Familial hyperamylasemia

A 7-year-old white boy was referred to us with a history of 3 attacks of hypogastric pain over the previous 2 years and persistently elevated serum amylase concentrations. At physical examination, he was well with no evidence of clinical abnormalities. His weight and height were normal. Laboratory diagnostic investigations were all normal except for the presence of Ascaris lumbricoides in the feces and persistently elevated serum amylase levels. Serum amylase determinations in the family members were normal in his father and maternal grandmother but elevated in his mother, sister, maternal aunt, and uncle, all of whom asymptomatic. Macroamylasemia was excluded in the child and in the mother. The finding of persistently elevated amylasemia in the child and in the other family members spanning 3 generations, and the exclusion of diseases that lead to hyperamilasemia are consistent with the diagnosis of familial hyperamylasemia. Until now, only 1 similar case has been reported. Familial hyperamylasemia must be considered in the differential diagnosis of hyperamylasemias in childhood.

Frequency of Strongyloides stercoralis Infection in Alcoholics

Several studies have shown that chronic alcoholics have increased susceptibility to infections due to higher exposure to infectious agents as well as breakdown in their immune defenses. As Strongyloides stercoralis infection is usually more relevant in immunocompromised patients, the aim of this study was to evaluate the frequency of S. stercoralis infection in alcoholics. Thus, coproparasitological examination was carried out in 145 subjects, from which 45 were chronic alcoholics (mean age of 45.7 ± 11.0 years), 10 were nonalcoholic cirrhotic patients (mean age of 50.2 ± 13.1 years), and 90 were asymptomatic nonalcoholic subjects (mean age of 46.7 ± 10.1 years), which served as controls. From the alcoholics, 9 had hepatic cirrhosis, 9 had chronic pancreatitis and 27 had neither cirrhosis nor pancreatitis. For the diagnosis of strongyloidiasis, the Baermann-Moraes and Lutz methods were used in three fecal samples from each subject. Samples were collected at alternated days, and three slides of each sample were analyzed for each method, thus totalizing 2,610 slides examined. The frequency of strongyloidiasis in the total alcoholic group (33.3%) and in the subgroups of alcoholics, i.e., patients with hepatic cirrhosis (44.4%), with chronic pancreatitis (33.3%), and those with no cirrhosis or pancreatitis (29.6%) was statistically higher than that found in the control group (5.5%). None of the individuals with nonalcoholic hepatic cirrhosis had S. stercoralis infection. Our results showed that the chronic alcoholism itself is an important factor that predisposes to strongyloidiasis.

Síndrome da alça aferente com necrose simulando pseudocisto de pâncreas

Afferent loop obstruction after gastrectomy and Billroth II reconstruction is an uncommon problem. Complete acute obstruction requires emergent laparotomy. We describe a patient who developed acute abdominal pain, hyperamylasemia, and palpable abdominal mass, five years after Billroth II gastrectomy. At laparotomy the patient was found to have a complete stricture of the afferent limb with evidence of strangulation and necrosis. There was no evidence of pancreatitis or pancreatic pseudocyst. The patient underwent pancreaticoduodenectomy plus degastrectomy and died 18 hours after the procedure in the ICU. The mass was initially inte1preted as pancreatic pseudocyst. Ultrasonography may provide enough evidence to differentiate a pancreatic pseudocyst. from an obstructed afferent loop, by the presence of a peripancreatic cystic mass or debris within the mass or the absence of the keyboard sign, suggesting effacement of the valvulae conniventes of the small bowel. Howewer, CT scan of the abdomen has been suggested to be highly characteristic, if not pathognomonic, for an obstructed afferent loop and should be considered first in patients with pancreatitis after Billroth II gastrectomy. A history of previous gastrectomy, recurrent or severe abdominal pain, hyperamylasemia with characteristic tomography, and endoscopic findings will establish the diagnosis and necessitate surgical evaluation and intervention.

Adenoma viloso da ampola de Vater

The aim of this paper is to present a case of a 37-year-old female patient with a benign tumor of the Ampulla of Vater and a brief review of the literature. The patient presented with progressive obstructive jaundice and weigth loss due to the presence of two adenomas of the second portion of duodenum. Laboratory tests confirmed the presence of obstruction of the biliary tree. Ultrasound and CT scan of the abdomen revealed bile duct dilatation. ERCP showed a tumor at the site of the Ampulla of Vater. The biopsies revealed tubular adenoma. She was submitted to local resection of the tumors and sphincteroplasty, since the frozen biopsy at the time of surgery showed no malignancy. During the post-operative follow-up she presented recurrence of symptoms. An upper GI endoscopy revealed a tumor at the Ampulla of Vater. She was then submitted to Whipple procedure with an uneventful recovery.

