Morning Morning Report Report Long Case Long Case 13-12-201013-12-2010

Prepared by: Prepared by: Dr.Yassin M Alsaleh Dr.Yassin M Alsaleh

Supervised by:Supervised by:Dr.Maher Al hatlaniDr.Maher Al hatlani

بسم الله الرحمن الرحيم

)وفي أنفسكم أفال تبصرون(

HISTORY AND PHYSICAL

• Fay is 5 years old saudi girl. • Chief complaint: • Abdominal distension. • Puffiness of eye . For 8 days• Loose bowel motion .

• Informant : father and grand mother.

• Family notice eye puffiness mostly after awaking from sleep , decrease during the day.

• Abdominal distension increasing with time associated with abdominal discomfort.

• Passing 3-5 times loose to semi- formed stool medium to large in amount non mucoid non bloody .

HISTORY AND PHYSICAL

• Hx of URTI 2 weeks ago.

• No hx of fever, urinary symptom , vomiting or jaundice

• No hx of weight loss or night sweat.• No hx of insect bite.• No hx of drug ingestion.

HISTORY AND PHYSICAL

• Perinatal hx: unremarkable.• Past medical and surgical :

unremarkable.• NKA.• Sharing family food (average).• Vaccination: up to date.• Developmental: in KG with

excellent performance.

HISTORY AND PHYSICAL

• Family history:• 1st degree cousin.Only child.• No family history of renal, eye ,

hearing in the family.

• Father worked as administrative in university.

• Mother house wife .• Living in flat in family house with

good income.

HISTORY AND PHYSICAL

• Looks well , not in distress ,not dysmorphic, well hydrated.

Vital sign: Oxygen Saturation 100% RA. Heart rate 110 bpm Respiratory rate 25 bpm Tempreature 36.4 C Blood pressure 90/57. Growth parameter: wt:15.7 kg 10th. Ht:104 cm 5th.

HISTORY AND PHYSICAL

HISTORY AND PHYSICAL

HENT: eye puffiness. CNS: conscious, oriented, normal

power, tone ,reflexes, cranial nerves and gait.

CVS: s1+s2+o CRT<2 sec. RS: vesicular breathing, good air

entry no added sound. Musculoskeletal: no edema . no rash. No lymphadenopathy

Abdomen: distended moving freely , no scar no strie, no dilated vein symmetrical . Soft no tenderness no masses no organomegally , shifting dullness positive. Normal bowel sound.

Normal female genitalia. Urine dip stix: negative.

HISTORY AND PHYSICAL

PHOTOS

PHOTOS

Initial impression• Protien loosing enteropathy :

1- intestinal lymphangictasia.2-celiac disease.3- infectious enteropathy.4- IBD inflammatory bowel

disease.• Nephrotic syndrome.• Liver failur ,heart failure.

PLAN OF CARE

• Basic screen:• BUN 1.8 mmol/l• Creat 31 μmol/L• Na 142 mmol/L• K 3.7 mmol/L• Cl 113 mmol/L• Co2 17 mmol/L

INVESTIGATION Complet blood

count: WBC 10.6 (μ L) Hgb 14.6 g/dl

Hct 44.5 % Plt 508 (μ L) Lymph 16 % Neut 73% Esion 5%

Liver function test:

AST 68 U/L ALT 66 U/L ALK 229 U/L Bili 1.7 umol/l Albumin 16 g/l PT,PTT: WNL

INVESTIGATION• Urine albumin/creat ratio:

WNL• Urine analysis:

negative.

• Lipid profile: WNL

• Stool ph: 6• Stool for reducing

subsetance: negative.• Stool for alfa-1-anti

trypsin : NA.• Stool for fat: normal

• T- TG: Still

• ECHO: normal.• AXR: ascities.• Abdominal us: bilateral pleural

effusion. Ascities. Bowel wall thickness. Normal kidney, spleen and liver.

• CT abdomin:

IMAGING

•ENDOSCOPY:

IMAGING

Protein loosing enteropathy

mostly due to primary intestinal lymphangiectasia

FINAL IMPRESSION:

Protein-Losing EnteropathyProtein-Losing Enteropathy

• Definition: a range of pathophysiologic processes that result in the loss of serum proteins into the GI tract.

• Not confined to intestine.

Protein-Losing Enteropathy Protein-Losing Enteropathy causescauses

PLE

lymphatics intestinal

primary secondary mucosal submucosal

• Primary intestinal lymphangiectasia .• Secondary intestinal lymphangiectasia :

• Constrictive pericarditis • Congestive heart failure • Post Fontan • Malrotation• Lymphoma • Sarcoidosis • Radiation therapy

Protein-Losing Enteropathy Protein-Losing Enteropathy causescauses

• Bowel mucosal :– Infection : (CMV) – Bacterial overgrowth .– Menetrier disease. – Eosinophilic gastroenteritis .

• Intestinal inflammation :– Celiac disease– Crohn disease– Inflammatory bowel disease .– Cow's milk/soy protein allergy

Protein-Losing Enteropathy Protein-Losing Enteropathy Causes Causes

Primary intestinal lymphangiectasia

• due to congenital defects in lymphatic duct formation

• often associated with lymphatic abnormalities elsewhere in the body.

• Lymph rich in proteins and lymphocytes leaks into the bowel lumen, resulting in protein-losing enteropathy and lymphocyte depletion.

• dilatation of intestinal lymphatics.

• loss of lymph fluid into the gastrointestinal (GI) tract.

• This leads to hypoalbuminemia ,edema hypogammaglobulin, lipid loss , ADEK loss and lymphopenia.

Pathogenisis

• Edema.• Diarrhea or steatorrhea.• FTT.

• Reversible blindness.• Tetany.

Clinical presentation

diagnosis

abnormal mucosalpattern with scattered white plaques

Sub epithelial Dilated lacteals

• Supplementing a low-fat diet.• restricting the amount of long-chain

fat .• administering a formula containing

protein and medium-chain triglycerides (MCTs)

• Rarely, parenteral nutrition is required.

• Surgical if localized.

Treatment

• The clinical course is highly variable.• Increased risk of lymphoma.

prognosis