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CONSTIPATIONCONSTIPATION
Dr. Soad JaberDr. Soad Jaber20092009
ConstipationConstipation Physiology of defecationPhysiology of defecation Understand the differences betweenUnderstand the differences between
ConstipationConstipation EncorporesisEncorporesis SoilingSoiling
Identify major causes of constipationIdentify major causes of constipation Differences between functional and Differences between functional and
organic causesorganic causes Understand the principle behind Understand the principle behind
constipation managementconstipation management
CONSTIPATIONCONSTIPATIONPhysiology of defecationPhysiology of defecation In normal subjects : Distention of the rectumIn normal subjects : Distention of the rectum
Reflex relaxation of internal sphincter Reflex relaxation of internal sphincter Contraction of external Contraction of external sphinctersphincter
*Lower rectum is normally empty*Lower rectum is normally empty
*Entry of fecal material from above gives the sensation of the *Entry of fecal material from above gives the sensation of the need to defecate.need to defecate.
If the rectum is chronically distended the sensation is lost If the rectum is chronically distended the sensation is lost retention of stool retention of stool full rectum full rectum
ConstipationConstipation::
Delay or difficulty in defecation that Delay or difficulty in defecation that
has been present for two weeks or has been present for two weeks or longer. is associated with anal and longer. is associated with anal and abdominal discomfort.abdominal discomfort.
Full rectumFull rectum
Functional ConstipationFunctional Constipation::
Is a voluntary withholding of stoolsIs a voluntary withholding of stools
Starts after the neonatal periodStarts after the neonatal period
Usually develop after passage of Usually develop after passage of painful bowel movement.painful bowel movement.
EncopresisEncopresis::
Day or night time passage of formed Day or night time passage of formed stool into inappropriate places stool into inappropriate places beyond the age expected for toilet beyond the age expected for toilet training (4-5 yeartraining (4-5 year
Abnormal anal sphincter physiology.Abnormal anal sphincter physiology.
Types of EncopresisTypes of Encopresis : :(A(AA)RETENTIVE encopresis with constipation A)RETENTIVE encopresis with constipation
and overflow incontinence.2/3and overflow incontinence.2/3B)Non RETINTIVE encopresis: withoutB)Non RETINTIVE encopresis: without
(A(Aconstipation and overflow.1/3constipation and overflow.1/3
(B(BOR :1ry from infancy occur with global OR :1ry from infancy occur with global developmental delay and enuresisdevelopmental delay and enuresis..
(C(C2ry:after successful toilet training occur with 2ry:after successful toilet training occur with psychosocial stress and mismanagementpsychosocial stress and mismanagement
SoilingSoiling::
Involuntary escape of fluid or semi Involuntary escape of fluid or semi fluid stools into the under clothing fluid stools into the under clothing usually due to overflow from a usually due to overflow from a loaded cecum with feces which leads loaded cecum with feces which leads to stretch of internal sphincterto stretch of internal sphincter
Neurogenic SoilingNeurogenic Soiling::
Soiling which occur due to neurological Soiling which occur due to neurological abnormalities:abnormalities:
Spinal bifidaSpinal bifida
ParaplegiaParaplegia
MyelomengiocelMyelomengiocel
Causes of ConstipationCauses of Constipation::
A.A. Infants and Children:Infants and Children:
1. Non-organic Causes1. Non-organic Causes.Lack of dietary fibers.Lack of dietary fibers.Inadequate fluid intake.Inadequate fluid intake.Failure to develop regular bowel habits .Failure to develop regular bowel habits (neglect, stubborn child, MR)(neglect, stubborn child, MR).Follow illness.Follow illness.Change in environment or routine .Change in environment or routine
