Parasympathetic Ganglia Research Articles

Short-Term Effect of Stimulating the Pterygopalatine Ganglion Via Electroacupuncture on Choroidal Structure in Human Subjects.

Choroidal dysfunction is implicated in various ocular pathologies. The parasympathetic pterygopalatine ganglion (PPG) innervates orbital vessels supplying the choroid. While PPG stimulation has been shown to dilate cerebral blood flow, its effects on the choroid, particularly in human subjects, require further elucidation. This study aimed to investigate the short-term influence of PPG stimulation via electroacupuncture on choroidal structure. In this crossover study, 22 healthy adults received PPG electrical stimulation and sham stimulation for one session each on two separate days in a randomized order. Measurements including choroidal thickness (ChT), choroidal vascularity index (ChVI), central subfield thickness, axial length, anterior chamber depth, and lens thickness were recorded before and at intervals (0, 15, 30, 45, and 60minutes) postintervention. The ChT on the side receiving PPG stimulation demonstrated a sustained increase, peaking at 15minutes poststimulation (17.2µm, P < 0.001) and persisting for up to 60minutes. Conversely, the ChVI exhibited a more immediate response, with a peak increase immediately poststimulation (3.8%, P = 0.003), followed by a gradual return to baseline. ChT and ChVI in the contralateral eye showed a nonsignificant trend to decrease. Additionally, a notable reduction in ipsilateral axial length was observed at specific time points poststimulation. PPG activation via electroacupuncture elicited a short-term increase in ChT and ChVI in the ipsilateral eye compared to sham stimulation, with ChT increases trailing those of ChVI but displaying greater persistence. Electrical stimulation of the PPG can produce a short-term increase in ipsilateral ChT and ChVI.

6997 Atypical Presentation of Hereditary Paraganglioma

Abstract Disclosure: O. Abdulhussein: None. R.R. Correa: None. M.D. Lundholm: None. J. Ferri-Guerra: None. G. Madrigol: None. Introduction: Paraganglioma (PGL) is a rare neuroendocrine, highly vascular tumor arising from sympathetic or parasympathetic ganglia chromaffin cells. Some PGL functional while others biochemically silent. Given the possible lack of classic symptoms or convincing catecholamine elevation. They are closely related to pheochromocytomas.• However, the distinction between pheochromocytoma and paraganglioma is essential because of the implication for associated neoplasm and syndromes; paraganglioma is more metastatic. Case: 32-year-old otherwise healthy female presented to primary care clinic for 1 year of dry cough. She was a non-smoker. She denied voice changes, palpitations, hypertension, palpitations, pallor, headache, or diaphoresis, but had noticed a mass growing in her right neck for a year. On exam her blood pressure 107/73 and there was a mobile, round mass felt at the right mandible. CT neck at the time showed reactive lymphadenopathy. She referred to pulmonology and ENT, spirometry and flexible laryngoscopy were unremarkable. For persistent mass 1year later, an MRI of the neck showed a 2.8x2.3x3.8 cm right carotid mass, likely PGL. Biochemical testing revealed elevations in plasma normetanephrine (NM) (269 pg/mL, 18-101 pg/mL) and 24-hour urinary NM (848 ug/24hr, 88-444 ug/24hr) with mild elevation in 24-hour urinary metanephrine (982 ug/24 hr, 140-785 ug/24 hr) and normal plasma metanephrine. The mass was intensely avid on DOTATATE scan without other lesions. Genetic testing uncovered SDHB mutation. She was started on preoperative alpha-blockade with successful resection. She had pathology confirmation of PGL and was set up for annual SDHB surveillance testing. Discussion: A minority of patients with PCC or PGL present with classical “5 P” catecholamine symptoms: pallor, pain, pressure, perspiration, or palpitations. One main reason is a lack of excess catecholamine secretion; 17 years of retrospective data at our center showed that 61% (N=50/82) of patients with PGL with preoperative catecholamine labs had normal levels. Additionally, mild to moderate increases to 2-3x upper limit of normal may be attributed to stress or medications, which obfuscates the distinction between secretory disease and physiologic elevation.Given the infrequency of catecholamine symptoms or secretion, PGL is frequently discovered incidentally. Indeed, 92% (N=110/119) of our PGL cases were found incidentally on imaging or pathology. Alpha-blockade is the preoperative standard of care, many patients are only diagnosed post-operatively by pathology findings. Further study is needed to determine if pre-operative alpha-blockade is needed in all PGL cases, and if so, how we can better identify these patients before they go to the operating room. For now, a high level of suspicion is needed to make a timely PGL diagnosis and facilitate genetic testing. Presentation: 6/1/2024

