CASE REPORT Dr Veselinka Djurisic - Institute for

Report
• Female infant, 8 month old;
• Admitted to hospital due to:
– Diarrhoea;
– Metabolic disbalance;
– With sings of enteropathy.
History of Present Illness
• 15 min before admission to hospital present
with sudden abdominal cramping, cyanosis of
limbs, she was lethargic, with drooping head.
• Day before admision, she was sleepless,
agitated, inconolably crying, with non bilious,
non-projectile vomiting (4 times/day), and she
had 10 regular stools.
• 9 days before she was addmited to hospital
due to vomiting, diarrhoea and high fever.
Personal hystory:
• Third child from regular pragnancy and term
delivery completed with caesarean section.
• Birth weight: 2830 g; Birth length: 53 cm; AS 9
• Breastfeeded 3 mo, after that continued
adapted milk formula, 1 month later started
mixed non-milk nutrition.
• No history of allergy, regularly vaccinated
Family history
• Older brother – convulsions trated with AET;
• Father – epilepsy;
• Mother – chronic enteropathy in childhood
suggested gluten free diet, but she refused;
• Grandfather – COPD;
Clinical finding
• Weight 7 kg
• Agitated, crying, groaning, dehydrated, afebrile
(36,7⁰C), hemodynamically stable;
• Vital sings: RR 36/min, CF 136/min, spO2 93%;
• Skin: pale, marble, with limbs cyanosis.
• Left torticollis, slight axial hypotonia.
• Normal auscultatory findings of lungs and heart .
• Abdominal examination: abdominal distension ,soft
and nontender, without tumefacts and
organomegaly.
Laboratory findings
Acido Base Balance
Full Blood Count
Coagulation status
PT
16,7 s
10,1
INR
1,31
6,21
5,20
aPTT
21,2 s
HGB
125
108
D – dimer
0,64 mg/l
- 14,2
MCV
64
65
Fibrinogen
2,2 g/l
92,9%
MCH
20
20,7
HCT
40%
34,2%
PLT
835
376
pH
7,240
ESR
3
pCO2
3,65 kPa
WBC
31,4
pO2
8,51 kPa
RBC
HCO3
11,5 mmol/l
BE
sO2
Biochemistry
CRP
0,2
Glucose
3,0
Total protein
52
Albumin
30
Blood urea nitrogen 1,7
Creatinine
24
Sodium
135
Potassium
3,5
Calcium
2,13
AST
42
ALT
32
ALP
326
CK
82
LDH
327
AFP
1,5
Normal urin dipstick
and sediment findings
• Tissue transglutaminase antibody:
– IgG 269;
– IgA > 300;
• Anti – gliadin antibodies:
– IgG 6 ;
– IgA > 300;
Microbiology
• Stool culture, ova and parasite testing,
Rotavirus and Adenovirus: NEGATIVE.
• Stool: positive for Candida sp.
• Urin culture: negative.
Radiology findings
• X-ray plain film
 air-fluid levels
Radiology findings
• Ultrasound revealed mass suspected to
intussusception in right hemiabdomen:
Target sign (also known as the doughnut sign)
Pseudokidney sign
Radiology findings
• Abdominal CT scan reveals dilated and fluidfilled loops of small bowel with air-fluid levels the
classic ying-yang sign of an intussusceptum
inside an intussuscipiens in right hemiabdomen.
• Rectoscopy was performed: reveal normal.
– The lining of the colon appears smooth and pink,
with numerous folds.
– No abnormal growths, pouches, bleeding, or
inflammation is present.
COURSE
• Treated with antibiotics:
– metronidazole
– gentamicin
• Corticosteroids:
– methylprednisolone
• H2 blockers:
– ranitidine
• Transfusion of fresh frozen plasma, 3 times
• After exclusion of acute abdomen, cow
protein free diet was introduced, but without
any improvement.
• Spontaneus desinvagination.
• After obtainig coeliac serology, gluten free diet
has started, occurs clinical improvement with
metabolic stabilisation.
Conclusion
• Case of rare but serious clinical presentation
of celiac crisis.
• It is important to recognize that CD may
present in “crisis.”
• The possible precipitating factors in present
patient are unrecognized coeliac disease,
hypokalemia and previous infection.
Discussion
• Incidence of celiac disease is on rise in
Montenegro.
• Prevalence of CD is found to be........ dopuniti
ukoliko postoje podaci.....
• Celiac crisis is a life-threatening complication of
CD.
• Clinically, it is characterized by severe diarrhea,
dehydration and metabolic disturbances like
hypokalemia, hypomagnesemia, hypocalcemia,
hypoproteinemia and metabolic acidosis.
Definition of celiac crisis
• Acute onset or rapid progression of gastrointestinal symptoms
attributable to celiac disease requiring hospitalization and/or
parenteral nutrition along with at least 2 of the following:
Signs of severe dehydration including: hemodynamic instability
and/or orthostatic changes
Neurologic dysfunction
Renal dysfunction: creatinine >2.0 g/dL
Metabolic acidosis: pH <7.35
Hypoproteinemia (Albumin < 3.0 g/dL)
Abnormal electrolytes including: hyper/hyponatremia,
hypocalcemia, hypokalemia or hypomagnesemia
Weight loss > 10 lbs
Metabolic pathophysiology in celiac
crisis
• Celiac crisis may not respond to a gluten-free diet
alone. In severely ill children with celiac crisis, the
use of corticosteroids may cause dramatic
improvement. *
• Lloyd-Still described 3 cases of celiac crisis
successfully treated with corticosteroids. **
• The role of steroids now is controversial as gluten
free and good nutritional diet are considered
good enough to tide over the crisis ***
* Mihailidi E, Paspalaki P, Katakis E, Evangeliou A. Celiac Disease: A Pediatric Perspective. International Pediatrics 2003;18:141-8.
** Lloyd-Still JD, Grand RJ, Khaw KT, Shwachman H. The use of corticosteroids in celiac crisis. J Pediatr. 1972; 81: 1074-1081.
*** Walia A, Thapa BR. Celiac crisis. Indian Pediatr. 2005; 42: 1169
Grazie per l'attenzione
Saluti da Montenegro

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