Diagnostic laparoscopy through the right lower abdominal incision following open appendectomy

BACKGROUND: Diagnostic laparoscopy through the right lower abdominal incision following open appendectomy for suspected acute appendicitis may help in making the correct diagnosis in the absence of pathology of the appendix. METHODS: Fourteen patients with a clinical diagnosis of acute appendicitis underwent diagnostic laparoscopy through the right lower quadrant incision after open appendectomy to exclude further pathology in the case of a noninflamed appendix. RESULTS: In 10 of the 14 patients, laparoscopy helped to correct the diagnosis. In two patients, the etiology of the acute right lower abdominal pain remained unclear. In two others, histological examination showed acute appendicitis despite a normal macroscopic appearance. CONCLUSIONS: Diagnostic laparoscopy through the right lower quadrant incision may help to correct the diagnosis in patients who are operated on for clinically acute appendicitis but in whom no acute appendicitis or other pathological findings are seen.     

A philosopher looks at neuroscience

A prospective multicenter observational trial was performed to assess the performance and clinical benefit of ultrasonography of the appendix in the routine clinical examination. Included in the study were 2280 patients with acute abdominal pain from 11 surgical departments in Germany and Austria. Ultrasonography of the appendix was performed in 870 (38%) of the patients (range 16-85%). The overall sensitivity of ultrasonography of the appendix was 55% (13-90%), the specificity 95% (range 82-100%), positive predictive value 81% (50-100%), and negative predictive value 85% (68-96%). With respect to single ultrasound scan findings, adequate sensitivity (44%) was achieved only with the target phenomen, not with the other criteria. There were no correlations between the ultrasound findings of the appendix and the diagnostic accuracy of the clinician, the negative appendectomy rate, or the perforated appendix rate. From the study it can be concluded that there is no proven clinical benefit of ultrasound scanning of the appendix in the routine clinical diagnosis.     

Drug-induced linear IgA disease: target antigens are heterogeneous

BACKGROUND: Colonic strictures represent an advanced stage of fibrosing colonopathy in patients with cystic fibrosis. AIMS: To clarify whether ultrasonography can identify patients with an early stage of fibrosing colonopathy and to determine clinical factors that influence bowel wall thickening. PATIENTS: Ninety patients with cystic fibrosis, median age 10 years, and 46 healthy controls, median age 13 years, were investigated. METHODS: Bowel wall thickness was measured by ultrasound in a prospective study. RESULTS: In cystic fibrosis, wall thickness of both small intestine and colon was significantly (p < 0.0001) higher than in controls; 81% of patients with cystic fibrosis had a maximum colon wall thickness at any site of 2 mm or more, a value that was never reached by controls. The maximum colon wall thickness was 6.5 mm. Bowel wall thickness was unchanged at re-examination after one year. There was no progression even with high dose pancreatic supplements. There was no association between bowel wall thickness and clinical features such as previous meconium ileus, intestinal resection, distal intestinal obstruction syndrome, abdominal pain, or pancreatic enzyme dose. CONCLUSIONS: There is genuine intestinal involvement in cystic fibrosis; in a few cases this could lead to fibrosing colonopathy.     

"Bet you I will!" Risk or experimental behavior during adolescence?

Abdominal cysts in girls are frequently observed by abdominal ultrasound (US) and are usually ovarian. In this case a cystic structure located in the right abdomen was seen in a female newborn without symptoms and was initially described as a possible ovarian cyst. Frequent US examinations showed an increase in volume and diameter, and temporary, recurring episodes of hyperbilirubinemia were observed. The US scans showed no relationship to the biliary tree. During a diagnostic laparoscopy, a cystic structure attached to the pyloric region was seen. A laparotomy revealed a cystic duplication of the stomach, which could be resected completely. The finding is discussed emphasizing the importance of clinical findings and diagnostic methods in the diagnosis and management of abdominal cystic masses in females.     

Functional and metabolic evaluation of the hypertrophied heart using MRI and 31P-MRS

Most patients with cystic fibrosis require oral administration of pancreatic enzymes to treat pancreatic insufficiency. Recent use of higher-strength enzyme preparations in large doses has been found to be associated with fibrotic strictures of the colon. We report a case of pancolonic fibrosis due to pancreatic enzyme use.     