P001 AN UNCOMMON CAUSE OF HIGH BLOOD PRESSURE IN YOUNG BASKETBALL PLAYER

Paragangliomas are rare neuroendocrine tumors that affect approximately 1.5–9 patients per million. They originate from chromaffin cells of the adrenal medulla or sympathetic and parasympathetic ganglia. Paragangliomas account for 10%–20% of retroperitoneal tumors and can manifest at any age. These tumors can secrete catecholamines, leading to symptoms such as hypertension, headaches, and diaphoresis.However, palpitations or tachycardia may also be present. Definitive treatment entails surgical resection, with acceptable rates of morbidity and mortality, necessitating careful patient preparation and intraoperative management. Case Presentation: We present the case of a 29-year-old man with a BMI 26,5 kg/m2, who is physically active and without other cardiovascular risk factors, episodic hypertension, headaches and blush. A contrast CT scan of the abdomen revealed a retroperitoneal intercavoaortic mass and 24-hour urine Normetanephrines were significantly elevated, indicating a functional paraganglioma. Laboratory tests revealed an elevated plasma normetanephrine level of 1838 pg/ml (reference values 0 - 180 pg/ml). The patient was prepared for surgery with alpha blockers, followed by beta blockers. Intraoperative blood pressure elevation was managed with intravenous beta blockers. Postoperatively, the patient did not require anti-hypertensives. The tumor, measuring 6x4x3.5 cm, histopathology and immunohistochemistry confirmed the diagnosis of a normetanephrine producing paraganglioma. The genetic study revealed a pathogenic variant in the SDHB gene, specifically the c.166_170delCCTCA (p.Pro56Tyrfs*5) in heterozygosity. This variant is associated with a 49% risk of Pheochromocytoma and Hereditary Paraganglioma, a 14% risk of Renal Carcinoma and susceptibility to Gastrointestinal Stromal Tumors. The c.166_170delCCTCA variant follows an autosomal dominant inheritance pattern, indicating a 50% risk of recurrence. Conclusions: The diagnosis of extra-adrenal retroperitoneal paragangliomas remains challenging due to the silent evolution of the condition. It is essential to consider screening patients who are young, and present with resistant arterial hypertension for secondary hypertension.

Review and prospect of the diagnosis and treatment of head and neck paragangliomas

ead and neck paraganglioma（HNPGL） often originates from the parasympathetic ganglia and is a highly invasive benign tumor. The diagnosis and treatment of this disease with strong heterogeneity is still a challenge. In the future, deep exploration is needed in genetic typing, grading diagnosis and treatment decisions, protection of cranial nerves and new drug treatments to better treat this disease.

Single-cell RNA-Seq reveals the heterogeneity of fibroblasts within the tympanojugular paraganglioma microenvironment