Hemosuccus pancreaticus: causa rara de hemorragia digestiva maciça

Hemosuccus pancreaticus is a unusual syndrome manifested by hemorrhage into the pancreatic duct and by blood loss through the ampulla of Vater: It may be caused by tumors, arteriovenous malformation, pancreatic lithiasis, aneurism rupture from adjacent vessels, or erosion of pancreatic and peripancreatic vessels due to chronic pancreatitis. The authors describe a case of massive and recurrent gastrointestinal upper hemorrhage in a 26-year-old man without known risk factors for pancreatitis. This man underwent urgent surgery due to gastrointestinal bleeding during the ínvestigation. During the procedure, blood was found in the intestinal lumen and a tumor in the head of pancreas with two centimeters of diameter: A gastroduodenopancreatectomy was performed. Histological study showed chronic pancreatitis with a fistula from the pancreatic vessels to the Wirsung duct. The patient was discharged without postoperative complications and after months, remains assymptomatic.

Anastomose duodenoduodenal na pancreatoduodenectomia por pancreatite crônica

Literature has been showing a tendency of reducing the limits of Whipple's resection. The main technical advance was the pylorus preserving resection, technique proposed by Traverso and Longmire in 1978. The pancreticoduodenectomy for chronic pancreatitis, is probably the best opportunity to apply this type of procedure. In these specific patients, the author preserved the pylorus and the third portion of the duodenum. The gastrointestinal transit was reconstructed by the duodenumduodenal anastomosis and the bile duct and the pancreas were drained in a Roux-en-way loop . Follow-up showed no important complication, with no problems related to gastric emptying and without pain.

Anomalia da via biliar extra-hepática

We show an anatomical modification of extrahepatic biliary ducts in a fifty-eight years old female who presented right hipocondric pain, jaundice and fever two months after cholecystectomy. The patient underwent ERCP wich showed an anatomical modification that consists in an union of the right and left hepatic ducts, with insertion into the cystic duct, right hepatic duct, being a choledochus agenesis.

Úlcera gástrica terebrante e pancreatite aguda provocadas por tricobezoar

Bezoar is a foreing body whitin the digestive tract originated from ingestion of varied substances, mainly vegetal fiber or hair. We present a case of a 14-year-old girl with trichotillomania, gastric trichobezoar, gastric ulcer and acute pancreatitis. The patient was operated on for anterior gastrotomy and removal of trichobezoar, with good postoperative follow-up. We illustrate this case to emphasize the need for recognition of gastric ulcer with acute pancreatitis and surgical management.

Pancreatite aguda biliar na infância

Acute pancreatitis is an uncommon condition in childhood. Gallstones rarely cause pancreatitis in children. Instead, the leading causes of pancreatitis tend to be trauma, infecctions, drugs, congenital disorders. One rare case of acute gallstone pancreatitis in children is described, showing the diagnosis clinical/radiologic and surgery treatment (videocolecistectomy).

Pseudotumor pancreático

The decrease in morbidity and mortality of pancreaticoduodenectomy in reference centers has increased the incidence of benign histopathologic diagnosis in cases where a pre-operative diagnosis of pancreatic ductal carcinoma was expected (9,2%). Recent reports have shown that autoimune pancreatitis, an entity that can lead to a pathologic diagnosis of sclerosing lymphoplasmatic pancreatitis has been the cause of about 2,5% of wrong preoperative diagnosis. Clinical and image definitive diagnosis is still uncertain on those borderline cases. An increase in IgG4 has been reported as frequent in patients with autoimune pancreatitis which can respond to steroid treatment. In doubtful cases, four diagnosis can be expected: 1. Pancreatic ductal carcinoma; 2. Chronic alcoholic pancreatitis; 3. "Chronic pancreatitis secondary to choledocal lithiasis"; 4. Sclerosing lymphoplasmatic pancreatitis. Modern medical literature sugests that evaluation of IgG4 in doubtful cases of pancreatic tumor (pseudotumor of the pancreas) could avoid unnecessary pancreaticoduodenectomies in the future.

