Preoperative factors associated with intraoperative maximum arterial pressures in patients with pheochromocytoma and paraganglioma: a single-center retrospective study

Surgery for pheochromocytoma and paraganglioma (PPGL) can lead to life-threatening complications, such as intraoperative hypertensive crises, even when adequate doses of preoperative α-receptor blockades are administered. The aim of this study was to identify preoperative factors associated with maximum arterial pressure (AP) during surgery in patients with PPGL. We retrospectively reviewed the cases of 61 PPGL patients who underwent surgical resection in our hospital between 2006 and 2020. The primary outcome was intraoperative maximum AP as a single index for continuous variables. The normal distribution of the results was confirmed by the Kolmogorov–Smirnov test. Simple and multiple linear regression model were used for statistical analysis. The median maximum systolic AP during surgery was 165 mmHg (interquartile range: 150–180 mmHg). Although the 24-h urinary-fractionated metanephrine (MN) and normetanephrine (NMN) (mg/day) was not normally distributed, the logarithmic representation (base = 10) of the combination of these continuous variables showed a normal distribution (p = 0.549). Log24 − h urinary−fractionated MN and NMN was correlated with intraoperative maximum AP (R = 0.481, p < 0.001). Multiple regression analyses showed that diabetes mellitus (β = 15.835, standard error [SE] = 7.550, t statistic = 2.097, P = 0.040), the classic triad (β = 14.081, SE = 5.668, t statistic = 2.484, P = 0.016), and log24 − h urinary−fractionated MN and NMN (β = 14.641, SE = 5.842, t statistic = 2.506, P = 0.015) were independent factors associated with intraoperative maximum AP. Patients with PPGL accompanied by diabetes mellitus, the classic triad, and high log 24−h urinary−fractionated MN and NMN values may be at risk for hypertensive crises during surgery regardless of whether preoperative α-receptor blockades are used. Clinicians should manage these patients more carefully and effectively.

Treatment of Reactive Arthritis: A Case Report

Reactive arthritis is clinically defined as inflammatory aseptic arthritis which develops shortly after an extra-articular infection. The classic triad of conjunctivitis, urethritis, and arthritisis found in a minority of patients. Patiepresent with an asymmetric mono- or oligoarthritis that predominantly affect lower limbs. Here, we present a case of reactive arthritis with inadequate primary response to Nonsteroidants Usually l Anti-inflammatory Drugs (NSAIDs).

Plummer-Vinson Syndrome: A Time for Redefinition and New Perspectives

Plummer-Vinson syndrome (PVS) is defined by the classic triad of dysphagia, esophageal web, and iron deficiency. It is a rare entity that remains poorly understood, and we describe our experience in diagnosing and management. Treatment for patients with PVS begins with aggressive dilation of the esophageal web (stricture), which has specific histopathology findings, thus helping to restore nutrition and facilitate iron absorption while also identifying other possible etiologies of iron deficiency, specifically underlying autoimmune conditions. We have reviewed the literature to place our experience in perspective and conclude that PVS now warrants a re-definition and a new perspective, which we discuss in this article.

SUN-LB38 Adrenal Tumor Causing Unexplained Hyperhidrosis in a Young Man

Introduction: Pheochromocytoma is a rare neuroendocrine tumor, afflicting around 0.8 out of 100,000 people per year [1]. The “classic triad” of headache, diaphoresis, and tachycardia is well documented in the literature. The “classic triad” in combination with hypertension has up to 91% sensitivity and 93.5% specificity for the diagnosis of pheochromocytoma [2]. Generalized sweating is part of the “classic triad”, however there is paucity of evidence in the literature describing diaphoresis as the only presenting symptom of pheochromocytoma.Case description: A 20-year-old male presented with five year history of worsening hyperhidrosis without any other associated symptoms. Vital signs were blood pressure of 138/82 mmHg and heart rate of 60 bpm. The remainder of the physical exam was normal. Laboratory testing showed 24 hour urine norepinephrine level of 1,002 ug/24 hours (normal: 0-135 ug/24hrs), plasma normetanephrine was 2,873 pg/mL (normal: 0-145 pg/mL) and plasma norepinephrine was 2,869 pg/mL (normal: 0-874 pg/mL). CT abdomen revealed a 4.0 x 3.1 x 4.3 cm left adrenal mass. After appropriate preoperative preparation, he successfully underwent a laparoscopic left adrenalectomy with complete resolution of diaphoresis. Pathology confirmed a completely resected pheochromocytoma.Discussion: This case reflects an atypical presentation of pheochromocytoma; a disease with high cardiovascular morbidity and mortality [3, 4]. The case demonstrates that the absence of hypertension or all three symptoms of the “classic triad” does not exclude the diagnosis of pheochromocytoma and any singular symptom of catecholamine excess warrants an evaluation for pheochromocytoma. This case report helps to establish the need to better quantify individual symptoms of patients with this disease in order to better understand the entire spectrum. Providers need to be aware of atypical presentation of this disease in order to decrease poor outcomes that can result from undiagnosed pheochromocytoma. 1. Beard, C.M., Sheps, S.G., Kurland, L.T., Carney, J.A., Lie, J.T., Occurence of pheochromocytoma in Rochester, Minnesota 1950-1979. Mayo Clinic proceedings, 1983.2. Bravo, E.L., Pheochromocytoma: New concepts and future trends. Kidney International, 1991. 40.3. Lenders, J.W.M., Duh, Q.Y., Young, W.F, et. al, Journal of Clinical Endocrinology and Metabolism. 2014.4. Zelinka, T., Petrak, O., Turkova, H., et. al, High Incidence of Cardiovascular Complications in Pheochromocytoma. Hormone and Metabolic Research, 2012.

