Pediatrics - Gastroenterology(74) Flashcards

1
Q

PED - 7.1
Zinc deficiency can occur in all undermentioned disorders, except:
A) Acrodermatitis enteropathica
B) Hirschsprung disease
C) Coeliac disease
D) Phenylketonuria
E) Kwashiorkor

A

ANSWER
B) Hirschsprung disease

EXPLANATION
Hirschsprung disease is caused by the total or partial shortage, or sometimes hypoplasia of the vegetative ganglion cells of large bowel which lead to strictures in the involved part, explaning the clinical symptoms, surgical intervention is the necessary solution. Acrodermatitis enteropathica is a specific malabsorption of zinc, in coeliac disease the subtotal atrophy caused by gluten leading to malabsorption including also the trace elements. Phenylketonuria is a defect of pehylalanin-hydroxalase enzyme which make necessary low phenylalanin containing diet. Diet which is not properly balanced may cause zinc deficiency. Kawashiorkor is a severe protein-energy malnutrition with oedema and may lead to deficiency of trace elements.

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2
Q

PED - 7.2
It is true for children with encopresis all undermentioned statements, except:
A) Its prevalence in average children’s population is 1-3%
B) Among children with psychiatric disturbances the prevalence is 5-7%
C) Its pathological base is the gluten sensitivity
D) Occurance is more frequent during day than night
E) Rectal stool impactation and constipation occur very frequently

A

ANSWER
C) Its pathological base is the gluten sensitivity

EXPLANATION
Gliadin sensitivity provoke the pathological lesions in coeliac disease and dermatitis herpetiformis Duhring. Encopresis is pathological over 4 years of age, its form with retention is caused by impactation of stool in the rectum. In the non-retention form the proper habit of defecation is late or it is happened not in the right time and place. This latter is caused frequently by psychiatric disorders.

Encopresis or fecal incontinence

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3
Q

PED - 7.3
All the undermentioned ones may be the causes of constipation, except:
A) Psychological problem
B) Inaccurate diet
C) Megacolon congenitum
D) Overfunction of nervus vagus
E) Hypothyreosis
F) Insufficiency of abdominal muscle
G) Gastrointestinal allergy

A

ANSWER
D) Overfunction of nervus vagus

EXPLANATION
Constipation is symtoms developing from the distrubance of storage, transport or emptiing ability of the large bowel. As a consequence the number of stools less than three times weekly. Its water content decreased (less than 60%) which explain the hardness of the stool. Constipation is caused organic, but most frequently from functional causes. Low fiber containing diet, neurogenic cause, as Hirschsprung disease, psychogenic problems, the decrease of stimulus for defecation, muscle insufficiency, metabolic and endocrine disorders (such as hypercalcemia, or hypothyreodism) may elicit constipation. Constipation may occur in infants with cow’s milk allergy probably caused by rectal pain. Overfunction of nervus vagus increase the parasympathic effect on the colon increasing its motility, therefore it cause diarrhea.

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4
Q

PED - 7.4
All of the undermentioned ones may be the causes of constipation, except:
A) Idiopathic megacolon
B) Overdosage of vitamin D
C) Insufficient fluid intake
D) Salmonellosis
E) Muscular insufficienty
F) Rectal stenosis

A

ANSWER
D) Salmonellosis

EXPLANATION
Salmonellosis causes diarrhea or persist without symptoms, however it never induce constipation. All other situations mentioned in this test cause constipations.

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5
Q

PED - 7.5
All statements are characteristic for ascariasis, except:
A) This worm goes through maturation in both man and soil
B) It causes always eosinophilia
C) Eggs can be rarely seen in stool
D) Rectal migration is not characteristic
E) The worms migrate to the lung

A

ANSWER
B) It causes always eosinophilia

EXPLANATION
Eosinophilia is not always observable at ascaris, only during larva migration. All other statements are true.

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6
Q

PED - 7.6
An exclusively breastfed infant develops properly, her or his mother has ample breast milk. When would you suggest to introduce the vegetable purée to the infant’s diet?
A) 6 weeks
B) about 6 months
C) 9 months
D) 11 months

A

ANSWER
B) about 6 months

EXPLANATION
6 weeks is very early even in non-breast fed infants and 9 months is too late for the introduction of vegetable puree. Infant need exclusive breast feeding during the first six months, but after this age only breast milk not ensures the proper development, therefore the introduction of complementary feeding should be started.

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7
Q

PED - 7.7
The absorption of vitamin B12 and bile salts are specifically located to:
A) Duodenum
B) Jejunum
C) Ileum
D) None of them

A

ANSWER
C) Ileum

EXPLANATION
Vitamin B12 and bile salts absorbed with active transport in the terminal ileum. the former one in a complex with intrinsic factor produced by the parietal cells of stomach.

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8
Q

PED - 7.8
Steatorrhea occurs in all undermentioned disease, except:
A) lactose intolerance
B) intestinal lymphangiectasia
C) coeliac disease
D) cystic fibrosis
E) bacterial overgrowth of the small bowel

A

ANSWER
A) lactose intolerance

EXPLANATION
Lactose intolerance does not cause steatorrhea, but osmotic, watery diarrhea

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9
Q

PED - 7.9
Hematochezia occurs in all undermentioned conditions, except:
A) allergic colitis
B) oesophagitis
C) Meckel-diverticulum
D) Ulcerative colitis

A

ANSWER
B) oesophagitis

EXPLANATION
Bleeding from the lower gastrointestinal tract, the stool contain visible red blood. This can occur at very large bleeding even from the upper gastrointestinal tract (e.g. rupture of oesopageal varices), but at oesopagitis the bleeding is mild.

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10
Q

PED - 7.10
The undermentioned statements are true in atrophy, except:
A) malnutrition without oedema
B) weight loss is 20-30%
C) caused by protein deficient diet
D) energy and protein intake are inadequate

A

ANSWER
C) caused by protein deficient diet

EXPLANATION
In atrophy both the energy and protein intake are low, but the protein/energy ratio is normal.

