mainly in obese girls during puberty or after repeated hemolysis (pigment stones) - in our country most often after spherocytosis, in black people after sickle cell disease)
may also be in CF, Wilson, congenital bile abnormalities. pathways, in IgA deficiency
clinical picture – nausea, vomiting, jaundice, fever, often colic-like abdominal pain with radiation back and under the right scapula or under the right shoulder
therapy – surgical, for colic - hydrate well, strict diet and give short-term antispasmodics resp. analgesics (Algifen)
Extrahepatic atresia of the bile ducts
absence of extrahepatic bile ducts
incidence - 1:14 000 births
It is thought to be the result of a sclerosing inflammatory process that begins fetally, at delivery or immediately after, etiology unknown
Newborns are usually born out of a normal pregnancy and are fine immediately after birth
atresia manifests at the age of several days, max. weeks - cholestatic syndrome , ie prolonged jaundice with a change in the color of urine (dark) and stool (light, acholic)
percutaneous liver biopsy is important , but there are no typical histological findings that would distinguish it from other liver diseases
obstruction can be verified isotopically or by ERCP
surgery - in all infants in whom we have ruled out other liver diseases
hepatoportoenteroanastomosis is most often used (Kasai's operation) - anastomosis between the hepatic porta and the intestine is established
Diseases of the pancreas
The most common pancreatic disease in children is cystic fibrosis
otherrs are quite rare
diagnosis - examination of pancreatic secretion - collection of duodenal juice after secretion stimulation (demanding and burdensome)
screening test for pancreatic lipase secretion disorder - examination of fat droplets in stool
PABA test - we administer a peptide from which chymotrypsin cleaves PABA (paraaminobenzoic acid) - it is absorbed and excreted in the urine
Congenital anomalies of the pancreas
annular pancreas
it is formed by incomplete rotation of the ventral part, the pancreas revolves around the duodenum and jejunum and makes a typical picture of congenital GIT obstruction
more common in Down syndrome
symptoms - see stenosis
divided pancreas
is one of the most common GIT anomalies, mostly it causes only minimal problems or is detected by chance
the incidence is estimated at 10% of the population
the result of the disorder is that part of the pancreas (cauda, corpus and part of the head) does not secrete into the ductus Wirsungi, but through the ductus Santorini
the anterior part may be compressed and chronic pancreatitis may develop
ectopic pancreatic tissue
It can be localized in the small intestine or stomach, usually does not cause problems
sometimes it can cause bleeding, partial obstruction or a intussusception (ie insertion of a part of the intestine into the adjacent part of the intestine)
Congenital disorders of pancreatic excretory function
AR, incidence 1:20 000, pancreatic tissue is infiltrated with fat
clinical picture – malabsorption syndrome, growth and development retardation
reminds of CF but without finding chlorides in sweat, there are usually more associated congenital diseases
neutropenia (neutrophils show loss of chemotaxis), metaphyseal dysplasia → growth disorders
growth disorders persists even with pancreatic replacement. enzymes (unlike CF)
neutrophilia is cyclical, leads to frequent bacterial infections and often to lethal sepsis
diagnosis - malabsorption, skeletal changes, disorders of pancreatic secretion, normal Cl concentration in sweat
on CT is a hypodense pancreas
therapy - enzyme substitution, consistent and early treatment of bacteria. infections, on neutropenia - G-CSF
prognosis - severe
Acute pancreatitis
it can accompany or complicate many general childhood diseases
etiology: infections - parotitis virus, VHA, coxsackie, pancreas is also sensitive to many toxins and drugs
in adolescents it can occur after alcohol and drugs, in all children after some immunosuppressants or antimicrobial drugs (tetracyclines, sulfonamides ), diuretics, antiepileptics, Au preparations
occasionally occurs in congenital bile duct and pancreatic disorders, CF, Rey's syndrome and MODS
from systemic diseases - most often in - CF, DM, binder diffusion disorders, Rey's sy, Kawasaki
trauma and obstruction of the bile ducts can also be the cause
pathogenesis: the effect of occlusion of the leads by inflammation, which is caused by a given etiological factor, is presumed, followed by activation of enzymes and escape into the stroma - the most important is the activation of trypsin and chymotrypsin
autoactivation is controlled by enterokinase, bile regurgitation
stasis destroys protease inhibitors, which otherwise prevent activation in the pancreas
necrosis with calcifications and saponification occurs, the process penetrates into the environment
in a very severe course, there is a risk of circulatory shock and ARDS
clinical picture: very acute pain