Menstrual cycle and age: influence on parenchymal contrast medium enhancement in MR imaging of the breast

PURPOSE: To quantify how frequently mesenteric adenitis clinically mimics appendicitis and to determine its appearance at computed tomography (CT). MATERIALS AND METHODS: The medical records of 651 consecutive patients with an admission diagnosis of appendicitis were reviewed to determine how often mesenteric adenitis was the discharge diagnosis. The CT scans of a separate group of 18 patients with a discharge diagnosis of mesenteric adenitis were reviewed. These patients were part of a group of 100 consecutive patients prospectively evaluated with CT of the appendix for clinically suspected appendicitis. RESULTS: Fifty of 651 patients (7.7%) with an admission diagnosis of appendicitis had a discharge diagnosis of mesenteric adenitis. Mesenteric adenitis constituted 50 of the 252 (19.8%) discharge diagnoses other than appendicitis. All 18 CT scans of mesenteric adenitis showed three or more nodes that measured at least 5 mm in shortest axis clustered in the right lower quadrant, with a normal appendix identified. Eight patients had associated ileal or ileocecal wall thickening. CONCLUSION: Mesenteric adenitis is an important clinical mimic of appendicitis. It appears at CT as clustered, enlarged mesenteric lymph nodes with a normal appendix, and there may be associated ileitis or ileocolitis noted.     

Carcinoembryonic antigen induces signal transduction in Kupffer cells

OBJECTIVES: We compared the ability of preoperative abdominal computed tomography (CT) with that of preoperative abdominal ultrasonography (US) in predicting the extent of tumor growth and the curative resectability of gastric cancer. METHODS: Abdominal CT and US were done in 95 patients with gastric adeno-carcinoma. The radiologic findings were prospectively compared with surgical and pathologic findings. RESULTS: The sensitivities of abdominal CT and US in detecting the perigastric lymph node involvement were 26.6% and 20%, respectively. The sensitivity of abdominal CT in predicting the pancreatic invasion (60%) was better than that of abdominal US (20%). However, there were 6 false positive diagnosis of pancreatic invasion by abdominal CT; in contrast, there was no false positive diagnosis of pancreatic invasion by abdominal US. Of 95 patients who underwent surgical exploration, 14 were found to have unresectable tumors because of extragastric organ involvement and distant metastases. Abdominal CT falsely predicted 8 of 14 cases in which curative gastric resection was impossible as resectable. Moreover, abdominal CT falsely predicted 5 of 81 cases in which curative gastric resection was possible as unresectable. Abdominal US falsely predicted 12 of 14 cases in which curative surgery was impossible as resectable. CONCLUSION: Preoperative abdominal Ct and US did not accurately predict the tumor extent and the curative resectability of the gastric cancer. Especially, abdominal CT finding of pancreatic invasion by gastric cancer should not be a contraindication for surgical exploration.     

Nutritional benefits of neonatal screening for cystic fibrosis. Wisconsin Cystic Fibrosis Neonatal Screening Study Group

BACKGROUND: Many patients with cystic fibrosis are malnourished at the time of diagnosis. Whether newborn screening and early treatment may prevent the development of a nutritional deficiency is not known. METHODS: We compared the nutritional status of patients with cystic fibrosis identified by neonatal screening or by standard diagnostic methods. A total of 650,341 newborn infants were screened by measuring immunoreactive trypsinogen on dried blood spots (from April 1985 through June 1991) or by combining the trypsinogen test with DNA analysis (from July 1991 through June 1994). Of 325,171 infants assigned to an early-diagnosis group, cystic fibrosis was diagnosed in 74 infants, including 5 with negative screening tests. Excluding infants with meconium ileus, we evaluated nutritional status for up to 10 years by anthropometric and biochemical methods in 56 of the infants who received an early diagnosis and in 40 of the infants in whom the diagnosis was made by standard methods (the control group). Pancreatic insufficiency was managed with nutritional interventions that included high-calorie diets, pancreatic-enzyme therapy, and fat-soluble vitamin supplements. RESULTS: The diagnosis of cystic fibrosis was confirmed by a positive sweat test at a younger age in the early-diagnosis group than in the control group (mean age, 12 vs. 72 weeks). At the time of diagnosis, the early-diagnosis group had significantly higher height and weight percentiles and a higher head-circumference percentile (52nd, vs. 32nd in the control group; P=0.003). The early-diagnosis group also had significantly higher anthropometric indexes during the follow-up period, especially the children with pancreatic insufficiency and those who were homozygous for the deltaF508 mutation. CONCLUSIONS: Neonatal screening provides the opportunity to prevent malnutrition in infants with cystic fibrosis.     