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11
Q

PED - 7.11
The undermentioned statements are true in decomposition, except:
A) it is called also athrepsy
B) weight loss is larger than 30%
C) the ratio of energy/protein intake is normal
D) mild form of protein-energy malnutrition

A

ANSWER
D) mild form of protein-energy malnutrition

EXPLANATION
Decomposition is the most severe form of protein-energy malnutrition.

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12
Q

PED - 7.12
The undermentioned statements are true for kwashiorkor, except:
A) may be caused by chronic diarrhea
B) malnutrition with oedema
C) sufficient energy intake with shortage of protein
D) caused by enzyme defect
E) the name of kwashiorkor denotes the reddish color of skin

A

ANSWER
D) caused by enzyme defect

EXPLANATION
Kwashiorkor develops because of low protein intake or increased catabolism caused by infection

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13
Q

PED - 7.13
All statements indicate protein-energy malnutrition with severe prognosis, except:
A) the weight loss is more than 30 percent
B) weight loss during treatment
C) bloodstream infection
D) age under 6 months
E) serum total protein concentration less than 30 g/l

A

ANSWER
B) weight loss during treatment

EXPLANATION
In hypoproteinemia oedema is the characteristic symptom, but when the oedema decrease, decrease of body weight is observable.

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14
Q

PED - 7.14
The undermentioned statements are true, except:
A) All forms of vegetarian diet are dangerous in childhood
B) It should talk the parents out of vegan diet by any means
C) After accepting the adequate advices the lacto ovo vegetarian diet is not dangerous for the children
D) The main danger of vegetarian diet is the B12-vitamin deficiency

A

ANSWER
A) All forms of vegetarian diet are dangerous in childhood

EXPLANATION
The lacto-ovo-vegetarian diet is not dangerous because cover all needs of macro- and micronutrients.

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15
Q

PED - 7.15
All the following statements are true, except:
A) Lifelong strict gluten-free diet is mandatory for the treatment of coeliac disease
B) Coeliac patients are fully healthy on gluten-free diet
C) Once in a while consumption of gluten-containg food does not cause problem
D) Intake of 100 mg gluten causes damage of the small intestinal mucosa

A

ANSWER
C) Once in a while consumption of gluten-containg food does not cause problem

EXPLANATION
Even occasional gluten consumtion impairs the small intestinal mucosa.

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16
Q

PED - 7.16
All undermentioned cereals contain gluten, except:
A) Wheat
B) Rye
C) Rice
D) Barley

A

ANSWER
C) Rice

EXPLANATION
Rice does not contain gluten and can be freely consumed by coeliac patients.

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17
Q

PED - 7.17
All undermentioned foodstuffs are gluten-free, except:
A) Potato
B) Rye
C) Corn
D) Rice
E) Locust seed

A

ANSWER
B) Rye

EXPLANATION
Rye is a gluten-containing cereals, all the other foodstuff is gluten-free.

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18
Q

PED - 7.18
Indicate the single statement which is true for rumination:
A) It is a cosequence of voluntary strain
B) It is a passive regurgitation of gastric content
C) It is a consequence of a retrograde duodeno-gastric contraction
D) It is a symptom a gastro-oesophaeal reflux

A

ANSWER
A) It is a cosequence of voluntary strain

EXPLANATION
Rumination is a voluntary action, while the other the other mentioned phenomenons are unintended.

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19
Q

PED - 7.19
All statements are true for infantile gastro-oesophageal reflux (GOR), except:
A) Starts in the first months of life
B) In 80% of cases it ceases at 12-24 months
C) GOR with different severity occur in 20% of infants
D) It can be continued to adult type GOR
E) It is always a physiological condition

A

ANSWER
E) It is always a physiological condition

EXPLANATION
Infantile gastro-oesophageal reflux can be regarded a physiological phenomen while it does not cause decrease in weight gain and does not influence the infant’s lifestyle.

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20
Q

PED - 7.20
All statements are true for th hypoallergenic formula used for therapeutic purposes, except one:
A) They are extensively hydrolysed
B) Does not contain cow’s milk or soya proteins
C) Contain short chain peptides
D) Molecular weight of the peptides are over 2000 Dalton
E) More than 90 percent of Infants with cow’s milk protein intolerance tolerate these formula

A

ANSWER
D) Molecular weight of the peptides are over 2000 Dalton

EXPLANATION
In extensive hydrolysed formula the molecular weight of peptides are under 1500 Dalton, which means that they contain 4-8 aminoacids.

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21
Q

PED - 7.21
All statements are true for melena, except one:
A) It originated from the upper gastrointestinal tract
B) It has a jet-black colour
C) Its other name is hematochezia
D) It is consequence of significant bleeding

A

ANSWER
C) Its other name is hematochezia

EXPLANATION
The colour of melena is jet-black, while that of hematochezia is red, visible blood.

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22
Q

PED - 7.22
Statements are characteristic for Crohn’s disease, except one:
A) It can occur anywhere in the gastrointestinal tract throughout from the oral cavity to the anus
B) Characterized with relapses
C) Blood stool is relatively rare
D) Segmental and transmural propagation is frequent
E) Occurs with the hyperplasia of lymphoid tissues and frequently involves the regional lymph nodes
F) Surgical solution brings definitive healing

A

ANSWER
F) Surgical solution brings definitive healing

EXPLANATION
Crohn’s disease may relapse even after surgical intervention, because recurrence can occur anywhere in the gastrointestinal tract.

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23
Q

PED - 7.23
All the undermentioned disease could cause liver cirrhosis, except:
A) galactosaemia
B) alfa1-antytripsin deficiency
C) hepatitis A virus infection
D) hepatitis B virus infection
E) lupus hepatitis
F) bile duct obstruction

A

ANSWER
C) hepatitis A virus infection

EXPLANATION
Hepatitis A virus infection has an acute course and there is not a long lasting carrier state of this virus and fibrotic process.

