Collapse Section. Congenital sucrase-isomaltase deficiency is a disorder that affects a person's ability to digest certain sugars. People with this condition cannot break down the sugars sucrose and maltose.
The disease is an autoimmune disorder that is triggered by hypersensitivity to ingested gliadins from wheat and other cereals [18]. The frequency of this disease can be up to 3 % in the different populations, but this ratio was detected to be as high as 11 % among patients with type 1 diabetes mellitus [19].
The carbon-13 breath test is thought to directly measure sucrase activity and is the most definitive method to aid in diagnosing CSID that does not require an invasive procedure to obtain tissue samples from your intestine.
Chronic, watery diarrhea and failure to thrive are the most common symptoms in infants and toddlers. Other symptoms include abdominal distention (swelling), gassiness, colic, irritability, scratched and reddened buttocks, severe diaper rash due to acidic diarrhea, indigestion (dyspepsia), and vomiting.
E74. 31 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
Congenital sucrase-isomaltase deficiency (CSID) is a genetic disorder that occurs when the body cannot produce the sucrase-isomaltase enzyme necessary for the proper metabolism of the sugar in fruits (sucrose) and the sugar in grains (maltose).
Cigna covers sacrosidase products (Sucraid®) as medically necessary when the following criteria are met for FDA Indications or Other Uses with Supportive Evidence: Prior Authorization is recommended for prescription benefit coverage of Sucraid.
Treatment of CSID focuses on dietary management through a low-sucrose or sucrose-free diet. In addition, a low-starch or starch-free diet is advised in some cases, especially in the first few years of life.
Secondary or acquired sucrase-isomaltase deficiency Clinically, the reduced enzymatic activity and villous atrophy in the small intestine are associated with maldigestive and malabsorptive diarrhea [20].
Proper sucrose digestion requires production of new sucrase-isomaltase enzymes each time food is consumed. Those with CSID lack the ability to produce sucrase-isomaltase, so CSID is a chronic disorder that will never be outgrown.
Increased fermentation, in turn, leads to more diarrhea and rapid small-bowel transit, which can result in malabsorption of all nutrients. Overall, the reduced absorption of essential nutrients can lead to malnutrition and failure to thrive in children, or low body weight in adults.
The word congenital means present at birth, and most congenital disorders are diagnosed when one is an infant or in early childhood. However, many patients with Sucrose Intolerance due to CSID are being diagnosed later in life, when they are in their teens or even when they are adults.
Fructose malabsorption, formerly named "dietary fructose intolerance" (DFI), is a digestive disorder in which absorption of fructose is impaired by deficient fructose carriers in the small intestine's enterocytes. This results in an increased concentration of fructose in the entire intestine.
This is the official approximate match mapping between ICD9 and ICD10, as provided by the General Equivalency mapping crosswalk. This means that while there is no exact mapping between this ICD10 code E74.31 and a single ICD9 code, 271.3 is an approximate match for comparison and conversion purposes.