Pancreatic tuberculosis in non-immunocompromised patients: reports of two cases, and a literature review

We present two cases of biopsy proven tuberculosis of the pancreas in non-immunocompromised patients diagnosed and treated in our unit within the last 14 years. The first case presented with abdominal pain and fever, and the second with iron deficiency anaemia and severe weight loss. In both cases abdominal ultrasound and computed tomography suggested a pancreatic carcinoma. There was no pulmonary or intestinal tuberculosis. The tuberculin skin test was positive. Upon exploratory laparotomy the macroscopic appearance of the pancreas was that of an inoperable pancreatic carcinoma. Following the histological diagnosis of pancreatic tuberculosis, both patients were successfully treated with triple antituberculous therapy for 6 months. Isolated pancreatic tuberculosis is an extremely rare disease with only 41 cases in non-immunocompromised patients reported worldwide (1966-1997). It is a curable disease and should be considered in the differential diagnosis of a pancreatic mass or abscess shown on ultrasound or computed tomography, especially in developing countries, where tuberculosis is common.     

The cystic fibrosis transmembrane conductance regulator as a marker of human pancreatic duct development

BACKGROUND & AIMS: The cystic fibrosis transmembrane conductance regulator (CFTR) protein is a small conductance adenosine 3',5'-cyclic monophosphate (cAMP)-activated chloride ion channel found in the apical membranes of epithelia within the pancreas, airway, intestine, bile duct, sweat gland, and male genital ducts. Pancreatic insufficiency is a feature of about 85% of patients with cystic fibrosis and is believed to be caused by pancreatic autolysis after pancreatic duct obstruction. The aim of this study was to investigate the expression of CFTR in the pancreas from early development to postnatal life to establish whether the CFTR plays a key role in development of the pancreatic duct epithelium. METHODS: Expression of CFTR from the start of the mid-trimester of human development through term to adult life by messenger RNA (mRNA) in situ hybridization was examined. RESULTS: CFTR mRNA is detected throughout the pancreatic duct epithelium and its pattern of expression follows the differentiation of the duct system. CONCLUSIONS: CFTR is a valuable marker of human pancreatic duct cell development and differentiation.     

Identification of the tumor antigen 90K domains recognized by monoclonal antibodies SP2 and L3 and preparation and characterization of novel anti-90K monoclonal antibodies

PURPOSE: To study the imaging findings in patients with systemic mastocytosis and to correlate the findings with the severity of disease on the basis of an established classification system. Pathologic findings, when available, were correlated with imaging findings. MATERIALS AND METHODS: Computed tomographic (CT) and ultrasound (US) scans and corresponding pathologic findings, when available, were retrospectively reviewed in 27 patients with systemic mastocytosis. RESULTS: Only five (19%) of the patients in our series had normal abdominal CT and/or US examination results. Common abdominal imaging findings associated with systemic mastocytosis were hepatosplenomegaly, retroperitoneal adenopathy, periportal adenopathy, mesenteric adenopathy, thickening of the omentum and the mesentery, and ascites. Less common findings included hepatofugal portal venous flow, Budd-Chiari syndrome, cavernous transformation of the portal vein, ovarian mass, and complications such as chloroma. The findings were more common in patients with category II and those with category III disease. CONCLUSION: Abdominal findings at CT and US are common in patients with systemic mastocytosis. Although the findings in patients with systemic mastocytosis are not specific to the disease, they are useful in directing further studies for diagnostic confirmation and in estimating the extent of systemic involvement.     

Invagination of the vermiform appendix. A report of two cases associated with endometriosis

Two cases of invagination of the Vermiform appendix associated with endometriosis are presented. Classification, incidence, etiology, symptomatology, diagnosis and treatment are discussed. The literature is reviewed in brief, and it is concluded that the possibility of invagination of the Vermiform appendix should be considered in obsure abdominal cases with recurrent right lower quadrant pain.     

The incidence of cataract in India is an overestimate

Color Doppler ultrasound has been reported to be useful in ruling out testicular torsion in children. Some investigators, however, have continued to report false-positive and false-negative studies. Herein we review our institution's experience over the past 6 years. From January 1990 to December 1996, over 300 boys 21 years of age or less underwent color Doppler ultrasound for testicular pain and/or swelling. Presentation, ultrasound diagnosis, operative findings (where applicable), and subsequent course were assessed. Complete data was available for 243 boys (average age 10.36 years, range 1 day to 21 years). Fourteen boys were explored without imaging based on strong clinical findings and short duration of pain. Of these, 13 had torsion and 1 had epididymitis. Torsion was ruled out by ultrasound in 182 with 100% specificity verified on follow-up. Forty-five diagnoses of torsion by ultrasound were confirmed operatively. Two patients with equivocal scans also were explored: one had torsion, whereas the other had a torsed appendix. Ultrasonic findings are characterized. Additionally, two cases of false-negative ultrasound from outside institutions are discussed. In conclusion, color Doppler ultrasound can identify reliably those children with an acute scrotum who require exploration and exclude those children without testis torsion who would otherwise undergo needless surgery.     