Tympanojugular paragangliomas (TJP) originate from the parasympathetic ganglia in the lateral base of the skull. Although the cellular composition and oncogenic mechanisms of paragangliomas have been evaluated, a comprehensive transcriptomic atlas specific to TJP remains to be established to facilitate further investigations. In this study, single-cell RNA sequencing and whole-exome sequencing were conducted on six surgically excised TJP samples to determine their cellular composition and intratumoral heterogeneity. Fibroblasts were sub-classified into two distinct groups: myofibroblasts and fibroblasts associated with bone remodeling. Additionally, an elaborate regulatory and cell–cell communication network was determined, highlighting the multifaceted role of fibroblasts, which varies depending on expression transitions. The Kit receptor (KIT) signaling pathway mediated interactions between fibroblasts and mast cells, whereas robust connections with endothelial and Schwann cell-like cells were facilitated through the platelet-derived growth factor signaling pathway. These findings establish a foundation for studying the mechanisms underlying protumor angiogenesis and the specific contributions of fibroblasts within the TJP microenvironment. IL6 signaling pathway of fibroblasts interacting with macrophages and endothelial cells may be involved in tumor regrowth. These results enhance our understanding of fibroblast functionality and provide a resource for future therapeutic targeting of TJP.

Therapy with botulinum neurotoxin for Parkinson's disease.

Botulinum neurotoxin (BoNT) has been in use since the 1970's. Its effect is reached mainly by inhibiting the release of acetylcholine in the synaptic gap of motor neurons or at the motor end plate and the parasympathetic ganglia. In the case of Parkinson's disease, it is used to treat several motor and non-motor symptoms. Within recent years increasingly numerous possible fields of application of BoNT have been found for the treatment of Parkinson's disease, and for some specific symptoms it has in fact become the therapy of choice, while for others it is but one of the therapeutic options that come into consideration when others are not sufficiently effective. In the following, we intend to outline the indications, the possible side effects and also the approvals for therapies with botulinum toxin in the primary and secondary symptoms of Parkinson's disease.

КАРДИОНЕЙРОАБЛАЦИЯ – ФИЗИОЛОГИЧЕСКИЕ ОСНОВЫ МЕТОДА, КЛИНИЧЕСКОЕ ПРИМЕНЕНИЕ ПРИ НАРУШЕНИЯХ РИТМА СЕРДЦА

Increased tone of the parasympathetic part of the autonomic nervous system, affecting the pacemaker cells, may cause sinus bradycardia, develop long pauses of heart rhythm, paroxysmal or permanent atrioventricular block followed by reflex syncope. The usual reflex syncope treatment is conservative and includes avoiding situations that trigger syncope (prolonged standing, sight of blood etc.), tilt-training, adequate drinking regime, wearing compression underwear, sleeping with the head of the bed elevated and in some cases stimulating neuro- and vegetotropic therapy. Some patients are refractory to conventional methods of treatment and retain severe symptoms that worsens quality of their life seriously. These patients may be required a pacemaker implantation subsequently. Cardioneuroablation is an alternative treatment in patients with overactive parasympathetic nervous system that is based on radiofrequency catheter ablation of the major parasympathetic autonomic ganglia around the heart.

18F-FDG PET/CT and 68Ga-DOTATATE PET/CT Findings of Polycystic Kidney-derived Paraganglioma.

Paragangliomas (PGLs) are neuroendocrine tumors originating from the neural crest. They usually arise from the adrenal medulla and sympathetic or parasympathetic ganglions. Approximately 10% of PGLs are located in the extra-adrenal gland. Renal PGL is a rare condition. In this case report, we present the 18F-fluorodeoxyglucose positron emission tomography/computed tomography (PET/CT) and 68Ga-DOTATATE PET/CT findings of polycystic kidney-derived PGL.

Cardioneuroablation: the known and the unknown.

Cardioneuroablation (CNA) is a novel interventional procedure for the treatment of recurrent vasovagal syncope (VVS) and advanced atrioventricular block secondary to hyperactivation of vagal tone in young patients. By damaging the cardiac parasympathetic ganglia, CNA seems to be able to mitigate and/or abolish the excessive vagal activity and improve patients' outcome. This review is intended to give a detailed and comprehensive overview of the current evidences regarding (1) the clinical applications of CNA (2) the identification of ablation targets and procedural endpoints (3) the medium-long term effect of the procedure and its future perspectives. However, clinical data are still limited, and expert consensus or recommendations in the guidelines regarding this technique are still lacking.