(Holidays… school entry)(Holidays… school entry)
Organic causeOrganic cause
Anatomic(anaal Anatomic(anaal stenosis,imperforatecanus,anteriorly stenosis,imperforatecanus,anteriorly displaced anus.intestinal stricture)displaced anus.intestinal stricture)
Abnormal musculature(prune-belly Abnormal musculature(prune-belly syndrome,Gastroschisis,Down syndromesyndrome,Gastroschisis,Down syndrome
Intestinal nerve or muscle Intestinal nerve or muscle abnormalities(Hirschprung diease)abnormalities(Hirschprung diease)
Spinal cord defects(spinal cord trauma or Spinal cord defects(spinal cord trauma or spina bifida)spina bifida)
Drugs(Anticholinergics or Drugs(Anticholinergics or narcotics<Vincristine,vit D narcotics<Vincristine,vit D intoxication,Lead)intoxication,Lead)
Metabolic Metabolic disorders(Hypokalemia,hypercalcemia,hypdisorders(Hypokalemia,hypercalcemia,hypotghyroidism,D.M)otghyroidism,D.M)
Intestinal disorders:(celiac disease,cow Intestinal disorders:(celiac disease,cow milk protein intolerance,meconium milk protein intolerance,meconium ileus,inflammatory bowel disease,tumour)ileus,inflammatory bowel disease,tumour)
Connective tissue Connective tissue disordersdisordersSLE,sclerdermaSLE,sclerderma))
Psychiatric diagnosis(Anorexia nervosa)Psychiatric diagnosis(Anorexia nervosa)
2. Organic Causes2. Organic Causes
• Hirschsprung’s diseaseHirschsprung’s disease
*Clinical clues*Clinical clues Delayed muconium passage > 48h or early Delayed muconium passage > 48h or early
onset of constipationonset of constipation Empty rectum on examination Normal anal toneEmpty rectum on examination Normal anal tone Absence of ganglia in rectal sub mucosal biopsyAbsence of ganglia in rectal sub mucosal biopsy Barium enema Barium enema transition zone transition zone Manometery --- absence of internal sphincter Manometery --- absence of internal sphincter
relaxationrelaxation
Conflict in trainingConflict in training
Stool retentionStool retention
Pain in defecationPain in defecation
withholding the stoolswithholding the stools
distention of the rectumdistention of the rectum
Rectal sensationRectal sensation
necessitating a greater fecal necessitating a greater fecal
mass to initiate the urge to mass to initiate the urge to defecatedefecate
Complication of stool Complication of stool retentionretention
ImpactionImpaction Abdominal painAbdominal pain Overflow diarrhea Overflow diarrhea leakage around the leakage around the
fecal massfecal mass Anal fissureAnal fissure Rectal bleedingRectal bleeding Urinary tract infectionUrinary tract infection
ManagementManagement::
History and clinical examination should History and clinical examination should reduce the number of investigation reduce the number of investigation e.g. anal fissuree.g. anal fissure
History of change in environment or dietHistory of change in environment or diet Presence of hard impacted stoolsPresence of hard impacted stools Barium study:?XRAYBarium study:?XRAY
.large rectum or recto -segmoid.large rectum or recto -segmoid
.impaction of stool.impaction of stool
B. B. Elder ChildrenElder Children He might present with recurrent He might present with recurrent
abdominal painabdominal pain He might develop soilingHe might develop soiling The elder child will eventually reach the The elder child will eventually reach the
stage where he is very quiet, withdrawn stage where he is very quiet, withdrawn and isolated from his classmatesand isolated from his classmates
Parents and child should participate in a Parents and child should participate in a group discussion and plan the group discussion and plan the management to help the childmanagement to help the child
*If with soiling advise of Psychiatrist*If with soiling advise of Psychiatrist
MedicalMedical1.1. Hypertonic phosphate enemas daily or every other Hypertonic phosphate enemas daily or every other
day for 1 week.day for 1 week.