Pancreatic trauma: a rare but severe event

INTRODUCTION: High-speed motor vehicle accidents have contributed to an increasing incidence of pancreatic trauma. Early mortality is generally due to associated vascular and other intra-abdominal injury. MATERIALS AND METHODS: The authors report their experience on pancreatic trauma. Six cases were treated during the last five years. Isolated pancreatic injury was observed only in one case. Two patients received simple external drainage. Two patients required distal pancreatectomy. One patient was mistreated with ligature of distal pancreatic duct and, postoperatively, developed pseudocyst. One patient, with associated duodenal injury, died. COMMENTS: Serum amylase is often unreliable, whereas abdominal CT scans have the best sensitivity and specificity in detecting pancreatic injury. Abdominal exploration is always recommended in retroperitoneal trauma evaluation; complete pancreas mobilisation is needed to determine the integrity of the major pancreatic duct. Treatment protocols are based on severity of injury: they range from simple drainage to distal pancreatectomy (in presence of distal transection or parenchymal injury with duct injury) and to pancreaticoduodenectomy (in presence of massive injury, devascularisation, destruction of ampulla). Complications are seen in 20 to 35% of patients and the overall mortality secondary to complications ranges from 10 to 20%. Fistulas and pseudocysts are the most common complications.     

Peroxisome proliferator-activated receptor gamma activators inhibit gene expression and migration in human vascular smooth muscle cells

Mesenchymal hamartomas of the liver are the second most common benign liver tumor of childhood. The experience with this tumor at Egleston's Children Hospital at Emory University from 1989 to 1994 is reviewed. Eight patients presented with abdominal distention or an upper abdominal mass. Six patients presented at a mean age of 8 months, and two patients presented at 17 and 23 years of age, respectively. Four patients displayed normal alpha-fetoprotein levels, whereas one patient had an elevated level. Liver function studies were normal in all patients. Abdominal ultrasonography and CT scans revealed a cystic, septated mass within the liver or on a pedicle in all patients. Five patients had simple excision of the tumor, and two had major hepatic resections. The cysts were multiloculated and lined with cuboidal bile duct epithelium surrounded by stroma containing proliferating bile ducts, blood vessels, and compressed liver tissue with no calcifications. In one patient, some pathologists favored the diagnosis of malignant myxoid fibrous histiocytoma because of similar-appearing stroma. Follow-up (mean, 35 months) revealed one symptomatic recurrence after initial resection was incomplete. There were no other recurrences and no malignant transformations. A septated, noncalcified, cystic hepatic mass in an infant with normal liver function studies and characteristic ultrasound or CT is likely a benign mesenchymal hamartoma that can be cured by total local excision.     

Role of secondary prevention in congestive heart failure due to coronary artery disease

BACKGROUND: It is unknown whether genetic factors predispose patients to idiopathic pancreatitis. In patients with cystic fibrosis, mutations of the cystic fibrosis transmembrane conductance regulator (CFTR) gene typically cause pulmonary and pancreatic insufficiency while rarely causing pancreatitis. We examined whether idiopathic pancreatitis is associated with CFTR mutations in persons who do not have lung disease of cystic fibrosis. METHODS: We studied 27 patients (mean age at diagnosis, 36 years), 22 of whom were female, who had been referred for an evaluation of idiopathic pancreatitis. DNA was tested for 17 CFTR mutations and for the 5T allele in intron 8 of the CFTR gene. The 5T allele reduces the level of functional CFTR and is associated with an inherited form of infertility in males. Patients with two abnormal CFTR alleles were further evaluated for unrecognized cystic fibrosis-related lung disease, and both base-line and CFTR-mediated ion transport were measured in the nasal mucosa. RESULTS: Ten patients with idiopathic chronic pancreatitis (37 percent) had at least one abnormal CFTR allele. Eight CFTR mutations were detected (prevalence ratio, 11:1; 95 percent confidence interval, 5 to 23; P<0.001). In three patients both alleles were affected (prevalence ratio, 80:1; 95 percent confidence interval, 17 to 379; P<0.001). These three patients did not have lung disease typical of cystic fibrosis on the basis of sweat testing, spirometry, or base-line nasal potential-difference measurements. Nonetheless, each had abnormal nasal cyclic AMP-mediated chloride transport. CONCLUSION: In a group of patients referred for evaluation of idiopathic pancreatitis, there was a strong association between mutations in the CFTR gene and pancreatitis. The abnormal CFTR genotypes in these patients with pancreatitis resemble those associated with male infertility.     