Tratamento cirúrgico da hipertensão arterial secundária com origem na glândula supra-renal

Among the main etiologies of secondary arterial hypertension figure out the tumorous affections of adrenal gland, located on cortex - primary aldosteronism (Conn’s syndrome) and Cushing’s syndrome - or at glandular medulla - pheocromocytoma. Although these tumors are at most benign the surgical resection is needed in order to eliminate the disturbances provided by them and to limit the mass growth, being curative in about 80-90% of the cases. In this paper some particularities above surgical treatment of these diseases will be focused emphasizing the pre-operative prepare of the patients and the currently preconized approach.

Pancreatite aguda sem etiologia aparente: a microlitíase deve ser pesquisada?

In a large number of patients, with episodes of acute pancreatitis the etiology is not identified, even after initial clinical history, detailed physical examination, laboratory tests and biochemical exams and an transabdominal ultrasound. This patient are considered with a unexplained acute pancreatitis. In this cases the treatment is restricted to improvement of symptoms. These patients after treated tend to have new episodes with the risk of raising the rates of morbidity and mortality. Therefore, the identification of a cause and its prompt treatment prevent at recurrent episodes of pancreatitis. This review aims to draw attention to how best diagnostic approach when the light of evidence-based medicine, to search for causes of difficult identification with microlithiasis, occult stones, the anatomical variations of biliary and pancreatic duct and in addition to sphincter of Oddi dysfunction.

Temporary abdominal closure with zipper-mesh device for management of intra-abdominal sepsis

OBJECTIVE: to present our experience with scheduled reoperations in 15 patients with intra-abdominal sepsis. METHODS: we have applied a more effective technique consisting of temporary abdominal closure with a nylon mesh sheet containing a zipper. We performed reoperations in the operating room under general anesthesia at an average interval of 84 hours. The revision consisted of debridement of necrotic material and vigorous lavage of the involved peritoneal area. The mean age of patients was 38.7 years (range, 15 to 72 years); 11 patients were male, and four were female. RESULTS: forty percent of infections were due to necrotizing pancreatitis. Sixty percent were due to perforation of the intestinal viscus secondary to inflammation, vascular occlusion or trauma. We performed a total of 48 reoperations, an average of 3.2 surgeries per patient. The mesh-zipper device was left in place for an average of 13 days. An intestinal ostomy was present adjacent to the zipper in four patients and did not present a problem for patient management. Mortality was 26.6%. No fistulas resulted from this technique. When intra-abdominal disease was under control, the mesh-zipper device was removed, and the fascia was closed in all patients. In three patients, the wound was closed primarily, and in 12 it was allowed to close by secondary intent. Two patients developed hernia; one was incisional and one was in the drain incision. CONCLUSION: the planned reoperation for manual lavage and debridement of the abdomen through a nylon mesh-zipper combination was rapid, simple, and well-tolerated. It permitted effective management of severe septic peritonitis, easy wound care and primary closure of the abdominal wall.

Ca2+ signaling in pancreatic acinar cells: physiology and pathophysiology

The pancreatic acinar cell is a classical model for studies of secretion and signal transduction mechanisms. Because of the extensive endoplasmic reticulum and the large granular compartment, it has been possible - by direct measurements - to obtain considerable insights into intracellular Ca2+ handling under both normal and pathological conditions. Recent studies have also revealed important characteristics of stimulus-secretion coupling mechanisms in isolated human pancreatic acinar cells. The acinar cells are potentially dangerous because of the high intra-granular concentration of proteases, which become inappropriately activated in the human disease acute pancreatitis. This disease is due to toxic Ca2+ signals generated by excessive liberation of Ca2+ from both the endoplasmic reticulum and the secretory granules.

Hospital strain colonization by Staphylococcus epidermidis

The skin and mucous membranes of healthy subjects are colonized by strains of Staphylococcus epidermidis showing a high diversity of genomic DNA polymorphisms. Prolonged hospitalization and the use of invasive procedures promote changes in the microbiota with subsequent colonization by hospital strains. We report here a patient with prolonged hospitalization due to chronic pancreatitis who was treated with multiple antibiotics, invasive procedures and abdominal surgery. We studied the dynamics of skin colonization by S. epidermidis leading to the development of catheter-related infections and compared the genotypic profile of clinical and microbiota strains by pulsed field gel electrophoresis. During hospitalization, the normal S. epidermidis skin microbiota exhibiting a polymorphic genomic DNA profile was replaced with a hospital-acquired biofilm-producer S. epidermidis strain that subsequently caused repetitive catheter-related infections.