2.2. Mineral oil 30-60 mls twice daily between meals to Mineral oil 30-60 mls twice daily between meals to minimize its effect in impairing the absorption of fat minimize its effect in impairing the absorption of fat soluble vitamins -- soluble vitamins -- with the response to stop within with the response to stop within the period of about 6 months.the period of about 6 months.
3.3. Ensure that the child has sufficient fluid and fiber in Ensure that the child has sufficient fluid and fiber in the dietthe diet
4.4. Bulk type of softness (Metamucil) maybe Bulk type of softness (Metamucil) maybe administered as the dose of mineral oil is tapered.administered as the dose of mineral oil is tapered.
5.5. Lactulose, senna, may have to be prescribed to break Lactulose, senna, may have to be prescribed to break the vicious cycle of fecal retention and painthe vicious cycle of fecal retention and pain
Dietary MeasuresDietary Measures Intake of fluidIntake of fluidHigh residual diet, bran – whole wheatHigh residual diet, bran – whole wheatFruit and vegetableFruit and vegetablePrune JuicePrune Juice
Anal DilatationAnal Dilatation Old method – local anesthesia Old method – local anesthesia Well lubricated little finger inserted Well lubricated little finger inserted
into the anus and kept for 1 minuteinto the anus and kept for 1 minute
INFANTSINFANTS
*Treat the cause*Treat the cause Simple constipation … correct diet … add Simple constipation … correct diet … add
fiber… treat anal fissure .. Softening the fiber… treat anal fissure .. Softening the stool to break the cyclestool to break the cycle
Daily stitz bathDaily stitz bath Application of Vaseline ointment after the Application of Vaseline ointment after the
bathbath Anesthetic ointment.Anesthetic ointment.
ManagementsManagements::HxHx OnsetOnset
Associated findingsAssociated findingsFTT FTT organic organic
ExEx AbdominalAbdominalRectalRectalSigns of HypothyroidismSigns of Hypothyroidism
ExplanationExplanation Common problemCommon problemWill get betterWill get better
Physiology of defecationPhysiology of defecation
Long term managements up to 1 yearLong term managements up to 1 year
Goals:Goals:
1.1. Empty RectumEmpty Rectum Clean out retained stoolClean out retained stool
EnemaEnema
SuppositoriesSuppositories
SenakotSenakot
Bowel cleaningBowel cleaning
2. Bulky stool2. Bulky stool
High fiber diet (bran)High fiber diet (bran)
3. Improve habits3. Improve habits
Regular time of toilet after Regular time of toilet after feedfeed
Stars chartStars chart
Position- squattingPosition- squatting
De emotionalize home De emotionalize home environmentenvironment
Functional ConstipationFunctional Constipation::
WHY MAY THE TREATMENT FAIL ?!WHY MAY THE TREATMENT FAIL ?!
Inadequate clean out Inadequate clean out suppositories suppositories Inadequate dosage of medicationInadequate dosage of medication On and off approach:On and off approach:
Intermittent use of medicationIntermittent use of medication
MalabsorptionMalabsorption
Objectives:Objectives: DigestionDigestion DefinitionDefinition Identify major causes of Identify major causes of
malabsorptionmalabsorption Know to work up for malabsorptionKnow to work up for malabsorption
MalabsorptionMalabsorption
DigestionDigestion
StomachStomach -------------- breakdown ingested food breakdown ingested food by rhythmic by rhythmic
contraction contraction gastric gastric acids.acids.
Small bowel----Small bowel---- Intestinal secretion Intestinal secretion digestion.digestion.
*Brush Border*Brush Border
Small bowel 2 halvesSmall bowel 2 halves
JejunumJejunum CHOCHO maltose maltoseFatFat F.A. mono glycerides F.A. mono glyceridesProtien..di ,tri, tetra peptides + a.a.Protien..di ,tri, tetra peptides + a.a.MineralsMineralsVitaminsVitaminsWaterWater
Ileum Ileum WaterWaterBB1212
Bile saltsBile salts
Malabsorption Malabsorption SyndromesSyndromes
Malabsorptive Malabsorptive disordersdisorders
They are conditions that cause insufficient assimilation of ingested They are conditions that cause insufficient assimilation of ingested nutrients either as a result of mal digestion, or malabsorption.nutrients either as a result of mal digestion, or malabsorption.
