-off value. Survival curves were analyzed using the Kaplan-Meier method. The Cox proportional hazard model was used to identify prognostic variables.

Results

After PSM, all basic characteristics between patients in the high SIRI group and low SIRI group were balanced except for sex (p=0.001) and clinical stage (p=0.036). Univariate analysis showed that NLR (p=0.001), PLR (p=0.008), SII (p=0.001), and SIRI (p<0.001) were prognostic factors for progression-free survival (PFS) and overall survival (OS). However, further multivariate Cox regression analysis showed that only SIRI was an independent predictor of PFS and OS (hazard ratio (HR):2.83; 95% confid ence interval (CI): 1.561-5.131; p=0.001, HR: 5.19; 95% CI: 2.588-10.406; p<0.001), respectively.

Conclusion

Our findings indicate that SIRI might be a promising predictive indicator of locally advanced NPC patients.

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