Autonomic Stimulation Action of EAT (Epipharyngeal Abrasive Therapy) on Chronic Epipharyngitis.

This study investigated the pathogenesis and pathophysiology of chronic epipharyngitis, which presents a variety of symptoms, with a focus on autonomic neuropathy symptoms, and also investigated the literature for information on EAT, which is useful as a treatment method. The mechanism of action of EAT has recently been clarified in terms of its immune system-stimulating and endocrine system-stimulating effects. However, the autonomic nerve-stimulating effects of EAT are still largely unexplained. This study was conducted to collect and integrate previous studies and papers focusing on the autonomic nerve-stimulating effects of EAT and to provide insight into the still not fully elucidated autonomic nerve-stimulating effects of EAT on chronic epipharyngitis. The local stimulating effects of zinc chloride and the bleeding and pain effects of EAT are also summarized, suggesting that EAT exerts its therapeutic effects through the interaction of the immune system, the endocrine system, and the autonomic nervous system. It is important to determine which mechanism is predominantly involved in each case of chronic epipharyngitis and to utilize it in treatment. Elucidating the effects of EAT on the autonomic nervous system will be an important guideline in determining the treatment strategy for chronic epipharyngitis.

Simple Instrument Modification to Aid in Laparoscopic Gastric Wraps for Posterior Fundoplications.

Described is a simple modification of the O'Reilly esophageal retractor to aid in performing laparoscopic posterior gastric wraps during antireflux procedures. A 3-mm hole was drilled into the distal end of the reticulating arm. Once the arm is positioned posteriorly to the gastroesophageal (GE) junction, the freed gastric fundus can be secured to the retractor with a suture. The fundus can then be pulled posteriorly to the GE junction and held into position for placement of the fundoplication sutures.

Autonomic Nervous System Regulation Effects of Epipharyngeal Abrasive Therapy for Myalgic Encephalomyelitis/Chronic Fatigue Syndrome Associated With Chronic Epipharyngitis.

OBJECTIVE: To evaluate the autonomic nerve stimulation effect of epipharyngeal abrasive therapy (EAT) on myalgic encephalomyelitis/chronic fatigue syndrome (CFS) associated with chronic epipharyngitis. Heart rate variability analysis was performed. The study was conducted by analyzing heart rate variability. SUBJECTS AND METHODS: A total of 29 patients with chronic epipharyngitis who underwent EAT from July 2017 to April 2018 were classified into two groups: 11 patients in the CFS group and 18 patients in the control group without CFS. The patients were classified as phase 1 during bed rest, phase 2 during nasal endoscopy, phase 3 during nasal abrasion, and phase 4 during oral abrasion. Electrocardiographic recordings were made, and autonomic function was compared and evaluated by measuring heart rate, coefficient of variation on R-R interval (CVRR), coefficient of component variance high frequency (ccvHF), and low frequency/ccvHF ratio (L/H) for each of the four phases. The Shapiro-Wilk test was performed to confirm the normality of the two groups, and the parametric test was selected. A repeated measures analysis of variance was performed to assess changes over time between the four events in the two groups. Multiple comparisons were corrected by the Bonferroni method. Comparisons between resting data and three events within each group were performed by paired t-test. RESULTS: The CFS group had an increased baseline heart rate compared to the control group, and the CFS group had a greater increase in parasympathetic activity and a decrease in heart rate with nasal abrasion. Oral abrasion elicited a pharyngeal reflex and increased heart rate and both sympathetic and parasympathetic activity. CONCLUSION: The CFS group was in a state of dysautonomia due to autonomic overstimulation, with an elevated baseline heart rate. The CFS group was considered to be in a state of impaired autonomic homeostasis, with an increased likelihood that overstimulation would induce a pathological vagal reflex and the Reilly phenomenon would develop. The direct effects of EAT on the autonomic nervous system were considered to be vagus nerve stimulation and the regulation of autonomic function by opposing stimulation input to sympathetic and parasympathetic nerves. As an indirect effect, bleeding from the epipharyngeal mucosa due to abrasion was thought to restore the function of the cerebral venous and lymphatic excretory systems and the autonomic nerve center.

Hematological Findings in Lysosomal Storage Disorders: A Perspective from the Medical Laboratory.

Lysosomal storage disorders (LSDs) are a group of rare and genetic diseases produced by mutations in genes coding for proteins involved in lysosome functioning. Protein defect leads to the lysosomal accumulation of undegraded macromolecules including glycoproteins, glycosaminoglycans, lipids, and glycogen. Depending on the stored substrate, several pathogenic cascades may be activated leading to multisystemic and progressive disorders affecting the brain, eye, ear, lungs, heart, liver, spleen, kidney, skin, or bone. In addition, for some of these disorders, hematological findings have been also reported. In this paper, we review the major hematological alterations in LSDs based on 56 case reports published between 2010 and 2020. Hematological alterations were reported in sphingolipidosis, mucopolysaccharidoses, mucolipidoses, neuronal ceroid lipofuscinosis, glycogenosis, glycoproteinosis, cystinosis, and cholesteryl ester storage disease. They were reported alterations in red cell linage and leukocytes, such as anemia and morphology changes in eosinophils, neutrophils, monocytes, and lymphocytes. In addition, changes in platelet counts (thrombocytopenia) and leukocyte abnormalities on non-peripheral blood samples were also reported for some LSDs. Although in most of the cases these hematological alterations are not pathognomonic of a specific disease or group of LSDs, since they can be easily identified in general clinical laboratories, their identification may contribute to the diagnosis of these disorders. In this sense, we hope that this review contributes to the awareness of the importance of hematological alterations in the diagnosis of LSDs.

