Pancreatita Familiala Vs Ereditara

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Case report

Resident doctor: Teodorescu Casandra

01.11.2018
P.V., male, age 16
• Addmited for upper abdominal pain, nausea, vomiting, dyspnoea, tachypnea

• No family history

• Medical history : 2 episodes of acute pancreatitis

• No smoking or alcohol consumption

• No medication previously

• Clinical examination – cachexia


• Lab results
Hematology
Hemoglobin = 10,9 g/dl (12- AP= 560 U/l (100-300)
14,6) AST= 67 U/l (5-45)
ALT= 51 U/l (5-45)

MCV =73 (80-96) Amylase=240 U/l (30-100)


MCHC = 31,5(31-37) Lipase=185 U/l (0-60)
Total serum iron = 32 (65-175)

• Abdominal ultrasound

– Liquid collection in the omental bursa, of about 60 mm, with its own
wall; bilateral pleural effusion
First diagnosis
• Reccurent acute pancreatitis

• Hepatic cytolisis syndrome

• Iron deficiency anemia


• CT scan with contrast
• CT scan with contrast
Chronic pancreatitis

progressive
exocrine and
replacement endocrine
of the pancreatic pancreatic
parenchyma by insufficiency
recurrent fibrous connective
inflammatory tissue
episodes
• 50-66% of reccurent acute pancreatitis → chronic pancreatitis

• Severe first acute pancreatitis episode → 9 x higher risk for


chronic pancreatitis

• SPINK1 mutations → higher risk for chronic pancreatitis


• Diagnosis - via high quality imaging
modalities:

• increased density of the parenchyma,


• atrophy of the gland
• calcification,
• pseudocysts
• irregularities of the main pancreatic duct and
its side branches
Chronic pancreatitis

• Alcohol and smoking abuse


• Ductal obstruction
• Autoimmune pancreatitis
• Idiopathic pancreatitis
• Genetic causes

HBV, HCV, HIV nonreactive

No gene mutations in PRSS1, SPINK1


SHOULD PSEUDOCYSTS BE TREATED?
• Indications

– Symptoms

– Complications (infections, bleeding, rupture)

– Compression of surrounding organs (gastric, duodenal, biliary obstruction)

• How?

– Endoscopic / percutaneous/ surgical


Endoscopic treatment
• Recommended for chronic pancreatitis
pseudocysts whenever is possible

1. Transmural drainage under EUS guidance

2. Transpapilary drainage – direct


communication between the PPC and MPD
• ULTRASOUND ENDOSCOPY (under
orotracheal intubation)

– Aspiration of the caudal pseudocyst

FEVER!
• ERCP : Wirsung duct stenting – infected liquid

• Antibiotics:

Meropenem+Metronidazole
Final diagnostic
• PANCREATIC PSEUDOCYST THAT COMMUNICATES WITH THE
PANCREATIC DUCT AND EXTENDS CLOSE TO MEDIASTINUM.
TRANSPAPILLARY DRAINAGE.

• LITHIASSIS OF WIRSUNG’S DUCT. ENDOSCOPIC STENTING(plastic)

• SEVERE CHRONIC PANCREATITIS – HEREDITARY?

• HEPATIC CYTOLISIS SYNDROME

• Treatment: pancreatic enzymes, antialgics when needed, hepatic


protectors, 1 month reevaluation
REEVALUATION
• Abdominal ultrasound

– Lithiasis of Wirsung’s duct with enlargement of 14


mm

• ERCP

– Restenting
ONE YEAR REEVALUATION
• Lab results

– Elevated AP

• Abdominal ultrasound

– Wirsung duct enlarged, with lithiasis; visible stent


Colangio MRI
Colangio MRI
Surgery
Familial pancreatitis

Vs.

Hereditary pancreatitis
Chronic hereditary pancreatitis

Rosendahl J, Bödeker H, Mössner J, Teich N. Hereditary chronic pancreatitis. Orphanet Journal of Rare Diseases.
2007;2:1. doi:10.1186/1750-1172-2-1.
REEVALUATION AFTER SURGERY
• No symptoms, weight gain

• Lab results
– No significant changes

• Abdominal ultrasound
– The main pancreatic duct can not be identified
Prognosis
• Increased risk for developing pancreatic cancer
→ higher mortality rate

• Progress to pancreatic insufficiency and/or


diabetes mellitus

• Avoid consuming alcohol, smoking cigarretes,


limit dietary fat and try to minimize emotional
stress
Relevance of the case
• Young patient

• Multidisciplinary team involvement

• The need of surgical treatment, after multiple


endoscopic attempts

• Close follow-up – higher risk for pancreatic cancer


“Early surgery for patients with painful obstructive chronic
pancreatitis provides better pain control with fewer interventions
compared with the current step-up approach, including endoscopy,
but pancreatic function and quality of life are comparable,” concluded
Marinus Kempeneers.

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