Anna Gunnarsdottir

Characterisation of CART-containing neurons and cells in the porcine pancreas, gastro-intestinal tract, adrenal and thyroid glands

BackgroundThe peptide CART is widely expressed in central and peripheral neurons, as well as in endocrine cells. Known peripheral sites of expression include the gastrointestinal (GI) tract, the pancreas, and the adrenal glands. In rodent pancreas CART is expressed both in islet endocrine cells and in nerve fibers, some of which innervate the islets. Recent data show that CART is a regulator of islet hormone secretion, and that CART null mutant mice have islet dysfunction. CART also effects GI motility, mainly via central routes. In addition, CART participates in the regulation of the hypothalamus-pituitary-adrenal-axis. We investigated CART expression in porcine pancreas, GI-tract, adrenal glands, and thyroid gland using immunocytochemistry.ResultsCART immunoreactive (IR) nerve cell bodies and fibers were numerous in pancreatic and enteric ganglia. The majority of these were also VIP IR. The finding of intrinsic CART containing neurons indicates that pancreatic and GI CART IR nerve fibers have an intrinsic origin. No CART IR endocrine cells were detected in the pancreas or in the GI tract. The adrenal medulla harboured numerous CART IR endocrine cells, most of which were adrenaline producing. In addition CART IR fibers were frequently seen in the adrenal cortex and capsule. The capsule also contained CART IR nerve cell bodies. The majority of the adrenal CART IR neuronal elements were also VIP IR. CART IR was also seen in a substantial proportion of the C-cells in the thyroid gland. The majority of these cells were also somatostatin IR, and/or 5-HT IR, and/or VIP IR.ConclusionCART is a major neuropeptide in intrinsic neurons of the porcine GI-tract and pancreas, a major constituent of adrenaline producing adrenomedullary cells, and a novel peptide of the thyroid C-cells. CART is suggested to be a regulatory peptide in the porcine pancreas, GI-tract, adrenal gland and thyroid.

Quality of Life in Adults Operated On for Hirschsprung Disease in Childhood.

Objectives: The aim of this study is to explore the long-term quality of life (QoL) in adults after surgery for Hirschsprung disease in childhood. Patients and Methods: Altogether 51 patients were operated for HD at our center during the period 1969 to 1989. In 2007, the validated instruments for assessing QoL, the SF-36 health survey and the Gastrointestinal Quality of Life Index (GIQLI), were sent to the 47 patients still alive at the time of the study. A retrospective review of the patient records was also done. Results: Forty-two patients returned both questionnaires (89% response rate) at the median age of 28.5 (range 18–45) years. The median clinical follow-up time was 5.7 years (range 5 months–23 years). At the last clinical control, 4 (9%) patients had a terminal enterostomy, 12 (29%) had soiling, 5 (12%) had constipation, and 2 (5%) experienced recurrent enterocolitis. In contrast to males, the subscores for females were lower for general health and mental health than for an age- and sex-matched general population (P < 0.05). Patients having aganglionosis to the right colon had lower GIQLI scores than those with aganglionosis to the left colon (P < 0.05). In multivariate linear regression analysis, female sex was the only factor associated with lower GIQLI score (P < 0.05). Conclusions: The long-term QoL of adults operated for Hirschsprung disease in their youth is satisfactory. Female scores were lower for general and mental health, compared with the matched control group. The study indicates that the longer the aganglionic segment, the greater its impact on QoL in later life.

Transanal Endorectal vs. Duhamel Pull-Through for Hirschsprung's Disease.

INTRODUCTION The aim of this study was to test the hypothesis that the early functional outcome for patients with rectosigmoid Hirschsprungs disease (HD) is comparable for the Duhamel pull-through procedure and the transanal endorectal pull-through (TERPT) procedure, with less discomfort for the patient postoperatively after the TERPT technique. MATERIAL AND METHODS Eleven patients operated on with the TERPT technique (T Group) were prospectively registered and compared retrospectively with 18 patients operated on with the Duhamel pull-through (D Group). Data recorded included patient demographics, operative treatment, complications, hospital stay and bowel functions. The follow-up time was limited to 24 months. RESULTS The T Group started oral feeding sooner, their bowel movements started sooner and they had less need for analgesia postoperatively and a significantly shorter hospital stay. 71% of the patients in the D Group needed re-intervention compared to only 18% of the T Group. Enterocolitis was seen in two patients in both groups. At the last clinical control ten patients had constipation (59%) and three had soiling (18%) in the D Group. Three patients in the T Group had constipation (27%) and one had soiling (9%). CONCLUSION Our results support the use of the TERPT method rather than the Duhamel pull-through for rectosigmoid HD.

Laparoscopic aided cholecystostomy as a treatment of inspissated bile syndrome.

We report a case of a newborn girl with inspissated bile syndrome (IBS) that did not respond to treatment with oral ursodeoxycholic acid (Ursofalk). A solution was found using laparoscopic aided cholecystostomy with an indwelling catheter for local Ursofalk flushing in the gallbladder and the choledochus. This is the first report of a laparoscopic aided management of IBS without cholecystectomy or exploration of the bile ducts. This minimal invasive approach showed a clear advantage for the patient. There were no complications. The method is recommended in the treatment of IBS.

