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Related Concept Videos

Nephrotic Syndrome I : Introduction01:24

Nephrotic Syndrome I : Introduction

Nephrotic Syndrome is a chronic kidney disorder defined by clinical findings such as severe proteinuria, hypoalbuminemia, hyperlipidemia, and edema. These symptoms result from damage to the glomeruli, the kidney’s filtering units, increasing their permeability to proteins.Definition and Meaning:Proteinuria, defined as the loss of more than 3.5 grams of protein per day in adults, is a crucial feature of nephrotic syndrome. This condition is often accompanied by edema, the accumulation of fluid...
Nephrotic Syndrome II : Assessment and Medical Management01:26

Nephrotic Syndrome II : Assessment and Medical Management

IntroductionNephrotic syndrome is a kidney disorder marked by excessive protein loss in the urine, leading to various systemic complications. This condition often results from damage to the glomeruli—the kidney's filtering units—causing proteinuria, low blood protein levels, and fluid retention. Understanding the assessment, diagnosis, and management of nephrotic syndrome is essential for effective treatment and prevention of further kidney damage.AssessmentPatient History: Document any history...
Portal Hypertension01:22

Portal Hypertension

Portal hypertension is an increase in blood pressure within the portal venous system. Normally, this pressure is less than 5 mmHg. It is considered clinically significant when it rises above 10 mmHg. At this threshold, complications from altered blood flow and venous congestion emerge.EtiologyPortal hypertension arises from conditions that impede blood flow through the liver. The most common cause is cirrhosis, in which chronic liver injury leads to fibrotic scarring. This fibrosis narrows or...
Esophageal Varices-II: Clinical Features and Management01:28

Esophageal Varices-II: Clinical Features and Management

Esophageal varices often manifest as gastrointestinal bleeding episodes, presenting symptoms like hematemesis (vomiting of blood), hematochezia (passing fresh blood via the rectum), and melena (black, tarry stools). Other signs can include weight loss, anorexia, abdominal discomfort, jaundice, pruritus, altered mental status, and muscle cramps.
In the initial assessment, a thorough review of the patient's medical history is vital to identify risk factors such as liver disease, alcohol abuse, or...
Cirrhosis II: Pathophysiology01:24

Cirrhosis II: Pathophysiology

Cirrhosis is a progressive chronic liver injury caused by prolonged inflammation, excessive fibrotic remodeling, and impaired regeneration. Over time, repeated hepatic insults disrupt the liver’s architecture and function, leading to reduced blood flow, impaired bile drainage, and diminished metabolic capacity.Pathophysiology of cirrhosisCirrhosis arises from three main responses to chronic liver damage: inflammation, immune activation, and hepatocyte death. These processes lead to structural...
Nephrotic Syndrome III : Nursing Management01:24

Nephrotic Syndrome III : Nursing Management

Nursing management for nephrotic syndrome adapts as the disease progresses, with strategies evolving to address advancing symptoms and complications.Early-Stage Management In the early stages, nursing interventions for nephrotic syndrome resemble those used in managing acute glomerulonephritis, focusing on symptom monitoring, fluid balance, and managing mild to moderate edema.Vital Signs: Regularly monitor blood pressure, pulse, respiratory rate, and temperature to promptly identify...

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Characterization of Leukocyte-platelet Rich Fibrin, A Novel Biomaterial
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Bernard-Soulier syndrome: an update.

Robert K Andrews1, Michael C Berndt

  • 1Department of Clinical Haematology, Monash University, Systems Haematology Laboratory, Australian Centre for Blood Diseases, Melbourne, Australia.

Seminars in Thrombosis and Hemostasis
|August 10, 2013
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Bernard-Soulier syndrome (BSS) is a rare inherited bleeding disorder affecting platelet count and size. Understanding BSS improves diagnosis and treatment of this complex platelet disorder.

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Area of Science:

  • Hematology
  • Molecular Biology
  • Genetics

Background:

  • Bernard-Soulier syndrome (BSS) is a rare inherited platelet disorder.
  • Characterized by macrothrombocytopenia and defective glycoprotein (GP)Ib-IX-V complex.
  • This complex is crucial for platelet adhesion and signaling.

Purpose of the Study:

  • To discuss the contribution of BSS studies to platelet biology.
  • To review recent advancements in BSS diagnosis and treatment.

Main Methods:

  • Literature review of BSS pathogenesis.
  • Analysis of genetic defects in the GPIb-IX-V complex.
  • Review of diagnostic and therapeutic strategies.

Main Results:

  • BSS pathogenesis involves defects in GPIb-IX-V expression or function.
  • These defects impact platelet size, adhesion, and thrombus formation.
  • BSS has multiple genetic causes and a variable clinical phenotype.

Conclusions:

  • Studies of BSS have significantly advanced platelet biology.
  • Improved diagnostic and therapeutic approaches for BSS are emerging.
  • Further research is needed to fully understand and manage BSS.