Approach to clinically significant vascular anomalies in children

Vascular anomalies consist of tumours or malformations made up of abnormal growth or collections of blood vessels that can result in functional or cosmetic problems. While many vascular anomalies are present at birth, some do not appear until later in life, making diagnosis more challenging. Although many vascular anomalies are benign, some are associated with serious complications and may involve multiple organ systems. This article highlights the important features of clinically significant vascular anomalies to help physicians promptly identify and refer these cases to a specialised multidisciplinary team for evaluation and management. The discussion includes the various presenting complaints of vascular anomalies in children, namely rapidly growing birthmarks, painful lesions, seizures/neurological manifestations, bleeding diathesis, cardiac/airway abnormalities or part of an overgrowth syndrome.

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Microprobe analysis of inclusion bodies related to two benzofuran derivatives

Proceedings, annual meeting, Electron Microscopy Society of America ◽

10.1017/s0424820100127785 ◽

1987 ◽

Vol 45 ◽

pp. 672-673

Author(s):

T. L. Benning ◽

P. Ingram ◽

J. D. Shelburne

Keyword(s):

Inclusion Bodies ◽

Uranyl Acetate ◽

Multiple Organ ◽

High Dose ◽

En Bloc ◽

Tissue Samples ◽

Transmission Electron ◽

Organ Systems ◽

Dense Inclusion ◽

Melanin Complex

Two benzofuran derivatives, chlorpromazine and amiodarone, are known to produce inclusion bodies in human tissues. Prolonged high dose chlorpromazine therapy causes hyperpigmentation of the skin with electron-dense inclusion bodies present in dermal histiocytes and endothelial cells ultrastructurally. The nature of the deposits is not known although a drug-melanin complex has been hypothesized. Amiodarone may also cause cutaneous hyperpigmentation and lamellar lysosomal inclusion bodies have been demonstrated within the cells of multiple organ systems. These lamellar bodies are believed to be the product of an amiodarone-induced phospholipid storage disorder. We performed transmission electron microscopy (TEM) and energy dispersive x-ray microanalysis (EDXA) on tissue samples from patients treated with these drugs, attempting to detect the sulfur atom of chlorpromazine and the iodine atom of amiodarone within their respective inclusion bodies.A skin biopsy from a patient with hyperpigmentation due to prolonged chlorpromazine therapy was fixed in 4% glutaraldehyde and processed without osmium tetroxide or en bloc uranyl acetate for Epon embedding.

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Impairment Rating and Spinal Cord Injuries: Revisiting the Guides

Guides Newsletter ◽

10.1001/amaguidesnewsletters.2010.mayjun01 ◽

2010 ◽

Vol 15 (3) ◽

pp. 1-7

Author(s):

Richard T. Katz

Keyword(s):

Spinal Cord ◽

Spinal Cord Injuries ◽

Functional Independence ◽

Outcome Data ◽

Multiple Organ ◽

Loss Of Function ◽

Sixth Edition ◽

Organ Systems ◽

Cord Injury ◽

Impairment Ratings

Abstract This article addresses some criticisms of the AMA Guides to the Evaluation of Permanent Impairment (AMA Guides) by comparing previously published outcome data from a group of complete spinal cord injury (SCI) persons with impairment ratings for a corresponding level of injury calculated using the AMA Guides, Sixth Edition. Results of the comparison show that impairment ratings using the sixth edition scale poorly with the level of impairments of activities of daily living (ADL) in SCI patients as assessed by the Functional Independence Measure (FIM) motor scale and the extended FIM motor scale. Because of the combinations of multiple impairments, the AMA Guides potentially overrates the impairment of paraplegics compared with that of quadriplegics. The use and applicability of the Combined Values formula should be further investigated, and complete loss of function of two upper extremities seems consistent with levels of quadriplegia using the SCI model. Some aspects of the AMA Guides contain inconsistencies. The concept of diminishing impairment values is not easily translated between specific losses of function per organ system and “overall” loss of ADLs involving multiple organ systems, and the notion of “catastrophic thresholds” involving multiple organ systems may support the understanding that variations in rating may exist in higher rating cases such as those that involve an SCI.

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Renal Manifestations of Tuberous Sclerosis Complex

Journal of Kidney Cancer and VHL ◽

10.15586/jkcvhl.v7i3.131 ◽

2020 ◽

Vol 7 (3) ◽

pp. 5-19

Author(s):

Nikhil Nair ◽

Ronith Chakraborty ◽

Zubin Mahajan ◽

Aditya Sharma ◽

Sidarth Sethi ◽

...

