About waldmann disease

What is waldmann disease?

Primary intestinal lymphangiectasia (PIL) is a rare digestive disorder characterized by abnormally enlarged (dilatated) lymph vessels supplying the lining of the small intestine. The main symptoms are swelling of the limbs and abdominal discomfort. The disorder is usually diagnosed before three years of age but is sometimes diagnosed later in life.



What are the symptoms for waldmann disease?

Protein-losing enteropathy symptom was found in the waldmann disease condition

The most obvious sign of the disorder is moderate to severe swelling in the lower limbs, eventually face, abdomen and external genitalia due to fluid retention (edema). Fluid is retained because the blood protein (albumin) levels are low. Lymphedema may also be associated and not easy to differentiate from edema.

Abdominal pain and/or Nausea, Vomiting and Diarrhea may also be present. Affected individuals may experience Fatigue, Weight loss, and an inability to gain weight in childhood. The blood lymphocyte count is usually low as are blood protein (albumin, globulins because protein in the lymph leaks into the intestine and the feces called exudative enteropathy) and blood cholesterol levels (because cholesterol from food is not properly absorbed).

Swelling of the membrane surrounding the heart (pericarditis) and fluid in the chest (pleural effusion) or ascites (abdominal effusion) can occur. Extreme generalized swelling of the body (anasarca) can be a rare life threatening complication in children



What are the causes for waldmann disease?

The cause of PIL is unknown. Multiple affected family members have been reported rarely.



What are the treatments for waldmann disease?

Treatment of PIL may include a strictly low-fat long-term diet supplemented by medium-chain triglycerides to supply essential fatty acids and nutrients associated with fat-soluble vitamin such as vitamin D. The need for dietary control appears to be permanent, because clinical and biochemical findings reappear after low-fat diet interruption. The administration of water pills (diuretics) may sometimes be helpful. Albumin infusion is sometimes proposed in patients with important serous effusion or uncomfortable lower limb edema. Very occasionally surgical removal of the diseased portion of the intestine may be beneficial if the damage is limited to a local area. In some patients, octreotide is prescribed in addition to dietary modifications. Compression stocking can be used to stabilize in associated lower limb lymphedema.



What are the risk factors for waldmann disease?

Waldmann disease, also known as primary intestinal lymphangiectasia (PIL), is typically discovered before the age of three but can sometimes be seen in older people with very few symptoms. The primary sign of protein-losing enteropathy linked to hypoalbuminemia is mostly bilateral lower leg edema.

Risk Factors

  • A relatively uncommon disorder called the yellow nail syndrome, which may be related to PIL. Dystrophic yellow nails on the hands with ridging and lunula loss are linked to pleural effusions and lymphedema.
  • Five disorders, including von Recklinghausen or Noonan, Klippel-Trenaunay, and Hennekam, are linked to intestinal lymphangiectasia.
  • Osteomalacia brought on by a lack of vitamin D
  • Multiple, non-specific small intestinal ulcers can cause persistent blood loss, which leads to iron deficiency and anemia.
  • Nocturnal migratory erythema
  • The skin of the lower limb, the perineum, or the external genitalia can also be affected by chyle backflow.
  • Localized edema that causes intestinal wall thickening and lumen reduction in the small intestine, resulting in mechanical ileus.
  • Loss of lymphatic fluid into the digestive system, enteropathy that causes protein loss, a lack of albumin in the blood, decreased antibody levels, and immunodeficiency


Conditions
Cyanosis,Apnea hepatomegaly
Drugs
Antibiotics,Allopurinol,ACE inhibitors,Angiotensin-converting enzyme (ACE),Allopurinol
Symptoms
Constant hunger,Frequent need to eat often,Easy bruising,Nosebleeds,Fatigue,Irritation,Thin chest and limbs,Puffy cheeks



Is there a cure/medications for waldmann disease?

Waldmann disease, also known as primary intestinal lymphangiectasia (PIL), is typically discovered before the age of three but can sometimes be seen in older people with very few symptoms. The primary sign of protein-losing enteropathy linked to hypoalbuminemia is mostly bilateral lower leg edema.

Cure

  • The mainstay of PIL medical care is a rigorously low-fat diet along with medium-chain triglyceride supplementation. The lack of fat in the diet stops the intestinal lymphatic arteries' chyle engorgement, avoiding their rupture and subsequent lymph loss. To avoid lacteal overload, medium-chain triglycerides are absorbed straight into the portal venous circulation.
  • Some PIL patients have responded well to octreotide treatment. In the extremely uncommon occurrences of segmental and localized intestinal lymphangiectasia, surgical small-bowel resection is beneficial.
  • Due to the persistence of clinical and biochemical data following the cessation of the low-fat diet, it appears that dietary control is always necessary.
  • A symptomatic treatment called albumin infusion is recommended for patients who have significant serous effusions or unpleasant lower limb edema.
  • The effectiveness of repeated albumin infusions to reduce edema is sporadic.
  • Vitamin D in particular needs to be supplemented often with fat-soluble vitamins.
  • It is advised to conduct a thorough clinical and biochemical follow-up.


Conditions
Cyanosis,Apnea hepatomegaly
Drugs
Antibiotics,Allopurinol,ACE inhibitors,Angiotensin-converting enzyme (ACE),Allopurinol
Symptoms
Constant hunger,Frequent need to eat often,Easy bruising,Nosebleeds,Fatigue,Irritation,Thin chest and limbs,Puffy cheeks



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