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24
Q

PED - 7.24
All statements are true for food related adverse reaction, except:
A) More frequent than food allergy
B) It has a quantity threshold value for the given food
C) For eliciting symptoms the all or nothing principle is the rule of thumb
D) It is no previous sensitization
E) The symptoms may be just the same as in food allergy

A

ANSWER
C) For eliciting symptoms the all or nothing principle is the rule of thumb

EXPLANATION
The rule of all or nothing is characteristic for allergic reaction, while the non-immune mediated food reaction always has a threshold value, which elicit the symptoms.

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25
Q

PED - 7.25
All factors are important for the pathogenesis of food allergy in infants and toddlers, except:
A) Genetic factors
B) Early foreign protein introduction to the infants diet
C) Protein rich diet of mother during pregnancy
D) Environmental factors (e.g. smoking)
E) Immaturity or disturbance of intestinal defense mechanisms

A

ANSWER
C) Protein rich diet of mother during pregnancy

EXPLANATION
The diet during pregnancy does not influence the development of allergy.

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26
Q

PED - 7.26
All the statements are true for anaphylactoid (Henoch-Schönlein) purpura, except:
A) The small purpura mostly located on the extensor side of the lower extremities and the gluteal area
B) Onset of purpura may be preceded by joint paint
C) Bloody stool and abdominal pain may also present
D) Relapse quite frequently occur
E) Platelet number decreased

A

ANSWER
E) Platelet number decreased

EXPLANATION
Anapylactoid purpura is a vasculitis, the number of thrombocytes is normal

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27
Q

PED - 7.27
Choose the answers which are true in adult type lactose intolerance.
1) Very small amout of lactose may cause severe symptoms.
2) The patients are able tolerate so much lactose which is in 2 deciliter milk.
3) Processed cheese could be consumed in any quantity.
4) It is important not to consume in one portion and in itself the lactose containing foods.

A) Answers 1, 2 and 3 are correct
B) Answers 1 and 3 are correct
C) Answers 2 and 4 are correct
D) Only answer 4 is correct
E) All of the answers are correct

A

ANSWER
C) Answers 2 and 4 are correct

EXPLANATION
Patients with adult type lactose intolerance can tolerate variable quantity of lactose, but usually the lactose content of 2-2,5 deciliter milk. It is important to know that the milk products also contain lactose.

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28
Q

PED - 7.28
Choose the answers which are true at decreased activity of lactase enzyme.
1) Symptoms related to the quantity of consumed lactose.
2) In children frequently occurs periumbilical abdominal pain after lactose consumtion.
3) Abdominal pain may be very severe.
4) Diarrhea does not always develop after lactose consumtion.

A) Answers 1, 2 and 3 are correct
B) Answers 1 and 3 are correct
C) Answers 2 and 4 are correct
D) Only answer 4 is correct
E) All of the answers are correct

A

ANSWER
E) All of the answers are correct

EXPLANATION
All statements are true. At children, it is important to consider the possibility of lactose intolerance at periumbilical abdominal pain, which could be quite severe. Abdominal distension and feeling of fullness are also frequently occuring symptoms.

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29
Q

PED - 7.29
When the diagnosis of coeliac disease can be established without the histologic analysis of dudenal mucosa biopsy taken by endoscopy?
1) The serum anti-transglutaminase (TTG) IgA titer is 10 times higher than the upper normal value.
2) Antiendomysium antibody titer is also positive.
3) The patient has no IgA deficiency.
4) The serum anti-TTG IgA titer is 5 times higher than the upper normal value.

A) Answers 1, 2 and 3 are correct
B) Answers 1 and 3 are correct
C) Answers 2 and 4 are correct
D) Only answer 4 is correct
E) All of the answers are correct

A

ANSWER
A) Answers 1, 2 and 3 are correct

EXPLANATION
If the anti transglutaminase (TTG) IgA antibody titer is higher than ten times the upper level of normal value and the endomysial antibody test is also positive, the diagnosis of coeliac disease can be established. However, in IgA deficiency for the diagnosis it is necessary the histological examination of the duodenal biopsy sample taken by upper endoscopy, because the titer of anti-TTG IgG titer does not prove with safety the presence of coeliac disease.

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30
Q

PED - 7.30
Which symptoms could occur in untreated coeliac disease?
1) Abdominal pain
2) Iron deficiency
3) Tooth enamel defect
4) Ataxia

A) Answers 1, 2 and 3 are correct
B) Answers 1 and 3 are correct
C) Answers 2 and 4 are correct
D) Only answer 4 is correct
E) All of the answers are correct

A

ANSWER
E) All of the answers are correct

EXPLANATION
Symptoms of coeliakia are very variable, so abdominal pain, iron defieciency, enamel defect and ataxia all may occur.

31
Q

PED-7.31-7.35
Connect the relevant treatment signed with letter to that disease signed with number, where it has indication.
A) Medium chain triglycerides
B) Zinc supplementation
C) Gluten-free diet
D) Galactose-free diet
E) Low lactose containing diet

PED - 7.31 - coeliakia
PED - 7.32 - galactosaemia
PED - 7.33 - intestinal lymphangiectasy
PED - 7.34 - postenteritis syndrome
PED - 7.35 - acrodermatitis enteropathica

A

ANSWER
PED - 7.31 - coeliakia - C)
Lifelong gluten-free diet the single possible treatment for coeliac disease.

PED - 7.32 - galactosaemia - D)
Very strict galactose free diet is the single possible treatment in galactosemia. As galactose is a component of lactose, breast feeding is contraindicated in infants with this inborn error of metabolism.

PED - 7.33 - intestinal lymphangiectasy - A)
Medium chain triglicerides absorbed by the capillary of portal system and arrive to the liver, such a way they spare the lymphatic vessels, which will not rupture in the intestinal villi losing their high protein content to the intestinal lumen.

PED - 7.34 - postenteritis syndrome - E)
Lactase enzyme located in the brush border of intestinal epithelial cells and in infection mostly this part of the small bowel impairs decreasing transiently also the quantity of lactase enzyme.

PED - 7.35 - acrodermatitis enteropathica - B)
Acrodermatitis eneteropathica is an autosomal recessively inherited disorder, where the absorption of zinc is impaired. Treatment of acrodermatitis enteropathica requires lifelong zinc supplementation.