THU523 A Rare Case Of Functional Cervical Paraganglioma: Case Report

Abstract Disclosure: V.D. Oliveira: None. G.C. Vaccarezza: None. A.E. Vieira: None. N.M. Scalissi: None. A.R. dos Santos: None. P.R. Lazarini: None. A.B. Suehara: None. C. Olivati: None. J.V. Lima: None. INTRODUCTION: Head and neck paragangliomas (HNPGL) are rare tumors arising from cells associated with parasympathetic ganglia. Catecholamine hypersecretion is founded in less than 5% of the patients. Evidence is growing that about 50% of the HNPGL are associated with hereditary syndromes. CASE REPORT: Male patient, 34 years old, previously healthy, presented with a 1-year history of jaw pain and hypoacusis, associated with progressive symptoms of dysphagia, facial paralysis, dysphonia and hoarseness. He also had a 2-month history of hypertension and palpitations. On physical examination, the patient had deficits in cranial nerves (CN) VII, VIII, IX, X, XI and XII and blood pressure of 150x90mmHg. Head and neck MRI showed an expansive lesion occupying the right jugular foramen extending above the cistern at the cerebellopontine angle and below the carotid space in the ipsilateral infratemporal fossa, reaching 50% of the internal carotid artery and extending into the right internal auditory canal, suggestive of glomus jugulare paraganglioma. Laboratory examination showed normetanephrines 9.1 mmol/L (VR &amp;lt; 0.9), confirming catecholamine-secreting paraganglioma. Next Generation Sequence (NGS) genetic testing evaluating 24 genes (ATM, DLST, EGLN1, EGLN2, FH, EPAS1 (HIF2A), HRAS, KIF1B , MAX, MDH2, MEN1 , MERTK , MET, NF1, RET, SLC25A11, SDHA, SDHAF2, SDHB, SDHC, SDHD, TMEM127, TP53 and VHL) was positive for the pathogenic variant c.166_170del:p.(Pro56Tyfs*5) of the SHDB gene. Patient with no family history of paraganglioma. Alpha-adrenergic blockade was started with doxazosin and, after two weeks, beta-adrenergic blockade with propranolol. After 3 months, he underwent preoperative embolization 48 hours before surgery with total tumor resection. Patient did not require antihypertensive drugs after surgery. CONCLUSIONS: We reported a case of a young patient diagnosed with catecholamine secreting HNPGL that carries pathogenic variant of SHDB. Presentation: Thursday, June 15, 2023

