CELIAC DISEASE (Gluten Hypersensitivity Gluten Allergy) Causes Symptoms Risk factors Treatment Diagnosis Management and Complications. Celiac Disease in Children.

CELIAC DISEASE (Gluten Hypersensitivity, Gluten Allergy)

Celiac Disease is also called Gluten-sensitive Enteropathy and Celiac Sprue. It is an inflammatory disease that is caused by an immune reaction to eating gluten. Gluten is a protein found in wheat and similar protein is present in rye, barley, and a smaller amount in oats. In Celiac disease, eating gluten causes an immune reaction and with time repeated inflammation results in damage to the small intestine that ultimately causes malabsorption (inability to absorb nutrients in the small intestine). Damage to intestinal villi (brush-like extensions) also causes diarrhea, fatigue, weight loss, anemia, bloating, and many other complications. In children. Malabsorption can causes growth retardation and affects development. Celiac disease has various presentations, with some cases diagnosed cases and some may be asymptomatic. Asymptomatic cases can be SILENT (Undiagnosed) and LATENT (Develop symptoms later in life)

CAUSES OF CELIAC DISEASE

There are small hair-like projections (called villi) that line the cells of the small intestine. Villi absorb nutrients such as vitamins and minerals from the digested food. If villi get damaged due to celiac disease, the body can not get the required nutrients, no matter how much the person eats and it results in malabsorption.

There is no known exact cause of celiac disease, however

• Susceptible genes combined with eating food containing gluten can trigger Celiac disease.
• Silent cases can get active after surgery, pregnancy and childbirth, infections of the gut, and some emotional stress.

Infant-feeding practices, gut infections, and loss of gut bacteria can also contribute. RISK FACTORS OF CELIAC DISEASE

• Family member with Celiac disease
• Monozygotic (Maternal Twins)
• A person having Human Leukocyte Antigen (HLA)-DQ2/DQ8
• Celiac Disease is also associated with other HLA-linked autoimmune disorders such as Type 1 Diabetes Mellitus, Thyroid Disease, Downs syndrome, Addison Disease, Sjogren syndrome, Pernicious Anemia, Sarcoidosis, Dermatitis Herpetiformis, Myasthenia Gravis, Ulcerative jejunitis, Microscopic colitis, Enteropathy associated T-Cell Lymphoma, Small bowel carcinoma, Squamous carcinoma of the esophagus and Neurological Complications (Encephalopathy, Cerebellar Atrophy, Epilepsy, Peripheral Neuropathy)

SYMPTOMS AND CLINICAL FEATURES OF CELIAC DISEASE

The signs and symptoms of celiac disease vary greatly in children and adults. 

Digestive signs and symptoms in adults and children - Diarrhea, fatigue, weight loss, bloating, gas, abdominal pain, nausea, vomiting, and constipation. Digestive signs and symptoms are more severe in children than in adults.

Signs and symptoms unrelated to the digestive system - Anemia (due to iron deficiency, decrease bone density (osteoporosis) or softening of the bone (osteomalacia), itchy, blistery skin rash (dermatitis herpetiformis), mouth ulcers, headaches, joint pain, and fatigue. There can be nervous system injury, the symptoms of which include numbness and tingling in the feet and hands and cognitive impairment.

Signs and symptoms due to malnutrition - Inability to absorb nutrients might result in failure to thrive for infants, weight loss, anemia, irritability, short stature, delayed puberty, damage to the enamel of teeth, and neurological symptoms, including learning disabilities, attention-deficit/hyperactivity disorder (ADHD), headaches, seizure and lack of muscle coordination.