32
Q

PED-7.36-7.40
Connect the symptoms singed with letter to that disorder for which they are mostly characteristic.
A) fever, spastic abdominal pain blood streaked and mucus containing stool
B) watery, acidic smell, fermented stool
C) large volume, stinky and fatty stool
D) watery, acidic smell, greenish stool containing transparent mucus
E) subfebrility, squirting watery stool

PED - 7.36 - Pancreas hypofunction
PED - 7.37 - Campylobacter jejuni enteritis
PED - 7.38 - Rotavirus infection
PED - 7.39 - Lactose intolerance
PED - 7.40 - Pseudodyspepsia

A

ANSWER
PED - 7.36 - Pancreas hypofunction - C)
When the exocrin function of pancreas decreases, mostly the fat absorption is decreasing.

PED - 7.37 - Campylobacter jejuni enteritis - A)
Campylobacter jejuni infection causes invasive enteral infection mostly in the large bowel, consequently colitis syndrome.

PED - 7.38 - Rotavirus infection - E)
Rotavirus infection involve mostly the small bowel causing squirting watery diarrhea with subfebrility.

PED - 7.39 - Lactose intolerance - B)
Lactose intolerance causes osmotic watery diarrhea, the unabsorbed lactose will be fermented in the large bowel and the produced short chain fatty acids will give the acidic smell of stool.

PED - 7.40 - Pseudodyspepsia - D)
Breast fed infants may have frequently watery, greenish stool with acidic smell, which is quite normal and it should be continued the breast feeding.

33
Q

PED - 7.41
What is the most probable diagnosis based on the case report?
An eight month old infant has abdominal distension, which observable for several months. From the history turns out that he unloaded meconium only after 48 hours of age. He had stool infrequently even during the exclusive breast feeding until 6 months, but this became more striking after introduction of complementary feeding. Last weeks his weight gain also decreased. At examination the abdomen was very distended and at rectal examination the sphincter tone was normal but the ampulla was very tight and empty.
A) Cystic fibrosis
B) Crohn’s disease
C) Functional constipation
D) Hirschsprung disease
E) Rectal stenosis

A

ANSWER
D) Hirschsprung disease

EXPLANATION
The tight ampulla is not characteristic for cystic fibrosis. At 8 months constipation can not be explained by CF. Occurance of Crohn’s disease is very rare at 8 months of age and it could cause strong abominal cramps, fever and frequently bloody diarrhea at this early age. The ampulla is wide and full with stool in functional constipation. In Hirschsprung’s disease is an important sign the tightness of rectum and the distal large bowel because of the lack of intestinal ganglions

34
Q

PED - 7.42
Which examination would you choose for the confirmation of diagnosis?
An eight month old infant has abdominal distension, which observable for several months. From the history turns out that he unloaded meconium only after 48 hours of age. He had stool infrequently even during the exclusive breast feeding until 6 months, but this became more striking after introduction of complementary feeding. Last weeks his weight gain also decreased. At examination the abdomen was very distended and at rectal examination the sphincter tone was normal but the ampulla was very tight and empty.
A) Small bowel follow through
B) Irrigoscopy
C) Genetic analysis of CFTR gene
D) Abdominal MRI

A

ANSWER
B) Irrigoscopy

EXPLANATION
Stomach-small bowel follow through is not justified, because with this method mostly the upper gastroenterological tract could be examined which is involved only very rarely in Hirschsprung disease. Irrigoscopy is the adequate examination, because this will show the lower tight part of the large bowel where is no ganglion and above that the dilatated upper part from where the intestinal content can move only very slowly move further and therefore intestinal loops containing ganglions are very wide. Genetic analysis of CFTR gene is not indicated, as the infant does not have symptoms characteristic for cystic fibrosis. Abdominal MRI is also not indicated becuase irrigoscopy gives more information about the intestinal passage.

35
Q

PED - 7.43
Which disorders may cause the development of meconium ileus?
An eight month old infant has abdominal distension, which observable for several months. From the history turns out that he unloaded meconium only after 48 hours of age. He had stool infrequently even during the exclusive breast feeding until 6 months, but this became more striking after introduction of complementary feeding. Last weeks his weight gain also decreased. At examination the abdomen was very distended and at rectal examination the sphincter tone was normal but the ampulla was very tight and empty.
1) Hypothyreosis
2) Cystic fibrosis
3) Metabolic acidosis
4) Hirschsprung diseases

A) Answers 1, 2 and 3 are correct
B) Answers 1 and 3 are correct
C) Answers 2 and 4 are correct
D) Only answer 4 is correct
E) All of the answers are correct

A

ANSWER
C) Answers 2 and 4 are correct

EXPLANATION
Hypothyreosis may cause slow intestinal motility rarely leading to pseudo-obstruction, but not meconium ileus. Cystic fibrosis is the most frequent cause of meconium ileus because the CFTR gene mutation leads to very viscous intestinal content, which develops even the intrauterine life. Metabolic acidosis never elicits meconium ileus. In Hirschsprung disease, the passage is very slow in the aganglionic intestinal segments which explains the development of meconium ileus.

36
Q

PED - 7.44
What is the most probable diagnosis according to the case descripiton?
The weight gain of a 10 month old infant was normal until 6 month of age during exclusive breastfeeding. After introduction of complementary feeding (vegetables, meat, egg, cereals) between 6 and 7 months her weight gain stoped and from the former 50 percentil fell to 25 percentil. In last four weeks diarrhea started and the stools were fatty, and abdominal distension was also observed. The earlier merry baby girl’s mood failed. In her stool blood and was not observed, hives or any rash as well as respiratory symptoms were also not detected.
A) Food allergy
B) Coeliac disease
C) Cystic fibrosis
D) Sucrose malabsorption
E) Functional diarrhea

A

ANSWER
B) Coeliac disease

EXPLANATION
Symptoms (skin rash, respiratory, bloody stool) were not in the history indicating food allergy. Decreased or stopped weight gain and steatorrhea are characteristic symptoms for coeliac disease. The possibility of coeliac disease is also supported by the fact that the symptoms started after the introduction of complementary feeding containing also cereals. In cystic fibrosis, growth retardation starts even before six months and also are present other symptoms, mostly respiratory ones. Sucrose malabsorption causes watery diarrhea with acidic smell because of the fermentation of unabsorbed sucrose. Stopping of weight gain does not occur at functional diarrhea and usually infants with this disorder are vivid and merry.