THU100 Case Of A Large Suprasellar-clival Mass: Prolactinoma Vs Paraganglioma

Abstract Disclosure: L.N. Madera Marin: None. Y. Rivera-Nieves: None. M.A. Ortiz-Rivera: None. A. Rosado-Burgos: None. L. El Musa Penna: None. W. Medina-Torres: None. L. Sepulveda-Garcia: None. M. Ramirez: None. L.A. Gonzalez-Rodriguez: None. M. Alvarado: None. N. Canales: None. Introduction Prolactin secreting adenomas comprise 30-40% of all pituitary adenomas. Generally, serum prolactin levels correlate with tumor size. Giant prolactinomas measure &amp;gt;4 cm, most have mass related clinical effects. Even large masses respond to cabergoline. Some may require surgical intervention. We present a case of a large pituitary mass associated with elevated prolactin levels, but with an interesting histopathologic analysis. Patient Description 30 y/o male patient with new onset decreased peripheral vision. Retinologist evaluation showed no abnormalities. Laboratories done by PCP found with altered thyroid function tests, but no treatment offered at the time. Two months later, the patient complained of worsening peripheral vision and was found with a left dilated pupil. A head CT scan was performed that showed a large brain mass. Admitted for urgent neurosurgery evaluation and high dose dexamethasone was initiated. ROS was remarkable for weight gain over the past year, and decreased morning erections, although denied decreased libido or erectile dysfunction. MRI showed an infiltrative skull base tumor extending from the clivus to the parasellar area encasing the left cavernous segment of the internal carotid artery with expansion of the sella obliterating the pituitary gland, and extension into the left optic canal. Pre-operative laboratories: Prolactin &amp;gt;877 ng/ml (2-17ng/ml), Cortisol 1.80 mcg/dL (4-22 mcg/dL), TSH 1.45 uIU/ml (0.4-4.0 uIU/ml), Sodium 142mMol/L (135-145mMol/L). Patient underwent left pterional craniotomy. Initial intra-operative pathology: “Positive for Neoplasia”. Post-operative laboratories: Prolactin &amp;gt;877 ng/ml, TSH 0.229 uIU/ml, FSH &amp;lt;0.30 mIU/ml (1.2-7.8 mIU/ml), and LH 0.16 mIU/ml (1.5-9.3 mIU/ml). Case Outcomes/Discussion Central hypothyroidism and hypogonadism confirmed, secondary hypocortisolism could not be assessed due to emergent corticosteroid infusion upon admission. A presumptive diagnosis of a giant prolactinoma was made for which cabergoline therapy was initiated. Final pathologic diagnosis revealed a paraganglioma. Urine fractionated metanephrines and catecholamines were normal. Immunostaining for prolactin requested. Paragangliomas arise from neuroendocrine cells, from either sympathetic or parasympathetic ganglia and can be solitary or multiple. Skull base and neck paragangliomas are usually benign while ∼20% of abdominal paragangliomas (sympathetic) are malignant. Anterior skull base paragangliomas are predominantly located in the sella and are rare but have been described in the literature. Very rare cases of pheochromocytomas or paragangliomas associated to pituitary adenomas have been reported. It has been named as the three P association, thought to be related to succinate dehydrogenase B and D mutations. Moreover, there are no cases in the literature of paragangliomas secreting prolactin. Presentation: Thursday, June 15, 2023

SAT314 Bilateral Sporadic Carotid Paraganglioma As A Rare Cause Of Syncope

Abstract Disclosure: A.G. Olafimihan: None. U.A. Ogar: None. I. Asemota: None. E. Krishnaraju: None. A. Okafor: None. Introduction: Carotid body tumors (CBT) are rare, mostly non-secretory skull-based neuroendocrine tumors originating from the parasympathetic ganglia. They are the most common (60%) paragangliomas of the head and neck. Bilaterality is seen in 5% of patients with sporadic CBT. CBT mainly presents as an asymptomatic neck mass. Seldomly, it compresses the carotid sinus, inducing bradycardia and hypotension, and presenting with recurrent syncope. Case presentation: A 70-year-old Eritrean woman visiting the US with a history of myasthenia gravis status post thymectomy on pyridostigmine presented following an episode of presyncope with five similar episodes in the past year. She described it as sudden onset lightheadedness that resolves with sitting and sometimes results in syncope. She had no palpitations, headaches, or sweating.Her admitting vital signs were significant for bradycardia (48bpm) with hypotension (112/54mmhg). A physical exam revealed mobile, mildly tender, palpable neck masses bilaterally, with reproducible worsening bradycardia on palpation of the masses. ECG was unremarkable except for sinus bradycardia. A CT angiogram head and neck with contrast showed multiple hyper-enhancing lesions (two on the right, one on the left; largest = 1.6 x 1.0 cm) at the junction of bilateral carotid bulbs suggestive of multiple paragangliomas. An MRI of the neck re-demonstrated these lesions with the classical ‘salt and pepper’ appearance. CT of the brain, chest, abdomen and pelvis were negative for metastatic lesions. Biochemical testing including plasma metanephrines, catecholamines and dopamine levels was normal, suggesting nonfunctional tumors. Genetic testing was negative for mutations associated with paragangliomas. She did not have more episodes during her three-day admission but remained bradycardic with uneventful telemetry. She was counseled to avoid sudden neck movement and neck pressure. She was discharged with multidisciplinary (endocrinology, neurology, vascular surgery, and ENT surgery) outpatient follow-up. Over the five months post-discharge, she had only two mild symptomatic episodes. Following multi-specialist deliberations at the tumor board, including a risk-benefit assessment of a surgical excision, and extensive discussions with our patient and her family, the decision to hold off surgery was made. Surgical intervention was associated with possible vagal nerve injury, and high aspiration risk given her comorbid myasthenia gravis. Her conservative management plan included a cardiology work-up for symptom-directed therapy with a pacemaker placement. Conclusion: Recurrent syncope is an uncommon presentation of carotid body tumors requiring workup to rule out functionality, genetic mutations, and metastases. Management involves a multi-disciplinary approach and individual risk-benefit assessment of treatment options. Presentation: Saturday, June 17, 2023

