This document contains a morning report from a pediatric case involving a 5-year old Saudi girl presenting with abdominal distension, eye puffiness, and loose stool over 8 days. Her initial impression was likely protein losing enteropathy. Investigations revealed hypoalbuminemia and ascites. Imaging showed bilateral pleural effusion and bowel wall thickening. She was ultimately diagnosed with primary intestinal lymphangiectasia based on endoscopy findings. The report discusses protein losing enteropathy causes, pathophysiology of primary intestinal lymphangiectasia, clinical presentation, diagnosis, and management focusing on a low-fat diet with medium-chain triglyceride supplementation.

1. Morning Report
Long Case
13-12-2010
Prepared by:
Dr.Yassin M Alsaleh
Supervised by:
Dr.Maher Al hatlani

2. ‫بسم ا الرحمن‬
‫الرحيم‬
‫)وفي أنفسكم‬
‫أفل تبصرون(‬

3. 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.

4. 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 .

5. 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.

6. 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.

7. 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.

8. 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.

9. 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

10. 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.

11. PHOTOS

12. PHOTOS

13. 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.

14. PLAN OF CARE

15. INVESTIGATION
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•
•
•
•
•
•
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
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


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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%

16. INVESTIGATION
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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

17. IMAGING
• ECHO: normal.
• AXR: ascities.
• Abdominal us: bilateral pleural
effusion. Ascities. Bowel wall
thickness. Normal kidney, spleen
and liver.
• CT abdomin:

18. IMAGING
• ENDOSCOPY:

19. FINAL
IMPRESSION:
Protein loosing
enteropathy
mostly due to
primary intestinal
lymphangiectasia

21. 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.

22. Protein-Losing Enteropathy
causes
causes
PLE
lymphatics
primary
secondary
intestinal
mucosal
submucosal

23. 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

24. 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

25. 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.

26. 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.

27. Clinical presentation
• Edema.
• Diarrhea or steatorrhea.
• FTT.
• Reversible blindness.
• Tetany.

28. diagnosis

29. abnormal mucosal
pattern with scattered white plaques

30. Sub epithelial
Dilated lacteals

31. 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.

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