General presentation:General presentation:Abdominal distentionAbdominal distentionPallorPallorFoul smelling stoolsFoul smelling stoolsBulky stools.. Normal… greasy…steatorrheaBulky stools.. Normal… greasy…steatorrheamuscle wastingmuscle wastingPoor weight gain … weight lossPoor weight gain … weight lossGrowth retardationGrowth retardation
Clues:Clues:Acute diarrhea --- infectionAcute diarrhea --- infectionchronic diarrhea > 3 weekschronic diarrhea > 3 weeks ? Malabsorption? Malabsorption
Blood,FTTBlood,FTT Overall appearance of the childOverall appearance of the child
I. Malabsorption I. Malabsorption according to age of according to age of
presentationpresentation1. From birth up to two years of age:1. From birth up to two years of age:
– Post infectiousPost infectious– Protein intoleranceProtein intolerance– Congenital microvillus inclusion disease.Congenital microvillus inclusion disease.– Glucose – galactose transport defects.Glucose – galactose transport defects.– Congenital chloride diarrheaCongenital chloride diarrhea– Familial chronic villous atrophyFamilial chronic villous atrophy– Immune deficiency diseaseImmune deficiency disease– Cholestatic liver diseaseCholestatic liver disease– Gluten sensitive enteropathyGluten sensitive enteropathy– Anatomical cause (short bowel syndrome)Anatomical cause (short bowel syndrome)
2. Older children:2. Older children:– Cystic fibrosisCystic fibrosis– Crohn’s diseaseCrohn’s disease– Gluten sensitive enteropathyGluten sensitive enteropathy– Acquired lactose deficiencyAcquired lactose deficiency
II. Malabsorption II. Malabsorption according to according to substancessubstances::1.1. FatFat
Celiac diseaseCeliac diseaseCystic fibrosisCystic fibrosisOTHERS:OTHERS:
GiardiasisGiardiasisCongenital intestinal malformationCongenital intestinal malformationCrohn’s diseaseCrohn’s diseaseBiliary atrasiaBiliary atrasiaLiver cirrhosisLiver cirrhosis
22..CarbohydrateCarbohydrate
Glucose -galactose transportGlucose -galactose transportdefectdefectGeneticGenetic Lactose )Primary lactase deficiency)Lactose )Primary lactase deficiency)
Sucrose )Congenital sucrase – Sucrose )Congenital sucrase – isomaltase deficiency) isomaltase deficiency)
SecondarySecondaryceliac diseaseceliac diseaseany chronic diarrheaany chronic diarrhea
3. Protein3. Protein
Follow any chronic diarrheaFollow any chronic diarrhea
Enzyme deficiency (trypsinogen)Enzyme deficiency (trypsinogen)
Allergy… Cow’s milk proteinAllergy… Cow’s milk protein
4. Vitamins4. Vitamins
Fat soluble Vit. Secondary to Fat soluble Vit. Secondary to steatorheasteatorhea
BB1212 … Crohn’s … Crohn’s
III. Malabsorption III. Malabsorption according to site of according to site of
defects.defects.1.1. MucosalMucosal Small intestinal injuries resulting in secondary Small intestinal injuries resulting in secondary
deficiencies.deficiencies.- - Celiac diseaseCeliac disease- Bacterial over growth- Bacterial over growth- Cow’s milk enteropathy- Cow’s milk enteropathy- Giardiasis- Giardiasis- Rota virus infection- Rota virus infection- Chronic protracted diarrhea- Chronic protracted diarrhea- Protein energy malnutrition- Protein energy malnutrition- Crohn’s disease of small intestine- Crohn’s disease of small intestine- Short bowel syndrome- Short bowel syndrome- Drugs …neomycin … colchicines- Drugs …neomycin … colchicines- Radiation enteritis- Radiation enteritis