Can Tissue Expansion Reconstruction in the Trunk of Children Increase the Risk of Scoliosis?

INTRODUCTION: We hypothesize that treatment of significant truncal lesions with truncal tissue expanders and subsequent flap surgery in pediatric patients may increase the risk of scoliosis. This study aims to investigate any relationship between tissue expansion (TE) and scoliosis and to compare the prevalence of scoliosis in our tissue expander population to the general population. METHODS: Health records of patients who underwent truncal TE at BC Children's Hospital between 1997 and 2017 were retrospectively reviewed and analyzed. The cross-sectional component of the study consisted of radiological imaging to establish the presence or absence of scoliosis. RESULTS: We identified 28 patients who underwent truncal TE over the study period. Ten had a scoliosis X-ray on their chart or as a part of the study. Three (10.7%) patients were identified as having developed scoliosis after TE. CONCLUSIONS: We recommend that pediatric TE patients be made aware of the potential complication of scoliosis and be followed closely in the years during and after their treatment, in order to allow for preventative measures, early diagnosis and early management (if required).

INTRODUCTION: Les chercheurs postulent que le traitement d'importantes lésions du tronc par des expandeurs tissulaires suivi d'une opération par lambeau chez les patients pédiatriques peut accroître le risque de scoliose. La présente étude vise à explorer la relation entre l'expansion tissulaire (ET) et la scoliose et à comparer la prévalence de scoliose entre la population ayant subi une expansion tissulaire et la population générale. MÉTHODOLOGIE: Les chercheurs ont procédé à l'analyse prospective du dossier de santé des patients qui ont subi une ET du tronc au BC Children's Hospital entre 1997 et 2017. L'élément transversal de l'étude était constitué d'une imagerie radiologique pour établir la présence ou l'absence de scoliose. RÉSULTATS: Les chercheurs ont recensé 28 patients qui ont subi une ET du tronc pendant la période de l'étude. Dix avaient une radiographie de scoliose au dossier ou en cours d'étude. Trois (10,7 %) avaient eu une scoliose après l'ET. CONCLUSIONS: Les chercheurs recommandent d'informer les patients pédiatriques qui subissent une ET du tronc qu'ils courent un risque de complication de scoliose et de les suivre de près dans les années où se déroulent le traitement et qui le suivent, afin de favoriser des mesures préventives, des diagnostics précoces et une prise en charge rapide, s'il y a lieu.

Mucopolysaccharidosis: A case report highlighting hematological aspects of the disease.

A 1½-year-old female child presented with swelling in thoracolumbar region and delayed developmental milestones. The routine hemogram analysis on Sysmex XN 1000 showed flags of white blood cell (WBC) abnormal scattergram and lymphocytosis. The peripheral smear examination showed Alder-Reilly (AR) granules leading to a suspicion of underlying Mucopolysaccharidosis (MPS). Further clinical workup, radiographic studies, chemical test lead to the confirmatory diagnosis of MPS. A flag of abnormal WBC scattergram and AR anomaly are the hematological findings that can be seen in a case of MPS.

Delimitating the Natural City with Points of Interests Based on Service Area and Maximum Entropy Method.

The natural city, which is essential to understand urban physical scale and identify urban sprawling in urban studies, represents the urban functional boundaries of the city defined by human activities rather than the administrative boundaries. Most studies tend to utilize physical environment data such as street networks and remote sensing data to delimitate the natural city, however, such data may not match the real distribution of human activity density in the new cities or even ghost cities in China. This paper suggests aggregating the natural city boundary from the service area polygons of points of interest based on Reilly's Law of Retail Gravitation and the maximum entropy method, since most points of interests provide services for surrounding communities, reflecting the vitality in a bottom-up way. The results indicate that the natural city defined by points of interests shows a high resolution and accuracy, providing a method to define the natural city with POIs.

Alder-Reilly Anomaly in Hurler's Syndrome in a Neonate: A Rare Case Report.

A 22-days-old male newborn baby presented with persistence of neonatal jaundice since birth. On clinical examination he had coarse facial features, a prominent forehead, enlarged tongue, icterus, hepatosplenomegaly, skeletal deformities and bilateral inguinal hernia. On investigation the peripheral smear revealed Alder-Reilly anomaly in the neutrophils suggesting mucopolysaccharidosis. Mucopolysaccharide excretion spot test of the urine was positive; and an assay for glycosaminoglycans in the urine was also high, which confirmed the clinical diagnosis of Hurler's syndrome. We present this rare case to highlight the association of Alder-Reilly anomaly and bilateral inguinal hernia in Hurler's syndrome even in neonates.