Management of pheochromocytomas and paragangliomas: Review of current diagnosis and treatment options.

Pheochromocytomas (PCCs) are rare neuroendocrine tumors derived from the chromaffin cells of the adrenal medulla. When these tumors have an extra-adrenal location, they are called paragangliomas (PGLs) and arise from sympathetic and parasympathetic ganglia, particularly of the para-aortic location. Up to 25% of PCCs/PGLs are associated with inherited genetic disorders. The majority of PCCs/PGLs exhibit indolent behavior. However, according to their affiliation to molecular clusters based on underlying genetic aberrations, their tumorigenesis, location, clinical symptomatology, and potential to metastasize are heterogenous. Thus, PCCs/PGLs are often associated with diagnostic difficulties. In recent years, extensive research revealed a broad genetic background and multiple signaling pathways leading to tumor development. Along with this, the diagnostic and therapeutic options were also expanded. In this review, we focus on the current knowledge and recent advancements in the diagnosis and treatment of PCCs/PGLs with respect to the underlying gene alterations while also discussing future perspectives in this field.

Prevalence of drooling in patients with parkinson disease

Background and aims: Drooling is the involuntary spillage of saliva from the mouth and is apparent in approximately 75% of individuals with PD and was historically interpreted as resulting from hypersecretion of saliva because of autonomic dysfunction. Alteration in salivary gland function is believed to arise from PD-associated changes in the autonomic nervous system and possibly involves the salivary para-sympathetic ganglia. Irrespective of the amount of saliva produced, drooling probably occurs because of PD-related inability to efficiently swallow with normal frequency, an inability to fully close the mouth, and an anterior flexed head position. Recognizing and managing sialorrhea is an important issue to ensure quality of life for patients with PD. The study aims to identify prevalence of drooling at patients hospitalized at neurology department in Tirana and increasing awareness about the importance of sialorrhea treatment in PD patients. Methods: In this retrospective study we investigated the prevalence of drooling in PD patients. We investigated the distribution of drooling according to sex and age and also the impact of drooling in this population by neurological examination and questionnaires given to them in a 6-month time distance. In addition, we studied the impact of gender in drooling in this population. Results: Our results show that most of the patients participating in the study showed signs of drooling. A significant correlation exists between drooling and stages of disease. Furthermore, in males, the correlation between the prevalence of drooling was found to be clinically significant compared to females. Conclusion: Our findings suggest that drooling is a major concern in the course of PD and should be addressed and treated early in patients with PD. Key word: Parkinson Disease, prevalence, sialorrhea, quality of life

193 13 SECONDS OF ASYSTOLE IN YOUNG: IS A PACEMAKER NECESSARY?