2. None mucosal2. None mucosalPancreatic diseasePancreatic disease ------ cystic ------ cystic
fibrosisfibrosis
Bile lossesBile losses ---- bacterial ---- bacterial overgrowthovergrowth
deconjugation of bile acids deconjugation of bile acids
CholestasisCholestasis
Lymphatic obstruction------Lymphatic obstruction------lymphagiactasialymphagiactasia
A Beta–lipo proteinemiaA Beta–lipo proteinemia
How to approachHow to approach::History:History:
Is he/she thrivingIs he/she thriving Weight/heightWeight/height AppetiteAppetite Other symptoms --- edemaOther symptoms --- edema Repetition of symptoms with reintroduction of certain foods Repetition of symptoms with reintroduction of certain foods
e.g. e.g. GlutenGluten
LactoseLactoseCow’s milkCow’s milk
StoolStool GreasyGreasy
Oil slik in the toiletOil slik in the toiletFrothy – sugar intoleranceFrothy – sugar intoleranceSmell --- Rancid, CFSmell --- Rancid, CF
-----foul, celiac-----foul, celiac
ExaminationExaminationGrowth chartGrowth chart
PallorPallor--- Iron, folate--- Iron, folate
ClubbingClubbing CF, IBD, CDCF, IBD, CD
Chest problemChest problem CFCF
Distended abdomenDistended abdomen CDCD
Muscle hypotonia + wastingMuscle hypotonia + wasting
Nappy rashNappy rash
Rectal examination )prolapsed)Rectal examination )prolapsed)
Laboratory Laboratory investigationsinvestigations::
1.1. StoolStool1.microscopic examination1.microscopic examination
– Fat droplet (stool + water + Sudan red stain)Fat droplet (stool + water + Sudan red stain)– 72 hours stool collection72 hours stool collection– Stool Giardia CystStool Giardia Cyst
ELISA for Giardia antibodies.ELISA for Giardia antibodies.
2. Stool PH 2. Stool PH 5.6 (CHO) 5.6 (CHO)
3. Spot 3. Spot antitrypsin level ( 2 days stool) antitrypsin level ( 2 days stool)
4. Stool reducing substance4. Stool reducing substance– Chromatography Chromatography – Clintest tabClintest tab
10 drops water + 5 drops stool+tab10 drops water + 5 drops stool+tab
(-ve -- 4+) Color coding 2 + or (-ve -- 4+) Color coding 2 + or suggest CHO suggest CHO
malabsorptionmalabsorption
Sucrose is not reducing substanceSucrose is not reducing substance
Should be hydrolyzed by HCl before analysisShould be hydrolyzed by HCl before analysis
5. Stool osmolarity (stool electrolyte content)5. Stool osmolarity (stool electrolyte content)
290 - 2(Na + K ) meq/L290 - 2(Na + K ) meq/L 100 mosm/L 100 mosm/L secretary as in cong chloride secretary as in cong chloride
diarrheadiarrhea 100 mosm/L 100 mosm/L Osmotic diarrhea Osmotic diarrhea CHO CHO
intoleranceintolerance
22 . .BloodBlood CBC— blood film-------CBC— blood film------- A canthocyteA canthocyte
hypochromic, microcytic anemiahypochromic, microcytic anemiamacrocytosismacrocytosis
Serum albuminSerum albumin Serum immunoglobulinsSerum immunoglobulins Trypsinogen (screening)Trypsinogen (screening) Nutrients in bloodNutrients in blood
Iron .. Transferrin concentrationIron .. Transferrin concentrationfolic acid –---- RBCs concentration ,folic acid –---- RBCs concentration , not serumnot serumCa, Mg, Vit D., Vit ACa, Mg, Vit D., Vit ABB12 12 -- bile salt -- bile salt