sudden pain in the epigastrium, the patient locates it in depth, as a fickle, practically permanent pain, radiates to the back (when the cauda is affected, it may radiate to the left shoulder with irritation of the diaphragm)
vomiting, fever, tachycardia, tachypnoea, sweating and hypothesia prone to collapse
the pain is extremely intense, the child takes a crouched position, attracts the limbs to the abdomen, usually lies on his side, prevents any change of position and any examination
the abdomen is hard to the touch, dilated, arched above the level of the chest, sometimes resistance can be felt in the depths of the left epigastrium
the pain worsens, the peristalsis is weakened or absent
hypocalcaemia with latent or overt tetany may result from salt deposition
the most serious form – acute hemorrhagic pancreatitis – is rare in childhood, may be in adolescents after drug or alcohol excess, jaundice, ascites, shock, acute renal insufficiency, DIC, gastrointestinal bleeding, respiratory disorders
laboratory: vomiting leads to dehydration, hyponatremia, hypochloraemia and potassium depletion, hypocalcaemia is characteristic
in serum and urine the value of amylases increases within 2–12 hours (decreases in 24–48 hours) - predominance of pancreatic isoenzymes
increase in serum lipases - with a threefold increase, this value is more specific and sensitive than amylases
rising ALT , AST , LDH
leukocytosis and hyperglycemia
diagnosis: hyperamylasemia is really important
imaging methods - especially valuable - abdominal sono and CT will provide the most data - pancreatic enlargement, hypoechogenic edematous pancreas, presence of pseudocysts filled with fluid or blood, duct dilatation, sometimes purulent abscesses
X-ray - non-specific findings - elevation of the left diaphragm arch, small pleural effusion on the left, smaller infiltrates at the base of the lungs or pulmonary atelectasis
dilated colon transverse, calcification in the pancreas
ERCP in recurrent pancreatitis
differential diagnosis: other causes of pain - appendicitis, peptic ulcer, intestinal obstruction, cholecystitis
other causes of elevated amylases - parotid disease ( mumps , EBV , CMV , HIV , sialoadenitis ), eating disorders (anorexia, bulimia)
biliary tract diseases, some systemic conditions (burns, head injuries, renal insufficiency, MAc, transplant rejection)
Acute pancreatitis requires repeated surgical consultation and close cooperation with a pediatric surgeon
therapy: no specific therapy
symptomatic: rehydration, pain relief, pancreatic secretion suppression and complication prevention and treatment
patients are hospitalized in the ICU and constantly monitored
pain relief - opiates are contraindicated because they increase the tone of Oddi's sphincter, we use tramadol the most
we stop the oral supply of anything to the child (this will reduce the secretion)
we pay attention to rehydration (we monitor CVP), we adjust the internal environment according to acute changes
we constantly aspirate gastric and duodenal contents with a probe
the prognosis of acute hemorrhagic pancreatitis still has up to 50% mortality
serious prognostic indicators - high leukocytosis (above 16.10 9 / l), disproportionate glycemia, high value of LDH and aminotransferases, hypoclacemia below 2 mmol / l, hypoxia and resp. insufficiency
acute without hemorrhage usually resolves during conservative treatment within 2–4 days, but convalescence is long (0.5–2 years following a strict diet)
Chronic pancreatitis
most often on a genetic basis - such as familial chronic pancreatitis
etiology: AD, penetration is high (affects all offspring with a defective gene)
but the expressiveness (degree of disability) is very diverse
in addition, chronic relapsing pancreatitis can occur in other diseases - inherited hyperlipidemia I., IV. and V., in CF, hyperparathyroidism or as a result of pancreatic anomalies
clinical picture: at first the symptoms are mild, often just dyspepsia
they often start at the beginning of school and are related to a change in diet (school canteen)
takes place in the form of short-term, several-hour seizures
seizures become more severe
chronic pancreatic insufficiency gradually develops - it suffers from fat digestion - first steatorrhea, then a picture of malabsorption syndrome
diagnosis: careful family anamnesis
during the attack we can detect biochemical changes in pancreatitis
outside seizures we can detect pancreatic calcification and the development of pseudocysts
differential diagnosis: we must determine chlorides in sweat, examine serum lipoproteins (exclude hyperlipoproteinemia), Ca and P concentrations, or tests for hyperparathyroidism, we examine the stool for ascaris eggs
therapy: enzyme substitution, dietary measures (prohibition of alcohol and toxins, adequate protein intake, fat reduction to 35% intake, reduction of simple carbohydrates, fiber preferences (if the pancreas does not fail completely), eat smaller doses more often