DIAGNOSIS OF CELIAC DISEASE

Many people with celiac disease are unaware that they have it, following tests can help diagnose it

• Duodenal Biopsy – Endoscopic small bowel biopsy is the gold standard investigation. More than four biopsies from the second part of the duodenum and one from the duodenal bulb is necessary as damage and histological change can be patchy, involving some part and sparing other.
• Serology testing –to measure the levels of antibodies in the blood. Elevated levels of anti-endomysial antibodies against auto-antigen tTG (tissue transglutaminase) indicate an immune reaction to gluten.
• Genetic testing for human leukocyte antigens (HLA-DQ2 and HLA-DQ8) can be done to rule out the disease.
• Endoscopy – upper GIT endoscopy is done to view the small intestine and a tissue sample (biopsy) is taken to analyze the damage to the villi.
• Capsule endoscopy – A vitamin-sized capsule with a camera travels through the digestive tract which takes thousands of pictures that are transmitted to a recorder.
• Measurement of bone density – to look for osteoporosis in older and post-menopausal patients.

MANAGEMENT AND TREATMENT OF CELIAC DISEASE

The aims are to correct existing nutrient deficiencies and heal the inflamed and damaged intestines for which a strict gluten-free diet is the only way. 

• Follow a Gluten-free Diet Plan-  Besides wheat, the foods that contain gluten are barley, rye, semolina, graham flour, malt, bulgur, durum, and farina and these should also be avoided. Oats may be reintroduced safely in patients after 6 to 12 months of restriction. 

Gluten can also be present in medications, vitamin and mineral supplements, herbal and nutritional supplements, modified food starch, preservatives and food stabilizers, toothpaste, mouthwash, glue, play dough, and lipstick products. Removing gluten from the diet can help in reducing inflammation and ultimately heals the damaged lining of the small intestine.

• Diet Counselling - Frequent diet counseling of patients is required to make sure the gluten-free diet is being observed. 

A dietitian can help you plan a healthy gluten-free diet and booklets produced by celiac societies containing diet sheets and healthy gluten-free recipes for the use of gluten-free flour are of great help. You can always look for these booklets.

• Correct mineral and nutrient deficiencies – if anemia or nutritional deficiencies are severe, vitamins and supplements such as iron, folate, copper, zinc, vitamin B-12, vitamin D, and vitamin K are usually given in the form of pills form but injections can also be given if your digestive tract has trouble absorbing.
• Follow-up – A follow-up at regular intervals after starting a gluten-free diet for assessment of symptoms, weight, and nutritional status is necessary. The doctor also measures the tTG or anti-endomysial antibodies.
• Biopsy – When symptoms do not improve or when anti-bodies levels remain high even after following a gluten-free diet.
• Medications to control inflammation – Glucocorticoids or immunosuppressive drugs are given in patients who fail to respond to treatment.
• Other medical conditions such as Ulcerative Jejunitis or Enteropathy Associated T-Cell Intestinal Lymphoma should be sought out.

COMPLICATIONS OF CELIAC DISEASE

Untreated Celiac Disease can cause

• Malnutrition. Celiac Disease damages the small intestine which can lead to anemia and weight loss and slow growth and short stature in children.
• Cancer – There are two times increased risk · of developing cancer, especially Enteropathy Associated T-Cell Intestinal Lymphoma, Squamous cell cancer of the esophagus, and small bowel cancer in patients of celiac disease who have not maintained a gluten-free diet.
• Ulcerative Jejuno-ileitis – There is fever, pain, obstruction, or perforation, and diagnosis is made by barium studies or enteroscopy but laparotomy and full-thickness biopsy are required. Treatment of it is difficult, glucocorticoid, surgical resection, and parenteral nutrition are required.
• Bone weakening. In people with poorly controlled Celiac Disease, there are chances of bone softening (osteomalacia or rickets) in children and decrease bone density (osteoporosis) in adults due to malabsorption of calcium and vitamin D.
• Infertility and miscarriage – Malabsorption of nutrients which can cause reproductive issues.
• Lactose intolerance- Damage to the lining of the small intestine may cause abdominal pain and diarrhea after consuming dairy products that contain lactose.
• Nervous system problems – There can be problems with the nervous system such as seizures or peripheral neuropathy (disease of the nerves of the hands and feet)