Abstract Introduction Cardioinhibitory neurocardiogenic syncope (CNS) is caused by inappropriately trigger-activated cardiac reflex which finally precipitates asystole, sinus bradycardia, or atrioventricular block. In the absence of structural heart disease, the prognosis is excellent, but frequent syncopal episodes can have a significant impact on quality of life. No medical therapy has proved to be really effective and studies concerning pacemaker implantation are often controversial. However, in young individuals, especially those under 40 years of age, implanting a pacemaker may carry more risks than benefits. Thus, alternative techniques such as ablation of the cardiac atrial parasympathetic ganglia (GP) have emerged. Case report A 34-year-old man with frequent episodes of reflex syncope performed a tilt test in March 2022 that diagnosed cardio-inhibitory syncope with 13-second asystole interrupted with external cardiac massage and intravenous atropine. In May 2022 he had an MRI scan showing normal biventricular systolic function and absence of late-gadolinium enhancement and underwent loop recorder implantation. He came to our centre in June 2022 and underwent electrophysiological study and electroanatomical mapping of the right atrium (Carto 3) during general anaesthesia. The anatomical areas of the supero-posterior and infero-posterior GPs of the right atrium were identified by electroanatomical mapping of complex fractionated atrial electrograms (CFAE). Radiofrequency pulses (Thermocool 30 watts) were delivered at these points, resulting in the disappearance of the CFAEs and an increase in basal heart rate. The greatest number of ganglia are allocated on the posterior surface of the right atrium and in particular the supero-posterior GP is the nexus point for vagal input to the GPs before innervating the atria. Therefore, it was decided to localise and ablate exclusively the GPs of the right atrium. The procedure was completed without complications. The patient has not complained of any syncopal episodes since, and the loop has not recorded any significant pauses. Conclusions The anatomic ablation of GP only in the right atrium could represent a feasible method to reduce CNS, to increase the warning symptoms period, and to delay as much as possible pacemaker implant, especially in young patients who have experienced a related physical trauma. This approach significantly decreased the procedural time and risks.

KARAKTERISTIK KLINIKOPATOLOGI PENYAKIT HIRSCHSPRUNG DI RSUP SANGLAH DENPASAR TAHUN 2019-2020

ABSTRACT&#x0D; &#x0D; Hirschsprung's disease is an abnormal condition of the colon with characteristics of the Meissneri submucosus, myenteric plexus and Aurbachi plexus without parasympathetic ganglion cells. Manifestations of Hirschsprung's disease are seen in the discharge of meconium that does not come out within 24 hours. Then there are signs of obstruction to the gastrointestinal tract, vomiting bile, constipation, and irregular bowel movements. In 2017, 2.5 million children died in the first month of life worldwide. 47% of deaths of children under the age of 5 years occur in newborns, which is about 7,000 deaths. Cases of Hirschsprung's disease itself occur in 1:5000-7000 births, with cases in boys 4 times more than girls. This study aims to determine the clinicopathological characteristics of Hirschsprung's disease at Sanglah Hospital Denpasar in 2019-2020 based on clinical symptoms, age, gender, and location of the aganglionic bowel segment.&#x0D; This study was carried out in 2021 at the Sanglah Hospital Denpasar Anatomical Pathology (PA) installation, using data from the years 2019-2020. It was conducted using a cross-sectional method, with secondary data in the form of patient data available at the Sanglah Hospital Denpasar PA Laboratory, and total sampling. SPSS version 26 was used to analyze the data.&#x0D; The results of this study showed that there were 78 patients with Hirschsprung's disease at Sanglah Hospital Denpasar in 2019-2020 and all of them met the criteria of inclusion. Most cases of Hirschsprung's disease in Sanglah Hospital Denpasar experienced the most flatulence, namely 38 cases with a percentage of 36.5%. Cases were dominated by patients within age of 0-12 months with total of 64 people with 82.1% percentage, and dominated by men as many as 50 people with 64.1% percentage. The aganglionic most frequently found in the rectum as many as 49 people with a percentage of 62.8%.&#x0D; It was concluded from the study result that Hirschsprung's disease patients at Sanglah Hospital in 2019-2020 were mostly male, mostly in the age range 0-12 months, with the most clinical symptoms being flatulence, and the most common aganglionic location found in the rectum. This result may then be used as a basis and also study material for further related research and learning&#x0D; Keywords : Hirschsprung's disease, congenital megacolon, aganglionic, clinicopathological characteristics

Expression of the gap junction protein connexin36 in small intensely fluorescent (SIF) cells in cardiac parasympathetic ganglia of rodents