Keyword(s):

Tuberous Sclerosis ◽

Tuberous Sclerosis Complex ◽

Gene Disruption ◽

Treatment Guidelines ◽

Renal Cysts ◽

Skin Lesions ◽

Multiple Organ ◽

Tsc2 Gene ◽

Genetic Condition ◽

Organ Systems

Tuberous sclerosis complex (TSC) is a genetic condition caused by a mutation in either the TSC1 or TSC2 gene. Disruption of either of these genes leads to impaired production of hamartin or tuberin proteins, leading to the manifestation of skin lesions, tumors and seizures. TSC can manifests in multiple organ systems with the cutaneous and renal systems being the most commonly affected. These manifestations can secondarily lead to the development of hypertension, chronic kidney disease, and neurocognitive declines. The renal pathologies most commonly seen in TSC are angiomyolipoma, renal cysts and less commonly, oncocytomas. In this review, we highlight the current understanding on the renal manifestations of TSC along with current diagnosis and treatment guidelines.

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Attenuation of COVID-19-induced cytokine storm in a young male patient with severe respiratory and neurological symptoms

Wiener klinische Wochenschrift ◽

10.1007/s00508-021-01867-2 ◽

2021 ◽

Author(s):

Christian Muschitz ◽

Anita Trummert ◽

Theresa Berent ◽

Norbert Laimer ◽

Lukas Knoblich ◽

...

Keyword(s):

Decision Making ◽

Male Patient ◽

Young Male ◽

Standard Of Care ◽

Multiple Organ ◽

Cytokine Storm ◽

Invasive Ventilation ◽

Organ Systems ◽

The Central Nervous System

SummarySevere acute respiratory syndrome coronavirus type 2 (SARS-CoV-2), the etiological agent of coronavirus disease 2019 (COVID-19), produces protean manifestations and causes indiscriminate havoc in multiple organ systems. This rapid and vast production of proinflammatory cytokines contributes to a condition termed cytokine storm. A 35-year-old, otherwise healthy, employed, male patient was tested positive for COVID-19. He was admitted to the hospital on disease day 10 due to retarded verbal reactions and progressive delirium. On account of these conditions and the need for noninvasive/invasive ventilation, a combination treatment with baricitinib and remdesivir in conjunction with standard of care was initiated. The cytokine storm was rapidly blocked, leading to a vast pulmonary recovery with retarded recovery of the central nervous system. We conclude that the rapid blockade of the COVID-19-induced cytokine storm should be considered of avail as a principle of careful decision-making for effective recovery.

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A Case of IgG4-Related Kidney Disease Developing While on Steroid Treatment for Autoimmune IgG4 Pancreatitis

Journal of Investigative Medicine High Impact Case Reports ◽

10.1177/23247096211026500 ◽

2021 ◽

Vol 9 ◽

pp. 232470962110265

Author(s):

Jonathan Vincent M. Reyes ◽

Dawn Maldonado ◽

Aaron S. Stern ◽

Maritza Brown

Keyword(s):

Kidney Disease ◽

Inflammatory Process ◽

Systemic Disease ◽

Multiple Organ ◽

Steroid Treatment ◽

Autoimmune Process ◽

Immunoglobulin G4 ◽

Organ Systems ◽

The Relationship ◽

Steroid Sparing

IgG4 (immunoglobulin G4)-related systemic disease is an autoimmune process affecting multiple organ systems. This inflammatory process can present as but not limited to pancreatitis, cholangitis, or unspecified kidney disease. In this case, our patient developed IgG4-related kidney disease while already on a prolonged steroid course for IgG4-related pancreatitis. The patient ultimately had renal recovery after starting a higher dose of prednisone, but also developed steroid-related complications. This case further highlights the relationship between IgG4 diseases now termed IgG4-related systemic disease. This case brings to light the need for further investigative research into ideal steroid dosing, as well as steroid-sparing agents for IgG4-related systemic disease.

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Thoracic trauma in small animals: road traffic accidents

The Veterinary Nurse ◽

10.12968/vetn.2021.12.1.15 ◽

2021 ◽

Vol 12 (1) ◽

pp. 15-19

Author(s):

Kate Sheard

Keyword(s):

Traffic Accidents ◽

Thoracic Trauma ◽

Road Traffic ◽

Positive Outcome ◽

Multiple Organ ◽

Trauma Patients ◽

Small Animals ◽

Road Traffic Accidents ◽

Blunt Force Trauma ◽

Organ Systems

Thoracic trauma is common in small animals and can be caused by a variety of insults from penetrating wounds to blunt force trauma. Patients that have sustained any form of thoracic trauma require immediate attention and intensive nursing care in order to have a positive outcome for the patient. These cases can prove challenging as multiple organ systems can be affected and surgery is often required. However, combined with the appropriate medical care, the outcome can be successful.