37
Q

PED - 7.45
Which ones of the undermentioned laboratory tests would you choose to the confirmation of the diagnosis?
The weight gain of a 10 month old infant was normal until 6 month of age during exclusive breastfeeding. After introduction of complementary feeding (vegetables, meat, egg, cereals) between 6 and 7 months her weight gain stoped and from the former 50 percentil fell to 25 percentil. In last four weeks diarrhea started and the stools were fatty, and abdominal distension was also observed. The earlier merry baby girl’s mood failed. In her stool blood and was not observed, hives or any rash as well as respiratory symptoms were also not detected.
1) Anti-transglutaminase IgA antibody
2) Sucrose hydrogen breath test
3) Serum IgA concentration
4) CFTR gene mutation analysis

A) Answers 1, 2 and 3 are correct
B) Answers 1 and 3 are correct
C) Answers 2 and 4 are correct
D) Only answer 4 is correct
E) All of the answers are correct

A

ANSWER
B) Answers 1 and 3 are correct

EXPLANATION
At suspicion of coeliac disease, the measurement of serum anti-transglutaminase IgA antibody titer is the first choice of serological test. It is also important to measure the serum IgA concentration, because in IgA deficiency this test is not informative. Sucrose breath hydrogen test is not indicated on the base of history. As the history and the physical finding exlude the possibility of cyastic fibrosis, CFTER gene test is not indicated.

38
Q

PED - 7.46
Which examination would you choose for further confirmation of the diagnosis according to the results of previous tests, if indicated?
The weight gain of a 10 month old infant was normal until 6 month of age during exclusive breastfeeding. After introduction of complementary feeding (vegetables, meat, egg, cereals) between 6 and 7 months her weight gain stoped and from the former 50 percentil fell to 25 percentil. In last four weeks diarrhea started and the stools were fatty, and abdominal distension was also observed. The earlier merry baby girl’s mood failed. In her stool blood and was not observed, hives or any rash as well as respiratory symptoms were also not detected.
A) Colonoscopy
B) Abdominal MRI
C) Small bowel follow through
D) Irrigoscopy
E) Analysis of duodenal biopsy sample taken with upper endoscopy

A

ANSWER
E) Analysis of duodenal biopsy sample taken with upper endoscopy

EXPLANATION
Colonoscopy and irrigoscopy is not indicated, because coeliac diesease does not involve the large bowel mucosa. MRI and gastric and small bowel follow through are also unjustified, as coeliac disease does not cause thickeness and/or strictures in the small intestine. If the serological examination do not confirm unequivocally the diagnosis of coeliac disease then the detection of atrophy in the duoenal biopsy sample taken through upper enterocopy could confirm it.

39
Q

PED - 7.47
Which one is the right diagnosis at this patient?
A 10 years old girl has been abdominal crampy pains for several months, sometimes she is awakening for the pain during night. She has appetite loss and lost weight and frequently has fever. Pathogen bacteria were not detected in stool.
A) Functional abdominal pain
B) Coeliac disease
C) Cholelithiasis
D) Crohn’s disease
E) Ulcerative colitis

A

ANSWER
D) Crohn’s disease

EXPLANATION
Functional abdominal pain does not cause weight loss, appetite loss, fever and awakening for pain. Coeliac disease explains weight loss, but fever and acute abdominal pain are not occur. Cholelithiasis frequently symptomless, or causes acute abdominal pain, but weight loss is not characteristic. The described symptoms occur in Crohn’s disease. In ulcerative colitis lack of appetite and weight loss are not so frequent, but the bloody diarrhea with tenesmus are more frequent.

40
Q

PED - 7.48
Which image forming and endoscopic examination would you choose for the further confirmation of diagnosis?
A 10 years old girl has been abdominal crampy pains for several months, sometimes she is awakening for the pain during night. She has appetite loss and lost weight and frequently has fever. Pathogen bacteria were not detected in stool.
1) Abdominal ultrasound
2) Colonoscopy
3) Upper endoscopy
4) Irrigoscopiy

A) Answers 1, 2 and 3 are correct
B) Answers 1 and 3 are correct
C) Answers 2 and 4 are correct
D) Only answer 4 is correct
E) All of the answers are correct

A

ANSWER
A) Answers 1, 2 and 3 are correct

EXPLANATION
Abdominal ultrasound can detect the thickness of the intestinal wall and so may confirm the possibility of Crohn’s disease. For the definitive diagnosis, there are indispensable the colonoscopy and upper endoscopy with taking biopsy samples. The endoscopic and histologic findings together with the clinical picture are decisive in the diagnosis. Irrigoscopy is not indicated as is it is not a good method for the detection of strictures, the MRI recommended for this purpose.

41
Q

PED - 7.49
If the suspected disease is confirmed, which therapy from the undermentioned ones is the first choice for remission induction?
A 10 years old girl has been abdominal crampy pains for several months, sometimes she is awakening for the pain during night. She has appetite loss and lost weight and frequently has fever. Pathogen bacteria were not detected in stool.
A) Wide spectrum antibiotics
B) Exclusive enteral nutrition
C) Cyclosporin
D) Tacrolimus
E) None of them

A

ANSWER
B) Exclusive enteral nutrition

EXPLANATION
Wide spectrum antibiotics are not suitable for induction of remission, their administration is indicated in complications, as abscesses and fistulas. Exclusive enteral nutrition induces remission as well as steroids but without the side effect of the latter one. Cyklosproine and tacrolimus only seldom indicated, only in relapses not reacting for other therapies.