Wireless Esophageal pH Monitoring in Children.

INTRODUCTION A wireless BRAVO (Medtronic, Shoreview, MN) capsule for pH measurement in the diagnosis of gastroesophageal reflux disease (GERD) is intended to be less uncomfortable, and facilitates activity during the measuring period, compared to the usual method with a naso-esophageal catheter. The aim of this study was to report on our experience with the wireless system in children. A secondary aim was to see if there was any cut-off level for esophageal acid exposure causing esophagitis as verified by pathologic examination. MATERIALS AND METHODS A total of 62 wireless 24-hour pH measurements with the BRAVO capsule were carried out over a period of 2 years in 58 children with symptoms of GERD. The median age of the children was 8 +/- 4 years (range, 1-15). They underwent upper endoscopies and the placement of the capsule under general anesthesia. Correlations between endoscope findings and pathologic diagnosis were done. RESULTS In 10 children, the endoscopies showed esophagitis. The median percent time of pH <4 was 7.0 +/- 9.6% (range, 0-61). The DeMeester score was abnormally high in 33 children. Three children described dysphagia during the measuring time. In 3 patients, we experienced technical problems with the wireless system. Biopsies were taken in 49 children, of which 18 showed esophagitis, with no correlation to the 24-hour pH-measurement findings. CONCLUSIONS Ambulatory pH monitoring, using the wireless pH system, is feasible and was well tolerated by the children. No cut-off level for the acid exposure in correlation to pathologic diagnosis of esophagitis could be found. We recommend the use of the wireless pH-measurement system in children.

Percutaneous Endoscopic Gastrostomy in Children: A Population-Based Study from Iceland, 1999–2010

AIM The aim of this study was to review the indications and the results of percutaneous endoscopic gastrostomy (PEG) procedures in Icelandic children. PATIENTS AND METHODS A retrospective review of all pediatric PEG procedures performed in Iceland in 1999-2010 was conducted. Diagnosis, demographics, complications, and body mass index were recorded. RESULTS Ninety-eight children (51 girls) were included. Median age was 2 years (range, 1 month-17 years). The most common diagnosis was neurological disease (56%). Median length of stay was 4 days (range, 1-189 days). Extended length of stay was not related to PEG. Before surgery, median body mass index (BMI) was 14.5 kg/m(2) (range, 9.8-20.8 kg/m(2)), and the median BMI-for-age z-score was -1.4 (range, -5.9 to 3.0). One year after surgery, median BMI was 15.3 kg/m(2) (range, 11.2-22.1 kg/m(2)), and median BMI-for-age z-score was -0.5 (range, -5.1 to 3.8). The median weight increased significantly in 1 year by 1.0 standard deviation (P<.0001; 95% confidence interval, -1.4820 to -0.7387). One hundred sixty-six complications were recorded in 65 children; 96% were minor, with the most common being granuloma formation (19%) and superficial skin infection (25%). The rate of infection was not statistically different between those who received preoperative antibiotics versus no antibiotics (P=.296). Major complications were peritonitis (n=3), esophageal tear (n=1), buried bumper (n=1), and malposition of the gastrostomy tube (n=1). Median follow-up was 47 months (range, 1-152 months). Fourteen children died (at 1 month to 3 years), but no deaths were related to PEG insertion. Twenty-seven children were without gastrostomy at follow-up. Twelve children (14%) underwent fundoplication later; 11 of them were neurologically impaired. CONCLUSIONS PEG is a safe technique with a high complication rate, but the majority of complications are minor and easily treatable. Gastrostomy is sometimes temporary. Enteral feeding results in significant weight gain in 1 year.

Congenital diaphragmatic hernia: Improved surgical results should influence abortion decision making

Aims: To compare surgical results for congenital diaphragmatic hernia (CDH) in two Scandinavian university hospitals and to evaluate the effects of abortions on the clinical profile of CDH in Iceland. Methods: A retrospective study including all CDH-cases in Iceland 1983–2002 and children referred to Lund University Hospital 1993–2002. Aborted fetuses with CDH from a nation-wide Icelandic abort-registry were also included. Results: In Iceland, 19 out of 23 children with CDH were diagnosed < 24 hours from delivery, one with associated anomalies. Eight fetuses were diagnosed prenatally and seven of them aborted, three having isolated CDH at autopsy. In Iceland, 15 of 18 children operated on survived surgery (83% operative survival). In Lund 28 children were treated with surgery, 23 of them diagnosed early after birth or prenatally. Four children did not survive surgery (86% operative survival) and 9 (31%) had associated anomalies. All the discharged children treated in Iceland and Lund are alive, 3–22 years postoperatively. Conclusion: CDH is a serious anomaly where morbidity and mortality is directly related to other associated anomalies and pulmonary hypoplasia. However, majority of CDH patients do not have other associated anomalies. In spite of improved surgical results (operative mortality < 20%), a large proportion of pregnancies complicated with CDH are terminated. We conclude that the improved survival rate after corrective surgery must be emphasized when giving information to parents regarding abortion of fetuses with a prenatally diagnosed CDH.