Intestinal Lymphangiectasia
Description
◉ Patient Information
• Age/Sex: guy, 27 years old
• Yeungnam University Hospital is the hospital.
• Entry: December 1991
◉ Chief complaints:
• Having diarrhea
• Lower extremity edema that worsened over eight months
◉ Past Health Conditions
• Eight years earlier:
• had abdominal distension and lower extremity edema.
• There was no therapy; the symptoms went away on their own.
• Four years earlier:
• Leg edema that comes and goes, linked to diarrhea.
• identified by a local doctor as having hypoalbuminemia.Abdominal pain, weight loss, vomiting, fever, or urinary symptoms are not present.
• Lifestyle: abstinence from alcohol and tobacco.
◉ Family background: Not noteworthy.
• There is no known allergy
◉ Laboratory Investigations
• White blood cell, platelet, and hemoglobin counts were all within normal ranges.
• The ESR was somewhat elevated at 25 mm/h.
• Tests for liver, kidney, and serum electrolytes were all normal.
• Kidney disease was ruled out by the normal urinalysis.
• The serum total protein level was extremely low at 3.9 g/dl.
• Serum albumin was significantly lower at 1.8 g/dl.
• Both cholesterol and calcium levels dropped.
• ANA, LE cell, and RA factor autoimmune testing came out negative.
• Immunoglobulin levels revealed a slightly low IgA and IgM and a decreased IgG value (412 mg/dl).
• These findings supported hypoproteinemia and hypoalbuminemia in the absence of renal loss or inflammation.
◉ Radiological and Endoscopic Findings
• The barium enema and chest X-ray revealed no anomalies.
• The ileum and jejunum showed irregular segmental luminal narrowing in the small bowel series, indicating abnormalities of the mucosa.
• the esophagus, stomach, and colon were all normal, but a small intestinal endoscopy revealed several white granular spots dispersed throughout the jejunum and terminal ileum's mucosa.
• A biopsy of the afflicted areas showed significantly dilated submucosal lymphatics devoid of inflammation, which is a hallmark of intestinal lymphangiectasia.
◉ Results of Scintigraphy
• To identify protein loss, 99mTechnetium-labeled human serum albumin (99mTc-HSA) was administered intravenously.
• At six hours, radioactivity was detected in the transverse colon and right lower quadrant; at twenty-four hours, it was detected in the descending and sigmoid colon.
• diagnosis of lymphatic leakage-induced protein-losing enteropathy.
◉ Diagnostic Features
• Serum albumin and total protein levels are noticeably low.
• Other causes of protein loss are eliminated by normal liver and kidney function.
• White granular mucosa in the ileum and jejunum is visible endoscopically.
• Verification of dilated intestinal lymphatics by histology.
• evidence of intestinal protein loss using scintigraphy.
• excluding secondary causes.
Protocol
◉ Treatment and Management
• There isn't a specific medication that can cure PIL.
• The foundation of treatment is dietary management.
• To make up for protein loss, a high-protein diet is prescribed.
• Because medium-chain triglycerides (MCTs) are absorbed straight into the portal venous system, avoiding intestinal lymphatics and halting additional lymphatic engorgement, a fat-restricted diet including MCTs is advised.
• If the disease is restricted to a small bowel segment, surgical resection can be an option.
• Although antiplasmin therapy is not frequently utilized, some reports claim improvement with it.
• In this instance, nutritional therapy quickly eliminated the patient's edema and diarrhea, and within four weeks, serum albumin returned to normal.
◉ Issues in the PIL
• severe hypoproteinemia-induced lower extremity edema.
• sporadic diarrhea that exacerbates electrolyte and fluid imbalance.
• risk of malnutrition and chronic hypoalbuminemia.
• Possible lymphocytopenia and related risk of infection.
◉ Strategies for Management
• Since fat restriction and protein replenishment prevent further protein loss, the main management strategy is a high-protein, low-fat diet.
• By being taken straight into the portal system, medium-chain triglycerides (MCTs) assist supply calories without aggravating lymphatic engorgement.
• To evaluate the effectiveness of treatment, blood protein and albumin levels are periodically measured in addition to symptoms are tracked.
• In rare, isolated situations, the damaged intestinal portions may need to be surgically removed.
Although it is not a standard treatment, antiplasmin therapy has demonstrated advantages in a few documented cases.
◉ Detailed Nursing Interventions
• To monitor fluid shifts and therapy response, measure and record abdominal circumference, daily weight, and edema symptoms.
• Keep a careful eye on input and output, especially during acute episodes of diarrhea, and be alert for symptoms of fluid overload or dehydration.
• Help carry out the recommended diet plan, offer dietary guidance, and make sure that the high-protein, low-fat, MCT-supplemented diet is followed.
• Since lymphocytopenia increases the risk of infection, maintain tight asepsis to prevent infection.
Regularly check serum protein, albumin, and electrolyte levels, and notify the medical staff of any notable abnormalities.
• Inform patients on the value of routine follow-ups in order to monitor and modify treatment plans, especially those including diet.
• Inform the patient and their family about the purpose and expectations of any possible diagnostic procedures
Notes
FOR MORE DETAILS VISIT: https://pmc.ncbi.nlm.nih.gov/articles/PMC4532082/?utm_source=chatgpt.com