Vit E + Serum lipidsVit E + Serum lipidsVit K ( PT , PTT)Vit K ( PT , PTT)
3. Others3. Others
Sweat chloride testSweat chloride test Hydrogen breath testHydrogen breath test D-xylose testD-xylose test Schilling test (Vit BSchilling test (Vit B1212))
4. Diagnostic Procedure:4. Diagnostic Procedure:
1.1. Small bowel biopsy:Small bowel biopsy:
1. Duodenal mucosa1. Duodenal mucosaGiardia trophozoiteGiardia trophozoiteColony count of bacteriaColony count of bacteriaCulture of proximal juiceCulture of proximal juice
2. For HIV2. For HIVparasitic bacteria or virusparasitic bacteria or virusviral opportunistic entral pathogenviral opportunistic entral pathogen
3. Gluten sensitive enteropathy3. Gluten sensitive enteropathyChallenge testChallenge test
4. Congenital microvillous inclusion disease4. Congenital microvillous inclusion disease5. Eosinophillic gastroenteritis5. Eosinophillic gastroenteritis6. Infectious disorders6. Infectious disorders
2. Imaging procedure2. Imaging procedureX-ray – site of colon - e.g. intestinal mal X-ray – site of colon - e.g. intestinal mal
rotationrotation
Ultra sound )biliary tree.. Pancreas)Ultra sound )biliary tree.. Pancreas)
Barium contrast meal and follow throughBarium contrast meal and follow through
Barium enemaBarium enema
Review of some Review of some disordersdisordersI. Disorders of CHO intoleranceI. Disorders of CHO intolerance
SymptomsSymptomsFlatulence, Diarrhea, bloating, vomiting, abdominal Flatulence, Diarrhea, bloating, vomiting, abdominal
cramping,cramping, barborygmi barborygmi
A. Congenital DisordersA. Congenital Disorders Lactose deficiencyLactose deficiency
cong )rare)cong )rare) AcquiredAcquiredTXTX Lactose hydrolyzed milk. Lactose hydrolyzed milk.
Lactid tabLactid tab
Sucrase deficiency:Sucrase deficiency:Recurrent watery diarrhea, stool PH below 6Recurrent watery diarrhea, stool PH below 6
Glucose – Galactose malabsorption.Glucose – Galactose malabsorption.Very rareVery rare neonatesneonates severe severe
hypoglycemia – acidosishypoglycemia – acidosisTXTX fructose formula fructose formula
B. Acquired disorders:B. Acquired disorders: Secondary to acute viral OR bacterial Secondary to acute viral OR bacterial
gastro enteritis…. lactase def > sucrasegastro enteritis…. lactase def > sucrase Generally children tolerate lactose withen Generally children tolerate lactose withen
(3w-3m) (3w-3m) Stool PH 6Stool PH 6+ve clinitest tab+ve clinitest tab
Secondary to celiac disease, Crohn’s Secondary to celiac disease, Crohn’s disease, ulcerative colitisdisease, ulcerative colitisTX – lactose free dietTX – lactose free diet
II. Disorders of II. Disorders of ProteinsProteins
Cow’s milk protein sensitivityCow’s milk protein sensitivityA topic individualsA topic individualsJejunal atrophyJejunal atrophyColonic bleedingColonic bleedingIron deficiency anemia unresponsive to Iron deficiency anemia unresponsive to treatment with oral iron.treatment with oral iron.
TXTX D/C milk D/C milk Repeat challenge with carefully increasing Repeat challenge with carefully increasing dose majority will tolerate milk by two years.dose majority will tolerate milk by two years.40% intolerant to Soya protein.40% intolerant to Soya protein.
