Diseases of the gallbladder and pancreas in children

Diseases of the gallbladder and bile ducts

 * gallbladder anomalies – accompany bile duct atresia or cystic fibrosis
 * acute gallbladder hydrops – during generalized infections, sepsis or Kawasaki disease
 * manifestations – fever, jaundice, more palpable gallbladder
 * treatment – conservative, surgery is indicated only in gangrene of the gallbladder
 * cholecystitis – G+ (streptococci) but also G- bacteria (salmonella, shigels), parasites (ascaris, giardia)
 * sometimes it is a complication of periarteriitis nodosa and other vasculitis or in Kawasaki disease
 * possibly also in Wilson's disease
 * therapy - ATB, good hydration
 * cholelithiasis – is relatively rare in children
 * 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


 * pancreas divided Pancreas divisum.png
 * 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

 * isolated deficits of pancreatic enzymes - rare
 * Johanson–Blizzad syndrome – congenital pancreatic insufficiency, deafness, microcephaly, skull skin defects, hypothyroidism, dwarfism and nasal wing aplasia
 * Schwachman-Diamond Syndrome – the most common congenital secretion disorder
 * 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)
 * i při jiných NPB, chorobách žlučových cest, některých systémových stavech (popáleniny, úrazy hlavy, renální insuficience, MAc, rejekce transplantátu)
 * akutní pankreatitida si vyžaduje opakované chirurgické konzilium a úzkou spolupráci s dětským chirurgem
 * terapie: specifická terapie neexistuje
 * symtomatická: rehydratace, tlumení bolesti, tlumení pankreatické sekrece a prevence a léčba komplikací
 * hospitalizujeme na JIP a trvale monitorujeme vnitřní prostředí
 * tlumení bolesti – jsou kontraindikovány opiáty, neboť zvyšují tonus Oddiho svěrače, nejvíc používáme tramadol
 * dítěti přerušíme perorální přísun čehokoli (tím snížíme sekreci)
 * dbáme na dostatečnou rehydrataci (monitorujeme CVP), dle akutních změn upravujeme vnitřní prostředí
 * neustále odsáváme žaludeční a duodenální obsah sondou
 * prognóza akutní hemoragická p. má stále až 50% mortalitu
 * vážné prognostické ukazatele – vysoká leukocytóza (nad 16.109/l), neúměrná glykémie, vysoká hodnota LDH a aminotransferáz, hypoklacémie pod 2 mmol/l, hypoxie a resp. insuficience
 * akutní bez hemoragie se obvykle upraví při konzervativní léčbě v průběhu 2–4 dnů, ale rekonvalescence je dlouhá (0,5–2 roky dodržovat přísnou dietu)

Chronická pankreatitida

 * vzniká nejčastěji na genetickém podkladě – jako familiární chronická pankreatitida
 * etiologie: AD, penetrace je vysoká (postihne všechny potomky s defektním genem)
 * ale expresivita (stupeň postižení) je velmi různorodá
 * kromě toho se může chronická relapsující pankreatitida vyskytovat i u jiných chorob – u dědičné hyperlipidémie I., IV. a V., při CF, hyperparathyreóze nebo jako následek anomálií slinivky
 * klinický obraz: na začátku jsou příznaky mírně, často jen dyspepsie
 * často začínají na počátku školní docházky a dávají se do souvislosti se změnou stravování (školní jídelna)
 * probíhá formou krátkodobých, několikahodinových záchvatů
 * záchvaty se stávají těžšími
 * postupně vznikne chronická insuficience pankreatu – trpí trávení tuků – první steatorea, pak obraz malabsorpčního sy
 * diagnóza: pečlivá RA
 * při záchvatu můžeme zjistit biochemické změny pankreatitidy
 * mimo záchvaty můžeme zjistit kalcifikace pankreatu a vývoj pseudocyst
 * dif. dg: musíme stanovit chloridy v potu, vyšetřit sérové lipoproteiny (vyloučit hyperlipoproteinémie), koncentrace Ca a P, příp. testy na hyperparathyreózu, vyšetříme stolici na vajíčka ascaris
 * terapie: substituce enzymů, dietní opatření (zákaz alkoholu a toxinů, dostatečný přísun bílkovin, omezení tuků na 35 % E příjmu, omezení jednoduchých sachridů, preference vlákniny (pokud pankreas úplně neselhává), jíst častěji menší dávky