Reactive Arthritis The Reiter-Fiessinger-Leroy Syndrome

The role of creating the expression of reactive arthritis rests with AHO in 1973, which defines reactive arthritis as an acute, unsupportive, sterile inflammatory arthropathy, arising from infections located at a distance in people with a certain genetic predisposition; the disease is systemic and, despite its name, is not limited to the joints. Reactive arthritis (RA), included in Reiter syndrome (with the classic triad of urethritis, conjunctivitis and arthritis), is a non-suppurative inflammatory joint pathology, with a minimal autoimmune component, which it is installed following intestinal or urogenital infections, mainly in people with genetic predisposition. Reactive arthritis is a form of arthritis that affects the joints, urethra, eyes and membranes and sometimes the skin and mucous membranes. This condition is also called Reiter`s syndrome, although this name only indicates a subtype. Reactive arthritis is a seronegative spondyloarthropathy, which brings together a group of disorders that cause inflammation in the body, especially in the spine - such as ankylosing spondylitis, and in the legs. The study covers a number of 20 patients (11 men, 9 women) admitted to the Rheumatology Service of the Clinical Recovery Hospital, between 2010 and 2011, aged over 20, who were diagnosed with reactive arthritis. The existence of a structural similarity between the etiological agent and the HLA-B27 molecule determines either the recognition of the disease-causing germ as a self, so that no immune response will be initiated against it and the antigen will be able to trigger the disease, or the recognition of the germ as nonself, with the triggering of an immune response aimed at both itself and against the HLA-B27 molecule carrying cells of similar structure. Reactive arthritis (RA), included in the Reiter syndrome (with the classic triad of urethritis, conjunctivitis and arthritis), is a non-suppurative inflammatory joint pathology, with a minimal autoimmune component, which is installed as a result of intestinal or urogenital infections, mainly in people with genetic predisposition.

CLINICAL MANIFESTATIONS, VITAL SIGNS, AND BOWEL VIABILITY IN PEDIATRIC INTUSSUSCEPTION

INTRODUCTION: Intussusception is a condition in which part of the intestine slides into an adjacent part of the intestine. Intussusception is an important cause of an acute abdomen and the second most common cause of bowel obstruction in children. The classic triad of intussusception, including vomiting, abdominal colic pain, and bloody stool. However, the triad has only been reported in <50% cases. This study is aimed to evaluate the clinical manifestation, vital signs, and bowel viability in pediatric intussusception. METHODS: This is an observational retrospective study with total sampling. RESULTS: Based on clinical characteristics, there were 44.44% subjects who had manifestations for 2-3 days. Ileocolic was the most common location (77.78%). Majority of the subjects did not have the classic triad (66.67%), had red currant jelly stool (62.96%), did not have abdominal mass (59.26%), complained of abdominal pain (70.37%), did not have abdominal distention (74.07%), did not vomit (55.5%), was lethargic (59.26%), and had viable bowel (62.96%). CONCLUSION: This study found that the most common manifestations of intussusception in children were abdominal pain, red currant jelly stool, and lethargy, with viable bowel and located at ileocolic.

A Rare Case of Cranial Polyneuritis as Complication of Ramsay Hunt Syndrome

Ramsay Hunt Sydnrome is a rare and severe disease caused by the reactivation of varicella zoster virus (VZV) in the ganglia geniculate. The classic triad of this disease includes ear pain (otalgia), vesicles in the auditory canal, and facial paralysis. This case report is about a 37-year-old woman that has the classic triad of Ramsay Hunt Syndrome, as well as a rare complication of cranial polyneuritis in the form of cephalgia and vertigo which occurs only in 1.8% of cases. The patient came one week after the initial symptoms had started and was given antiviral combination therapy and steroids. Treatment of this disease is time sensitive to <72 hours and will determine the prognosis. One month later the follow up showed sequelae in the form of persistent headache and slight facial paralysis as a result of delayed treatment. This case report showed the importance of prompt diagnosis and treatment to minimize complications of Ramsay Hunt Syndrome.

Multiple Nail Candidosis in a Child with APECED Syndrome

The APECED syndrome (autoimmune polyendocrinopathy, candidosis, ectodermal dystrophy) is a rare autosomal recessive disorder that develops in early childhood and results in tissue-specific multiorgan autoimmunity, leading to the hypofunction of multiple glands. Is clinically defined as the presence of at least two components of the classic triad of hypoparathyroidism, adrenal insufficiency, and mucocutaneous candidiasis. We report a case in a child, illustrating the importance of dermatological disorders.

Melkersson-Rosenthal syndrome with coeliac and allergic diseases

A 45-year-old man presented with a 10-year history of relapsing oedema of the lips. Moreover, he exhibited recurrent facial nerve palsy since the age of 10 years, coeliac disease since the age of 12 years, atopic eczema, allergic rhinitis and asthma. Physical examination revealed lip swelling and lingua plicata. Thus, he presented the classic triad of Melkersson-Rosenthal syndrome which includes recurrent orofacial oedema, facial nerve palsy and fissured tongue. A lip biopsy confirmed our clinical diagnosis.This case is particularly rare, as the classic triad is seen only in a minority of the cases. Moreover, allergic and coeliac diseases were observed concomitantly. This paper illustrates a potential pathophysiological interconnection between these pathologies in which interferon gamma could play a key role. To our knowledge, this is the first case report in which Melkersson-Rosenthal syndrome has been observed concurrently with coeliac disease.