In mammals, several endocrine cell types are electrically coupled by connexin36 (Cx36)-containing gap junctions, which mediate intercellular communication and allow regulated and synchronized cellular activity through exchange of ions and small metabolites via formation of intercellular channels that link plasma membranes of apposing cells. One cell type thought to be endocrine-like in nature are small intensely fluorescent (SIF) cells that store catecholamines in their dense-core vesicles and reside in autonomic ganglia. Here, using immunofluorescence approaches, we examined whether SIF cells located specifically in cardiac parasympathetic ganglia of adult and neonatal mice and adult rats follow patterns of Cx36 expression seen in other endocrine cells. In these ganglia, SIF cells were identified by their distinct small soma size, autofluorescence at 475 nm, and immunolabelling for their markers tyrosine hydroxylase and vesicular monoamine transporter-1. SIF cells were often found in pairs or clusters among principal cholinergic neurons. Immunofluorescence labelling of Cx36 occurred exclusively as fine puncta that appeared at contacts between SIF cell processes and somata or at somato-somatic appositions of SIF cells. These puncta were absent in cardiac parasympathetic ganglia of Cx36 null mice. Transgenic mice expressing enhanced green fluorescent protein reporter for Cx36 expression displayed labelling for the reporter in SIF cells. The results suggest that Cx36-containing gap junctions electrically couple SIF cells, which is consistent with previous suggestions that these may be classified as endocrine-type cells that secrete catecholamines into the bloodstream in a regulated manner.

Effect of Physical Training on the Morphology of Parasympathetic Atrial Ganglia after Unilateral Vagotomy in Rats.

Cardiac denervation is a serious problem in a number of patients, including patients after heart transplantation. The status of the parasympathetic ganglia after crossing the preganglionic fibers of the vagus nerve has not been enough studied. The aim of our study was to assess the effect of physical training on the morphological parameters of the parasympathetic atrial ganglia and autonomic regulation of heart rate after right- and left-sided vagotomy in rats. Morphometric characteristics of the right atrial ganglia were evaluated using an immunohistochemical method after a study that included a three-time assessment of heart rate variability. It was found that right-sided vagotomy leads to both an increase in the volume of ganglion and autonomic dysfunction. No significant change in the number of nerve cells was found in animals with false and left-sided vagotomy while maintaining preganglionic innervation after the physical training, whereas exercises led to a decrease in the volume of nerve tissue of rats with right-sided denervation. It was also found that in animals with preserved vagal innervation, the volume of atrial ganglion tissue correlates with overall heart rate variability and a normalized parasympathetic component. Therefore, a positive effect from regular physical activity on parasympathetic regulation can be expected only if preganglionic vagal influence is preserved.

The Correlation of Post Definitive Surgery and Quality of Life on Patient with Hirschsprung’s Disease: A Literature Review

Hirschsprungs disease is a congenital malformation of parasympathetic ganglion in colon with the result of interfering defecation process. Since Hirschsprungs disease usually diagnoses in the early life of the patients, complain of defecation problem occurs on pediatrics patient. The management of Hirschsprungs disease is definitive surgery. Quality of life described the effectiveness of definitive surgery as the primary management of Hirschsprungs disease. The desired goal of this literature review was to described the pediatric patients quality of life post definitive surgery from previous similar study.

Satellite Glial Cells: Morphology, functional heterogeneity, and role in pain.

Neurons in the somatic, sympathetic, and parasympathetic ganglia are surrounded by envelopes consisting of satellite glial cells (SGCs). Recently, it has become clear that SGCs are highly altered after nerve injury, which influences neuronal excitability and, consequently, the development and maintenance of pain in different animal models of chronic pain. However, the exact mechanism underlying chronic pain is not fully understood yet because it is assumed that SGCs in different ganglia share many common peculiarities, making the process complex. Here, we review recent data on morphological and functional heterogeneity and changes in SGCs in various pain conditions and their role in response to injury. More research is required to decipher the role of SGCs in diseases, such as chronic pain, neuropathology, and neurodegenerative diseases.