42
Q

PED - 7.50
Which tissues contain SGOT (AST)?
A) liver
B) myocardium
C) sceletal muscle
D) kidney
E) each of them

A

ANSWER
E) each of them

EXPLANATION
Serum SGOT (AST) is present not only in hepatocytes, but also in heart and sceletal muscle as well as in the kidney. Consequently serum SGOT elevation does not indicate always liver disease, but it occurs after strong physisical strain causing damage of sceletal muscle. In such situation serum creatine kinase is also high.

43
Q

PED - 7.51
Which parameters indicate the synthetic capacity of liver?
A) GOT (AST), GPT (ALT)
B) bilirubin, GGT
C) albumin, prothrombin, INR

A

ANSWER
C) albumin, prothrombin, INR

EXPLANATION
Serum albumin, prothrombin time and INR are the indicators of liver synthetic capacity. Serum albumin and prothrombin decrease and INR increases in parenchymal liver insufficiency. The elevation of serum SGOT and SGPT does not indicate parenchymal insufficiency, only shows the size of injury of hepatocytes. Serum bilirubin and GGT concentration elevate in cholestasis.

44
Q

PED - 7.52
Which statement is true for biliary atresia?
A) prolonged jaundice with indirect hyperbilirubinemia
B) it has autosomal dominant inheritance
C) associate always with other malformations (e.g. polysplenia)
D) it has autosomal recessive inheritance
E) progressive fibrotizing process of intra- and extrahepatic bile ducts

A

ANSWER
E) progressive fibrotizing process of intra- and extrahepatic bile ducts

EXPLANATION
Biliary atresia caused by a progressive fibrotizing process of intra and extraheptic bile ducts, which leads to direct hyperbilirubinemia and a gradually worsening jaundice. Neither dominant, nor recessive inheritence is observable. Polysplenia does not occur in every cases only in the embryonal forms.

45
Q

PED - 7.53
Which statement is not true for direct hyperbiliturinaemia?
A) serum concentration of direct bilirubin is higher than 25 micromol/l
B) serum concentration of direct bilirubin is higher than 20 percent of total bilirubin
C) decreased canalicular bile flow
D) cholestasis
E) its frequncy is 1:25000-1:40000

A

ANSWER
E) its frequncy is 1:25000-1:40000

EXPLANATION
In conjugated hyperbilirubinemia the serum level of direct bilirubin is over 25 micromol/l and its quantity is larger than 20 % of total bilirubin. Slow canalicular bile flow and cholestasis are also characteristic findings. The frequency of conjugated hyperbilirubinemia is 1:2500-1:4000.

46
Q

PED - 7.54
Is it necessary to suspend breastfeeding in case of biliary atresia?
A) Yes
B) No
C) Depend on the serum bilirubin concentration
D) Depending on the age of child
E) Yes, for a few days

A

ANSWER
B) No

EXPLANATION
It is not justified the suspension of breastfeeding in biliary atresia irrespectively of the age and serum bilirubin value of patient.

47
Q

PED - 7.55
When is the most frequent the occurance of acute liver failure?
A) Under 1 year
B) Between 1-2 years
C) Between 2-5 years
D) Over 5 years

A

ANSWER
A) Under 1 year

EXPLANATION
Occurance of acut liver failure is most frequent under one year of age, principally caused by inborn error of metabolism or indeterminated etiology.

48
Q

PED - 7.56
What is the most frequent cause of acute liver failure under 1 year?
A) Autoimmune disease
B) Viral infection
C) Metabolic and indeterminated causes
D) Acute Wilson disease

A

ANSWER
C) Metabolic and indeterminated causes

EXPLANATION
Under 1 year the most frequent causes of acute liver failure are inborn error of metabolism or unspecified etiologies.

49
Q

PED - 7.57
How frequent is jaundice in matured neonates?
A) 30%
B) 40%
C) 50%
D) 60%

A

ANSWER
D) 60%

EXPLANATION
Frequency of jaundice in mature infants is about 60 percent, most of them have physiological incterus, with elevated indirect bilirubin because of the increased hemolysis of fetal erythrocytes and the decreased activity of glucuronyl transferase enzyme.

50
Q

PED - 7.58
Is it necessary to suspend breastfeeding during jaundice elicited by mother’s milk?
A) Yes
B) No
C) Depends on the level of serum bilirubin
D) Depends on the infant’s age
E) Only for a few days

A

ANSWER
B) No

EXPLANATION
In some infants breastfeeding may slightly increase the serum level of indirect bilirubin, which is caused by pregnandiol and unsaturated fatty acid content of breast milk and the increased enterohepatic circulation. Suspension of breastfeeding is not justified.

51
Q

PED - 7.59
Which one is a pathological condition in neonatal period?
A) Physiologic icterus
B) Breast milk induces icterus
C) Icterus of pramature babies
D) Conjugated hyperbilirubinaemia

A

ANSWER
D) Conjugated hyperbilirubinaemia

EXPLANATION
Physiological and breast milk induced jaundice are not pathological conditions, as well as the icterus of prematures neonates. Elevation of conugated bilirubin indicate cholestasis which indicates a thoroughful diagnostic evaluation to find out its cause.

52
Q

PED - 7.60
Which one of the undermentioned factors does not decrease bilirubin binding capacity of albumin?
A) Acidosis
B) Hypoxia
C) Hypothermia
D) Hyperglycemia

A

ANSWER
D) Hyperglycemia

EXPLANATION
Acidosis, hypoxia and hypothermia and hypoglycemia decrease significantly the bilirubin binding capacity of albumin, while hyperglycemia does not influences it.

53
Q

PED - 7.61
Which condition does not increase the bilirubin production?
A) Haemolysis
B) Crigler-Najjar syndrome
C) Extravascular blood in hematomas
D) Polycythemia

A

ANSWER
B) Crigler-Najjar syndrome

EXPLANATION
Hemolysis, extravasal blood accumulation and polycythemia increase the destruction of erythrocyetes, and so from the released hemoglobin more bilirubin will be formed. In Crigler-Najjar syndrome the production of bilirubin does not change, but the conjugation of indirect bilirubin will totally or partially impaired leading to its increase.

