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Morning Report
Long Case
13-12-2010

Prepared by:
Dr.Yassin M Alsaleh
Supervised by:
Dr.Maher Al hatlani
‫بسم ا الرحمن‬
‫الرحيم‬

‫)وفي أنفسكم‬
‫أفل 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.
HISTORY AND PHYSICAL
• 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 semiformed 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
 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
HISTORY AND PHYSICAL
 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.
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
INVESTIGATION
•
•
•
•
•
•
•

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










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%
INVESTIGATION








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

• Lipid profile: WNL
• T- TG: Still

• Urine albumin/creat ratio:
WNL
• Urine analysis:
negative.

• Stool ph: 6
• Stool for reducing
subsetance: negative.
• Stool for alfa-1-anti
trypsin : NA.
• Stool for fat: normal
IMAGING
• ECHO: normal.
• AXR: ascities.
• Abdominal us: bilateral pleural
effusion. Ascities. Bowel wall
thickness. Normal kidney, spleen
and liver.
• CT abdomin:
IMAGING

• ENDOSCOPY:
FINAL
IMPRESSION:
Protein loosing
enteropathy
mostly due to
primary intestinal
lymphangiectasia
Protein-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

causes
causes

PLE
lymphatics

primary

secondary

intestinal

mucosal

submucosal
Protein-Losing Enteropathy
Protein-Losing Enteropathy
• Primary intestinal lymphangiectasia .
• Secondary intestinal lymphangiectasia :
• Constrictive pericarditis
• Congestive heart failure
• Post Fontan
• Malrotation
• Lymphoma
• Sarcoidosis
• Radiation therapy

causes
causes
Protein-Losing Enteropathy
Protein-Losing Enteropathy
• 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

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.
Pathogenisis
• 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.
Clinical presentation

• Edema.
• Diarrhea or steatorrhea.
• FTT.
• Reversible blindness.
• Tetany.
diagnosis
abnormal mucosal
pattern with scattered white plaques
Sub epithelial
Dilated lacteals
Treatment
• 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.
prognosis
• The clinical course is highly
variable.
• Increased risk of lymphoma.
protein loosing enteropathy

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