Introduction. Celiac disease, or gluten-sensitive enteropathy, can be defined as a persistent intolerance of wheat gliadins and other cereal prolamines in the small intestinal mucosa of genetically susceptible individuals. The clinical picture of the disease can often be misleading because it varies greatly from patient to patient, resulting in delayed diagnosis.To analyze the clinical case of a child with celiac disease and acquired ichthyosis.Results. The disease, until a final diagnosis was established, had a severe course due to gastrointestinal and dermatological disorders. From the age of 1.5 years, the child had frequent diarrhea, bloating, which is why she was repeatedly hospitalized in the hospital at the place of residence. However, there was no effect from the ongoing therapeutic measures, and other symptoms such as vomiting, peripheral edema, deficiency of height and weight, and severe peeling of the skin joined in. The diagnosis was finally confirmed at the age of 2.5 years after the test for antibodies to tissue transglutaminase IgA (fifty-fold excess relative to the norm). A genetic study revealed alleles of genes responsible for predisposition to celiac disease. The results of a biopsy of the mucous membrane of the duodenum had signs of atrophy, lymphoid infiltration, corresponding to a lesion of the small intestine according to the classification Marsh III. Microscopic examination of the skin – hyperkeratosis with a decrease in the granular layer. On the basis of the obtained data, the diagnosis was made: Celiac disease, active phase, severe course, complicated by proteinenergy insufficiency severe degree, exudative enteropathy syndrome, 2 degree anemia, concomitant diagnosis: acquired ichthyosis. The girl was prescribed a gluten-free diet, and symptomatic drug therapy was carried out. In dynamics, the condition has improved. After 6 months, at the second visit, gastrointestinal and skin symptoms were absent, physical development was age-appropriate.Conclusions. The classic form of celiac disease usually manifests itself with several major symptoms, such as diarrhea, abdominal pain, weight loss, and nutritional deficiencies. In this article we wanted to talk about a rare combination of celiac disease with ichthyosis, therefore, practitioners should be wary of a combination of skin and gastrointestinal symptoms.
The aim of the study – to analyze the clinical case of a child with distal intestinal obstruction syndrome in cystic fibrosis. A 9.5 year old patient was admitted to the Gastroenterology Department of the Republican Specialized Scientific and Practical Center of Pediatrics with complaints of an increase in abdominal circumference, increased peristalsis, weakness, lethargy, swelling in the limbs, loose stools, and weight loss. From the anamnesis: from the age of 6 years it was observed periodic vomiting, not associated with food intake. At the age of 8 years, the condition worsened, bloating joined the vomiting. At the place of residence, the diagnosis was made “Acute intestinal obstruction. Volvulus of the sigmoid colon”. Over the next 6 months, the condition was relatively satisfactory, and then stool disorders began – 3–4 times a day and bloating. Considering the presence of steatorrhea, changes in the pancreas on ultrasound and MSCT, the child was referred to a geneticist, the result of a sweat test was 86.0 mEq/l. The diagnosis was made: Cystic fibrosis, mixed form. Syndrome of distal intestinal obstruction. Partial intestinal obstruction. Severe PEI, celiac disease and HIV were excluded. On the background of the therapy, the child’s condition improved relatively. Within a month after discharge, the condition was satisfactory, and then worsened again. After 3 months, the girl was re-hospitalized with the same complaints. After consulting the surgeons, recommendations were given to perform the operation due to the appearance of signs of partial lower intestinal obstruction, but the relatives signed a voluntary refusal of the operation, the child was taken home against receipt. The girl was discharged with the final diagnosis: Cystic fibrosis, mixed form, severe course. Syndrome of distal intestinal obstruction. Partial lower intestinal obstruction. Syndrome of exudative enteropathy, severe PEI. The girl died 2 weeks after discharge. Distal intestinal obstruction syndrome (DIOS) may be misunderstood by those who are not familiar with cystic fibrosis. Pediatric gastroenterologists, surgeons should be alert when this condition is detected in combination with other symptoms indicating cystic fibrosis.
amounted to 6.9+2.3 pg/ml at a normal rate -4.3+0.7 pg/ml (p<0.05), but the highest increase was observed in IL1b (10.4 +3.9 pg/ml and 2.05+0.03 pg/ml, respectively, p<0.05), which reflects the increased activity of macrophages involved in maintaining the inflammatory process. Thus, the data obtained demonstrate that the intestinal microvascular endothelium of the intestinal mucosa can respond to locally generated cytokines and produce strong pro-inflammatory mediators. Conclusion Thus, there is clear evidence of intestinal inflammation in children with a mixed form of cystic fibrosis, which can negatively affect the patient's nutritional status, which in turn adversely affects pulmonary function and survival. The data obtained are the basis for optimizing therapy aimed at improving bowel function.
Figure 1B Trichobezoar measuring approximately 30 cm, extending from the stomach to the duodenum Results Trichobezoar is a collection of hair in the gastrointestinal tract, usually, the stomach, caused by chronic hair pulling and ingestion. This condition is called trichotillomania, a mental disorder that typically strikes during adolescence and has a chronic course. Patients who suffer from trichotillomania usually have patchy hair loss, a sign that was absent in our patients. In addition, the patient in our case presented with symptoms that resemble other conditions like intestinal lymphoma and inflammatory bowel disease. Radiological imaging can be diagnostic in 97% of the case; however, in other cases, further investigations and surgical exploration might be warranted. The clinical course in our case was severe indicating the devastating effect trichobezoar can lead to. Conclusion Diagnosis of intestinal obstruction due to trichobezoar may not be straightforward especially in patients who have not been diagnosed with mental diseases or lack the clues on physical examination. Although it is an uncommon cause of intestinal obstruction, it should be included in the differential diagnosis when possible.
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