Celiac diseaseCeliac diseaseAllergy to Gliaden portion of gluten Allergy to Gliaden portion of gluten
(wheat, Rye, Oats or barley)(wheat, Rye, Oats or barley)
– Screening antibodiesScreening antibodies
Antigliadin IgA, Anti reticulin, Anti Antigliadin IgA, Anti reticulin, Anti endomyseal EMAendomyseal EMA
Celiac Sprue:Celiac Sprue: Gluten sensitive enteropathyGluten sensitive enteropathy
C/PC/P Abdominal distentionAbdominal distention Weight loss –FTTWeight loss –FTT
irritabilityirritability VomitingVomiting SteotorrheaSteotorrhea
mild cases,mild cases, IDA,,, short stature ,,,delayed pubertyIDA,,, short stature ,,,delayed puberty
Investigation:Investigation: Serum Iron Serum Iron folic acid folic acid albumin albumin
Flat Glucose tolerance test Flat Glucose tolerance test EMA +ve,, ,+ve EMA +ve,, ,+ve Antigliadin Antigliadin
Small bowel biopsySmall bowel biopsy 100% diagnostic100% diagnostic
- villous atrophy on a gluten free diet- villous atrophy on a gluten free diet
- Recurrence after a gluten challenge- Recurrence after a gluten challenge
TreatmentTreatment:: Gluten free diet for life Gluten free diet for life
PrognosisPrognosis:: Strict diet Strict diet - Normal growth – health- Normal growth – health
- 1% lymphoma- 1% lymphoma
Causes of flat small Causes of flat small intestinal mucosa:intestinal mucosa:
1.1. Celiac diseaseCeliac disease
2.2. Cow’s milk protein sensitivityCow’s milk protein sensitivity
3.3. Post enteritis syndromePost enteritis syndrome
4.4. GiardiasisGiardiasis
5.5. Tropical sprueTropical sprue
6.6. Soya protein intoleranceSoya protein intolerance
7.7. Autoimmune enteropathyAutoimmune enteropathy
8.8. Protein energy malnutritionProtein energy malnutrition
Mucoviscidosis Mucoviscidosis Cystic fibrosisCystic fibrosis
ARAR Defect:Defect: Mucus secreted by exocrine glands is Mucus secreted by exocrine glands is
abnormally thick and sticky due to defect in abnormally thick and sticky due to defect in chloride permeability which lead to failure in chloride permeability which lead to failure in maintaining the luminal hydration.maintaining the luminal hydration. It is an obstructive lesion in which a duct or It is an obstructive lesion in which a duct or airway passage is blocked by intra luminal airway passage is blocked by intra luminal mucous and other protein.mucous and other protein.
60% presents before 1 year.60% presents before 1 year. 85% before 85% before 5 years5 years
C.PC.P.. Most presented with failure to thrive Most presented with failure to thrivefrequent foul smelling stoclfrequent foul smelling stoclRectal prolapse 18.5% Rectal prolapse 18.5% by 1-2 Yr.by 1-2 Yr.
10%10% muconium illeusmuconium illeus- a plug of muconium in the terminal ileum - a plug of muconium in the terminal ileum
which lead to ischemic which lead to ischemic ulceration and ulceration and peritonitis peritonitis
- delayed passage of muconium in - delayed passage of muconium in neonateneonate
10%10% Pancreatic fibrosisPancreatic fibrosisExocrine insufficiency Exocrine insufficiency
malabsorptionmalabsorption
80%80% Recurrent chest infectionRecurrent chest infectionAtelactasisAtelactasis Bronchiactasis Bronchiactasis
Emphysema Emphysema CorpulmonalCorpulmonal
deathdeath
RareRare BoysBoys AzospermiaAzospermia infertilityinfertility
Liver Liver obstructive jaundiceobstructive jaundicebiliary cirrhosisbiliary cirrhosisGallbladder Gallbladder Gall stonesGall stones
Investigation:Investigation:1.1. 24 hour stool chymotripsin24 hour stool chymotripsin2.2. True test for pancreatic function : by low True test for pancreatic function : by low
enzyme output from Pancreas, follow enzyme output from Pancreas, follow stimulation by secretin and cholecytokininstimulation by secretin and cholecytokinin
3.3. Sweat test. (Sweat chloride measurement)Sweat test. (Sweat chloride measurement) 60 meq/L --------60 meq/L -------- diagnosticdiagnosticIf doubtful as is cases of edema , sweat If doubtful as is cases of edema , sweat test should be repeated once edema subsidetest should be repeated once edema subside
Treatment:Treatment:Enteric coated – acid resistant oral pancreatic Enteric coated – acid resistant oral pancreatic enzyme supplementsenzyme supplementsDiet high in protein, low fat or MCT dietDiet high in protein, low fat or MCT diet
CYSTIC FIBROSISCYSTIC FIBROSISA.R.A.R.