Gender gap in cystic fibrosis mortality

The authors conducted the largest study to date of survival in cystic fibrosis. The study cohort consisted of all patients with cystic fibrosis seen at Cystic Fibrosis Foundation-accredited care centers in the United States between 1988 and 1992 (n = 21,047), or approximately 85% of all US patients diagnosed with cystic fibrosis. Cox proportional hazards regression analysis was used to compare the age-specific mortality rates of males and females and to identify risk factors serving as potential explanatory variables for the gender-related difference in survival. Among the subjects 1-20 years of age, females were 60% more likely to die than males (relative risk = 1.6, 95% confidence interval 1.4-1.8). Outside this age range, male and female survival rates were not significantly different. The median survival for females was 25.3 years and for males was 28.4 years. Nutritional status, pulmonary function, and airway microbiology at a given age were strong predictors of mortality at subsequent ages. Nonetheless, differences between the genders in these parameters, as well as pancreatic insufficiency, age at diagnosis, mode of presentation, and race, could not account for the poorer survival among females. Even after adjustment for all these potential risk factors, females in the age range 1-20 years remained at greater risk for death (relative risk = 1.6, 95% confidence interval 1.2-2.1). The authors concluded that in 1- to 20-year-old individuals with cystic fibrosis, survival in females was poorer than in males. This "gender gap" was not explained by a wide variety of potential risk factors.     

False-positive gliadin and endomysium antibodies and exocrine pancreatic insufficiency as pitfalls in the differential diagnosis of duodenal Crohn's versus celiac disease

We report a 13-yr-old boy with Crohn's disease in the upper gastrointestinal tract presenting with abdominal pain, failure to thrive, recurrent fever, iron-deficient anemia, and exocrine pancreatic insufficiency. Initially, latent celiac disease was suggested because of normal endoscopic findings, the finding of non-specific inflammation on histological evaluation of duodenal biopsies, positive IgA and IgG gliadin, as well as endomysium antibodies and exocrine pancreatic insufficiency. There was no response to a gluten-free diet. A reevaluation revealed Crohn's disease.     

More informative abstracts revisited

Recent progress in understanding the luminal biochemistry of regulated pancreatic exocrine secretion, including acid-base interactions between acinar and duct cells and pH-dependent processes that regulate membrane trafficking (endocytosis) at the apical plasma membrane, have led to the development of in vitro models of cystic fibrosis in the rat exocrine pancreas. Based on investigations in these model systems, a unifying hypothesis is presented that proposes that pancreatic dysfunction in cystic fibrosis occurs as a result of progressive acidification of the acinar and duct lumen, which leads to secondary defects in (i) apical trafficking of zymogen granule membranes and (ii) solubilization of secretory (pro)enzymes. By directly acidifying the pH of the acinar lumen in cholescystokinin-stimulated acini, the early cytological findings observed in cystic fibrosis, including (i) massive dilatation of the acinar lumen, (ii) decreased appearance of zymogen granules, (iii) loss of the apical pole of the acinar cell, and (iv) persistent aggregation of secretory (pro)enzymes released into the luminal space, have been reproduced in primary cultures of pancreatic tissue.     

Congenital dilatation of the bile ducts

Our experience with five cases of cystic dilatation of the extrahepatic bile ducts is reported and compared with the literature. The following conclusions have been reached: (1) The etiology of this anomaly is primarily congenital but may involve an acquired component. (2) Diagnosis should be suspected when any of the triad of abdominal pain, right upper quadrant mass, or jaundice is present. (3) The diagnosis can usually be made in infants based on the clinical picture and routine radiologic studies. (4) Retrograde cholangiopancreatography is a useful tool in making the diagnosis in older children and adults. (5) Roux-en-Y choledochocystojejunostomy is the procedure of choice for type I cysts, excision for type II, and choledochocystoduodenostomy for type III.