{"id":28702,"date":"2024-04-11T15:43:23","date_gmt":"2024-04-11T19:43:23","guid":{"rendered":"https:\/\/nephcure.org\/?page_id=28702"},"modified":"2025-12-11T08:15:09","modified_gmt":"2025-12-11T13:15:09","slug":"sindrome-nefrotico-infantil-congenito-cns-ins","status":"publish","type":"page","link":"https:\/\/nephcure.org\/es\/intro-to-rkd\/types-of-rkd\/congenital-infantile-nephrotic-syndrome-cns-ins\/","title":{"rendered":"S\u00edndrome nefr\u00f3tico cong\u00e9nito e infantil (SNC e INS)"},"content":{"rendered":"
\n
Home<\/a><\/span> \/ Intro to RKD<\/a><\/span> \/ Types of RKD<\/a><\/span> \/ Congenital & Infantile Nephrotic Syndrome (CNS & INS)<\/span><\/span><\/div>\n\n
\n\n

Congenital & Infantile Nephrotic Syndrome<\/strong> (CNS & INS)<\/h1>\n\n\n\n

Congenital Nephrotic Syndrome (CNS)<\/strong> is a rare kidney disorder that begins at birth or within the first 3 months of life.  <\/p>\n\n\n\n

Infantile Nephrotic Syndrome (INS) <\/strong>is also a rare kidney disorder that occurs between 3 months and 1 year of life.<\/p>\n\n<\/div>\n\n

\n
\n
<\/div><\/div>\n\n
\n\n

Who Gets CNS or INS<\/h3>\n\n\n\n

CNS and INS are caused by genetic mutations. NPHS1 is also known as the Finnish-type of CNS, and most commonly found in people with Finnish ancestry.  Non-Finnish individuals often have NPHS2 mutations. But some causes of CNS\/INS are not genetic. <\/p>\n\n\n\n

Other non-genetic causes include infections such as cytomegalovirus, congenital syphilis, and congenital toxoplasmosis <\/p>\n\n<\/div><\/div>\n<\/div>\n<\/div>\n<\/section>\n\n

\n\n
\"\"<\/figure>\n\n\n\n

CNS & INS Facts<\/h2>\n\n\n\n

In most cases with Congenital and Infantile Nephrotic Syndrome patients, biopsy findings usually indicate FSGS. The majority of CNS mutations (NPHS1 or NPHS2) are autosomal recessive diseases, meaning both parents are carriers of CNS and the possibility of having an affected child is 25% (or 1 in 4 children will have the disease).<\/p>\n\n\n

\n
\n\n

80% of CNS Cases<\/h4>\n\n\n\n

are caused by in four different gene mutations: NPHS1 and NPHS2, LAMB2, WT1, or PLCE1<\/p>\n\n<\/div><\/div>\n\n

\n\n

Infants with CNS<\/h4>\n\n\n\n

may have failure to thrive, frequent life-threatening infections and be at risk for abnormal blood clotting <\/p>\n\n<\/div><\/div>\n\n

\n\n

2\/3 of INS Cases<\/h4>\n\n\n\n

are explained in four gene mutations: NPHS1, NPHS2, LAMB2, or WT1<\/p>\n\n<\/div><\/div>\n\n

\n\n

Many CNS Patients<\/h4>\n\n\n\n

develop end-stage kidney disease between ages 2 and 8, and require dialysis and transplant<\/p>\n\n<\/div><\/div>\n<\/div>\n<\/div>\n\n

\n
\n
\n
\n\n
\"\"<\/figure>\n\n<\/div><\/div>\n\n
\n\n
\"\"<\/figure>\n\n\n\n

Signs and Symptoms<\/h3>\n\n\n
<\/div>\n\n\n

These signs begin early in life, presenting a challenge for infants and young children.<\/p>\n\n\n\n

Common symptoms include:<\/strong><\/p>\n\n\n\n