No abstract available.
Protein-Losing Enteropathies*

No abstract available.
Protein-Losing Enteropathies

No abstract available.
Protein-Losing Enteropathies ; Retinal Detachment ; Retinaldehyde

As the operative mortality has diminished and the number of survivors has increased after Fontan procedure, morbidities related to the unnatural physiology of cavopulmonary flow have developed. One of the complications by the hemodynamic derangement after Fontan procedure is a protein-losing enteropathy. This is a rare but life-threatening complication after the Fontan operation. Treatment strategies are highly variable. But, reports on successful management are limited. We experienced three cases of protein-losing enteropathy after the Fontan operation. We report that the conversion to the total extracardiac or intracardiac cavopulmonary connection with fenestration is a satisfactory treatment modality for protein- losing enteropathy after the Fontan operation.
Fontan Procedure* ; Hemodynamics ; Humans ; Mortality ; Physiology ; Protein-Losing Enteropathies* ; Survivors

Systemic lupus erythematosus (SLE) is a multisystemic disease that can affect most organ system, although gastrointestinal (GI) manifestations are relatively uncommon. Protein losing enteropathy (PLE) is associated with several clinical disorders, but it is an unusual manifestation of SLE. Of over 20 reported cases of PLE associated with SLE, the pathogenesis of lupus associated PLE remains unclear. We describe a patient with edema, diarrhea, abdomianl pain, and hypoalbuminemia who had been diagnosed SLE. PLE was diagnosed by the method of alpha1-antitrypsin clearance in stool.
Diarrhea ; Edema ; Humans ; Hypoalbuminemia ; Lupus Erythematosus, Systemic* ; Protein-Losing Enteropathies*

Systemic lupus erythematosus(SLE) is the prototypic immune complex disease which involves various organs. Chronic interstitial cystitis and protein-losing enteropathy are uncommon manifestations of SLE and have good response to steroid. Only one case of SLE associated with chronic interstitial cystitis and protein-losing enteropathy has been reported which was resistant to steroid treatment but dramatically responded to cyclophosphamide. We describe a case of a female patient who developed systemic lupus erythematosus associated with chylothorax, interstitial cystitis and protein-losing enteropathy with literature review. This case was initially resistant to steroid but with the addition of cyclophosphamide, chylothorax, chronic interstitial cystitis and protein-losing enteropathy markedly improved. This is the first case of SLE that developed chylothorax as well as chronic interstitial cystitis and protein-losing enteropathy.
Chylothorax* ; Cyclophosphamide ; Cystitis, Interstitial* ; Female ; Humans ; Immune Complex Diseases ; Lupus Erythematosus, Systemic* ; Protein-Losing Enteropathies*

Protein-losing enteropathy (PLE) is a rare manifestation of systemic lupus erythematosus (SLE), and it is an uncommon cause of hypoalbuminemia without proteinuria. We describe a case of an 11 year-old girl who had diarrhea and periorbital edema as the initial symptoms of SLE. PLE was diagnosed by 24-hour stool alpha-1 antitrypsin clearance and 99mTc-human serum albumin scintigraphy.
Child ; Female ; Humans ; Lupus Erythematosus, Systemic/*complications ; Protein-Losing Enteropathies/*etiology

Constrictive pericarditis represents a rare cause of protein-losing enteropathy in children. Reported is an 11-year-old girl with protein-losing enteropathy (PLE) as the principal manifestations of constrictive pericarditis. After total pericardiectomy, symptoms and signs of PLE disappeared. Doppler echocardiography including tissue Doppler imaging is a useful noninvasive initial diagnostic tool for differential diagnosis of diastolic heart failure.
Child ; Diagnosis, Differential ; Echocardiography, Doppler ; Female ; Heart Failure, Diastolic ; Humans ; Pericardiectomy ; Pericarditis, Constrictive* ; Protein-Losing Enteropathies*

Primary intestinal lymphangiectasia is a congenital lymphatic disorder characterized by dilated intestinal lacteals resulting in lymph leakage into the small bowel lumen and responsible for protein losing enteropathy. As a result, generalized edema, hypoalbuminemia, and lymphocytopenia are clinically manifested. We could not find the reason by several examinations. Therefore, we performed double balloon enteroscopy (DBE), and intestnal lymphangiectasia was diagnosed histologically by a biopsy. DBE is a safe and effective method to diagnose small bowel lymphangiectasia. We report a case of primary intestinal lymphangiectasia, which occurred in a 54-year-old male patient with generalized edema and ascites.
Ascites ; Biopsy ; Double-Balloon Enteroscopy ; Edema ; Humans ; Hypoalbuminemia ; Lymphopenia ; Male ; Protein-Losing Enteropathies

Primary intestinal lymphangiectasia is a congenital lymphatic disorder in which intestinal lymphatic channels are dilated and ruptured resulting in loss of protein, lipid, and lymphocyte into the intestine or peritoneum. As a result, hypoalbuminemia, generalized edema, diarrhea are clinically manifested. We report a case of primary intestinal lymphangiectasia with generalized edema which occurred in a 7-year old boy who was treated with lipid restriction diet with medium chain triglyceride oil supplement.
Child ; Diarrhea ; Diet ; Edema ; Humans ; Hypoalbuminemia ; Intestines ; Lymphocytes ; Male ; Peritoneum ; Protein-Losing Enteropathies ; Triglycerides