Post on 19-Dec-2014
description
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