Exocrine gland abnormalitiesExocrine gland abnormalities
Electrolyte transport defect + inspissated mucousElectrolyte transport defect + inspissated mucous
SweatSweat SalivarySalivary PancreaticPancreatic Pancreas Pancreas Lung Lung Liver Liver vas vas GlandsGlands gland gland duct duct deference deference
BronchialBronchial
Obstruction ObstructionObstruction Obstruction and infectionand infection
Defective Na + cl Defective HCODefective Na + cl Defective HCO33 Pancreatic fibrosis Pancreatic fibrosis Biliary duct Biliary ductmalemale
AbsorptionAbsorption SecretionSecretion Obstruction infertility Obstruction infertilityBy glandBy gland Chronic Chronic
ObstructiveObstructive lung diseaselung disease
PancreaticPancreatic Biliary cirrhosis Biliary cirrhosis insufficiencyinsufficiency
Cor pulmonaleCor pulmonale
mal absorption muconiummal absorption muconium ilieusilieus
Inflammatory bowel diseaseInflammatory bowel disease
A. Croh’s disease:A. Croh’s disease: Regional enteritisRegional enteritis
Transmural inflammation with skip areasTransmural inflammation with skip areasGenetic predispositionGenetic predisposition
C/PC/P Post prandial abdominal pain . ± diarrhea or constipationPost prandial abdominal pain . ± diarrhea or constipationweight lossweight loss feverfeverarthralgia /arthritisarthralgia /arthritis 2% Erythema nodosum2% Erythema nodosum
Investigations:Investigations: ESR ESR AnemiaAnemia albumin albumin iron iron:: ProctoscopyProctoscopy
Double contrast colon X-rayDouble contrast colon X-rayRectal biopsyRectal biopsy
Treatment:Treatment: Enteral or parentral nutritionEnteral or parentral nutritionMedicineMedicine - Prednisone- Prednisone
- Sulfasalazine )if colonic involvement)- Sulfasalazine )if colonic involvement)- Azathoprine- Azathoprine- Cyclophosphamide- Cyclophosphamide-70% surgical treatment-70% surgical treatment
B. Ulcerative colitisB. Ulcerative colitis
Mucosal inflammation ---rarely involve muscularis layerMucosal inflammation ---rarely involve muscularis layer Toxic mega colonToxic mega colon
C/PC/P bloody diarrhea + tenesmusbloody diarrhea + tenesmusComplicationComplication HgeHge Mega colonMega colon
intractable symptomsintractable symptomsArthalgiaArthalgia / arthritis/ arthritishigh risk of colonic cancerhigh risk of colonic cancer
Treatment:Treatment: Enteral and parentral nutritionEnteral and parentral nutritionmedical: medical: - Sulfasalazine oral- Sulfasalazine oral
- hydro cortison enema- hydro cortison enema - systemic predisone- systemic predisone - Cyclo sporins- Cyclo sporins
25% surgical treatment 25% surgical treatment colectomy if mega coloncolectomy if mega colon
Thank YouThank You