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Complement-Mediated Thrombotic Microangiopathy Associated with Lupus Nephritis Treated with Eculizumab: A Case Report

Case Reports in Nephrology and Dialysis ◽

10.1159/000512227 ◽

2021 ◽

pp. 95-102

Author(s):

Everardo Arias Torres ◽

Yongen Chang ◽

Sheetal Desai ◽

Ian Chang ◽

Jonathan E. Zuckerman ◽

...

Keyword(s):

Lupus Erythematosus ◽

Alternative Pathway ◽

Atypical Hemolytic Uremic Syndrome ◽

Homozygous Deletion ◽

Multiple Organ ◽

Thrombotic Microangiopathies ◽

Clinical Criteria ◽

Organ Systems ◽

Oral Steroids ◽

Uremic Syndrome

Thrombotic microangiopathies (TMAs) involve multiple organ systems due to the presence of microangiopathic hemolysis. One such condition, atypical hemolytic uremic syndrome (aHUS), is a complement-mediated process that is part of a spectrum of disorders that have underlying complement dysfunction of the alternative pathway due to overactivity or decreased self-nonself discrimination by innate immunity. Complement-amplifying conditions such as pregnancy may unmask a diagnosis of aHUS. We present an important case of a pregnant 23-year-old Hispanic female who presented in mid-gestation (21 weeks) with an initial diagnosis of systemic lupus erythematosus (SLE) complicated by aHUS. She met clinical criteria for aHUS on presentation and was found to have a pathogenic CFHR1–3 homozygous deletion. She has been treated with intravenous and oral steroids, cyclophosphamide, subsequently also with plasma exchange, and finally with eculizumab with partial improvement in renal function. This case adds to the emerging literature showing that SLE and aHUS (or complement-mediated TMA) can be successfully treated with C5 blockade.

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Developing an adolescent and adult Fontan Management Programme

Cardiology in the Young ◽

10.1017/s104795112100175x ◽

2021 ◽

pp. 1-6

Author(s):

Adam M. Lubert ◽

Tarek Alsaied ◽

Andrew T. Trout ◽

Jonathan R. Dillman ◽

Joseph J. Palermo ◽

...

Keyword(s):

Single Ventricle ◽

Fontan Procedure ◽

Multiple Organ ◽

Fontan Circulation ◽

Management Programme ◽

Adverse Outcomes ◽

Cardiac Complications ◽

Organ Systems ◽

Adolescents And Adults ◽

Care Models

Abstract Patients with single-ventricle CHD undergo a series of palliative surgeries that culminate in the Fontan procedure. While the Fontan procedure allows most patients to survive to adulthood, the Fontan circulation can eventually lead to multiple cardiac complications and multi-organ dysfunction. Care for adolescents and adults with a Fontan circulation has begun to transition from a primarily cardiac-focused model to care models, which are designed to monitor multiple organ systems, and using clues from this screening, identify patients who are at risk for adverse outcomes. The complexity of care required for these patients led our centre to develop a multidisciplinary Fontan Management Programme with the primary goals of earlier detection and treatment of complications through the development of a cohesive network of diverse medical subspecialists with Fontan expertise.

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Glucocorticoids produce whole body insulin resistance with changes in cardiac metabolism

AJP Endocrinology and Metabolism ◽

10.1152/ajpendo.00453.2006 ◽

2007 ◽

Vol 292 (3) ◽

pp. E654-E667 ◽

Cited By ~ 70

Author(s):

Dake Qi ◽

Brian Rodrigues

Keyword(s):

Insulin Resistance ◽

Fatty Acid ◽

Glucose Utilization ◽

Experimental Studies ◽

Cardiac Metabolism ◽

Multiple Organ ◽

Whole Body ◽

Metabolic Abnormalities ◽

Organ Systems ◽

Per Se

Insulin resistance is viewed as an insufficiency in insulin action, with glucocorticoids being recognized to play a key role in its pathogenesis. With insulin resistance, metabolism in multiple organ systems such as skeletal muscle, liver, and adipose tissue is altered. These metabolic alterations are widely believed to be important factors in the morbidity and mortality of cardiovascular disease. More importantly, clinical and experimental studies have established that metabolic abnormalities in the heart per se also play a crucial role in the development of heart failure. Following glucocorticoids, glucose utilization is compromised in the heart. This attenuated glucose metabolism is associated with altered fatty acid supply, composition, and utilization. In the heart, elevated fatty acid use has been implicated in a number of metabolic, morphological, and mechanical changes and, more recently, in “lipotoxicity”. In the present article, we review the action of glucocorticoids, their role in insulin resistance, and their influence in modulating peripheral and cardiac metabolism and heart disease.

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