小児期の末期肝疾患によるグルメロネフライト
G Noble-Jamieson1, S Thiru, P Johnston
1Department of Paediatrics, Addenbrooke's Hospital, Cambridge, UK.
Lancet (London, England)
|March 21, 1992
まとめ
肝移植を受けた小児の患者は,しばしばグルメロネフリスを発症します. しかし,大半は移植後の安定した腎機能を示し,患児のタンパク質尿は解消した.
科学分野:
- ネフロロジーは腎臓科
- 小児胃腸内科 小児胃腸内科
- 移植免疫学 移植免疫学
背景:
- 肝臓移植は,末期肝疾患の子どもたちの命を救う手術です.
- 移植後の腎臓合併症は,グルメルノネフライトを含む,長期的な結果に影響を与える可能性があります.
- 小児肝臓移植後の腎臓病変の発生率と経過を理解することは極めて重要です.
研究 の 目的:
- 肝臓移植を受けた小児におけるグルメルノネフライトの発生と特徴を調査する.
- これらの患者の移植後の腎機能と臨床経過を評価する.
主な方法:
- 腎臓生検は,肝臓移植を受けた18人の小児の患者に,手術前期に実施されました.
- 組織病理学的分析により,グルメロネフリスの種類 (メサンジアル増殖性またはメサンジオ毛細血管性) が特定されました.
- 移植後の腎機能は,球の濾過率とタンパク質尿を含む,移植後のモニタリングが行われました.
主要な成果:
- 肝移植を受けた18人の小児患者は全員,腎臓生検でグローメルロネフリチスを発症した.
- 主なタイプは,メサンジアル増殖性グローメルロネフライト (15/18).
- 4ヶ月以上生きていた16人の患者のうち,1人だけが進行性腎機能低下を示し,1人は静的不正常な濾過をしていました. タンパク質尿症は,手術前の6人の患児全員で解消した.
結論:
- グロメルロネフライトは,小児の肝臓移植受容者の間でよく見られる症状です.
- バイオプシーの結果にもかかわらず,ほとんどの子供では腎臓機能は一般的に安定しているか,移植後の状態が改善している.
- 肝臓移植は,この集団における既存のタンパク質尿に有益な影響を及ぼす可能性があります.
関連する概念動画
Lysosomal Hydrolases
Lysosomes are the site for the degradation of macromolecules and biological polymers released during membrane trafficking events such as secretory, endocytic, autophagic, and phagocytic pathways. The membrane-enclosed area of the lysosome, called the lumen, contains hydrolytic enzymes active in an acidic environment. These acid hydrolases are functional at a pH between 4.5 and 5 and are involved in cellular processes such as cell signaling, energy metabolism, restoration of the plasma membrane,...
Liver Regeneration
The liver is an important organ in vertebrates that plays an essential role in metabolism. It is also responsible for storing and redistributing nutrients such as carbohydrates, fats, and vitamins in the body. Additionally, the liver releases bile salts which are critical for digesting food and eliminating toxic metabolites from the body.
Cells of Liver
The liver comprises four major types of cells— hepatocytes, stellate, Kupffer, and sinusoidal endothelial cells. The hepatocytes are large...
Cells of Liver
The liver comprises four major types of cells— hepatocytes, stellate, Kupffer, and sinusoidal endothelial cells. The hepatocytes are large...
Disorders of Leukocytes
Leukocyte disorders can lead to either leukopenia, characterized by an abnormally low leukocyte count, or leukocytosis, marked by a very high leukocyte number.
Leukopenia may result from bone marrow disorders, autoimmune diseases, and infectious diseases. For example, conditions such as multiple myeloma and aplastic anemia can impair the bone marrow's ability to produce adequate leukocytes. Similarly, autoimmune diseases like lupus and viral infections such as HIV can prompt the immune system...
Leukopenia may result from bone marrow disorders, autoimmune diseases, and infectious diseases. For example, conditions such as multiple myeloma and aplastic anemia can impair the bone marrow's ability to produce adequate leukocytes. Similarly, autoimmune diseases like lupus and viral infections such as HIV can prompt the immune system...
Immunodeficiency Diseases
Immunodeficiency disorders are conditions in which the immune system's ability to fight infectious disease and cancer is compromised or entirely absent. The immune system comprises a complex network of cells, tissues, and organs that work together to protect the body from potentially harmful invaders. When this system is deficient or not functioning properly, it leaves the body susceptible to infections, diseases, or other complications.
There are three main causes of immunodeficiency disorders...
There are three main causes of immunodeficiency disorders...
Diseases of the Liver and Gallbladder
Liver and gallbladder diseases are a significant health concern, with prominent conditions including cirrhosis, hepatitis, non-alcoholic fatty liver disease (NAFLD), and gallstones. Jaundice is a common manifestation of liver and biliary disease.
Cirrhosis is characterized by the scarring of hepatic lobules in the liver, which are replaced by fibrous tissue, affecting the liver's normal functioning. NAFLD, on the other hand, is caused by an excessive build-up of fat in the liver, not related to...
Cirrhosis is characterized by the scarring of hepatic lobules in the liver, which are replaced by fibrous tissue, affecting the liver's normal functioning. NAFLD, on the other hand, is caused by an excessive build-up of fat in the liver, not related to...
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...