54
Q

PED - 7.62
Which statement is not true for Alagille syndrome?
A) Autosomal recessively inherited
B) Triangular face
C) Butterfly verterbrae
D) Cardiovascular malformations
E) Pruritus is a common symptom

A

ANSWER
A) Autosomal recessively inherited

EXPLANATION
Alagille syndrome inherited autosomal dominantly, triangular face, butterfly vertebrae, cardiovascular malformation and cholestasis with pruritus belong to its characteristic features.

55
Q

PED - 7.63
What is characteristic for Wilson disease?
A) Rarely onset the first clinical symptoms under 4-5 years of age
B) Rarely occur symptoms under 10 years of age
C) Liver symptoms always present earlier than the neurological ones
D) Specific findings can be seen in the liver biopsy specimens

A

ANSWER
A) Rarely onset the first clinical symptoms under 4-5 years of age

EXPLANATION
Symptoms of Wilson disease unfrequently starts under 4-5 years of age. Hepatic symptoms are more frequent in small children, but rarely may occur even in this age group, that neurological symptoms are the first manifestation. There is no specific histologic finding in the liver biopsy specimen which is specific for this disorder.

56
Q

PED - 7.64
What is the definition of fatty liver disease?
A) fatty change in more than 10 percent of hepatocytes
B) fatty change in more than 5 percent of hepatocytes
C) fatty change in more than 15 percent of hepatocytes
D) fatty change in more than 20 percent of hepatocytes
E) fatty change in more than 25 percent of hepatocytes

A

ANSWER
B) fatty change in more than 5 percent of hepatocytes

EXPLANATION
Fatty acid liver disease is proved when more than 5 percent of heptocytes show fatty transformation.

57
Q

PED - 7.65
Which one is a characteristic attribute for liver disease developing in cystic fibrosis?
A) In 20-25 percent presents liver disease with clinical symptoms
B) Pathological function of CFTR in hepatocytes is the causing factor
C) Pathological function of CFTR in bile duct epithaliac cells is the causing factor
D) Liver transplantation is contraindicated
E) Fatty transformation of liver is not characteristic

A

ANSWER
C) Pathological function of CFTR in bile duct epithaliac cells is the causing factor

EXPLANATION
The pathogenetic base of cystic fibrosis associated liver disease is the wrongly functioning CFTR gene in the epithelial cells of bile ducts. Liver disease with clinical symptoms occurs in 10-15 percent of patients with cystic fibrosis. Liver transplantation is indicated in severe parenchymal and vascular insufficiency. Fatty liver may also develop in CF associated liver disease.

58
Q

PED - 7.66
Which statement is not true for alfa-1 antitrypsin deficiency?
A) Autosomal recessive inheritance, locus 14q32.1
B) Frequency of PiZZ homozygosity 1:10000 in Europe, decreasing from to north to south
C) Pathological changes are variable in infant and children
D) In young adults pulmonary disease
E) 20% of PiZ carriers will have pulmonary disorder

A

ANSWER
E) 20% of PiZ carriers will have pulmonary disorder

EXPLANATION
Lung disease usually does not occur in carriers of protease inhibitor (Pi) Z, but it is recommended to abstain from smoking, because the carriers with smoking susceptible for development of emphysema.

59
Q

PED - 7.67
Which one is the right definiton of acute liver failure?
A) High level of transaminase enzymes
B) High level of ammonia
C) INR over 1,5 + encephalopathy or INR over 2
D) High level of ammonia and transaminase enzymes

A

ANSWER
C) INR over 1,5 + encephalopathy or INR over 2

EXPLANATION
The diagnosis of acute liver failure can be established if the INR is over 1,5 and the patient has encephalopathia, or INR is over 2 without encephalopathy. Elevated serum transaminase and ammonia values either separately or together have no diagnostic value for acute liver failure.

60
Q

PED - 7.68
Which one is the benefit of breastfeeding?
A) Decreases in children the frequency of allergic and autoimmune diseases
B) Decreases in mothers the risk of some malignant diseases
C) Both in children and mothers decreases the risk of cardiovascular diseases
D) Decreases of the frequency of several types of infection in children
E) All of them are true

A

ANSWER
E) All of them are true

EXPLANATION
It is proved that breastfeeding decreases the frequency of allergic and autoimmune disorders, as well as the risk of several infections. The risk of cardiovascular diseases was also decreased in both the mothers and children. Breastfeeding lowers also the risk of malignancy in the mothers.

61
Q

PED - 7.69
What is the most frequent cause of bloody diarrhea in children?
A) Infection
B) Inflammatory bowel disease (IBD)
C) Intestinal polyp
D) Meckel’s diverticulum
E) Hemorrhoid

A

ANSWER
A) Infection

EXPLANATION
The most frequent cause of bloody diarrhea is the infection, while their prevalence is lower in the other mentioned diseases.

62
Q

PED - 7.70
Which is the most frequently occuring food allergy and such as frequent cause allergic intestinal inflammation?
A) cow’s milk
B) egg
C) soya
D) peanut
E) tomato

A

ANSWER
A) cow’s milk

EXPLANATION
Cow’s milk allergy is the most frequently observed food allergy. Egg, soya, peanut and tomato allegies are not so frequent.

63
Q

PED - 7.71
Which one of the undermentioned bacteria may cause bloody diarrhea in childhood?
A) Salmonella
B) Shigella
C) E. Coli
D) Campylobacter
E) All of them

A

ANSWER
E) All of them

EXPLANATION
Gram-negative bacteria cause mostly bloody stool. All of the mentioned Gram negativ bacteria could cause enteritis and colitis, consequently bloody stool.

64
Q

PED - 7.72
What is the gold standard of the diagnosis of food allergy in childhood?
A) Detection of IgE antibodies against different food antigens
B) Detection of IgG antibodies against different food antigens
C) Skin prick test with different food extracts
D) Elimination and challenge with the suspected food, or double blind placebo controlled challenge
E) None of them

A

ANSWER
D) Elimination and challenge with the suspected food, or double blind placebo controlled challenge

EXPLANATION
The diagnostic gold standard of food allergy is the cessation of symptoms at allergen elimination and the reappearance at challange. It is more reliable the double blind placebo controlled challenge. The detection of IgG antibody against the suspected food antigen has not any diagnostic value, but the detection of specific IgE antibodies can confirm the in the presence of symptoms the possibility of allergy.

