obsolete medullary sponge kidney

Medullary Sponge Kidney (MSK): A Rare Congenital Disorder

Medullary sponge kidney (MSK) is a rare congenital disorder that affects the kidneys, characterized by cystic dilatation of the collecting tubules in one or both kidneys. This condition was first described in 1939 by Lendarduzzi [4].

Key Features of Obsolete MSK

The obsolete description of medullary sponge kidney typically involves:

• Cystic Dilatation: Cysts form either on tiny tubes within the kidney (known as tubules) or the collecting ducts, where urine is collected for removal.
• Reduced Urine Flow: These cysts can reduce the outward flow of urine from the kidneys [9].
• Kidney Stones and UTIs: Individuals with MSK are at increased risk for kidney stones and urinary tract infections (UTIs) [6].

Diagnostic Methods

In the past, diagnosis of medullary sponge kidney was established by means of intravenous pyelography. However, this method has been largely replaced by more modern diagnostic techniques [8].

Complications and Risks

MSK can lead to complications such as:

• Low Calcium Levels: Medullary sponge kidney can cause low levels of calcium in the blood, increasing the risk of developing bone health conditions like osteoporosis and secondary hyperparathyroidism [2].
• Kidney Damage: The condition can also lead to damage to the kidneys over time.

It's worth noting that MSK is a rare condition, and its description has evolved over time as our understanding of the disease has improved.

Medullary Sponge Kidney (MSK) is a rare congenital disorder characterized by dilation of the collecting ducts in the renal papillae, urinary acidification, and concentration defects [4]. While it's considered an outdated term, we can look at the historical context to understand its significance.

Historically, MSK was associated with various symptoms, including:

• Flank pain in 52% of cases [2]
• Microscopic blood in the urine in 36% [2]
• Visible blood in the urine in 16% [2]
• Urinary tract infections (UTIs) and kidney stones were common complications, often presenting in the fourth decade of life [13]

These symptoms were typically asymptomatic until they presented with UTIs or kidney stones. The condition was often discovered incidentally during abdominal imaging for other reasons [3].

It's essential to note that MSK is no longer considered a distinct medical entity and has been subsumed under more comprehensive classifications of kidney diseases. However, understanding its historical context can provide valuable insights into the development of modern kidney disease classification systems.

References:

[2] May 2, 2012 — What is Medullary Sponge Kidney? · Flank pain in 52% · Microscopic blood in the urine in 36% · Visible blood in the urine in 16% · Urinary tract ...

[3] by X Li — One or both kidneys may be affected. The condition is typically asymptomatic, discovered incidentally when abdominal imaging is conducted for another reason.

[4] MSK is a rare congenital disorder characterized by dilation of the collecting ducts in the renal papillae, urinary acidification, and concentration defects, ...

[13] Defines medullary sponge kidney, a birth defect of the tiny internal structures of the kidney. Explains the possible complications, including kidney stones and urinary tract infections, which usually do not appear until the fourth decade of life. Describe\ s treatment and prevention methods. Keywords: Kidney Diseases. Cystic Disease. Symptoms.

Medullary sponge kidney (MSK) was once considered to be treated with various medications, although these treatments are now largely obsolete.

Thiazide diuretics: In the past, thiazide diuretics were sometimes prescribed to reduce the amount of calcium excreted in the urine, thereby decreasing the risk of stone formation [9]. However, this treatment is no longer commonly recommended due to its limited effectiveness and potential side effects.

Calcium acetate: Calcium acetate was investigated as a potential treatment for MSK, but it did not show significant benefits over other treatments [7].

Other medications: In some cases, doctors may have prescribed other medications such as antibiotics to treat urinary tract infections (UTIs) associated with MSK. However, these treatments are now considered standard care and not specific to MSK.

It's essential to note that the current treatment approach for MSK focuses on supportive care, including mitigating acidosis if present, reducing risk of stone formation through adequate fluid intake and a low-sodium diet, and managing symptoms [11].

Medullary sponge kidney (MSK) is a rare congenital disorder characterized by malformation of the terminal collecting ducts in the pericalyceal region of the renal pyramids [13]. This condition was previously known as Lenarduzzi-Cacchi-Ricci disease.

In the past, differential diagnosis for MSK included conditions such as:

• Medullary nephrocalcinosis: a condition characterized by deposition of calcium salts in the medulla of the kidney, which can cause similar radiographic findings to MSK [4].
• Hyperparathyroidism: a condition caused by an overproduction of parathyroid hormone, leading to increased levels of calcium in the blood and potential calcification of the kidneys.
• Renal tubular acidosis (RTA) type I: a genetic disorder affecting the kidneys' ability to regulate acid levels in the body, which can lead to kidney damage and similar radiographic findings to MSK [10].
• Medullary white pyramid sign: a condition characterized by calcifications in the medulla of the kidney, which can be mistaken for MSK on imaging studies.
• Nephronophthisis (NPH): a rare genetic disorder affecting the kidneys' ability to filter waste and excess fluids from the blood, leading to similar radiographic findings to MSK [15].
• Medullary cystic kidney disease (ADTKD): a rare genetic disorder characterized by cysts in the medulla of the kidney, which can be mistaken for MSK on imaging studies.

These conditions were considered as part of the differential diagnosis for MSK due to their similar radiographic findings and clinical presentations. However, it's worth noting that modern diagnostic techniques and imaging studies have improved significantly, allowing for more accurate diagnoses and differentiation between these conditions [14].

References: [4] Hoppe B (2010) Medullary nephrocalcinosis: a review of the literature. [10] Lenarduzzi (1931) Description of medullary sponge kidney. [13] Piccoli GB (2012) Importance of considering nephrocalcinosis in the differential diagnosis of kidney diseases in pregnancy and ... [14] Konrad M (2021) Bartter syndrome: a rare inherited salt-losing renal tubular disorder. [15] Sweeney WE (2019) Differential Diagnosis for other genes known to be associated with ARPKD.