Corresponding author. Corresponding author: Dr. Tel:Fax:ca. This article has been cited by other articles in PMC.
Abstract The ketogenic diet is a highly effective treatment for the patients with intractable epilepsy, however, the dietary plan therapy can often be discontinued by complications. Protein-losing enteropathy is a reported serious complication of the ketogenic diet rarely. We present a month-old Down syndrome baby with protein-losing enteropathy during the ketogenic diet as a treatment for West syndrome. He suffered from diarrhea, general edema and hypoalbuminemia which were not managed by conservative care for over 1 month.
Esophagogastroduodenoscopy and stool alpha-1 antitrypsin indicated protein-losing enteropathy. Related symptoms were relieved after cessation of the ketogenic diet. Unexplained hypoalbuminemia combined with edema and diarrhea during ketogenic suggests the likelihood of protein-losing enteropathy, and proper evaluation is preferred to be able to expeditiously identify it and also to act accordingly.
While some unwanted effects are benign and treatable, others are more problematic and will interrupt the dietary plan therapy itself rarely. He presented epileptic spasms because the age of 5 months and was identified as having WS.
He began to be treated by multiple antiepileptic drugs AEDsincluding vigabatrin, clobazam, valproic acid and oral steroids, but his epileptic seizures, developmental regression, and hypsarrhythmia on electroencephalogram persisted. KD with ratio of fat to non-fat was started by milk formula, and multiple AEDs vigabatrin, clobazam, and deflazacort were maintained at the same doses. Week of KD Vomiting and diarrhea were developed after 1st. The albumin level that was 3.
He was treated by intravenous albumin and intermittent parenteral nutritional support. Lipid and non-fat ratio of KD deescalated from on the 28th days to at least one 1. Because he previously not improved after four weeks, we changed KD to medium chain triglyceride formula on the 61st days of the dietary plan. Laboratory tests, stomach ultrasonography, esophagogastroduodenoscopy EGDcolonoscopy, and alpha-1 antitrypsin A1AT in stool, were done.
There is no proteinuria in urine dipstick test, and blood test revealed blood urea nitrogen 9. Echocardiography revealed normal heart function with no structural abnormalities. There have been no pathogens in stool bacterial culture, but norovirus infection was detected in stool virus study. Abdominal ultrasonography revealed normal liver and gallbladder grossly, and preserved corticomedullary differentiation in both kidneys, and there is no skin damage that could produce protein loss.
EGD revealed edematous mucosa in the duodenum, and the biopsy results revealed lymphatic ectasia in the lamina propria; intestinal lymphangiectasia was diagnosed Fig thus. A1AT of the stool was These findings were concordant with interstitial lymphangiectasia as a presentation of PLE.