65
Q

PED - 7.73
Which could be the symptoms of inflammatory bowel disease (IBD)?
A) abdominal pain, diarrhea
B) growth retardation
C) eye-skin-joint complaints
D) bloody diarrhea
E) all of them

A

ANSWER
E) all of them

EXPLANATION
Inflammatory bowel disease most frequently induce abdominal pain, diarrhoea and bloody stool. It can cause also growth retardation and often occur extraintestinal symptoms such as eye, skin and joint ones.

66
Q

PED - 7.74
Which one is NOT belong to the presently known risk factors for the development of IBD?
A) administration of antibiotics during infancy
B) occurance of IBD in the family
C) food allergy in infancy
D) smoking for Crohn disease
E) none of them

A

ANSWER
C) food allergy in infancy

EXPLANATION
Antibiotic treatment during infancy increases the frequency of inflammatory bowel disease. Development of IBD influenced first of all by environmental factors, but genetic predisposition is also important, because it occurs more frequntly in those, who have first degree relatives with this disorder. Smoking tends to increase the incidence of Crohn’s disease. Food allergy is not a risk factor for the development of IBD

67
Q

PED - 7.75
Which symptom is NOT typical for irritable bowel syndrome (IBS)?
A) chronic diarrhea
B) abdominal pain during night
C) bloody diarrhea
D) small weight loss
E) none of them

A

ANSWER
C) bloody diarrhea

EXPLANATION
Chronic diarrhea, abdominal pain during night, mild weight loss may occur in irritable bowel syndrome, but bloody stool does not.

68
Q

PED - 7.76
What is typical rather for Crohn disease than for ulcerative colitis?
A) fistula forming
B) gastric involvement
C) large weight loss
D) symptoms can be alleviated by diet
E) all of them

A

ANSWER
E) all of them

EXPLANATION
Fistula forming most frequently perianally and less often intraabdominally between intestinal loops, gastric involvement, significant weigth loss are all characteristic symptoms of Crohn disease, which can be allivated by diet. None of the mentioned symptoms are characteristic for ulcerative colitis.

69
Q

PED - 7.77
Which one can be the symptom of malabsorption?
A) chronic diarrhea
B) abdominal distension
C) growth retardation
D) slow weight gain
E) all of them

A

ANSWER
E) all of them

EXPLANATION
Chronic diarrhea, abdominal distension is a consequence of fermentation of unabsorbed nutrients leading to gas forming and to the increased and stagnant intestinal content. Malabsorption causes weight loss and growth retardation.

70
Q

PED - 7.78
Which one is NOT characteristic for adult type lactose intolerance?
A) Onset is mostly in late childhood
B) Inborn form is extremely rare
C) It may be outgrown later
D) One third to one half of population has it
E) None of them

A

ANSWER
C) It may be outgrown later

EXPLANATION
Adult type lactose intolerance is explained by the genetically determined decrease or cessation of lactase enzyme synthesis, which is a definitive condition. Its onset is usually around puberty and occur about in third-to half of population.

71
Q

PED - 7.79
Which symptom occurs in cow’s milk allergy?
A) Disturbed weight development
B) Chronic diarrhea
C) Constipation
D) Eczema
E) All of them

A

ANSWER
E) All of them

EXPLANATION
Slow weight gain, chronic diarrhea, eczema are frequent symptoms of cow’s milk allergy, less frequently constipation.

72
Q

PED - 7.80
What kind of examinations and in which sequence are suggested in otherwise healthy infant with bloody stool?
A) physical examination for exclusion of fissure, stool culture, trial of cow’s milk free diet
B) cow’s milk free diet, physical examination, stool culture
C) physical examination, stool culture, abdominal ultrasound and native abdominal X-ray
D) physical examination, abdominal ultrasound and native abdominal X-ray
E) colonoscopy is mandatory

A

ANSWER
A) physical examination for exclusion of fissure, stool culture, trial of cow’s milk free diet

EXPLANATION
In an otherwise healthy infant, in case of bloody stool the physical and rectal examinations are the most important. At rectal examination, it should be observed whether the patient has fissures or perianal fistula. Following this it is necessary to take stool culture. If no pathological finding is detected at these examinations, it is suggested to put the infant on a cow’s milk free diet or at exclusive breastfeeding the mother should start such a diet.

73
Q

PED - 7.81
Which further examination would you suggest first in this case even at the general practitioner?
A 16 years old boy arrives to the emergency deaprtment because of bloody diarrhea lasting for a few weeks associated with tenesmus. Previously abdominal pain once in a while was noticed but no examination was done. At physical examination paleness and mild left sided abdominal pressure sensitivity were observed. Perianal lesions were not detected.
A) Stool culture
B) Blood picture
C) Abdominal ultrasound
D) Stool calprotectin
E) None of them

A

ANSWER
A) Stool culture

EXPLANATION
The patient’s symptoms indicate the presence of colitis syndrome, caused most frequently by infection, therefore the stool culture is the first choice in the row of examinations. If no pathogen bacteria can be detected from the stool then it is important further examinations: the blood picture and other laboratory test, abdominal ultrasound and stool calprotectin.

74
Q

PED - 7.82
Which disease is the most probable in case the above mentioned examination is negative?
A 16 years old boy arrives to the emergency deaprtment because of bloody diarrhea lasting for a few weeks associated with tenesmus. Previously abdominal pain once in a while was noticed but no examination was done. At physical examination paleness and mild left sided abdominal pressure sensitivity were observed. Perianal lesions were not detected.
A) Allergic colitis
B) Meckel’s diverticulum
C) IBD
D) Large bowel polyp
E) None of them

A

ANSWER
C) IBD

EXPLANATION
If the patient described in case presentation does not have pathogen bacterium in the stool, then the most probable diagnosis among the mentioned ones, is the IBD, because this occurs most frequently at this age and Meckel diverticulum, large intestinal polyp does not cause tenesmus.